Morphological and Immunohistochemical Characteristics of Surgically Removed Paediatric Renal Tumours in Latvia (1997–2010)
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Mixed Hepatoblastoma in the Adult: Case Report and Review of the Literature
J Clin Pathol: first published as 10.1136/jcp.33.11.1058 on 1 November 1980. Downloaded from J Clin Pathol 1980;33:1058-1063 Mixed hepatoblastoma in the adult: case report and review of the literature RP HONAN AND MT HAQQANI From the Department of Pathology, Walton Hospital, Rice Lane, Liverpool L9 JAE, UK SUMMARY A case of mixed hepatoblastoma in a woman is described. A survey of the English literature reveals 13 cases acceptable as mixed hepatoblastoma; these have been described and published under a variety of names. Difficulties in nomenclature and the histology of these cases are discussed. Diagnosis depends on the identification of both malignant mesenchymal and malignant epithelial elements. The former include myxoid connective tissue resembling primitive mesenchyme and areas resembling adult fibrosarcoma. Mature fibrous tissue with calcification and bone for- mation may be seen. Epithelial areas show tissue resembling fetal liver, poorly differentiated epithelial cells, and/or areas of adenocarcinoma. The current view on histogenesis is also given. Most hepatoblastomas occur in children under the mixedtumour,6carcino-osteochondromyxosarcoma,5 copyright. age of 2 years.' Hepatoblastoma in adults is ex- and rhabdomyosarcohepatoma.7 tremely rare, and the prognosis is much worse than in the mixed hepatoblastoma of childhood. Case report The literature of mixed hepatoblastoma in adults has until recently been confused, and the true inci- CLINICAL PRESENTATION dence of the tumour obscured, owing to the various A Chinese woman aged 27 had been resident in names used by different authors to describe their England for eight years. She gave a history of cases. The commonest pseudonym is 'mixed malig- 18 months' intermittent right-sided chest pain http://jcp.bmj.com/ nant tumour',2-4 an ambivalent term which merely and upper abdominal discomfort. -
Angiomyolipoma of the Cervix – Report of a Rare Entity
Internet Journal of Medical Update. 2017 July;12(2):13-15. doi: 10.4314/ijmu.v12i2.4 Internet Journal of Medical Update Journal home page: http://www.akspublication.com/ijmu Case Report Angiomyolipoma of the cervix – report of a rare entity N. Hariharanadha Sarmaᴪ1, Rama Srivastava2, Smriti Agnihotri3 1Consultant Pathologist, Department of Pathology, RDT Hospital, Bathalapalli, Andhra Pradesh, India 2Professor, Department of Pathology, SSR Medical College, Belle Rive, Mauritius 3Professor, Department of Pathology, American University of Antigua College of Medicine, Antigua & Barbuda, WI (Received 22 September 2017 and accepted 04 October 2017) ABSTRACT: Angiomyolipoma (AML) is a mesenchymal neoplasm seen usually in the kidney. Few cases of extra renal AML have been documented in various organs including the female genital tract, where the uterus is the most common site. To the best of our knowledge, only 4 cases of AML in the cervix have been reported in the literature. Association of AML with tuberous sclerosis is well known. Presently AML is included in the spectrum of disease entities called PEComa. We report a case of AML without tuberous sclerosis arising from the uterine cervix, which has to be differentiated from lipoleiomyoma. KEY WORDS: Angiomyolipoma; Uterine cervix; PEComas; Uterine tumor INTRODUCTIONV localization8, and mostly in females over 409. The occurrence of AML in the cervix without Angiomyolipoma (AML) occurs most frequently in concurrent incidence in the kidney is extremely the kidney, where it is closely related to tuberous rare, and only four cases have been reported in the sclerosis complex (TSC)1,2, occasionally in other scientific literature10. We are reporting a case of organs, most commonly the liver, but occurrence at uterine cervix AML without tuberous sclerosis other sites is extremely rare3. -
