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Ovarian Sex Cord Tumor with Annular Tubules

MedGen UID:
276804
•Concept ID:
C1519276
•
Neoplastic Process
Synonyms: Ovarian Sertoli Cell Tumor, Annular Tubular Variant; Ovarian Sex Cord Neoplasm with Annular Tubules

Definition

An ovarian sex cord-stromal tumor characterized by the presence of Sertoli cells forming annular tubules. It may be associated with Peutz-Jeghers syndrome. Cases associated with Peutz-Jeghers syndrome have followed a benign clinical course. Cases which are not associated with Peutz-Jeghers syndrome have been reported having a clinically malignant course. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVOvarian Sex Cord Tumor with Annular Tubules

Professional guidelines

PubMed

Peutz-Jeghers Syndrome: Pathobiology, Pathologic Manifestations, and Suggestions for Recommending Genetic Testing in Pathology Reports.
Meserve EE, Nucci MR
Surg Pathol Clin 2016 Jun;9(2):243-68. doi: 10.1016/j.path.2016.01.006. PMID: 27241107
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

Non-Peutz-Jeghers syndrome-associated ovarian sex cord tumor with annular tubules treated by radiotherapy: a case report and literature review.
Li C, Aishajiang R, Teng Y, Xu T, Ding L, Dong L
J Int Med Res 2021 Mar;49(3):300060521996563. doi: 10.1177/0300060521996563. PMID: 33715493Free PMC Article
Hereditary ovarian tumour syndromes: current update on genetics and imaging.
Shanbhogue KP, Prasad AS, Ucisik-Keser FE, Katabathina VS, Morani AC
Clin Radiol 2021 Apr;76(4):313.e15-313.e26. Epub 2021 Jan 19 doi: 10.1016/j.crad.2020.11.116. PMID: 33353730
Ovarian Sex Cord-Stromal Tumors: Reflections on a 40-Year Experience With a Fascinating Group of Tumors, Including Comments on the Seminal Observations of Robert E. Scully, MD.
Young RH
Arch Pathol Lab Med 2018 Dec;142(12):1459-1484. doi: 10.5858/arpa.2018-0291-RA. PMID: 30500284
Ovarian sex cord-stromal tumours and their mimics.
Young RH
Pathology 2018 Jan;50(1):5-15. Epub 2017 Nov 11 doi: 10.1016/j.pathol.2017.09.007. PMID: 29132723
Management and prognosis of patients with ovarian sex cord tumor with annular tubules: a retrospective study.
Qian Q, You Y, Yang J, Cao D, Zhu Z, Wu M, Chen J, Lang J, Shen K
BMC Cancer 2015 Apr 12;15:270. doi: 10.1186/s12885-015-1277-y. PMID: 25886261Free PMC Article

Diagnosis

Sclerosis in Sex Cord-Stromal Tumors Other Than the Sclerosing Stromal Tumor: A Report of 70 Cases.
Devins KM, Young RH
Am J Surg Pathol 2023 Jul 1;47(7):774-784. Epub 2023 May 15 doi: 10.1097/PAS.0000000000002049. PMID: 37184091
Ovarian sex cord tumor with annular tubules: case report and review of the literature.
Ruiz-Echeverría FR, Beltrán-Salazar MI, Calderón-Quiroz PH, Lalinde-Triviño JD, Palencia-Palacios M, Suescún-Garay O
Rev Colomb Obstet Ginecol 2022 Sep 30;73(3):317-329. doi: 10.18597/rcog.3888. PMID: 36331303Free PMC Article
Fertility sparing surgery in sex-cord stromal tumors: oncological and reproductive outcomes.
Bergamini A, Luisa FM, Dellino M, Erica S, Loizzi V, Bocciolone L, Rabaiotti E, Cioffi R, Sabetta G, Cormio G, Mangili G
Int J Gynecol Cancer 2022 Aug 1;32(8):1063-1070. doi: 10.1136/ijgc-2021-003241. PMID: 35437273
Hereditary ovarian tumour syndromes: current update on genetics and imaging.
Shanbhogue KP, Prasad AS, Ucisik-Keser FE, Katabathina VS, Morani AC
Clin Radiol 2021 Apr;76(4):313.e15-313.e26. Epub 2021 Jan 19 doi: 10.1016/j.crad.2020.11.116. PMID: 33353730
Ovarian sex cord-stromal tumours and their mimics.
Young RH
Pathology 2018 Jan;50(1):5-15. Epub 2017 Nov 11 doi: 10.1016/j.pathol.2017.09.007. PMID: 29132723

