Treetipsatit Jitsupa, Kittikowit Wipawee, Zielenska Maria, Chaipipat Mookda, Thorner Paul Scott, Shuangshoti Shanop
Department of Pathology, Faculty of Medicine, Chulalongkorn University, Bangkok, Thailand.
Pediatr Dev Pathol. 2009 Sep-Oct;12(5):383-9. doi: 10.2350/08-08-0518.1.
Alveolar rhabdomyosarcoma (RMS) is 1 of 2 main subtypes of RMS in the pediatric age group and tends to occur in the extremities. The urogenital tract is another common site for RMS, but this typically involves the embryonal subtype including sarcoma botryoides. We report a 28-year-old male with a prostatic tumor that was excised en bloc and showed a RMS with separate areas of embryonal and solid alveolar morphologies at the light microscopic level. Both areas showed diffuse nuclear expression for myogenin, and both areas expressed the PAX3-FKHR fusion gene, a genetic change associated with alveolar but not embryonal RMS. A review of the literature documented only 5 cases of RMS primary to the prostate showing alveolar or mixed histology. Ours is the 6th case and the 1st with molecular findings. Although the diagnostic category of mixed embryonal/alveolar RMS remains in use, the nature of this type of RMS is incompletely understood. In our case, although the morphology was mixed embryonal/alveolar, at the genetic level this tumor was alveolar in nature.
肺泡状横纹肌肉瘤(RMS)是儿童年龄组中RMS的两种主要亚型之一,倾向于发生在四肢。泌尿生殖道是RMS的另一个常见部位,但这通常涉及胚胎型亚型,包括葡萄状肉瘤。我们报告一名28岁男性,其前列腺肿瘤被整块切除,在光镜下显示为RMS,具有胚胎型和实性肺泡状形态的不同区域。两个区域均显示肌生成素的弥漫性核表达,且两个区域均表达PAX3-FKHR融合基因,这是一种与肺泡状而非胚胎型RMS相关的基因改变。文献回顾仅记录了5例原发性前列腺RMS表现为肺泡状或混合组织学的病例。我们的病例是第6例,也是首例有分子学发现的病例。尽管混合性胚胎型/肺泡状RMS的诊断类别仍在使用,但这种类型RMS的性质尚未完全明确。在我们的病例中,尽管形态学上是混合性胚胎型/肺泡状,但在基因水平上该肿瘤本质上是肺泡状的。