Intraoral angiosarcoma with unusual clinical presentation: A case report

Abstract

Introduction: Angiosarcoma is a rare and highly aggressive soft tissue malignancy originating from vascular and lymphatic endothelial cells. Epithelioid angiosarcoma is the rarest subtype of angiosarcoma, characterized by the proliferation of large polygonal cells with an epithelioid feature. The occurrence of these tumors in the oral cavity is highly uncommon, and immunohistochemistry staining is essential to differentiate epithelioid angiosarcoma from mimicking lesions.

Aim: To present a case of intraoral angiosarcoma with an unusual clinical presentation and behavior and to report, to the best of our knowledge, a first primary appendix epithelioid angiosarcoma with metastasis foci in the oral cavity.

Objectives: To discuss the clinical, histological, and immunochemical features of an unusual case of intraoral angiosarcoma.

Case report: A 53-year-old Saudi female with an uncommon clinical presentation of intraoral angiosarcoma. The patient reported the lesion being painless, slowly growing, and of a six-month duration. The microscopic examination and immunohistochemical evaluation showed epithelioid angiosarcoma. The tumor cells were positive to ERG, FLI 1, and CD31 (focal) and negative to CK HMW, CD45, S100, HMB 45, D2-4, and CD 34.

Discussion: Due to the extremely rare occurrence and non-characteristic presentation of angiosarcoma in the oral cavity, many lesions maybe included in the differential diagnosis. Thus, making the diagnosis of intraoral angiosarcoma difficult.

Keywords: Angiosarcoma; Appendicitis; Epithelioid; Intraoral.

Fig. 1

Initial clinical presentation of the lesions: A) Exophytic pedunculated rubbery red to bluish nodular mass. B) Exophytic pink to reddish sessile mass on the dental papilla. C) OPG radiograph showing no destruction or invasion of the adjacent structures. (For interpretation of the references to colour in this figure legend, the reader is referred to the Web version of this article.)

Fig. 2

Hematoxylin and eosin-stained microscopic photographs of the lesion in the area of missing upper left second premolar: A) Low power revealing the tissue diffusely infiltrated by the tumor cells. B) higher magnification showing irregular vascular space with multilayering of endothelial cells, the tumor cells have abundant cytoplasm with large nuclei, prominent nucleoli and some abnormal mitotic figures.

Fig. 3

Immunohistochemical staining showing the tumor cells: positive to ERG (A), FLI-1 (B), CK 5,2 (C), CK 7 (D); focally positive to CD31(E); negative to CD34 (F), CK HMW (G), S100 (H), HMB 45 (I).

Fig. 4

Intraoral photographs two weeks post-laser excision showing: Healing of surgical site (A); Dental papilla lesion increasing in size (B); Appearance of three new lesions indicated by arrows (C and D).

Fig. 5

Hemoxylin and eosin-stained sections revealing tumor cells infiltrating the appendix: Low power (A); Higher power (B).