Coincidence of intracranial myoepithelioma and adrenocortical carcinoma in a young man

Abstract

Myoepithelial tumors are rare neoplasms that develop from myoepithelial cells in glandular structures and soft tissues. Primary intracranial myoepithelial neoplasms are even rarer with around ten cases reported. On the other hand, adrenocortical carcinoma (ACC) is also uncommon with an annual incidence of 0.7–2 per million and carries a poor prognosis. It is known to have an association with certain familial cancer syndromes. Even in sporadic cases, a significant portion of them had other malignancies before and after diagnosis of ACC. We reported a 34-year-old gentleman who was diagnosed to have ACC without known familial cancer syndrome. After that, he was also found to have right occipital myoepithelioma that was confirmed by excisional biopsy. There was no known association between these two pathologies. This is the first report of coincidence of ACC and intracranial myoepithelioma.

Uncommon metastases to the brain: Frontal lobe myoepithelial carcinoma

“…[2,16] e occurrence of myoepithelial tumors within the central nervous system is also extremely rare, with only a few cases reported. [1,2,4,11] Most commonly these tumors present as benign myoepitheliomas, but malignant myoepithelial carcinomas have been known to metastasize to the lungs, bone, soft tissue, skin, liver, and brain. As reported by Ren et al, [15] the mean metastatic rate can reach 47% and mortality 29%.…”

“…[ 2 , 16 ] The occurrence of myoepithelial tumors within the central nervous system is also extremely rare, with only a few cases reported. [ 1 , 2 , 4 , 11 ]…”

“…[ 16 ] Primary intracranial myoepithelial neoplasms also have been described and are even rarer with around ten cases reported, according to literature. [ 11 ] Myoepithelial tumors can be classified as either benign myoepitheliomas or malignant myoepithelial carcinomas. The former has a better prognosis with complete resection, even though they can recur.…”