Hepatic undifferentiated (embryonal) sarcoma and rhabdomyosarcoma in children. Results of therapy

Abstract

From July 1972 through September 1984, 8 of 44 children diagnosed as having primary malignant hepatic tumors, who were treated at St. Jude Children's Research Hospital, had undifferentiated (embryonal) sarcoma (five patients) or rhabdomyosarcoma (three patients). The natural history and response to multimodal therapy of these rare tumors are described. The pathologic material was reviewed and evidence for the differentiating potential of undifferentiated (embryonal) sarcoma is presented. At diagnosis, disease was restricted to the right lobe of the liver in three patients, was bilobar in four patients, and extended from the left lobe into the diaphragm in one patient. Lung metastases were present in two patients at diagnosis. All three patients with rhabdomyosarcoma had intrahepatic lesions without involvement of the biliary tree. Survival ranged from 6 to 73 months from diagnosis (median, 19.5 months); two patients are surviving disease‐free for 55+ and 73+ months, and one patient recently underwent resection of a recurrent pulmonary nodule 22 months from initial diagnosis. Three patients died of progressive intrahepatic and extrahepatic abdominal tumors, and two patients, who died of progressive pulmonary tumor, also had bone or brain metastasis but no recurrence of intra‐abdominal tumor. Six patients had objective evidence of response to chemotherapy. The authors suggest an aggressive multimodal approach to the treatment of these rare tumors in children.

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

“…[11,12] Many case reports and case series have detailed various combinations of treatment modalities, but only two report patients with UESL prospectively entered into a clinical trial. [13,14] Overall survival rates are difficult to assess, as few reports document long term follow-up. Early literature reports a poor prognosis with limited survival.…”

“…Patients with UESL cannot be cured using modalities that exclude surgery. [14] Where the lesion is deemed resectable on available radiology, the ideal approach is primary exploration with a view to complete resection and subsequent adjuvant therapy. Steiner et al [17] describe second-look laparotomy with biopsy of the tumour bed after initial resection and adjuvant chemotherapy to assess response.…”

“…[7][8][9][10][11][12][13][14][15][16][17][18][19][20] Liver transplantation has been performed for non-resectable tumours, [21] or for recurrent disease after previous resection, and has been suggested to offer more likelihood of cure than adding radiation. [22] Five such patients have been documented with claimed disease-free survival from 18 months to 5 years.…”

“…Of these tumours 62% showed reduction in volume following chemotherapy. 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.…”

“…Thus, due to the high incidence of local recurrence, radiotherapy may be justified as an adjunct to surgery and chemotherapy. [14,17,19,27,28,32,33] Neo-adjuvant chemoradiation has also been used to reduce tumour size, leading to total resection. [19] Based on largely anecdotal evidence, radiotherapy is reserved for high-risk patients, including those in whom tumour spillage or rupture has occurred or in whom surgical margins are positive.…”