Epithelioid hemangioendothelioma of the mandibular gingiva: A rare case of metastasis 4 years after radical excision and literature review

Abstract

Malignant epithelioid hemangioendothelioma (MEH), or high-risk epithelioid hemangioendothelioma, is a low- to intermediate-grade vascular malignancy. A few cases of MEH have been documented in the head and neck region, including the neck, thyroid gland, larynx and scalp. MEHs are extremely rare in the oral cavity. Only 31 cases of MEH in the oral cavity were described in English literature between 1975 and 2014. Further, only eleven cases were referred to MEH of the maxillary or mandibular gingiva. No gingival MEH metastases have been described in literature. We report a literature review and a case of MEH with a metastatic occurrence 4 years after surgical excision.

Keywords: Malignant hemangioendothelioma; mandibular gingiva; vascular tumor.

Figure 1

Gingival lesion at first evaluation. Note, the mandibular gingival recession bounded between right central incisor and first left premolar

Figure 2

Radiographic findings. Optical projection tomography: Radiolucency was appreciable between the left mandibular canine and first premolar teeth (arrow)

Figure 3

(a) Computed tomography scan (occlusal view) showing buccal cortical bone loss. (b) Computed tomography showing buccal cortical bone loss

Figure 4

(a) Proliferation of ovoid and fusiform epithelioid cells with nuclear and cellular pleomorphism arranged in nests and cords forming small vascular channels (H&E stain, ×100). (b) The Ki-67 index was 20% (IHC stain, ×100). The neoplastic cells were positive for (c): CD31 in both stromal and endothelial cells (IHC stain, ×100) and (d): CD34 (IHC stain, ×100)

Figure 5

(a-d) Surgical excision and reconstruction of the mandible with an iliac crest bone graft

Figure 6

Radiographic findings 4 years after bone reconstruction

Figure 7

Involvement of bone and mucosa by the malignant cells (a) H&E stain, ×40, (b) H&E stain, ×400. Cells positive for (c) Ki-67 (IHC stain, ×200) and (d) CD 31 (IHC stain, ×100). (e) lymph node metastases (H&E stain, ×40)