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Ovarian small cell carcinoma

MedGen UID:
389177
•Concept ID:
C2212006
•
Neoplastic Process
Synonym: Small cell carcinoma of the ovary
SNOMED CT: Small cell ovarian carcinoma (770601003); Small cell carcinoma of ovary (770601003)
 
Monarch Initiative: MONDO:0003795
Orphanet: ORPHA370396

Definition

Small cell carcinoma of the ovary is a rare, highly aggressive, poorly differentiated ovarian neoplasm, often associated with paraneoplastic hypercalcemia. It is usually diagnosed in childhood or young adulthood at an advanced stage and presents with abdominal or pelvic mass or, rarely, symptoms related to hypercalcemia. Occasional familial cases have been reported. [from ORDO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVOvarian small cell carcinoma

Professional guidelines

PubMed

Clinical utility of SMARCA4 testing by immunohistochemistry in rare ovarian tumours.
Genestie C, Blanc-Durand F, Auguste A, Pautier P, Dunant A, Scoazec JY, Gouy S, Morice P, Bentivegna E, Maulard A, LeFormal A, Devouassoux-Shisheboran M, Leary A
Br J Cancer 2020 Feb;122(4):564-568. Epub 2019 Dec 17 doi: 10.1038/s41416-019-0687-z. PMID: 31844183Free PMC Article

Recent clinical studies

Etiology

Cellular context determines DNA methylation profiles in SWI/SNF-deficient cancers of the gynecologic tract.
Kommoss FK, Tessier-Cloutier B, Witkowski L, Forgo E, Koelsche C, Köbel M, Foulkes WD, Lee CH, Kolin DL, von Deimling A, Howitt BE
J Pathol 2022 Jun;257(2):140-145. Epub 2022 Mar 25 doi: 10.1002/path.5889. PMID: 35218556
Comprehensive genomic profiling reveals inactivating SMARCA4 mutations and low tumor mutational burden in small cell carcinoma of the ovary, hypercalcemic-type.
Lin DI, Chudnovsky Y, Duggan B, Zajchowski D, Greenbowe J, Ross JS, Gay LM, Ali SM, Elvin JA
Gynecol Oncol 2017 Dec;147(3):626-633. Epub 2017 Nov 6 doi: 10.1016/j.ygyno.2017.09.031. PMID: 29102090
Ovarian small cell carcinoma of hypercalcemic type - evidence of germline origin and SMARCA4 gene inactivation. a pilot study.
Kupryjańczyk J, Dansonka-Mieszkowska A, Moes-Sosnowska J, Plisiecka-Hałasa J, Szafron L, Podgórska A, Rzepecka IK, Konopka B, Budziłowska A, Rembiszewska A, Grajkowska W, Spiewankiewicz B
Pol J Pathol 2013 Dec;64(4):238-46. doi: 10.5114/pjp.2013.39331. PMID: 24375037
Ovarian small cell carcinoma of the hypercalcemic type in children and adolescents: a prognostically unfavorable but curable disease.
Distelmaier F, Calaminus G, Harms D, Sträter R, Kordes U, Fleischhack G, Göbel U, Schneider DT
Cancer 2006 Nov 1;107(9):2298-306. doi: 10.1002/cncr.22213. PMID: 16998935
Small cell carcinoma of the ovary, hypercalcemic type. A clinicopathological analysis of 150 cases.
Young RH, Oliva E, Scully RE
Am J Surg Pathol 1994 Nov;18(11):1102-16. doi: 10.1097/00000478-199411000-00004. PMID: 7943531

Diagnosis

Hypercalcaemia due to ovarian small cell carcinoma of the hypercalcaemic type.
Montebello A, Gruppetta M
BMJ Case Rep 2021 Jul 30;14(7) doi: 10.1136/bcr-2021-243571. PMID: 34330726Free PMC Article
Ovarian small cell carcinoma of hypercalcemic type - evidence of germline origin and SMARCA4 gene inactivation. a pilot study.
Kupryjańczyk J, Dansonka-Mieszkowska A, Moes-Sosnowska J, Plisiecka-Hałasa J, Szafron L, Podgórska A, Rzepecka IK, Konopka B, Budziłowska A, Rembiszewska A, Grajkowska W, Spiewankiewicz B
Pol J Pathol 2013 Dec;64(4):238-46. doi: 10.5114/pjp.2013.39331. PMID: 24375037
Ovarian pulmonary-type small cell carcinoma: case report and review of the literature.
Kurasaki A, Sakurai N, Yamamoto Y, Taoka H, Takahashi K, Kubushiro K
Int J Gynecol Pathol 2013 Sep;32(5):464-70. doi: 10.1097/PGP.0b013e31826d7ea8. PMID: 23896708
Primary ovarian small cell carcinoma of pulmonary type with enlarged paraaortic lymph node masses: a case report and review of the literature.
Tsolakidis D, Papanikolaou A, Ktenidis K, Pervana S
Eur J Gynaecol Oncol 2012;33(3):312-5. PMID: 22873108
Small cell carcinoma of the ovary of the hypercalcaemic type: an analysis of clinical and prognostic aspects of a rare disease on the basis of cases published in the literature.
Estel R, Hackethal A, Kalder M, Münstedt K
Arch Gynecol Obstet 2011 Nov;284(5):1277-82. Epub 2011 Feb 6 doi: 10.1007/s00404-011-1846-5. PMID: 21298438

