DOCSLIB.ORG

Non Germ Cell Tumours Hong Kong 2021

Total Page：16

File Type：pdf, Size：1020Kb

Download full-text PDF Read full-text
Non Germ Cell Tumours Hong Kong 2021 Non Germ cell tumours Hong Kong 2021 Sex cord stromal tumours • Leydig Cell Tumour – Malignant Leydig cell tumour • Sertoli Cell Tumour – Malignant Sertoli cell tumour – Large cell calcifying Sertoli cell tumour – Intratubular large cell hyalinising Sertoli cell tumour • Granulosa Cell Tumour – Adult – Juvenile • Tumours of Fibroma/thecoma group • Mixed/Unclassified • Tumours with both sex cord and germ cell elements Sex cord stromal lesions Leydig Sertoli Granulosa Sertoli/Leydig Fibroma thecoma Coelomic mesothelium = Sex cords = Sertoli cells Mesenchyme = Stroma = Leydig cells Leydig cell tumours • Typical appearance • Oncocytic • Reinke Crystalloids • Lack of GCNIS • 30% have endocrinopathy • Children 20% – German Zoologist 1821-1908 REINKE, F . 1896 . Beiträge zur Histologie des Menschen. Arch . Mikroskop . Anat. 47 :34 German anatomist, 1862-1919 Increase in incidental lesions in the testis. • Victim Of Modern Imaging Technology • VOMIT • Brainless Application of Radiological Findings • BARF Leydig cell Malignancy Criteria • Size • Invasiveness • Mitoses • Necrosis • RPLND?? Leydig cell hyperplasia and variants • High hCG • Klinefelter’s syndrome • Bilateral nodularity • Not a reason for Orchidectomy • Cord and tunica involvement • Congenital Adrenal Hyperplasia – Testicular Adrenal Rest Tumours (TART) Testicular tumor of adrenogenital syndrome (Testicular adrenal rest tumor) • Congenital adrenal hyperplasia • Florid hyperplasia of Leydig like cells • Responsive to high dose steroids • Mediacl treatment sufficient! Sertoli Cell Tumors • Sometimes oestrogens • Variable pattern • Distict nucleoli usually. • Tubules, retiform, solid, clear cells • Charcot-Böttcher filaments • Any tubular component • Enrico Sertoli 1865 Italian Physiologist • Arthur Bottcher 1813-89 Baltic Pathologist • Jean-Martin Charcott 1825-1893 French neurologist Kao CS, Kum JB, Idrees MT, Ulbright TM. Sclerosing Sertoli cell tumor of the testis: a clinicopathologic study of 20 cases. Am J Surg Pathol. 2014 Apr;38(4):510-7. Henley JD, Young RH, Ulbright TM. Malignant Sertoli cell tumors of the testis: a study of 13 examples of a neoplasm frequently misinterpreted as seminoma. Am J Surg Pathol. 2002 May;26(5):541-50. Contacted clinicians • Further investigations • Goitre • Nodular adrenals • Previous myxoma • Lip pigmentation • Acromegaly Diagnosis • Multiple Large cell calcifying Sertoli cell tumors secondary to Carney complex Large cell calcifying SCTs • Carney complex: myxomas, lentigines, blue naevi, Cushing's 2ary to PANH, PRKAR1A germ line mutation • pituitary adenomas. • Younger age Peutz Jeghers Syndrome • STK11germ line mutations on chromosome 17 • Autosomal dominant • Muco-cutaneous lesions • Hamatomatous polyps • Testicular lesions… Intratubular large cell Hyalinising Sertoli cell tumours • Peutz-Jeghers Syndrome • Intra tubular growth • Less calcification • Largely in paediatric population 2 Sertoli cell adenoma/hyperplasia • Cryptorchidism • ‘Pick’s’ adenoma • Androgen insensitivity Granulosa cell tumours • Usual type – Rare in pure form: morphology. – Usually benign • Juvenile – Commonest testis tumour in infants – XX/XY mosaicism – Invariably benign • Fibroma/thecomas very rare on own. Cornejo KM, Young RH. Adult granulosa cell tumors of the testis: a report of 32 cases. Am J Surg Pathol. 2014 Sep;38(9):1242-50. Kao CS, Cornejo KM, Ulbright TM, Young RH. Juvenile granulosa cell tumors of the testis: a clinicopathologic study of 70 cases with emphasis on its wide morphologic spectrum. Am J Surg Pathol. 