Malignant Testicular Paraganglioma: The Importance of Adequate Clinical Follow-Up

Background: Paragangliomas (PGL) are rare neuroendocrine tumors derived from neural crest cells. The presence of metastases is the only absolute criterion of malignancy. Clinical Case: A 59 y.o. male patient reports the appearance of a tumor in the right inguinal region operated in 2014 with tumor removal and right orchiectomy, with anatomopathological (AP) outcome spermatic cord adenomathoid tumor and immunohistochemistry (IHC) compatible with PGL: Ki-67 1% (S 100 diffusely positive protein), diffusely positive chromogranin A, weak positive CD99. However, no new tests were performed in the follow-up. After 5 years, the patient started to present sporadic episodes of sweating, palpitations and increased tension levels after doxazosin suspension. Investigation for reoccurrence with Urinary Catecholamines: Noradrenaline (VR< 97 mcg/24h) 112/179, Adrenaline (VR< 27 mcg/24h) 4/4, Dopamine (< 500 mcg/24h) 184/ 342, Plasma Metanephrines: Normetanephrines (VR< 196 pg/ml) 880.7/ 2402 Metanephrines (VR< 65 pg/ml) 44.6/ 53.1. Tests was performed: chest CT Multiple bilateral non-calcified pulmonary nodules with contrast enhancement, measuring 1.2 cm. Abdominal CT: Two retroperitoneal solid lesions located anteriorly to the inferior vena cava and aorta, in a discretely paramedian position on the right, intense arterial enhancement, with a hypoattenuating center. The largest lesion is located just below the emergence of the renal arteries and after the head of the pancreas and third portion of the duodenal. It measures 4.4 x 3.2 x 4.4 cm (LL x AP x CC). The smallest lesion is immediately inferior to the largest and measures 3.1 x 2.6 x 3.6 cm (LL x AP x CC). The lesions have contact with retroperitoneal vascular structures, notably with the inferior vena cava and with the vascular pedicle of the right kidney. Scintigraphy with MIBG: Radiopharmaceutical hyperconcentration in focal areas in the projections of lung fields, in greater number on the left, with intensity of uptake varying from mild to moderate; two contiguous focal areas in the median projection of mesogastrics, in moderate/acentuated degree. Patient was reoperated and removed abdominal lesions with new conclusive AP for PGL whit PASS 3, currently performing therapeutic MIBG for thoracic injury control. Clinical Lesson: Patients with lesions suggestive of PGL but without confirmatory AP should request IHC and clinical follow-up will be according to the findings in it.