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.

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Keywords Conservative surgery, Neoadjuvant chemotherapy, Ovarian cancer, Small-cell carcinoma of the hypercalcemic type, Survival, adjuvant therapy, adult, advanced cancer, altretamine, article, bleomycin, cancer combination chemotherapy, cancer radiotherapy, cancer regression, cancer staging, cancer surgery, cancer survival, carboplatin, case report, cisplatin, common acute lymphoblastic leukemia antigen, computer assisted tomography, conservative treatment, cyclophosphamide, cytoreductive surgery, dactinomycin, disease severity, doxorubicin, dysgerminoma, epithelial membrane antigen, etoposide, female, female fertility, follow up, germ cell tumor, histopathology, human, ifosfamide, immunohistochemistry, inhibin, mitosis, multiple cycle treatment, neuron specific enolase, oral contraceptive agent, outcome assessment, ovary carcinoma, ovary function, ovary small cell carcinoma of the hypercalcemic type, paclitaxel, priority journal, prognosis, protein p53, recurrent cancer, tumor cell, vinblastine, vincristine
Persistent URL dx.doi.org/10.1111/IGC.0b013e3181a1a116, hdl.handle.net/1765/17153
Journal International Journal of Gynecological Cancer
Citation
Dykgraaf, R.H.M, de Jong, D, van Veen, M, Ewing-Graham, P.C, Helmerhorst, T.J.M, & van der Burg, M. (2009). Clinical management of ovarian small-cell carcinoma of the hypercalcemic type: A proposal for conservative surgery in an advanced stage of disease. International Journal of Gynecological Cancer, 19(3), 348–353. doi:10.1111/IGC.0b013e3181a1a116