XXVI Congress of the IAP: Abstracts 179 transplant nephrectomies in which extensive infarcts are present. hemangioma and locally had DT-like appearance, as in the fi rst lesion. About 10 months after the second resection, another recurrence took place. This time, apart from the Soft Tissue primary tumor, CT examination demonstrated metastases in the lungs. Histopathological examination of the excised primary tumor showed locally persistent traits of DT, as well as evident angiosarcoma with infi ltration of soft tissue and muscle of the buttock. The patient 824 A CASE REPORT: ANGIOSARCOMA ARISING IN AN A-V FISTULA SITE died three years after having been fi rst diagnosed with DT. IN A RENAL TRANSPLANT RECIPIENT Conclusion: There remains some controversy regarding DT’s malignancy though currently Alia Albawardi; Atilla Omeroglu, McGill University, Montreal, QC, Canada it is more and more often classifi ed as a tumor of intermediate biologic malignancy. Complete Background: Angiosarcoma is a rare malignancy of endothelial origin, comprising < 1% surgical excision is the treatment of choice. Prognosis is good following complete surgical of all sarcomas. Angiosarcoma can occur at any site of the body with a predilection for the excision of the primary lesion, although spread to regional lymph nodes is possible. Our skin and soft tissue of adults. Predisposing factors include chronic lymphedema, prolonged case proves that DT, once regarded as a low-grade lesion has the potential to transform into immunosuppression, certain chemicals and possibly viruses. Renal transplant patients have angiosarcoma which makes it a tumor of intermediate malignancy. Therefore, the Dabska an increased risk for developing angiosarcoma due to both immunosuppression and chronic Tumor should never be overlooked. lymphedema. In this patient population approximately 50% of angiosarcomas arise at the site of arterio-venous fi stula. Here we present a case of an epithelioid angiosarcoma in a 827 SUPERFICIAL ANGIOMYXOMA OF THE TONGUE renal transplant patient arising in a defunctionalized arterio-venous fi stula site, clinically Sharon Bihlmeyer; Lisa Cole; Masotoshi Kida, University of Vermont/Fletcher Allen mimicking a pseudo-aneurysm formation. Health Care, Burlington, VT, United States Case report: A 58-year-old renal transplant male presented with progressive swelling, Background: Superfi cial angiomyxomas are poorly recognized soft tissue tumors typically redness and pain at the arterio-venous fi stula site in the left elbow. The patient was located on the head and neck, trunk and lower limbs. These benign tumors have a high risk transplanted 8 years ago because of chronic renal failure secondary to diabetes mellitus of local recurrence with no reports of metastatic potential. Only 2 intra-oral cases have been type 2 and hypertension. The patient was maintained on immunosuppressive agents. reported in the English literature, on the buccal mucosa and on the fl oor of the mouth. The swelling at the arterio-venous fi stula was thought to represent a hematoma with Design: We will report the fi rst case on a superfi cial angiomyxoma arising in the tongue superimposed infection. A surgical exploration and excision of the lesion was performed. along with a review of the literature. Due to profuse bleeding during the operation a pseudo-aneurysm formation at the fi stula Results: A 62-year-old female presented to the Otolaryngology clinic with a 12-month site was suspected. The histological examination of the lesion revealed an epithelioid history of a persistent `sore` on her tongue. Examination revealed a 3x4 mm area of thickened angiosarcoma. The patient’s clinical condition suddenly deteriorated. A chest CT showed epithelium on the tip of the tongue with some associated induration. An excisional biopsy several pleural masses consistent with metastasis. In an attempt to confi rm the diagnosis was performed revealing the histopathology of a superfi cial angiomyxoma. Histologic an US guided transthoracic lung biopsy was attempted. The procedure was complicated by examination revealed a biopsy of unremarkable mucosa. Within the submucosa was a massive hemothorax. The patient expired secondary to this complication. myxoid lesion composed of numerous small blood vessels, acellular mucin pools, stellate Conclusion: There is a predilection of angiosarcomas to arise at arterio-venous fi stula sites and bipolar fi broblastic cells, muciphages and a sparse mixed infl ammatory cell infi ltrate. in renal transplant patients. Those patients should be followed-up and changes in arterio- Conclusion: This is the fi rst reported case in the English literature of superfi cial venous sites should be carefully examined to rule out the possibility of an angiosarcoma. angiomyxoma involving the tongue. Angiosarcomas arising in the setting of old AV fi stulas are highly aggressive. Hence early diagnosis is of utmost importance. 828 EXPRESSION OF K-2, A NEW MARKER OF ADIPOCYTES AND LIPOBLASTS, IN PLEOMORPHIC HIGH GRADE SARCOMA 825 AN UNUSUAL SITE OF PLEOMORPHIC LIPOMA: A CASE REPORT Jennifer Black; Jae Ro, The Methodist Hospital, Houston, TX, United States; Fan Lin, Adham Ammar; Jenish Patel; Martin B. Gillett, Department of Histopathology, Arrowe Geisinger Health System, Danville, PA, United States; Alberto Ayala; Jim Zhai, The Park Hospital, Wirral, United Kingdom Methodist Hospital, Houston, TX, United States Background: Although, lipoma is the most common primary benign paratesticular Background: Pleomorphic high grade sarcomas (PHGS) have usually been diagnosed as neoplasm and the most common tumour of the spermatic cord, the pleomorphic lipoma malignant fi brous histiocytoma (MFH) in cases of high grade sarcomas where no clear is a rare tumour in this site, which histologically may resemble a well differentiated differentiation is identifi ed. This diagnosis is confi rmed by negative immunoperoxidase liposarcoma. We present a case of pleomorphic lipoma in the paratesticular tissue, which to staining for epithelial, mesenchymal, hematopoietic and melanocytic markers. K-2, a our knowledge, there have been no previous cases reported of patients with paratesticular clone of Ki-67, demonstrates a dual staining pattern: a nuclear staining pattern can be pleomorphic lipoma in the English-language literature. The differential diagnosis and used as a proliferation index (essentially identical to Mib-1), while a cytoplasmic staining the relationship between pleomorphic lipoma and recurrent giant lipoma are briefl y pattern is sensitive and specifi c for mature adipocytes and lipoblasts. Identifi cation of discussed. subtle lipogenic differentiation within MFH using K-2 would provide more accurate Design: A case report and review of literature. A 57 year old man, presented with 9 month diagnostic information. history of right scrotal mass. Previous resection of a giant lipoma of the right epididymis Design: Thirty-four cases of PHGS were studied using immunoperoxidase staining against was also done three years ago. An ultrasound scan revealed a large complex solid mass K-2 and S-100 protein. The staining pattern and density were recorded. occupying the right scrotum but it was not possible to determine the exact site. Right Results: Of 34 cases, 4 cases of PHGS showed focal cytoplasmic staining and 3 cases orchidectomy was carried out. revealed patchy/multifocal cytoplasmic staining pattern, for a total of 7 cases (21%) Result: The lesion is characterized by bizarre multinucleated cells in a background of mature demonstrating a possible lipogenic differentiation. S-100 stained only mature adipocytes