Moro Mario, Giannini Caterina, Scheithauer Bernd W, Lloyd Ricardo V, Restall Paul, Eagleton Carl, Law Andrew J, Kovacs Kalman
Department of Pathology, Mayo Clinic, Rochester, MN 55905, USA.
Endocr Pathol. 2004 Summer;15(2):149-58. doi: 10.1385/ep:15:2:149.
We report the occurrence of a primary pituitary fibrosarcoma causally unrelated to radiotherapy, admixed in association with a prolactin cell pituitary adenoma showing neuronal metaplasia. These unique findings were associated with multiple endocrine neoplasia type 1 (MEN 1). Primary fibrosarcoma involving the sella is a very rare tumor. The majority of cases have been associated with prior irradiation of either a pituitary adenoma or a craniopharyngioma. Pituitary adenoma with neuronal metaplasia is also rare and usually occurs in the setting of acromegaly. Despite the intimate association of both elements in our lesion, no transition of adenoma to sarcoma was demonstrable by immunohistochemistry or in situ hybridization studies.
我们报告了一例与放疗无因果关系的原发性垂体纤维肉瘤,其与伴有神经元化生的催乳素细胞垂体腺瘤混合存在。这些独特的发现与1型多发性内分泌肿瘤(MEN 1)相关。累及蝶鞍的原发性纤维肉瘤是一种非常罕见的肿瘤。大多数病例与先前垂体腺瘤或颅咽管瘤的放疗有关。伴有神经元化生的垂体腺瘤也很罕见,通常发生在肢端肥大症的背景下。尽管我们病变中的两种成分密切相关,但免疫组织化学或原位杂交研究均未显示腺瘤向肉瘤的转变。