Advanced small cell carcinoma of the ovary in a seventeen‐year‐old female, successfully treated with surgery and multi‐agent chemotherapy

Abstract

Advanced small cell carcinoma of the ovary (FIGO stage III or IV) is a rare and usually lethal tumor seen in adolescents and young women. In pediatric patients with advanced disease, there have been only two case reports of successful therapy, we report a third patient, diagnosed at 17 years of age, with an abdominal mass and metastatic disease to regional and distant lymph nodes, who was successfully treated with surgery and intensive multi-agent chemotherapy. Imatinib, thalidomide, and celecoxib were also administered for up to 24 months following initial chemotherapy. She remains in remission 3 years from diagnosis.

Successful Treatment of Preadolescents With Small Cell Carcinoma of the Ovary Hypercalcemic Type

“…The identification of c-KIT expression was demonstrated in a tumor from a patient successfully treated with imatinib in combination with VPCBAE. 16 VEGF, but not c-KIT, expression was documented by IHC in tumor tissue from patient 2 in the present series, supporting the use of bevacizumab. A similar use of bevacizumab is suggested to be of benefit in adults with epithelial ovarian tumors.…”

“…More recently, VPCBAE has been successfully used in combination with surgery in stage II and III SCCOHT. [14][15][16] HD-SCR has been utilized as a consolidative therapy in several case reports and in a prospective trial. In the study by Pautier and colleagues, 7 of 10 patients in CR after surgery and cisplatin-containing chemotherapy who underwent HD-SCR remained in CR, compared with 3 of 8 patients treated without HD-SCR; although, there was a higher proportion of advanced stage patients in the latter group.…”

The treatment of small cell carcinoma of the ovary hypercalcemic type

“…An analysis of a single SCCOHT tumor cell line by comparative genomic hybridization (CGH) was undertaken, leading to the conclusion that the tumor did not closely resemble germ cell tumors or epithelial ovarian cancers [15]. The analysis of a single SCCOHT tumor demonstrated the expression of the receptor tyrosine kinase cKIT (CD117) by immunohistochemistry leading to the use of imatinib in a successfully treated patient [16]. Ultimately, substantial improvement in outcome will likely rely, at least in part, upon novel therapies directed at biological targets.…”

Small cell ovarian carcinoma: Long term survival in juvenile case with poor prognostic features

“…Patients with early-stage disease confined to the ovary may experience longer survival (Young et al, 1994, Harrison et al, 2006, Distelmaier et al, 2006). There are a few case reports of patients with stage II or III disease surviving several years (Young et al, 1994, Harrison et al, 2006, Sholler et al, 2005, Christin et al, 2008, Kanwar et al, 2008, Tewari et al, 1997, Woopen et al, 2012, Pressey et al, 2013) and no cases of long-term survival in stage IV disease.…”

“…Treatment usually includes a combination of surgical resection and chemotherapy (Young et al, 1994, Harrison et al, 2006, Distelmaier et al, 2006, Senekjian et al, 1989, Peccatori et al, 1993, Sholler et al, 2005, Christin et al, 2008, Kanwar et al, 2008, Tewari et al, 1997, Woopen et al, 2012, Pressey et al, 2013). The extent of surgery required is uncertain due to the need to provide aggressive treatment while attempting to preserve fertility in these young patients (Woopen et al, 2012).…”