Abstract
The extra-adrenal paraganglioma is a neoplasm originating in regional structures, uncommon in paediatrics. We report on a case of a 13-year-old patient who began with severe arterial hypertension, tachycardia, dilated cardiomyopathy and elevated levels of catecholamines in the blood and urine. The presence of a retrovesical pelvic mass in contact with the right vaginal dome was determined by imaging studies. A diagnosis of malignant extra-adrenal pelvic paraganglioma with lymph node metastases was reached through biopsy and the surgical resection of subsequent local recurrences. Paragangliomas are usually located in the paravertebral zones from the base of the skull to the retroperitoneum and are benign in 90% of cases. This kind of neoplasia is uncommon in paediatrics, especially those located in the pelvis. In cases of masses of a gynaecological origin, a differential diagnosis should be considered, and a histological and immunohistochemical study is essential in certifying the diagnosis.
Highlights
Paraganglioma is a rare neuroendocrine neoplasia uncommon in the paediatric population originating in regional structures derived from neural crests
It is considered that between 10% and 20% of cases are diagnosed in the paediatric age group, with an estimated incidence of 0.011 for each 100,000 children under 18 years of age
The patient evolved for three years with local recurrences and metastases in intrapelvic lymph nodes due to the neoplasia described
Summary
Paraganglioma is a rare neuroendocrine neoplasia uncommon in the paediatric population originating in regional structures derived from neural crests. Extra-adrenal paragangliomas are usually diagnosed in the retroperitoneum, head, and neck Those located in the abdomen and pelvis are associated with the sympathetic nervous system, with catecholamine hypersecretions being the cause of the signs and symptoms during the clinical presentation, as well as the possible effects of mass on neighbouring organs [5, 6]. The patient evolved for three years with local recurrences and metastases in intrapelvic lymph nodes due to the neoplasia described These results were obtained due to the studies of positron emission tomography that revealed three pelvic hypermetabolic nodules (right side: 14.2 mm, left side: 14, 9 mm and 10.3 mm), with a hypermetabolic focus on the floor of the pelvis relative to the vagina, to the right of the midline, adjacent to the metallic suture clip (Figures 3 and 8)
Sign-in/Register to view highlights & summary Sign-in/Register to access full text options 
Free) 
Talk to us
Join us for a 30 min session where you can share your feedback and ask us any queries you have
Schedule a call
