| Abstract|| |
Verrucous hemangioma (VH) is an uncommon, congenital, vascular malformation that involves dermis and subcutaneous connective tissue of skin. VH lesions are initially present at birth, and therefore, the diagnosis in the elderly may be difficult. Review of literature reveals that VH lesions are commonly located unilaterally on the lower extremities. VH may clinically present as keratotic, papular, nodular, or plaque-like lesions that are reddish-blue in color. VH does not resolve spontaneously and has a tendency to relapse. The diagnosis of VH is generally done on the basis of histopathology. Early diagnosis is important to get a better cosmetic result. VH requires a large, deep excision to avoid recurrence because of frequent extension into subcutaneous fat planes. Intra-oral lesions of VH have rarely been reported in the literature. We present an extremely rare case of VH occurring in the retromolar triangle area of oral cavity.
Keywords: Angiokeratoma, imaging, oral, verrucous hemangioma
|How to cite this article:|
Dighe R, Aditya A, Mhapuskar A, Jathar M. Verrucous hemangioma of the oral cavity: A rare diagnostic dilemma. Indian J Dent Res 2015;26:644-7
Verrucous hemangioma (VH) is a vascular malformation that shows reactive epidermal acanthosis, papillomatosis, and hyperkeratosis histologically. In 1937, Halter first used the term "Verrucous hemangioma." In 1967, Imperial and Helwig defined VH as "a congenital vascular malformation comprising capillary or cavernous hemangioma in the dermis and subcutaneous tissue associated with reactive epidermal acanthosis, papillomatosis, and hyperkeratosis, distinguishing it from angiokeratoma."  VH of the oral cavity is one of the rarest entities. We present a case of VH in a 13-year-old boy, who presented with an extensive lesion spreading over left buccal mucosa and vestibule, palate, and the pharyngeal region.
|How to cite this URL:|
Dighe R, Aditya A, Mhapuskar A, Jathar M. Verrucous hemangioma of the oral cavity: A rare diagnostic dilemma. Indian J Dent Res [serial online] 2015 [cited 2020 May 25];26:644-7. Available from: http://www.ijdr.in/text.asp?2015/26/6/644/176935
| Case Report|| |
A 13-year-old male patient was referred from the department of pedodontics with the chief complaint of mild, intermittent pain and discomfort in the lower left posterior region of the jaw for 3 days. He also complained of difficulty in breathing and swallowing. Patient's medical, dental, and personal histories were noncontributory. Intraoral examination revealed multiple, round, well-defined reddish papules in the left buccal and vestibular mucosa which extended medially and posteriorly until the soft palate and the pharynx. The lesions appeared coalesced in few regions, whereas segregated papules with few petechiae were present on the soft palate [Figure 1]. Coalescence of these lesions was also apparent in the upper pharyngeal region with vertical fissured appearance [Figure 2]. The pain and discomfort experienced by the patient could be attributed to secondary trauma to this extensive lesion. No such lesion was present anywhere else on the body.
|Figure 1: Intra-oral photograph showing lesion involving left buccal and vestibular mucosa which extended medially and posteriorly until the soft palate and the pharynx|
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|Figure 2: Lesions involving upper pharyngeal region with vertical fissured appearance|
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Based on the clinical examination, a provisional diagnosis of vascular malformation was made. However, probability of other pigmented lesions such as Kaposi's sarcoma and malignant melanoma could not be completely ruled out. Various investigations were advised for the patient which included hematological investigations (CBC, HIV, and HbsAg), radiographic investigations (Panoramic radiograph and contrast-enhanced CT Scan of Head and Neck), and incisional biopsy followed by histopathological investigation. Considering the probable origin of the lesion to be vascular, an incisional biopsy was carried out in the hospital set up.
Hematological investigations and panoramic radiograph did not reveal any abnormalities. Contrast-enhanced CT imaging showed large soft tissue density lesion involving left lateral wall of oropharynx, left half of uvula, and aryepiglottic fold. Lesion was seen projecting into the oropharyngeal airway causing significant narrowing of the airway. The lesion was mildly contrast-enhancing in nature [Figure 3]. The large soft tissue mass also appeared to involve left lateral wall of nasopharynx. Fat planes around the left medial pterygoid muscle appeared to be lost [Figure 4]. An incisional biopsy was taken from left buccal mucosa. Histopathological examination with hematoxylin and eosin staining revealed multiple vascular spaces lined by endothelial cells and filled with erythrocytes in the deeper layer i.e., subcutaneous plane around muscle fibers [Figure 5]. Immunohistochemistry study was done using CD34 marker which revealed proliferating endothelial cells; stained due to uptake of the marker [Figure 6].
