Clinical Management of Ovarian Small-Cell Carcinoma of the Hypercalcemic Type: A Proposal for Conservative Surgery in an Advanced Stage of Disease

Abstract

Ovarian small-cell carcinoma of the hypercalcemic type is a rare and highly malignant tumor. In two thirds of the patients, the tumor is associated with asymptomatic paraneoplastic hypercalcemia. The diagnosis may be impeded; the tumor must be distinguished from other tumors with similar features.This tumor occurs predominantly in young women and is merely lethal. The 1-year survival is solely 50%, with an overall 5-year survival rate of approximately 10%. It is believed that the empirical treatment characterized by combination of radical surgery, chemotherapy, and radiotherapy results in the most favorable outcome in terms of survival. However, the outcome remains extremely poor despite this aggressive approach.Alternatively, these poor survival rates may justify a less aggressive fertility sparing approach without compromising the outcome. Such an approach is illustrated by a case report involving a patient with ovarian small-cell carcinoma of the hypercalcemic type, FIGO stage IIIC. A fertility-sparing approach was used, consisting of conservative surgery followed by induction chemotherapy, interval debulking surgery, and local radiotherapy. During follow-up of 60 months, there was no evidence of disease and the normal menstrual cycle resumed.In addition to this case report, histopathological features, different therapeutic modalities, and outcome of ovarian small-cell carcinoma of the hypercalcemic type is reviewed. This report suggests that a fertility-sparing approach may be just as feasible as the generally applied aggressive approach.

Advanced small-cell ovarian carcinoma, hypercalcemic type: a challenging therapeutic entity

“…However, the best surgical approach is debatable. In cases with unilateral involvement in future child-bearing patients, unilateral salpingo-oophorectomy can be performed,10 although some authors argue that a bilateral oophorectomy and hysterectomy with lymphadenectomy would reduce the risk of relapse. However, there is a lack of evidence comparing these two approaches.…”

“…In all case series reviewed,2 , 3 , 10 - 15 patients received adjuvant chemotherapy—the vast majority with platinum-based regimens. The most prescribed drugs were cisplatin in combination with etoposide, with limited results in terms of survival outcomes.…”

The treatment of small cell carcinoma of the ovary hypercalcemic type

“…Arguably, ipsilateral salpingo-oophorectomy, sampling of ascitic fluid, peritoneal sampling, omentectomy, and pelvic and para-aortic LN sampling or dissection should be undertaken to fully stage the tumor and render the patient as free of tumor as is feasible. The benefit of routine bilateral salpingo-oophorectomy and total abdominal hysterectomy along with extensive lymph node dissection is not certain and may carry substantial morbidity beyond infertility and lack of ovarian function [14]. Young et al [3] noted that stage IA patients that underwent bilateral salpingo-oophorectomy fared better in survival than those who underwent unilateral salpingo-oophorectomy (57 vs. 23%, p value [ 0.05).…”

Molecular Characterization of a Patient's Small Cell Carcinoma of the Ovary of the Hypercalcemic Type

“…7-11 A VPCBAE (vinblastine, cisplatin, cyclophosphamide, bleomycin, adriamycin, and etoposide) regimen appears to be the preferred treatment in most centers despite its high toxicity. 8, 12, 13 Clearly, better therapeutic options are needed to fight this disease.…”