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

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 salpingooophorectomy (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.

Large colorectal adenomas. An approach to pathologic evaluation.

Adenomatous polyps are common neoplastic lesions of the large intestine. The risk of carcinoma increases with polyp size. Small polyps are typically totally embedded for histologic examination, but no standard method for sampling large, grossly benign polyps has been established. We reviewed grossly noninvasive adenomas 2.5 cm or larger to determine the percentage that contained high-grade dysplasia (HGD) and invasive cancer (IC). Based on these findings, we suggest an approach to evaluating large adenomas. Forty-three colon resections met the inclusion criteria (no previous diagnosis of cancer, no gross evidence of invasion, and totally embedded polyp). Twelve (28%) had HGD with 3% (1 of 33 slides) to 100% (4 of 4 slides) containing HGD. Five (12%) had IC with 4% (3 of 72 slides) to 42% (5 of 12 slides) containing IC. All cases with IC had HGD in other slides. Probability studies showed that in the majority of cases, polyps would need to be entirely embedded to have an estimated probability of 95% or more of detecting either HGD or IC. Therefore, grossly noninvasive adenomas should be routinely entirely embedded.