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巨大肾上腺髓质脂肪瘤合并小肠平滑肌肉瘤:一例报告

Giant adrenal myelolipoma associated with small bowel leiomyosarcoma: a case report.

作者信息

Ersoy Eren, Ozdoğan Mehmet, Demirağ Alp, Aktimur Recep, Kulaçoğlu Hakan, Kulaçoğlu Sezer, Gündoğdu Haldun

机构信息

Department of General Surgery 3, Ankara Atatürk Research and Education Hospital, Ankara, Turkey.

出版信息

Turk J Gastroenterol. 2006 Jun;17(2):126-9.

Abstract

Adrenal myelolipoma is a rare, benign and biochemically inactive tumor. It is usually diagnosed incidentally by radiological methods and is known to be associated with obesity, hypertension, endocrinological disorders and some malignancies. We report herein the association of a myelolipoma with a gastrointestinal stromal tumor. To our knowledge this is the first report of such an association to date. A 67-year-old male patient admitted to our clinic with abdomimal pain and fever; he had a history of hypertension and diabetes mellitus. In physical examination, a mass involving the right quadrants was palpated. Computerized tomography revealed a right retroperitoneal mass, probably originating from the kidney or cecum. In laparotomy, the tumor (12 cm radius and 1500 g) localized on the superior of right kidney was excised. Abdomen exploration revealed another mass with 10 cm radius 100 cm distal to the ligamentum of Treitz and segmental jejunal resection and anastomosis were applied. The pathological diagnosis was reported as myelolipoma for the retroperitoneal mass and leiomyosarcoma for the jejunal mass. Myelolipoma is a benign tumor, involving mature fat and hematopoietic stem cells. Pathogenesis is still not clear and the microscopical characteristics are hematopoietic, lipoid, and reticuloid cells and megakaryocytes. Myelolipomas are reported to be associated with some other malignancies (especially renal), but this is the first report showing the association with a leiomyosarcoma. Therefore, leiomyosarcoma should also be one of the possible associations kept in mind by the physician in the diagnosis and treatment of myelolipomas.

摘要

肾上腺髓质脂肪瘤是一种罕见的良性且无生化活性的肿瘤。它通常通过影像学方法偶然诊断出来,已知与肥胖、高血压、内分泌紊乱及一些恶性肿瘤有关。我们在此报告一例肾上腺髓质脂肪瘤与胃肠道间质瘤相关的病例。据我们所知,这是迄今为止此类关联的首例报告。一名67岁男性患者因腹痛和发热入住我院;他有高血压和糖尿病病史。体格检查时,在右象限触及一个肿块。计算机断层扫描显示右腹膜后肿块,可能起源于肾脏或盲肠。在剖腹手术中,切除了位于右肾上方的肿瘤(半径12 cm,重1500 g)。腹部探查发现距Treitz韧带远端100 cm处有另一个半径10 cm的肿块,并进行了空肠节段切除和吻合术。病理诊断为腹膜后肿块为肾上腺髓质脂肪瘤,空肠肿块为平滑肌肉瘤。肾上腺髓质脂肪瘤是一种良性肿瘤,由成熟脂肪和造血干细胞组成。其发病机制仍不清楚,显微镜下特征为造血细胞、类脂质细胞、网状细胞和巨核细胞。据报道,肾上腺髓质脂肪瘤与其他一些恶性肿瘤(尤其是肾癌)有关,但这是首例显示与平滑肌肉瘤有关联的报告。因此,在肾上腺髓质脂肪瘤的诊断和治疗中,医生也应将平滑肌肉瘤视为可能的关联之一。

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