*Radosław Samsel1, Helena Muszyńska2
Epidemiology, diagnosis and surgical management of neuroendocrine neoplasms of the colon and rectum
Epidemiologia, diagnostyka i leczenie chirurgiczne nowotworów neuroendokrynnych okrężnicy i odbytnicy
1Department of Surgery, Department of Surgery of Gastrointestinal Cancers and Neuroendocrine Tumors, National Institute of Oncology – National Research Institute, Warsaw
Head of Department: Professor Andrzej Rutkowski, MD, PhD
2Student Scientific Club “Hormon” Department of Endocrinology, Centre of Postgraduate Medical Education, Bielański Hospital, Medical University of Warsaw
Coordinator: Alicja Szatko, MD, PhD
Streszczenie
Nowotwory neuroendokrynne przewodu pokarmowego są coraz częściej rozpoznawalną grupą nowotworów. W jelicie grubym (okrężnicy i odbytnicy) lokalizuje się łącznie około 35% nowotworów neuroendokrynnych przewodu pokarmowego. Właściwa diagnostyka i leczenie skutkują odsetkami 5-letnich przeżyć na poziomie 60-85%. Większość tych guzów wykrywa się przypadkowo w ramach endoskopowych badań przesiewowych. Istotne znaczenie dla dalszego postępowania ma właściwa ocena makroskopowa w trakcie endoskopii, szczególnie w lokalizacji zmian w odbytnicy. Również znaczna część wykrytych zmian może być leczona endoskopowo. Ze względu na rzadkość występowania i odmienne sposoby postępowania, zaleca się, aby pacjenci z podejrzeniem lub rozpoznaniem guza neuroendokrynnego jelita grubego leczeni byli w referencyjnych ośrodkach o odpowiednim zapleczu diagnostyczno-terapeutycznym i doświadczeniu.
W niniejszej pracy autorzy przedstawiają aktualną wiedzę i zalecenia dotyczące nowotworów neuroendokrynnych okrężnicy i odbytnicy.
Summary
Gastrointestinal neuroendocrine tumors are an increasingly prevalent group of malignancies. Approximately 35% of these tumors develop in the large bowel (colon and rectum). Correct diagnosis and treatment allow for achieving 5-year survival rates of 60-85%. Most of these tumors are found incidentally during endoscopic screening. Proper macroscopic assessment during endoscopy is essential for further treatment, especially in the case of rectal lesions. A significant portion of the detected lesions can be also managed endoscopically. Due to the rarity of the disease and different treatment approaches, it is recommended that patients with either suspected or diagnosed colonic neuroendocrine tumors be treated in reference centers with appropriate diagnostic and therapeutic facilities and experience.
In this paper, we present current knowledge and recommendations for neuroendocrine tumors of the colon and rectum.
Słowa kluczowe: guzy neuroendokrynne jelita grubego, rzadkie nowotwory jelita grubego, nowotwory neuroendokrynne przewodu pokarmowego
Key words: neuroendocrine colonic tumors, rare colon and rectal tumors, neuroendocrine tomors of digestive tract
Digestive neuroendocrine neoplasms (DNEN; neuroendocrine neoplasms/tumours [NEN-NET]; gastroenteropancreatic neuroendocrine tumours [GEP-NETs]) form a heterogeneous group. They originate from diffuse neuroendocrine system (DNES) cells found throughout the body, including the enterochromaffin cells in the intestinal mucosa and Merkel cells in the skin. About 70% of these cells are found in the gastrointestinal tract.
The annual incidence of DNENs is estimated at 22-35/100,000, and it has doubled over the last 15-20 years, which is primarily due to the increased availability of diagnostic imaging and its improved quality and sensitivity as a result of technological advances. In the case of NETs of the large bowel, the increase in incidence is even three times higher, probably due to the increased number of screening endoscopies (1).
Gastrointestinal neuroendocrine tumours (GI NETs) are slightly more common in men. It is estimated that NENs account for approximately 2% of gastrointestinal tumours. Despite the increasing incidence of GI NETs, large, reliable prospective studies are lacking. Current recommendations for the management of NENs are based on retrospective analyses including case series, patient groups, and meta-analyses of the available data.
Adenocarcinomas comprise the vast majority (98%) of colon and rectal cancers (2). The remaining 2% include NENs (3).
Colonic and rectal NENs account for about 5% and 30% of all GI NETs, respectively, and are mostly asymptomatic (4). If symptomatic, they produce symptoms similar to those of other colon tumours, such as nonspecific abdominal pain, irregular bowel movements and lower GI tract bleeding. Obstructions caused by NETs are exceptionally rare. The incidence is similar in both sexes, with the average age at onset of 60 years. Colonic tumours are most often detected during screening colonoscopy or colonoscopy due to nonspecific symptoms. Prognosis is good in most patients, with 5-year survival rates of approximately 60% for colonic NETs and over 85% for rectal NETs (5).
Due to their rarity, recommendations for the management of colorectal NETs vary depending on the geographical region (6), with most data referring to the more common rectal NETs (rNETs). There is hope for more precise data if a large prospective study from Japan, which included 495 patients with colorectal NETs, is published (7). Among all locations of NETs, rNETs have the best prognosis (HR 1.87; 95% CI: 1.76-1.98) (8).
Patients with rNETs are most often referred to a surgeon after polypectomy performed during colonoscopy as part of screening or for indications other than NET symptoms.
The following therapeutic approaches are used for rNETs, depending on the stage of the disease:
1. simple polypectomy (not recommended due to difficulty in assessing the margin and frequent R1 resections),
2. different techniques of endoscopic mucosal resection (EMR),
3. endoscopic submucosal dissection (ESD),
4. endoscopic full thickness resection (eFTR),
5. different forms of transanal resections, such as transanal minimally invasive surgery (TAMIS) and transanal endoscopic microsurgery (TEM/TEMS),
6. anterior resection with mesorectal excision,
7. abdominosacral amputation of the rectum.
It is important to remember that R0 radical resection is defined as a free margin for rNETs. Therefore, any resection with no tumour cells found in the margin of the histopathological specimen is an R0 resection. As with other malignancies, for the treatment to be effective, it should be properly planned and preceded by adequate diagnosis.
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