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Rare Association Between Neurofibromatosis Type 1 and Adrenocortical Carcinoma | Synapse

March 10, 2026Open Access

Rare Association Between Neurofibromatosis Type 1 and Adrenocortical Carcinoma

Key Points

To investigate the rare occurrence of adrenocortical carcinoma in individuals with neurofibromatosis type 1.
Review of cases involving neurofibromatosis type 1 and adrenal incidentalomas
Assessment of functional evaluation techniques for adrenal tumors
Identified adrenocortical carcinoma in select cases of neurofibromatosis type 1
Emphasized the significance of thorough evaluations to differentiate from pheochromocytoma

Abstract

ABSTRACT Although rare, adrenocortical carcinoma (ACC) should be considered in individuals with neurofibromatosis type 1 (NF1) presenting with adrenal incidentalomas. Despite pheochromocytoma being more prevalent in NF1, appropriate functional evaluation is important in this context to rule out adrenocortical carcinoma given its aggressive nature and poor prognosis.

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Cite This Study

Pluim et al. (Sun,) studied this question.

synapsesocial.com/papers/69af951a70916d39fea4c4cf https://doi.org/https://doi.org/10.1002/ccr3.72224