The role of differential diagnosis in intravascular papillary endothelial hyperplasia of the sinonasal cavity mimicking angiosarcoma: A case report

Abstract

Intravascular papillary endothelial hyperplasia (IPEH), also known as Masson's tumour, is an unusual vascular lesion characterized by the proliferation of endothelial cells. The principal significance of IPEH is its clinical and radiological resemblance to angiosarcoma, resulting in a potential misdiagnosis and overtreatment. The aim of the present study is to report a rare case of IPEH of the right maxillary sinus, focusing on the radiological and histopathological features of the neoplasm and their role in the differential diagnosis with angiosarcoma. Herein we present a case of a 67-year-old woman with history of right-sided nasal obstruction. A computed tomography scan indicated opacity in the right maxillary sinus and significant bony erosion, and an magnetic resonance imaging revealed a highly vascularized polylobed neoplasm, suggesting a malignant neoplasm. The mass was removed surgically through a combined open and endoscopic approach. A perioperative biopsy excluded malignancy and histological examination was consistent with IPEH. IPEH in the sinonasal cavity is an extremely rare condition in which differential diagnosis serves a central role due to its close clinical and radiological resemblance to angiosarcoma. Awareness of IPEH is recommended for a correct diagnosis, and in order to avoid unnecessarily aggressive treatment.

Keywords: Masson's tumour; angiosarcoma; differential diagnosis; intravascular papillary endothelial hyperplasia; sinonasal cavity.

Figure 1.

Computed tomography (CT) scan without iodate contrast, in (A) axial and (B) coronal plane, showing a complete opacity within the right maxillary sinus and significant evidence of bony erosion especially in the medial wall (red arrows).

Figure 2.

Contrast-enhanced MRI. T2 weighted images in (A) axial and (B) coronal plane showed a large mass measuring 47×52×47 mm. (C) T1 weighted scan showed a hypointense, polylobate neoplasm with high-intensity spots, compatible with hemosiderin deposits (red arrow). The mass caused deformation and thinning of the sinus walls with bony erosion and significant signs of discontinuity. The medial wall was no longer recognizable, with erosion of the middle turbinate and the root of the lower turbinate. MRI, magnetic resonance imaging.

Figure 3.

Histological examination of the specimen (hematoxylin and eosin staining; at ×100 magnification) revealed a dishomogeneous thrombotic soft tissue characterized by an arborizing network of small blood vessels anastomosed among the tissue, with deposition of matrix and collagen, and the presence of organized thrombi within dilated vessels. No atypia or mitotic activity were shown in the epithelium.

Figure 4.

Intraoperative image of maxillary sinus after removing the mass. Posterior wall sinus mucosa is quite normal and no alteration can be seen.