Hemangiopericytoma C49.-

Stout and Murray, 1942

Facultative malignant tumor originating from the pericytes. The entity of this tumor is increasingly disputed, especially the etiopathogenetic concept of pericyte-derived tumor. Many tumors diagnosed in the past as hemangiopericytomas are now classified as myopericytomas or cell-rich fibrous histiocytomas.

Hemangiopericytomas are extraordinarily rare and of dermatological relevance. Infantile hemangiopericytoma is distinguished as a separate entity.

• Nodular or band-shaped convolutes of densely packed, spindle-shaped cells with spindle-shaped, chromatin-rich nuclei located in the deep dermis. Included are deer antler-like networked, bizarre blood vessels. Significant rate of mitosis.
• Immunohistology: Tumor cells are smooth muscle actin negative. Only in case of biphasic differentiation (pericyte and myofibroblastoid) smooth muscle actin positive cells (myofibroblastoid differentiation) are found.

• Clinical: all deep, newly formed tissue masses that can be diagnosed by palpation or imaging techniques.
• Histological: malignant fibrous histiocytoma, infantile myofibromas.

In the latest WHO classification, the term "hemangiopericytoma" is no longer used and has been replaced by the new terminology "extrapleural solitary fibrous tumour" (Kutzner et al.).

1. Abdel-Fattah HM et al (1990) Hemangiopericytoma of the maxillary sinus and skull base. Head neck 12: 77-83
2. Alpers CE et al (1984) Congenital (infantile) hemangiopericytoma of the tongue and the sublingual region. At J Clin Pathol 81: 377-382
3. Larbre B et al (1993) Cutaneus mesenchymal tumour with haemangiopericytoma-like features. Acta Derm Venereol 73: 294-295
4. Kutzner H et al (2016) vascular tumors of the skin. In: Cerroni L et al. histopathology of the skin. Springer-Verlag Berlin Heidelberg New-York S. 877
5. Mentzel T et al (1994) Infantile hemangiopericytoma versus infantile myofibromatosis. Study of series suggesting a continous spectrum of infantile myofibroblastic lesions. Am J Surg Pathol 18: 922-930
6. Seibert JJ (1978) Multiple congenital hemangiopericytomas of the head and neck. Laryngoscope 88: 1006-1012
7. Stout AP, Murray MR (1942) Hemangiopericytoma: vascular tumor featuring Zimmermann's pericytes. Ann Surg 116: 26-33
8. Thompson LD et al (2003) Sinonasal-type hemangiopericytoma: a clinicopathologic and immunophenotypic analysis of 104 cases showing perivascular myoid differentiation. Am J Surg Pathol 27: 737-749
9. Veltrini VC et al (2003) Solitary fibrous tumor of the oral mucosa--morphological and immunohistochemical profile in the differential diagnosis with hemangiopericytoma. Oral Oncol 39: 420-426
10. Yoshida M et al (2003) Primary hemangiopericytoma of the trachea. Ann Thorac Surg 76: 944-946