Head and Neck Pathol (2009) 3:54–58 muscular hemangioma’, which is once again a subjective diagnosis [7]. Arteriovenous malformation, a term well founded in the radiologic literature, implies functional arteriovenous communications and may correspond in some instances to what pathologists term angiomatosis [2]. Because of nomenclature issues, it has been very difﬁcult to gain a clear understanding of this lesion and in particular to compare the experience derived from one speciality to another [2]. To promote a more standardized approach to diagnosis, Rao and Weiss [2] have suggested that the term ‘angio- matosis’ be used to connote a histologically benign vascular lesion that extensively involves a region of the body or several different tissue types in a contiguous fashion. Angiomatosis can either be congenital or acquired. The congenital form may be seen sporadically or accompanying certain types of syndromes such as Klippel–Trenaunay– Weber syndrome, Sneddon’s syndrome, or Gorham disease [8]. The acquired form of angiomatosis may be infectious or iatrogenic. Bartonellosis and HIV are associated with angiomatosis. Iatrogenic cases occur after AV ﬁstula for- mation or trauma [8]. Rao and Weiss’s study [2] bears similarities to our reports in that the lesions commonly occurs in the second decade of life, there is a female predilection to its occur- rence and it presents as a diffuse persistent swelling having two histological patterns. These lesions probably begin during early intra-uterine life, grow proportionately with the fetus and consequently affect large areas of the body [2, 4]. Histopathologically, two types of patterns are seen. The most common pattern consists of a haphazard proliferation of varying sized vessels, particularly large veins. However, the most distinctive feature is the clusters of capillary vessels residing within or adjacent to the vein walls. A second but uncommon pattern is the clusters of capillary- sized vessels arranged in nodules around a large vessel in the center. Both types are typically associated with large amounts of adipose tissue and hence these lesions have lead previous authors to use the term ‘inﬁltrating angioli- poma’, suggesting that these are more generalized mesenchymal proliferations rather than exclusive vascular pathology [8]. Similar histopathologic features are seen in the present case reports. Because of its inﬁltrative nature, angiomatosis may resemble many of the vascular tumors and malformations. Histopathological differential diagnosis includes angioli- poma, angiomyolipoma, inﬁltrating lipoma, angiomyxo- lipoma and liposarcoma [6]. Angiolipoma can be considered as an important differ- ential diagnosis, but it is well circumscribed and proliferating vessels are usually concentrated at the 57 periphery of intratumoral lobules of adipocytes [6]. Angi- omyolipoma can be distinguished from angiomatosis as they are composed of varying amounts of blood vessels, smooth muscle and fat [9]. Inﬁltrating lipoma consist of lesional fat tissue inﬁltrating in the form of long thin streaks radiating from the intratumoral mass [6]. Angi- omyxolipoma usually resembles angiomatosis due to presence of adipose tissue component and blood vessels with thick and thin walls. It can be distinguished by the presence of myxoid change in adipose tissue and absence of haphazard blood vessel proliferation [10]. Liposarcoma can also be considered as a differential diagnosis; however, mitotic ﬁgures, cellular atypia and lipoblasts (signet ring appearance) are not seen in angiomatosis. The treatment of choice in extensive angiomatosis is either radiotherapy or Interferon a-2a treatment [11]. In localized cases, complete resection is preferred but there is a risk of local recurrence. Angiomatosis has no tendency to progress or evolve histologically to a more aggressive lesion. On follow up, nearly 90% of patients experienced local recurrence and approximately 40% encountered more than one local recurrence within a period of 5 years [2]. Conclusion Angiomatosis is rare in the head and neck region. Its diffuse inﬁltrating nature may give a false malignant picture. His- topathological diagnosis of angiomatosis is important due to its high recurrence rate. The possibility of angiomatosis should be considered whenever a persistent swelling in the head and neck region in early childhood is seen. Precise diagnosis is vital to achieve a curative resection. References 1. Howat AJ, Campbell DE. Angiomatosis: a vascular malformation of infancy and childhood—report of 17 cases. Pathology. 1987;19(4):377–82. doi:10.3109/00313028709103887. 2. Rao VK, Weiss SW. Angiomatosis of soft tissue—an analysis of the histologic features and clinical outcome in 51 cases. Am J Surg Pathol. 1992;16(8):764–71. doi:10.1097/00000478-19920 8000-00004. 3. Radin DR. Angiomatosis of the abdominal wall: imaging ﬁndings in three adults. Radiology. 1994;193:543–5. 4. Weiss SW, Goldblum JR, editors. Enzinger and Weiss. Soft tissue tumors. 5th ed. Philadelphia: Mosby Inc; 2008. 5. Regezi JA, Sciubba JJ, Jordan RC, editors. Oral pathology. 5th ed. Philadelphia: Saunders Company; 2008. 6. Bouquot JE, Nikai H. Lesions of the oral cavity. In: Gnepp DR, editor. Diagnostic surgical pathology of the head and neck. 1st ed. Philadelphia: WB Saunders Company; 2001. 7. Sunil TM. Intramuscular hemangioma complicated by a Volk- mann’s like contracture of the forearm muscles. Indian Pediatr. 2004;41:270–3.