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多发性巨大髓脂肪瘤的罕见病例:临床及发病机制意义

An unusual case of multiple giant myelolipomas: clinical and pathogenetic implications.

作者信息

Allison Kimberly H, Mann Gary N, Norwood Thomas H, Rubin Brian P

机构信息

Department of Anatomic Pathology, University of Washington Medical Center, Seattle, WA 98195, USA.

出版信息

Endocr Pathol. 2003 Spring;14(1):93-100. doi: 10.1385/ep:14:1:93.

Abstract

Myelolipomas are benign tumors composed of both mature adipose and myeloid tissues. They typically present as an incidental mass in one of the adrenal glands proper. However, they can occur in ectopic adrenal tissue or, rarely, without associated adrenal tissue in various locations and can grow to weights of several kilograms. These tumors have been linked to endocrinopathies, such as Cushing disease and congenital adrenal hyperplasia, which involve overproduction of adrenocorticotropic hormone. We report a case of three giant adrenal myelolipomas arising in a persistently virilized female with congenital adrenal hyperplasia, supporting a role for hormonal stimuli in myelolipoma formation.

摘要

肾上腺髓质脂肪瘤是由成熟脂肪组织和髓样组织构成的良性肿瘤。它们通常表现为肾上腺本身的一个意外肿块。然而,它们也可发生于异位肾上腺组织,或者很少见地,发生于没有相关肾上腺组织的各种部位,并且可以生长到数千克重。这些肿瘤与内分泌疾病有关,如库欣病和先天性肾上腺增生,这些疾病涉及促肾上腺皮质激素分泌过多。我们报告一例患有先天性肾上腺增生且持续男性化的女性发生三个巨大肾上腺髓质脂肪瘤的病例,支持激素刺激在肾上腺髓质脂肪瘤形成中起作用。

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