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

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

Author Callegaro-Filho, D. Autor UNIFESP Google Scholar
Gershenson, D. M. Google Scholar
Nick, A. M. Google Scholar
Munsell, M. F. Google Scholar
Ramirez, P. T. Google Scholar
Eifel, P. J. Google Scholar
Euscher, E. D. Google Scholar
Marques, R. M. Autor UNIFESP Google Scholar
Nicolau, S. M. Autor UNIFESP Google Scholar
Schmeler, K. M. Google Scholar
Abstract Objective. 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.
Keywords Small cell carcinoma of the ovary
Hypercalcemic type
VPCBAE
Radiotherapy
SMARCA4
Malignant rhabdoid tumorRhabdoid Tumor
Smarca4 Mutations
Chemotherapy
Experience
Management
Kidney
Language English
Sponsor National Institutes of Health through MD Anderson's Cancer Center Support Grant [CA016672]
Grant number National Institutes of Health through MD Anderson's Cancer Center Support Grant CA016672.
Date 2016
Published in Gynecologic Oncology. San Diego, v. 140, n. 1, p. 53-57, 2016.
ISSN 0090-8258 (Sherpa/Romeo, impact factor)
Publisher Academic Press Inc Elsevier Science
Extent 53-57
Origin http://dx.doi.org/10.1016/j.ygyno.2015.11.004
Access rights Open access Open Access
Type Article
Web of Science ID WOS:000367869100011
URI http://repositorio.unifesp.br/handle/11600/49673

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