Nathan J D, Gingalewsik C, Salem R R
Department of Surgery, Yale University, School of Medicine, New Haven, Connecticut, USA.
Yale J Biol Med. 1999 Jul-Aug;72(4):287-93.
Intra-abdominal desmoplastic small round cell tumor is a rare malignancy with a predilection for young males. Unique histological and immunocytochemical features distinguish the tumor from other members of the family of small round cell tumors of infancy and childhood. The aggressive nature of tumor spread, relative insensitivity to chemotherapy, and generally incomplete resectability result in a very poor prognosis. The authors report a case of a 39-year-old man with diffuse abdominal and pelvic involvement of intra-abdominal desmoplastic small round cell tumor treated with aggressive chemotherapy and surgery.
Computed-tomography (CT)-guided biopsy of an omental mass was performed. Histologically, discrete nests of uniform closely packed malignant cells were distributed in a background of focally desmoplastic stroma. Immunocchemistry demonstrated positivity for epithelial, mesenchymal, and neural markers. On the basis of these unique histological and immunohistochemical characteristics, the diagnosis of desmoplastic small round cell tumor was made. The patient was treated with aggressive neoadjuvant chemotherapy consisting of a high-dose alkylator -based combination regimen, followed by surgery.
The patient had a 10 to 15 percent regression in tumor mass in response to chemotherapy. Laparotomy revealed two large omental masses, another large mass adherent to the left colon and pelvic sidewall, and diaphragmatic, peritoneal and mesenteric studding with small nodules. Complete surgical resection was not possible.
Intra-abdominal desmoplastic small round cell tumor remains an aggressive malignancy with an extremely poor prognosis. Although some response to chemotherapy may be possible, complete resection is rare, and surgical efforts are general palliative.
腹腔促纤维增生性小圆细胞肿瘤是一种罕见的恶性肿瘤，好发于年轻男性。独特的组织学和免疫细胞化学特征将该肿瘤与婴幼儿及儿童期小圆细胞肿瘤家族中的其他成员区分开来。肿瘤侵袭性生长、对化疗相对不敏感以及通常无法完全切除，导致预后极差。作者报告了一例39岁男性腹腔促纤维增生性小圆细胞肿瘤弥漫性累及腹部和盆腔，接受了积极化疗和手术治疗的病例。
对网膜肿物进行计算机断层扫描（CT）引导下活检。组织学上，均匀紧密排列的恶性细胞离散巢状分布于局灶性促纤维增生性间质背景中。免疫化学显示上皮、间充质和神经标志物呈阳性。基于这些独特的组织学和免疫组化特征，诊断为促纤维增生性小圆细胞肿瘤。患者接受了以高剂量烷化剂为基础的联合方案的积极新辅助化疗，随后进行手术。
患者化疗后肿瘤体积缩小10%至15%。剖腹探查发现两个大网膜肿物、另一个附着于左结肠和盆腔侧壁的大肿物以及膈肌、腹膜和肠系膜上散在小结节。无法进行完整的手术切除。
腹腔促纤维增生性小圆细胞肿瘤仍然是一种侵袭性恶性肿瘤，预后极差。尽管化疗可能有一定反应，但完整切除很少见，手术通常是姑息性的。