Therapy

Impact of taxane plus bevacizumab for ovarian sex cord tumor with annular tubules.
Sho T, Yanazume S, Fukuda M, Togami S, Kamio M, Kobayashi H
J Obstet Gynaecol Res 2019 Jul;45(7):1423-1428. Epub 2019 May 6 doi: 10.1111/jog.13985. PMID: 31060113
Patterns of metastasis in sex cord-stromal tumors of the ovary: can routine staging lymphadenectomy be omitted?
Brown J, Sood AK, Deavers MT, Milojevic L, Gershenson DM
Gynecol Oncol 2009 Apr;113(1):86-90. Epub 2009 Jan 21 doi: 10.1016/j.ygyno.2008.12.007. PMID: 19162310
Chemotherapy-induced alterations in ovarian sex cord tumor with annular tubules (SCTAT): a diagnostic challenge.
Khalifa M, Nofech-Mozes S, Atri M, Covens A
Pathol Res Pract 2006;202(3):183-6. Epub 2006 Feb 3 doi: 10.1016/j.prp.2005.12.004. PMID: 16459027
Recurrent ovarian sex cord tumor with annular tubules: tumor marker and chemotherapy experience.
Puls LE, Hamous J, Morrow MS, Schneyer A, MacLaughlin DT, Castracane VD
Gynecol Oncol 1994 Sep;54(3):396-401. doi: 10.1006/gyno.1994.1232. PMID: 7522202
Ovarian sex cord tumors with annular tubules. An ultrastructural study of three cases.
Crissman JD, Hart WR
Am J Clin Pathol 1981 Jan;75(1):11-7. doi: 10.1093/ajcp/75.1.11. PMID: 7457418

Prognosis

Ovarian sex cord tumor with annular tubules: case report and review of the literature.
Ruiz-Echeverría FR, Beltrán-Salazar MI, Calderón-Quiroz PH, Lalinde-Triviño JD, Palencia-Palacios M, Suescún-Garay O
Rev Colomb Obstet Ginecol 2022 Sep 30;73(3):317-329. doi: 10.18597/rcog.3888. PMID: 36331303Free PMC Article
Hereditary ovarian tumour syndromes: current update on genetics and imaging.
Shanbhogue KP, Prasad AS, Ucisik-Keser FE, Katabathina VS, Morani AC
Clin Radiol 2021 Apr;76(4):313.e15-313.e26. Epub 2021 Jan 19 doi: 10.1016/j.crad.2020.11.116. PMID: 33353730
Sex Cord Tumour with Annular Tubules-An Unusual Case of Abdominal Pain.
Senn D, Videira H, Haagsma B, El-Bahrawy M, Madhuri TK
J Obstet Gynaecol Can 2021 Mar;43(3):361-364. Epub 2020 Aug 4 doi: 10.1016/j.jogc.2020.06.025. PMID: 33288481
Management and prognosis of patients with ovarian sex cord tumor with annular tubules: a retrospective study.
Qian Q, You Y, Yang J, Cao D, Zhu Z, Wu M, Chen J, Lang J, Shen K
BMC Cancer 2015 Apr 12;15:270. doi: 10.1186/s12885-015-1277-y. PMID: 25886261Free PMC Article
Sex cord-stromal tumors of the ovary and testis: their similarities and differences with consideration of selected problems.
Young RH
Mod Pathol 2005 Feb;18 Suppl 2:S81-98. doi: 10.1038/modpathol.3800311. PMID: 15502809

Clinical prediction guides

The role of FOXL2, SOX9, and β-catenin expression and DICER1 mutation in differentiating sex cord tumor with annular tubules from other sex cord tumors of the ovary.
Onder S, Hurdogan O, Bayram A, Yilmaz I, Sozen H, Yavuz E
Virchows Arch 2021 Aug;479(2):317-324. Epub 2021 Feb 10 doi: 10.1007/s00428-021-03052-2. PMID: 33566167
Ovarian Sex Cord-Stromal Tumors: Reflections on a 40-Year Experience With a Fascinating Group of Tumors, Including Comments on the Seminal Observations of Robert E. Scully, MD.
Young RH
Arch Pathol Lab Med 2018 Dec;142(12):1459-1484. doi: 10.5858/arpa.2018-0291-RA. PMID: 30500284
Ovarian sex cord tumor with annular tubules: a report of six cases.
Shen K, Wu PC, Lang JH, Huang RL, Tang MT, Lian LJ
Gynecol Oncol 1993 Feb;48(2):180-4. doi: 10.1006/gyno.1993.1030. PMID: 8428688
Ovarian sex cord tumor with annular tubules--a case report and review of the literature in Japanese.
Takeshima Y, Inai K
Hiroshima J Med Sci 1992 Jun;41(2):37-42. PMID: 1286974
Ovarian neoplasms resembling sex cord tumors with annular tubules.
Hart WR, Kumar N, Crissman JD
Cancer 1980 May 1;45(9):2352-63. doi: 10.1002/1097-0142(19800501)45:9<2352::aid-cncr2820450920>3.0.co;2-#. PMID: 7379031

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