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Extremely rare isolated and acquired oral presentation – Verrucous hemangioma
G Model JOMSMP-485; No. of Pages 4
ARTICLE IN PRESS Journal of Oral and Maxillofacial Surgery, Medicine, and Pathology xxx (2016) xxx–xxx
Contents lists available at ScienceDirect
Journal of Oral and Maxillofacial Surgery, Medicine, and Pathology journal homepage: www.elsevier.com/locate/jomsmp
Case Report
Extremely rare isolated and acquired oral presentation – Verrucous hemangioma Tejraj P. Kale ∗,1 , S.M. Kotrashetti 2 , Arjun Singh ∗ Department of Oral and Maxillofacial Surgery, V.K. Institute of Dental Sciences, KLE University, Belgaum, Karnataka, India
a r t i c l e
i n f o
Article history: Received 2 July 2015 Received in revised form 5 January 2016 Accepted 15 February 2016 Available online xxx Keywords: Oral verrucous hemangioma Rare presentation hemangioma Isolated and acquired hemangioma
a b s t r a c t Imperial and Helwig originally described verrucous hemangioma as a vascular malformation that is present at birth, anatomic predilection for lower limbs, proportionate growth and equal sex distribution. Mucosal involvement, including the oral cavity, is seen occasionally as integration of a systemic variety or with multiple cutaneous lesions in other locations. Isolated oral involvement is extremely rare while acquired involvement of tongue without cutaneous or metabolic disease has not been reported in the literature. We present a case of verrucous hemangioma involving the lateral surface of the tongue in a 60-year-old male. Due to the unusual presentation we need to reconsider our current knowledge on the etio-pathogenesis of verrucous hemangioma and its clinical inclusion in the differential diagnosis of various verrucous lesions. © 2016 Asian AOMS, ASOMP, JSOP, JSOMS, JSOM, and JAMI. Published by Elsevier Ltd. All rights reserved.夽
1. Introduction Vascular lesions and malformations, comprising of a broad category of lesions often referred to as vascular anomalies, are a heterogeneous group of clinicopathologically distinct entities. Clinicians traditionally have grouped these lesions under the generic term, hemangioma, sometimes classified as cavernous (large vessels) or capillary (small vessels) [1]. Most are congenital or occur at a young age and may show limited growth potential. Some lesions undergo spontaneous regression, which has led some to believe it to be a hamartoma rather than a tumour, while others require active intervention [2]. Imperial and Helwig [1] first introduced the verrucous hemangioma, usually found as a congenital solitary or multiple cutaneous lesions of lower limbs, and distinguished it from the angiokeratoma. Although it is a clinically specific lesion with characteristic histological features, its precise nature remains
Abbreviations: RBC, red blood cells; CD, cluster of differentiation; GLUT, glucose transporter; MAP3K3, mitogen-activated protein kinase kinase kinase 3. 夽 Asian AOMS: Asian Association of Oral and Maxillofacial Surgeons; ASOMP: Asian Society of Oral and Maxillofacial Pathology; JSOP: Japanese Society of Oral Pathology; JSOMS: Japanese Society of Oral and Maxillofacial Surgeons; JSOM: Japanese Society of Oral Medicine; JAMI: Japanese Academy of Maxillofacial Implants. ∗ Corresponding author. Tel.: +91 9743764783. E-mail addresses: [email protected] (T.P. Kale), [email protected] (S.M. Kotrashetti), [email protected] (A. Singh). 1 Tel.: +91 9448472891. 2 Tel.: +91 8722323369.
