Abstract
Small cell carcinoma of the ovary hypercalcemic type (SCCOHT) is an exceedingly rare and poorly characterized tumor with an unknown cell of origin. SCCOHT typically occurs in adolescents and young women with a peak incidence in the third decade of life. The initial description of SCCOHT noted a dismal outcome among patients, particularly those with advanced disease. However, recently published studies indicate that SCCOHT is often amenable to therapy and even in advanced stages potentially curable. Surgery paired with multiagent chemotherapy including platinum-based agents and etoposide are often cited in patients who achieve a complete remission. While reports of successfully treated patients have emerged, the scarcity of prospective clinical trials has slowed the validation of effective treatment regimens. The value of extensive surgery, radiation therapy, and high-dose chemotherapy with autologous stem cell rescue remain uncertain. Insight into the biology underlying SCCOHT is desperately needed to guide the implementation of novel therapeutics in SCCOHT.
This is a preview of subscription content, log in via an institution to check access.
Similar content being viewed by others
References
Young RH, Goodman A, Penson RT, Russell AH, Uppot RN, Tambouret RH (2010) Case records of the Massachusetts General Hospital. Case 8-2010. A 22-year-old woman with hypercalcemia and a pelvic mass. N Engl J Med 362(11):1031–1040
Dickersin GR, Kline IW, Scully RE (1982) Small cell carcinoma of the ovary with hypercalcemia: a report of eleven cases. Cancer 49(1):188–197
Young RH, Oliva E, Scully RE (1994) Small cell carcinoma of the ovary, hypercalcemic type. A clinicopathological analysis of 150 cases. Am J Surg Pathol 18(11):1102–1116
Florell SR, Bruggers CS, Matlak M, Young RH, Lowichik A (1999) Ovarian small cell carcinoma of the hypercalcemic type in a 14 month old: the youngest reported case. Med Pediatr Oncol 32(4):304–307
Distelmaier F, Calaminus G, Harms D et al (2006) Ovarian small cell carcinoma of the hypercalcemic type in children and adolescents: a prognostically unfavorable but curable disease. Cancer 107(9):2298–2306
Senekjian EK, Weiser PA, Talerman A, Herbst AL (1989) Vinblastine, cisplatin, cyclophosphamide, bleomycin, doxorubicin, and etoposide in the treatment of small cell carcinoma of the ovary. Cancer 64(6):1183–1187
Tewari K, Brewer C, Cappuccini F, Macri C, Rogers LW, Berman ML (1997) Advanced-stage small cell carcinoma of the ovary in pregnancy: long-term survival after surgical debulking and multiagent chemotherapy. Gynecol Oncol 66(3):531–534
Sholler GL, Luks F, Mangray S, Meech SJ (2005) Advanced small cell carcinoma of the ovary in a pediatric patient with long-term survival and review of the literature. J Pediatr Hematol Oncol 27(3):169–172
Pautier P, Ribrag V, Duvillard P et al (2007) Results of a prospective dose-intensive regimen in 27 patients with small cell carcinoma of the ovary of the hypercalcemic type. Ann Oncol 18(12):1985–1989
Harrison ML, Hoskins P, du Bois A et al (2006) Small cell of the ovary, hypercalcemic type–analysis of combined experience and recommendation for management. A GCIG study. Gynecol Oncol 100(2):233–238
George RE, Li S, Medeiros-Nancarrow C et al (2006) High-risk neuroblastoma treated with tandem autologous peripheral-blood stem cell-supported transplantation: long-term survival update. J Clin Oncol 24(18):2891–2896
Nitz UA, Mohrmann S, Fischer J et al (2005) Comparison of rapidly cycled tandem high-dose chemotherapy plus peripheral-blood stem-cell support versus dose-dense conventional chemotherapy for adjuvant treatment of high-risk breast cancer: results of a multicentre phase III trial. Lancet 366(9501):1935–1944
Christin A, Lhomme C, Valteau-Couanet D, Dubrel M, Hartmann O (2008) Successful treatment for advanced small cell carcinoma of the ovary. Pediatr Blood Cancer 50(6):1276–1277
Dykgraaf RH, de Jong D, van Veen M, Ewing-Graham PC, Helmerhorst TJ, van der Burg ME (2009) Clinical management of ovarian small-cell carcinoma of the hypercalcemic type: a proposal for conservative surgery in an advanced stage of disease. Int J Gynecol Cancer 19(3):348–353
Walt H, Hornung R, Fink D et al (2001) Hypercalcemic-type of small cell carcinoma of the ovary: characterization of a new tumor line. Anticancer Res 21(5):3253–3259
Kanwar VS, Heath J, Krasner CN, Pearce JM (2008) Advanced small cell carcinoma of the ovary in a seventeen-year-old female, successfully treated with surgery and multi-agent chemotherapy. Pediatr Blood Cancer 50(5):1060–1062
Longy M, Toulouse C, Mage P, Chauvergne J, Trojani M (1996) Familial cluster of ovarian small cell carcinoma: a new Mendelian entity? J Med Genet 33(4):333–335
Seidman JD (1995) Small cell carcinoma of the ovary of the hypercalcemic type: p53 protein accumulation and clinicopathologic features. Gynecol Oncol 59(2):283–287
Cheng Z, Yin H, Du J, Yue X, Qian X, Liu B (2008) Bilateral breast metastasis from small-cell carcinoma of the ovary. J Clin Oncol 26(31):5129–5130
Di Vagno G, Melilli GA, Cormio G et al (2000) Large-cell variant of small cell carcinoma of the ovary with hypercalcaemia. Arch Gynecol Obstet 264(3):157–158
Conflict of interest
None.
Author information
Authors and Affiliations
Corresponding author
Rights and permissions
About this article
Cite this article
Pressey, J.G. The treatment of small cell carcinoma of the ovary hypercalcemic type. Oncol Rev 5, 61–66 (2011). https://doi.org/10.1007/s12156-010-0064-x
Received:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s12156-010-0064-x