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

Panek, M; Szymczak, Marek; Stepaniuk, Maria; Górecki, Wojciech; Gawłowska-Marciniak, Aleksandra; Wolak, Przemysław; Zbyrad, Dariusz; Rybkiewicz, Marek; Chrobak, Kamila; Noparlik, Ryszard; Niedzielski, Jerzy; Janík, P.; Nowak, Jan; Miaśkiewicz, Witold; Kamiński, Andrzej; Fischer, W.O.; Dembowska-Bagińska, Bożena; Kaliciński, Piotr

doi:10.5114/aoms/135706

Cited by 9 publications

(11 citation statements)

References 15 publications

(35 reference statements)

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“…In addition, a recent meta-analysis has also observed that no relapse or tumour-related death occurred in all published cases of paediatric appendiceal NET [ 28 ]. As far as we know, the only case of relapsed appendiceal NET, published in 2021, has size as only risk factor [ 29 ].…”

Section: Surgerymentioning

confidence: 99%

International Society of Paediatric Surgical Oncology (IPSO) Surgical Practice Guidelines

Abib¹,

Chui²,

Cox³

et al. 2022

ecancer

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Most children with tumors will require one or more surgical interventions as part of the care and treatment, including making a diagnosis, obtaining adequate venous access, performing a surgical resection for solid tumors (with staging and reconstruction), performing procedures for cancer prevention and its late effects, and managing complications of treatment; all with the goal of improving survival and quality of life. It is important for surgeons to adhere to sound pediatric surgical oncology principles, as they are closely associated with improved local control and survival. Unfortunately, there is a significant disparity in survival rates in low and middle income countries, when compared to those from high income countries.The International Society of Paediatric Surgical Oncology (IPSO) is the leading organization that deals with pediatric surgical oncology worldwide. This organization allows experts in the field from around the globe to gather and address the surgical needs of children with cancer. IPSO has been invited to contribute surgical guidance as part of the World Health Organization Initiative for Childhood Cancer. One of our goals is to provide surgical guidance for different scenarios, including those experienced in High-(HICs) and Low-and Middle-Income Countries (LMICs). With this in mind, the following guidelines have been developed by authors from both HICs and LMICs. These have been further validated by experts with the aim of providing evidence-based information for surgeons who care for children with cancer.We hope that this initiative will benefit children worldwide in the best way possible.

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Section: Surgerymentioning

confidence: 99%

International Society of Paediatric Surgical Oncology (IPSO) Surgical Practice Guidelines

Abib¹,

Chui²,

Cox³

et al. 2022

ecancer

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“…3,28,36 Ileocaecal resection either as first line surgery or as completion surgery has also been applied in some patients, 11,36 mainly in paediatric populations. 37,38 Although the indication for ileocaecal resection instead of RHC in aNET remains unclear, resection of the ileocaecal artery at its origin from the superior mesenteric artery is recommended. 36 Factors to consider when deciding on one of the two types of intestinal resection, are the LN yield, short-term surgical morbidity, and long-term outcome regarding HRQoL.…”

Section: When Completion Oncological Surgery Is Recommended Is Rhc Al...mentioning

confidence: 99%

“…Ileocaecal resection either as first line surgery or as completion surgery has also been applied in some patients, 11,36 mainly in paediatric populations 37,38 . Although the indication for ileocaecal resection instead of RHC in aNET remains unclear, resection of the ileocaecal artery at its origin from the superior mesenteric artery is recommended 36 …”

Section: When Completion Oncological Surgery Is Recommended Is Rhc Al...mentioning

confidence: 99%

European Neuroendocrine Tumor Society (ENETS) 2023 guidance paper for appendiceal neuroendocrine tumours (aNET)

Kaltsas

Walter

Knigge

et al. 2023

J Neuroendocrinology

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The present guidance paper is aimed to update the previous ENETS guidelines on well differentiated appendiceal neuroendocrine tumours (NET), providing practical guidance for the diagnosis and management of appendiceal NET (aNET); poorly differentiated neoplasms are dealt in a separate guidance paper. This paper is structured on a question‐answer format in order to also address controversial issues and areas where uncertainty regarding the management and follow‐up of aNET exists. All recommendations are offered on the basis of the best available evidence, along with the authors' experiences in managing these neoplasms. Each recommendation for treatment will provide a level of evidence and grade of recommendation as per the GRADE system (adapted in Infectious Disease Society of United States Public Health Service grading system) (1).This article is protected by copyright. All rights reserved.

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“…Of the multitude of studies on pediatric appendiceal NENs, most report no observed recurrences in their patient populations [ 109 ]. One study out of Poland has documented a recurrence after surgical resection (which was subsequently surgically removed, with no further evidence of recurrence on follow-up) [ 170 ]. In contrast, other extra-appendiceal gastrointestinal NETs, although rarer than appendiceal NETs in children, have a greater risk of recurrence [ 13 ].…”

Section: Long-term Outcomesmentioning

confidence: 99%

Pediatric Neuroendocrine Neoplasms: Rare Malignancies with Incredible Variability

Castle

Levy

Chauhan

2022

Cancers

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Neuroendocrine neoplasms (NENs) encompass a variety of neuroendocrine tumors (NETs) and neuroendocrine carcinomas (NECs) which can arise anywhere in the body. While relatively rare in the pediatric population, the incidence of NENs has increased in the past few decades. These neoplasms can be devastating if not diagnosed and treated early, however, symptoms are variable and can be indolent for many years. There is a reported median of 10 years from the appearance of the first symptoms to time of diagnosis. Considering some of these neoplasms have a mortality rate as high as 90%, it is crucial healthcare providers are aware of NENs and remain vigilant. With better provider education and easily accessible resources for information about these neoplasms, awareness can be improved leading to earlier disease recognition and diagnosis. This manuscript aims to provide an overview of both the most common NENs as well as the rarer NENs with high lethality in the pediatric population. This review provides up to date evidence and recommendations, encompassing recent changes in classification and advances in treatment modalities, including recently completed and ongoing clinical trials.

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

Cited by 9 publications

References 15 publications

International Society of Paediatric Surgical Oncology (IPSO) Surgical Practice Guidelines

International Society of Paediatric Surgical Oncology (IPSO) Surgical Practice Guidelines

European Neuroendocrine Tumor Society (ENETS) 2023 guidance paper for appendiceal neuroendocrine tumours (aNET)

Pediatric Neuroendocrine Neoplasms: Rare Malignancies with Incredible Variability

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