unknown. The original description was that of a vascular malformation, that is, present at birth, anatomic predilection for lower limbs, proportionate growth and equal sex distribution [1]. Involvement of the mucosa, including the oral cavity, is seen occasionally as integration of a systemic variety or with multiple cutaneous lesions in other locations. Isolated oral involvement is extremely rare while acquired involvement of tongue without cutaneous or metabolic disease has not been reported in the literature. 2. Case report A 60-year-old Asian Indian male, presented to our Oral and Maxillofacial unit with a complaint of a growth over the left half of the tongue. He was having trouble eating and had developed a psychological component of fear of it being a cancerous growth. He gave a history of extraction done of a lower left third molar at a local dentist 3 months prior to his presentation to us, which he considered traumatic, after which a small ulcerative lesion 1 cm × 1 cm appeared over the left anterior half of the tongue in the left second molar region. Over about a month after the extraction, the ulcer healed and appeared then as a small 1 cm × 1 cm growth which eventually progressed to its current size. There was no history of any associated fever during these events. The patient had no deleterious habits such as tobacco chewing or alcohol consumption. The family history was inconclusive for any hereditary or genetic correlation. General examination did not reveal any significant findings such as lesions on the extremities or the chest and abdomen. On fur-
http://dx.doi.org/10.1016/j.ajoms.2016.02.005 2212-5558/© 2016 Asian AOMS, ASOMP, JSOP, JSOMS, JSOM, and JAMI. Published by Elsevier Ltd. All rights reserved.夽
Please cite this article in press as: Kale TP, et al. Extremely rare isolated and acquired oral presentation – Verrucous hemangioma. J Oral Maxillofac Surg Med Pathol (2016), http://dx.doi.org/10.1016/j.ajoms.2016.02.005
G Model JOMSMP-485; No. of Pages 4 2
ARTICLE IN PRESS T.P. Kale et al. / Journal of Oral and Maxillofacial Surgery, Medicine, and Pathology xxx (2016) xxx–xxx
Fig. 1. Lesion in situ on deviation of tongue to opposite side.
ther intraoral examination, a 3 cm in length and 2 cm in width irregular, mixed white and red, verrucous growth was seen over the left anterior 2/3rd of the tongue which was firm, sessile and nontender (Fig. 1). In the mandibular arch, an edentulous span was present posterior to the mandibular left canine, whose tooth preparation was done to receive a crown prosthesis, and there were porcelain fused to metal crowns over the lower anterior teeth. There were also fractured restorations and crowns of the right mandibular second molar, first and second premolar. All maxillary teeth were present with moderate amount of attrition and abrasion. No sharp cusps or points were present. No regional lymph nodes were palpable. Routine blood and urine investigations were ordered which were within the normal limits. Based on the clinical features, a provisional diagnosis of verrucous hyperplasia involving the left anterior lateral half of tongue was given. The treatment plan included a wide excision biopsy for the lesion. The patient was explained the risks associated and after obtaining informed consent the patient was taken up under general anaesthesia. After placing an anterior traction suture and retracting the tongue to the opposite side, an elliptical incision was given using an electrocautery around the periphery of the lesion maintaining a 5 mm margin of clinically healthy tissue (Fig. 2a). The lesion was excised (Fig. 2b) and the wound sutured using Polyglactin 910 sutures and sent for histopathology examination. The histopathology of the specimen under low magnification of one area revealed papillary projections with connective tissue core,
surface keratinisation, keratin plugging, acanthosis and individual cell keratinisation. Other side of the tissue shows loss of epithelium, fibrinous exudates and necrotic debris. Numerous endothelial lined blood vessels engorged with RBCs were also evident (Fig. 3). Under higher magnification, the epithelium confirmed the findings as low magnification. The underlying connective tissue stroma was loose with numerous branching endothelial lined spaces with engorged RBCs. The stromal tissue in few areas showed myxoid degeneration. The deeper connective tissues stroma showed muscle tissue interspread with small to large capillary spaces with RBCs. A few chronic inflammatory cells such as lymphocytes, plasma cells and mast cells were also seen (Fig. 4). Based on these findings a histopathological diagnosis of verrucous hemangioma was given. Due to the rarity of the presentation in the oral cavity the margins of the lesion were further examined which were found to be close or clear margins.
Fig. 3. Photomicrograph (4×) of specimen showing papillary projections with surface keratinisation, individual cell keratinisation and plugging, acanthosis, fibrinous exudates and underlying loose connective tissue stroma with numerous endothelial lined spaces.
Postoperatively, the wound healed uneventfully and the patient has been followed up regularly for 9 months now without any signs of recurrence or new lesions (Fig. 5).
Fig. 2. (a) Site after excision of lesion. (b) Excised lesion.
