Maciel Joana M, Pereira Daniela V, Simões Helder F, Leite Valeriano A
AACE Clin Case Rep. 2019 Mar 13;5(4):e250-e254. doi: 10.4158/ACCR-2018-0621. eCollection 2019 Jul-Aug.
Schwannomas are usually benign tumors, originating from the Schwann cell myelin sheath around the peripheral or cranial nerves. Visceral schwannomas are rare, and less than 60 cases of adrenal schwannomas have been reported. Microcystic/reticular schwannoma (MRS) is the rarest variant of schwannoma and only 2 cases have been reported in the adrenal gland. Here we describe a third case.
We describe a case of an adrenal MRS with a comprehensive literature review.
The patient was a 69-year-old, Caucasian female with abdominal pain. An abdominal computed tomography scan revealed a solid, lobulated, 78-mm mass of the left adrenal gland. Two inconclusive adrenal biopsies were performed and then she was referred to our tertiary center for further evaluation. No clinical or laboratorial signs of endocrine dysfunction were identified. A positron emission tomography scan with fluorodeoxyglucose showed a single left adrenal hypermetabolic mass (SUVmax = 71.7), suggestive of malignancy. In this context, an open left adrenalectomy was undertaken. The histologic evaluation showed an MRS.
Adrenal MRSs are extremely rare tumors. Definitive diagnosis of schwannoma can only be made by surgical excision with histopathologic and immunohistochemical evaluation of the specimen.
施万细胞瘤通常为良性肿瘤，起源于周围神经或颅神经周围的施万细胞髓鞘。内脏施万细胞瘤较为罕见，肾上腺施万细胞瘤报告病例不足60例。微囊性/网状施万细胞瘤（MRS）是施万细胞瘤中最罕见的变异型，肾上腺仅报告过2例。在此我们描述第3例病例。
我们描述1例肾上腺MRS病例并进行全面文献复习。
患者为69岁白人女性，有腹痛症状。腹部计算机断层扫描显示左肾上腺有一个实性、分叶状、78毫米的肿块。进行了两次诊断不明确的肾上腺活检，随后她被转诊至我们的三级中心作进一步评估。未发现内分泌功能障碍的临床或实验室体征。氟脱氧葡萄糖正电子发射断层扫描显示左肾上腺有一个代谢活跃的肿块（最大标准摄取值=71.7），提示为恶性肿瘤。在此情况下，实施了开放性左肾上腺切除术。组织学评估显示为MRS。
肾上腺MRS是极其罕见的肿瘤。施万细胞瘤的确诊只能通过手术切除标本并进行组织病理学和免疫组织化学评估来实现。
