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骨髓中具有异源性横纹肌肉瘤分化的去分化脂肪肉瘤。

Dedifferentiated liposarcoma with heterologous rhabdomyosarcomatous differentiation in the bone marrow.

作者信息

Scott Spencer, Hakima Laleh, Raff Evan

机构信息

The University of North Carolina at Chapel Hill School of Medicine, Chapel Hill, North Carolina, USA.

Pathology and Laboratory Medicine, The University of North Carolina School of Medicine, Chapel Hill, North Carolina, USA.

出版信息

BMJ Case Rep. 2023 Dec 9;16(12):e258298. doi: 10.1136/bcr-2023-258298.

Abstract

We present a case of advanced well-differentiated liposarcoma transforming to dedifferentiated liposarcoma in the bone marrow.Our patient presented with 5 weeks of worsening flank pain. He was found to have profound leukocytosis, thrombocytopenia, and normocytic anemia. Imaging showed a dominant retrocaval mass concerning for viable disease. Bone marrow biopsy revealed dedifferentiated liposarcoma with heterologous rhabdomyosarcomatous differentiation. He underwent 3 cycles of eribulin and subsequently developed complications of his increasingly aggressive liposarcoma. He was transitioned to hospice care and died 3 months after initial bone marrow biopsy. While exceedingly rare, it is possible for liposarcoma to metastasize to the bone marrow and cause rapid progression to death. In patients with high-grade liposarcoma, new cytopenia, leukocytosis, elevated granulocyte colony stimulating factor, and/or leukoerythroblasosis on peripheral smear should prompt bone marrow biopsy for early detection of this rare disease entity.

摘要

我们报告一例发生于骨髓的高级别高分化脂肪肉瘤转变为去分化脂肪肉瘤的病例。我们的患者出现了5周的侧腹疼痛加重症状。他被发现有严重的白细胞增多、血小板减少和正细胞性贫血。影像学检查显示一个主要的腔静脉后肿物,怀疑为存活的病变。骨髓活检显示为去分化脂肪肉瘤,伴有异源性横纹肌肉瘤分化。他接受了3个周期的艾日布林治疗,随后出现了其侵袭性日益增加的脂肪肉瘤的并发症。他被转入临终关怀护理,在初次骨髓活检后3个月死亡。虽然极为罕见,但脂肪肉瘤有可能转移至骨髓并导致迅速进展至死亡。对于高级别脂肪肉瘤患者,外周血涂片出现新的血细胞减少、白细胞增多、粒细胞集落刺激因子升高和/或幼稚粒-幼红细胞血象时,应进行骨髓活检以早期发现这种罕见的疾病实体。

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本文引用的文献

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