Please cite this article in press as: Kale TP, et al. Extremely rare isolated and acquired oral presentation – Verrucous hemangioma. J Oral Maxillofac Surg Med Pathol (2016), http://dx.doi.org/10.1016/j.ajoms.2016.02.005
G Model JOMSMP-485; No. of Pages 4
ARTICLE IN PRESS T.P. Kale et al. / Journal of Oral and Maxillofacial Surgery, Medicine, and Pathology xxx (2016) xxx–xxx
Fig. 4. Photomicrograph (10×) of specimen showing underlying loose connective tissue stroma with numerous branching endothelial lined spaces engorged with RBCs and a few chronic inflammatory cells.
3. Discussion Verrucous hemangioma 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 [1]. The clinical appearance may simulate that of angiokeratoma and distinction is important as the histological and clinical behaviour differ. Verrucous hemangiomas are vascular malformations, whereas the angiokeratomas are telangiectasias [1].
Fig. 5. 9th month postoperative image of site of lesion.
The term “malformation” was proposed for this lesion by Mankani and Dufresne [2] due to its typical features, but in our case the presentation is more suggestive of a tumour in accordance to the definition by Willis [3], that is, an abnormal mass of tissue, the growth of which exceeds and is uncoordinated with that of the normal tissues, and persists in the same excessive manner after cessation of the stimuli which evoked the change. Many terms have been used synonymously for this malformation in the literature such as hemangioma unilateralis neviforme, angiokeratoma circumscriptum neviforme, nevus vascularis unius lateralis, keratotic hemangioma, nevus angiokeratoticus, nevus keratoangiomatosus and papulous angiokeratoma.
3
Only one study with an intraoral presentation of isolated verrucous hemangioma reported a lesion on the distal surface of lower left second molar. The lesion appeared to be reddish pink in colour, interspersed with bluish black areas grouped together. Surface of the growth was irregular. Papules were seen on soft palate extending up to the pharynx which caused difficulty in swallowing and discomfort [4]. Their presentation did not match our case and reflects the uncertain manifestation of the lesion which may be present. A study done by Tennant et al. compared the clinical and histopathologic characteristics of verrucous hemangioma with other hyperkeratotic mimickers such as capillary-lymphatic malformation or capillary-venous malformation and angiokeratoma circumscriptum, and whether the term was appropriate in the current nosology of vascular anomalies [5]. All the lesions were located on an extremity, except for one lesion on the trunk, and were single or grouped and 2.6–8 cm in diameter. All the lesions were raised, red-to-purple, variably keratotic with irregular borders, and several manifested intermittent bleeding and oozing. They conferred no firm conclusion on whether verrucous hemangioma was a congenital malformation or an indolent tumour, but clinical evidence favours the former category [5]. The occurrence of this lesion in our case was in the 6th decade indicating that it is not confined to being a solely congenital malformation. With such a late presentation one could only assume an acquired aetiology for the lesion such as trauma, altered haemodynamics or possibly secondary infection. Verrucous lesions of the oral cavity, especially the tongue, can be challenging diagnostically as they include a large spectrum of benign, potentially malignant and malignant lesions. Majority of the benign lesions have a viral aetiology and include the squamous papilloma. The potentially malignant lesions have been described in a perplex manner and include proliferative verrucous leukoplakia and verrucous hyperplasia which most often cannot be distinguished clinically from verrucous carcinoma. The differential diagnosis in our case would include all of the above. The most reliable way to distinguish these entities is by histological examination. Histologically, verrucous hemangioma presents as an epidermis with irregular acanthosis and hyperkeratosis. Abnormal vessels are located in the dermis and hypodermis with almost no involvement of the reticular dermis. The vessels are round, with thick walls and a multi lamellar basement membrane. The dilated vessels of the papillary dermis often contain blood, are thin-walled, and have a vertical orientation, whereas the deeper vessels may contain blood or be empty. Immunohistochemical staining with endothelial markers like CD 31, CD 34 and GLUT 1 may highlight the endothelial cells, but the diagnosis can be made by light microscopic features [6]. Wang et al. [7] found the presence of CD31 marker in all their 74 samples while Couto et al. [8] observed mosaicism for a missense mutation in mitogen-activated protein kinase kinase kinase 3 (MAP3K3) which could be suggestive of a vascular marker more sensitive than specific for the cutaneous verrucous hemangioma. Secondary infection and bleeding is a frequent complication and this could result in reactive papillomatosis and hyperkeratosis, and thus, the older lesions acquire a verrucous surface. Surgical treatment of the cutaneous condition is now preferred to be excision instead of ablation by laser or cryosurgery [2]. Yang and Ohara suggested surgical excision in combination with laser therapy as the preferred treatment for verrucous hemangioma because the crucial pathologic change is concentrated in the subcutaneous tissue. They used clinical inspection, palpation, and imaging studies before surgery to delineate the underlying mass [9]. Our treatment plan was a wide excision until healthy looking tongue musculature was seen without combined laser therapy and similar results were achieved.
