Treatment Options for Pancreatic Neuroendocrine Tumors

Abstract

The management of pancreatic neuroendocrine tumors (PanNETs) involves classification into non-functional or functional PanNET, and as localized or metastatic PanNET. In addition, while most PanNETs are sporadic, these endocrine neoplasms can also be manifestations of genetic syndromes. All these factors may assist in forming a risk stratification system permitting a tailored management approach. Most PanNETs are classified as non-functional because they are not associated with clinical sequelae of hormone excess. They are characterized by non-specific symptoms, such as abdominal pain or weight loss, resulting from mass effect related to the pancreatic tumor or secondary to distant metastases. Accurate staging of the disease is essential for determining the appropriate approach to therapy. As cure is only potentially possible with surgical resection of the tumor, it is recommended to remove all localized and limited metastatic disease. However, many patients present with metastatic and/or advanced local disease. In such instances, the goal of therapy is to control tumor growth and/or decrease tumor burden, lengthen survival, and palliate local symptoms and those of hormone excess. This typically requires a multimodal approach, including surgery, liver-directed treatment, and systemic medical therapy.

Prognosis analysis of patients with pancreatic neuroendocrine tumors after surgical resection and the application of enucleation

“…pNET patients with G3, distant metastasis, larger tumor diameter, AJCC 8th III-IV stage, the white race, or age > 60 years old were associated with a worse OS/CSS. A higher pathological grade and lower differentiation of tumor cells indicate the tumor may be more malignant [ 13 , 14 ], some pNET patients with distant metastasis are difficult to treat since the removal of the primary and metastatic tumors may be impossible, and most of them can only achieve R1 or R2 resection [ 8 ]. Their prognosis is poorer than patients with an R0 resection.…”

“…At present, many studies have researched the prognostic factors of pNETs all around the world. However, there are still many limitations and controversies about the prognostic factors of this disease [6][7][8]. There are few large databases exclusively researching the prognostic factors of pNET patients nonradical surgical resection.…”

Operationsindikationen bei neuroendokrinen Neoplasien des Pankreas

“…Im Falle eines MEN1-assoziierten Gastrinoms spielt die Tumorgröße eine entscheidende Rolle [10]. Da die Symptome sehr gut medikamentös mit Protonenpumpenblockern und Antazida therapiert werden können, wird die Operationsindikation bei Tumoren < 2 cm kontrovers diskutiert [10,53,56]. Dennoch stellt die Resektion des Tumors mit den zugehörigen Lymphknoten die einzige Möglichkeit auf Heilung dar.…”

VIPoma: Mechanisms, clinical presentation, diagnosis and treatment (Review)

“…Cytotoxic chemotherapy includes agents, such as 5-fluorouracil (5-FU), oxaliplatin, capecitabine, temozolomide and streptozocin. Often, a combination of these will be favoured: Temozolomide with capecitabine, 5-FU/doxorubicin/streptozocin (FAS), or streptozocin with doxorubicin or 5-FU (60). Systemic chemotherapy with a streptozotocin and 5-FU mixture is a standard procedure for patients with bulky extensive growths together with extrahepatic metastases (4).…”