A case of 59-year-old male patient presenting with metastasis to the mandible from malignant pheochromocytoma is described. The conventional radiographs and CT images suggested that the lesion was malignant osteogenic tumors or metastatic tumors due to the existence of calcification and widespread periosteal sunburst spiculation. On MRI, an expansive mass was clearly depicted and the signal intensities of the lesion were low to intermediate on T1 weighted image with intermediate to high signal intensity on T2 weighted image. A strong enhancement of the lesion was also observed on contrast enhanced T1 weighted image. On maximum intensity projection image in the arterial phase, the mass showed exceedingly early enhancement and excessively dislocated adjacent vessels. The diagnosis of a pheochromcytoma was difficult on the basis of these imagings. The final diagnosis was based on a biopsy of the mandible and I-131 Meta-iodobenzylguanidine scintigraphy (MIBG) scintigraphy. A primary lesion of the right adrenal showed low uptake due to wide centric necrosis and metastatic lesions of liver, lumber vertebrae, ribs and sacroiliac joint showed high uptake on the I-131 MIBG scintigraphy. The final diagnosis was nonfunctioning malignant pheochromocytoma due to the absence of elevation of catecholamine or its metabolite.

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http://dx.doi.org/10.1016/s0720-048x(01)00366-7DOI Listing

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Article Synopsis
  • Non-neoplastic hypercortisolaemia, or pseudo-Cushing's syndrome (PCS), can occur due to various health conditions like depression, obesity, and diabetes, linked to overactivity of the hypothalamic-pituitary-adrenal axis.
  • A 66-year-old woman exhibited symptoms such as weakness, weight loss, and poorly controlled hypertension, which led to the discovery of pheochromocytoma, a tumor that causes hormone overproduction, upon further testing.
  • After undergoing surgery to remove the tumor and receiving supportive treatment, her symptoms improved significantly, confirming the diagnosis of PCS rather than traditional Cushing's syndrome.
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Pheochromocytomas and paragangliomas (PPGLs) are rare neuroendocrine tumors derived from chromaffin cells, with 80-85% originating in the adrenal medulla and 15-20% from extra-adrenal chromaffin tissues (paragangliomas). Approximately 30-40% of PPGLs have a hereditary component, making them one of the most genetically predisposed tumor types. Recent advances in genetic research have classified PPGLs into three molecular clusters: pseudohypoxia-related, kinase-signaling, and -signaling pathway variants.

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Pheochromocytoma is rare in clinical practice, with patients typically presenting with headache, high blood pressure and sweating. Patients who develop a pheochromocytoma crisis are particularly rare. This report describes the case of a patient in a pheochromocytoma crisis who presented with severe cardiogenic shock, acute respiratory failure, and acute coronary syndrome.

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Unexpected pheochromocytoma leading to cardiac arrest during the perioperative period: a case report and literature review.

BMC Anesthesiol

December 2024

Department of Anaesthesiology, The Fourth Affiliated Hospital, School of Medicine, Zhejiang University, Yiwu, Zhejiang, 322000, China.

Background: Pheochromocytomas (PCCs) are rare neuroendocrine catecholamine (CA)-secreting tumours that originate from chromaffin tissue and can produce and store CAs. Unexpected PCCs pose a serious threat to the perioperative safety of patients and a considerable challenge to anaesthesiologists because of the risks of fatal hypertensive crises and other stresses.

Case Presentation: A 37-year-old woman who was scheduled for tonsillectomy and palatopharyngoplasty under general anaesthesia experienced a malignant cardiovascular event after induction, which was characterized mainly by a sharp increase in heart rate and blood pressure, ultimately leading to cardiac arrest and the occurrence of secondary long QT syndrome.

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Background: Anal and perineum pain caused by malignant tumor invasion is often difficult to control with opioids. Continuous sacral epidural ethanol injection therapy is less likely to cause bladder and rectal disturbances, making it a suitable treatment option for patients with preserved voiding function.

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