Stout and Murray, 1942; Enzinger and Smith, 1976
Infantile hemangiopericytomaC49.-
HistoryThis section has been translated automatically.
DefinitionThis section has been translated automatically.
Rare, congenital vascular tumour or one acquired in the first years of life.
EtiopathogenesisThis section has been translated automatically.
The frequently detectable myofibroblastic differentiation of tumour cells suggests that infantile haemangiopericytoma, infantile myofibroma and infantile myofibromatosis are closely related entities whose aetiology has not yet been conclusively clarified. Typical for the above mentioned diseases are purely pericytic or purely myoblastoid differentiations of tumor cells including all transitional forms in one and the same tumor.
LocalizationThis section has been translated automatically.
Head and neck, shoulder region, tongue and visceral organs (mediastinum, abdomen).
Clinical featuresThis section has been translated automatically.
Mostly solitary, only very rarely multiply occurring, firm, 0.5-2 cm large or larger, occasionally painful, flat nodules or nodular tissue masses that are only detectable by palpation and are located in the deep dermis and subcutis. Tumour masses can have a red or bread-brown skin surface. Multicentric appearance must not be interpreted as a sign of metastasis. Tumour masses can tend to progress rapidly in the first months of life (as in infantile haemangiomas! They are self-limited in growth like these!
HistologyThis section has been translated automatically.
- In the deep dermis or subcutaneously localized nodular or ligamentous mostly (in contrast to the HP of the adult) biphasic tumor clusters. In addition to densely packed areas of spindly cells (myopericytoma aspect; these cells are smooth muscle actin negative) with spindle-shaped chromatin-rich nuclei (here deer antler-like cross-linked bizarre blood spaces), there are less cell-rich areas with clumsy myofibroblast-like elements enclosed in a hyalinized connective tissue matrix (myofibroma aspect). Significant rate of mitosis.
- Immunohistology: Myopericyte cells are smooth muscle actin positive.
TherapyThis section has been translated automatically.
Primarily, a spontaneous regression should be awaited (behaviour as in infantile haemangiomas). In individual cases, excision with sufficient safety distance in case of unfavourable localisation. High risk of recurrence! Subtle clinical follow-up.
LiteratureThis section has been translated automatically.
- Alpers CE et al (1984) Congenital (infantile) hemangiopericytoma of the tongue and the sublingual region. At J Clin Pathol 81: 377-382
- Borg MF et al (2003) Childhood haemangiopericytoma. Med Pediatr Oncol 40: 331-334
- Enzinger FH, Smith BH (1976) Hemangiopericytoma. An analysis of 106 cases. Hum Pathol 7: 61-82
- Ferrari A (2001) Hemangiopericytoma in pediatric ages: a report from the Italian and German Soft Tissue Sarcoma Cooperative Group. Cancer 92: 2692-2698
- 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
- Seibert JJ (1978) Multiple congenital hemangiopericytomas of the head and neck. Laryngoscope 88: 1006-1012
- Stout AP, Murray MR (1942) Hemangiopericytoma: vascular tumor featuring Zimmermann's pericytes. Ann Surg 116: 26-33