2015 Sep;39(9):1159-69. Mixed sex cord stromal tumours • More common. • Benign under 10 • Malignancy reported in older group Immunochemistry • Germ cell markers negative • Inhibin variable • Calretinin variable • Actin spindle elements • CD99 variable • S100 Tumour containing both germ cell and sex cord-stromal elements • Gonadoblastoma • 20% phenotypically male • Most <20 years of age • Cryptorchidism, hypospadias and gynecomastia • May present in children under age 2 • Failure of involution of müllerian ducts Tips for diagnosis • Think of hyperplasia. • Syndromes. • Always look for GCNIS. • Check nuclear morphology. • Immunochemistry. Tumour containing both germ cell and sex cord-stromal elements • Gonadoblastoma • 20% phenotypically male • Most <20 years of age • Cryptorchidism, hypospadias and gynecomastia • May present in children under age 2 • Failure of involution of Müllerian ducts Presentation • Usually young males with feminizing symptoms. • Often persistent mullerian duct structures • Karyotype can be normal but always a Y chromosome. Newly added 2016 WHO Entities • Hemangioma • Juvenile Xanthogranuloma Hemangioma • Rare • Well circumscribed • IHC CD31, FLI1 and ERG. • Benign Juvenile Xanthogranuloma • Neoplastic ? • May progress to multiple sites • Children (<13 months) • mononuclear histiocyte like cells • diffuse infiltrative pattern Other Tumours of the Testicular Adnexa and Paratesticular Tissue • Rete Testis – Hyperplasia/adenoma – Adenocarcinoma • Epididymis – Clear cell papillary hyperplasia – Papillary cystadenoma and carcinoma • Lesions of mesothelial origin – Papillary hyperplasia – Adenomatoid tumor – Mesothelioma • Tumors of mullerian type epithelium • Desmoplastic small round cell tumour Rete Testis Benign Lesions • Hyperplasia • Adenomatous hyperplasia • Adenoma – Adenoma of usual type – Sertoliform cystadenoma – Adenofibroma • Morphologic criteria – Circumscribed – Cystic or solid – Cytologically bland Rete hyperplasia and metaplasia Sertoliform cystadenoma Rete Testis Adenocarcinoma Clinical Features Morphology • Adult males • Grossly solid or cystic • Tubulopapillary • Unilateral hilar mass • Solid, spindle, retiform • Painless testicular • Cuboidal to columnar cells swelling • Eosinophilic to basophilic cytoplasm • Hydrocele • Moderate to marked • Poor survival pleomorphism Diagnostic Criteria • Hilar location • Normal to malignant transition • No other primary • Morphology incompatible with other intrascrotal primary • Immunohistochemical studies Nochomovitz and Orenstein; AJSP 1984 Ulbright and Young; AFIP series 4, 2013 Immunoprofile • Positive: CK7, AE1/AE3, WT1 CKBE312, and calretinin focal • Negative: CK20, CD15, p63, OCT4, CEA, inhibin, and CD30 CKBE312 CK7 CK32B AE1/AE3AE1/AE3 E12 WT1WT1 Treatment and Prognosis • Radical orchiectomy • RPLND ? • No proven benefit for chemotherapy and radiation • Overall prognosis is poor (5 year at 15%) • Larger size – worse prognosis Papillary cystadenoma of epididymis Serous borderline tumor Adenomatoid Tumour Adenomatoid Tumour Malignant mesothelioma of tunica vaginalis • Occur from childhood elderly. • Mean age of 60 year • Some associated with asbestos exposure • Hydrocele • Tunica is thickened and often studded by papillary tumor and coats the TV • Pattern is usually purely epithelioid • Cytologic atypia, mitotic activity, and necrosis are variably prominent. Calretinin Malignant mesothelioma of tunica vaginalis • 20 cases : IHC was similar to that of malignant mesothelioma in the pleura • CK5/6 staining could be patchy in some tumors • Radical orchidectomy is the usual treatment • Some patients possibly benefiting from RPLND. • After a median follow-up of 22 months, 50% of 30 patients were disease free after treatment . • Division of malignant mesotheliomas into high-grade and low- grade categories based on pathologic and clinical evaluation may aid in prognostication • Versus mesothelial hyperplasia: gross mass, complex arborizing papillae with fibrovascular cores, invasive growth, and a less frequent inflammatory background. Well-differentiated Papillary Mesothelioma • No longer termed “benign” • Identical