Please cite this article in press as: Kale TP, et al. Extremely rare isolated and acquired oral presentation – Verrucous hemangioma. J Oral Maxillofac Surg Med Pathol (2016), http://dx.doi.org/10.1016/j.ajoms.2016.02.005
G Model JOMSMP-485; No. of Pages 4
ARTICLE IN PRESS T.P. Kale et al. / Journal of Oral and Maxillofacial Surgery, Medicine, and Pathology xxx (2016) xxx–xxx
4
In our case we had clear margins in all the edges of the specimen, except in the posterior inferior part which had close margins (1–5 mm). The recurrence after surgical intervention of cutaneous lesions is noted to be greater when the lesion is incompletely removed, as in some instances a staged procedure is planned to achieve adequate primary closure eventually [9]. We did not intervene a second time surgically due to the purely benign nature of the lesion and the complete, but close margins found, and have followed up closely. The general consensus on the follow-up period for verrucous hemangioma is unknown. Our patient was followed up for a period of 6 months without any signs of recurrence. We suggest a regular follow-up period of every 3 months in the first year followed by every 6 months thereafter due to the rapid progression seen in this case. 4. Conclusion This is the first reported case in the indexed literature of a verrucous hemangioma with an isolated occurrence and proposed acquired aetiology. Due to the recent appearance of similar articles [4] in the literature and the unusual presentation we need to reconsider our current knowledge on the etio-pathogenesis of verrucous hemangioma and its clinical inclusion in the differential diagnosis of various aforementioned conditions. Ethical approval Obtained.
Conflict of interest None. Competing interests None declared. Patient consent Informed consent was taken from the patient. References [1] Imperial R, Helwig EB. Verrucous hemangioma. A clinicopathologic study of 21 cases. Arch Dermatol 1967;96:247. [2] Mankani MH, Dufresne CR. Verrucous malformations: their presentation and management. Ann Plast Surg 2000;45:31–6. [3] Willis RA. Pathology of tumors. 3rd ed. Washington, DC: Butterworth; 1960, 1002 p. [4] Lele GS, Salunkhe BB, Lele SM. Intraoral verrucous hemangioma: a case report. J Dent Appl 2015;2:229–31. [5] Tennant LB, Mulliken JB, Perez-Atayde AR, Kozakewich HP. Verrucous hemangioma revisited. Pediatr Dermatol 2006;23:208–15. [6] Garrido-Rioss AA, Sanchez-Velicia L, Marino-Harrison JM, Torrero-Anton MV, Miranda-Romero A. A histopathologic and imaging study of verrucous hemangioma. Actas Dermosifiliogr 2008;99:723–6. [7] Wang L, Gao T, Wang G. Verrucous hemangioma: a clinicopathological and immunohistochemical analysis of 74 cases. J Cutan Pathol 2014;41:823–30. [8] Couto JA, Vivero MP, Kozakewich HP, Taghinia AH, Mulliken JB, Warman ML, et al. A somatic MAP3K3 mutation is associated with verrucous venous malformation. Am J Hum Genet 2015;96:480–6. [9] Yang CH, Ohara K. Successful surgical treatment of verrucous hemangioma: a combined approach. Dermatol Surg 2002;28:913–9.
