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Renal PNETs present as very clinically aggressive tumors.
Observations of a high rate of microsatellite instability (MSI-H) in pNETs have led to suggestions that microsatellite instability (MSI) may have an etiologic role in a significant proportion of these tumors.
The present study was undertaken during PBTC protocol 001 ( Identifier: NCT00042367), a trial to investigate the feasibility of the addition of regional therapy with intrathecal mafosfamide to an intensive regimen of systemic chemotherapy in children younger than 3 years at diagnosis with MB, PNETs, ependymoma with metastatic disease, or other primary intracranial embryonal tumors.
This discussion illustrates that the collaboration between the oncologist and the pathologist has a critical role in appropriately classifying these primitive pediatric brain tumors to (1) identify prognostically important medulloblastoma variants, (2) separate atypical teratoid rhabdoid tumors from other small blue cell tumors, and (3) distinguish between central and peripheral PNETs when appropriate.
A total of 102 cases of medulloblastoma (n = 77)/supratentorial PNETs (n = 25) that were diagnosed during a period of 8.5 years (January 1997 to June 2005) were retrieved from the anatomic pathology files of Texas Children's Hospital (Houston, Tex).
Infrequently, PNETs have been described in other organs, such as the kidney, as primary neoplasms, or as arising from other tumors, such as ovarian or testicular germ cell neoplasms.[1-5] Those PNETs that arise in the lung parenchyma without pleural or chest wall involvement are extremely rare, and to our knowledge only 5 cases have been reported, 3 of them with follow-up.[6-8]
Gastroenteropancreatic system, being the prime location, contributes almost 70% of all NETs, 2 broadly categorised into pancreatic neuroendocrine tumours (pNET) and enteric (extra-pancreatic) neuroendocrine tumours (eNET).
Signs or symptoms of pancreatic neuroendocrine tumours (pNETs) depend mainly on the type of hormone released by the tumour.
Primitive neuroectodermal tumors (PNETs, also called extraskeletal Ewing sarcomas [ESs]) are rare malignant tumors derived from neuroectoderm, belonging to the group of small blue round cell tumor (SBRCT).[1],[2],[3] The body sites in which PNETs commonly grow include the central nervous system, soft tissues, and bones.
Pancreatic cancer treatment company The Pancreatic Cancer Action Network (PanCAN) reported on Friday the receipt of US Food & Drug Administration (FDA) approval for Lutathera for the treatment of patients with pancreatic neuroendocrine tumours (PNETs).