adipose tissue and loosely textured collagen. The nuclear chromatin has a characteristic in 6 cases, adjacent to benign adipose tissue. These mature adipocytes, also stained with smudge appearance. Floret cells with ring nuclei are also present. Immunohistochemically, K-2, may represent entrapment of mature adipocytes by malignant cells rather than true the tumour cells stained positively with CD34 and were negative for S-100 protein. tumor lipogenic differentiation. Nuclear staining was positive diffusely in 14 cases, with Conclusion: The unusual benign pattern of differentiation in this tumour can present a patchy positivity in 8 cases and focal positivity in 10 cases. diagnostic challenge and the unusual site is also of clinical and radiological dilemma. Conclusions: 1) Bubbly cytoplasmic staining with K-2 in allomorphic cells suggests Therefore, establishment of accurate diagnosis is of crucial importance to avoid unnecessary lipogenic differentiation; 2) S-100 demonstrates membranous staining only in mature radical surgery. adipocytes; 3) In our study, 21% of PHGS, diagnosed as MFH previously, are positive for K-2, indicating lipogenic differentiation; 4) K-2 positive PHGS may represent cases 826 ANGIOSARCOMA ARISING WITHIN A MALIGNANT ENDOVASCULAR of pleomorphic liposarcoma, however, this hypothesis requires clinical validation; 5) K-2, PAPILLARY ANGIOENDOTHELIOMA (DABSKA TUMOR) as a novel marker for lipogenic differentiation, should be added to the immuno-panel in Zbigniew Antosz, District Specialist Hospital, Tychy, Poland; Maciej Zaniewski; Jacek evaluating cases of PHGS. Kostecki, Department of Surgery, Medical University of Silesia, District Specialist Hospital, Tychy, Poland 829 EXPRESSION OF EPIDERMAL GROWTH FACTOR RECEPTOR (EGFR) Background: The Dabska tumor is a rare vascular neoplasm also known as malignant IN SYNOVIAL SARCOMA endovascular papillary angioendothelioma. There have been 31 cases reported in literature Dariusz Borys, NYU School of Medicine, New York, NY, United States; Jerome B. Taxy, to this day. DT shows some traits of malignancy like increased mitotic activity, focal University of Chicago, Chicago, IL, United States; Herman Yee; Roberto Garcia, NYU necrosis and potential for metastazing (most frequently to regional lymph nodes), but School of Medicine, New York, NY, United States; Andre Kajdascy Balla; Nick Luna, because of its relatively good long-term prognosis the lesion was often regarded as a low Univeristy of Illinois at Chicago, Chicago, IL, United States; Thomas Krausz, University grade vascular tumor. of Chicago, Chicago, IL, United States; M. Alba Greco, NYU School of Medicine, New Design: We report an angiosarcoma arising within a malignant endovascular papillary York, NY, United States angioendothelioma (Dabska tumor) in soft tissue of the upper thigh/buttock of a 42-year-old Background: Synovial sarcomas are aggressive soft tissue neoplasms often characterized woman. Although neoplastic progression within a vascular tumor of an existing low-grade by monophasic and biphasic spindle and epithelioid cell morphology. Despite advances lesion into DT has been described so far, we seem to be the fi rst to report transformation in the therapy of local disease, distant metastasis remains the predominant cause of death. of DT into an angiosarcoma. The case we are reporting is, to our knowledge, the fi rst one Accordingly, there is a need for alternate therapies, such as those recently developed in which transformation of DT into angiosarcoma with accompanying pulmonary and liver against the tyrosine kinase receptors, like epidermal growth factor receptor (EGFR) and metastases has been confi rmed. HER-2/neu. Present study was done to determinate the frequency of expression of these Results: A 42-year old female was admitted to the Department of General and Vascular two markers in synovial sarcoma. Surgery in our hospital for elective surgical treatment of a tumor in the left buttock area Design: Archival paraffi n blocks of synovial sarcoma cases (n=16) were used for which had fi rst been noticed by the patient about 5 years earlier. Histological picture of the tissue array to assess for EGFR and HER-2/neu protein expression by standard excised lesion showed both ordinary hemangioma and, locally, traits suggesting Dabska immunohistochemical techniques. Tumor. After 8 months, because of local recurrence, the patient underwent consecutive Results: The age of the patients ranged from 12 to 55 (mean 38). 10 patients had tumor resection at another center. Histological picture then was consistent with ordinary 180 XXVI Congress of the IAP: Abstracts monophasic and 6 biphasic synovial sarcomas. 5 patients had pulmonary metastasis; 1 had Score 2: Other types a local recurrence in the primary site. In 13/16 ( 81% ) cases EGFR immunoreactivity was • Tumor longest dimension: detected as strong membrane pattern. In biphasic synovial sarcoma EGFR was strongly Score 1: < 5 cm expressed in spindle component, and it was negative in the epithelial component. In Score 2: > 5 cm. monophasic synovial sarcoma type EGFR was strogly expressed in spindle component. • Tumor depth*: 5 / 5 patients with pulmonary metastasis, and 1/1 patient with local recurrence had strong Score 1: Superfi cial EGFR membranous expression. In contrast, HER2 immunostaining was negative in 14/16 Score 2: Deep (87%) cases. Only two cases of biphasic type showed focal weak cytoplasmic HER-2/neu • Tumor histological grade (according to FFCCSG system): expression in the epithelial component. Score 1: Grade I Conclusion: Strong expression of EGFR in the majority of cases of synovial sarcoma, Score 2: Grade II and all cases with metastatic and recurrent disease, suggest that immunotherapy using Score 3: Grade III tyrosinase kinase inhibitors may represent a therapeutic alternative for these patients. • Tumor metastases (LN or organ metastases) Score 0: Absent 830 OSTEOCHONDRAL JUNCTION ALTERATION IN AUTOPSIED Score 6: Present FETUSES Conclusion: The suggested soft tissue prognostic index (STS-PI) for the studied cases was Rogério Canno; Tarcisio Evangelista; Ana karina Salge; Renata Santos, Triângulo Mineiro calculated by summing up the previous scores relevant to each tumor and was found and Federal University, Uberba. Brazil; Luis Cesar Peres, Ribeirão Preto Medical School, São described as 4 CLASSES as follows: Paulo, Brazil; Vicente Teixeira Eumenia Castro, Triângulo Mineiro Federal University, -CLASS A : 4 (best score) Uberaba, Brazil -CLASS B : 5, 6, 7 (good score) Background: Despite of the alterations of the osteochondral junction (OCJ) were frequent -CLASS C : 8, 9 (medium score) in pediatric autopsies there are still doubts remaining about the contribution of each -CLASS D : 10 – 15 (poor score) layer of the growth zone of the cartilage to the alterations found in this organ and what The 5 years survival rate for the studied tumors was recorded in 7/8 CLASS A tumors are the intrauterine conditions that are associated with these lesions. Objective: Identify (87.5%), in 30/44 of CLASS B tumors (68.2 %) , and in 17/59 CLASS C tumors (28.8%) the contribution of each growth zone of the cartilage to the lesions found in the OCJ of and was 0/11 in class D tumors (0 %), pointing to a reliable STS-PI. autopsied stillborn and neonates and its association with the cause of death. Design: It was utilized 96 ribs from autopsied stillborn and neonates. For identifi cation and 832 BETA-CATENIN EXPRESSION IN EMBRYONAL classifi cation of OCJ alterations it was utilized the Hematoxilin-Eosin stain. The Masson RHABDOMYOSARCOMA Blue Trichromic method was used for the morphometrical analysis of the extracelular L.