Soft Tissue Cytopathology: a Practical Approach Liron Pantanowitz, MD
4/1/2020 Soft Tissue Cytopathology: A Practical Approach Liron Pantanowitz, MD Department of Pathology University of Pittsburgh Medical Center [email protected] What does the clinician want to know? • Is the lesion of mesenchymal origin or not? • Is it begin or malignant? • If it is malignant: – Is it a small round cell tumor & if so what type? – Is this soft tissue neoplasm of low or high‐grade? Practical diagnostic categories used in soft tissue cytopathology 1 4/1/2020 Practical approach to interpret FNA of soft tissue lesions involves: 1. Predominant cell type present 2. Background pattern recognition Cell Type Stroma • Lipomatous • Myxoid • Spindle cells • Other • Giant cells • Round cells • Epithelioid • Pleomorphic Lipomatous Spindle cell Small round cell Fibrolipoma Leiomyosarcoma Ewing sarcoma Myxoid Epithelioid Pleomorphic Myxoid sarcoma Clear cell sarcoma Pleomorphic sarcoma 2 4/1/2020 CASE #1 • 45yr Man • Thigh mass (fatty) • CNB with TP (DQ stain) DQ Mag 20x ALT –Floret cells 3 4/1/2020 Adipocytic Lesions • Lipoma ‐ most common soft tissue neoplasm • Liposarcoma ‐ most common adult soft tissue sarcoma • Benign features: – Large, univacuolated adipocytes of uniform size – Small, bland nuclei without atypia • Malignant features: – Lipoblasts, pleomorphic giant cells or round cells – Vascular myxoid stroma • Pitfalls: Lipophages & pseudo‐lipoblasts • Fat easily destroyed (oil globules) & lost with preparation Lipoma & Variants . Angiolipoma (prominent vessels) . Myolipoma (smooth muscle) . Angiomyolipoma (vessels + smooth muscle) . Myelolipoma (hematopoietic elements) . Chondroid lipoma (chondromyxoid matrix) . Spindle cell lipoma (CD34+ spindle cells) . Pleomorphic lipoma . Intramuscular lipoma Lipoma 4 4/1/2020 Angiolipoma Myelolipoma Lipoblasts • Typically multivacuolated • Can be monovacuolated • Hyperchromatic nuclei • Irregular (scalloped) nuclei • Nucleoli not typically seen 5 4/1/2020 WD liposarcoma Layfield et al. -
The Health-Related Quality of Life of Sarcoma Patients and Survivors In
Cancers 2020, 12 S1 of S7 Supplementary Materials The Health-Related Quality of Life of Sarcoma Patients and Survivors in Germany—Cross-Sectional Results of A Nationwide Observational Study (PROSa) Martin Eichler, Leopold Hentschel, Stephan Richter, Peter Hohenberger, Bernd Kasper, Dimosthenis Andreou, Daniel Pink, Jens Jakob, Susanne Singer, Robert Grützmann, Stephen Fung, Eva Wardelmann, Karin Arndt, Vitali Heidt, Christine Hofbauer, Marius Fried, Verena I. Gaidzik, Karl Verpoort, Marit Ahrens, Jürgen Weitz, Klaus-Dieter Schaser, Martin Bornhäuser, Jochen Schmitt, Markus K. Schuler and the PROSa study group Includes Entities We included sarcomas according to the following WHO classification. - Fletcher CDM, World Health Organization, International Agency for Research on Cancer, editors. WHO classification of tumours of soft tissue and bone. 4th ed. Lyon: IARC Press; 2013. 468 p. (World Health Organization classification of tumours). - Kurman RJ, International Agency for Research on Cancer, World Health Organization, editors. WHO classification of tumours of female reproductive organs. 4th ed. Lyon: International Agency for Research on Cancer; 2014. 