Kohli Vikraant, Koltz Michael T, Kamath Ashwin A
Department of Neurosurgery, Dell Medical School, The University of Texas at Austin, Austin, Texas; and.
Department of Neurosurgery, Seton Brain and Spine Institute, Austin, Texas.
J Neurosurg Case Lessons. 2021 Aug 23;2(8):CASE21281. doi: 10.3171/CASE21281.
Myeloid sarcoma is a rare malignant hematopoietic neoplasm that arises at extramedullary sites. Although myeloid sarcoma may involve any organ, central nervous system (CNS) involvement is exceptionally rare. To date, few case reports and case series have described CNS myeloid sarcoma, the majority of which include peripheral disease.
The authors present an illustrative case of an adult male with acute myeloid leukemia (AML) in remission relapsing with an isolated, diffuse myeloid sarcoma of the cerebellum. Magnetic resonance imaging showed posterior fossa crowding and diffuse enhancement within the cerebellar white matter without an apparent mass lesion. The patient required ventriculostomy due to obstructive hydrocephalus and ultimately suboccipital craniectomy with duraplasty due to posterior fossa compression. An open cerebellar biopsy revealed myeloid sarcoma. Peripheral studies did not meet the criteria for recurrent AML. The patient subsequently received high-dose systemic chemotherapy and has responded well to treatment.
Myeloid sarcoma may be a neurosurgical lesion because it has the potential to cause mass effect with obstructive hydrocephalus requiring emergent cerebrospinal fluid diversion and possible decompression. The authors report a rare case of isolated recurrence of AML in the form of diffuse CNS myeloid sarcoma and describe the role of neurosurgery in its diagnosis and treatment.
髓系肉瘤是一种罕见的恶性造血肿瘤，发生于髓外部位。尽管髓系肉瘤可能累及任何器官，但累及中枢神经系统（CNS）极为罕见。迄今为止，很少有病例报告和病例系列描述中枢神经系统髓系肉瘤，其中大多数还伴有外周疾病。
作者展示了一例成年男性病例，该患者急性髓系白血病（AML）缓解后复发，出现孤立性、弥漫性小脑髓系肉瘤。磁共振成像显示后颅窝受压，小脑白质内弥漫性强化，无明显肿块病变。由于梗阻性脑积水，患者需要进行脑室造瘘术，最终因后颅窝受压进行枕下颅骨切除术及硬脑膜成形术。开放性小脑活检显示为髓系肉瘤。外周检查未达到复发性AML的标准。该患者随后接受了大剂量全身化疗，治疗反应良好。
髓系肉瘤可能是一种神经外科病变，因为它有可能导致占位效应并引起梗阻性脑积水，需要紧急进行脑脊液引流和可能的减压。作者报告了一例罕见的以弥漫性中枢神经系统髓系肉瘤形式出现的AML孤立性复发病例，并描述了神经外科在其诊断和治疗中的作用。