-M. Giroux; K. Jacob; S. Barrette, CHU Sainte-Justine, Montréal, QC, Canada; Jean- matrix. From the autopsied reports were collected information regarding the gender, Christophe Fournet, CHU Sainte-Justine, Department of Pathology, Montréal, QC, gestacional age , antropometrical measurements and causes of death. All of the cases Canada were classifi ed in growth curves according to the gestational age and those below the 10 Background: Beta-catenin, encoded by CTNNB1 gene, is a key protein that links the percentile, were diagnostic as intrauterine growth restricted babies. E-cadherin adhesion complex to the cytoskeleton and transcription signaling through Results: Of the 98 cases analyzed, 74 infants were premature (77%). The morphologic TCF4 gene. Among many tumors, beta-catenin overexpression and mutations have been alterations of the OCJ were classifi ed in the following groups: 63 cases (65.6%) normal described in two pediatric tumors with anomalies of the chromosomal region 11p15 and (N), 9 cases (9.3%) increase of matrix cartilage (MC), 16 cases (16.6%) increase of matrix deregulation of genes with parental imprinting: Wilms tumor and hepatoblastoma. We cartilage and presence bone marrow cells in the free bone marrow growth zone (MCE), have tested beta-catenin anomalies of expression in embryonal rhabdomyosarcoma, a third and 6 cases (6.2%) increase of matrix cartilage, presence bone marrow cells in the free pediatric tumor linked to 11p15 region anomalies. bone marrow growth zone and bone formation near to the cartilage (MCEP) and 2 cases Design: We selected 21 embryonal rhabdomyosarcoma from our archives. Slides from (2%) classifi ed as bizarre (B) due to the gross disorganization of the growth zone layers. formalin-fi xed, paraffi n-embedded tumor samples were immunostained with a monoclonal The cases with congenital malformation, 32 (30.7%), presented the most severe alterations anti-beta-catenin antibody (Becton-Dickinson). Immunochemistry was performed by of the OCJ (46.2%). The cases with MCEP presented the biggest gestacional age (H = the avidin-biotin technique and the signal was revealed by immunoperoxydase reaction. 135.024; p = <0.001). From the growth layers, the proliferativa layer was not altered (P = Membranous, cytoplasmic and Nuclear staining was assessed. 0.136), however the hyperplastic layer was decrease in the MCE; MCEP and B groups. Results: 16 of 21 tumors showed a positive signal (73%). Cellular immunolabelling was Conclusion: Contrary to what is commonly believed in the literature the lesions of the diffuse in 13 cases, focal in 2, interspersed in 1. It was specifi c of tumoral cells and no OCJ do not present a relation with the intrauterine growth restriction. In our study it seems immumolabelling of tumoral stroma was found. The signal was weak in 1 case , moderate to occurred in association with congenital malformation, being maybe, a consequence of in 4, and strong in 11. The localization of the beta-catenin protein was membranous in 2 the same defect that leads to the malformed syndrome. Regarding the lesion ethiology the cases (10%) and/or cytoplasmic in 11 cases (52%) and/or nuclear in 5 cases (24%). decrease of the hyperplastic zone, that is the responsible for the matrix cartilage production, Conclusion: Embryonal rhabdomyosarcoma is a new tumor with beta-catenin seems to indicate that an alteration in the output of cartilage is the responsible for the found overexpression suggesting that the deregulation of the Wnt pathway could play a role in lesions (Financial support: FAPEMIG, CNPq e FUNEPU). rhabdomyosarcoma pathway of tumorigenesis. Interestingly, a nuclear translocation of the protein in tumoral cells nucleus was noted in a signifi cant proportion of tumors raising the 831 A SUGGESTED SOFT TISSUE SARCOMA – PROGNOSTIC INDEX (STS- possibility of CTNNB1 gene mutation in that tumoral model. PI) Ali El Hindawi, Pathology Department Faculty of Medicine. Cairo University, Cairo, Egypt; 833 ELASTOFIBROMA OF THE FOOT: AN UNUSUAL TUMOUR AT AN Maha Akl, Pathology Department, Theodor Bilharz Research Institute, Cairo, Egypt UNUSUAL SITE Background: The currently used grading methods for soft tissue tumors are mainly James Harrison; Alec Howat; Abdul Al-Dawoud, East Lancashire Hospital NHS Trust, dependant on morphological criteria which has the drawback of ignoring other important Burnley, United Kingdom criteria as specifi c microscopic types and depth of the tumor. Background: Elastofi broma is a benign pseudotumour almost exclusively occurring in Design: This study was carried out on 122 patients suffering from different types of soft the region of the shoulder girdle, fi rst described by Jarvi and Saxen in 1961. It usually tissue sarcomas and ranging in their ages from 17 to 72 years. They were admitted to presents as a painless mass. Several reports have described identical lesions in a variety `Aswan Cancer Institute` in Egypt for surgical resection of their tumors. The resected of unusual sites including the foot where only three previous cases in the foot have been specimens were proved to be soft tissue sarcomas of various microscopic types with reported in the English literature. Other tumour sites include the mouth, gastrointestinal a minimum follow up period of 60 months (5 years) . The aim of our study is to fi nd tract, omentum, thigh and the hand. These are very rare and unusual. out a reliable scoring system for grading and staging of such sarcomatous tumors that Design: We present this case report of an elastofi broma in an unusual location. could refl ect a convenient clinco-pathological prognostic index. Following proper gross Results: A 63 year old woman presented with a three month history of pain in the foot. morphological, histopathological and immunohistochemical diagnosis of the specimens There was no history of abnormal physical activity or of injury involving that foot. The under the study, they were given scores according to the following fi ve parameters, as pain was localised to an area between the second and third metatarsal with separation of follows: (a) According to Tumor microscopic type: Score 1 indicates favorable prognosis the second and third toes. Examination revealed exquisite tenderness in the second web (as in dermatofi brosarcoma protuberans) and Score 2: for other types of sarcomas.( b) space with altered sensation of the web space distally. The clinical diagnosis of Mortons According to Tumor longest dimension: Score 1: for tumors < 5 cm and Score 2: for tumors metatarsalgia/ neuroma was made and she proceeded to excision with decompression of > 5 cm. (c) According to Tumor depth: Score 1: for Superfi cial tumors and Score 2: for the interdigital nerve. Pathological appearance- We received a fi rm white rubbery mass Deep tumors. (d) As regards Tumor histological grade, according to the French Federation measuring 3.5x1.5x1cm. Histology showed the typical features of an elastofi broma with of Cancer Centers Sarcoma Groups (FFCCSG system) : tumors were given three scores; brightly eosinophilic fi bres scattered throughout a fi brous collagenous background. Elastic Score 1: for Grade I tumors (well differentiated), Score 2: for Grade II tumors(moderately stains confi rmed these fi bres to be composed of elastin. differentiated) and Score 3: for Grade III tumors (well differentiated). Grading is best Conclusions: Elastofi broma occurs most commonly after 50 years of age with done in relation to histological morphological criteria including ;(degree of cellularity, predominance in women and occurs almost exclusively