Please cite this article in press as: Kale TP, et al. Extremely rare isolated and acquired oral presentation – Verrucous hemangioma. J Oral Maxillofac Surg Med Pathol (2016), http://dx.doi.org/10.1016/j.ajoms.2016.02.005
ARTICLE IN PRESS Journal of Oral and Maxillofacial Surgery, Medicine, and Pathology xxx (2016) xxx–xxx
Contents lists available at ScienceDirect
Journal of Oral and Maxillofacial Surgery, Medicine, and Pathology journal homepage: www.elsevier.com/locate/jomsmp
Case Report
Extremely rare isolated and acquired oral presentation – Verrucous hemangioma Tejraj P. Kale ∗,1 , S.M. Kotrashetti 2 , Arjun Singh ∗ Department of Oral and Maxillofacial Surgery, V.K. Institute of Dental Sciences, KLE University, Belgaum, Karnataka, India
a r t i c l e
i n f o
Article history: Received 2 July 2015 Received in revised form 5 January 2016 Accepted 15 February 2016 Available online xxx Keywords: Oral verrucous hemangioma Rare presentation hemangioma Isolated and acquired hemangioma
a b s t r a c t Imperial and Helwig originally described verrucous hemangioma as a vascular malformation that is present at birth, anatomic predilection for lower limbs, proportionate growth and equal sex distribution. Mucosal involvement, including the oral cavity, is seen occasionally as integration of a systemic variety or with multiple cutaneous lesions in other locations. Isolated oral involvement is extremely rare while acquired involvement of tongue without cutaneous or metabolic disease has not been reported in the literature. We present a case of verrucous hemangioma involving the lateral surface of the tongue in a 60-year-old male. Due to the unusual presentation we need to reconsider our current knowledge on the etio-pathogenesis of verrucous hemangioma and its clinical inclusion in the differential diagnosis of various verrucous lesions. © 2016 Asian AOMS, ASOMP, JSOP, JSOMS, JSOM, and JAMI. Published by Elsevier Ltd. All rights reserved.夽
1. Introduction Vascular lesions and malformations, comprising of a broad category of lesions often referred to as vascular anomalies, are a heterogeneous group of clinicopathologically distinct entities. Clinicians traditionally have grouped these lesions under the generic term, hemangioma, sometimes classified as cavernous (large vessels) or capillary (small vessels) [1]. Most are congenital or occur at a young age and may show limited growth potential. Some lesions undergo spontaneous regression, which has led some to believe it to be a hamartoma rather than a tumour, while others require active intervention [2]. Imperial and Helwig [1] first introduced the verrucous hemangioma, usually found as a congenital solitary or multiple cutaneous lesions of lower limbs, and distinguished it from the angiokeratoma. Although it is a clinically specific lesion with characteristic histological features, its precise nature remains
Abbreviations: RBC, red blood cells; CD, cluster of differentiation; GLUT, glucose transporter; MAP3K3, mitogen-activated protein kinase kinase kinase 3. 夽 Asian AOMS: Asian Association of Oral and Maxillofacial Surgeons; ASOMP: Asian Society of Oral and Maxillofacial Pathology; JSOP: Japanese Society of Oral Pathology; JSOMS: Japanese Society of Oral and Maxillofacial Surgeons; JSOM: Japanese Society of Oral Medicine; JAMI: Japanese Academy of Maxillofacial Implants. ∗ Corresponding author. Tel.: +91 9743764783. E-mail addresses: [email protected] (T.P. Kale), [email protected] (S.M. Kotrashetti), [email protected] (A. Singh). 1 Tel.: +91 9448472891. 2 Tel.: +91 8722323369.
