Small cell carcinoma of the ovary-hypercalcemic type (SCCOHT): A review of 47 cases*

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dc.contributor.authorCallegaro-Filho, D. [UNIFESP]
dc.contributor.authorGershenson, D. M.
dc.contributor.authorNick, A. M.
dc.contributor.authorMunsell, M. F.
dc.contributor.authorRamirez, P. T.
dc.contributor.authorEifel, P. J.
dc.contributor.authorEuscher, E. D.
dc.contributor.authorMarques, R. M. [UNIFESP]
dc.contributor.authorNicolau, S. M. [UNIFESP]
dc.contributor.authorSchmeler, K. M.
dc.date.accessioned2019-01-21T10:30:16Z
dc.date.available2019-01-21T10:30:16Z
dc.date.issued2016
dc.description.abstractObjective. Small cell carcinoma of the ovary-hypercalcemic type (SCCOHT) is a rare disease with a poor prognosis. SCCOHT has recently been shown to be associated with SMARCA4 gene mutations as well as molecular and genetic similarities to malignant rhabdoid tumors (MRT). The objective of our study is to describe the clinical characteristics, treatment modalities and outcomes of 47 patients with SCCOHT. Methods. We performed a retrospective analysis of 47 patients with SCCOHT evaluated at MD Anderson Cancer Center between 1990 and 2014. Medical records were reviewed for demographic information, pathologic findings, treatment regimens and outcomes. Results. Median age at diagnosis was 30 years (range 5-46). All patients underwent surgery with unilateral salpingo-oophorectomy (USO) performed in 26 patients (55%), and hysterectomy with bilateral salping000phorectomy (BSO) in 21 patients (45%). Sixteen patients (34.0%) had stage I disease, six (12.8%) stage II, 23 (48.9%) stage III, and two patients (4.3%) had stage IV disease. Information on adjuvant treatment was available for 43 patients: 83.3% received chemotherapy alone, 9.5% chemotherapy followed by radiotherapy, 2.4% chemoradiation, and 4.8% did not receive any adjuvant therapy. Median follow-up was 13.2 months (range, 0.1 to 210.7) with a median overall survival of 14.9 months. Multi-agent chemotherapy and radiotherapy were associated with a better prognosis. Conclusion. Our findings suggest that aggressive therapy including multi-agent chemotherapy and possibly radiotherapy may extend survival. Further study is needed to improve outcomes in these patients including the adoption of systemic therapies used in MRT as well as the development of novel agents targeting specific mutations. (c) 2015 Elsevier Inc. All rights reserved.en
dc.description.affiliationHosp Israelita Albert Einstein, Dept Med Oncol, Sao Paulo, Brazil
dc.description.affiliationUniv Texas MD Anderson Canc Ctr, Dept Gynecol Oncol & Reprod Med, Houston, TX 77030 USA
dc.description.affiliationUniv Texas MD Anderson Canc Ctr, Dept Biostat, Houston, TX 77030 USA
dc.description.affiliationUniv Texas MD Anderson Canc Ctr, Dept Radiat Oncol, Houston, TX 77030 USA
dc.description.affiliationUniv Texas MD Anderson Canc Ctr, Dept Pathol, Houston, TX 77030 USA
dc.description.affiliationUniv Fed Sao Paulo, Div Gynecol Oncol, Sao Paulo, Brazil
dc.description.affiliationUnifespDivision of Gynecologic Oncology, Universidade Federal de São Paulo, São Paulo, Brazil
dc.description.sourceWeb of Science
dc.description.sponsorshipNational Institutes of Health through MD Anderson's Cancer Center Support Grant [CA016672]
dc.description.sponsorshipIDNational Institutes of Health through MD Anderson's Cancer Center Support Grant CA016672.
dc.format.extent53-57
dc.identifierhttp://dx.doi.org/10.1016/j.ygyno.2015.11.004
dc.identifier.citationGynecologic Oncology. San Diego, v. 140, n. 1, p. 53-57, 2016.
dc.identifier.doi10.1016/j.ygyno.2015.11.004
dc.identifier.issn0090-8258
dc.identifier.urihttp://repositorio.unifesp.br/handle/11600/49673
dc.identifier.wosWOS:000367869100011
dc.language.isoeng
dc.publisherAcademic Press Inc Elsevier Science
dc.relation.ispartofGynecologic Oncology
dc.rightsinfo:eu-repo/semantics/openAccess
dc.subjectSmall cell carcinoma of the ovaryen
dc.subjectHypercalcemic typeen
dc.subjectVPCBAEen
dc.subjectRadiotherapyen
dc.subjectSMARCA4en
dc.subjectMalignant rhabdoid tumorRhabdoid Tumoren
dc.subjectSmarca4 Mutationsen
dc.subjectChemotherapyen
dc.subjectExperienceen
dc.subjectManagementen
dc.subjectKidneyen
dc.titleSmall cell carcinoma of the ovary-hypercalcemic type (SCCOHT): A review of 47 cases*en
dc.typeinfo:eu-repo/semantics/article
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