|Figure 3: Contrast-enhanced computed tomography imaging showed large soft tissue density lesion involving left lateral wall of oropharynx, left half of uvula, and aryepiglottic fold. Lesion was seen projecting into the oropharyngeal airway causing significant narrowing of the airway|
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|Figure 4: Fat planes around the left medial pterygoid muscle appeared to be lost in the axial computed tomography image|
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|Figure 5: Multiple vascular spaces lined by endothelial cells filled with red blood cells seen in the deeper layer i.e., subcutaneous plane around muscle fibers (H and E, ×20)|
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|Figure 6: Proliferating endothelial cells stained due to uptake of CD34 marker (×20)|
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Based on the above findings, a final diagnosis of VH was made. Considering the extensive spread of the lesion and its complicated nature, prompt referral of the patient was done to the tertiary specialty hospital. Until the time of reporting of this case, patient was under medical care where an extensive management strategy was being formulated by a panel of experts.
| Discussion|| |
VH, first described by Halter in 1937, is defined as a congenital vascular malformation comprising a capillary or cavernous hemangioma in the dermis and subcutaneous tissue associated with reactive epidermal acanthosis, papillomatosis, and hyperkeratosis. 
VH has been referred in the literature with various names such as angiokeratoma circumscriptum neviforme, hemangioma unilateralis neviforme, keratotic hemangioma, unilateral VH, and nevus angiokeratoticus. 
Although VH is a clinically specific lesion with characteristic histopathological features, its precise nature remains controversial until date. The clinical features of VH that are indicative of it being a vascular malformation are its presence at birth, proportionate growth, anatomical predilection for the lower extremities, and equal gender distribution. On the other hand, some of its features such as thick vascular walls with multilamellated basement membrane, relative uniformity of channel size, and the endothelium shows focal positivity for type 1 glucose transporter (GLUT1) are characteristics of vascular tumor. , Hence, the true nature of VH still remains indistinct.
Clinically, VH presents as a macular or slightly raised lesion at birth which is pink to bluish-red in color, firm on palpation and well-demarcated with an irregular border. It can be single or multiple and most commonly located on the lower extremities. In early childhood, these lesions begin to thicken and become hyperkeratotic. Whether these reactive changes are secondary to trauma, altered hemodynamics or secondary to infection is not known.  VH clinically presents as circumscribed, warty papules arranged in linear or serpiginous configuration often surrounded by satellite lesions. The verrucous component increases with age. The lesions persists indefinitely and does not regress. 
Clinically, the differential diagnosis of VH includes Angiokeratoma circumscriptum, lymphangioma circumscriptum, verrucous epidermal nevus, verrucous carcinoma, and malignant melanoma. The most important differential diagnosis is AK.  A comparison between VH and AK is presented in [Table 1]. , Histopathologically, VH is characterized by the presence of large capillary or venule such as vessels that fill the papillary dermis and merge with smaller, more capillary-sized vessels in the subcutis.  The endothelial lining is flat and immunostains with CD34. These subcutaneous capillaries form lobules interposed with fat cells and fibrous tissue, giving a pseudoinfiltrative appearance. There are multilaminated basement membranes particularly surrounding the deeper vessels. The vessels show focal immunostaining with mindbomb homolog 1 (MIB-1) and weak positivity for GLUT1, suggesting increased cellular turnover. Staining with D2-40 is negative for VH. 
The correct diagnosis is made on the basis of histopathological features as evident in our case also.  During ultrasonography, VH presents as a homogeneous vascular mass. If it is venous, then phleboliths and compressible vessels are seen and flow demonstrates a venous signal. If arteriovenous, both arterial and venous flow is seen. If lymphatic, no flow signal is apparent. Computed tomography demonstrates a hypodense lesion with slow peripheral postcontrast enhancement. Magnetic resonance imaging (MRI) has got therapeutic and diagnostic implications in the diagnosis of the depth of the lesion. On MRI venous malformations demonstrate variable signal intensity, arteriovenous malformations demonstrates low signal intensity. Lymphatic malformation demonstrates low T1 and high T2 intensity. 
VH do not resolve spontaneously and have tendency for relapse. The horizontal extent of the lesion is greater than the clinical verrucous component. Earlier the diagnosis better will be the results esthetically. VH requires a large, deep surgical excision to rule out the chances of relapse because of the presence of deeper components. 
VH is one of the rarest entity affecting oral cavity. However, we suggest that although rare, VH should be considered in the differential diagnosis of reddish-blue, warty, papular lesions oral cavity. The most important aspect in the diagnosis of VH is deep biopsy to detect changes in the deeper components.
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Dr. Rahul Dighe
Department of Oral Medicine and Radiology, Sinhgad Dental College and Hospital, Pune, Maharashtra
Source of Support: None, Conflict of Interest: None
[Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6]