Epithelioid Hemangioendothelioma of the Maxillary Sinus

Abstract

The clinical course and pathologic features of a 72 year old female who presented with epistaxis are presented. Radiographic findings were notable for a large, soft tissue lesion filling the maxillary sinus with significant bony erosion and expansion. The patient was ultimately diagnosed with epithelioid hemangioendothelioma (EHE) and underwent endoscopic resection. She has no evidence of local, regional or distant recurrence 14 months postsurgery. The rarity of this neoplasm, the unusual anatomic location and non-specific symptoms present diagnostic and management challenges. Epithelioid vascular tumors encompass a spectrum of benign and malignant tumors. EHE itself is thought to have an intermediate malignant behavior pattern, though cases with indolent behavior have been reported. Differentiation of EHE from other lesions has historically based on histopathology. Additionally, recent studies have described a recurrent genetic fusion WWTR1-CAMTA1 in EHE, involving t(1;3) (p36;q25). This represents the second reported case of EHE arising in a paranasal sinus. The histopathologic findings of this lesion are reviewed.

Intraosseous epithelioid haemangioendothelioma of the mandible

“…For example, these tumors are typically positive for factor VIII-related antigen, CD31, CD34, FLI-1, vimentin, and UEA-1, but are typically negative for SMA, S100, CD68, EMA, MNF116, and CK7. [16] It may also be useful to perform fluorescence in situ hybridization or reverse-transcription polymerase chain reaction analysis to detect the presence of the WWTR1-CAMTA1 fusion gene. [5] Testing for CD34 expression alone is insufficient, as this marker is expressed by >90% of vascular tumors; therefore, staining for both CD31 and FLI-1 has been recommended for diagnosing EHE.…”

Extensive epithelioid hemangioendothelioma of the maxillary sinus: A case report

“…Even rarer, however, is an EHE primarily originating within a paranasal sinus. To our best knowledge, only two cases of EHE originating in the maxillary sinus has been previously described in the literature [7,8].…”

“…Immunohistochemically, these tumors are positive for cytokeratin (especially CK18), and vascular markers including CD31. These tumors have an intermediate and variable malignancy pattern in terms of both local recurrences and metastatic potential [8].…”

“…Given the limited clinical data, evidence based treatment protocols for treatment for sinonasal EHE are not possible. Therefore it requires a close clinical follow-up because of its potential to recur and metastasize [8].…”