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mediastinal pericytic tumors

Sunday 18 March 2018

mediastinal perivascular tumors

Less than ten cases of glomus tumour, including a single malignant variant, have been described in the mediastinum until 2015. All cases were located in the posterior or superior compartments of the mediastinum in young or middle-aged females. The single malignant case, which resulted in this patient’s death, occurred in a 74-year-old female.

Pain and dyspnea or cough were presenting symptoms. All benign cases were successfully treated by surgery without evidence of disease at follow-up.

A single case of (infantile) myofibromatosis, which in the WHO classification is included in the spectrum of pericytic tumours, located in the central mediastinum and involving the hilar region of the lung was described in a 4-year-old male infant and was treated by pneumonectomy.

Angioleiomyoma, which is included in the pericytic group of tumours in the current WHO classification, is considered a benign proliferation of vascular smooth muscle cells and has on rare occasion been reported in the mediastinum.

The reported tumours were (with one exception) small and were either discovered incidentally or caused symptoms by compression of nerves in the posterior mediastinum. In reports with follow-up, angioleiomyoma behaved in a benign fashion.

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