Treatment of ruptured undifferentiated sarcoma of the liver in children: a report of two cases and review of the literature

Abstract

Undifferentiated (embryonal) sarcoma of the liver (USL) is a highly malignant tumor of early life. Treatment choices for USL, especially with intraperitoneal rupture, are uncertain. Outcomes have been almost uniformly poor until recently. We describe two 7-year-old girls treated for ruptured USL. In the more recent patient, operative biopsy was followed by three cycles of cisplatin (CDDP), adriamycin (ADR), and cyclophosphamide (CPM). A fluid-filled cavity in the tumor showed enlargement and was drained. Two cycles of CDDP, ADR, vincristine (VCR), and ifosfamide were accompanied by reduction in tumor size, and trisegmentectomy was performed. She has no evidence of disease 3.5 years after surgery. In the other patient, left lobectomy was followed by a less intensive regimen, including CPM, VCR, and fluorouracil. This patient died of dissemination within 5 months. In 170 reported pediatric patients with USL, the 2-year disease-free survival was 17%. For the 96 such patients reported since 1980, 2-year disease-free survival had improved to 27%. More aggressive chemotherapy has been associated with this change. Of 8 patients with tumor rupture whose details have been reported (including the 2 present patients) after resection of the tumor, 4 died, 1 was alive with disease, and 3 were free of disease at 8, 49, and 58 months, respectively, after diagnosis. Ruptured USL should be treated with combination chemotherapy including CDDP and ADR, as well as with curative resection.

Management of undifferentiated embryonal sarcoma of the liver in children: A case series and management review

“…There are more data on the treatment of patients with sarcoma protocols [7,8,13,14,26,27] than those with hepatoblastoma protocols. [17][18][19]30,31] Such reports do not lend themselves to rigorous statistical analysis, but the relatively high survival rate in patients treated on sarcoma protocols implies acceptable efficacy. Since the camptothecin analogues irinotecan and topotecan are promising new agents in treating rhabdomyosarcoma, they may prove to be of use in treating UESL.…”

Surgical Treatment and Chemotherapy of Adult Primary Liver Sarcoma: Experiences from a Single Hospital in China

“…However, the effects of chemotherapy for treating PLS in the literature are controversial, and a variety of chemotherapeutic regimens were used in these case reports, including doxorubicin, ifosfamide, vincristine, actinomycin-D, cyclophosphamide (VAC), cisplatinum, carboplatinum, ifosfamide, and etoposide. European hospitals prefer VAC regimen or chemotherapy with a modified VAC backbone, but in China, we tend to use chemotherapy regimen (vincristin, cisplatin, cyclophosphamide and adriamycin) [24-29]. Merli et al [27] treated 13 undifferentiated liver sarcoma patients with surgery and postoperative chemotherapy according to the European protocol (7 patients with neoadjuvant chemotherapy).…”

“…Li et al [28] reported that an undifferentiated embryonal sarcoma of the liver patient who underwent interventional therapy and surgical resection exhibited a prolonged survival compared with patients who underwent surgical resection only. Uchiyama et al [29] proposed that surgical resection combined with chemotherapy is the most effective therapy for undifferentiated embryonal sarcoma of the liver patients with tumor rupture. Recently, Noguchi et al [30] reported a long-term survival case of USEL who was treated with a high dose of etoposide and radiation therapy, then followed with peripheral blood stem cell trans-plantation.…”

Sarcoma indiferenciado (embrionário) do fígado: relato de caso e revisão da literatura

“…O sarcoma embrionário é considerado uma neoplasia agressiva e de prognóstico desfavorável, com sobrevida de 1 a 2 anos. Nos casos em que há predomínio de padrão cístico, a ruptura da neoplasia é uma complicação importante (7,22) . Nas últimas décadas, o prognóstico dos pacientes com sarcoma indiferenciado do fígado melhorou expressivamente, com a associação da ressecção cirúrgica completa do tumor e tratamentos adjuvantes, principalmente novos protocolos quimioterápicos (2,23,24) .…”