Glomangiosarcoma

Signs and symptoms

Glomangiosarcoma usually manifest as painful blue-red dermal nodules that range from 0.5-2cm in size.

Causes

Glomangiosarcomas can arise de novo or from a preexisting benign glomus tumour.

Diagnosis

Typically, a definitive diagnosis cannot be made based solely on radiologic findings and clinical symptoms. To get a conclusive diagnosis of glomangiosarcoma, immunohistochemical staining in addition to hematoxylin and eosin staining is needed.

Histologically, glomangiosarcomas differ from benign glomus tumors in that they include sheets of uniform, round to oval cells with eosinophilic cytoplasm, many vascular spaces, and cellular pleomorphism linked to frequent mitotic figures.

Glomangiosarcomas display many of the same antigens as their benign counterparts on immunohistochemical labeling; these antigens include vimentin, smooth muscle actin, and muscle-specific actin. On the other hand, benign tumors stain more highly for actin and myosin, and malignant tumors stain more intensely for vimentin. Studies conducted in the past have also revealed changes in the expression of Bcl-2 and p53 in comparison to benign glomus tumors.

These lesions in soft tissue and skin need to be distinguished from other cutaneous round cell malignancies such hemangiopericytoma, leiomyosarcoma, melanoma, and Merkel cell carcinoma.

Treatment

Although Mohs micrographic surgery has been previously described, the standard of care for malignant glomus tumors still involves wide local excision with negative surgical margins. It is not currently advised to use adjuvant chemotherapy for primary glomangiosarcoma.

References

References

1. (2005). "Glomangiosarcoma of the hypothenar eminence: a case report". Chirurgie de la Main.
2. (November 2003). "Glomangiosarcoma of the hand: a case report and review of the literature". The Journal of Dermatology.
3. (December 1998). "Glomangiosarcoma of the hip: report of a highly aggressive tumour with widespread distant metastases". [[British Journal of Dermatology]].
4. (September 2001). "Malignant glomus tumor: a case report and review of the literature". Dermatologic Surgery.
5. (September 1997). "Malignant glomus tumor: a case report and review of the literature". The American Journal of Surgical Pathology.
6. (1990). "Locally infiltrative glomus tumors and glomangiosarcomas. A clinical, ultrastructural, and immunohistochemical study". Cancer.
7. (2022-01-01). "A case of malignant glomus tumor (glomangiosarcoma) of the nasal cavity". Journal of Surgical Case Reports.
8. (2020). "Glomus tumors associated with the bone and joints: a review of 91 cases". AME Publishing Company.
9. (2001). "Atypical and Malignant Glomus Tumors". Ovid Technologies (Wolters Kluwer Health).
10. (2017). "Glomangiosarcoma Arising from a Prior Biopsy Site". Ovid Technologies (Wolters Kluwer Health).
11. (1988). "Glomangiosarcoma in a glomus tumor. An immunohistochemical and ultrastructural study". Cancer.
12. (1998-11-01). "Histochemical investigation into the molecular mechanisms of malignant transformation in a benign glomus tumour". BMJ.
13. (2013-01-03). "Clinically malignant atypical glomus tumour". BMJ.
14. (2009). "Malignant Glomus Tumor of the Hand". Ovid Technologies (Wolters Kluwer Health).