Biliary tract rhabdomyosarcoma: a report from the Soft Tissue Sarcoma Committee of the Associazione Italiana Ematologia Oncologia Pediatrica

Perruccio, Katia; Cecinati, Valerio; Scagnellato, Angela; Provenzi, Massimo; Milano, Giuseppe Maria; Basso, Eleonora; Manzitti, Carla; Cecchetto, Giovanni; Alaggio, Rita; Martino, Martina Di; Schiavetti, Amalia; Melchionda, Fraia; Affinita, Maria Carmen; Chiaravalli, Stefano; Miglionico, L; Balter, Rita; Tamburini, Angela; Bisogno, Gianni; Ferrari, Andrea

doi:10.5301/tj.5000692

Cited by 14 publications

(16 citation statements)

References 18 publications

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“…In these cases, radiotherapy may be proposed to obtain a cure and avoid local recurrence. In rare cases where radical resection was difficult to achieve, LT has been proposed to ensure a radical resection, and the authors argue that the radicality of the operation allows avoiding irradiation in a child and its long-term effects (scholastic effect, effect on growth and function of the regional organs, thoraco-vertebral deformities), which balances the need for long-term immunosuppression [34][35][36].…”

Section: Rhabdomyosarcoma Of Livermentioning

confidence: 99%

Liver Transplantation for Pediatric Liver Cancer

Sindhi

Rohan

Tadros

et al. 2020

Cancers

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Unresectable hepatocellular carcinoma (HCC) was first removed successfully with total hepatectomy and liver transplantation (LT) in a child over five decades ago. Since then, children with unresectable liver cancer have benefitted greatly from LT and a confluence of several equally important endeavors. Regional and trans-continental collaborations have accelerated the development and standardization of chemotherapy regimens, which provide disease control to enable LT, and also serve as a test of unresectability. In the process, tumor histology, imaging protocols, and tumor staging have also matured to better assess response and LT candidacy. Significant trends include a steady increase in the incidence of and use of LT for hepatoblastoma, and a significant improvement in survival after LT for HCC with each decade. Although LT is curative for most unresectable primary liver sarcomas, such as embryonal sarcoma, the malignant rhabdoid tumor appears relapse-prone despite chemotherapy and LT. Pediatric liver tumors remain rare, and diagnostic uncertainty in some settings can potentially delay treatment or lead to the selection of less effective chemotherapy. We review the current knowledge relevant to diagnosis, LT candidacy, and post-transplant outcomes for these tumors, emphasizing recent observations made from large registries or larger series.

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Section: Rhabdomyosarcoma Of Livermentioning

confidence: 99%

Liver Transplantation for Pediatric Liver Cancer

Sindhi

Rohan

Tadros

et al. 2020

Cancers

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“…Initial misdiagnosis of these rare tumors is frequent, with patients being operated on for a presumed choledochal cyst malformation (Figure ) . Except for the Intergroup Rhabdomyosarcoma Study Group (IRSG) that reported 21 patients with localized RMS registered in IRS I‐IV studies and the Italian group that recently reported 10 patients, only case reports have been published. Several options are available for local treatment: external beam radiotherapy (EBRT), surgery, or a combination of both .…”

Section: Introductionmentioning

confidence: 99%

Outcome of localized liver‐bile duct rhabdomyosarcoma according to local therapy: A report from the European Paediatric Soft‐Tissue Sarcoma Study Group (EpSSG)‐RMS 2005 study

