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Ovarian Stromal Tumor with Minor Sex Cord Elements

MedGen UID:
362863
•Concept ID:
C1883177
•
Neoplastic Process
Synonym: Stromal Tumor with Minor Sex Cord Elements

Definition

A rare, benign sex cord-stromal tumor of the ovary characterized by the presence of a fibrothecomatous stroma and scattered sex cord elements. Although it is usually hormonally inactive, cases associated with endometrial hyperplasia or adenocarcinoma have been reported. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVOvarian Stromal Tumor with Minor Sex Cord Elements

Professional guidelines

PubMed

Value of A103 (melan-A) immunostaining in the differential diagnosis of ovarian sex cord stromal tumours.
Stewart CJ, Nandini CL, Richmond JA
J Clin Pathol 2000 Mar;53(3):206-11. doi: 10.1136/jcp.53.3.206. PMID: 10823140Free PMC Article

Recent clinical studies

Etiology

Clinicopathologic Characterization of GREB1-rearranged Uterine Sarcomas With Variable Sex-Cord Differentiation.
Lee CH, Kao YC, Lee WR, Hsiao YW, Lu TP, Chu CY, Lin YJ, Huang HY, Hsieh TH, Liu YR, Liang CW, Chen TW, Yip S, Lum A, Kuo KT, Jeng YM, Yu SC, Chung YC, Lee JC
Am J Surg Pathol 2019 Jul;43(7):928-942. doi: 10.1097/PAS.0000000000001265. PMID: 31094921
Ovarian fibroma with luteinized thecal cells and minor sex cord element: A rare case report.
Mathur A, Seth A, Pant L
Indian J Pathol Microbiol 2018 Apr-Jun;61(2):264-267. doi: 10.4103/IJPM.IJPM_446_17. PMID: 29676374
Sex cord-stromal tumors of the ovary: a comprehensive review and update for radiologists.
Horta M, Cunha TM
Diagn Interv Radiol 2015 Jul-Aug;21(4):277-86. doi: 10.5152/dir.2015.34414. PMID: 26054417Free PMC Article
Uterine tumors resembling ovarian sex cord tumors: an update.
Czernobilsky B
Int J Gynecol Pathol 2008 Apr;27(2):229-35. doi: 10.1097/PGP.0b013e3181569a21. PMID: 18317219
Uterine tumors resembling ovarian sex cord tumors are polyphenotypic neoplasms with true sex cord differentiation.
Irving JA, Carinelli S, Prat J
Mod Pathol 2006 Jan;19(1):17-24. doi: 10.1038/modpathol.3800475. PMID: 16118629

Diagnosis

Stromal Overgrowth in a Brenner Tumor or Ovarian Fibroma With Minor Sex Cord Elements?
Ross JA, Saglam O
Cancer Control 2015 Jul;22(3):366-8. doi: 10.1177/107327481502200316. PMID: 26351894
Sex cord-stromal tumors of the ovary: a comprehensive review and update for radiologists.
Horta M, Cunha TM
Diagn Interv Radiol 2015 Jul-Aug;21(4):277-86. doi: 10.5152/dir.2015.34414. PMID: 26054417Free PMC Article
Microcystic stromal tumor of the ovary: report of 16 cases of a hitherto uncharacterized distinctive ovarian neoplasm.
Irving JA, Young RH
Am J Surg Pathol 2009 Mar;33(3):367-75. doi: 10.1097/PAS.0b013e31818479c3. PMID: 18971779
Uterine tumors resembling ovarian sex cord tumors: an update.
Czernobilsky B
Int J Gynecol Pathol 2008 Apr;27(2):229-35. doi: 10.1097/PGP.0b013e3181569a21. PMID: 18317219
Ovarian stromal tumors with minor sex cord elements: a report of seven cases.
Young RH, Scully RE
Int J Gynecol Pathol 1983;2(3):227-34. doi: 10.1097/00004347-198303000-00001. PMID: 6315609

Therapy

Testicular fibroma of gonadal stromal origin with minor sex cord elements: clinicopathologic and immunohistochemical study of 2 cases.
de Pinieux G, Glaser C, Chatelain D, Perie G, Flam T, Vieillefond A
Arch Pathol Lab Med 1999 May;123(5):391-4. doi: 10.5858/1999-123-0391-TFOGSO. PMID: 10235496

Prognosis

Clinicopathologic Characterization of GREB1-rearranged Uterine Sarcomas With Variable Sex-Cord Differentiation.
Lee CH, Kao YC, Lee WR, Hsiao YW, Lu TP, Chu CY, Lin YJ, Huang HY, Hsieh TH, Liu YR, Liang CW, Chen TW, Yip S, Lum A, Kuo KT, Jeng YM, Yu SC, Chung YC, Lee JC
Am J Surg Pathol 2019 Jul;43(7):928-942. doi: 10.1097/PAS.0000000000001265. PMID: 31094921
Ovarian fibroma with luteinized thecal cells and minor sex cord element: A rare case report.
Mathur A, Seth A, Pant L
Indian J Pathol Microbiol 2018 Apr-Jun;61(2):264-267. doi: 10.4103/IJPM.IJPM_446_17. PMID: 29676374
Sex cord-stromal tumors of the ovary: a comprehensive review and update for radiologists.
Horta M, Cunha TM
Diagn Interv Radiol 2015 Jul-Aug;21(4):277-86. doi: 10.5152/dir.2015.34414. PMID: 26054417Free PMC Article
Uterine tumors resembling ovarian sex cord tumors: an update.
Czernobilsky B
Int J Gynecol Pathol 2008 Apr;27(2):229-35. doi: 10.1097/PGP.0b013e3181569a21. PMID: 18317219
Uterine tumors resembling ovarian sex cord tumors are polyphenotypic neoplasms with true sex cord differentiation.
Irving JA, Carinelli S, Prat J
Mod Pathol 2006 Jan;19(1):17-24. doi: 10.1038/modpathol.3800475. PMID: 16118629

Clinical prediction guides

Ovarian cellular fibromas lack FOXL2 mutations: a useful diagnostic adjunct in the distinction from diffuse adult granulosa cell tumor.
McCluggage WG, Singh N, Kommoss S, Huntsman DG, Gilks CB
Am J Surg Pathol 2013 Sep;37(9):1450-5. doi: 10.1097/PAS.0b013e31828e4f55. PMID: 23774170
Microcystic stromal tumor of the ovary: report of 16 cases of a hitherto uncharacterized distinctive ovarian neoplasm.
Irving JA, Young RH
Am J Surg Pathol 2009 Mar;33(3):367-75. doi: 10.1097/PAS.0b013e31818479c3. PMID: 18971779
Uterine tumors resembling ovarian sex cord tumors: an update.
Czernobilsky B
Int J Gynecol Pathol 2008 Apr;27(2):229-35. doi: 10.1097/PGP.0b013e3181569a21. PMID: 18317219
Comparative immunohistochemical analysis of granulosa and sertoli components in ovarian sex cord-stromal tumors with mixed differentiation: potential implications for derivation of sertoli differentiation in ovarian tumors.
Vang R, Herrmann ME, Tavassoli FA
Int J Gynecol Pathol 2004 Apr;23(2):151-61. doi: 10.1097/00004347-200404000-00010. PMID: 15084844

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