tumors have shown invasive foci or progression in other sites – Gallateau-Salle et al. Am J Surg Pathol 2004;28:534 – Churg et al. Am J Surg Pathol 2014;38:990 Well-differentiated Papillary Mesothelioma Well-differentiated Papillary Mesothelioma Well-differentiated Papillary Mesothelioma Papillary serous v Mesothelioma • Thinner papillae • Less cellular budding and stratification • Fewer psammoma bodies • Immunohistochemistry: papillary serous tumors typically being positive with B72.3, PLAP, Leu- M1, CA125, and sometimes CEA Rhabdomyosarcoma • Most common malignant tumor of the spermatic cord • Peak incidence at 9 yrs but also teens • Generally conventional embryonal rhabdomyosarcomas • Many have spindle cell morphology A small % are of the alveolar or pleomorphic • IHC; MyoD1, myogenin, desmin, and muscle- specific actin. Myoglobin stains poorly sensitive but specific. Rhabdomyosarcoma Rhabdomyosarcoma • Radical orchiectomy with RPLND is the usual treatment, with chemotherapy and radiation therapy • Overall survival rate of approximately 80% can be expected Spindle cell subtype has a better outcome. • Complete local resectability and an absence of parenchymal metastases are important favorable prognostic features. • Wraps around testicular structures • Differentiate from transformation of GCT Deep (Aggressive) angiomyxoma • More common in vulva • Locally aggressive • V rare in men • HMGA2 more specific (gene) • ER/PR • Local recurrence common • WLE recommended Deep (Aggressive) angiomyxoma Cellular Angiofibroma Angiomyofibroblastoma-like tumor • Benign paratesticular/spermatic cord • circumscription • More superficial location, • mixture of spindle and plump cells, • zonal cellularity, • vessels with hyalinized or fibrinoid change • different from the similarly named vulvar tumor • cellular angiofibroma .
Load more

Recommended publications
  • Huge Ovarian Sertoli-Leydig Cell Tumor- a Rare Presentation Mimicking Advanced Ovarian Carcinoma: a Clinical Diagnostic Pitfall
    International Journal of Health Sciences and Research Vol.10; Issue: 5; May 2020 Website: www.ijhsr.org Case Report ISSN: 2249-9571 Huge Ovarian Sertoli-Leydig Cell Tumor- A Rare Presentation Mimicking Advanced Ovarian Carcinoma: A Clinical Diagnostic Pitfall Ikeanyi M Eugene1, Udoye P Ezenwa2, Jeremiah Israel1 1Department of Obstetrics and Gynecology, Niger Delta University Teaching Hospital, Okolobiri Bayelsa State Nigeria 2Department of Pathology, Niger Delta University Teaching Hospital, Okolobiri Bayelsa State Nigeria Corresponding Author: Ikeanyi M Eugene ABSTRACT Sertoli-Leydig cell tumor is a very rare ovarian tumor constituting less than 0.5% of all primary ovarian tumors. It mostly occurs in second and third decades of life. This is a case report of a rare presentation of a huge ovarian Sertoli-Leydig cell tumor presenting like an advanced ovarian cancer in a 62 year old seven years postmenopausal para eight woman. At surgery was a left well encapsulated multilobulated ovarian tumour measuring 28 x28x14cm, weighing 6.2kg and histologically containing clusters of Leydig cells and solid cords of Sertoli cells of intermediate differentiation. The patient presented with a year history of progressive abdominal swelling and irregular vaginal bleeding. She had total abdominal hysterectomy and bilateral salpingo-oophorectomy. About a year on follow- up and stable. Keywords: Sertoli-Leydig cell, sex cord, stromal, ovarian, tumor, postmenopausal, neoplasm INTRODUCTION but rarely in any age. It can contain Ovarian Sertoli-Leydig cell tumor is heterologous elements and be functionally one of the categories of sex cord-stromal diverse. It contains testicular structures that tumors of ovary; defined by World Health secrete androgen with varying degrees of Organization (WHO) as groups of tumors virilization based on the quantity of secreted composed of granulosa cells, theca cells, androgen.
