Bapir Rawa, Aghaways Ismaeel, Omer Sharaza Qadir, Rashid Rezheen J, Ahmed Shaho F, Yasseen Hadeel Adnan, Dhahir Hardi M, Kareem Honar O, Hiwa Dilan S, Abdalla Berun A, Kakamad Fahmi H
Smart Health Tower, Madam Mitterrand Street, Sulaymaniyah, Kurdistan, Iraq.
Department of Urology, Sulaymaniyah Teaching Hospital, Sulaymaniyah, Kurdistan, Iraq.
Radiol Case Rep. 2025 May 7;20(7):3574-3587. doi: 10.1016/j.radcr.2025.04.039. eCollection 2025 Jul.
Extramedullary hematopoiesis (EMH) is extremely rare in the adrenal glands, especially in children. Because this is an underrecognized condition, the vast majority of cases are diagnosed only after adrenalectomy. An 11-year-old female with hereditary spherocytosis presented with dull right hypochondrial pain. Ultrasonography showed chronic calculous cholecystitis, and splenomegaly. A computed tomography revealed a 4 × 1.7 cm mass in the right adrenal gland with no fat content. Laparoscopic cholecystectomy with adrenalectomy was done. Histopathological examination of the adrenal mass revealed hematopoietic precursors demonstrating megakaryocytes, admixed myeloid precursors, and erythroid precursors without adipose tissue. Nearly 30 cases of adrenal EMH have been documented in the literature. Despite being a benign condition and most of the cases diagnosed incidentally, adrenalectomy was done for the majority of patients even though conservative treatment has shown good results in several case reports. Approximately 75% of cases occurred in the right adrenal gland, with bilateral and left side constituting 12.5% each. Patients with hereditary spherocytosis may present with an adrenal mass due to EMH, even in pediatric age groups. In patients with known hematologic diseases, preoperative diagnosis through functional imaging or biopsy may prevent the need for surgical intervention.
肾上腺髓外造血（EMH）极为罕见，尤其是在儿童中。由于这种情况未得到充分认识，绝大多数病例仅在肾上腺切除术后才得以诊断。一名患有遗传性球形红细胞增多症的11岁女性出现右上腹隐痛。超声检查显示慢性结石性胆囊炎和脾肿大。计算机断层扫描显示右肾上腺有一个4×1.7 cm的肿块，不含脂肪成分。进行了腹腔镜胆囊切除术和肾上腺切除术。肾上腺肿块的组织病理学检查显示造血前体细胞，可见巨核细胞、混合的髓系前体细胞和红系前体细胞，无脂肪组织。文献中已记录了近30例肾上腺EMH病例。尽管肾上腺EMH是一种良性疾病，且大多数病例是偶然诊断出来的，但尽管在一些病例报告中保守治疗已显示出良好效果，大多数患者仍接受了肾上腺切除术。约75%的病例发生在右肾上腺，双侧和左侧各占12.5%。即使在儿童年龄组中，患有遗传性球形红细胞增多症的患者也可能因EMH而出现肾上腺肿块。对于已知患有血液系统疾病的患者，通过功能成像或活检进行术前诊断可避免手术干预的需要。