unknown. The original description was that of a vascular malformation, that is, present at birth, anatomic predilection for lower limbs, proportionate growth and equal sex distribution [1]. Involvement of the mucosa, including the oral cavity, is seen occasionally as integration of a systemic variety or with multiple cutaneous lesions in other locations. Isolated oral involvement is extremely rare while acquired involvement of tongue without cutaneous or metabolic disease has not been reported in the literature. 2. Case report A 60-year-old Asian Indian male, presented to our Oral and Maxillofacial unit with a complaint of a growth over the left half of the tongue. He was having trouble eating and had developed a psychological component of fear of it being a cancerous growth. He gave a history of extraction done of a lower left third molar at a local dentist 3 months prior to his presentation to us, which he considered traumatic, after which a small ulcerative lesion 1 cm × 1 cm appeared over the left anterior half of the tongue in the left second molar region. Over about a month after the extraction, the ulcer healed and appeared then as a small 1 cm × 1 cm growth which eventually progressed to its current size. There was no history of any associated fever during these events. The patient had no deleterious habits such as tobacco chewing or alcohol consumption. The family history was inconclusive for any hereditary or genetic correlation. General examination did not reveal any significant findings such as lesions on the extremities or the chest and abdomen. On fur-
http://dx.doi.org/10.1016/j.ajoms.2016.02.005 2212-5558/© 2016 Asian AOMS, ASOMP, JSOP, JSOMS, JSOM, and JAMI. Published by Elsevier Ltd. All rights reserved.夽
Please cite this article in press as: Kale TP, et al. Extremely rare isolated and acquired oral presentation – Verrucous hemangioma. J Oral Maxillofac Surg Med Pathol (2016), http://dx.doi.org/10.1016/j.ajoms.2016.02.005
G Model JOMSMP-485; No. of Pages 4 2
ARTICLE IN PRESS T.P. Kale et al. / Journal of Oral and Maxillofacial Surgery, Medicine, and Pathology xxx (2016) xxx–xxx
Fig. 1. Lesion in situ on deviation of tongue to opposite side.
ther intraoral examination, a 3 cm in length and 2 cm in width irregular, mixed white and red, verrucous growth was seen over the left anterior 2/3rd of the tongue which was firm, sessile and nontender (Fig. 1). In the mandibular arch, an edentulous span was present posterior to the mandibular left canine, whose tooth preparation was done to receive a crown prosthesis, and there were porcelain fused to metal crowns over the lower anterior teeth. There were also fractured restorations and crowns of the right mandibular second molar, first and second premolar. All maxillary teeth were present with moderate amount of attrition and abrasion. No sharp cusps or points were present. No regional lymph nodes were palpable. Routine blood and urine investigations were ordered which were within the normal limits. Based on the clinical features, a provisional diagnosis of verrucous hyperplasia involving the left anterior lateral half of tongue was given. The treatment plan included a wide excision biopsy for the lesion. The patient was explained the risks associated and after obtaining informed consent the patient was taken up under general anaesthesia. After placing an anterior traction suture and retracting the tongue to the opposite side, an elliptical incision was given using an electrocautery around the periphery of the lesion maintaining a 5 mm margin of clinically healthy tissue (Fig. 2a). The lesion was excised (Fig. 2b) and the wound sutured using Polyglactin 910 sutures and sent for histopathology examination. The histopathology of the specimen under low magnification of one area revealed papillary projections with connective tissue core,
surface keratinisation, keratin plugging, acanthosis and individual cell keratinisation. Other side of the tissue shows loss of epithelium, fibrinous exudates and necrotic debris. Numerous endothelial lined blood vessels engorged with RBCs were also evident (Fig. 3). Under higher magnification, the epithelium confirmed the findings as low magnification. The underlying connective tissue stroma was loose with numerous branching endothelial lined spaces with engorged RBCs. The stromal tissue in few areas showed myxoid degeneration. The deeper connective tissues stroma showed muscle tissue interspread with small to large capillary spaces with RBCs. A few chronic inflammatory cells such as lymphocytes, plasma cells and mast cells were also seen (Fig. 4). Based on these findings a histopathological diagnosis of verrucous hemangioma was given. Due to the rarity of the presentation in the oral cavity the margins of the lesion were further examined which were found to be close or clear margins.
Fig. 3. Photomicrograph (4×) of specimen showing papillary projections with surface keratinisation, individual cell keratinisation and plugging, acanthosis, fibrinous exudates and underlying loose connective tissue stroma with numerous endothelial lined spaces.
Postoperatively, the wound healed uneventfully and the patient has been followed up regularly for 9 months now without any signs of recurrence or new lesions (Fig. 5).
Fig. 2. (a) Site after excision of lesion. (b) Excised lesion.
