Livaditi E, Mavridis G, Soutis M, Papandreou E, Moschovi M, Papadakis V, Stefanaki K, Christopoulos-Geroulanos G
2nd Department of Pediatric Surgery, Aghia Sophia Children's Hospital, Athens, Greece.
Eur J Pediatr Surg. 2006 Dec;16(6):423-7. doi: 10.1055/s-2006-924736.
Intraabdominal desmoplastic small round cell tumors (IDSRCT) are rare in children and predominantly affect male adolescents and young adults. We present our experience in the management of five children with diffuse IDSRCT, managed with aggressive chemotherapy, surgery, radiotherapy and peripheral blood stem cell transplantation.
During the last decade five patients, four males and one female (mean age 9.6 years), with diffuse IDSRCT were managed in our department. The main symptoms were abdominal distention, vague abdominal pain, and vomiting. Three patients with inoperable tumor on admission were submitted initially to open biopsy followed by aggressive chemotherapy. Regression of the tumor was followed by a second laparotomy and radical excision of any macroscopically distinguishable masses, followed by chemotherapy. In the remaining two patients a debulking procedure was done initially, followed by chemotherapy. The accurate diagnosis of the disease was established by immunohistochemistry, additionally confirmed in the last two patients by molecular analysis.
Three patients who had radical excision of the tumor and adjuvant chemotherapy had recurrence after two to six months. In the remaining two patients, recurrence was evident after two and eighteen months, respectively, following debulking. In addition, one patient with recurrence received radiotherapy and two others underwent peripheral blood stem cell transplantation. All but one patient died within three years from diagnosis. The last patient, who was submitted to a debulking procedure, is still alive eight months after the operation.
Intrabdominal desmoplastic small round cell tumor is a highly aggressive malignancy with a very poor prognosis. Multiagent chemotherapy usually leads initially to a temporary regression of the tumor, but recurrence is the rule. Radical surgical excision, radiotherapy and peripheral blood stem cell transplantation does not seem to improve prognosis significantly. Despite all therapeutic modalities the outcome is dismal and surgical efforts can be considered only as palliative.
腹腔促结缔组织增生性小圆细胞肿瘤（IDSRCT）在儿童中罕见，主要影响男性青少年和青年成人。我们介绍了对5例弥漫性IDSRCT患儿的治疗经验，采用了积极的化疗、手术、放疗和外周血干细胞移植。
在过去十年中，我们科室治疗了5例弥漫性IDSRCT患者，4例男性和1例女性（平均年龄9.6岁）。主要症状为腹胀、腹部隐痛和呕吐。3例入院时无法手术切除的肿瘤患者最初接受了开放活检，随后进行积极化疗。肿瘤缩小后进行第二次剖腹手术，根治性切除任何肉眼可辨别的肿块，然后进行化疗。其余2例患者最初进行了减瘤手术，随后进行化疗。通过免疫组织化学确诊疾病，最后2例患者通过分子分析进一步证实。
3例接受肿瘤根治性切除和辅助化疗的患者在2至6个月后复发。其余2例患者在减瘤手术后分别于2个月和18个月后出现复发。此外，1例复发患者接受了放疗，另外2例接受了外周血干细胞移植。除1例患者外，所有患者均在诊断后3年内死亡。最后1例接受减瘤手术的患者术后8个月仍存活。
腹腔促结缔组织增生性小圆细胞肿瘤是一种侵袭性很强的恶性肿瘤，预后很差。多药化疗通常最初会使肿瘤暂时缩小，但复发是常见现象。根治性手术切除、放疗和外周血干细胞移植似乎并不能显著改善预后。尽管采用了所有治疗方式，结果仍然很糟糕，手术只能被视为姑息性治疗。