Therapy

Primary ovarian small cell carcinoma of pulmonary type with coexisting endometrial carcinoma in a breast cancer patient receiving tamoxifen: A case report and literature review.
Yin L, Li J, Wei Y, Ma D, Sun Y, Sun Y
Medicine (Baltimore) 2018 Jun;97(23):e10900. doi: 10.1097/MD.0000000000010900. PMID: 29879027Free PMC Article
Ovarian pulmonary-type small cell carcinoma: case report and review of the literature.
Kurasaki A, Sakurai N, Yamamoto Y, Taoka H, Takahashi K, Kubushiro K
Int J Gynecol Pathol 2013 Sep;32(5):464-70. doi: 10.1097/PGP.0b013e31826d7ea8. PMID: 23896708
Primary ovarian small cell carcinoma of pulmonary type with enlarged paraaortic lymph node masses: a case report and review of the literature.
Tsolakidis D, Papanikolaou A, Ktenidis K, Pervana S
Eur J Gynaecol Oncol 2012;33(3):312-5. PMID: 22873108
Small cell carcinoma of the ovary of the hypercalcaemic type: an analysis of clinical and prognostic aspects of a rare disease on the basis of cases published in the literature.
Estel R, Hackethal A, Kalder M, Münstedt K
Arch Gynecol Obstet 2011 Nov;284(5):1277-82. Epub 2011 Feb 6 doi: 10.1007/s00404-011-1846-5. PMID: 21298438
Ovarian small cell carcinoma, hypercalcemic type exhibiting a response to high-dose chemotherapy.
Nelsen LL, Muirhead DM, Bell MC
S D Med 2010 Nov;63(11):375-7. PMID: 21117517

Prognosis

Primary Ovarian Small Cell Carcinoma of Pulmonary Type: Analysis of 6 Cases and Review of 31 Cases in the Literatures.
Chen X, Liu HL, Wang JS, Zhao FH
Chin Med Sci J 2023 Jun 30;38(2):130-137. doi: 10.24920/004183. PMID: 37286512
Primary ovarian small cell carcinoma of pulmonary type with coexisting endometrial carcinoma in a breast cancer patient receiving tamoxifen: A case report and literature review.
Yin L, Li J, Wei Y, Ma D, Sun Y, Sun Y
Medicine (Baltimore) 2018 Jun;97(23):e10900. doi: 10.1097/MD.0000000000010900. PMID: 29879027Free PMC Article
Primary ovarian small cell carcinoma of pulmonary type with enlarged paraaortic lymph node masses: a case report and review of the literature.
Tsolakidis D, Papanikolaou A, Ktenidis K, Pervana S
Eur J Gynaecol Oncol 2012;33(3):312-5. PMID: 22873108
Small cell carcinoma of the ovary of the hypercalcaemic type: an analysis of clinical and prognostic aspects of a rare disease on the basis of cases published in the literature.
Estel R, Hackethal A, Kalder M, Münstedt K
Arch Gynecol Obstet 2011 Nov;284(5):1277-82. Epub 2011 Feb 6 doi: 10.1007/s00404-011-1846-5. PMID: 21298438
Large cell variant ovarian small cell carcinoma: case report.
Donato G, Conforti F, Zuccala V, Cosco C, Laratta C, Perrotta I, Tripepi S, Amorosi A
Ultrastruct Pathol 2008 Sep-Oct;32(5):206-10. doi: 10.1080/01913120802397802. PMID: 18958794

Clinical prediction guides

Comprehensive genomic profiling reveals inactivating SMARCA4 mutations and low tumor mutational burden in small cell carcinoma of the ovary, hypercalcemic-type.
Lin DI, Chudnovsky Y, Duggan B, Zajchowski D, Greenbowe J, Ross JS, Gay LM, Ali SM, Elvin JA
Gynecol Oncol 2017 Dec;147(3):626-633. Epub 2017 Nov 6 doi: 10.1016/j.ygyno.2017.09.031. PMID: 29102090
A case of ovarian small cell carcinoma of the pulmonary type that was observed as it developed.
Kumasaka R, Yokoyama Y, Fuchinoue K, Mizunuma H, Taniguchi R, Hashimoto T, Oda T, Kusumi T
Eur J Gynaecol Oncol 2016;37(5):710-713. PMID: 29787016
SMARCA4 (BRG1) loss of expression is a useful marker for the diagnosis of ovarian small cell carcinoma of the hypercalcemic type (ovarian rhabdoid tumor): a comprehensive analysis of 116 rare gynecologic tumors, 9 soft tissue tumors, and 9 melanomas.
Karanian-Philippe M, Velasco V, Longy M, Floquet A, Arnould L, Coindre JM, Le Naoures-Méar C, Averous G, Guyon F, MacGrogan G, Croce S
Am J Surg Pathol 2015 Sep;39(9):1197-205. doi: 10.1097/PAS.0000000000000475. PMID: 26135561
Familial cluster of ovarian small cell carcinoma: a new mendelian entity?
Longy M, Toulouse C, Mage P, Chauvergne J, Trojani M
J Med Genet 1996 Apr;33(4):333-5. doi: 10.1136/jmg.33.4.333. PMID: 8730291Free PMC Article
Primary ovarian small cell carcinoma of pulmonary type. A clinicopathologic, immunohistologic, and flow cytometric analysis of 11 cases.
Eichhorn JH, Young RH, Scully RE
Am J Surg Pathol 1992 Oct;16(10):926-38. doi: 10.1097/00000478-199210000-00002. PMID: 1384368

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