Papillary Cystic and Solid Tumor of the Pancreas - Surgical Therapy with the Use of CUSA, and a Review of the Pediatric Literature

Abstract

Papillary cystic and solid tumor of the pancreas (PCSTP), socalled Frantz tumor, is a very rare tumor in children. Only 62 cases, 57 girls and 5 boys, have been reported in children since 1959. The tumor presents usually as a slowly growing abdominal mass with or without abdominal pain. Surgical resection of the tumor is an adequate mode of treatment. and the prognosis is excellent. The authors present 4 girls Resume Les tumeurs kystiques et solides du pancreas (PCSTP) appelees tumeurs de Frantz, sont tres rares chez l'enfant; seulement 62 cas, 57 filles et 5 gar<;ons, ont He rapportes depuis 1959. La tumeur se presente habituellement comme une masse abdominale avec ou sans douleur. I..:exerese chirurgicale est une modalite adequate de traitement; le pronostic est excellent. Les auteurs presentent 4 observations de PCSTP chez des filles et une chez un gar<;on; ils considerent que l'aspiration par Cavitron chirurgical ultrasonique est la therapeutique de choix. Mots-ch~s: Tumeur papillarre solide et kystique du pancreas chez l'enfant -Traitement chirurgical -Cavitron chirurgical ultrasonique -CUSA Resumen EI tumor papilar clStiCO y solido deI pancreas, llamado tumor de Frantz, es muy raro en niiios pues solo se han publicado 62 casos, 57 en niiias y 5 en niiios, desde 1.959. Este tumor se presenta generalmente corno una masa abdominal lentamente creciente con 0 sin dolor abdominal. La reseccion quirUrgica deI tumor es el modo adecuado de tratamiento y el pronostico es excelente.Los autores presentan 4 niiias y un niiio con este tumor y muestran que el aspirador ultrasonico puede usarse con exito para este tratamiento. Palabras c1ave: Tumor papilar clStiCO y solido deI pancreas en niiios -Tratamiento quirUrgico -Cavitron -Aspirador ultrasanico. Eur J Pediatr Surg 9 (1999) 416-419 © Hippokrates Verlag Stuttgart . Masson Editeur Paris and 1 boy with PCSTP and demonstrate that the Cavitron Ultrasonic Surgical Aspirator (CUSA. Valleylab) is successfully used in surgical therapy. Key words: Papillary cystic and solid tumor of the pancreas in children -Surgical treatment -Cavitron Ultrasonic Surgical Aspirator -CUSA Zusammenfassung Zystische und solide papilläTe Tumoren des Pankreas, die sogenannten Frantz-Tumoren sind sehr selten im Kindesalter. In der Literatur wurden seit 1959 nur 62 Fälle (57 Mädchen und 5 Knaben) beschrieben. Der Tumor zeigt sich in Form einer langsam wachsenden Abdominalgeschwulst, die nicht immer schmerzhaft ist. Die Therapie besteht in einer chirurgischen Resektion entsprechend dem Ausmaß und der Größe des Tumors. Die Prognose ist sehr gut. In der vorliegenden Arbeit werden 4 Mädchen und 1 Knabe mit einem Frantzschen Tumor vorgestellt und die Entfernung unter gleichzeitiger Verwendung eines Cavitron Ultraschallaspirators (CUSA, Valleylab) vorgestellt. Schlüsselwörter: Pankreastumor -UltraschallmesserStöm,

Solid pseudopapillary and malignant pancreatic tumors in childhood: A systematic review and evidence quality assessment

“…After screening titles and abstracts, 50 studies were retrieved for full‐text evaluation. Ultimately, 32 papers reporting on a total of 489 pediatric patients with pancreatic tumors satisfied predetermined search criteria and were included in this systematic review (Figure ) . Nine studies were conducted in the United States, five in Korea, four in Canada, and the rest of them came from Europe and Japan.…”

“…Data on postoperative complications was available in 20 papers . Pancreatic fistulae were reported in 11% of SPTs, ileus was described in 25% of PBLs, and fluid collections were found in 14.3% of exocrine pancreatic tumors (Table ).…”

“…Radiotherapy is generally considered unnecessary in SPTs and was performed only in 1% of systematically reviewed patients and in none of the cases registered with the SEER database . A few patients with advanced SPTs (3–4%) received neoadjuvant or postoperative chemotherapy (i.e., combination of cyclophosphamide, cisplatin, doxorubicin, and etoposide phosphate) . However, the role of chemotherapy in the management of these neoplasms remains unclear.…”

“…8 A few patients with advanced SPTs (3-4%) received neoadjuvant or postoperative chemotherapy (i.e., combination of cyclophosphamide, cisplatin, doxorubicin, and etoposide phosphate). 24,28,32 However, the role of chemotherapy in the management of these neoplasms remains unclear. Tamoxifen has also been used in estrogen receptor positive SPTs.…”

Solid-pseudopapillärer Pankreastumor im Kindesalter - Ein Bericht über 5 Fälle -

“…Bis 1998 wurden 292 Erkrankungen, davon 62 Fälle bei Kindern, beschrieben [2,18]. Seit 1959 werden nach der Erstbeschreiberin diese Tumoren auch als Frantz-Tumoren bezeichnet [17].…”