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Ovarian Leydig Cell Tumor, Not Otherwise Specified

MedGen UID:
233807
•Concept ID:
C1335164
•
Neoplastic Process
Synonyms: Leydig Cell Neoplasm of Ovary; Leydig Cell Neoplasm of the Ovary; Leydig Cell Tumor of Ovary; Leydig Cell Tumor of the Ovary; Ovarian Leydig Cell Neoplasm; Ovarian Leydig Cell Tumor

Definition

A rare, benign steroid tumor that arises from the ovary and is composed of Leydig cells that contain Reinke crystals. It usually affects postmenopausal women and in most cases, is associated with androgenic manifestations. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVOvarian Leydig Cell Tumor, Not Otherwise Specified

Professional guidelines

PubMed

DICER1-associated Tumors in the Female Genital Tract: Molecular Basis, Clinicopathologic Features, and Differential Diagnosis.
Han LM, Weiel JJ, Longacre TA, Folkins AK
Adv Anat Pathol 2022 Sep 1;29(5):297-308. Epub 2022 Jul 1 doi: 10.1097/PAP.0000000000000351. PMID: 35778792
CT and MR imaging of ovarian tumors with emphasis on differential diagnosis.
Jung SE, Lee JM, Rha SE, Byun JY, Jung JI, Hahn ST
Radiographics 2002 Nov-Dec;22(6):1305-25. doi: 10.1148/rg.226025033. PMID: 12432104
Management of early ovarian cancer: germ cell and sex cord-stromal tumors.
Gershenson DM
Gynecol Oncol 1994 Dec;55(3 Pt 2):S62-72. PMID: 7530680

Recent clinical studies

Etiology

Sex-Cord Stromal Tumors in Children and Teenagers: Results of the TGM-95 Study.
Fresneau B, Orbach D, Faure-Conter C, Verité C, Castex MP, Kalfa N, Martelli H, Patte C
Pediatr Blood Cancer 2015 Dec;62(12):2114-9. Epub 2015 Jul 23 doi: 10.1002/pbc.25614. PMID: 26206391
CD56 is a sensitive and diagnostically useful immunohistochemical marker of ovarian sex cord-stromal tumors.
McCluggage WG, McKenna M, McBride HA
Int J Gynecol Pathol 2007 Jul;26(3):322-7. doi: 10.1097/01.pgp.0000236947.59463.87. PMID: 17581419
Ovarian steroid cell tumors (not otherwise specified). A clinicopathological analysis of 63 cases.
Hayes MC, Scully RE
Am J Surg Pathol 1987 Nov;11(11):835-45. doi: 10.1097/00000478-198711000-00002. PMID: 2823622

Diagnosis

Incidental Finding of Bilateral Ovarian Adrenal Rest Tumor in a Patient With Congenital Adrenal Hyperplasia: A Case Report and Brief Review.
Bouzidi L, Triki M, Charfi S, Ameur HB, Dhaou MB, Bouaziz T, Boudawara T
Pediatr Dev Pathol 2021 Mar-Apr;24(2):137-141. Epub 2021 Jan 12 doi: 10.1177/1093526620980614. PMID: 33433255
Sex-Cord Stromal Tumors in Children and Teenagers: Results of the TGM-95 Study.
Fresneau B, Orbach D, Faure-Conter C, Verité C, Castex MP, Kalfa N, Martelli H, Patte C
Pediatr Blood Cancer 2015 Dec;62(12):2114-9. Epub 2015 Jul 23 doi: 10.1002/pbc.25614. PMID: 26206391
CD56 is a sensitive and diagnostically useful immunohistochemical marker of ovarian sex cord-stromal tumors.
McCluggage WG, McKenna M, McBride HA
Int J Gynecol Pathol 2007 Jul;26(3):322-7. doi: 10.1097/01.pgp.0000236947.59463.87. PMID: 17581419
Steroid cell tumors of the ovary: clinical, ultrasonic, and MRI diagnosis--a case report.
Wang PH, Chao HT, Lee RC, Lai CR, Lee WL, Kwok CF, Yuan CC, Ng HT
Eur J Radiol 1998 Feb;26(3):269-73. doi: 10.1016/s0720-048x(96)01133-3. PMID: 9587754

Therapy

Sex-Cord Stromal Tumors in Children and Teenagers: Results of the TGM-95 Study.
Fresneau B, Orbach D, Faure-Conter C, Verité C, Castex MP, Kalfa N, Martelli H, Patte C
Pediatr Blood Cancer 2015 Dec;62(12):2114-9. Epub 2015 Jul 23 doi: 10.1002/pbc.25614. PMID: 26206391

Prognosis

Sex-Cord Stromal Tumors in Children and Teenagers: Results of the TGM-95 Study.
Fresneau B, Orbach D, Faure-Conter C, Verité C, Castex MP, Kalfa N, Martelli H, Patte C
Pediatr Blood Cancer 2015 Dec;62(12):2114-9. Epub 2015 Jul 23 doi: 10.1002/pbc.25614. PMID: 26206391
Ovarian steroid cell tumors (not otherwise specified). A clinicopathological analysis of 63 cases.
Hayes MC, Scully RE
Am J Surg Pathol 1987 Nov;11(11):835-45. doi: 10.1097/00000478-198711000-00002. PMID: 2823622

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