췌장신경내분비종양의 내과적 치료 = Current Paradigm of Treatment for Pancreatic Neuroendocrine Tumor

국문 초록 (Abstract)

췌장신경내분비종양은 랑게르한스 소도에서 기원한 종양을 의미하며, 호르몬 분비에 의한 증상 발현 유무, Ki-67 분열 지수 및 세포분화도를 기준으로 한 분류 외에도, Tumor-Node-Metastasis (TNM) 병기 분류를 통한 병의 진행 정도에 따라 다양한 예후를 보인다. 췌장신경내분비종양의 내과적 치료목표는 증상을 조절하거나, 종양의 성장을 억제하는 것이다. 내과적 치료 약제 중 하나인 소마토스타틴 유사체는 호르몬 분비와 연관된 증상 조절 목적뿐만 아니라 항암 효과를 위해서도 사용되고 있으며, Ki-67 분열 지수가 20% 미만인 WHO 분류기준 Grade 1/Grade 2 (G1/G2) 종양의 진행을 억제하는 데에 효과적인 약물로 받아들여지고 있다. 분자표적치료제 중 everolimus와 sunitinib은 소마토스타틴 유사체 치료 후 진행된 WHO G1/G2 췌장신경내분비종양 환자에서 고려될 수 있다. 항암화학요법은 주로 Ki-67 분열지수가 높거나 급속히 진행하는 종양 또는 타 장기로 전이가 동반된 경우, 다른 치료에 실패하였거나 소마토스타틴 수용체 음성인 신경내분비 암종에 우선적으로 사용이 고려되며, 주로 etoposide/cisplatin 병합 요법이 표준 치료제로 받아들여지고 있다. 하지만 Grade 3 췌장신경내분비종양(well differentiated, high grade) 환자군에 대해서는 아직까지 표준 치료에 대한 가이드라인이 확립되어 있지 않으며, 기존의 전신 항암제에 치료 반응률이 상대적으로 저조한 것으로 보고되고 있어, G1/G2 종양에서 사용되는 소마토스타틴 유사체나 분자표적치료제 등의 치료 반응률을 평가할 연구가 필요한 상황이다. 췌장신경내분비종양은 다양한 임상 경과를 보이는 만큼 다양한 치료의 선택이 필요하며, 내과적 치료뿐만 아니라 원발 부위 및 전이성 병소에 대한 근치적 절제술, 간동맥 색전술과 고주파 소작술 등의 국소 치료, 방사선 동위 원소를 이용한 방사선표적치료(PRRT) 등 모든 가능한 치료 방법을 고려하는 다학제적 접근이 중요하다고 하겠다.

다국어 초록 (Multilingual Abstract)

Pancreatic neuroendocrine tumor (PNET) refer to tumors originating from the islet of Langerhans and shows various prognosis based on the presence or absence of symptoms due to hormone secretion, the Ki-67 cell proliferation index, and the histologic grade, and according to the degree of disease progression defined by the tumor-node-metastasis (TNM) stage classification. The purpose of medical treatment for PNET is to control symptoms or inhibit tumor growth. Somatostatin analogues can be administered for the purpose of controlling symptoms caused by the secretion of specific hormones, and are accepted as effective drugs for inhibiting the progression of G1/G2 tumors based on World Health Organization (WHO) classification with a Ki- 67 cell proliferation index less than 20%. Among the molecularly targeted agents, everolimus and sunitinib can be considered in patients with WHO G1/G2 PNET showing progression after somatostatin analog therapy. Cytotoxic chemotherapy is generally administered to patients with large tumor volume and rapidly progressing metastatic NET, and etoposide/cisplatin combination therapy has been considered as a standard treatment. For the patient group of Grade 3 PNET (well differentiated) newly classified by the WHO 2017 classification, guidelines for standard treatment have not yet been established. As it has been reported, studies are needed to evaluate the treatment response rate of somatostatin analogues or molecularly targeted therapies for the patient with Grade 3 PNET. It is important to consider a multidisciplinary approach with all possible treatment options including medical treatment, radical resection of primary or metastatic lesions, liver-directed therapies, and peptide receptor radionuclide therapy for the patients with PNET.
Korean J Pancreas Biliary Tract 2021;26(1):24-32

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