in the subscapular region with a pleomorphism, mitosis and necrosis), and fi nally the last parameter is (e) According to the few reports of the tumour in other sites. Only three previous cases in the foot have been presence or absence of Tumor metastases (LN or organ metastases); tumors were given reported in the English literature. It is thought to be a reactive process possibly following either Score 0: for absent metastases and Score 6: when metastases are present. minor tissue injury or trauma. It is not a neoplastic process. Some reports have shown some Results: So our suggested STS-PI is as follows : familial clustering. It is reported to rarely cause pain or tenderness. This case highlights So our suggested STS-PI is as follows : that elastofi broma should be considered in the differential diagnosis of fi brous ‘tumours’ • Tumor microscopic type: from virtually any site. It may mimic other more common lesions. Surgical removal results Score 1: Favorable in a cure with only rare reports of local recurrence. XXVI Congress of the IAP: Abstracts 181 834 IMMUNOSTAINING FOR SYT PROTEIN DISCRIMINATES SYNOVIAL 836 EXPRESSION OF ERBB2 AND BCL-2 IN SYNOVIAL SARCOMA SARCOMA FROM OTHER SOFT TISSUE TUMORS: ANALYSIS OF 146 CASES Lenka Krsková; Irena Hilská; Alena Augustiňáková; Markéta Kalinová; Marcela Rui He, Department of Pathology, Loyola University Medical Center, Maywood, IL, United Mrhalová; Marie Neradová; Roman Kodet, Department of Pathology and Molecular States; Rajiv Patel, Department of Pathology, Emory University, Atlanta, GA, United Medicine, 2nd Faculty of Medicine, Charles University, Prague, Czech Republic States; Sarhan Alkan; Rasheed Hammadeh, Department of Pathology, Loyola University Background: Synovial sarcoma (SS) is a rare soft-tissue tumor that effect children and Medical Center, Maywood, IL, United States; Sharon Weiss, Department of Pathology, young adults. It is characterized by chromosomal translocation t(X;18)(p11.2;q11.2), which Emory University, Atlanda, GA, United States; John Goldblum, Division of Pathology and results in a fusion of the SYT gene on chromosome 18 and with SSX genes on chromosome Laboratory Medicine, The Cleveland Clinic Foundation, Cleveland, OH, United States; X. Despite advances in the therapy of a local disease, distant metastases remain a dominant Girish Venkataraman; Horea Baila, Department of Pathology, Loyola University Medical cause of death. Therefore, there is a need for alternative therapies, targeting receptor Center, Maywood, IL, United States tyrosine kinases – ERBB-2 and against cell cycle regulators - for example BCL-2. Background: Synovial sarcoma (SS) in classic biphasic form can be distinguished Design: Archival specimens of synovial sarcomas (n=25) were assessed for BCL-2 and readily from other soft tissue lesions; however, monophasic and poorly differentiated ERBB-2 protein expression by standard immunohistochemical techniques (IHC). To forms are diagnostically more problematic. For this reason, we have assessed effi cacy of validate the results of IHC, real-time quantitative reverse transcriptase polymerase chain immunostaining for SYT and SSX1 proteins, the gene products resulting from unique SS reaction (RQ-RT-PCR) assays using fresh and/or paraffi n sections was performed. The translocation, to distinguish SS from other soft tissue lesions. presence of amplifi cation of ERBB2 was determined by fl uorescence in-situ hybridization. Design: Cases included 45 SS (all of which were verifi ed by FISH to have t(X;18) The results of IHC were assessed as diffuse and strong positivity (D3+), diffuse and translocation and SYT-SSX fusion gene) and 101 soft tissue tumors of various types. Two moderate (D2+), diffuse and weak (D1+) and focal positivity (F1+ - 2+). antibodies were employed: 1) a polyclonal IgG antibody to SYT and 2) SSX1 antibody to Results: BCL-2 protein was detected by immunohistochemistry in 18/18 (100 %) – 3 cases stain formalin-fi xed paraffi n embedded tissues. with F1+-D1+, 10 cases with D2+ and 5 with D3+ positivity. ERBB-2 protein was detected Results: 39 out of 45 (87%) SS cases displayed strong positive nuclear staining (ranging in 9/18 (50 %) synovial specimens, but only with a weak focal positivity. Coexpression from 80% to 90% of the tumor cells) for SYT (N-18) antibody. 10 SS cases demonstrated of BCL-2 and ERBB-2 molecules was observed in 9/18 (50 %) synovial sarcomas. We strong positive nuclear staining for SSX1(N-18) antibody (ranging from 80% to 90%). 19 observed a mirror pattern in immunoreactivity of BCL-2 in biphasic synovial sarcomas. out of 101 (19%) non-SS cases showed variable nuclear and cytoplasmic staining with The positivity of epitheloid cells and negativity or a very weak positivity of spindle cells SYT (N-18): MPNST: 5 out of 25 cases with 20-60% nuclear stain; SFT: 2 out of 14 cases was detected in 4 cases, although in one the positivity of spindle cells with negative with 30% nuclear stain; EWS: 2 out of 6 cases with 40% nuclear stain; LGFMS: 2 out of epitheloid cells was observed. RQ-RT-PCR demonstrated a massive content of mRNA for 4 cases with 30% nuclear stain; ESMChS: 2 out of 6 cases with 30% nuclear stain; GIST BCL-2 in 14/25 (56 %) cases, moderate in 7/25 (28%) and a weak in 4/25 (16 %) of 4 out of 17 cases with 20% cyoplasmic stain; ES: 2 out of 2 cases with 20% cytoplasmic synovial sarcoma specimens. The presence of mRNA for ERBB2 was detected in 16/24 (67 stain. (MPNST= malignant peripheral nerve sheath tumor; GIST= gastrointestinal %) cases in contrast to the results of immunohistochemical techniques. 50 % patients with stromal tumor; SFT= solitary fi brous tumor; ESMChS= extraskeletal mesenchymal synovial sarcomas had a strong expression of ERBB2 proven by RQ-RT-PCR. chondrosarcoma; LGFMS=low grade fi bromyxoid sarcoma; EWS= Ewing sarcoma; ES= Conclusion: BCL-2 and ERBB2 are expressed in a high percentage of synovial sarcomas. epithelioid sarcoma). Other types of soft tissue tumors included in this study didn`t show The treatment with antisense BCL-2 may represent an appropriate alternative therapy for either nuclear or cytoplasmic staining. these patients. The treatment with tyrosine kinase inhibitors in connection with expression Conclusion: This is the fi rst study demonstrating expression of SYT in tissue sections of ERBB2 is debatable, because of a low expression of the ERBB-2 protein. The project of SS and indicates that this method could provide an easy, rapid, and widely applicable was supported by grant GAUK 200 052, 63/20005/C and by research project 00064203 means of assisting in the diagnosis of SS, particularly when material and/or resources are n. 6074. unavailable for PCR or FISH-based testing. Since variable weak staining for SYT may be encountered in non-SS sarcomas a positive interpretation should be made only when the 837 DUAL COLOUR FLUORESCENCE IN SITU HYBRIDIZATION ANALYSIS staining is strong, nuclear and present in the majority of cells. OF DELETION OF P16INK4A IN A TISSUE MICROARRAY OF SYNOVIAL SARCOMAS 835 THE VALUE OF MDM2 AND CDK4 AMPLIFICATION LEVELS USING Antonio Llombart-Bosch; Manish Mani Subramaniam; Rosa Noguera; Marta Piqueras; REAL-TIME PCR AND FISH FOR THE DIFFERENTIAL DIAGNOSIS OF Samuel Navarro; Julia Cruz, Department of Pathology, Medical School, University of LIPOSARCOMAS AND THEIR HISTOLOGIC MIMICKERS Valencia, Valencia, Spain; José Antonio López-Guerrero, Unit of Molecular Biology. Takanori Hirose; Shio Shimada; Keisuke Ishizawa; Hiroshi Harada, Department of Fundación Instituto Valenciano de Oncología, Valencia, Spain Pathology, Saitama Medical School, Saitama, Japan; Tadaki