Ligiero Braga Thais, Santos-Oliveira Ralph
Brazilian Nuclear Energy Commission, Nuclear Engineering Institute, Rio de Janeiro 21941906, Brazil.
Diseases. 2018 Jan 11;6(1):8. doi: 10.3390/diseases6010008.
Generally, pancreatic polypeptide-secreting tumor of the distal pancreas (PPoma) is classified as a rare tumor, and may occur sporadically or be associated in families or with multiple endocrine neoplasia type 1 (NEM 1). It grows slowly, reaching large dimensions at the time of diagnosis and the symptomatology is fundamentally due to the mass effect, causing either non-specific abdominal pain or symptoms suggestive of obstruction of the pancreatic or biliary duct. Therefore, when detected, they are usually malignant, with metastases mainly in the liver. The combination of serum analysis of increased levels of chromogranin A and pancreatic polypeptide and pancreastatin is very useful with a sensitivity of up to 95%. However, in addition, scintigraphicexams with somatostatin analogues should be performed to better clarify the diagnosis. Surgical resection is the treatment of choice, despite surgical difficulty and because they are generally palliative due to the metastases. Surgeries for tumor volume reduction are also performed to relieve symptoms. Chemotherapy commonly uses streptozotocin and somatostatin analogues to treat residual disease. Unfortunately, the survival rates are still very low, less than 10%, and if metastases already exist, this percentage drops to 3%.
一般来说，胰腺远端的胰多肽分泌肿瘤（PPoma）被归类为罕见肿瘤，可散发性发生，或与家族性或多发性内分泌腺瘤1型（NEM 1）相关。它生长缓慢，在诊断时体积较大，症状主要源于占位效应，可引起非特异性腹痛或提示胰管或胆管梗阻的症状。因此，在检测到时，它们通常为恶性，主要转移至肝脏。血清嗜铬粒蛋白A、胰多肽和胰腺抑制素水平升高的联合分析非常有用，敏感性高达95%。然而，此外，还应进行生长抑素类似物的闪烁扫描检查，以更好地明确诊断。手术切除是首选治疗方法，尽管手术难度较大，且由于存在转移，手术通常为姑息性手术。也会进行肿瘤减容手术以缓解症状。化疗通常使用链脲佐菌素和生长抑素类似物来治疗残留病灶。不幸的是，生存率仍然很低，不到10%，如果已经发生转移，这个百分比会降至3%。
