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Ovarian Sex Cord-Stromal Tumor Associated with Peutz-Jeghers Syndrome

MedGen UID:
275292
•Concept ID:
C1518733
•
Neoplastic Process

Definition

A sex cord-stromal tumor that arises from the ovary in a patient diagnosed with Peutz-Jeghers syndrome. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVOvarian Sex Cord-Stromal Tumor Associated with Peutz-Jeghers Syndrome

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

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 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
Sex cord-stromal and steroid cell tumors of the ovary.
Outwater EK, Wagner BJ, Mannion C, McLarney JK, Kim B
Radiographics 1998 Nov-Dec;18(6):1523-46. doi: 10.1148/radiographics.18.6.9821198. PMID: 9821198
Peutz-Jeghers syndrome and cancer.
Rodu B, Martinez MG Jr
Oral Surg Oral Med Oral Pathol 1984 Nov;58(5):584-8. doi: 10.1016/0030-4220(84)90084-7. PMID: 6595620

Diagnosis

Ovarian Sertoli cell tumours: practical points.
Gheorghisan-Galateanu AA, Carsote M, Terzea D, Valea A, Ghemigian A
J Pak Med Assoc 2020 Jan;70(1):129-133. doi: 10.5455/JPMA.1381. PMID: 31954037
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
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
Non-Peutz-Jeghers syndrome-associated ovarian sex cord tumor with annular tubules: Report of a malignant case.
Han Y, Li S, Wu L, Zhang X, Cao D
J Obstet Gynaecol Res 2016 Feb;42(2):224-7. Epub 2015 Dec 29 doi: 10.1111/jog.12883. PMID: 26807963
Sex cord-stromal and steroid cell tumors of the ovary.
Outwater EK, Wagner BJ, Mannion C, McLarney JK, Kim B
Radiographics 1998 Nov-Dec;18(6):1523-46. doi: 10.1148/radiographics.18.6.9821198. PMID: 9821198

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

Prognosis

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
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
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
Unclassified sex cord-stromal tumors of the ovary: a report of eight cases.
Simpson JL, Michael H, Roth LM
Arch Pathol Lab Med 1998 Jan;122(1):52-5. PMID: 9448017
Ovarian sex cord tumor with annular tubules: review of 74 cases including 27 with Peutz-Jeghers syndrome and four with adenoma malignum of the cervix.
Young RH, Welch WR, Dickersin GR, Scully RE
Cancer 1982 Oct 1;50(7):1384-402. doi: 10.1002/1097-0142(19821001)50:7<1384::aid-cncr2820500726>3.0.co;2-5. PMID: 7104978

Clinical prediction guides

Non-Peutz-Jeghers syndrome associated ovarian sex cord tumor with annular tubules: a case report.
Nosov V, Park S, Rao J, Memarzadeh S
Fertil Steril 2009 Oct;92(4):1497.e5-1497.e8. Epub 2009 Aug 22 doi: 10.1016/j.fertnstert.2009.07.1002. PMID: 19703688

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