Abstract
Paragangliomas are neoplasms that arise from extra-adrenal chromaffin cells. Pancreatic paragangliomas are rare, and few are malignant. To the best of our knowledge, no cases of functional pancreatic paragangliomas have been reported in the literature to date. We present two cases of pancreatic paragangliomas with pathological confirmation. In the case 1, clinical testing and pathological analysis revealed functional and malignant characteristics of the tumor, which carried a poor prognosis. In case 2, functional paraganglioma was suspected. The clinical presentations and outcomes of these two patients are summarized, and the relevant literature is reviewed. Because of the small number of cases reported previously, few characteristics of these tumors are known. The best methods of predicting the malignant and functional potential of these tumors remain unknown. We propose careful preoperative treatment and close postoperative follow-up of paraganglioma patients because of the functional and malignant potential of these tumors.
Highlights
Paragangliomas (PGLs) are rare neuroendocrine tumors (NETs) that arise from extra-adrenal chromaffin cells of the autonomic nervous system, with an average annual incidence rate of only 2 to 8 per 1 million adults [1]
The clinical presentation and outcomes of the two cases are summarized, and we describe the functional and malignant potential of pancreatic PGLs based on our review of the literature
As the patient was suspected with functional pancreatic PGL, he received α-adrenergic receptor blocker treatment 2 weeks prior to the operation in order to avoid the excess release of catecholamine
Summary
Paragangliomas (PGLs) are rare neuroendocrine tumors (NETs) that arise from extra-adrenal chromaffin cells of the autonomic nervous system, with an average annual incidence rate of only 2 to 8 per 1 million adults [1]. The clinical manifestation revealed a functional pancreatic PGL with liver metastases. Case 1 A 50-year-old woman presented to our institution with a 3-year history of intermittent hypertension, headache, sweating and palpitation. Postoperative laboratory tests revealed the patient had a sixfold increase in 24-hour urinary norepinephrine excretion, consistent with functional pancreatic PGL. Case 2 A 63-year-old man with a 10-year history of hypertension was admitted to the hospital for treatment of a suspected functional pancreatic PGL, symptoms. The patient had a normal level of 24-hour urinary norepinephrine excretion, primary functional pancreatic PGL was suspected due to his hypertension history. Pathological analysis confirmed the diagnosis of pancreatic PGL, and the tumor was positive for CgA and Syn, observed by performing immunohistochemical analysis. The patient received no subsequent adjuvant treatment, and his blood pressure was normal 3 months after surgery
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