Khan Umar Zeb, Masroor Matiullah, Yang Wenlong, Riaz Muhammad, Liu Hai
Department of General Surgery, The Third Xiangya Hospital of Central South University, Changsha, China.
Department of General Surgery, The Second Xiangya Hospital of Central South University, Changsha, China.
Gland Surg. 2021 Mar;10(3):1158-1164. doi: 10.21037/gs-20-773.
Solitary extramedullary plasmacytoma, an extremely rare case which accounts for about 3% of all plasma cell neoplasms, often occurs in the head and neck region such as para nasal sinuses, nasal cavity and oral cavity, it also occurs in the lymph nodes, lungs, thyroid, intestine, liver, pancreas, testis, breast, or skin. Isolated primary plasmacytoma of adrenal is extremely rare and only nine documented cases have been reported in the literature. We are presenting the 10 case which is the youngest patient until now with symptoms of fever and hepatosplenomegaly. A 19-year-old female was admitted with an irregular fever for 20 days. After a series of investigations were carried out there were no CRAB symptoms (hypercalcemia, renal failure, anemia and bone lesions), no free light chain and no more than 10% increase in plasma cell on bone marrow examination. Computed tomography (CT) scan revealed a tumor in the left adrenal region, and it was diagnosed to be a solitary extramedullary plasmacytoma on biopsy. She underwent the tumor resection one month after admission and recovered well after operation without fever and was discharged from hospital on the thirteenth post-operative day. She has been followed up for 5 years without any sign and symptom of tumor recurrence. Extramedullary plasmacytoma of adrenal gland in an extremely rare disease and usually diagnosed late in life but it can present in younger patients with variable symptoms. However, the surgical treatment yields excellent long-term results. So, complete surgical resection of the lesion is not only a good diagnostic measure, but also an intent-for-cure treatment for solitary adrenal extramedullary plasmacytoma.
孤立性髓外浆细胞瘤是一种极为罕见的病例,约占所有浆细胞肿瘤的3%,常发生于头颈部区域,如鼻窦旁、鼻腔和口腔,也可发生于淋巴结、肺、甲状腺、肠道、肝脏、胰腺、睾丸、乳腺或皮肤。肾上腺孤立性原发性浆细胞瘤极为罕见,文献中仅报道过9例。我们现报告第10例病例,该患者是目前最年轻的,有发热和肝脾肿大症状。一名19岁女性因持续20天的不规则发热入院。经过一系列检查,未发现CRAB症状(高钙血症、肾衰竭、贫血和骨病变),无游离轻链,骨髓检查浆细胞增加不超过10%。计算机断层扫描(CT)显示左肾上腺区域有一个肿瘤,活检诊断为孤立性髓外浆细胞瘤。她入院一个月后接受了肿瘤切除术,术后恢复良好,无发热,术后第13天出院。她已随访5年,无任何肿瘤复发的迹象和症状。肾上腺髓外浆细胞瘤是一种极为罕见的疾病,通常在晚年被诊断出来,但也可能出现在症状各异的年轻患者中。然而,手术治疗可产生优异的长期效果。因此,完整切除病变不仅是一种良好的诊断措施,也是孤立性肾上腺髓外浆细胞瘤的根治性治疗方法。