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多灶性胚胎性横纹肌肉瘤患儿阴道和膀胱受累的放射学特征。

Radiological features of multifocal embryonal rhabdomyosarcoma affecting the vagina and the urinary bladder in a pediatric patient.

机构信息

Radiodiagnosis, Seth GS Medical College and KEM Hospital, Mumbai, Maharashtra, India

Radiodiagnosis, Seth GS Medical College and KEM Hospital, Mumbai, Maharashtra, India.

出版信息

BMJ Case Rep. 2024 Jun 4;17(6):e259549. doi: 10.1136/bcr-2023-259549.

Abstract

Rhabdomyosarcomas are the most common soft-tissue sarcomas, found usually in the younger age group. Histologically, they are subdivided into embryonal, alveolar, pleomorphic and not otherwise specified. They have a heterogenous appearance on imaging with few additional characteristic features based on the subtype. Botryoid variant of embryonal rhabdomyosarcoma commonly involves the genitourinary and the biliary system. They can be multifocal. Most of these lesions have a heterogenous appearance on imaging with areas of necrosis and haemorrhage. On ultrasound, they are polypoidal with cystic areas and are vascular. The lesions are hyperintense on T2 sequences, isointense to the skeletal muscle on T1 sequences and show heterogenous enhancement. Surgery is the mainstay of treatment along with radiotherapy or chemotherapy depending on the site and the stage of the tumour. We report a case of botryoid variant of rhabdomyosarcoma involving the vagina and the urinary bladder.

摘要

横纹肌肉瘤是最常见的软组织肉瘤,通常发生在年轻人群中。组织学上,它们可细分为胚胎型、肺泡型、多形性和未分类型。根据亚型,横纹肌肉瘤在影像学上具有异质性表现,几乎没有其他特征性表现。胚胎型横纹肌肉瘤的葡萄状变体通常累及泌尿生殖系统和胆道系统。它们可能是多灶性的。这些病变中的大多数在影像学上表现为异质性,存在坏死和出血区域。在超声检查中,它们呈息肉状,伴有囊性区域,并且具有血管性。病变在 T2 序列上呈高信号,在 T1 序列上与骨骼肌等信号,并且呈异质性增强。手术是治疗的主要方法,根据肿瘤的部位和分期,可联合放疗或化疗。我们报告了一例阴道和膀胱受累的葡萄状变体横纹肌肉瘤病例。

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