Navegando por Palavras-chave "Small Cell Carcinoma Of The Ovary"

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    Epidemiologia e os desfechos clínicos de pacientes com carcinoma de pequenas células do ovário, tipo hipercalcêmico (SCCOHT)
    (Universidade Federal de São Paulo (UNIFESP), 2020-11-26) Callegaro Filho, Donato [UNIFESP]; Speck, Neila Maria De Gois [UNIFESP]; Universidade Federal de São Paulo
    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 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 performed in 26 patients (55%), and hysterectomy with bilateral salpingo-oophorectomy 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 with a median overall survival of 14.9 months. Multi-agent chemotherapy and radiotherapy were associated with a better prognosis. On multivariate analysis, factors associated with favorable prognosis included absence of large cell component (p = 0.0003, HR = 0.19, 95% CI 0.08 to 0.47) and early stage disease (p=0.0029, HR=0.28, 95% CI 0.12 to 0.64). Median overall survival (OS) for the 12 patients with a large cell component was 8.8 months compared with 19.4 months for the 35 patients without a large cell component. Median OS for patients with early stage disease was 35.3 months compared with 10.4 months for patients with advanced stage disease. A better prognosis was also associated with the use of radiotherapy (p = 0.0365, HR = 0.26, 95% CI 0.07 to 0.92). The multiagent chemotherapy regimen of vinblastine, cisplatin, cyclophosphamide, bleomycin, doxorubicin and etoposide (VPCBAE) was associated with a decreased rate of recurrence (p = 0.0394). Conclusion: These findings shows a predominant incidence in young adults, caucasian, with large. Frequent misinterpreted as other ovarian neoplasms. Surgery was part of the initial treatment of all patients followed by complementary platinum- based chemotherapy for most patients. High recurrence rates despite treatments. Favorable prognostic factors included absence of large cell component, early stage disease, use of radiotherapy and chemotherapy with VPCBAE regimen. 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.