Intravascular Papillary Endothelial Hyperplasia in Clinical Pathology

Intravascular papillary endothelial hyperplasia (IPEH), often termed Masson’s tumour, represents a benign, non-neoplastic proliferation of endothelial cells within the vascular lumen. It arises most commonly in the head, neck and extremities but may involve virtually any vascular bed. Histologically, IPEH is characterised by papillary projections of hyperplastic endothelium intimately associated with an organising thrombus, lacking significant atypia, necrosis or elevated mitotic activity. Clinically, lesions present as slow-growing, firm nodules that may mimic both benign haemangiomas and malignant vascular tumours such as angiosarcoma or Kaposi’s sarcoma, rendering histopathological examination essential for accurate diagnosis. Three clinicopathological variants are recognised: the pure form arising de novo in a vessel, the secondary form developing within pre-existing vascular lesions or aneurysms, and the rare extravascular form within haematomas. Surgical excision with complete removal of the lesion and associated thrombus remains curative in the vast majority of cases, with recurrence uncommon when margins are clear. Immunohistochemical staining for endothelial markers (for example CD31, CD34 and ERG) and a low proliferation index (Ki-67) serve to confirm vascular origin and support the benign diagnosis. Recent work has also explored growth factors and hypoxia-related pathways in IPEH development, reinforcing the concept of a reactive, thrombus-driven process rather than true neoplastic transformation.

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Intravascular Papillary Endothelial Hyperplasia in Clinical Pathology publication trend

The graph below shows the total number of articles in intravascular papillary endothelial hyperplasia in clinical pathology across all publications each year (not limited to Nature Index journals).

Technical terms

Endothelial cells: The specialised cells lining the interior surface of blood and lymphatic vessels.

Immunohistochemistry: A laboratory technique using antibodies to detect specific antigens in tissue sections, aiding in diagnosis.

Organising thrombus: A blood clot undergoing cellular invasion and vascularisation, serving as a scaffold for endothelial proliferation in IPEH.

Papillary structure: Finger-like projections of hyperplastic endothelium forming within the vascular lumen.

Angiosarcoma: A malignant vascular tumour characterised by aggressive endothelial proliferation and marked cytological atypia, which must be excluded in IPEH diagnosis.

References

1. Intravascular papillary endothelial hyperplasia: histomorphological and immunohistochemical features. Diagnostic Pathology (2013).
2. Report of intraosseous intravascular papillary endothelial hyperplasia associated with an odontogenic cyst in the maxilla and literature review. Diagnostic Pathology (2024).
3. Immunohistochemical Study of Differential Expressions of CD31, ERG, VEGF-A, α-SMA, Glut-1, CD105, D2-40 and Ki-67 in Oral Intravascular Papillary Endothelial Hyperplasia. Journal of Hard Tissue Biology (2024).
4. Case report: Multiple brain intravascular papillary endothelial hyperplasia: incidence, diagnostic challenges, and management approach. Frontiers in Neurology (2023).