    [Show full text]
  • Adenomatoid Tumor of the Skin: Differential Diagnosis of an Umbilical Erythematous Plaque
    Title: Adenomatoid tumor of the skin: differential diagnosis of an umbilical erythematous plaque Keywords: Adenomatoid tumor, skin, umbilicus Short title: Adenomatoid tumor of the skin Authors: Ingrid Ferreira1, Olivier De Lathouwer2, Hugues Fierens3, Anne Theunis1, Josette André1, Nicolas de Saint Aubain3 1Dermatopathology laboratory, Department of Dermatology, Saint-Pierre University Hospital, Université Libre de Bruxelles, Brussels, Belgium. 2Department of Plastic surgery, Centre Hospitalier Interrégional Edith Cavell, Waterloo, Belgium. 3Department of Dermatology, Saint-Jean Hospital, Brussels, Belgium. 4Department of Pathology, Jules Bordet Institute, Université Libre de Bruxelles, Brussels, Belgium. Acknowledgements: None Corresponding author: Ingrid Ferreira This article has been accepted for publication and undergone full peer review but has not been through the copyediting, typesetting, pagination and proofreading process which may lead to differences between this version and the Version of Record. Please cite this article as doi: 10.1111/cup.13872 This article is protected by copyright. All rights reserved. Abstract Adenomatoid tumors are benign tumors of mesothelial origin that are usually encountered in the genital tract. Although they have been observed in other organs, the skin appears to be a very rare location with only one case reported in the literature to our knowledge. We report a second case of an adenomatoid tumor, arising in the umbilicus of a 44-year-old woman. The patient presented with an 8 months old erythematous and firm plaque under the umbilicus. A skin biopsy showed numerous microcystic spaces dissecting a fibrous stroma and being lined by flattened to cuboidal cells with focal intraluminal papillary formation. This poorly known diagnosis constitutes a diagnostic pitfall for dermatopathologists and dermatologists, and could be misdiagnosed as other benign or malignant entities.
    [Show full text]
  • 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
    [Show full text]
  • Use of Novel Serum Markers in Clinical Follow-Up of Sertoli-Leydig Cell Tumours
    CORE Metadata, citation and similar papers at core.ac.uk Article in press - uncorrected proof Provided by Open Access LMU Clin Chem Lab Med 2007;45(5):657–661 ᮊ 2007 by Walter de Gruyter • Berlin • New York. DOI 10.1515/CCLM.2007.120 2006/514 Short Communication Use of novel serum markers in clinical follow-up of Sertoli-Leydig cell tumours Miriam Lenhard1,*, Caroline Kuemper1, Nina Keywords: ovarian malignancy; Sertoli-Leydig cell Ditsch1, Joachim Diebold2, Petra Stieber3, tumour; serum marker; sex-cord stromal tumour. Klaus Friese1 and Alexander Burges1 1 Department of Obstetrics and Gynaecology, Sertoli-Leydig cell tumours are classified as sex-cord Campus Grosshadern, Ludwig-Maximilians- stromal tumours. They account for only 0.2% of University, Munich, Germany malignant ovarian tumours and are often found uni- 2 Department of Pathology, Ludwig-Maximilians- laterally (1). Synonyms in the literature are arrheno- University, Munich, Germany blastoma, androblastoma and gonadal stromal 3 Department of Clinical Chemistry, Ludwig- tumour of the android type. Most of these tumours Maximilians-University, Munich, Germany are described in young adults and less than 10% occur prior to menarche or after menopause (2). Two- thirds of all patients are diagnosed with this rare dis- Abstract ease due to the tumour’s hormone production (3). A 41-year-old patient (IV gravida, IV para) presented Background: Sertoli-Leydig cell tumours of the ovary with dyspnoea, enlarged abdominal girth and mela- account for only 0.2% of malignant ovarian tumours. ena. On physical examination, the abdomen was dis- Two-thirds of all patients become apparent due to the tended with a fluid wave.
