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Case Reports
. 2023 Jun 30;6(5):278-281.
doi: 10.1002/iju5.12603. eCollection 2023 Sep.

Composite pheochromocytoma associated with neurofibromatosis type 1

Akira Tachibana  1 , Kota Iida  1 , Yoshitaka Itami  1 , Masaya Hashimura  1 , Yukinari Hosokawa  1 , Kiyohide Fujimoto  2
Affiliations
Case Reports

Composite pheochromocytoma associated with neurofibromatosis type 1

Akira Tachibana et al. IJU Case Rep. .

Abstract

Introduction: Composite pheochromocytoma is a rare tumor, occurring in only 3% of pheochromocytomas. We report a case of composite pheochromocytoma with neurofibromatosis type 1.

Case presentation: A 42-year-old man was referred to our department for further evaluation of an incidentally detected right adrenal tumor. He was a patient at another hospital for neurofibromatosis type 1. The serum and urinary catecholamine levels exceeded the normal range. Abdominal computed tomography and magnetic resonance imaging showed a 2.8 cm diameter right adrenal tumor, and 123I-metaiodobenzyguanidine scintigraphy showed radioisotope uptake. He was diagnosed with pheochromocytoma and underwent a right laparoscopic adrenalectomy. Histopathological examination revealed that the tumor consisted of a pheochromocytoma and ganglioneuroma. The final diagnosis was composite pheochromocytoma-ganglioneuroma. Five years after surgery, no recurrence was observed.

Conclusion: Preoperative diagnosis of composite pheochromocytoma-ganglioneuroma is difficult; therefore, histopathological examination is necessary for a definitive diagnosis. Pheochromocytoma management requires lifelong follow-up.

Keywords: adrenal tumor; composite pheochromocytoma; ganglioneuroma; neurofibromatosis type 1.

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Conflict of interest statement

The authors declare no conflict of interest.

Figures

Fig. 1
Fig. 1
(a) CT shows a right adrenal tumor that is 2.8 cm in diameter. (b) MRI shows a hypointense mass on T1 weighted images in the right adrenal gland. (c) Diffusion‐weighted images show a heterogeneous tumor in the same region. (d) 123I‐MIBG scintigraphy confirming a localized increased uptake in the same region.
Fig. 2
Fig. 2
(a, b) Two different components are observed in the tumor (H&E stain): pheochromocytoma (white arrow) and ganglioneuroma (black arrow). (c) Pheochromocytoma component of the composite tumor (H&E). (d) Ganglioneuroma component consisting of ganglion and Schwann cells (H&E stain).
Fig. 3
Fig. 3
(a) The chromaffin cells in pheochromocytoma are strongly immunoreactive for chromogranin A. (b) The ganglion cells are negative or weakly immunoreactive for chromogranin A. (c) The chromaffin cells are negative immunoreactive for S100. (d) Schwann cells are immunoreactive for S100.
See this image and copyright information in PMC

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