McCluggage W Glenn, Young Robert H
Department of Pathology, Royal Group of Hospitals Trust, Grosvenor Road, Belfast, Northern Ireland.
Am J Surg Pathol. 2006 May;30(5):600-5. doi: 10.1097/01.pas.0000189180.08063.01.
Paraganglioma is one of the rarest neoplasms to involve the ovary, whether primary or metastatic, with only two previous reports. We describe three examples that occurred in patients 22, 58, and 68 years of age. Two patients had hypertension. Two tumors involved the left ovary and one the right ovary; they ranged from 8 to 22 cm, were solid, and were tan, brown, or yellow. One tumor was confined to the ovary; in the second case, there were tumor deposits on the posterior surface of the uterus and the contralateral ovary; in the other case, there was peri-aortic lymph node involvement and peritoneal deposits. In all cases, however, radiologic investigations did not reveal an alternative primary site. On microscopic examination, all three tumors showed a predominantly nested "zellballen" pattern with groups of cells surrounded by a vascular stroma. Tumor cells largely had abundant granular eosinophilic cytoplasm with, in 2 cases, focal clear cell areas. In 1 case, bizarre tumor giant cells were present. Immunohistochemically, all neoplasms were cytokeratin negative and diffusely positive with neuroendocrine markers. In 1 case, there was an S-100-positive population of sustentacular cells. Two cases were positive for inhibin, one focal and one diffuse, and the other was focally positive for calretinin. Electron microscopy performed in 2 cases revealed dense core neuroendocrine granules. One patient has been followed up for 15 years and is alive and well. Although metastatic spread from an undetected primary outside the ovary cannot be totally excluded for the 2 cases with extraovarian disease, we think that the neoplasms most likely represent primary ovarian paragangliomas. Because various neoplasms in the sex cord-stromal and steroid categories are likely to enter into the differential diagnosis, inhibin and calretinin positivity represents a significant potential diagnostic pitfall. The differential is broad and may include many other ovarian tumors, particularly those with an oxyphilic cell type. Possible theories of histogenesis of primary ovarian paraganglioma include an origin from extra-adrenal paraganglia in the region of the ovary or unidirectional differentiation within a teratoma.
副神经节瘤是累及卵巢的最罕见肿瘤之一，无论是原发性还是转移性，此前仅有两篇报道。我们描述了3例发生在22岁、58岁和68岁患者中的病例。2例患者有高血压。2个肿瘤累及左侧卵巢，1个累及右侧卵巢；肿瘤大小为8至22厘米，质地坚实，呈棕褐色、棕色或黄色。1个肿瘤局限于卵巢；在第2例中，子宫后表面和对侧卵巢有肿瘤沉积物；在另一例中，腹主动脉旁淋巴结受累且有腹膜沉积物。然而，在所有病例中，影像学检查均未发现其他原发性部位。显微镜检查显示，所有3个肿瘤均主要呈巢状“细胞球”模式，细胞群被血管性间质包绕。肿瘤细胞大多具有丰富的颗粒状嗜酸性细胞质，2例有局灶性透明细胞区域。1例出现怪异的肿瘤巨细胞。免疫组化显示，所有肿瘤细胞角蛋白均为阴性，神经内分泌标记物呈弥漫性阳性。1例中存在S-100阳性的支持细胞群。2例抑制素阳性，1例局灶阳性，1例弥漫阳性，另1例钙视网膜蛋白局灶阳性。2例进行了电子显微镜检查，发现有致密核心神经内分泌颗粒。1例患者已随访15年，目前健在且情况良好。尽管对于2例有卵巢外病变的病例不能完全排除来自未检测到的卵巢外原发性肿瘤的转移，但我们认为这些肿瘤很可能代表原发性卵巢副神经节瘤。由于性索间质和类固醇类别的各种肿瘤可能参与鉴别诊断，抑制素和钙视网膜蛋白阳性是一个重要的潜在诊断陷阱。鉴别范围很广，可能包括许多其他卵巢肿瘤，特别是那些具有嗜酸性细胞类型的肿瘤。原发性卵巢副神经节瘤可能的组织发生学理论包括起源于卵巢区域的肾上腺外副神经节或畸胎瘤内的单向分化。
