Objective: To review the imaging features of granular cell tumors of the breast (on mammography, ultrasound, and magnetic resonance imaging), establishing a pathological correlation, in order to familiarize radiologists with this entity and make them aware of the differential diagnoses, other than malignancy, of lesions with spiculated margins. Materials and Methods: We reviewed the medical records (from a clinical-pathology database and picture archiving and communication system) of five patients with a pathologically confirmed diagnosis of granular cell tumor of the breast, treated at the Portuguese Oncology Institute of Lisbon, in the city of Lisbon, Portugal, between January 2012 and December 2018. Results: All five tumors exhibited imaging features highly suggestive of malignancy (BI-RADS 5 lesions), namely spiculated margins, significant depth, and posterior acoustic shadowing (on ultrasound). One tumor showed a kinetic curve indicative of washout on magnetic resonance imaging, two were adherent to the pectoralis muscle, and one was accompanied by skin retraction. Pathology provided the definitive diagnosis in all cases. Conclusion: Granular cell tumors of the breast pose a diagnostic challenge because they can present with clinical and imaging features mimicking malignancy, and the diagnosis is therefore provided by pathology. Radiologists should be familiarized with this entity, so they can be aware of the fact that breast lesions with spiculated margins can be indicative of diagnoses other than malignancy.

We present a case report of a patient with a rare high-grade transformation of an acinic cell carcinoma (ACC) of the parotid gland, who developed Cushing’s syndrome (CS) as a result of ectopic secretion of adrenocorticotropic hormone by the tumour. The hypercortisolism was successfully treated with metyrapone, and the ACC was treated with local radiotherapy and a combined six cycles of gemcitabine and cisplatin, having achieved a partial response to the tumour. A multidisciplinary approach and combined medical treatment with radiotherapy and were essential for disease control and CS management. ACC should be considered in the differential diagnosis of ectopic CS.

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