Management chirurgico di voluminoso paraganglioma dell’addome: case report e revisione della letteratura

Abstract

A 58 years old woman was admitted to our Institution with acute endocrine impairment and simulatneous intestinal necrosis resulting from the mass effect of the tumor on the mesenteric vessels. Clinical and biochemical preoperative work up posed the suspect of paraganglioma. Previous intensive care tratment, the patient, underwent surgical resection of the tumor and left hemicolectoly, with immediate remission of both endocrine and abdominal syndromes. Histological examination con firm the preoperative clinical hypothesis. Paragangliomas are rare tumors of the extra-adrenal chromaffin tissue. A paraganglioma may be discovered in the absence of any sympton. When symptomatic, the clinical findings are related to the hypersecretion of catecholamines or to the compression of several anatomical structures from a growing mass. Combined acute syndromes of hormonal impairment and contemporaneous mass effect, as in our case, are exceptional. The diagnosis of the secreting forms may be obtained on the basis of biochemical dosage of serum and urinary catecholamines and metanephrines. The assessment of malignancy for paragangliomas is not always feasible. Several imaging, cytological and hystological parameters have been proposed as predictors of malignancy, but the only element widely accepted is the existence of distant metastais. Surgery represents the tratment of choice for paragangliomas. Benign forms, catecholamine secreting forms and tunors compressing neighbouring anatomical structures can be treated surgically with success. In general, an accurante follow-up after complete resection must be considered