Matsumura; Tadashi Background: Alterations involving cell-cycle regulators acting at the G1-S checkpoint Hasegawa, Department of Clinical Pathology, Sapporo Medical University School of have been implicated in the pathogenesis and tumor progression of sarcomas including Medicine, Sapporo, Japan synovial sarcomas (SS). The 9p21 region contains 2 well known tumor suppressor genes, Background: Atypical lipomatous tumor (ALT), well differentiated liposarcoma of p16INK4A and p15INK4B. p16 blocks the cell cycle progression at the G1-S transition by the retroperitoneum (WDL) and dedifferentiated liposarcoma (DDL) share common interfering with the cyclin D/CDK4 complexes, inhibiting Rb phosphorylation and release chromosomal abnormalities, that is, ring or giant marker chromosomes composed of the of transcription factor E2F, thus forming a critical component of the pRB pathway. The role 12q13-15 region including MDM2 and CDK4 genes. To evaluate the diagnostic utility of p16 gene deletion at 9p21 in SSs has not been comprehensively investigated. of these genetic abnormalities, we compared amplifi cation levels of these genes in Design: Forty one SSs confi rmed by dual colour break-apart fl uorescence in situ liposarcomas and those in their histologic mimickers using real-time PCR and FISH. hybridization (FISH) assay were assembled in a tissue microarray (TMA) and interphase Design: Materials included 13 cases of ALT/WDL/DDL, 1 of pleomorphic liposarcoma, 5 dual colour FISH assay for deletion of the p16 gene at 9p21was performed on TMA sections of myxoid liposarcoma, 20 of malignant fi brous histiocytoma (MFH), 3 of leiomyosarcoma, using a commercial cocktail p16 (CDKN2A) specifi c DNA probe (Q-BIOgene) composed 5 of malignant peripheral nerve sheath tumor (MPNST), 23 of lipoma (including 4 spindle of p16 specifi c probe (rhodamine red) and ABL (9q34) probe (dgreen) for chromosome cell and 6 intramuscular type), and 28 of normal adipose tissue. Real-time PCR was identifi cation. A scoring system was applied to the FISH data to identify the heterozygous performed using DNA samples extracted from paraffi n blocks. The amplifi cation level was deletions of p16 gene which was defi ned as the presence of 2 green (ABL) and 1 red calculated as a ration of the copy number of MDM2/CDK4 to that of the reference gene, (p16) signals in 15% or more of the tumor cells. The percentage of tumor cells showing beta-globin. FISH was carried out on paraffi n sections in sarcoma groups. Locus-specifi c true heterozygous deletions was obtained by subtracting the population of cells showing probes were prepared from bacterial artifi cial chromosomes (BAC), clone RP11-775J10 the various possible combinations of cutting artefacts. Homozygous deletions were not for MDM2 and clone RP11-571M6 for CDK4. When a large homogeneously staining considered in the study as they were found in low proportions of tumor cells as well as region (HSR), which indicates a high level of amplifi cation of a target gene, was observed, due to very high possibility of false positive deletion resulting out of cutting artefacts in the population of cells containing HSRs were counted. paraffi n sections. Immunohistochemical analysis for p16, cyclin D1 and Ki-67 proteins Results: The median for MDM2 amplifi cation levels were as follows: 38.9 (range 11.9- were done and scored in TMA sections. 96.3) for ALT/WDL/DDL; 2.7 for pleomorphic liposarcoma; 2.5 (2.3-3.3) for myxoid Results: Heterozygous genomic deletion of p16 was observed in 28 of the 38 cases (74%) liposarcoma; 1.5 (0.4-4.6) for MFH; 0.4 (0.3-0.9) for leiomyosarcoma; 0.3 (0.2-1.1) for and Polysomy of chromosome 9 was present in 10 of the 38 cases (26%), out of which 7 MPNST; 1.2 (0.2-2.8) for lipoma; and 1.1 (0.1-1.7) for normal adipose tissue. The median (70%) of them were associated with deletion of the p16. 25 of the 28 (90%) cases with for CDK4 amplifi cation levels were as follows: 35.3 (range 6.2-69.7) for ALT/WDL/DDL; chromosomal loss of 9p21 showed abnormal p16 protein expression, of which 20 of 2.1 for pleomorphic liposarcoma; 2.3 (1.5-4.4) for myxoid liposarcoma; 1.5 (0.4-2.8) them were negative and 5 revealed heterogenous staining. Out of the 25 cases showing for MFH; 0.7 (0.7-2.2) for leiomyosarcoma; 0.7 (0.5-1.4) for MPNST; 0.6 (0.2-2.1) for over expression of cyclin D1, 19 (68%) demonstrated deletion of p16 loci. No signifi cant lipoma; and 1.1 (0.5-1.6) for normal adipose tissue. The amplifi cation levels of MDM2 association was observed between deletion of 9p21 and Ki-67 proliferation index, tumor and CDK4 in all ALT/WDL/DDL but one were over 10 and were signifi cantly higher than grade or histological subtype. those of other soft tissue tumors studied. In FISH, HSRs were detected in most tumor cells Conclusion: This high-throughput TMA based FISH assay for deletion of p16 gene in of all ALT/WDL/DDL (MDM2: median 90.7%, range 77.4-97.4%; CDK4: median 86.5%, SSs demonstrates the genomic deletion of p16INK4A at 9p21, to be a frequent molecular range 5.9-93.7%), whereas they were not seen in other sarcomas. genetic event in the pathogenesis and tumor progression of synovial sarcomas. Supported Conclusion: In the present study, we demonstrated the remarkable amplifi cation of by a grant (PI-04/0822) from the FIS (Instituto Carlos-III, Madrid, Spain), PROTHETS MDM2 and CDK4 in ALT/WDL/DDL with real-time PCR using paraffi n materials. On (Prognosis and therapeutic targets of Ewing´s family of tumors) FP6 Contract number: the other hand, other histologic mimickers, such as MFH, leiomyosarcoma, MPNST and 503036 and a Personal grant from the Hospital Clinic of University and the AECC of lipoma, showed very low or no amplifi cation. HSRs indicating higher amplifi cation were Valencia, Spain. also demonstrated only in ALT/WDL/DDL in FISH. Therefore, a quantitative analysis of MDM2 and CDK4 amplifi cation using real-time PCR as well as FISH is extremely useful for the differentiation of ALT/WDL/DDL from their histologic mimickers. 182 XXVI Congress of the IAP: Abstracts 838 ANGIOMATOID FIBROUS HISTIOCYTOMA OF THE EWS/PNET and in 2/15 control cases. FISH was rearranged in 38/43 EWS/PNET cases RETROPERITONEUM and in 0/15 control cases, and has split signal in 19/43 EWS/PNET cases and in all 15 Alisa Matthews; Masatoshi Kida, University of Vermont/Fletcher Allen Health Care, control cases. The most sensitive and specifi c test panel for the diagnosis of EWS/PNET Burlington, VT, United States; Kumarasen Cooper, University of Vermont, Burlington, is the combination of CD99 and FLI-1p with sensitivity and specifi city of 93% and 73.3%, VT, United States respectively. FISH had a very high specifi city (100%) but only a moderate sensitivity Background: Angiomatoid fi brous histiocytoma is a rare soft tissue neoplasm with low (50%). metastatic potential that usually occurs in children and young adults. It is generally a tumor Conclusion: The combination of CD99 and FLI-1p is the method of choice for the of the deep dermis and subcutis, most commonly located in the extremities, trunk, or head diagnosis of EWS/PNET. EWRS1 (22q12) dual color, break apart rearrangement probe and neck. We report a case of angiomatoid fi brous histiocytoma presenting as a large, cystic FISH should be used as a confi rmatory test in addition to CD99 and FLI1-p due to its high retroperitoneal mass in a 49 year-old woman. To our knowledge, this is the fi rst descrption specifi city. in the English literature of angiomatoid fi brous histiocytoma in the retroperitoneum. Design: Gross and histiologic descriptions of the excised surgical specimen are provided. 