Current Treatments of Neuroendocrine Tumors Role of Biotherapy and Chemotherapy

Abstract

Neuroendocrine tumors are rare neoplasms originating from cells belonging to a diffuse or confined neuroendocrine system and characterized by a significant histopatologic and biologic heterogeneity. Timely diagnosis is delayed because they are often clinically silent for their low differentiation grade and the absence of any symptom due to abnormal hormone release. For these reasons, many neuroendocrine tumor patients are not treated medically for metastatic or inoperable disease. Medical treatments include biotherapy, with interferon-α and somatostatin analogues, and chemotherapy. Somastostatin analogues are widely used in patients with symptoms and with carcinoids of low differentiation grade. Interferon-α is used alone or in combination with somatostatin analogues. Chemotherapy is active in patients with poorly differentiated neuroendocrine tumors. The therapeutic regimen commonly used is the combination of cisplatinum and etoposide. In conclusion, no standard treatment for NET has yet been identified, and the response criteria suggested by ITMO remain a reference point. The clinical aspect of the disease and biologic features suggest the identification of neuroendocrine tumors patients suitable for the appropriate therapies. On these bases, it is recommended that diagnosis and treatment of neuroendocrine tumors be carried out at specialized oncological centers involved in clinical trials.

X-linked Hyper-IgM Syndrome Associated with Poorly Differentiated Neuroendocrine Tumor Presenting as Obstructive Jaundice Secondary to Extensive Adenopathy

“…These fast-growing neuroendocrine tumors are shown to respond well to chemotherapy [25][26][27][28][29][30][31][32]. Combination chemotherapy with etoposide and cisplatin is the preferred regimen [25,26,28,32].…”

Octreotide-LAR + adrenalectomia bilateral no manejo de tumores carcinóides produtores de ACTH

“…Nossos dados demonstram que a associação destas estratégias, em conjunto com terapias anti-neoplásicas, pode contribuir para estabilização e/ou controle definitivo dos TuCA-ACTH. grupo raro e heterogêneo de neoplasias originadas de células pertencentes ao sistema neuroendócrino (1). A incidência destas neoplasias aumentou nos últimos 20 anos, provavelmente devido à modernização dos métodos diagnósticos, variando de 1,2 a 4,4 casos por 100.000 indivíduos a cada ano, representando aproximadamente 1% de todos os tumores (1,2).…”

“…grupo raro e heterogêneo de neoplasias originadas de células pertencentes ao sistema neuroendócrino (1). A incidência destas neoplasias aumentou nos últimos 20 anos, provavelmente devido à modernização dos métodos diagnósticos, variando de 1,2 a 4,4 casos por 100.000 indivíduos a cada ano, representando aproximadamente 1% de todos os tumores (1,2). A síndrome carcinóide (SCA), por sua vez, está associada à produção de serotonina pelos tumores neuroendócrinos que são denominados tumores carcinóides (TuCA), sendo agressivos e localizados predominantemente no tubo digestivo, pâncreas ou pulmões.…”

“…Mesmo recentemente, empregando-se procedimentos como a embolização hepática (5-8), ressecção cirúrgica de metástases hepáticas (9), transplante hepático (10,11), quimioterapia (1,12,13), imunoterapia (1,14), radioiodoterapia com 131 I-MIBG (15) e aná-logos da somatostatina (12,14) não tem sido descrita a importância da associação destes recursos terapêuti-cos com a adrenalectomia bilateral.…”

Sunitinib and Everolimus in Pancreatic Neuroendocrine Tumors

“…The low incidence of these tumors along with their histological and biological heterogeneity have in the past prevented clinicians from conducting prospective clinical studies aiming to define an appropriate therapeutic approach. Therefore, biotherapy with somatostatin analogues and chemotherapy combinations including platinum or streptozocin have been considered the standard treatments so far, capable of slowing tumor growth in unresectable disease 3,4 .…”