Case Report: Pediatric Hepatic Rhabdomyosarcoma With Maximum Lifetime

Abstract

Primary intrahepatic rhabdomyosarcoma is an extremely rare malignant tumor. Here, we describe a case of embryonal rhabdomyosarcoma of the liver in a 7-year-old boy without any symptoms. Serologically, the patient showed abnormal levels of serum tumor markers and liver function. Imaging revealed a large mass in the left lobe of the liver and no lesions elsewhere. At first, the patient was misdiagnosed by percutaneous liver biopsy as having clear cell sarcoma. However, the final diagnosis was established to be hepatic embryonal rhabdomyosarcoma based on postoperative histopathology and typical immunohistochemical staining, which was positive for desmin and myogenin. For treatment, the patient received two cycles of preoperative chemotherapy, prophylactic radiotherapy, and 13 cycles of combined postoperative chemotherapy. Routine follow-ups after all treatment conducted by imaging examinations showed no sign of recurrence or metastasis over 13 months, and the patient survives more than 38 months since initial diagnosis. To our knowledge, this patient is the first with hepatic rhabdomyosarcoma to receive neoadjuvant chemotherapy (preoperative chemotherapy) combined with relative comprehensive treatment and achieve a favorable result.

Pleomorphic rhabdomyosarcoma of the liver in an adult: An extremely rare case report and review of literature

“…The Pleomorphic subtype is the most common one in advanced age groups, while the Alveolar and Embryonal subtypes occurred more in pediatric and teenage groups. [ 6 ] Only 7 cases of pleomorphic hepatic RMS have been reported and our case is the 8th one. There are only 22 cases of hepatic RMS presented in adults, and our case is the 23rd [ 2 , 6 , 7 ] ( Table.…”

About a Large Botryoid Rhabdomyosarcoma in a Little Girl: Management Difficulties and Literature Review

“…We could not make an objective assessment of the OS time even though the significant tumoral decrease was an encouraging result. For many authors in literature, tumoral prognosis is severe [18][19][20]. Some authors suggested that most patients are stratified into high-, intermediate-, and lowrisk groups based on tumor size, tumor invasiveness, nodal status, primary tumor site, and pathological and molecular parameters (PAX-fusion status) [18,19].…”

“…Some authors suggested that most patients are stratified into high-, intermediate-, and lowrisk groups based on tumor size, tumor invasiveness, nodal status, primary tumor site, and pathological and molecular parameters (PAX-fusion status) [18,19]. In other words, prognosis for RMS depends on primary tumor site, age, completeness of resection, presence and number of metastatic sites, histology, and biology of the tumor cells [18][19][20]. In addition, the overall cure rates have improved significantly thanks to the multimodal therapies, including surgery, radiotherapy, and chemotherapy (with various combinations of vincristine, actinomycin D, cyclophosphamide, etoposide, irinotecan, or ifosfamide).…”

“…In addition, the overall cure rates have improved significantly thanks to the multimodal therapies, including surgery, radiotherapy, and chemotherapy (with various combinations of vincristine, actinomycin D, cyclophosphamide, etoposide, irinotecan, or ifosfamide). In recent studies, it was found that surgical therapy is the key basis and significant treatment for a better prognosis in patients [20]. The mean survival time with patients who underwent surgery is 16.7 months, whereas patients with non-surgical therapy is only of 9.5 months.…”

Primary Liver Rhabdomyosarcoma in a Burnt-out disease: An unusual presentation

“…The longest surviving case of hepatic RMS was the only one in literature to have received multimodality treatment. (9) Multi-modality management with surgery, radiation therapy, and chemotherapy may be a key factor affecting survival rate. (1)…”