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Intratubular Large Cell Hyalinizing Sertoli Cell Neoplasia

MedGen UID:
1390579
•Concept ID:
C4518349
•
Neoplastic Process
Synonym: Intratubular large cell hyalinizing Sertoli cell neoplasia
SNOMED CT: Intratubular large cell hyalinizing Sertoli cell neoplasia (733914009)

Definition

A clinically benign intratubular neoplastic proliferation of large Sertoli cells in the testis. It is associated with prominent basement membrane deposits. It occurs almost exclusively in patients with Peutz-Jeghers syndrome. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVIntratubular Large Cell Hyalinizing Sertoli Cell Neoplasia

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
Intratubular Large Cell Hyalinizing Sertoli Cell Tumor of the Testes in a 4-Year-Old Male With Peutz-Jeghers Syndrome.
Armijo B, Bocklage T, Heideman R
J Pediatr Hematol Oncol 2015 Apr;37(3):e184-7. doi: 10.1097/MPH.0000000000000243. PMID: 25171448

Diagnosis

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 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
Perspectives on testicular sex cord-stromal tumors and those composed of both germ cells and sex cord-stromal derivatives with a comparison to corresponding ovarian neoplasms.
Roth LM, Lyu B, Cheng L
Hum Pathol 2017 Jul;65:1-14. Epub 2017 Apr 23 doi: 10.1016/j.humpath.2017.04.009. PMID: 28445692
Intratubular large cell hyalinizing Sertoli cell tumor of the testis presenting with prepubertal gynecomastia: a case report.
Tuhan H, Abaci A, Sarsık B, Öztürk T, Olguner M, Catli G, Anik A, Olgun N, Bober E
Acta Clin Belg 2017 Aug;72(4):254-258. Epub 2016 Aug 4 doi: 10.1080/17843286.2016.1215869. PMID: 27488929
Testicular tumors--some new and a few perennial problems.
Young RH
Arch Pathol Lab Med 2008 Apr;132(4):548-64. doi: 10.5858/2008-132-548-TTNAAF. PMID: 18384207

Prognosis

Intratubular Large Cell Hyalinizing Sertoli Cell Tumor of the Testes in a 4-Year-Old Male With Peutz-Jeghers Syndrome.
Armijo B, Bocklage T, Heideman R
J Pediatr Hematol Oncol 2015 Apr;37(3):e184-7. doi: 10.1097/MPH.0000000000000243. PMID: 25171448
Intratubular large cell hyalinizing sertoli cell neoplasia of the testis: a report of 8 cases of a distinctive lesion of the Peutz-Jeghers syndrome.
Ulbright TM, Amin MB, Young RH
Am J Surg Pathol 2007 Jun;31(6):827-35. doi: 10.1097/PAS.0b013e3180309e33. PMID: 17527069

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
Intratubular Large Cell Hyalinizing Sertoli Cell Tumor of the Testes in a 4-Year-Old Male With Peutz-Jeghers Syndrome.
Armijo B, Bocklage T, Heideman R
J Pediatr Hematol Oncol 2015 Apr;37(3):e184-7. doi: 10.1097/MPH.0000000000000243. PMID: 25171448

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