841 MUTATIONAL, IMMUNOHISTOCHEMICAL, AND FUNCTIONAL Paraffi n-embedded sections were submitted for routine hematoxylin and eosin staining, ANALYSIS OF THE HEDGEHOG PATHWAY IN SYNOVIAL SARCOMA and immunohistochemical stains were performed on selected blocks. The fi ndings are Toru Motoi; Tsuyoshi Saito; Noriko Motoi; Marc Ladanyi, Memorial Sloan-Kettering presented in the context of a review of the literature. Cancer Center, New York, NY, United States Results: Gross examination revealed a 17 x 10.5 x 3.5 cm multi-cystic mass that contained Background: The Hedgehog (HH) pathway signals via Patched (PTCH) and Smoothened a normally-sized right adrenal gland in the superomedial aspect of the specimen. Multiple (SMO) at the cell surface to the GLI1 transcription factor in the nucleus. It is known to be ill-defi ned nodular areas were present, displaying a tan-white microcytic cut surface. The required for cell differentiation and organ formation during embryogenesis. The Suppressor cysts contained a mixture of blood clot and diffl uent material. Histiologically, the tumor of Fused (SuFu) protein binds to GLI1 and blocks HH pathway signaling by inhibiting consisted of a multinodular proliferation of uniform cells with round to oval, vesicular nuclear transfer of GLI1 and interfering with its transcriptional activity. Activation of HH nuclei, prominent nucleoli, and abundant pale eosinophilic cytoplasm. A thick fi brous signaling due to mutations in PTCH or SMO is seen in some cancers. pseudocapsule was present, which was associated with a dense lymphoplasmacytic Design: Mutational analysis was performed on all coding exons of PTCH and SMO infi ltrate. Bands of fi brous tissue coursing through the tumor contained a similar infi ltrate; in 40 SS. Immunohistochemistry (IHC) for GLI1 and SuFu was performed on a tissue occasional germinal center formation was observed. Many pseudoangiomatoid cystic microarray containing 30 SSs (20 monophasic SS (MSS), 9 Biphasic SS (BSS), 1 poorly spaces were present, containing extravasated blood. Tumor cells were focally positive for differentiated). We examined changes in cell growth and expression of two HH target keratin AE1-AE3, EMA, and occasionally for CK7. The surgical margins were negative genes (GLI1, PTCH) upon blockade of HH signaling with cyclopamine, a specifi c inhibitor for tumor, and the patient has no evidence of disease recurrence or metastases 2 years of SMO, in three SS cell lines (FUJI, HS-SY-II, SYO-1). post-diagnosis. Results: Sequencing revealed no somatic mutations in PTCH and SMO in 40 SS samples Conclusion: We document a case of angiomatoid fi brous histiocytoma presenting as a and 3 SS cell lines. By IHC, the MSS and the spindle cell components of BSS cases deep-seated mass in a middle-aged woman. This is the fi rst reported case of angiomatoid showed nuclear positivity for GLI1 suggestive of active HH pathway signaling in 27 of fi brous histiocytoma in the retroperitoneum. 28 evaluable cases ( 0:1, 1+:7, 2+:14, 3+:6). Nuclear positivity for SuFu was observed in 24/28 (0:4, 1+:3, 2+:17, 3+:4). Cytoplasmic staining for GLI1 and SuFu was present in 839 SARCOMATOUS TRANSFORMATION IN A CELLULAR 27/28 (0:1, 1+:2, 2+;17, 3+:8) and 28/28 (1+:2, 2+:14, 3+:12), respectively. Expression of ANGIOFIBROMA GLI1 and SuFu showed signifi cant concordance both in nucleus and cytoplasm (p<0.05) Ralph Matthews, University of Vermont, Burlington, VT, United States; Masatoshi of spindle cells but they were not correlated in the epithelial component of BSS cases. Kida, University of Vermont/Fletcher Allen Health Care, Burlington, VT, United States; FUJI cells were most sensitive to cyclopamine, with an IC50 (50% reduction in cell counts Kumarasen Cooper, University of Vermont, Burlington, VT, United States relative to untreated cells) between 1ìM and 5ìM whereas SYO-1 cells were less sensitive Background: Cellular angiofi broma is a rare mesenchymal tumor of older adults that is (IC50: 10ìM), and HS-SY-II cells were resistant (IC50>10ìM). By quantitative RT-PCR, currently classifi ed as benign. This tumor is usually located in the vulvar or inguinoscrotal cyclopamine caused an 80% decrease in GLI1 expression in FUJI cells, but not in the regions, with rare cases described in the retroperitoneum and subcutaneous tissue of the other two lines. PTCH was not signifi cantly altered upon cyclopamine treatment in any chest. We report the case of a 52 year-old woman with a cellular angiofi broma of the left of the three cell lines. There was a difference in localization of SuFu in the three cell hip that contains areas of sarcomatous transformation. To our knowledge, this is the fi rst lines; nuclear localization of SuFu was observed in FUJI cells whereas strong cytoplasmic report of sarcomatous transformation within a cellular angiofi broma. positivity was observed in the other two. Design: The entire soft tissue excision was submitted for paraffi n-embedded sections with Conclusion: The HH pathway seems to be active in MSS and in the spindle cell component routine hematoxylin and eosin staining. Immunohistochemical studies were performed on of BSS. Co-localization of GLI1 and SuFu suggests that HH pathway is regulated in select tissue blocks. SS. The HH pathway can be blocked by cyclopamine in some SS, resulting in reduced Results: The soft tissue excision consisted of a 3.5 x 3.0 x 2.3 cm specimen with a proliferation, thereby identifying HH signaling as a novel potential therapeutic target. predominately tan-white, fi rm cut surface. Eccentrically located within this tissue was However, mutational activation of the HH pathway is absent or rare in SS. a tan-yellow, whorled, well-delineated nodule measuring 1.5 cm in greatest dimension. Histologically, the tumor was primarily composed of cytologically bland spindle cells 842 THE UTILITY OF MDM2 AND CDK4 IMMUNOSTAINS IN NEEDLE set in a collagenous stroma, with a proliferation of small to medium-sized dilated vessels BIOPSY INTERPRETATION OF LIPOMATOUS TUMORS. A STUDY OF 12 TRU- with variably hyalinized walls. Other areas showed an abrupt transition to hypercellular CUT BIOPSIES sarcomatous elements, including pleomorphic cells with high mitotic activity. The tumor Ayoub Nahal; Daniel Dion; Hassan Huwait; Robert Turcotte, McGill University Health cells were positive for vimentin and factor XIIIa. Variable, weak positivity for CD34 was Centre, Montreal, QC, Canada observed in the sarcomatous tumor cells. The resection margins of the initial excision were Background: Well-differentiated liposarcoma (WDLS) is among the most common soft positive, and a wide re-excision approximately one month later showed no residual tumor. tissue sarcomas and together with its dedifferentiated form(DDLS) is among the most The patient is free of disease recurrence or metastases 23 months post-diagnosis. histologically variable tumors. Normally, a fi rm diagnosis on core biopsy is only feasible Conclusion: This is the fi rst reported case of sarcomatous transformation of a cellular within the comfort zone of classic appearance. This limitation coupled with the nature of angiofi broma. The signifi cance of sarcomatous transformation in this entity is unclear. surgical management of WDLS/DDLS has de-emphasized the importance of pre-operative Documentation of malignant transformation in this benign lesion is important, and tissue diagnosis. The known molecular amplifi cation of MDM2 and CDK4 genes on additional cases are necessary to elucidate the prevalence and implications of our fi nding. chromosome 12q13-15 can now be detected with commercially available immunostains. We report the results of MDM2 and CDK4 immunostains and emphasize their indispensable 840 DIAGNOSTIC UTILITY OF FLI-1 MONOCLONAL ANTIBODY AND utility in approaching typical and challenging lipomatous tumors on needle biopsies. DUAL- COLOR BREAK-APART PROBE FLUORESCENCE IN SITU (FISH) Design: 12 biopsies (5 retrospectively and 7 prospectively) and three fi nal tumor resections ANALYSIS IN EWING’S SARCOMA/ PRIMITIVE NEUROECTODERMAL were tested for MDM2 and CDK4. TUMOR (EWS/PNET) Results: The patients’ age varied from 35 to 82 with male predominance (10/12). 