Please cite this article in press as: Kale TP, et al. Extremely rare isolated and acquired oral presentation – Verrucous hemangioma. J Oral Maxillofac Surg Med Pathol (2016), http://dx.doi.org/10.1016/j.ajoms.2016.02.005
G Model JOMSMP-485; No. of Pages 4
ARTICLE IN PRESS T.P. Kale et al. / Journal of Oral and Maxillofacial Surgery, Medicine, and Pathology xxx (2016) xxx–xxx
Fig. 4. Photomicrograph (10×) of specimen showing underlying loose connective tissue stroma with numerous branching endothelial lined spaces engorged with RBCs and a few chronic inflammatory cells.
3. Discussion Verrucous hemangioma 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 [1]. The clinical appearance may simulate that of angiokeratoma and distinction is important as the histological and clinical behaviour differ. Verrucous hemangiomas are vascular malformations, whereas the angiokeratomas are telangiectasias [1].
Fig. 5. 9th month postoperative image of site of lesion.
The term “malformation” was proposed for this lesion by Mankani and Dufresne [2] due to its typical features, but in our case the presentation is more suggestive of a tumour in accordance to the definition by Willis [3], that is, an abnormal mass of tissue, the growth of which exceeds and is uncoordinated with that of the normal tissues, and persists in the same excessive manner after cessation of the stimuli which evoked the change. Many terms have been used synonymously for this malformation in the literature such as hemangioma unilateralis neviforme, angiokeratoma circumscriptum neviforme, nevus vascularis unius lateralis, keratotic hemangioma, nevus angiokeratoticus, nevus keratoangiomatosus and papulous angiokeratoma.
3
Only one study with an intraoral presentation of isolated verrucous hemangioma reported a lesion on the distal surface of lower left second molar. The lesion appeared to be reddish pink in colour, interspersed with bluish black areas grouped together. Surface of the growth was irregular. Papules were seen on soft palate extending up to the pharynx which caused difficulty in swallowing and discomfort [4]. Their presentation did not match our case and reflects the uncertain manifestation of the lesion which may be present. A study done by Tennant et al. compared the clinical and histopathologic characteristics of verrucous hemangioma with other hyperkeratotic mimickers such as capillary-lymphatic malformation or capillary-venous malformation and angiokeratoma circumscriptum, and whether the term was appropriate in the current nosology of vascular anomalies [5]. All the lesions were located on an extremity, except for one lesion on the trunk, and were single or grouped and 2.6–8 cm in diameter. All the lesions were raised, red-to-purple, variably keratotic with irregular borders, and several manifested intermittent bleeding and oozing. They conferred no firm conclusion on whether verrucous hemangioma was a congenital malformation or an indolent tumour, but clinical evidence favours the former category [5]. The occurrence of this lesion in our case was in the 6th decade indicating that it is not confined to being a solely congenital malformation. With such a late presentation one could only assume an acquired aetiology for the lesion such as trauma, altered haemodynamics or possibly secondary infection. Verrucous lesions of the oral cavity, especially the tongue, can be challenging diagnostically as they include a large spectrum of benign, potentially malignant and malignant lesions. Majority of the benign lesions have a viral aetiology and include the squamous papilloma. The potentially malignant lesions have been described in a perplex manner and include proliferative verrucous leukoplakia and verrucous hyperplasia which most often cannot be distinguished clinically from verrucous carcinoma. The differential diagnosis in our case would include all of the above. The most reliable way to distinguish these entities is by histological examination. Histologically, verrucous hemangioma presents as an epidermis with irregular acanthosis and hyperkeratosis. Abnormal vessels are located in the dermis and hypodermis with almost no involvement of the reticular dermis. The vessels are round, with thick walls and a multi lamellar basement membrane. The dilated vessels of the papillary dermis often contain blood, are thin-walled, and have a vertical orientation, whereas the deeper vessels may contain blood or be empty. Immunohistochemical staining with endothelial markers like CD 31, CD 34 and GLUT 1 may highlight the endothelial cells, but the diagnosis can be made by light microscopic features [6]. Wang et al. [7] found the presence of CD31 marker in all their 74 samples while Couto et al. [8] observed mosaicism for a missense mutation in mitogen-activated protein kinase kinase kinase 3 (MAP3K3) which could be suggestive of a vascular marker more sensitive than specific for the cutaneous verrucous hemangioma. Secondary infection and bleeding is a frequent complication and this could result in reactive papillomatosis and hyperkeratosis, and thus, the older lesions acquire a verrucous surface. Surgical treatment of the cutaneous condition is now preferred to be excision instead of ablation by laser or cryosurgery [2]. Yang and Ohara suggested surgical excision in combination with laser therapy as the preferred treatment for verrucous hemangioma because the crucial pathologic change is concentrated in the subcutaneous tissue. They used clinical inspection, palpation, and imaging studies before surgery to delineate the underlying mass [9]. Our treatment plan was a wide excision until healthy looking tongue musculature was seen without combined laser therapy and similar results were achieved.
