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Ovarian Signet Ring Stromal Tumor

MedGen UID:
275293
•Concept ID:
C1518735
•
Neoplastic Process
Synonym: Ovarian Signet-Ring Stromal Tumor

Definition

A rare, benign sex cord-stromal tumor that arises from the ovary. It is characterized by the presence of round cells with a single cytoplasmic vacuole and an eccentric nucleus resembling signet ring cells. Mucin stains are negative. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVOvarian Signet Ring Stromal Tumor

Professional guidelines

PubMed

Ovarian Signet-ring Stromal Tumor: A Morphologic, Immunohistochemical, and Molecular Study of 7 Cases With Discussion of the Differential Diagnosis.
Tchrakian N, Oliva E, Chong AS, Rivera-Polo B, Bennett JA, Nucci MR, Sah S, Schoolmeester JK, van der Griend RA, Foulkes WD, Clarke BA, Young RH, McCluggage WG
Am J Surg Pathol 2022 Dec 1;46(12):1599-1610. Epub 2022 Aug 29 doi: 10.1097/PAS.0000000000001954. PMID: 36040033

Recent clinical studies

Etiology

Lung adenocarcinoma metastatic to the ovary: a retrospective clinicopathological analysis of 17 cases and literature review.
Liu W, Cui YM, Wang XJ, Lin XD, Zhang LB, Liu JC, Lyu QH, Chen W, Hu D
Pathology 2024 Dec;56(7):942-950. Epub 2024 Jul 20 doi: 10.1016/j.pathol.2024.05.006. PMID: 39168778
Intraoperative frozen section evaluation of ovarian sex cord-stromal tumours and their mimics: a study of 121 cases with emphasis on potential diagnostic pitfalls.
Zhao Z, Wong SL, Chng JKS, Ang JX, Lim GH, Li J, Zeng L, Chua HM, Ngo NT, Cheng CL, Tan PH, Shi R
Pathology 2024 Oct;56(6):842-853. Epub 2024 Jun 20 doi: 10.1016/j.pathol.2024.04.007. PMID: 38977384
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
Signet-ring stromal tumor of the testis: a case report and literature review.
Kuo CY, Wen MC, Wang J, Jan YJ
Hum Pathol 2009 Apr;40(4):584-7. Epub 2008 Oct 5 doi: 10.1016/j.humpath.2008.07.012. PMID: 18835626
Ovarian spindle cell lesions: a review with emphasis on recent developments and differential diagnosis.
Irving JA, McCluggage WG
Adv Anat Pathol 2007 Sep;14(5):305-19. doi: 10.1097/PAP.0b013e3180ca8a5b. PMID: 17717430

Diagnosis

Ovarian Signet-ring Stromal Tumor: A Morphologic, Immunohistochemical, and Molecular Study of 7 Cases With Discussion of the Differential Diagnosis.
Tchrakian N, Oliva E, Chong AS, Rivera-Polo B, Bennett JA, Nucci MR, Sah S, Schoolmeester JK, van der Griend RA, Foulkes WD, Clarke BA, Young RH, McCluggage WG
Am J Surg Pathol 2022 Dec 1;46(12):1599-1610. Epub 2022 Aug 29 doi: 10.1097/PAS.0000000000001954. PMID: 36040033
Signet-ring stromal cell tumour of the ovary confused with Krukenberg's tumour; a case report.
Choi Y, Choi H, Kim HS, Kim YA, Chun KC
J Obstet Gynaecol 2021 Jan;41(1):155-157. Epub 2019 Dec 2 doi: 10.1080/01443615.2019.1677580. PMID: 31790619
Signet Ring Stromal Cell Tumor: A Legitimate (Benign) Mimic of Krukenberg Tumor.
DeCoste R, Offman SL
Arch Pathol Lab Med 2018 Oct;142(10):1289-1291. doi: 10.5858/arpa.2017-0272-RS. PMID: 30281366
Perspectives on signet ring stromal cell tumor and related signet ring cell lesions of the gonads.
Roth LM, Ramzy I
Adv Anat Pathol 2014 Nov;21(6):443-9. doi: 10.1097/PAP.0000000000000042. PMID: 25299313
Signet-ring stromal tumor of the ovary: report of a case and review of the literature.
Hardisson D, Regojo RM, Mariño-Enríquez A, Martínez-García M
Pathol Oncol Res 2008 Sep;14(3):333-6. Epub 2008 Aug 29 doi: 10.1007/s12253-008-9080-6. PMID: 18758997

