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Testicular Large Cell Calcifying Sertoli Cell Tumor

MedGen UID:
690106
•Concept ID:
C1266109
•
Neoplastic Process
Synonym: Large cell calcifying Sertoli cell tumor
SNOMED CT: Large cell calcifying Sertoli cell tumor (128728007)

Definition

A testicular Sertoli cell tumor characterized by the presence of large polygonal cells with eosinophilic cytoplasm in a myxoid and hyalinized stroma. Calcifications may be present in the stroma. Malignant behavior is uncommon. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVTesticular Large Cell Calcifying Sertoli Cell Tumor

Recent clinical studies

Etiology

Key changes in WHO classification 2022 of testicular tumors.
Michalová K, Hes O, Michal M
Cesk Patol 2022 Fall;67(4):198-204. PMID: 36513505
Large Cell Calcifying Sertoli Cell Tumor: A Clinicopathologic Study of 18 Cases With Comprehensive Review of the Literature and Reappraisal of Prognostic Features.
Al-Obaidy KI, Idrees MT, Abdulfatah E, Kunju LP, Wu A, Ulbright TM
Am J Surg Pathol 2022 May 1;46(5):688-700. doi: 10.1097/PAS.0000000000001849. PMID: 34913878
Sertoli cell tumors of the testis, not otherwise specified: a clinicopathologic analysis of 60 cases.
Young RH, Koelliker DD, Scully RE
Am J Surg Pathol 1998 Jun;22(6):709-21. doi: 10.1097/00000478-199806000-00008. PMID: 9630178
Large cell calcifying Sertoli cell tumor of the testis: contrasting features of six malignant and six benign tumors and a review of the literature.
Kratzer SS, Ulbright TM, Talerman A, Srigley JR, Roth LM, Wahle GR, Moussa M, Stephens JK, Millos A, Young RH
Am J Surg Pathol 1997 Nov;21(11):1271-80. doi: 10.1097/00000478-199711000-00002. PMID: 9351565
Testicular sex cord-stromal tumors in children: clinicopathologic study of sixteen children with review of the literature.
Goswitz JJ, Pettinato G, Manivel JC
Pediatr Pathol Lab Med 1996 May-Jun;16(3):451-70. doi: 10.1080/15513819609168683. PMID: 9025845

Diagnosis

Large cell calcifying Sertoli cell tumour: molecular and immunohistochemical assessment of a series comprising non-metastasising and metastasising neoplasms.
Yu S, Sholl LM, Siegmund S, Ulbright TM, Collins K, Colecchia M, Del Pilar Gonzalez-Peramato M, Michalová K, Gordetsky JB, Cornejo KM, Kao CS, Wobker SE, Vargas SO, Maclean F, Idrees MT, Anderson WJ, Fletcher CDM, Acosta AM
Histopathology 2023 Jun;82(7):1079-1088. Epub 2023 Mar 16 doi: 10.1111/his.14895. PMID: 36929593
Large cell calcifying Sertoli cell tumour: a contemporary multi-institutional case series highlighting the diagnostic utility of PRKAR1A immunohistochemistry.
Anderson WJ, Gordetsky JB, Idrees MT, Al-Obaidy KI, Kao CS, Cornejo KM, Wobker SE, Cheville JC, Vargas SO, Fletcher CDM, Hirsch MS, Acosta AM
Histopathology 2022 Mar;80(4):677-685. Epub 2022 Jan 5 doi: 10.1111/his.14599. PMID: 34780072
Prepubertal Malignant Large Cell Calcifying Sertoli Cell Tumor of the Testis.
Li G, Lee MS, Kraft KH, Heider A
Urology 2018 Jul;117:145-149. Epub 2018 Apr 4 doi: 10.1016/j.urology.2018.03.033. PMID: 29626571
Comparative immunomorphology of testicular Sertoli and sertoliform tumors.
Mesa H, Gilles S, Datta MW, Murugan P, Larson W, Dachel S, Manivel JC
Hum Pathol 2017 Mar;61:181-189. Epub 2016 Dec 8 doi: 10.1016/j.humpath.2016.11.004. PMID: 27939779
Large-cell calcifying Sertoli cell tumors of the testes in pediatrics.
Gourgari E, Saloustros E, Stratakis CA
Curr Opin Pediatr 2012 Aug;24(4):518-22. doi: 10.1097/MOP.0b013e328355a279. PMID: 22732638Free PMC Article

