Benko Goran, Kopjar Andrina, Plantak Marin, Cvetko Danijel, Glunčić Vicko, Lukić Anita
Department of Urology, Varaždin General Hospital, I. Meštrovića 1, Varaždin, Croatia.
Department of Pathology, Varaždin General Hospital, I. Meštrovića 1, Varaždin, Croatia.
Case Rep Urol. 2021 Apr 13;2021:6614641. doi: 10.1155/2021/6614641. eCollection 2021.
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.
肾上腺外髓脂肪瘤是罕见的无症状病变，尽管大的肿瘤可能引起局部症状或出血。当这些病变发生在肾上腺外的腹膜后时，在影像学上很容易与原发性和继发性腹膜后肿瘤混淆，而后者往往具有侵袭性。虽然髓脂肪瘤是良性的，可以保守治疗，但如果怀疑有恶性病变，则可选择手术治疗。我们报告一例68岁患有多个肾周肾上腺外髓脂肪瘤的患者。最初的腹部超声显示左肾周围有一个不均匀的肿块。随后的腹部CT检查显示有四个独立的、肾外的、边界清晰的、圆形的、含脂肪的腹膜后肿瘤。鉴于肿块体积较大，压迫了腹部主要血管，且怀疑为脂肪肉瘤，遂对病变进行了手术切除。肿瘤很容易分离，所有周围结构均得以保留，并且被完全切除。组织学检查显示，所有肿块均由造血组织和成熟脂肪组织组成，最终诊断为肾上腺外髓脂肪瘤。患者术后第7天情况良好，恢复至基线水平后出院。由于髓脂肪瘤根据定义是无功能的良性肿瘤，因此无需进一步随访。放射学评估和细针穿刺活检通常足以确诊，但在一些高分化脂肪肉瘤的病例中，区分髓脂肪瘤和脂肪肉瘤可能具有挑战性。因此，考虑到髓脂肪瘤和脂肪肉瘤的预后相反，这会影响外科医生对手术范围和进一步治疗的决策，我们也强调术中外科医生评估肿瘤以及术中病理会诊的重要性。
