A 3 year-old girl was admitted in the Department of Pediatric Oncology following several months history of febrile episodes, sweating and irritability. Symptoms were first interpreted as being of infectious causes, and that is why she received repeated antibiotic therapies. There was no improvement, and that is why more investigations were performed. An abdominal ultrasound performed in a pediatric hospital revealed a right adrenal tumor mass that prompted the transfer to our unit.Family history: the patient’s mother aged 38 was diagnosed with bilateral pheochromocytoma resected when she was the age of 18 years. She presented several relapses, the most recent one at the age of 32, when she was also diagnosed with cerebral angioma. Genetic testing revealed the presence of the VHL c 244 C>G (p. Arg 82 Gly) heterozygote mutation, while the tests for RET, SDHB, SDHC and SDHD were negative.The clinical examination of our patient revealed: pallor, paroxysmal febrile episodes with irritability and sweating, tachycardia (130/min), hypertension (170/126 mmHg). There was no palpable abdominal mass.Abdominal ultrasound: well delimited tumor of the right adrenal gland, 37/34/35 mm in diameter, irregular with transonic inclusions.Magnetic resonance imaging (MRI): right adrenal tumor, 35/42/43 mm in diameter, well delimited, irregular, with marked heterogenous contrast intake, presenting cystic and necrotic areas (Figure 1).Open in a separate windowFigure 1Right adrenal tumor.Serum markers: urinary vanillylmandelic acid (VMA) 31.1 mg/24 h (normal <6.6), neuron specific enolase (NSE) 59.79 ng/ml (normal <17), ferritin 306.6 ng/ml (normal range 6–67), lactic dehydrogenase (LDH) 333 IU/L (normal range 125–300).Genetic examination: VHL c 244 C>G (p. Arg 82 Gly) heterozygotic mutation.Management: Complete surgical resection was performed with special precautions, but it was uneventful and blood pressure instantly dropped to normal.Go to:PathologyMacroscopic encapsuled 40/40/30 mm tumor, delimited by a bright yellow rim. The tumor is nodular with small hemorrhagic and necrotic areas.Microscopy: adrenal gland is compressed by a well delimited tumor made by cell nests of variable dimensions, separated by a reduced vascular connective stroma (Zellballen), with relatively numerous mitoses (8/10 High Power Field - HPF), most of them typical, very few atypical, extensive necrosis and vascular invasion. The aspect is suggestive for a malignant pheochromocytoma with Pheochromocytoma of the Adrenal gland Scaled Score (PASS) score 7: vascular invasion (1), necrosis (2), mitoses (2) (Figure 2).Open in a separate windowFigure 2Example of light microscopy image of sections from the excised tumor showing characteristics of malignant pheochromocytoma (Zellballen with central necrosis), hematoxylin eosin (HE) stain (10X).Go to:OutcomeAfter 6 months of follow-up, the patient is without any clinical symptoms, with normal blood pressure, no tumor markers, no evidence of tumor on cerebral and abdominal MRI and a normal ophthalmoscopy.