Rare Case of Multiple Perirenal, Extra-Adrenal Myelolipoma: Case Report, Current Management Options, and Literature Review.

Case Reports in Urology Pub Date : 2021-04-13 eCollection Date: 2021-01-01 DOI:10.1155/2021/6614641
Goran Benko, Andrina Kopjar, Marin Plantak, Danijel Cvetko, Vicko Glunčić, Anita Lukić
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引用次数: 1

Abstract

Extra-adrenal myelolipomas are rare, asymptomatic entities, although large tumors may cause local symptoms or hemorrhage. When these lesions occur outside the adrenals in the retroperitoneum, they are radiographically easily confused with both primary and secondary retroperitoneal tumors, which tend to be aggressive. Although myelolipomas are benign and can be managed conservatively, if malignancy is suspected, a surgical procedure is an option. We report a case of a 68-year-old patient with multiple perirenal extra-adrenal myelolipomas. Initial abdominal ultrasound reviled an inhomogeneous mass surrounding the left kidney. Subsequent CT examination of the abdomen showed four separate, extrarenal, well-circumscribed, round-shaped, fat-containing retroperitoneal tumors. Given the significant size of the masses, that compressed major abdominal vessels and the suspicion of liposarcoma, a surgical excision of the lesions was performed. The tumors were easily separated, all surrounding structures were spared, and they were removed completely. Histologically, all masses consisted of hematopoietic and mature fat tissue and the final diagnosis was extra-adrenal myelolipoma. The patient was released from the hospital 7th day after surgery in good condition and at his baseline. Since myelolipomas are, by definition, nonfunctional benign tumors, there was no need for further follow-up. The radiological evaluation and fine needle biopsy are usually sufficient to establish the diagnosis, but in some cases of well-differentiated liposarcoma, the differentiation between myelolipoma and liposarcoma can be challenging. Therefore, considering that myelolipomas and liposarcomas have opposite prognoses, which affects the surgeon's decision on the extent of surgical procedure and further treatment, we also emphasize the importance of intraoperative assessment of the tumor, both by the surgeon and by intraoperative pathology consultation.

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罕见多发性肾周、肾上腺外骨髓瘤:病例报告、当前治疗方案及文献回顾。
肾上腺外骨髓瘤是罕见的,无症状的实体,尽管大的肿瘤可能引起局部症状或出血。当这些病变发生在腹膜后肾上腺外时,影像学上容易与原发性和继发性腹膜后肿瘤混淆,后者往往具有侵袭性。虽然骨髓脂肪瘤是良性的,可以保守治疗,但如果怀疑是恶性的,手术治疗是一种选择。我们报告一例68岁患者多发性肾周肾上腺外骨髓瘤。最初的腹部超声显示左肾周围有不均匀的肿块。随后的腹部CT检查显示四个独立的,肾外的,边界清楚的,圆形的,含脂肪的腹膜后肿瘤。考虑到肿块的明显大小,压迫了腹部主要血管,并怀疑是脂肪肉瘤,我们进行了手术切除病变。肿瘤很容易分离,所有周围的结构都被保留下来,并被完全切除。组织学上,所有肿块均由造血和成熟脂肪组织组成,最终诊断为肾上腺外骨髓瘤。患者术后第7天出院,情况良好,处于基线水平。根据定义,骨髓瘤是一种非功能性良性肿瘤,因此无需进一步随访。放射学评估和细针活检通常足以确定诊断,但在一些分化良好的脂肪肉瘤病例中,骨髓脂肪瘤和脂肪肉瘤之间的区分可能具有挑战性。因此,考虑到骨髓脂肪瘤和脂肪肉瘤具有相反的预后,这影响了外科医生对手术程度和进一步治疗的决定,我们也强调了外科医生和术中病理咨询对肿瘤进行术中评估的重要性。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
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