Therapy

Efficacy of aromatase inhibitor therapy in a case with large cell calcifying Sertoli cell tumour-associated prepubertal gynaecomastia.
Kağızmanlı GA, Besci Ö, Acinikli KY, Şeker G, Yaşar E, Öztürk Y, Demir K, Böber E, Abacı A
J Pediatr Endocrinol Metab 2022 Dec 16;35(12):1565-1570. Epub 2022 Oct 3 doi: 10.1515/jpem-2022-0368. PMID: 36181314
Response to Anastrozole Treatment in a Case with Peutz-Jeghers Syndrome and a Large Cell Calcifying Sertoli Cell Tumor.
Koç Yekedüz M, Şıklar Z, Burgu B, Kuloğlu Z, Kocaay P, Çamtosun E, İsakoca M, Kansu A, Soygür T, Berberoğlu M
J Clin Res Pediatr Endocrinol 2017 Jun 1;9(2):168-171. Epub 2016 Nov 20 doi: 10.4274/jcrpe.3625. PMID: 27873740Free PMC Article
Use of aromatase inhibitors in large cell calcifying sertoli cell tumors: effects on gynecomastia, growth velocity, and bone age.
Crocker MK, Gourgari E, Lodish M, Stratakis CA
J Clin Endocrinol Metab 2014 Dec;99(12):E2673-80. doi: 10.1210/jc.2014-2530. PMID: 25226294Free PMC Article
Testicular sex cord stromal tumors: analysis of patients from the MAKEI study.
Hofmann M, Schlegel PG, Hippert F, Schmidt P, von-Schweinitz D, Leuschner I, Göbel U, Calaminus G, Schneider DT; MAKEI study group
Pediatr Blood Cancer 2013 Oct;60(10):1651-5. Epub 2013 Jun 3 doi: 10.1002/pbc.24607. PMID: 23733594
Large-cell calcifying Sertoli cell tumors of the testes in pediatrics.
Gourgari E, Saloustros E, Stratakis CA
Curr Opin Pediatr 2012 Aug;24(4):518-22. doi: 10.1097/MOP.0b013e328355a279. PMID: 22732638Free PMC Article

Prognosis

Large Cell Calcifying Sertoli Cell Tumor: A Clinicopathologic Study of 18 Cases With Comprehensive Review of the Literature and Reappraisal of Prognostic Features.
Al-Obaidy KI, Idrees MT, Abdulfatah E, Kunju LP, Wu A, Ulbright TM
Am J Surg Pathol 2022 May 1;46(5):688-700. doi: 10.1097/PAS.0000000000001849. PMID: 34913878
Comparative immunomorphology of testicular Sertoli and sertoliform tumors.
Mesa H, Gilles S, Datta MW, Murugan P, Larson W, Dachel S, Manivel JC
Hum Pathol 2017 Mar;61:181-189. Epub 2016 Dec 8 doi: 10.1016/j.humpath.2016.11.004. PMID: 27939779
Testicular sclerosing Sertoli cell tumor: an additional case and review of the literature.
Brunocilla E, Pultrone CV, Schiavina R, Rocca C, Passaretti G, Corti B, Martorana G
Anticancer Res 2012 Nov;32(11):5127-30. PMID: 23155292
Testicular sertoli cell tumours and relative sub-types. Analysis of clinical and prognostic features.
Giglio M, Medica M, De Rose AF, Germinale F, Ravetti JL, Carmignani G
Urol Int 2003;70(3):205-10. doi: 10.1159/000068770. PMID: 12660458
Large cell calcifying Sertoli cell tumor of the testis: contrasting features of six malignant and six benign tumors and a review of the literature.
Kratzer SS, Ulbright TM, Talerman A, Srigley JR, Roth LM, Wahle GR, Moussa M, Stephens JK, Millos A, Young RH
Am J Surg Pathol 1997 Nov;21(11):1271-80. doi: 10.1097/00000478-199711000-00002. PMID: 9351565

Clinical prediction guides

Large cell calcifying Sertoli cell tumour: a contemporary multi-institutional case series highlighting the diagnostic utility of PRKAR1A immunohistochemistry.
Anderson WJ, Gordetsky JB, Idrees MT, Al-Obaidy KI, Kao CS, Cornejo KM, Wobker SE, Cheville JC, Vargas SO, Fletcher CDM, Hirsch MS, Acosta AM
Histopathology 2022 Mar;80(4):677-685. Epub 2022 Jan 5 doi: 10.1111/his.14599. PMID: 34780072
Comparative immunomorphology of testicular Sertoli and sertoliform tumors.
Mesa H, Gilles S, Datta MW, Murugan P, Larson W, Dachel S, Manivel JC
Hum Pathol 2017 Mar;61:181-189. Epub 2016 Dec 8 doi: 10.1016/j.humpath.2016.11.004. PMID: 27939779
Sertoli cell proliferations of the infantile testis: an intratubular form of Sertoli cell tumor?
Venara M, Rey R, Bergadá I, Mendilaharzu H, Campo S, Chemes H
Am J Surg Pathol 2001 Oct;25(10):1237-44. doi: 10.1097/00000478-200110000-00003. PMID: 11688457
Malignant large cell calcifying Sertoli cell tumor of the testis (LCCSCTT). Report of a case in an elderly man and review of the literature.
Bufo P, Pennella A, Serio G, Mastropasqua MG, Cenacchi G, Rogatsch H, Mikuz G
Pathologica 1999 Apr;91(2):107-14. PMID: 10484870
Large-cell calcifying Sertoli cell tumor of the testis: a case report with ultrastructural study.
Horn T, Jao W, Keh PC
Ultrastruct Pathol 1983 Jun;4(4):359-64. doi: 10.3109/01913128309140588. PMID: 6649081

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