    [Show full text]
  • Fatal Haemorrhage and Neoplastic Thrombosis in a Captive African Lion
    Gonzales‑Viera et al. Acta Vet Scand (2017) 59:69 DOI 10.1186/s13028-017-0337-5 Acta Veterinaria Scandinavica CASE REPORT Open Access Fatal haemorrhage and neoplastic thrombosis in a captive African lion (Panthera leo) with metastatic testicular sex cord–stromal tumour Omar Antonio Gonzales‑Viera1,2, Angélica María Sánchez‑Sarmiento1* , Natália Coelho Couto de Azevedo Fernandes3, Juliana Mariotti Guerra3, Rodrigo Albergaria Ressio3 and José Luiz Catão‑Dias1 Abstract Background: The study of neoplasia in wildlife species contributes to the understanding of cancer biology, manage‑ ment practices, and comparative pathology. Higher frequencies of neoplasms among captive non-domestic felids have been reported most commonly in aging individuals. However, testicular tumours have rarely been reported. This report describes a metastatic testicular sex cord–stromal tumour leading to fatal haemorrhage and thrombosis in a captive African lion (Panthera leo). Case presentation: During necropsy of a 16-year-old male African lion, the left testicle and spermatic cord were found to be intra-abdominal (cryptorchid), semi-hard and grossly enlarged with multiple pale-yellow masses. Encap‑ sulated haemorrhage was present in the retroperitoneum around the kidneys. Neoplastic thrombosis was found at the renal veins opening into the caudal vena cava. Metastases were observed in the lungs and mediastinal lymph nodes. Histology revealed a poorly diferentiated pleomorphic neoplasm comprised of round to polygonal cells and scattered spindle cells with eosinophilic cytoplasm. An immunohistochemistry panel of inhibin-α, Ki-67, human placental alkaline phosphatase, cytokeratin AE1/AE3, cKit, vimentin and S100 was conducted. Positive cytoplasmic immunolabeling was obtained for vimentin and S100. Conclusions: The gross, microscopic and immunohistochemical fndings of the neoplasm were compatible with a poorly diferentiated pleomorphic sex cord–stromal tumour.
    [Show full text]
  • Concurrent Hepatic Adenomatoid Tumor and Hepatic Hemangioma: a Case Report
    pISSN 2287-2728 eISSN 2287-285X Case Report http://dx.doi.org/10.3350/cmh.2012.18.2.229 Clinical and Molecular Hepatology 2012;18:229-234 Concurrent hepatic adenomatoid tumor and hepatic hemangioma: a case report Ji-Beom Kim1, Eunsil Yu2, Ju-Hyun Shim1, Gi-Won Song3, Gwang Un Kim1, Young-Joo Jin1, and Ho-Seop Park2 1Department of Internal Medicine, 2Department of Pathology, and 3Division of Hepatobiliary Surgery and Liver Transplantation, Department of Surgery, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea A 45-year-old male with alleged asymptomatic hepatic hemangioma of 4 years duration had right upper-quadrant pain and was referred to a tertiary hospital. Computed tomography and magnetic resonance imaging scans revealed a hypervascular mass of about 7 cm containing intratumoral multilobulated cysts. A preoperative liver biopsy was performed, but this failed to provide a definitive diagnosis. The patient underwent a partial hepatectomy of segments IV and VIII. The histologic findings revealed multifocal proliferation of flattened or cuboidal epithelioid cells and a highly vascular edematous stroma. Immunohistochemistry findings demonstrated that the epithelioid tumor cells were positive for cytokeratin (AE1/AE3), vimentin, calretinin, and cytokeratin 5/6, and were focally positive for CD10, and negative for WT1 and CD34, all of which support their mesothelial origin. Immunohistochemistry for a mesothelial marker should be performed for determining the presence of an adenomatoid tumor when benign epithelioid
    [Show full text]
