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Clinicoradiological characteristics, management and prognosis of primary myeloid sarcoma of the central nervous system: A report of four cases.

ABSTRACT: Myeloid sarcoma (MS) is a localized tumor composed of premature precursors of granulocytic cells, which may occur in any organ and most commonly involves the soft tissue and musculoskeletal system. This malignancy may occur in the presence or absence of hematological disorders. Primary MS involving the central nervous system (CNS-MS) is rare, and has only been described in a small number of isolated case reports. The diagnosis of CNS-MS is challenging and strategies for its management are undefined. The present study describes 4 cases of CNS-MS. The hematological indices at admission were normal and all patients presented with location-associated nonspecific symptoms. In magnetic resonance imaging scans, the tumors appeared isointense on T1-weighted and T2-weighted images, with marked enhancement following contrast agent administration. Only 1 patient progressed to acute myeloblastic leukemia (AML), with this occurring 3 weeks following histological diagnosis. During the follow-up period, the patient with AML succumbed to the disease, local recurrence was noted in another patient for which a second surgery was requested and no progression was observed in the remaining 2 patients. CNS-MS has unique radiological characteristics and, due to its diffuse tissue infiltration, gross total resection is challenging. It is important for clinicians to be aware of potential hematological disorders in patients with CNS-MS. A combined surgical and chemotherapeutic strategy may be able to provide long-term control of this malignancy.

SUBMITTER: Yang B

PROVIDER: S-EPMC5587933 | biostudies-literature | 2017 Sep

REPOSITORIES: biostudies-literature

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Clinicoradiological characteristics, management and prognosis of primary myeloid sarcoma of the central nervous system: A report of four cases.

Yang Bao B Yang Chenlong C Fang Jingyi J Yang Jun J Xu Yulun Y

Oncology letters 20170720 3

Myeloid sarcoma (MS) is a localized tumor composed of premature precursors of granulocytic cells, which may occur in any organ and most commonly involves the soft tissue and musculoskeletal system. This malignancy may occur in the presence or absence of hematological disorders. Primary MS involving the central nervous system (CNS-MS) is rare, and has only been described in a small number of isolated case reports. The diagnosis of CNS-MS is challenging and strategies for its management are undefi ...[more]

PMID: 28927153

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Project description:Central nervous system (CNS) involvement of graft versus host disease (GvHD) is a rare cause of CNS disorders after allogeneic hematopoietic stem cell transplantation (allo-HSCT). Chronic CNS GvHD symptoms are heterogeneous and include cerebrovascular manifestations, demyelinating disease and immune-mediated encephalitis. CNS-Acute GvHD is not formally defined in literature.We report 7 cases of CNS-GvHD among which two had histological-proven disease. We reviewed 32 additional cases of CNS GvHD published in literature since 1990. In this cohort, 34 patients were transplanted for hematologic malignancies, and 5 for non-malignant hematopoiesis disorders. Of these patients, 25 had a history of chronic GvHD and immunosuppressive treatment had been decreased or discontinued in 14 patients before neurological symptoms onset. Median neurological disorder onset was 385 days [7-7320]. Patients had stroke-like episodes (n?=?7), lacunar syndromes (n?=?3), multiple sclerosis-like presentations (n?=?7), acute demyelinating encephalomyelitis-like symptoms (n?=?4), encephalitis (n?=?14), mass syndrome (n?=?1), and 3 had non-specific symptoms. Median neurological symptoms onset was 81.5 days [7-1095] for patients without chronic GVHD history versus 549 days [11-7300] for patients with chronic GVHD (P?=?0.001). Patients with early involvement of CNS after allo-HSCT and no chronic GVHD symptoms were more frequently suffering from encephalitis (64% versus 28%, P?=?0.07), whereas stroke-like episodes and lacunar symptoms were less frequent (9% versus 36%, P?=?0.13).34 patients with CNS-GvHD were treated with immunosuppressive therapy, including corticosteroids for 31 of them. Other treatments were intravenous immunoglobulin, plasmapheresis, cyclophosphamide, calcineurin inhibitors, mycophenolic acid, methotrexate and etoposide.27 patients achieved a response: 10 complete responses, 15 partial responses and 2 transient responses. Of 25 patients with sufficient follow-up, 7 were alive and 18 patients deceased after CNS-GvHD diagnosis.CNS-related GvHD is a rare cause of CNS disorders after allo-HSCT and is associated with a poor prognosis.

Dataset Information

Clinicoradiological characteristics, management and prognosis of primary myeloid sarcoma of the central nervous system: A report of four cases.

Publications

Clinicoradiological characteristics, management and prognosis of primary myeloid sarcoma of the central nervous system: A report of four cases.

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