Arias-Garzón Williams, Pacheco-Barsallo Felipe, Trujillo-Jácome Cecilia
Hospital Quito 1 de la Policía Nacional, Quito, Ecuador.
Cir Cir. 2011 Sep-Oct;79(5):458-63.
Medulloblastoma is a highly aggressive tumor of the posterior fossa. It is more common in children and has a very low incidence in adults. This tumor may cause endocranial hypertension, which requires placement of a ventriculoperitoneal shunt after surgical resection. It is possible, however, that neoplastic cells found in the cerebral spinal fluid can migrate through the shunt and implant themselves into the peritoneal cavity, producing multiple metastases that create great difficulty in treatment and offer a poor prognosis. This event is extraordinarily rare, which explains the few published cases and the lack of guidelines for treatment.
We present a 26-year-old male with a history of medulloblastoma of the cerebellum that was treated by complete tumor resection, radiotherapy and placement of a ventriculoperitoneal shunt. Two years later the patient demonstrated severe abdominal pain caused by the presence of multiple irresectable tumors in the abdomen and pelvis. A percutaneous biopsy documented metastatic medulloblastoma and the patient was treated with neoadjuvant chemotherapy based on schemes used for pediatric patients. There was a significant reduction in tumor activity that later allowed complete tumor resection.
In adult patients, peritoneal metastases from medulloblastoma are an unusual event. With this surgical and clinical case, we can suggest new guidelines for diagnosis and treatment to obtain satisfactory results.
髓母细胞瘤是一种发生于后颅窝的侵袭性很强的肿瘤。它在儿童中更为常见,在成人中的发病率很低。该肿瘤可能导致颅内高压,手术切除后需要放置脑室腹腔分流管。然而,脑脊液中发现的肿瘤细胞有可能通过分流管迁移并植入腹腔,形成多处转移,给治疗带来极大困难且预后不佳。这种情况极为罕见,这也解释了为何发表的病例较少且缺乏治疗指南。
我们报告一名26岁男性,有小脑髓母细胞瘤病史,接受了肿瘤全切、放疗及脑室腹腔分流管置入治疗。两年后,患者因腹部和盆腔出现多个无法切除的肿瘤而表现出严重腹痛。经皮活检证实为转移性髓母细胞瘤,患者接受了基于儿童患者方案的新辅助化疗。肿瘤活性显著降低,随后得以进行肿瘤全切。
在成年患者中,髓母细胞瘤的腹腔转移是一种罕见情况。通过这个手术和临床病例,我们可以提出新的诊断和治疗指南以获得满意的结果。