Please cite this article in press as: Kale TP, et al. Extremely rare isolated and acquired oral presentation – Verrucous hemangioma. J Oral Maxillofac Surg Med Pathol (2016), http://dx.doi.org/10.1016/j.ajoms.2016.02.005
G Model JOMSMP-485; No. of Pages 4
ARTICLE IN PRESS T.P. Kale et al. / Journal of Oral and Maxillofacial Surgery, Medicine, and Pathology xxx (2016) xxx–xxx
4
In our case we had clear margins in all the edges of the specimen, except in the posterior inferior part which had close margins (1–5 mm). The recurrence after surgical intervention of cutaneous lesions is noted to be greater when the lesion is incompletely removed, as in some instances a staged procedure is planned to achieve adequate primary closure eventually [9]. We did not intervene a second time surgically due to the purely benign nature of the lesion and the complete, but close margins found, and have followed up closely. The general consensus on the follow-up period for verrucous hemangioma is unknown. Our patient was followed up for a period of 6 months without any signs of recurrence. We suggest a regular follow-up period of every 3 months in the first year followed by every 6 months thereafter due to the rapid progression seen in this case. 4. Conclusion This is the first reported case in the indexed literature of a verrucous hemangioma with an isolated occurrence and proposed acquired aetiology. Due to the recent appearance of similar articles [4] in the literature and the unusual presentation we need to reconsider our current knowledge on the etio-pathogenesis of verrucous hemangioma and its clinical inclusion in the differential diagnosis of various aforementioned conditions. Ethical approval Obtained.
Conflict of interest None. Competing interests None declared. Patient consent Informed consent was taken from the patient. References [1] Imperial R, Helwig EB. Verrucous hemangioma. A clinicopathologic study of 21 cases. Arch Dermatol 1967;96:247. [2] Mankani MH, Dufresne CR. Verrucous malformations: their presentation and management. Ann Plast Surg 2000;45:31–6. [3] Willis RA. Pathology of tumors. 3rd ed. Washington, DC: Butterworth; 1960, 1002 p. [4] Lele GS, Salunkhe BB, Lele SM. Intraoral verrucous hemangioma: a case report. J Dent Appl 2015;2:229–31. [5] Tennant LB, Mulliken JB, Perez-Atayde AR, Kozakewich HP. Verrucous hemangioma revisited. Pediatr Dermatol 2006;23:208–15. [6] Garrido-Rioss AA, Sanchez-Velicia L, Marino-Harrison JM, Torrero-Anton MV, Miranda-Romero A. A histopathologic and imaging study of verrucous hemangioma. Actas Dermosifiliogr 2008;99:723–6. [7] Wang L, Gao T, Wang G. Verrucous hemangioma: a clinicopathological and immunohistochemical analysis of 74 cases. J Cutan Pathol 2014;41:823–30. [8] Couto JA, Vivero MP, Kozakewich HP, Taghinia AH, Mulliken JB, Warman ML, et al. A somatic MAP3K3 mutation is associated with verrucous venous malformation. Am J Hum Genet 2015;96:480–6. [9] Yang CH, Ohara K. Successful surgical treatment of verrucous hemangioma: a combined approach. Dermatol Surg 2002;28:913–9.
Please cite this article in press as: Kale TP, et al. Extremely rare isolated and acquired oral presentation – Verrucous hemangioma. J Oral Maxillofac Surg Med Pathol (2016), http://dx.doi.org/10.1016/j.ajoms.2016.02.005