Pauser Ursula, Grimm Horst
Department of Pathology, University of Lübeck, Lübeck, Germany.
World J Surg Oncol. 2008 Dec 14;6:131. doi: 10.1186/1477-7819-6-131.
Leiomyosarcomas of the stomach are very rare. At the time of primary diagnosis the tumors are most often in advanced stage and the patients complain of abdominal pain due to large tumor size. Endosonographically, the tumors impress as submucous mass with suspicion to malignancy. Sarcomas following hereditary retinoblastoma in childhood are in generally located in the soft tissue. Structural alterations of the retinoblastoma gene (RB1) seem to be involved in the pathogenesis.
A 37-year-old german male suffered from reflux disorder. In endoscopic examination a small polypous tumor was detected in the stomach. The resection specimen revealed an intramucosal leiomyosarcoma. At the age of one year, the patient had a retinoblastoma.
This is the unique report of an intramucosal gastric leiomyosarcoma and the first account of a gastric leiomyosarcoma after retinoblastoma in childhood. A careful clinical follow-up is advised because of increased risk of developing further metachronous malignancies.
胃平滑肌肉瘤非常罕见。在初次诊断时，肿瘤通常处于晚期，患者因肿瘤体积大而主诉腹痛。内镜超声检查显示，肿瘤表现为可疑恶性的黏膜下肿块。儿童遗传性视网膜母细胞瘤后的肉瘤一般位于软组织中。视网膜母细胞瘤基因（RB1）的结构改变似乎与发病机制有关。
一名37岁德国男性患有反流性疾病。内镜检查发现胃内有一个小的息肉样肿瘤。切除标本显示为黏膜内平滑肌肉瘤。该患者1岁时患视网膜母细胞瘤。
这是关于黏膜内胃平滑肌肉瘤的独特报告，也是儿童视网膜母细胞瘤后胃平滑肌肉瘤的首例报道。鉴于发生进一步异时性恶性肿瘤的风险增加，建议进行仔细的临床随访。
