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Clinical Management of Ovarian Small-Cell Carcinoma of the Hypercalcemic Type: A Proposal for Conservative Surgery in an Advanced Stage of Disease
  1. Ramon H.M. Dykgraaf, MD*,
  2. Diederick de Jong, MD, PhD,
  3. Mirjam van Veen, MSc*,
  4. Patricia C. Ewing-Graham, FRCPath,
  5. Theo J.M. Helmerhorst, MD, PhD§ and
  6. Maria E.L. van der Burg, MD, PhD
  1. * Department of Obstetrics and Gynecology, Erasmus MC, University Medical Center, Rotterdam, the Netherlands;
  2. Division of Gynecologic Oncology,Department of Obstetrics and Gynecology, Princess Margaret Hospital University Health Network, Toronto, Canada;
  3. Josephine Nefkens Institute, Department of Pathology, Erasmus MC;
  4. § Division of Gynecologic Oncology, Department of Obstetrics and Gynecology, Erasmus MC/Daniel de Hoed;
  5. Department of Medical Oncology, Erasmus MC, University Medical Center,Rotterdam, the Netherlands.
  1. Address correspondence and reprint requests to Diederick de Jong, MD, PhD, Division of Gynecologic Oncology, Department of Obstetrics and Gynecology, Princess Margaret Hospital University Health Network, 610 University Ave, Suite M700, Toronto, Ontario, M5G 2M9 Canada. E-mail: Diederick.DeJong{at}uhn.on.ca.

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.

  • Ovarian cancer
  • Small-cell carcinoma of the hypercalcemic type
  • Conservative surgery
  • Neoadjuvant chemotherapy
  • Survival

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