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Pancreatic Neuroendocrine Tumor. PanNETs can either be managed conservatively by observation or by surgery, depending upon the clinical scenario. Very small (close to 1 cm), incidentally discovered PanNETs in elderly patients with multiple comorbid conditions may be managed conservatively. PanNETs may grow very slowly for prolonged periods. Although the majority (50–60%) exhibit malignant behavior, surgical intervention may not be the best option, especially for elderly patients with multiple comorbidities. Hence, PSC has placed PanNETs in the “neoplastic: other” rather than in the “malignant” category so that patients can have more conservative management options as well. Genetic testing for germline mutations may be performed in cases where family or personal history is suggestive of MEN1 or VHL disease. Medical management of functioning PanNETs is available with multiple drugs, especially in non-resectable cases. For patients with metastatic disease, if the disease is restricted to the liver, resection (including the possibility of hepatectomy and transplantation) is warranted for both functioning and non-functioning tumors with the aim of curative resection or debulking/palliative treatment depending on the size and location of the tumor [30]. Radiofrequency ablation or embolization may be considered with or without chemotherapy for liver metastases.

Solid-Pseudopapillary Neoplasm. Surgical resection is the treatment of choice for SPN.

Mucinous Cystic Neoplasm. MCNs are mostly benign, with 18% of cases undergoing malignant transformation. Hence, due to the expense and anxiety associated with life-long surveillance, these neoplasms are removed surgically regardless of their grade. However, patients with small cysts and without high-risk imaging features can be managed with observation.

Intraductal Papillary Mucinous Neoplasms. IPMNs are treated with either surgical resection or observation depending upon the location. IPMNs that involve the main pancreatic duct are treated with resection. IPMNs that involve only branch ducts can be treated by either of the two options. The decision to operate depends upon the presence of worrisome imaging features such as a large cyst size (>3 cm) or a non-enhancing mural nodule. IPMNs with cytological presence of high-grade dysplasia and adenocarcinoma are surgically resected. Figure 1 shows the Fukuoka revised guidelines for the management of MCNs and IPMNs.

Gastrointestinal Stromal Tumor. Surgical resection is the treatment of choice for localized gastrointestinal stromal tumors (GISTs). For advanced or metastatic c-Kit-positive GISTs, tyrosine kinase inhibitor (TKI) imatinib is used. Patients who develop resistance to TKI are treated with chemotherapy.