11 Paulette Mhawech-Fauceglia, Roswell Park Cancer Institute, Buffalo, NY, United States; tumors were located in the limbs and one in the retroperitoneum. Mean follow-up was Francois R. Herrmann, Geneva University Hospital, Geneva, Switzerland; Penetrante 7.2 months (1-21). Confi dent morphologic diagnosis was diffi cult in all cases. 9 cases Remedios; Kunle Odunsi; Sheila Sait; Anne Marrie Block, RPCI, Buffalo, NY, United belonged to the WDLS/DDLS category spanning the entire spectrum of classic WDLS, States; Lucia Balos, Kaleida Health System, Buffalo, NY, United States; Richard T. ALT, non lipogenic sclerosing and infl ammatory variants to pure DDLS unaccompanied Cheney, RPCI, Buffalo, NY, United States by the WDLS component. There were two cases of typical and spindle cell lipomas, and Background: The diagnosis of Ewing’s Sarcoma/Primitive Neuroectodermal Tumor one case of pleomorphic liposarcoma. The three post biopsy fi nal resections concurred (EWS/PNET) depends on morphology and the immunohistochemistry (IHC) profi le. with the pre-operative diagnosis and consisted of a spindle cell lipoma, well differentiated CD99 and FLI-1 polycolonal (FLI-1p) antibodies have been proven to be useful diagnostic liposarcoma and a pleomorphic liposarcoma. MDM2 and CDK4 were positive in all but markers. This study compares the sensitivity and specifi city of the recently commercially the typical lipoma and the pleomorphic liposarcoma. Positive staining was instrumental available FLI-1 monoclonal (FLI-1m) antibody to the currently used antibodies (CD99 and in diagnosing the four cases that showed only a non lipogenic sclerosing or infl ammatory FLI-1p) in the diagnosis of EWS/PNET and to determine the diagnostic value of EWSR1 histology or dedifferentiation alone. Pleomorphic liposarcoma was interpreted negative (22q12) dual color, break-apart rearrangement probe fl uorescent in situ hybridization due to non specifi c cytoplasmic labeling. The case of spindle cell lipoma was positive in (FISH) technique. both the biopsy and the resection. Design: 43 cases of well-documented EWS/PNET and 15 non EWS/PNET case were Conclusion :MDM2 and CDK4 immunostains are indispensable in the diagnosis of retrieved from the archival fi les. IHC for FLI-1p, FLI-1m and FISH analysis were lipomatous tumors on core biopsies. Where non-lipogenic components predominate, their performed. positive expression helps to confi rm liposarcoma, a diagnosis otherwise diffi cult to fi rmly Results: CD99 was positive in 40/43 EWS/PNET, and in 3/15 of control cases. FLI-1m achieve on morphologic grounds alone. Spindle cell lipomas might pose diffi culty on core was expressed in 42/43 EWS/PNET and in 6/15 control cases. FLI-1p was seen in 35/43 XXVI Congress of the IAP: Abstracts 183 biopsy. Therefore, awareness to their known expression of MDM2 and CDK4 should be or subplasmalemmal linear staining pattern in tumor cells. In contrast, all neurofi bromas made to avoid overdiagnosis. and leiomyomas were negative for WT1. Ten of 10 (100%) schwannomas were positive for WT1 although the staining intensity was relatively weak compared to those of the above 843 USE OF BETA CATENIN IN EVALUATION OF RESECTION MARGINS OF described sarcomas. One of 8 (13%) synovial sarcomas, 4/10 (40%) well-differentiated SOFT TISSUE FIBROMATOSIS liposarcomas and 2/7 (29%) MFHs were positive for WT1. Vladimir Osipov; David King; Donald Hackbarth, Medical College of Wisconsin, Conclusion:Within neurogenic and myogenic sarcomas, malignant tumors tended to Milwaukee, WI, United States be strongly positive for WT1, but benign tumors were negative or weak for WT1. WT1 Background: Desmoid-type fi bromatosis is a clonal fi broblastic proliferation with expression in soft tissue tumors could be a useful marker for malignant neurogenic and infi ltrative growth and a tendency to recur. Since fi bromatosis is known for its bland myogenic tumors. histological appearance and similarity to scar tissue and tendons, the margins’ clearance represents a challenge especially in the face of recurrence. Since beta-Catenin nuclear 846 MIXED TUMOR OF SOFT TISSUE WITH PULMONARY METASTASIS staining is a consistent feature of this entity, we retrospectively evaluated surgical resection Kenichi Wakasa; Tomoko Wakasa; Naoko Obatake,Department of Diagnostic Pathology, margins in cases of soft tissue desmoid fi bromatosis using this marker. Osaka CIty University Graduate School of Medicine, Osaka, Japan; Makoto Ieguchi, Design: Six cases of soft tissue desmoid fi bromatosis were used for this study. Follow-up Department of Orthopedic Surgery, Osaka City University Graduate School of Medicine, time ranged from 2 to 5 years. All but two cases had negative resection margins at the time Osaka, Japan of the initial surgery. Beta-Catenin immunohistochemical stain was used to re-assess the Introduction: Mixed tumor of soft tissue is a rare disease. The prognosis is most often surgical resection margins. Sections of tendons and scar tissue from unrelated cases were benign, but a minority of the tumors metastasize. also stained with this marker to document the absence of beta-Catenin expression in these Design: We report a case of mixed tumor with pulmonary metastasis. types of tissue. Results: A 58-year-old, male was admitted to the hospital because of pain in the sole of Results: All three cases of fi bromatosis with recurrence had beta-Catenin immunostaining the left foot. Four months before the fi rst admission, the patient noticed discomfort of at the surgical resection margins. The staining at the resection margins was not seen in the the left sole. Thereafter, he noticed swelling and pain in the sole, and consulted a local recurrence-free cases. In recurrent cases the staining allowed clear distinction between hospital, where a tumor of the sole was surgically resected. But 1 month before the current fi bromatosis and scar tissue. Freezing of the tissue during frozen sections did not affect the admission, the tumor recurred and he was admitted to this hospital. The tumor located in staining intensity of the tissue re-submitted for permanent sections. the soft tissue of the sole, measured 3 cm in diameter, and was elastic hard, protuberant, Conclusion: Beta-Catenin immunostaining allows for evaluation of the extent of and exposed through a skin ulcer. The surface of the tumor was red and seemed to be fi bromatosis remarkably well. The tissue staining was not affected by processing during rich in vascularity. Radiologically, the tumor had an ill-defi ned border, and showed low frozen section. There was no staining observed in the scar or tendon tissue. Retrospective intensity on T1- and high intensity on T2-weighted MRI. On the second and eleventh evaluation of margins using beta-Catenin showed that the staining at the inked margins hospital days, respectively, biopsy and resection of the tumor was performed. On the was predictive of recurrence. We recommend use of this stain to rule out the recurrence of 15th hospital day, the foot was amputated. Seven months after the amputation, resection the disease as well as