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Difference between revisions of "Sertoli-Leydig cell tumour"
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Sertoli-Leydig cell tumour (view source)
Revision as of 09:43, 11 September 2018
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**75% younger than 30 years of age | **75% younger than 30 years of age | ||
**10% over 50 years of age. | **10% over 50 years of age. | ||
*[[DICER1 mutation]] common in moderately and poorly differentiated Sertoli-Leydig cell tumours.<ref name=pmid28654427>{{Cite journal | last1 = de Kock | first1 = L. | last2 = Terzic | first2 = T. | last3 = McCluggage | first3 = WG. | last4 = Stewart | first4 = CJR. | last5 = Shaw | first5 = P. | last6 = Foulkes | first6 = WD. | last7 = Clarke | first7 = BA. | title = DICER1 Mutations Are Consistently Present in Moderately and Poorly Differentiated Sertoli-Leydig Cell Tumors. | journal = Am J Surg Pathol | volume = 41 | issue = 9 | pages = 1178-1187 | month = Sep | year = 2017 | doi = 10.1097/PAS.0000000000000895 | PMID = 28654427 }}</ref> | |||
==Microscopic== | ==Microscopic== | ||
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*** Irregular nuclei with irregular/vacuolated-appearing chromatin. | *** Irregular nuclei with irregular/vacuolated-appearing chromatin. | ||
*** Architecture: tubules, cords or sheets. | *** Architecture: tubules, cords or sheets. | ||
****Classic Sertoli tubule shows an 'antipodal arrangement of nuclei' | ****Classic Sertoli tubule shows an 'antipodal arrangement of nuclei' | ||
*****Nuclei sit near the basement membrane away from the tubule lumen. | |||
*****A fair bit of cytoplasm sits above the nucleus. | |||
*****Lumen is round. | |||
***Mitotic activity may be much lower than expected for the degree of atypia (in comparison to adenocarcinoma). | ***Mitotic activity may be much lower than expected for the degree of atypia (in comparison to adenocarcinoma). | ||
**Stroma | |||
***Varies from fibrous pink stroma in well differentiated tumors to cellular primative stroma in poorly differentiated tumors. | |||
***+/-Stromal edema may be prominent | |||
*Growth Patterns: | *Growth Patterns: | ||
**Well-differentiated. | **Well-differentiated. | ||
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***Tubules may be a very minor element. | ***Tubules may be a very minor element. | ||
***Poorly differentiated tumours have sarcomatous features.<ref name=Ref_PBoD1103>{{Ref PBoD|1103}}</ref> | ***Poorly differentiated tumours have sarcomatous features.<ref name=Ref_PBoD1103>{{Ref PBoD|1103}}</ref> | ||
**Retiform.<ref>{{Cite journal | last1 = Young | first1 = RH. | last2 = Scully | first2 = RE. | title = Ovarian Sertoli-Leydig cell tumors with a retiform pattern: a problem in histopathologic diagnosis. A report of 25 cases. | journal = Am J Surg Pathol | volume = 7 | issue = 8 | pages = 755-71 | month = Dec | year = 1983 | doi = | PMID = 6660351 }}</ref> | |||
***Tumour resembles rete testis/ovary with an irregular network of elongated slit-like tubules and cysts, which may contain papillae. | |||
**With heterologous element. | **With heterologous element. | ||
***Mucinous intestinal-type epithelium, cartilage, skeletal muscle. | ***Mucinous intestinal-type epithelium, cartilage, skeletal muscle. | ||
***Heterologous elements can | ***Heterologous elements can occur in retiform or poorly differentiated tumours. | ||
***+/-Sarcomatous features (mucinous glands, bone, cartilage). | |||
** | |||
*+/-Sarcomatous features (mucinous glands, bone, cartilage). | |||