307 p. (World Health Organization classification of tumours). - Humphrey PA, Moch H, Cubilla AL, Ulbright TM, Reuter VE. The 2016 WHO Classification of Tumours of the Urinary System and Male Genital Organs—Part B: Prostate and Bladder Tumours. Eur Urol. 2016 Jul;70(1):106–19. - World Health Organization, Swerdlow SH, International Agency for Research on Cancer, editors. WHO classification of tumours of haematopoietic and lymphoid tissues: [... reflects the views of a working group that convened for an Editorial and Consensus Conference at the International Agency for Research on Cancer (IARC), Lyon, October 25 - 27, 2007]. 4. ed. -
Non-Wilms Renal Cell Tumors in Children
PEDIATRIC UROLOGIC ONCOLOGY 0094-0143/00 $15.00 + .OO NON-WILMS’ RENAL TUMORS IN CHILDREN Bruce Broecker, MD Renal tumors other than Wilms’ tumor are tastases occur in 40% to 60% of patients with infrequent in childhood. Wilms’ tumors ac- clear cell sarcoma of the kidney, whereas they count for 6% to 7% of childhood cancer, are found in less than 2% of patients with whereas the remaining renal tumors account Wilms’ tumor.**,26 This distinct clinical behav- for less than l%.27The most common non- ior is one of the features that has led to its Wilms‘ tumors are clear cell sarcoma of the designation as a separate tumor. Other clini- kidney, rhabdoid tumor of the kidney (both cal features include a lack of association with formerly considered unfavorable Wilms’ tu- sporadic aniridia or hemihypertrophy. mor variants but now considered separate tu- Clear cell sarcoma of the kidney has not mors), renal cell carcinoma, mesoblastic been reported to occur bilaterally and is not nephroma, and multilocular cystic nephroma. associated with nephroblastomatosis. It has Collectively, these tumors account for less been reported in infancy and adulthood, but than 10% of the primary renal neoplasms in the peak incidence is between 3 and 5 years childhood. of age. It has an aggressive behavior that responds poorly to treatment with vincristine and actinomycin alone, leading to its original CLEAR CELL SARCOMA designation by Beckwith as an unfavorable histology pattern. The addition of doxorubi- Clear cell sarcoma of the kidney is cur- cin in aggressive chemotherapy regimens has rently considered a separate tumor distinct improved outcome. -
Appendix 4 WHO Classification of Soft Tissue Tumours17
S3.02 The histological type and subtype of the tumour must be documented wherever possible. CS3.02a Accepting the limitations of sampling and with the use of diagnostic common sense, tumour type should be assigned according to the WHO system 17, wherever possible. (See Appendix 4 for full list). CS3.02b If precise tumour typing is not possible, generic descriptions to describe the tumour may be useful (eg myxoid, pleomorphic, spindle cell, round cell etc), together with the growth pattern (eg fascicular, sheet-like, storiform etc). (See G3.01). CS3.02c If the reporting pathologist is unfamiliar or lacks confidence with the myriad possible diagnoses, then at this point a decision to send the case away without delay for an expert opinion would be the most sensible option. Referral to the pathologist at the nearest Regional Sarcoma Service would be appropriate in the first instance. Further International Pathology Review may then be obtained by the treating Regional Sarcoma Multidisciplinary Team if required. Adequate review will require submission of full clinical and imaging information as well as histological sections and paraffin block material. Appendix 4 WHO classification of soft tissue tumours17 ADIPOCYTIC TUMOURS Benign Lipoma 8850/0* Lipomatosis 8850/0 Lipomatosis of nerve 8850/0 Lipoblastoma / Lipoblastomatosis 8881/0 Angiolipoma 8861/0 Myolipoma 8890/0 Chondroid lipoma 8862/0 Extrarenal angiomyolipoma 8860/0 Extra-adrenal myelolipoma 8870/0 Spindle cell/ 8857/0 Pleomorphic lipoma 8854/0 Hibernoma 8880/0 Intermediate (locally -
