Dykgraaf Ramon H M, de Jong Diederick, van Veen Mirjam, Ewing-Graham Patricia C, Helmerhorst Theo J M, van der Burg Maria E L
Department of Obstetrics and Gynecology, Erasmus MC, University Medical Center, Rotterdam, the Netherlands.
Int J Gynecol Cancer. 2009 Apr;19(3):348-53. doi: 10.1111/IGC.0b013e3181a1a116.
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.
高钙血症型卵巢小细胞癌是一种罕见且高度恶性的肿瘤。在三分之二的患者中，肿瘤与无症状副肿瘤性高钙血症相关。诊断可能会受到阻碍；该肿瘤必须与具有相似特征的其他肿瘤相鉴别。这种肿瘤主要发生在年轻女性中，且致死率高。1年生存率仅为50%，总体5年生存率约为10%。据信，以根治性手术、化疗和放疗相结合为特征的经验性治疗在生存方面能带来最有利的结果。然而，尽管采取了这种积极的方法，结果仍然极其糟糕。
或者，这些低生存率可能证明在不影响治疗效果的情况下采取一种不那么激进的保留生育功能的方法是合理的。一份涉及一名FIGO IIIC期高钙血症型卵巢小细胞癌患者的病例报告就说明了这种方法。采用了保留生育功能的方法，包括保守手术，随后进行诱导化疗、间隔减瘤手术和局部放疗。在60个月的随访期间，没有疾病证据，月经周期恢复正常。
除了这份病例报告外，还对高钙血症型卵巢小细胞癌的组织病理学特征、不同治疗方式和治疗结果进行了综述。本报告表明，保留生育功能的方法可能与普遍应用的积极方法同样可行。
