Raparia Kirtee, Ayala Alberto G, Sienko Anna, Zhai Qihui J, Ro Jae Y
Department of Pathology, The Methodist Hospital, Weill Medical College of Cornell University, Houston, TX 77030, USA.
Ann Diagn Pathol. 2008 Oct;12(5):344-8. doi: 10.1016/j.anndiagpath.2008.04.002. Epub 2008 Jul 7.
Myxoid adrenal cortical neoplasms are rare, and to our knowledge, only about 23 cases have been reported in the literature, including 13 carcinomas and 10 adenomas. We recently experienced 4 cases of myxoid adrenal cortical neoplasms (3 benign and 1 borderline malignancy) and studied the clinical, histopathological, and immunohistochemical features of these neoplasms. There were 2 male and 2 female patients (age range, 37-61 years, mean, 48 years). All but 1 patient had hormone-related symptoms. The tumors weighed from 24.1 to 94 g (size, 4.1-9.8 cm). They were variably encapsulated with areas of hemorrhage. Histologically, the tumor cells were arranged in delicate arborizing cords or trabecula with myxoid areas varying from 30% to 70%. Three tumors were benign and 1 was of borderline morphology with mitoses of 3/10 high-power fields and mild to moderate nuclear pleomorphism. Two cases contained areas of myelolipomatous component. The tumor cells were positive for vimentin, synaptophysin, and inhibin but negative for cytokeratin. All patients are alive with no recurrence of their tumors or evidence of metastasis (follow-up of 14-20 months). Myxoid changes in adrenal cortical neoplasms are rare but can be seen in both an adenoma and a tumor of uncertain malignant potential. Because of prominent myxoid changes, other myxoid tumors occurring in the retroperitoneum should be excluded. The usual clinical and histological features can be applied to classify the lesions as benign, borderline tumor, or malignant. In our series, there was no case with frank malignant tumor.
黏液样肾上腺皮质肿瘤较为罕见，据我们所知，文献中仅报道了约23例，其中包括13例癌和10例腺瘤。我们最近遇到了4例黏液样肾上腺皮质肿瘤（3例良性，1例交界性恶性），并研究了这些肿瘤的临床、组织病理学和免疫组化特征。患者有2名男性和2名女性（年龄范围为37 - 61岁，平均48岁）。除1例患者外，其余患者均有激素相关症状。肿瘤重量为24.1至94克（大小为4.1 - 9.8厘米）。肿瘤有不同程度的包膜，伴有出血区域。组织学上，肿瘤细胞呈纤细的分支状条索或小梁状排列，黏液样区域占30%至70%不等。3例肿瘤为良性，1例为交界性形态，每10个高倍视野中有3个核分裂象，核有轻度至中度异型性。2例含有髓脂肪瘤成分。肿瘤细胞波形蛋白、突触素和抑制素呈阳性，细胞角蛋白呈阴性。所有患者均存活，肿瘤无复发或转移迹象（随访14 - 20个月）。肾上腺皮质肿瘤中的黏液样改变罕见，但在腺瘤和恶性潜能不确定的肿瘤中均可出现。由于显著的黏液样改变，应排除发生于腹膜后的其他黏液样肿瘤。通常的临床和组织学特征可用于将病变分类为良性、交界性肿瘤或恶性。在我们的系列病例中，没有一例为明显的恶性肿瘤。