Novel KHDRBS1-NTRK3 Rearrangement in a Congenital Pediatric CD34-Positive Skin Tumor: a Case Report
Virchows Archiv (2019) 474:111–115 https://doi.org/10.1007/s00428-018-2415-0 BRIEF REPORT Novel KHDRBS1-NTRK3 rearrangement in a congenital pediatric CD34-positive skin tumor: a case report Matthias Tallegas1 & Sylvie Fraitag2 & Aurélien Binet3 & Daniel Orbach4 & Anne Jourdain5 & Stéphanie Reynaud6 & Gaëlle Pierron6 & Marie-Christine Machet1,8 & Annabel Maruani7,8,9 Received: 15 May 2018 /Revised: 11 July 2018 /Accepted: 12 July 2018 /Published online: 6 September 2018 # Springer-Verlag GmbH Germany, part of Springer Nature 2018 Abstract Cutaneous spindle-cell neoplasms in adults as well as children represent a frequent dilemma for pathologists. Along this neoplasm spectrum, the differential diagnosis with CD34-positive proliferations can be challenging, particularly concerning neoplasms of fibrohistiocytic and fibroblastic lineages. In children, cutaneous and superficial soft-tissue neoplasms with CD34-positive spindle cells are associated with benign to intermediate malignancy potential and include lipofibromatosis, plaque-like CD34-positive dermal fibroma, fibroblastic connective tissue nevus, and congenital dermatofibrosarcoma protuberans. Molecular biology has been valuable in showing dermatofibrosarcoma protuberans and infantile fibrosarcoma that are characterized by COL1A1-PDGFB and ETV6-NTRK3 rearrangements respectively. We report a case of congenital CD34- positive dermohypodermal spindle-cell neoplasm occurring in a female infant and harboring a novel KHDRBS1-NTRK3 fusion. This tumor could belong to a new subgroup of pediatric cutaneous spindle-cell neoplasms, be an atypical presentation of a plaque-like CD34-positive dermal fibroma, of a fibroblastic connective tissue nevus, or represent a dermatofibrosarcoma protuberans with an alternative gene rearrangement. Keywords Cutaneous . Neoplasms . Spindle-cell Introduction Cutaneous spindle-cell proliferations form a large spectrum of * Annabel Maruani neoplasms occurring in children and adults. -
Radiation-Associated Synovial Sarcoma
Radiation-Associated Synovial Sarcoma: Clinicopathologic and Molecular Analysis of Two Cases Jean-François Egger, M.D., Jean-Michel Coindre, M.D., Jean Benhattar, Ph.D., Philippe Coucke, M.D., Louis Guillou, M.D. University Institute of Pathology (J-FE, JB, LG) and Department of Radiooncology, University Hospital (PC), Lausanne, Switzerland; Bergonié Institute and University of Bordeaux II (J-MC), Bordeaux, France region, or viscera (1, 2). SS bears the t(X;18) (SYT- Development of a soft-tissue sarcoma is an infre- SSX) reciprocal translocation that seems to be spe- quent but well-known long-term complication of cific for this tumor type and can be routinely de- radiotherapy. Malignant fibrous histiocytomas, ex- tected in paraffin-embedded tissue using the traskeletal osteosarcomas, fibrosarcomas, malig- reverse transcriptase–polymerase chain reaction nant peripheral nerve sheath tumors, and angiosar- (RT-PCR; 3–6). Radiation-associated sarcomas are comas are most frequently encountered. Radiation- an infrequent but well-known long-term complica- associated synovial sarcomas are exceptional. We tion of radiotherapy (7–16). They occur in about report the clinicopathologic, immunohistochemi- 1/1000 patients who have undergone radiation cal, and molecular features of two radiation- therapy (7–11). Radiation-associated sarcomas are associated synovial sarcomas. One tumor developed defined as sarcomas arising in a previously irradi- in a 42-year-old female 17 years after external irra- ated field after a latency period of Ն2 years (12). diation was given for breast carcinoma; the other They usually show a more aggressive clinical course occurred in a 34-year-old female who was irradiated associated with shortened patient survival as com- at the age of 7 years for a nonneoplastic condition of pared with sporadic sarcomas (9–12, 14). -