DDx: | DDx: | ||
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Retiform variant | Retiform variant | ||
*Ovarian serous carcinoma - generally | *Ovarian serous carcinoma - generally carcinoma patients are older. | ||
*Ovarian yolk sac tumor | *Ovarian yolk sac tumor | ||
===Images=== | ===Images=== | ||
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Image:Sertoli-Leydig_cell_tumour_-_high_mag.jpg | Sertoli-Leydig cell tumour - high mag. (WC) | Image:Sertoli-Leydig_cell_tumour_-_high_mag.jpg | Sertoli-Leydig cell tumour - high mag. (WC) | ||
Image:Sertoli-Leydig_cell_tumour_-_very_high_mag.jpg | Sertoli-Leydig cell tumour - very high mag. (WC) | Image:Sertoli-Leydig_cell_tumour_-_very_high_mag.jpg | Sertoli-Leydig cell tumour - very high mag. (WC) | ||
Image:Ovary SertoliLeydigCellTumor 4 PA.jpg|Ovarian Sertoli Leydig Cell Tumor - Well differentiated - low power (SKB) | |||
Image:Ovary SertoliLeydigCellTumor 5 PA.jpg|Ovarian Sertoli Leydig Cell Tumor - Well differentiated - low power (SKB) | |||
Image:Ovary SertoliLeydigCellTumor 7 PA.jpg|Ovarian Sertoli Leydig Cell Tumor - Well differentiated - medium power (SKB) | |||
Image:Ovary SertoliLeydigCellTumor 6 PA.jpg|Ovarian Sertoli Leydig Cell Tumor - Well differentiated (SKB) | |||
Image:Ovary SertoliLeydigCellTumor.jpg|Ovarian Sertoli Leydig Cell Tumor - Well differentiated (SKB) | |||
Image:Ovary SertoliLeydigCellTumor 3 PA.jpg|Ovarian Sertoli Leydig Cell Tumor - Well differentiated - see how much cytoplasm is between the nucleus and the lumen? See the crisp outline of the lumen by the apical membrane of the cells - this is a typical Leydig tubule. (SKB) | |||
Image:Ovary SertoliLeydigCellTumor PA.jpg|Ovarian Sertoli Leydig Cell Tumor - Well differentiated (SKB) | |||
Image:Ovary SertoliLeydigCellTumor MP2 CTR.jpg|Ovarian Sertoli Leydig Cell Tumor - medium power - This example is somewhat between the previous well differentiated and following intermediate differentiated examples (SKB) | |||
Image:Ovary SertoliLeydigCellTumor MP3 CTR.jpg|Ovarian Sertoli Leydig Cell Tumor - medium power (SKB) | |||
Image:Ovary SertoliLeydigCellTumor MP CTR.jpg|Ovarian Sertoli Leydig Cell Tumor - medium power (SKB) | |||
Image:Ovary SertoliLeydig Intermediate MP CTR.jpg|Ovarian Sertoli-Leydig Cell Tumor - Intermediate differentiation - Medium power (SKB) | |||
Image:Ovary SertoliLeydig Intermediate HP CTR.jpg|Ovarian Sertoli-Leydig Cell Tumor - Intermediate differentiation - High power (SKB) | |||
Image:Ovary SertoliLeydig Intermediate HP2 CTR.jpg|Ovarian Sertoli-Leydig Cell Tumor - Intermediate differentiation - High power (SKB) | |||
</gallery> | </gallery> | ||
www: | www: | ||
*[http://path.upmc.edu/cases/case270/micro.html Sex cord stromal tumour with Sertoli-Leydig component - several images (upmc.edu)]. | *[http://path.upmc.edu/cases/case270/micro.html Sex cord stromal tumour with Sertoli-Leydig component - several images (upmc.edu)]. | ||
==Prognosis== | |||
