Rhabdomyosarcoma of the biliary tree in childhood. A report from the intergroup rhabdomyosarcoma study

Fb, Ruymann; Rb, Raney; Wm, Crist; Lawrence, Walter; Rd, Lindberg; Eh, Soule

doi:10.1002/1097-0142(19850801)56:3<575::aid-cncr2820560326>3.0.co;2-9

Cited by 88 publications

(11 citation statements)

References 10 publications

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“…Rhabdomyosarcoma (RMS) is the most common soft tissue sarcoma of the childhood, accounting for about 5% of all pediatric malignancies [1]. Rhabdomyosarcoma of the biliary tree represents only about 0.5% of all pediatric RMS [1, 2]. Biliary RMS usually arises in the common bile duct, but it can originate from anywhere along the biliary tree [1–4].…”

Section: Introductionmentioning

confidence: 99%

“…Late recognition of the biliary tree malignancy, its critical location, and frequent extension into the liver are the main factors responsible for diminished survival expectancy in a tumor of otherwise favorable histology. In 1985 the Intergroup Rhabdomyosarcoma Study Group (IRSG) reported the first series of 10 cases of biliary RMS treated on IRS (Intergroup Rhabdomyosarcoma Study) I and II protocols between 1972 and 1984 [2]. At that time the outcome for patients was poor and only 4/10 patients survived.…”

Section: Introductionmentioning

confidence: 99%

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Treatment and outcome of the patients with rhabdomyosarcoma of the biliary tree: Experience of the Cooperative Weichteilsarkom Studiengruppe (CWS)

et al. 2019

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Background Biliary rhabdomyosarcoma (RMS) is the most common biliary tumor in children. The management of affected patients contains unique challenges because of the rarity of this tumor entity and its critical location at the porta hepatis, which can make achievement of a radical resection very difficult. Methods In a retrospective chart analysis we analysed children suffering from biliary RMS who were registered in three different CWS trials (CWS-96, CWS-2002P, and SoTiSaR registry). Results Seventeen patients (12 female, 5 male) with a median age of 4.3 years were assessed. The median follow-up was 42.2 months (10.7–202.5). The 5-year overall (OS) and event free survival (EFS) rates were 58% (45–71) and 47% (34–50), respectively. Patients > 10 years of age and those with alveolar histology had the worst prognosis (OS 0%). Patients with botryoid histology had an excellent survival (OS 100%) compared to those with non-botryoid histology (OS 38%, 22–54, p = 0.047). Microscopic complete tumor resection was achieved in almost all patients who received initial tumor biopsy followed by chemotherapy and delayed surgery. Conclusion Positive predictive factors for survival of children with biliary RMS are age ≤ 10 years and botryoid tumor histology. Primary surgery with intention of tumor resection should be avoided.

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Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Treatment and outcome of the patients with rhabdomyosarcoma of the biliary tree: Experience of the Cooperative Weichteilsarkom Studiengruppe (CWS)

et al. 2019

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“…Biliary rhabdomyosarcoma (RMS) is a rare primary malignancy, but the most likely primary tumour to cause biliary tract obstruction, and can be accompanied by abdominal distension and hepatomegaly [62, 63]. Occasionally, it may be misinterpreted as a hepatic mass such as a hepatoblastoma, which can also present with jaundice [64] or as an undifferentiated embryonal sarcoma due to the shared histopathological similarities (although embryonal sarcomas rarely cause biliary obstruction [65]).…”

Section: Abdominal Emergenciesmentioning

confidence: 99%

Emergency imaging in paediatric oncology: a pictorial review

et al. 2019

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Despite the decline in mortality rates over the last 20 years, cancer remains one of the leading causes of death in children worldwide. Early recognition and treatment for acute oncological emergencies are vital in preventing mortality and poor outcomes, such as irreversible end-organ damage and a compromised quality of life. Imaging plays a pivotal and adjunctive role to clinical examination, and a high level of interpretative acumen by the radiologist can make the difference between life and death. In contrast to adults, the most accessible crosssectional imaging tool in children typically involves ultrasound. The excellent soft tissue differentiation allows for careful delineation of malignant masses and along with colour Doppler imaging, thromboses and large haematomas can be easily identified. Neurological imaging, particularly in older children is an exception. Here, computed tomography (CT) is required for acute intracranial pathologies, with magnetic resonance imaging (MRI) providing more definitive results later. This review is divided into a 'body systems' format covering a range of pathologies including neurological complications (brainstem herniation, hydrocephalus, spinal cord compression), thoracic complications (airway obstruction, superior vena cava syndrome, cardiac tamponade), intra-abdominal complications (bowel obstruction and perforation, hydronephrosis, abdominal compartment syndrome) and haematological-related emergencies (thrombosis, infection, massive haemorrhage). Within each subsection, we highlight pertinent clinical and imaging considerations. The overall objective of this pictorial review is to illustrate how primary childhood malignancies may present with lifethreatening complications, and emphasise the need for imminent patient management.

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“…A review of the >1600 patients treated on the Intergroup Rhabdomyosarcoma Studies I and II evaluated the 10 cases of hepatobiliary RMS enrolled. Representing <1% of the cases, this is a rare entity [ 2 ]. Mean age reported was three years old with a range of one to nine years old.…”

Section: Introductionmentioning

confidence: 99%

“…Its location provides for clinically challenging approaches given relative inaccessibility to achieve gross resection and the functional complications presented by biliary obstruction. In spite of the therapeutic challenges that the location presents, it is classified as a favorable site; and, as outcomes are generally successful, low or intermediate-risk systemic therapy regimens are commonly employed [ 2 ]. Case reports reveal that good long-term outcomes are possible with acute management of biliary obstruction and aggressive oncologic management [ 4 - 6 ].…”

Section: Introductionmentioning

confidence: 99%

Pencil Beam Scanning Proton Therapy for Rhabdomyosarcoma of the Biliary Tract

Pater

Turpin

Mascia

2017

Cureus

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Rhabdomyosarcoma (RMS) is the most common soft tissue sarcoma of childhood with 250-350 cases diagnosed annually in the United States. Biliary tract rhabdomyosarcoma is rare, representing <1% of the RMS cases. Due to its location, resection is clinically challenging, and functional complications exist and persist from biliary obstruction. The anatomical location of this tumor presents both opportunities and challenges for pencil beam scanning proton therapy. Proton therapy offers a dosimetric and clinical advantage by sparing the healthy liver, stomach, contra-lateral kidney and bowel. Motion management and anatomical variations, such as intestinal filling or weight loss, requiring routine dosimetric evaluation and possible adaptive treatment planning, present challenges for the use of proton therapy. By taking advantage of the superior dose distribution of proton radiation, assessing the impact of tumor and anatomy motion, and performing regular dose evaluations, biliary tract RMS is an ideal diagnosis for pencil beam scanning proton therapy.

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Rhabdomyosarcoma of the biliary tree in childhood. A report from the intergroup rhabdomyosarcoma study

Cited by 88 publications

References 10 publications

Treatment and outcome of the patients with rhabdomyosarcoma of the biliary tree: Experience of the Cooperative Weichteilsarkom Studiengruppe (CWS)

Treatment and outcome of the patients with rhabdomyosarcoma of the biliary tree: Experience of the Cooperative Weichteilsarkom Studiengruppe (CWS)

Emergency imaging in paediatric oncology: a pictorial review

Pencil Beam Scanning Proton Therapy for Rhabdomyosarcoma of the Biliary Tract

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