A Rare Tumor in the Adrenal Region: Neuron-specific Enolase (NSE)-Producing Leiomyosarcoma in an Elderly Hypertensive Patient

Endocrine Journal

Tomoko MIYOSHI¹  Masaru KINOMURA¹  Ryo KODERA¹  Toshio OGURA¹  Hirofumi MAKINO¹  Fumio OTSUKA¹  Hiroyuki OTANI¹  Junko GOTO¹  Yukari MIMURA¹  Yasutomo NASU³  Hiroyuki YANAI² 

[1] Department of Medicine and Clinical Sciences, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences;Department of Pathology, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences;Department of Urology, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences

关键词: Adrenal incidentaloma;    Hypertension;    Neuron-specific enolase (NSE);    Leiomyosarcoma;    FDG-PET;   

DOI  :  10.1507/endocrj.K07E-020

学科分类：内分泌与代谢学

来源: Japan Endocrine Society

【 摘 要 】

References(42)Cited-By(4)A 73-year-old Japanese woman was referred for examination of right flank pain and progressive hypertension. Abdominal CT incidentally detected a right adrenal mass 8 cm in size. The tumor exhibited isodensity by CT and contained high-intense lesion by T2-weighted MRI. Scintigraphy with 131 I-metaiodobenzylguanidine and 131 I-adosterol showed no abnormal uptake by whole body scan. Positron emission tomography scan with 18 F-2-fluoro-D-deoxyglucose demonstrated an exclusive uptake in the right adrenal mass. Adrenocortical hormone levels and catecholamine secretion were within normal range; however, the level of serum neuron-specific enolase (NSE) was found to be markedly high. After controlling systemic blood pressure with an α1-blocker, the right adrenal tumor was surgically removed, along with the right kidney and inferior vena cava which adhered to it. The tumor was pathologically proven to be leiomyosarcoma, which was immunohistochemically positive with α-smooth muscle actin and negative with CD57, S-100 and c-kit proteins. Notably, NSE protein was massively expressed in the resected tumor. After surgery blood pressure was controlled with regular medication and serum NSE levels have since normalized. The possibility of leiomyosarcoma should be kept in mind in adrenal incidentalomas with rapid growth and atypical radiological images. Our findings suggest that circulating NSE levels may be clinically useful for early detection of recurrence.

【 授权许可】

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