RETROPERITONIAL PARAGANGLIOMA - CASE REPORT

Pheochromocytomas / paragangliomas are tumors derived from tissues coming from the neural crest chromaffin cells and can be found anywhere in which there is an autonomic nervous system. These are rare tumors usually occurring in 2 to 8 individuals per million annually. The most common topography is in the adrenal medulla (85%), being pheochromocytomas. Paragangliomas are found at extra-adrenal sites such as the Zuckerkandl organ, the urinary bladder, the chromaffin cells of the autonomic ganglia in the neck, chest, and abdomen. This tumor may have a familial origin, usually presenting as bilateral or multifocal masses at a younger age. Both pheochromocytoma and paragangliomas may secrete catecholamines, but only the adrenaline secreted pheochromocytoma. Most of these tumors are benign in character. However, paragangliomas metastasize in about 20 to 42% of cases in relation to pheochromocytomas, which have a 2 to 10% incidence of metastases. The patient's clinic is based on the consequences of excess catecholamine release in the circulation, having the classic triad of episodic headache, sweating and palpitations, among other symptoms. The diagnosis is made by the concentration of plasma metanephrines. Imaging studies localize the tumor with high sensitivity and specificity, but are not sensitive to tumors <2 cm. To locate small masses, Imetaiodobenzylguanidine (I-MIBG) scintigraphy is used for more accurate staging. The treatment of this condition is essentially surgical and seen as the only dressing. The risk of surgery is the management of the tumor to remove it, which can trigger an exacerbated catecholamine secretion leading to hypertensive crisis, arrhythmias and acute myocardial infarction. Thus preoperative preparation for this surgery is essential. The case of the study is based on a 53-year-old white, hypertensive, asthmatic man, referred in May 2019, to the left retroperitoneal abdominal surgery outpatient clinic. Abdominal tomography showing mass with soft tissue density in the left para-aortic region measuring 4.5 × 4.3 cm. No history of cancer in the family. Submitted complete resection of the lesion. Pathology showed that it was a neoplasm with neuroendocrine characteristics; Immunohistochemistry (IHC) matched the tumor as a paraganglioma. Patient follows outpatient follow-up, with no signs of relapse. The authors have no conflict of interest.

 

Scroll to top

Die Autoren geben an, dass kein Interessenkonflikt besteht.

Publikationsverlauf

Artikel online veröffentlicht:
23. Oktober 2019