  • The Role of Cytogenetics and Molecular Diagnostics in the Diagnosis of Soft-Tissue Tumors Julia a Bridge
    Modern Pathology (2014) 27, S80–S97 S80 & 2014 USCAP, Inc All rights reserved 0893-3952/14 $32.00 The role of cytogenetics and molecular diagnostics in the diagnosis of soft-tissue tumors Julia A Bridge Department of Pathology and Microbiology, University of Nebraska Medical Center, Omaha, NE, USA Soft-tissue sarcomas are rare, comprising o1% of all cancer diagnoses. Yet the diversity of histological subtypes is impressive with 4100 benign and malignant soft-tissue tumor entities defined. Not infrequently, these neoplasms exhibit overlapping clinicopathologic features posing significant challenges in rendering a definitive diagnosis and optimal therapy. Advances in cytogenetic and molecular science have led to the discovery of genetic events in soft- tissue tumors that have not only enriched our understanding of the underlying biology of these neoplasms but have also proven to be powerful diagnostic adjuncts and/or indicators of molecular targeted therapy. In particular, many soft-tissue tumors are characterized by recurrent chromosomal rearrangements that produce specific gene fusions. For pathologists, identification of these fusions as well as other characteristic mutational alterations aids in precise subclassification. This review will address known recurrent or tumor-specific genetic events in soft-tissue tumors and discuss the molecular approaches commonly used in clinical practice to identify them. Emphasis is placed on the role of molecular pathology in the management of soft-tissue tumors. Familiarity with these genetic events
    [Show full text]
  • Microrna Dysregulation Interplay with Childhood Abdominal Tumors
    Cancer and Metastasis Reviews (2019) 38:783–811 https://doi.org/10.1007/s10555-019-09829-x MicroRNA dysregulation interplay with childhood abdominal tumors Karina Bezerra Salomão1 & Julia Alejandra Pezuk2 & Graziella Ribeiro de Souza1 & Pablo Chagas1 & Tiago Campos Pereira3 & Elvis Terci Valera1 & María Sol Brassesco3 Published online: 17 December 2019 # Springer Science+Business Media, LLC, part of Springer Nature 2019 Abstract Abdominal tumors (AT) in children account for approximately 17% of all pediatric solid tumor cases, and frequently exhibit embryonal histological features that differentiate them from adult cancers. Current molecular approaches have greatly improved the understanding of the distinctive pathology of each tumor type and enabled the characterization of novel tumor biomarkers. As seen in abdominal adult tumors, microRNAs (miRNAs) have been increasingly implicated in either the initiation or progression of childhood cancer. Moreover, besides predicting patient prognosis, they represent valuable diagnostic tools that may also assist the surveillance of tumor behavior and treatment response, as well as the identification of the primary metastatic sites. Thus, the present study was undertaken to compile up- to-date information regarding the role of dysregulated miRNAs in the most common histological variants of AT, including neuroblas- toma, nephroblastoma, hepatoblastoma, hepatocarcinoma, and adrenal tumors. Additionally, the clinical implications of dysregulated miRNAs as potential diagnostic tools or indicators of prognosis were evaluated. Keywords miRNA . Neuroblastoma . Nephroblastoma . Hepatoblastoma . Hepatocarcinoma . Adrenal tumors 1 MicroRNA biogenesis and function retaining the hairpin (pre-miRNA, ~ 70 nucleotides long), which is translocated by Exportin-5 to the cytoplasm, and then Fundamentally, all cellular programs are controlled by genes: processed by another endoribonuclease (Dicer) into the growth, senescence, division, metabolism, stemness, mobility, miRNA duplex.