to achieve a complete microscopic margin clearance in cases of soft of multiple bilateral pulmonary metastases was carried out. In histopathology, the tumor tissue desmoid fi bromatosis when complete resection is feasible. was composed of trabecular and ductal arrangement of epithelioid cells and solid, fi brous and myxoid areas of spindle cells. Frank anaplasia was not seen. Mitotic count was 10/10 844 MYOEPITHELIAL CARCINOMA OF SOFT TISSUE. A CASE REPORT HPF. Vascular invasion was identifi ed. The histopathology of pulmonary metastases was WITH CYTOGENETIC FINDINGS almost the same as the primary tumor. Immunohistochemically the tumor was positive Zoltán Sápi; Linda Deák; Zsófi a Balogh, 1st Department of Pathology, Semmelweis for vimentin, CAM5.2, AE1+AE3, EMA, S-100 protein, GFAP, and negative for desmin, University, Budapest, Budapest, Hungary alpha smooth muscle actin, CD34, CD68. Background: Soft tissue myoepithelial carcinoma (STMC) is a very rare tumor displaying Conclusion: In this case, frank anaplasia was not seen, but mitotic count was 10/10 HPF myoepithelial elements and lacking obvious ductal differentiation. Only one case report and vascular invasion was identifi ed. High mitotic count and vascular invasion may be a with cytogenetics is available in the English literature by which STMC seems to be a hallmark of malignancy in the mixed tumor. distinct entity with some resemblance to chordoma on the one hand and myoepithelioma on the other. Design: To present the second case of STMC with cytogenetic fi ndings. Techniques Results: A 82-year-old female patient presented with a soft tissue tumor within the deep soft tissues in the right gluteal muscle measuring 16x13x11 cm. Histologically, the lesion 847 IMMUNOHISTOCHEMICAL EXPRESSION PROFILE OF BREAST was diagnosed as a myoepithelial carcinoma displaying a partly lobulated architecture with CANCER OBTAINED BY TISSUE MICROARRY USING TWO CORES IS cords and nests of solid proliferations of spindled and plasmocytoid cells with frankly COMPARABLE TO LARGE-SECTION RESULTS malignant cytomorphology. Areas of necrosis were obvious. Immunohistochemistry was Abdulmohsen Alkushi; Walid Khalbuss, King Fahad National Guard Hospital, Riyadh, partly positive for pancytokeratin , EMA, S-100 protein, and alpha smooth muscle actin, Saudi Arabia; Osama Nassif, King Abdulaziz University Hospital, Jeddah, Saudi Arabia and negative for H-Caldesmon and Myf-4. MIB-1 index was 28%. Using high resolution Background: The immunohistochemical analysis of a large number of tumor comperative genomic hybridization a gain of 1q21-23, 9q12-q33, 16q22 and loss of 1p31- tissues with conventional techniques is tedious and slow. It is possible by using tissue 34, 1p11-22, 1q24-q44, 3p, 10q11.1-q22, 13q, 14q13-q24, 15q was detected. Further FISH microarry technology (TMA) to sample up to 1000 tumors on one glass slide, which analysis confi rmed the 3p deletion and the monosomy of chromosomes of 13 and 15. Total then can be analyzed by fl uorescence in situ hybridization, RNA in situ hybridization, or DNA content measured by image cytometry proved to be diploid, DI: 0.95. immunohistochemistry. Because of the small size of the individual arrayed tissue samples Conclusion: Our results support the hypothesis that STMC is a distinct entity, not (diameter 0.6 mm), the question arises as to whether these specimens are representative of sharing the cytogenetic alterations of salivary gland myoepithelial carcinomas and ductal their donor tumors. The aim of this study is to compare the staining result obtained by TMA carcinomas of breast with myoepithelial differentiation. with the conventional large-section technique. Design: 80 cases of breast cancer were retrieved from the archives of our institutions to 845 WILMS` TUMOR GENE (WT1) EXPRESSION IN NEUROGENIC AND build breast cancer model. A tissue microarray consisting of duplicate 0.6 mm cores of MYOGENIC SARCOMAS tumor was constructed from selected one paraffi n block containing tumor per case. Serial Satoko Shimada, Nagoya University Graduate School of Medicine, Nagoya, Japan; sections of donor block were then immunostained with a panel of 4 antibodies (ER, PR, Toyonori Tsuzuki, Nagoya Daini Red Cross Hospital, Nagoya, Japan; Makoto Kuroda, Her2/neu, and p53). Twenty-six of recipient blocks, after being cored, were serial sectioned Fujita Health University School of Medicine, Toyoake, Aichi, Japan; Tetsuro Nagasaka, and stained for H&E, ER, PR, Her2/Neu, and p53 immunostains. H&E of recipient Nagoya University Graduate School of Medicine, Nagoya, Aichi, Japan; Kazuo Hara, blocks were examined to determine how many core accurately sample the target tumor. Aichi Medical University Hospital, Nagakute, Aichi, Japan; Emiko Takahashi, Aichi Concordance rates were calculated for each immunostains comparing scoring result by Cancer Center Hospital, Nagoya, Aichi, Japan; Seijun Hayakawa, Anjo Kosei Hospital, TMA and conventional large section of recipient blocks. Anjo, Aichi, Japan; Kenzo Ono, Tosei General Hospital, Seto, Aichi, Japan; Nagako Results: Target tumors were accurately sampled by two cores in 19 out of 26 recipient Maeda, Nagoya Daini Red Cross Hospital, Nagoya, Aichi, Japan; Naoyoshi Mori, Nagoya blocks, and only by one core in 5 blocks. Failure to sample tumor was seen in two blocks. University Graduate School of Medicine, Nagoya, Aichi, Japan Concordance rates of immunostain scoring result between TMA and conventional large Background: The Wilms` tumor gene (WT1), originally identifi ed as a tumor suppressor section were 84%, 81%, 96%, and 96% for ER, PR, Her2/Neu, and p53 respectively when gene, is overexpressed in hematologic malignancies and solid tumors. Therefore, WT1 is all 26 recipient blocks included. The rate improved to 100% for p53 immunostain and no currently thought to play an important role in the tumorigenesis of these malignancies. changes for the other markers when concordance is limited to recipient blocks that have It has become known that WT1 is detected in developing spinal cord, brain and skeletal been sampled by two cores. muscle. The aim of the current study is to evaluate the diagnostic utility of WT1 in Conclusion: Tissue microarry is a reliable technique for examining large set of tumors. neurogenic and myogenic sarcomas. It shows immunostaining scores comparable to those obtained by conventional large- Design: Formalin-fi xed, paraffi n-embedded sections were prepared from 131 soft section. However, some alterations are not detected due to heterogeneity of the tumors. tissue tumors, including 39 malignant peripheral nerve sheath tumors (MPNSTs), This shortfall can be improved by sampling target tumor by two cores and possibly by 10 schwannomas, 10 neurofi bromas, 17 leiomyosarcomas, 10 leiomyomas, 20 validating the technique through examining the recipient blocks H&E sections following rhabdomyosarcomas, 8 synovial sarcomas, 10 well-differentiated liposarcomas and 7 coring procedure. malignant fi brous histiocytomas (MFHs). Sections were stained with the antibody to WT1 using a biotin free detection system. The cases showing moderate to strong positivity for 848 PRODUCTION OF HIGH QUALITY POLYCLONAL ANTIBODY OF HPV the antibody were regarded as positive. 18 E7 PROTEIN Results:Thirty-two of 39 (82%) MPNSTs, 14/17 (82%) leiomyosarcomas, and 18/20 (90%) Venugopal Balakrishnan; Shaharum Shamsuddin ; Nor Hayati Othman, Universiti Sains rhabdomyosarcomas were strongly positive for WT1. WT1 showed granular cytoplasmic Malaysia, Kubang Kerian, Kelantan, Malaysia Background: Human Papillomaviruses (HPVs), a large family of small double stranded