Isolated megakaryoblastic bone sarcoma revealing acute myeloproliferative syndrome

Desplechin, Arnaud; Mékinian, A.; Rossignol, Julien; Denis, Guillaume; Gosset, P.; Cambier, Nathalie; Rosé, Christian

doi:10.1016/j.jbspin.2010.03.005

Cited by 7 publications

(5 citation statements)

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“…As can be seen in Table , summarizing literature reports of MS with megakaryoblastic differentiation, none of the cases occurred in the CNS, and the present one is the first arising intracranially . Similar to our detection of JAK2 V617F mutation from the MS specimen, Morita et al .…”

Section: Discussionsupporting

confidence: 87%

“…To the best of our knowledge, only 12 cases of MS with megakaryoblastic differentiation have been described, half of which associated with primary myelofibrosis, and usually involving the lymph nodes and soft tissues . This is the first reported case of MS with CNS localization, showing megakaryoblastic differentiation and homozygous JAK2 V617F mutation in a tumor specimen.…”

Section: Introductionmentioning

confidence: 88%

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Myeloid sarcoma with megakaryoblastic differentiation mimicking a sellar tumor

et al. 2013

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Myeloid sarcoma (MS) is a localized extra-medullary tumor mass of immature myeloid cells, arising de novo or related to acute myeloid leukemia, of which it can be a forerunner, a coinciding or late event. Less commonly, MS represents an acute blastic transformation of myelodysplastic syndromes or myeloproliferative neoplasms. This rare condition commonly consists of a proliferation of more or less immature cells with a myeloid immunophenotype, very exceptional cases showing a megakaryoblastic or erythroid differentiation. The most common localization of MS is the skin, lymph node, soft tissues and bones, but CNS involvement is exceedingly rare, with no cases reported in the sellar region. We report a 54-year-old man, affected by myeloproliferative neoplasm, JAK2 V617F-positive of 13 years duration, who acutely presented with a third cranial nerve palsy; neuroradiology documented a space-occupying lesion at the level of the sellar, upper clival and right parasellar regions, that was sub-totally removed with a trans-sphenoidal approach. The histological examination documented a proliferation of large, blastic cells, frequently multinucleated; a diagnosis of MS with megakaryoblastic differentiation, arising in a background of chronic idiopathic myelofibrosis, was suggested by immunohistochemistry, owing to CD42b, CD45, CD61 and LAT (linker for activation of T cells) positivity. In addition, homozygous JAK2 V617F mutation was detected from the myeloid sarcoma specimen. A few weeks after surgery, an acute blastic leukemic transformation occurred and, despite chemotherapy, the patient died 2 months after surgery. To the best of our knowledge, this is the first MS case with megakaryoblastic differentiation arising within the CNS.

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Section: Discussionsupporting

confidence: 87%

Section: Introductionmentioning

confidence: 88%

Myeloid sarcoma with megakaryoblastic differentiation mimicking a sellar tumor

et al. 2013

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“…It has often been reported in the course of an acute leukemia, myelodysplastic syndrome or myeloproliferative neoplasms including chronic myelogenous leukemia and essential thrombocythemia [1][2][3][4]. Myeloid sarcoma can involve any site of body.…”

Section: Introductionmentioning

confidence: 99%

Isolated Myeloid Sarcoma Presenting as Cord Compression by Paraspinal Mass

Kim

Han

et al. 2016

Ewha Med J

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“…Il est présent dans 3 à 8 % des leucémies myéloïdes aiguës (LAM) [1]. Il est rarement associé à d'autre processus myéloprolifératif [2,3]. Il touche préférentiellement les parties molles du cou et de la tête, la peau et moins fréquemment le système nerveux et l'os [4].…”

Section: Introductionunclassified