    [Show full text]
  • Adenomatoid Tumour of the Adrenal Gland: a Case Report and Literature Review
    CORE Metadata, citation and similar papers at core.ac.uk Provided by Jagiellonian Univeristy Repository POL J PATHOL 2010; 2: 97–102 ADENOMATOID TUMOUR OF THE ADRENAL GLAND: A CASE REPORT AND LITERATURE REVIEW MAGDALENA BIAŁAS1, WOJCIECH SZCZEPAŃSKI1, JOANNA SZPOR1, KRZYSZTOF OKOŃ1, MARTA KOSTECKA-MATYJA2, ALICJA HUBALEWSKA-DYDEJCZYK2, ROMANA TOMASZEWSKA1 1Chair and Department of Pathomorphology, Jagiellonian University Medical College, Kraków 2Chair and Department of Endocrinology, Jagiellonian University Medical College, Kraków Adenomatoid tumour (AT) is a rare, benign neoplasm of mesothelial origin, which usually occurs in the genital tract of both sexes. Occasionally these tumours are found in extra genital locations such as heart, pancreas, skin, pleura, omentum, lymph nodes, retroperitoneum, intestinal mesentery and adrenal gland. Histologically ATs show a mixture of solid and cystic patterns usually with focal presence of signet-ring like cells and scattered lymphoid infiltration. The most important thing about these tumours is not to misdiagnose them as primary malignant or metastatic neoplasms. We present a case of an adrenal AT in a 29-year-old asymptomatic male. The tumour was an incidental finding during abdominal CT-scan for an unrelated condition. We also present a review of the literature concerning adrenal gland AT and give possible differential diagnosis. Key words: adenomatoid tumour, adrenal gland, immunophenotype. Introduction histopathological examination. Additional sections were made for immunohistochemical analysis (for Adenomatoid tumour (AT) is a benign neoplasm details see Table I). of mesothelial origin [1-3]. The usual place of its Proliferative activity of the tumour was deter- appearance is the male and female genital tract, most mined using immunohistochemical staining for often epididymis in men and fallopian tube in women nuclear protein MIB-1 (Ki-67).
    [Show full text]
  • WHO 2016 Classification of Tumours of the Testis
    WHO 2016 classification of tumours of the testis Dan Berney BAUP/BDIAP 2015 WHO Zurich March 2015 Nomenclature precursor Germ Cell Tumour (GCT) testis CIS IGCNU TIN • CIS – Not a carcinoma • TIN – Not intraepithelial • IGCNU – Unclassified/Undifferentiated… – The spermatogonial niche PLAP CIS IGCNU IGCNU IGCN GCNI GCNIS GCNIS GERM CELL NEOPLASIA IN SITU WHO 2016 Germ cell tumours • Tumours derived from GCNIS of one type • Seminoma • Embryonal carcinoma • Yolk Sac Tumour, post pubertal type • Trophoblastic tumours • Teratoma, post pubertal type • Teratoma with somatic type malignancy Seminoma hCG I am not a choriocarcinoma OCT3/4 Anaplastic seminoma? • ‘Differentiation’ of seminomas • Mitotic rate • Lymphocytic infiltrate • Cell morphology Embryonal carcinoma Hepatoid YST Glandular YST Parietal Solid YST Trophoblastic tumours • Choriocarcioma • Non-choriocarcinomatous trophoblastic tumours – Placental site trophoblastic tumour – Epithelioid trophoblastic tumour – Cystic trophoblastic tumour Choriocarcinoma ETT • Gestational trophoblastic tumor with proposed origin from intermediate trophoblastic cells of the chorionic laeve • Squamoid monophasic trophoblast cells in cohesive epithelioid nests with abundant eosinophilic cytoplasm • Lacking the biphasic pattern characteristic of choriocarcinoma • Prominent cell boundaries, intracytoplasmic, and extracytoplasmic eosinophilic fibrinoid and globular material Immunoprofile CTT ETT Chorioca. Inhibin ++ ++ +/- p63 -- ++ - hCG ++ ++ +++ HPLC +/- ++ +++ Ki-67 <5% >10% >10% Teratoma, post pubertal
    [Show full text]
