Objective To summary the pathological classification， clinical manifestations， diagnosis， and treatments of gastrointestinal neuroendocrine tumors （GE-NETs）. Methods Domestic and international literatures were collected to summary the status of clinical researches and treatments of GE-NETs. Results GE-NETs derived from enterochromaffin cells throughout the gut which had the function of amine precursor uptake decarboxylase （APUD）. These tumors secreted discrete bioactive substances and produced characteristic immunohistochemical patterns， making patients to manifest endocrine syndrome. But there were no unified standards on the diagnosis， grade， TNM classification， and prognosis of GE-NETs. Early diagnosis rate of GE-NETs was low， and most of tumors were asymptomatic and detected at late stage， with a tendency to metastasize to the liver. Imaging examation was important in early diagnosis of GE-NETs. Surgery was the traditional first-line therapy and the only possible curative approach. Somatostatin analogues， such as long-term-release （LAR） octreotide， could relieve the hormonal symptoms， slow down the tumor growth， and had shown synergistic antiproliferative activity in combination with biological agent everolimus. Conclusions The lack of standardized classification and an incomplete understanding of this disease are some of the impediments to the progress of treatment. Individualized comprehensive therapy is the aim of the future treatment.
Carney complex (CNC) is a rare autosomal dominant syndrome, characterized by pigmented lesions of the skin and mucosa, cardiac, cutaneous and other myxomas and multiple endocrine tumors. The disease is caused by inactivating mutations or large deletions of the PRKAR1A gene located at 17q22–24 coding for the regulatory subunit type Ⅰ alpha of protein kinase A (PKA) gene. Most recently, components of the complex have been associated with defects of other PKA subunits, such as the catalytic subunits PRKACA (adrenal hyperplasia) and PRKACB (pigmented spots, myxomas, pituitary adenomas). We reviewed CNC’s clinical features, diagnosis, treatment and molecular etiology.
Thymic neuroendocrine tumors (TNETs) are a series of rare diseases with aggressive biology and poor prognosis. Clinical manifestations of TNETs are atypical, and ectopic secretion of adrenocorticotropic hormone can be found in some cases, resulting in associated endocrine symptoms. Due to the low morbidity and strong heterogeneity, it’s difficult to diagnose, treat and obtain new treatment regimen. Early complete surgical resection is an effective treatment. For advanced cancer, clinical trials of new drugs are expected to improve the survival of patients.