Guérin

Rogers

Minard‐Colin

et al. 2019

Pediatric Blood & Cancer

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Objectives To evaluate the impact of local therapies on the outcome of patients with liver‐bile duct rhabdomyosarcoma (LBDRMS). Methods Data of 30 patients included in the EpSSG‐RMS 2005 study were analyzed. Results The median age at diagnosis was 3 years (11 months–8 years). All patients had non‐alveolar histology. Fifteen patients had a tumor > 5 cm and six had enlarged regional lymph nodes on imaging. Eight patients (27%) had primary surgery (1 R0). Six of them received external beam radiotherapy (EBRT). All are in first complete remission (CR1) except one (R1, EBRT+, local relapse, death). Six patients (20%) received EBRT without surgery: one had local relapse and died. Sixteen patients (53%) underwent delayed surgery, with 12 achieving R0 margins, which were higher than those in the primary surgery group (P = 0.003). Three patients with R0 margins received EBRT; one had a metastatic relapse and died. Nine patients with R0 resection did not receive EBRT, three relapsed locally (two deaths). Four R1 patients received additional EBRT without relapses. Local relapse occurred in two among 19 patients with EBRT and three among 11 without EBRT (P = 0.326). At a median follow‐up of 61 months (48–84 months), five patients died; all had a tumor size > 5 cm (P = 0.01). The five‐year overall survival was 85% (95% CI, 65–94), and event‐free survival was 76% (95% CI, 54–89). Conclusion This analysis did not show any significant difference in outcome between irradiated and nonirradiated patients. Local relapse in LBDRMS is related to initial tumor size and is often fatal.

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“…RMS is a rare malignant tumor affecting children, forming about 2% present at birth [ 7 ], it is divided into five major subtypes; Embryonal subtype is considered the most common one (60% of total RMS), and has a better prognosis than other histologic subtypes [ 6 ]. CBD affection still rare, but it is the most common cause of malignant obstructive jaundice in children [ 3 , 5 ].…”

Section: Discussionmentioning

confidence: 99%

“…Around one hundred cases of CBD RMS reported in literature [ [2] , [3] , [4] , 6 , [13] , [14] , [15] ], the largest series was 25 cases over 25 years [ 15 ]. Most of the reported cases were difficult to diagnose and were misdiagnosed as a Choledochal Cyst [ [2] , [3] , [4] , [5] , 15 ], especially in confined tumor with no invasion to the surrounding structures [ 4 ].…”

Section: Discussionmentioning

confidence: 99%

“…However, they are rare as they constitute about 3% of all malignancies affecting pediatrics [ 1 ], with median age of 3.5 years and slight male predominance [ [2] , [3] , [4] , [5] ]. RMS is divided into five major subtypes; Embryonal subtype is considered the most common one [ 6 ]. The common sites in pediatric age group are head, neck, genitourinary, and retroperitoneal [ 7 ].…”

Section: Introductionmentioning

confidence: 99%

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Laparoscopic management of Rhabdomyosarcoma of common Bile duct, Case report

Quran

Rousan

Aljaafreh

et al. 2020

Annals of Medicine and Surgery

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Introduction Embryonal Rhabdomyosarcoma (ERMS) is a malignant soft tissue musculoskeletal tumor which constitutes about 0.06% of all malignancies affecting children. Biliary tract ERMS is still rare, though it is considered the most common cause of malignant obstructive jaundice in children. Case presentation A report of a 2-year-old boy, who was presented with recurrent episodes of scleral icterus of three months duration, is added to the related literature. His labs went with obstructive jaundice and the radiological investigations were consistent with a diagnosis of choledochal cyst. The found mass was suspected to be an ERMS of common bile duct and turned out to be so by the histopathology. He was managed totally by laparoscope, both excision and hepaticojejunostomy reconstruction, which is an extremely uncommon entity. Conclusion Common Bile Duct Rhabdomyosarcoma is rare and diagnosis at this anatomical site is difficult. Our case highlights the feasibility of laparoscopic resection and hepaticojejunostomy reconstruction with very good results at 16-month follow up and parents' gratitude as well.

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Biliary tract rhabdomyosarcoma: a report from the Soft Tissue Sarcoma Committee of the Associazione Italiana Ematologia Oncologia Pediatrica

Cited by 14 publications

References 18 publications

Liver Transplantation for Pediatric Liver Cancer

Liver Transplantation for Pediatric Liver Cancer

Outcome of localized liver‐bile duct rhabdomyosarcoma according to local therapy: A report from the European Paediatric Soft‐Tissue Sarcoma Study Group (EpSSG)‐RMS 2005 study

Laparoscopic management of Rhabdomyosarcoma of common Bile duct, Case report

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