骨盆腔惡性蠑螈瘤於第一型神經纖維瘤病男性患者: 病例報告
樊樂威、李威昌、劉忠一、張英勛
新北市立土城醫院 外科部 泌尿科
Malignant triton tumor of pelvis in a male with neurofibromatosis type 1: a case report
Le-Wei Fan, Wei-Chang Li, Chung-Yi Liu, Ying-Hsu Chang
Divisions of Urology, Department of Surgery, New Taipei City Municipal Tucheng Hospital, Taiwan
Abstract:
Malignant triton tumor (MTT) is a rare subgroup of tumors with rhabdomyoblastic differentiation within the heterogeneous group of malignant peripheral nerve sheath tumors (MPNST). It shows an aggressive course and poor prognosis. More than half of the cases present symptoms related to neurofibromatosis type 1 disease. To date, there is still no standardized treatment, the mainstay of treatment is complete surgical resection, and adjuvant radiotherapy or chemotherapy is used frequently to strengthen the control of local disease and prolong survival.
Here we report a case of 17-year-old healthy male with neurofibromatosis type 1 disease who had complained of low abdominal pain for 2 months and underwent excision biopsy at another hospital for huge pelvic tumor with sigmoid colon and urinary bladder compression. We performed complete tumor resection and sigmoid colectomy with colo-rectal anastomosis and transient loop transverse colostomy, bladder was preserved without sequela of low urinary tract symptoms. On resection, diagnosis was confirmed by histological findings revealing an MTT with glandular differentiation, FNCLCC grade 3, invasion to sigmoid colon and rectum. We also arranged adjuvant chemotherapy and clinic follow-up.
With our experience, MTT is growing so fast that the tumor grew up about twice in size within 2-moth after excision biopsy. It caused anemia due to occult internal bleeding and deterioration of abdominal distention. We suggest close monitor the patients with NF1 and perform complete tumor excision once tentative diagnosis is anticipated.