Abstract
Intraabdominal Desmoplastic Small Round Cell Tumour (IDSRCT) is a very rare neoplasia with a unique immunoprofile. Children and young adults are most commonly affected. We report two cases with IDSRCT in children who initially presented with ascites, pain and abdominal mass. Complete surgical excision was possible only in one patient. Although both patients underwent multiagent chemotherapy, they had a relapse of the disease. One patient died two years after diagnosis. The tumour has a very poor prognosis. Survival is correlated to the radical resection of the tumour combined with intense chemotherapy and radiotherapy.
Résumé
La tumeur desmoplastique intra-abdominale à petite cellule ronde (IDSRCT) est une néoplasie très rare avec un profil immunologique particulier. Les enfants et les jeunes adultes sont le plus souvent touchés. Nous rapportons 2 cas avec IDSRCT chez des enfants qui se sont présentés initialement avec de l'ascite, des douleurs et une masse abdominale. Une excision chirurgicale complète a été possible seulement chez un patient. Quoique les 2 patients soient traités par une polychimiothérapie, il y a eu une récidive de la tumeur. Un patient est décédé 2 ans après le diagnostic. La tumeur a un pronostic très pauvre. La survie est en relation avec une résection radicale de la tumeur combinée avec une chimiothérapie intense et une radiothérapie.
Resumen
El tumor intraabdominal desmoplástico de células redondas pequeñas (IDSRCT) es una neoplasia muy rara con un perfil inmunológico único. Los niños y los adultos jóvenes son los más frecuentemente afectados. Presentamos dos casos de IDSRCT en niños que se presentaron inicialmente con ascitis, dolor y masa abdominal. Solamente fue posible la extirpación completa en un caso. Aunque ambos pacientes se trataron con quimioterapia combinada presentaron recidivas de la enfermedad. Uno de los niños murio dos años tras el diagnóstico. El tumor tiene un pronóstico malo. La supervivencia se correlaciona con la resección radical del tumor combinada con quimioterapia intensa y radioterapia.
Zusammenfassung
Der intraabdominelle, desmoplastische Rundzelltumor ist eine sehr seltene Geschwulst mit einem ungewöhnlichen Immunprofil. Kinder und junge Erwachsene sind am häufigsten betroffen. Es werden zwei Fälle dieser Tumorart vorgestellt, wobei die Kinder mit Aszites, Schmerzen und einem tastbaren Bauchtumor zur Aufnahme kamen. Nur bei einem Patienten konnte der Tumor vollständig entfernt werden. Beide Kinder erhielten eine umfassende Chemotherapie, bei beiden kam es jedoch zu einem Tumorrezidiv. Ein Kind verstarb 2 Jahre nach der Diagnose. Der kleinzellige Rundzelltumor des Abdomens hat eine schlechte Prognose. Das Überleben hängt von der radikalen Entfernung des gesamten Tumorgewebes in Kombination mit einer intensiven Chemo- und Radiotherapie ab.
Key words
Desmoplastic small round cell tumour - Childhood
Mots-clés
Tumeur desmoplastique à petite cellule ronde - Enfance
Palabras clave
Tumor desmoplastico de células redondas pequeñas - Niños
Schlüsselwörter
Desmoplastischer Kleinzelltumor im Kindesalter
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