Intraabdominal desmoplastic small round cell tumor: results of ifosfamide-based chemotherapy.
Intraabdominal desmoplastic small round cell tumor (IDSRT) is a rare neoplasm, which is malignant and typically occurs in young adults. Although IDSRT is chemotherapy-sensitive, most cases do not achieve a complete response (CR). Even if some cases achieve a CR, they often relapse and result in poor prognosis. We report two cases of IDSRT, in a 24-year-old man and a 23-year-old man, who were treated with ifosfamide-based chemotherapy. Case 1, a 24-year-old man, presented with an abdominal mass, and the diagnosis of the biopsy specimen at laparoscopy was IDSRT. He received 7 courses of ifosfamide-based chemotherapy, which obtained a partial response (PR). He had no surgical resection, except for the biopsy procedure, and he underwent a total of 21 courses of chemotherapy. He died 36 months after diagnosis because of tumor regrowth and peritoneal bleeding. Case 2, a 23-year-old man, whose biopsy material allowed a diagnosis of IDSRT, was treated with 18 courses of chemotherapy, using ifosfamide, that resulted in a PR. This was followed by resection of the abdominal lesions, and he received an additional 2 courses of adjuvant chemotherapy. Four months after, completion of these 2 courses, the tumor recurred, and he died of diffuse pulmonary metastasis 36 months after diagnosis. This case report examines the results of ifosfamide-based chemotherapy in our two patients.
Suehara
Yoshiyuki
Y
Department of Orthopedic Oncology, Tochigi Cancer Center, 4-9-13 Younan, 320-0834, Utsunomiya, Japan.
Yazawa
Yasuo
Y
Hitachi
Kouhei
K
D000008:Abdominal Neoplasms / Q000000981:diagnostic imaging / Q000188:drug therapy* / Q000473:pathology; D000328:Adult; D018906:Antineoplastic Agents, Alkylating / Q000627:therapeutic use*; D017809:Fatal Outcome; D006801:Humans; D007069:Ifosfamide / Q000627:therapeutic use*; D008297:Male; D018193:Neoplasms, Complex and Mixed / Q000000981:diagnostic imaging / Q000188:drug therapy* / Q000473:pathology; D014057:Tomography, X-Ray Computed
D018906:Antineoplastic Agents, Alkylating; D007069:Ifosfamide