The terms pheochromocytoma (PHEO) and paraganglioma (PGL) refer to a broad spectrum of clinical conditions that have as their common denominator the origin from a pathological (neoplastic) proliferation of neural crest cells. Clinical presentation and evolution of the disease is extremely variable in relation to several factors, such as the secretive functional characteristics of the cells involved, the primitive site of neoplastic proliferation and the tendency to form remote metastases, the presence of a genetic predisposition that may affect the expression of particular phenotypes. PHEO/PGL is sometimes a complex diagnostic and therapeutic challenge, but the main difficulty is probably the fact that it is a rare condition that is met on very few occasions by most physicians and therefore may remain unrecognized. A good knowledge of the polymorphic expressions of this disease should result in clinical suspicion, which can be confirmed or excluded reliably in most cases. Similarly, the therapeutic management of PHEO/PGL is well established in the overwhelming majority of cases, provided it is entrusted to experienced medical staff.
Pheochromocytoma/paraganglioma: Diagnosis and treatment
Castellano M.;Panarotto A.;Cappelli C.
2018-01-01
Abstract
The terms pheochromocytoma (PHEO) and paraganglioma (PGL) refer to a broad spectrum of clinical conditions that have as their common denominator the origin from a pathological (neoplastic) proliferation of neural crest cells. Clinical presentation and evolution of the disease is extremely variable in relation to several factors, such as the secretive functional characteristics of the cells involved, the primitive site of neoplastic proliferation and the tendency to form remote metastases, the presence of a genetic predisposition that may affect the expression of particular phenotypes. PHEO/PGL is sometimes a complex diagnostic and therapeutic challenge, but the main difficulty is probably the fact that it is a rare condition that is met on very few occasions by most physicians and therefore may remain unrecognized. A good knowledge of the polymorphic expressions of this disease should result in clinical suspicion, which can be confirmed or excluded reliably in most cases. Similarly, the therapeutic management of PHEO/PGL is well established in the overwhelming majority of cases, provided it is entrusted to experienced medical staff.I documenti in IRIS sono protetti da copyright e tutti i diritti sono riservati, salvo diversa indicazione.
