Vankalakunti Mahesha, Das Ashim, Rao Narasimhan K L
Department of Histopathology, Postgraduate Institute of Medical Education & Research, Chandigarh, India.
Diagn Pathol. 2006 Oct 17;1:37. doi: 10.1186/1746-1596-1-37.
Congenital alveolar rhabdomyosarcoma is an extremely uncommon and invariably fatal tumor with the current therapy. Less than 25% of patients present with evidence of cutaneous metastasis.
We report a case of congenital alveolar rhabdomyosarcoma in an 18-month-old male who presented with a progressively increasing mass lesion in the left post-auricular region since birth. Radiological examination did not show any intracranial involvement of the mass lesion. Upon resecting the mass that was 10-cm in largest dimension, the gross, microscopic, and ultrastructural findings were consistent with congenital alveolar rhabdomyosarcoma.
The suspicion of alveolar subtype on histological grounds and proper evaluation of this tumor by immunostain and ultrastuctural examination is necessary. In the Medline literature search, there is no report of large congenital alveolar rhabdomyosarcoma in the post-auricle region.
先天性肺泡横纹肌肉瘤极为罕见，采用当前治疗方法时其总是致命的。不到25%的患者有皮肤转移迹象。
我们报告一例18个月大男性的先天性肺泡横纹肌肉瘤病例，该患儿自出生以来左耳后区域出现一个逐渐增大的肿块病变。放射学检查未显示该肿块病变有任何颅内累及。切除最大直径为10厘米的肿块后，大体、显微镜及超微结构检查结果均符合先天性肺泡横纹肌肉瘤。
基于组织学依据怀疑为肺泡亚型，并通过免疫染色和超微结构检查对该肿瘤进行恰当评估很有必要。在医学在线文献检索中，尚无耳后区域巨大先天性肺泡横纹肌肉瘤的报告。
