OBJECTIVE: @entity4153 of the @entity43 (SCCOHT) is a rare disease with a poor prognosis. SCCOHT has recently been shown to be associated with @entity23423 gene mutations as well as molecular and genetic similarities to @entity5606 ( @entity5606 ). The objective of our study is to describe the clinical characteristics, treatment modalities and outcomes of 47 @entity1 with SCCOHT. METHODS: We performed a retrospective analysis of 47 @entity1 with SCCOHT evaluated at @entity601 Anderson @entity5 Center between 1990 and 2014. Medical records were reviewed for demographic information, pathologic findings, treatment regimens and outcomes. RESULTS: Median age at diagnosis was 30years (range 5-46). All @entity1 underwent surgery with unilateral salpingo-oophorectomy (USO) performed in 26 @entity1 (55%), and hysterectomy with bilateral salpingooophorectomy (BSO) in 21 @entity1 (45%). Sixteen @entity1 (34.0%) had stage I disease, six (12.8%) stage II, 23 (48.9%) stage III, and two @entity1 (4.3%) had stage IV disease. Information on adjuvant treatment was available for 43 @entity1 : 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 13.2months (range, 0.1 to 210.7) with a median overall survival of 14.9months. 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 @entity1 including the adoption of systemic therapies used in @entity5606 as well as the development of novel agents targeting specific mutations.

XXXX of the @entity43 (SCCOHT): A review of 47 cases.

@entity43 :: ('MESH:D010051', 'Disease') :: ['ovary-hypercalcemic type'] @entity1 :: ('9606', 'Species') :: ['patients'] @entity4153 :: ('MESH:D018288', 'Disease') :: ['Small cell carcinoma'] @entity5 :: ('MESH:D009369', 'Disease') :: ['Cancer'] @entity5606 :: ('MESH:D018335', 'Disease') :: ['malignant rhabdoid tumors', 'MRT'] @entity601 :: ('MESH:D009436', 'Disease') :: ['MD'] @entity23423 :: ('6597', 'Gene') :: ['SMARCA4']
@entity4153 :: (MESH:D018288,Disease) :: ['Small cell carcinoma']