*Dependant on degree of differentiation and stage at presentation.<ref>{{Cite journal | last1 = Young | first1 = RH. | last2 = Scully | first2 = RE. | title = Ovarian Sertoli-Leydig cell tumors. A clinicopathological analysis of 207 cases. | journal = Am J Surg Pathol | volume = 9 | issue = 8 | pages = 543-69 | month = Aug | year = 1985 | doi = | PMID = 3911780 }}</ref> | |||
*Heterologous mesenchymal elements may portend a worse outcome.<ref>{{Cite journal | last1 = Zaloudek | first1 = C. | last2 = Norris | first2 = HJ. | title = Sertoli-Leydig tumors of the ovary. A clinicopathologic study of 64 intermediate and poorly differentiated neoplasms. | journal = Am J Surg Pathol | volume = 8 | issue = 6 | pages = 405-18 | month = Jun | year = 1984 | doi = | PMID = 6731664 }}</ref> | |||
==IHC== | ==IHC== | ||
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*Vimentin +ve.<ref name=pmid20349790>{{Cite journal | last1 = Kondi-Pafiti | first1 = A. | last2 = Grapsa | first2 = D. | last3 = Kairi-Vassilatou | first3 = E. | last4 = Carvounis | first4 = E. | last5 = Hasiakos | first5 = D. | last6 = Kontogianni | first6 = K. | last7 = Fotiou | first7 = S. | title = Granulosa cell tumors of the ovary: a clinicopathologic and immunohistochemical study of 21 cases. | journal = Eur J Gynaecol Oncol | volume = 31 | issue = 1 | pages = 94-8 | month = | year = 2010 | doi = | PMID = 20349790 }}</ref> | *Vimentin +ve.<ref name=pmid20349790>{{Cite journal | last1 = Kondi-Pafiti | first1 = A. | last2 = Grapsa | first2 = D. | last3 = Kairi-Vassilatou | first3 = E. | last4 = Carvounis | first4 = E. | last5 = Hasiakos | first5 = D. | last6 = Kontogianni | first6 = K. | last7 = Fotiou | first7 = S. | title = Granulosa cell tumors of the ovary: a clinicopathologic and immunohistochemical study of 21 cases. | journal = Eur J Gynaecol Oncol | volume = 31 | issue = 1 | pages = 94-8 | month = | year = 2010 | doi = | PMID = 20349790 }}</ref> | ||
*CD99 +ve. | *CD99 +ve. | ||
*AE1/AE3 and | *[[AE1/AE3]] and [[pankeratin]] +ve | ||
Others:<ref name=pmid20349790/> | Others:<ref name=pmid20349790/> | ||
*CD34 -ve. | *CD34 -ve. | ||
*'''EMA''' -ve. | *'''[[EMA]]''' -ve. | ||
*[[CK7]] -ve. | |||
Keep in mind that this is a biphasic tumor - Leydig cells will not be Pan-keratin positive - Sertoli cells do not express calretinin - Both components express inhibin - etcetera - interpreting this immunopanal requires correlation with the histomorphology. | Keep in mind that this is a biphasic tumor - Leydig cells will not be Pan-keratin positive - Sertoli cells do not express calretinin - Both components express inhibin - etcetera - interpreting this immunopanal requires correlation with the histomorphology. Immunoreactivity may be focal. | ||
Pan-keratins and AE1/AE3 may mark granulosa cell tumors and Sertoli cell tumors causing confusion with adenocarcinoma. EMA is a better marker to exclude an epithelial tumor as EMA is negative in sex cord-stromal tumors. Highlighting why a panel of stains is needed, endometrioid adenocarcinomas may occasionally weakly express inhibin, calretinin or WT-1. | Pan-keratins and AE1/AE3 may mark granulosa cell tumors and Sertoli cell tumors causing confusion with adenocarcinoma. EMA is a better marker to exclude an epithelial tumor as EMA is negative in sex cord-stromal tumors. Highlighting why a panel of stains is needed, endometrioid adenocarcinomas may occasionally weakly express inhibin, calretinin or WT-1. | ||