From the Guidelines
Neurologic manifestations of pancreatic neuroendocrine tumors (PNETs) can occur through several mechanisms, including paraneoplastic syndrome, hormone-related symptoms, and metastatic disease, with the most common presentations being confusion, behavioral changes, seizures, and coma due to hypoglycemia in insulinomas, and neuropsychiatric symptoms from electrolyte imbalances in gastrinomas and VIPomas 1.
Neurologic Manifestations
The neurologic manifestations of PNETs can be diverse and depend on the specific tumor type and symptoms. Some common presentations include:
- Paraneoplastic syndrome, particularly in insulinomas, which can cause neuroglycopenic symptoms like confusion, behavioral changes, seizures, and even coma due to hypoglycemia 1
- Hormone-related symptoms, such as:
- Metastatic disease, particularly to the brain or spine, which can cause focal neurologic deficits, seizures, or spinal cord compression 1
- Rare complications, such as paraneoplastic encephalitis and cerebellar degeneration 1
Diagnosis and Treatment
Diagnosis of PNETs involves histological examination, immunohistochemical staining, and grading according to the WHO 2017 and 2019 classifications 1. Treatment depends on the specific tumor type and symptoms, often involving:
- Surgical resection of the primary tumor
- Somatostatin analogs like octreotide (100-500 mcg subcutaneously 2-3 times daily or long-acting formulations)
- Management of specific hormone-related symptoms Early recognition of these neurologic manifestations is crucial for timely diagnosis and treatment of the underlying PNET 1.
From the Research
Neurologic Manifestations of Neuroendocrine Pancreatic Tumors
The neurologic manifestations of neuroendocrine pancreatic tumors are primarily related to the excessive secretion of peptide hormones. Some of the key manifestations include:
- Hormone-related symptoms such as hypoglycemia, hyperglycemia, diarrhea, flushing, and wheezing 2, 3
- Neurological symptoms are not directly mentioned in the provided studies, however, it can be inferred that the hormonal imbalances caused by the tumor can lead to various neurological symptoms
- The clinical presentation of pancreatic neuroendocrine tumors (PNETs) can vary depending on the type of hormone secreted by the tumor 3
Types of Neuroendocrine Pancreatic Tumors
There are several types of neuroendocrine pancreatic tumors, including:
- Insulinomas: secrete insulin, leading to hypoglycemia 2, 3
- Gastrinomas: secrete gastrin, leading to hypergastrinemia and peptic ulcers 2, 3
- Glucagonomas: secrete glucagon, leading to hyperglycemia and skin rash 2, 3
- Vasoactive intestinal peptideomas: secrete vasoactive intestinal peptide, leading to diarrhea and flushing 2, 3
- Somatostatinomas: secrete somatostatin, leading to diabetes, gallstones, and diarrhea 2, 3
Diagnosis and Treatment
The diagnosis of neuroendocrine pancreatic tumors typically involves imaging studies, hormone level tests, and biopsy 4, 5, 6. Treatment options include surgery, targeted therapy, and chemotherapy 4, 5, 6. The choice of treatment depends on the type and stage of the tumor, as well as the patient's overall health.