The Identification of a Girl with Tuberous Sclerosis and an Unexpected Large Sized Renal Angiomyolipoma: a Case Report and Literature Review Study
Case Report Annals of Clinical Case Reports Published: 24 Aug, 2020 The Identification of a Girl with Tuberous Sclerosis and an Unexpected Large Sized Renal Angiomyolipoma: A Case Report and Literature Review Study Mitra Naseri1, Farah Ashrafzadeh2, Fatemeh Ghalibafan3* and Paria Dehghanian4 1Department of Pediatric Nephrology, Mashhad University of Medical Sciences, Iran 2Department of Pediatric Neurology, Mashhad University of Medical Sciences, Iran 3Department of Medicine, Mashhad University of Medical Sciences, Iran 4Department of Pathology, Mashhad University of Medical Sciences, Iran Abstract Background: The TSC1 mutated gene, hamartin, and the TSC2 gene, tuberin, cause tuberous sclerosis, the autosomal dominant tumor syndrome. The angiomyolipoma of the kidney is a benign tumor that often happens along with tuberous sclerosis. Large renal angiomyolipoma’s associated with tuberous sclerosis are in danger of fatal hemorrhage. This study reported a case with an unexpected large size of renal angiomyolipoma’s associated with tuberous sclerosis. Case Presentation: We report a girl aged 5 years and 9 months old with tuberous sclerosis who referred to nephrology clinic due to abdominal pain. Physical examination revealed a large mass in the right flank. Kidney ultrasound reported a large mass with approximate size of 90 mm × 60 mm in the right kidney, and abdominal CT scan also showed a non-hemogenic solid mass in the right kidney with size of 91 mm × 68 mm × 67 mm, was highly suspected for Wilms' tumor. Since OPEN ACCESS the large size -
Dermatofibrosarcoma Protuberans of the Parotid Gland -A Case Report
The Korean Journal of Pathology 2004; 38: 276-9 Dermatofibrosarcoma Protuberans of the Parotid Gland -A Case Report - Ok-Jun Lee∙David Y. Pi∙ Dermatofibrosarcoma protuberans (DFSP) typically presents during the early or mid-adult life, Daniel H. Jo∙Kyung-Ja Cho and the most common site of origin is the skin on the trunk and proximal extremities. DFSP of Sang Yoon Kim1∙Jae Y. Ro the parotid gland is extremely rare and only one case has been reported in the literature. We present here a case of a 30-year-old woman with DFSP occurring in the parotid gland, and we Departments of Pathology and discuss the differential diagnosis. The patient is alive and doing well one year after her operation. 1Otolaryngology, University of Ulsan College of Medicine, Asan Medical Center, Seoul, Korea Received : January 27, 2004 Accepted : July 5, 2004 Corresponding Author Jae Y. Ro, M.D. Department of Pathology, University of Ulsan College of Medicine, Asan Medical Center, 388-1 Pungnap-dong, Songpa-gu, Seoul 138-736, Korea Tel: 02-3010-4550 Fax: 02-472-7898 E-mail: [email protected] Key Words : Dermatofibrosarcoma Protuberans-Parotid Gland Epithelial tumors make up the majority of salivary gland neo- CASE REPORT plasms, while mesenchymal tumors of this organ are uncommon. Dermatofibrosarcoma protuberans (DFSP) of the salivary gland A 30-year-old woman came to the Otolaryngology Clinic at is exremely rare and only one case has been reported in the parotid the Asan Medical Center with a 2-year history of a slowly enlarg- gland.1 ing mass inferior to the left angle of the mandible. -
