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

Gynecologic Cancer
Session Type and Session Title: 
This abstract will not be presented at the 2015 ASCO Annual Meeting but has been published in conjunction with the meeting.
Abstract Number: 


J Clin Oncol 33, 2015 (suppl; abstr e16541)
Donato Callegaro Filho, Alpa Manchandia Nick, Mark F. Munsell, Pedro T Ramirez, David Marc Gershenson, Andrea S. Dickens, Dina Patel, Patricia J. Eifel, Elizabeth D. Euscher, Kathleen M. Schmeler; Hospital Israelita Albert Einstein, São Paulo, Brazil; The University of Texas MD Anderson Cancer Center, Houston, TX

Abstract Disclosures


Background: Small cell carcinoma of the ovary-hypercalcemic type (SCCOHT) is a rare disease with fewer than 300 reported cases. Recent studies have shown association with a mutation in the SMARCA4 gene. However, management of this disease remains a challenge and prognosis is poor. Methods: We conducted a retrospective analysis of 47 patients with SCCOHT evaluated at a tertiary cancer care center between January 1990 and August 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). A unilateral salpingo-oophorectomy (USO) was performed in 26 patients (55%) and hysterectomy with bilateral salpingo-oophorectomy (BSO) in 21 patients (45%). Staging procedures were performed in 40 patients (85%). All tumors were unilateral and median tumor size was 16 cm (range, 4-30). Sixteen patients (34%) had stage I disease, six (13%) stage II, 23 (49%) stage III, and two (4%) stage IV disease. Serum calcium levels were elevated at diagnosis in 91% of the 11 patients with results available. Information on adjuvant treatment was available for 43 patients: 33 (77%) received adjuvant treatment with chemotherapy alone, 6 (14%) chemotherapy followed by radiotherapy, one (2%) chemotherapy and radiotherapy, and three (7%) did not receive any treatment. The adjuvant chemotherapy regimens administered included platinum doublet (n = 23) and multi-agent regimen with three (n = 4) or more (n = 12) drugs. Median follow-up was 13 months (range, 0.1 to 210). Median survival was 14.9 months, and 29 patients died, all of disease. Five-year survival for the entire cohort was 29% (95% CI 16% to 44%). Patients with stage III/IV disease had significantly poorer five-year survival (10%, 95% CI 2% to 27%) compared with patients with stage I/II disease (52%, 95% CI 26% to 73%), p < 0.001. Conclusions: SCCOHT is a rare and aggressive disease. Further study is needed to improve outcomes in these patients including the development of new systemic therapies such as novel agents targeting specific mutations.