Distelmaier Felix, Calaminus Gabriele, Harms Dieter, Sträter Ronald, Kordes Uwe, Fleischhack Gudrun, Göbel Ulrich, Schneider Dominik T
Clinic of Pediatric Oncology, Hematology, and Immunology, Heinrich-Heine University, Duesseldorf, Germany.
Cancer. 2006 Nov 1;107(9):2298-306. doi: 10.1002/cncr.22213.
Ovarian small cell carcinoma of the hypercalcemic type is a rare neoplasm that is associated with a poor prognosis. The objective of the current study was to investigate the clinicopathologic features of this tumor and to develop preliminary diagnostic and therapeutic guidelines.
Between 1994 and 2005, 11 girls (ages 9-22 years) who were registered on the German Maligne Keimzelltumoren studies and the Kiel Pediatric Tumor Registry were analyzed. Prior to histopathologic review, 8 patients had been misdiagnosed with either germ cell tumor or juvenile granulosa cell tumor.
According to the International Federation of Gynecologic Oncology, 4 patients had Stage IA disease, 3 patients had Stage IC disease, and 4 patients had Stage III disease. After resection, 4 patients were followed without additional therapy, and all 4 patients developed recurrent disease after 3 to 11 months. Seven patients received adjuvant chemotherapy during first-line treatment. One patient with Stage III disease received additional regional deep hyperthermia. During first-line treatment, high-dose chemotherapy was received by 4 patients who achieved a complete response (CR) after conventional chemotherapy. All 4 of those patients remained in CR for 7 to 73 months, whereas the other 3 patients developed recurrent disease. Salvage treatment after recurrence or tumor progression consisted of surgery and chemotherapy. One patient received high-dose chemotherapy in 2nd CR and remained in 2nd CR. In total, 5 patients remained alive with no evidence of disease.
Patients with ovarian small cell carcinoma of the hypercalcemic type require multiagent chemotherapy during first-line treatment. High-dose chemotherapy may be used to consolidate the therapeutic success.
高钙血症型卵巢小细胞癌是一种罕见的肿瘤，预后较差。本研究的目的是探讨该肿瘤的临床病理特征，并制定初步的诊断和治疗指南。
对1994年至2005年间登记在德国恶性生殖细胞肿瘤研究和基尔儿科肿瘤登记处的11名女孩（年龄9 - 22岁）进行分析。在进行组织病理学检查之前，8例患者曾被误诊为生殖细胞肿瘤或幼年型颗粒细胞瘤。
根据国际妇产科联盟的标准，4例患者为IA期疾病，3例患者为IC期疾病，4例患者为III期疾病。切除术后，4例患者未接受额外治疗进行随访，所有4例患者在3至11个月后均出现复发。7例患者在一线治疗期间接受了辅助化疗。1例III期疾病患者接受了额外的区域深部热疗。在一线治疗期间，4例在常规化疗后达到完全缓解（CR）的患者接受了大剂量化疗。这4例患者均保持CR状态7至73个月，而其他3例患者出现复发。复发或肿瘤进展后的挽救治疗包括手术和化疗。1例患者在第二次CR时接受了大剂量化疗，并保持在第二次CR状态。总共有5例患者存活且无疾病证据。
高钙血症型卵巢小细胞癌患者在一线治疗期间需要多药联合化疗。大剂量化疗可用于巩固治疗效果。
