Pheochromocytoma - Von Hippel-Lindau syndrome

Case contributed by Dr Bahman Rasuli

Presentation

Hypertension, palpitation and weight loss.

Patient Data

Age: 50 years
Gender: Male

There is 57*62 mm abnormal heterogeneous enhancing mass lesion involving the right adrenal gland.

There are also abnormal enhancing mass lesions are seen in both kidneys.

A renal cortical cyst is noted at the anteroinferior portion of the right kidney.

Case Discussion

  • Random Urine Normetanephrine: 2105(high)
  • MIBG Scintigraphy: I-131 avid lesion in the region of the right adrenal gland.

The patient underwent right adrenalectomy which confirmed a malignant pheochromocytoma in the right adrenal gland.

The renal enhancing masses are likely renal cell carcinoma in the setting of von Hippel Lindau syndrome. Conservative management is being undertaken of the bilateral renal masses.

Molecular genetic test for MEN IIA and RET gene was normal.

A few months later the patient presented with multiple cerebellar masses consisted with hemangioblastomas (see case rID:62416).

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Case information

rID: 61415
Published: 19th Aug 2018
Last edited: 14th Aug 2019
Inclusion in quiz mode: Included
Institution: Jame Jam Imaging Center

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