Small cell carcinoma of the ovary of the hypercalcaemic type: an analysis of clinical and prognostic aspects of a rare disease on the basis of cases published in the literature

Abstract

In spite of limitations this analysis provides new insights especially with respect to therapeutic aspects. This review underlines the importance of case reports in rare tumour entities in order to answer open questions.

Small Cell Carcinoma of the Ovary: A Rare Tumor With a Poor Prognosis

“…9,10,14 Moreover, most tumor recurrences occur in the abdomen and pelvis. 4 These findings indicate that the role of RT in the management of SCCO merits further investigation. Nevertheless, it should be noted that a pooled analysis of 257 published cases of SCCO-HT failed to demonstrate a survival benefit for patients treated with adjuvant RT (n = 22) in addition to CT compared with CT alone (n = 56).…”

“…1,8 A review of 101 published cases of SCCO-HT documented a survival benefit for patients who received cisplatinum/carboplatinum, vinca alkaloids, or etoposide. 4 Similarly, based on a cumulative analysis of all published SCCO-PT cases, women who received one of the aforementioned chemotherapeutic drugs had better OS. 3 As such, regimens that include platinum and etoposide are preferred.…”

“…2 Small cell carcinoma of the ovary can be further subdivided into SCCO hypercalcemic type (SCCO-HT) and SCCO pulmonary type (SCCO-PT), each characterized by unique histopathologic features. 1,3,4 Small cell carcinoma of the ovary hypercalcemic type commonly arises in young premenopausal women and is frequently associated with hypercalcemia; prognosis is extremely poor compared with the more common epithelial tumors, regardless of disease stage. 4 Little is known about SCCO-PT, an exceedingly rare tumor; it affects mostly postmenopausal women and is associated with a poor prognosis as well.…”

“…1,3,4 Small cell carcinoma of the ovary hypercalcemic type commonly arises in young premenopausal women and is frequently associated with hypercalcemia; prognosis is extremely poor compared with the more common epithelial tumors, regardless of disease stage. 4 Little is known about SCCO-PT, an exceedingly rare tumor; it affects mostly postmenopausal women and is associated with a poor prognosis as well. 3,5 To date, fewer than 300 and 22 cases of SCCO-HT and SCCO-PT, respectively, have been described in literature.…”

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

“…A retrospective review of 135 SCCOHT patients published in case reports and series found that 82 of 126 (65.1%) patients were reported to experience tumor recurrence. 11 The average time to recurrence was 11.5 months (SD 13.3 mo) with a range of 1 to 70 months. Given the lack of prospective data to guide patient follow-up, patient 2 is still considered to be at some risk of recurrence and will be monitored radiologically every 6 to 12 months until she is 5 years from the end of therapy.…”