Small Cell Ovarian Carcinomas – Characterisation of Two Rare Tumor Entities

Abstract

Objective: Small cell ovarian carcinomas (SCOC) are differentiated into two types: hypercalcaemic (SCOCHT) and pulmonary (SCOCPT). Unfortunately, little is known about pulmonary-type small cell ovarian carcinoma. Study Design: We carried out a systematic analysis of all available reports in the literature on individual cases of SCOCHT and SCOCPT. Results: We found that patients with SCOCPT were significantly older than those with SCOCHT. Vimentin and chromogranin detection by immunohistochemistry allow good differentiation between the two types. Interestingly, SCOCPT but not SCOCHT was found to be associated with other benign and malignant ovarian tumours in about 44 % of cases. Although the percentage of R0/R1 resections was high (~ 74 %), survival was poor; even in patients with disease limited to the ovaries (stage Ia and Ib) the recurrence rate was 40 %. Chemotherapy with etoposide or anthracyclines could be useful. Conclusion: Taking the limitations of our study such as its retrospective nature into account and based on the results from studies of small cell carcinomas originating from other tumour sites, we conclude that treatment of SCOCPT should be based on the therapies used to treat other small cell carcinomas. Surgery is appropriate, especially in very early stages of disease, but chemotherapy should not be omitted. Newer concepts such as treatment with somatostatin analogues could help to control symptoms and stabilise some slow-growing tumours.

Zusammenfassung

Einleitung: Es gibt 2 Arten von kleinzelligen Ovarialkarzinomen – die vom hyperkalzämischen (SCOCHT) und die vom pulmonalen Typ (SCOCPT). Leider ist wenig über die Karzinome vom pulmonalen Typ bekannt. Material und Methode: Wir führten ein systematisches Review aller zur Verfügung stehenden Fälle aus der Literatur zu beiden Tumorentitäten durch. Ergebnisse: Patientinnen mit SCOCPT waren signifikant älter als diejenigen mit SCOCHT. Vimentin- und Chromogranin-Expression in der Immunohistochemie erlaubte eine gute Unterscheidung zwischen beiden Tumorentitäten. Interessanterweise waren Fälle von SCOCPT, aber nicht solche mit SCOCHT häufig (44 %) assoziiiert mit anderen benignen und malignen Ovarialtumoren. Trotz hoher Rate an R0/R1-Resektionen (~ 74 %) war das Überleben der Patientinnen selbst in frühen Tumorstadien (FIGO-Stadium Ia oder Ib) sehr schlecht, und es zeigte sich eine Rezidivrate hier von 40 %. Soweit beurteilbar, erschienen Chemotherapien mit Etoposide oder Anthrazyklinen beim pulmonalen Typ sinnvoll. Schlussfolgerungen: Unter Berücksichtigung der Probleme dieses Studienansatzes und im Vergleich zu Studiendaten zu kleinzelligen Tumoren anderer Lokalisationen kann man schließen, dass sich die Behandlung des SCOCPT allgemein an den dort gewonnenen Erkenntnissen orientieren sollte. Operationen sind angebracht, insbesondere in den frühen Stadien, aber Chemotherapie darf niemals unterlassen werden. Möglicherweise sind neue Behandlungsansätze wie die Behandlung mit Somatostatin-Analoga geeignet, die Erkrankung und deren Symptomatik positiv zu beeinflussen.

Key words

ovarian cancer - ovarian tumor - ovary

Schlüsselwörter

Ovarmalignom - Ovar - benigne Ovartumoren

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