Therapy

Ovarian malignant Mullerian mixed tumor (heterologous) whose epithelial component is composed predominantly of signet ring cell carcinoma.
Terada T
Arch Gynecol Obstet 2011 Jun;283(6):1403-6. Epub 2010 Jul 23 doi: 10.1007/s00404-010-1591-1. PMID: 20652287
Phase I clinical and pharmacological study of intraperitoneal cis-bis-neodecanoato( trans- R, R-1, 2-diaminocyclohexane)-platinum II entrapped in multilamellar liposome vesicles.
Verschraegen CF, Kumagai S, Davidson R, Feig B, Mansfield P, Lee SJ, Maclean DS, Hu W, Khokhar AR, Siddik ZH
J Cancer Res Clin Oncol 2003 Oct;129(10):549-55. Epub 2003 Aug 29 doi: 10.1007/s00432-003-0481-3. PMID: 14513369
Clear cell tumors of the female genital tract.
Matias-Guiu X, Lerma E, Prat J
Semin Diagn Pathol 1997 Nov;14(4):233-9. PMID: 9383823

Prognosis

Lung adenocarcinoma metastatic to the ovary: a retrospective clinicopathological analysis of 17 cases and literature review.
Liu W, Cui YM, Wang XJ, Lin XD, Zhang LB, Liu JC, Lyu QH, Chen W, Hu D
Pathology 2024 Dec;56(7):942-950. Epub 2024 Jul 20 doi: 10.1016/j.pathol.2024.05.006. PMID: 39168778
Lobular Carcinoma of the Breast Metastatic to the Ovary: A Clinicopathologic Study of 38 Cases.
Chuang AY, Watkins JC, Young RH, Lerwill MF
Am J Surg Pathol 2022 Feb 1;46(2):179-189. doi: 10.1097/PAS.0000000000001812. PMID: 34931622
Perspectives on signet ring stromal cell tumor and related signet ring cell lesions of the gonads.
Roth LM, Ramzy I
Adv Anat Pathol 2014 Nov;21(6):443-9. doi: 10.1097/PAP.0000000000000042. PMID: 25299313
Signet-ring stromal tumor of the testis: a case report and literature review.
Kuo CY, Wen MC, Wang J, Jan YJ
Hum Pathol 2009 Apr;40(4):584-7. Epub 2008 Oct 5 doi: 10.1016/j.humpath.2008.07.012. PMID: 18835626
Ovarian carcinomas, including secondary tumors: diagnostically challenging areas.
Prat J
Mod Pathol 2005 Feb;18 Suppl 2:S99-111. doi: 10.1038/modpathol.3800312. PMID: 15492758

Clinical prediction guides

Ovarian microcystic stromal tumour: from morphological observations to syndromic associations.
Parra-Herran C, McCluggage WG
Histopathology 2022 May;80(6):898-904. Epub 2022 Feb 28 doi: 10.1111/his.14616. PMID: 35020947
Lobular Carcinoma of the Breast Metastatic to the Ovary: A Clinicopathologic Study of 38 Cases.
Chuang AY, Watkins JC, Young RH, Lerwill MF
Am J Surg Pathol 2022 Feb 1;46(2):179-189. doi: 10.1097/PAS.0000000000001812. PMID: 34931622
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
Perspectives on signet ring stromal cell tumor and related signet ring cell lesions of the gonads.
Roth LM, Ramzy I
Adv Anat Pathol 2014 Nov;21(6):443-9. doi: 10.1097/PAP.0000000000000042. PMID: 25299313
Signet-ring stromal tumor of the testis: a case report and literature review.
Kuo CY, Wen MC, Wang J, Jan YJ
Hum Pathol 2009 Apr;40(4):584-7. Epub 2008 Oct 5 doi: 10.1016/j.humpath.2008.07.012. PMID: 18835626

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