Poster Presentation ESA-SRB 2023 in conjunction with ENSA

Metanephrine mirage: distinguishing the phaeocopies (#301)

Sylvia Ye 1 , Joanna Y Gong 1 , Debbie Gordon 1 2 , Bill Fleming 3 , Jason Tan 3 , Toby Hulf 3 , Thiep Kuany 3 , Maryam Shamassi 4 , Christopher J Yates 1 5 , Dev Kevat 1 6
  1. Department of Endocrinology and Diabetes, Western Health, Melbourne, Victoria, Australia
  2. Department of Endocrinology and Diabetes, Eastern Health, Melbourne, Victoria, Australia
  3. Department of Endocrine Surgery, Western Health, Melbourne, Victoria, Australia
  4. Department of Anatomical Pathology, Dorevitch Pathology, Western Health, Melbourne, Victoria, Australia
  5. Department of Diabetes and Endocrinology, Melbourne Health, Melbourne, Victoria, Australia
  6. Department of Endocrinology and Diabetes, Monash Health, Melbourne, Victoria, Australia

Neuroblastomas (NB) are a rare cause of adrenal malignancy in adults that may present as an incidentaloma. We report the case of an adult catecholamine-secreting adrenal NB, one of only 6 such reported cases.1

A 63-year-old male was urgently reviewed at the endocrinology outpatient clinic for a right 12cm adrenal incidentaloma, incidentally found on a CT chest performed to investigate weight loss. He was a current heavy smoker (59 pack-year history), with chronic obstructive pulmonary disease and schizophrenia.

The patient had asymptomatic hypertension (systolic blood pressure 134−163mmHg) and plasma normetanephrine and 3-methoxytyramine levels 10 times the upper limit of normal at 9272pmol/L (<900) and 1023pmol/L (<110) respectively, while metanephrine was unremarkable. Urine biochemistry was concordant. CT revealed an adrenal mass measuring 12.2cm x 9.7cm x 10.7cm with heterogeneous post-contrast enhancement with enhancing solid areas, non-enhancing necrotic and cystic areas. The adrenal mass was inseparable from the liver on imaging (Figure 1) and thus suspected to be an invasive malignant phaeochromocytoma.

The patient underwent a right adrenalectomy and segment 6/7 liver resection (Figures 2 and 3). Post-operatively, he recovered well.

Neuroblastic tumours arise from sympathetic ganglion cells, comprising of neuroblastomas (immature, malignant), ganglioneuroblastomas (intermediate) and ganglioneuromas (mature). NB may be misdiagnosed as phaeochromocytoma when they secrete catecholamines and may have similar radiological features.2

The initial differentials for this case included phaeochromocytoma and composite phaeochromocytoma. There are no established criteria to distinguish catecholamine-secreting NB from phaeochromocytoma and paraganglioma (PPGL). Histological diagnosis was critical given the management of NB differs from that of PPGL. A critical eye is required for the accurate diagnosis and management of malignant adrenal incidentalomas.

 

A)

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B)

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C)

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Figure 1. A) Non-contrast adrenal CT, B) Contrast adrenal CT, C) GaTate PET-CT and FDG-PET.

 

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Figure 2. Resected right adrenal mass.

 

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Figure 3. Histology of the resected neuroblastoma.

  1. Telecan T, Andras I, Bungardean MR, Muntean D, Militaru C, Perciuleac I, Maga A, Crisan N. Adrenal Gland Primary Neuroblastoma in An Adult Patient: A Case Report and Literature Review. Medicina 2022;59(1):33.
  2. Tateishi U, Hasegawa T, Makimoto A, Moriyama N. Adult neuroblastoma: radiologic and clinicopathologic features. J Comput Assist Tomogr 2003;27(3):321-6.