Radical surgical treatment of neuroendocrine tumors of the appendix in children - a Polish multicenter study

Małgorzata Panek; Marek Szymczak; Maria Stepaniuk; Wojciech Górecki; Aleksandra Gawłowska-Marciniak; Przemysław Wolak; Dariusz Zbyrad; Marek Rybkiewicz; Kamila Chrobak; Ryszard Noparlik; Jerzy Niedzielski; Przemysław Janik; Jan Nowak; Witold Miaśkiewicz; Andrzej Kamiński; Wojciech Fischer; Bożena Dembowska-Bagińska; Piotr Kaliciński

doi:10.5114/aoms/135706

Radical surgical treatment of neuroendocrine tumors of the appendix in children - a Polish multicenter study

Arch Med Sci. 2021 Apr 23;17(4):1128-1131. doi: 10.5114/aoms/135706. eCollection 2021.

Authors

Małgorzata Panek¹, Marek Szymczak¹, Maria Stepaniuk², Wojciech Górecki³, Aleksandra Gawłowska-Marciniak⁴, Przemysław Wolak⁵, Dariusz Zbyrad⁶, Marek Rybkiewicz⁷, Kamila Chrobak⁸, Ryszard Noparlik⁹, Jerzy Niedzielski¹⁰, Przemysław Janik¹¹, Jan Nowak¹², Witold Miaśkiewicz¹³, Andrzej Kamiński¹⁴, Wojciech Fischer¹⁵, Bożena Dembowska-Bagińska¹⁶, Piotr Kaliciński¹

Affiliations

¹ Department of Pediatric Surgery and Organ Transplantation, The Children's Memorial Health Institute, Warsaw, Poland.
² Department of Pathology, The Children's Memorial Health Institute, Warsaw, Poland.
³ Department of Pediatric Surgery, Jagiellonian University Medical College, Krakow, Poland.
⁴ Department of Pediatric Surgery and Oncology, Central Clinical Hospital Medical University of Lodz, Lodz, Poland.
⁵ Department of Pediatric Surgery, Urology and Traumatology, Wladyslaw Buszkowski Children's Hospital, Kielce, Poland.
⁶ Department of Pediatric Surgery and Traumatology, Provincial Hospital, Tarnobrzeg, Poland.
⁷ Department of Pediatric and Oncological Surgery, Urology and Hand Surgery, Pomeranian Medical University, Szczecin, Poland.
⁸ Department of Children's Developmental Defects Surgery and Traumatology, Medical University of Silesia in Katowice, Zabrze, Poland.
⁹ Department of Pediatric Surgery, University Clinical Hospital, Opole, Poland.
¹⁰ Department of Pediatric Surgery and Urology, Medical University of Lodz, Lodz, Poland.
¹¹ Department of Urology and Pediatric Surgery, The Ludwik Rydygier Provincial Polyclinic Hospital, Torun, Poland.
¹² Department of Pediatric Surgery and Urology, Karol Marcinkowski University Hospital, Zielona Gora, Poland.
¹³ Department of Pediatric Surgery and Traumatology, ZZOZ, Ostrow Wielkopolski, Poland.
¹⁴ Department of Pediatric Surgery and Urology, The Autonomous Public Children's Clinic Hospital of the Medical University of Warsaw, Warsaw, Poland.
¹⁵ Department of Pediatric Surgery, Hospital Municipal St. John Paul II, Elblag, Poland.
¹⁶ Department of Pediatric Oncology, The Children's Memorial Health Institute, Warsaw, Poland.

Abstract

Introduction: The aim of the study was to examine management of pediatric appendiceal neuroendocrine tumors (ANETs) in Poland.

Methods: Records of 27 patients with ANET diagnosed incidentally after appendectomy in the last decade.

Results: Well-differentiated NET G1/G2 was diagnosed in 25 and well-differentiated neuroendocrine carcinoma G3 in 2 patients. Extended surgery was performed primarily in one instance and secondarily in 10 patients (right hemicolectomy in 9, ileocecal resection in 1) without adjuvant chemotherapy. Follow-up range was 1-121 months. Recurrence after secondary surgery was observed in 1 (3.7%) patient.

Conclusions: Applying ENETS guidelines resulted in 100% overall survival of patients with NET.

Keywords: appendiceal tumor; appendix; carcinoid; neuroendocrine neoplasm; neuroendocrine tumor.