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Small cell carcinoma of the ovary-hypercalcemic type (SCCOHT): A review of 47 patients.
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.
Abstracts by Donato Callegaro Filho:
Prolonged survival of small cell ovarian cancer-hypercalcemic type (SCCOHT): Should radiotherapy play a larger role?Meeting: 2015 ASCO Annual Meeting | Abstract No: e16542Category: Gynecologic Cancer - Ovarian Cancer
Post-chemotherapy retroperitoneal lymph node dissection (PC-RPLND) for advanced nonseminomatous germ cell tumor (NSGCT): Correlation of teratoma in the primary tumor and RPLND histology.Meeting: 2014 ASCO Annual Meeting | Abstract No: e15537