Pediatric Abdominal Masses
Pediatric Abdominal Masses Andrew Phelps MD Assistant Professor of Pediatric Radiology UCSF Benioff Children's Hospital No Disclosures Take Home Message All you need to remember are the 5 common masses that shouldn’t go to pathology: 1. Infection 2. Adrenal hemorrhage 3. Renal angiomyolipoma 4. Ovarian torsion 5. Liver hemangioma Keys to (Differential) Diagnosis 1. Location? 2. Age? 3. Cystic? OUTLINE 1. Kidney 2. Adrenal 3. Pelvis 4. Liver OUTLINE 1. Kidney 2. Adrenal 3. Pelvis 4. Liver Renal Tumor Mimic – Any Age Infection (Pyelonephritis) Don’t send to pathology! Renal Tumor Mimic – Any Age Abscess Don’t send to pathology! Peds Renal Tumors Infant: 1) mesoblastic nephroma 2) nephroblastomatosis 3) rhabdoid tumor Child: 1) Wilm's tumor 2) lymphoma 3) angiomyolipoma 4) clear cell sarcoma 5) multilocular cystic nephroma Teen: 1) renal cell carcinoma 2) renal medullary carcinoma Peds Renal Tumors Infant: 1) mesoblastic nephroma 2) nephroblastomatosis 3) rhabdoid tumor Child: 1) Wilm's tumor 2) lymphoma 3) angiomyolipoma 4) clear cell sarcoma 5) multilocular cystic nephroma Teen: 1) renal cell carcinoma 2) renal medullary carcinoma Renal Tumors - Infant 1) mesoblastic nephroma 2) nephroblastomatosis 3) rhabdoid tumor Renal Tumors - Infant 1) mesoblastic nephroma 2) nephroblastomatosis 3) rhabdoid tumor - Most common - Can’t distinguish from congenital Wilms. Renal Tumors - Infant 1) mesoblastic nephroma 2) nephroblastomatosis 3) rhabdoid tumor Look for Multiple biggest or diffuse and masses. ugliest. Renal Tumors - Infant 1) mesoblastic -
NY-ESO-1 (CTAG1B) Expression in Mesenchymal Tumors
Modern Pathology (2015) 28, 587–595 & 2015 USCAP, Inc. All rights reserved 0893-3952/15 $32.00 587 NY-ESO-1 (CTAG1B) expression in mesenchymal tumors Makoto Endo1,2,7, Marieke A de Graaff3,7, Davis R Ingram4, Simin Lim1, Dina C Lev4, Inge H Briaire-de Bruijn3, Neeta Somaiah5, Judith VMG Bove´e3, Alexander J Lazar6 and Torsten O Nielsen1 1Department of Pathology and Laboratory Medicine, University of British Columbia, Vancouver, British Columbia, Canada; 2Department of Orthopaedic Surgery, Kyushu University, Fukuoka, Japan; 3Department of Pathology, Leiden University Medical Center, Leiden, The Netherlands; 4Department of Surgical Oncology, The University of Texas MD Anderson Cancer Center, Houston, TX, USA; 5Department of Sarcoma Medical Oncology, The University of Texas MD Anderson Cancer Center, Houston, TX, USA and 6Department of Pathology, The University of Texas MD Anderson Cancer Center, Houston, TX, USA New York esophageal squamous cell carcinoma 1 (NY-ESO-1, CTAG1B) is a cancer-testis antigen and currently a focus of several targeted immunotherapeutic strategies. We performed a large-scale immunohistochemical expression study of NY-ESO-1 using tissue microarrays of mesenchymal tumors from three institutions in an international collaboration. A total of 1132 intermediate and malignant and 175 benign mesenchymal lesions were enrolled in this study. Immunohistochemical staining was performed on tissue microarrays using a monoclonal antibody for NY-ESO-1. Among mesenchymal tumors, myxoid liposarcomas showed the highest positivity for NY-ESO-1 (88%), followed by synovial sarcomas (49%), myxofibrosarcomas (35%), and conventional chondrosarcomas (28%). Positivity of NY-ESO-1 in the remaining mesenchymal tumors was consistently low, and no immunoreactivity was observed in benign mesenchymal lesions.