  • Aggressive Angiomyxoma in Pregnancy: a Rare and Commonly Misdiagnosed Entity Kamal Malukani, Amit V
    Published online: 2020-02-19 Case Report Access this article online Quick Response Code: Aggressive angiomyxoma in pregnancy: A rare and commonly misdiagnosed entity Kamal Malukani, Amit V. Varma, Devashish Choudhary, Shilpi Dosi Website: www.jlponline.org Abstract: DOI: 10.4103/JLP.JLP_179_17 Aggressive angiomyxoma (AAM) is an uncommon mesenchymal tumor that predominantly involves the pelvis and perineum of young females. It is often clinically mistaken for more common superficial lesions such as vaginal cysts, labial cysts, and lipomas. A review of the medical literature reveals very few cases of AAM reported in pregnancy. We describe a rare case of AAM in pregnancy, clinically misdiagnosed as prolapsed cervical fibroid. Key words: Aggressive angiomyxoma, mesenchymal tumors, pregnancy Introduction (G2 P1 L1), married for 5 years, presented with bleeding per vagina for 3 days. She ggressive angiomyxoma (AAM) is also complained of huge mass coming out Aa rare mesenchymal neoplasm of of vagina, difficulty in walking, and pain vulvo‑perineal region and usually found in abdomen and lower back. There was [1] in women of reproductive age group. It no history of fever, weight loss, trauma, is a slow‑growing, low‑grade neoplasm bowel or bladder disturbance, and use of but is locally infiltrative, and relapse any contraceptive. Her menstrual history has been reported in about 30%–40% of and past history were unremarkable. Local cases even after many years of complete examination showed a large pedunculated, resection.[2] Rarely, AAM can metastasize well‑circumscribed, ulcerated mass coming to lungs, peritoneum, and lymph nodes.[3] out of the vagina. Only a few cases of AAM coexistent Ultrasound of abdomen showed with pregnancy are reported in medical bulky anteverted uterus, measuring literature.[4,5] AAM grows to a huge size 9.6 cm × 7.6 cm × 5.4 cm.
    [Show full text]
  • Desterrando El Término Ewing-Like Sarcoma: ¿Qué Entidades Se Cobijaban Bajo Esa Denominación?
    Desterrando el término Ewing-like sarcoma: ¿Qué entidades se cobijaban bajo esa denominación? Sílvia Bagué Servei de Patologia Hospital de la Santa Creu i Sant Pau Universitat Autònoma de Barcelona #SEOM20 No disclosures Small round cell sarcomas (SRCS) - Group of malignant neoplasms sharing histological similar features - Mainly in children & young adults - High-grade by definition. Often “translocated-sarcomas” • Ewing sarcoma • “Ewing-like” sarcomas • Desmoplastic SRCT • Alveolar rhabdomyosarcoma • Poorly diff sinovial sarcoma • Mesenchymal chondrosarcoma • High-grade myxoid liposarcoma • Small cell osteosarcoma • Undiff sarcoma with round cell morphology (USRCS) #SEOM203 Ewing sarcoma • Most common round cell sarcoma • 1st - 2nd decade • 85% diaphysis-metaphysis long bones > pelvis, ribs, chest wall (‘Askin’) • 15% extraeskeletal (adults) • Key morphologic features: uniform monotonous round cells, fine chromatin, inconspicious nucleoli, scant clear to pale cytoplasm • Key immunohistochemical stains: strong and diffuse membranous CD99 expression; NKX2+, PAX7+ • Genetics: fusion between EFT genes (FUS, EWSR1) and TAF15 genes and ETS transcription factor family members (Fli-1, ERG, ETV1, ETV4, FEV, E1AF, ZSG) 85-90% t(11;22)(q24;q12) EWSR1- FLI1 fusion gene 5-10 % t(21;22)(q22;q12) EWSR1-ERG fusion gene CD99 FISH EWSR1 #SEOM204 Desmoplastic small round cell tumor Clinical history Boy, 12 y-o. Intraabdominal mass #SEOM205 DSRCT: IHC & genetics Malignant round cell tumor with desmoplastic stroma, poliphenotypic differentiation and translocation
    [Show full text]