J. Maxillofac. Oral Surg. (2016) 15(Suppl 2):S371–S374 DOI 10.1007/s12663-016-0901-y
CASE REPORT
Intraoral Superficial Angiomyxoma: A Case Report Venkatesh Anehosur1 • Sahana Adirajaiah1 • Rajarshi Ghosh1
Received: 1 September 2015 / Accepted: 21 March 2016 / Published online: 19 April 2016 Ó The Association of Oral and Maxillofacial Surgeons of India 2016
Abstract Angiomyxomas are a group of relatively uncommon myxoid mesenchymal tumors associated with a high risk of local recurrence without any metastatic potential. Till date only five cases of intraoral superficial angiomyxoma have been reported. This is a case report of a middle aged Indian male patient diagnosed with superficial angiomyxoma of lower left buccal vestibule. Keywords invasion
Myxoma Angiomyxoma Perineural
Case Report A 32 year old male visited our craniofacial unit, SDM College of Dental Sciences & Hospital, Dharwad with chief complaints of swelling on the left lower side of his face. On history patient revealed that the swelling first appeared 10 years back in 2006, in the same region and he got the lesion excised under local anesthesia in 2008 in a local hospital. The histopathological and FNAC reports of the lesion were not available. After this again 3 years back he noticed a similar swelling in the same region which was gradually increasing in size.
& Venkatesh Anehosur [email protected] 1
SDM College of Dental Sciences & Hospital, Sattur, Dharwad, Karnataka 580009, India
The extra oral swelling measured about 3 cm 9 2 cm in size. Soft in consistency, non-tender, skin over the swelling is pinchable and with no history of paresthesia. On intraoral examination a diffused swelling in left lower buccal vestibule was seen measuring around 5 cm 9 3 cm in size extending from 34 to 37 regions anterior-posteriorly. After clinical examination and FNAC reports provisional diagnosis of mucous retention cyst was achieved. Orthopantomogram and mandibular occlusal radiographs were taken but no evident changes were observed. The lesion was excised under general anesthesia. Before excision wide bore needle aspiration was done but no fluid or blood was aspirated. A vestibular incision was given over the mucosa covering the swelling. As the submucosal tissue was exposed part of the tumor bulged out as shown in Fig. 1. The tumor was encapsulated with no signs of local infiltration. The only distinctive feature noted was that the tumor was involving the mental nerve as shown in Fig. 2. The lesion was removed in toto and mental nerve and vessels were ligated and incised as shown in Fig. 3. Figure 4 shows the enucleated lesion. The lesion was then sent for histopathologic examination. There were no postoperative complications. Macroscopic examination showed lobulated, glossy cream in color, well capsulated lesion measuring about 6 cm 9 4 cm in size. Histologic examination showed a myxomatous stroma with focal bundles of collagen fibers consisting of spindle shaped to stellate shaped cells, numerous small to medium sized blood capillaries with thick walled vessels, mast cells, lymphocytes and extravasated RBCs. These features are quite evident in Fig. 5. The myxoid stroma infiltrating between muscle fibers and perineural infiltration is also evident.
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Fig. 4 Macroscopic view of the enucleated lesion Fig. 1 Lesion bulging out from the left lower buccal vestibule
Fig. 2 Lesion involving the mental nerve
Fig. 5 Microscopic view showing a myxomatous stroma consisting of spindle shaped cells and numerous small to medium sized blood capillaries
Discussion
Fig. 3 Post removal of the lesion showing ligated mental nerve
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Superficial angiomyxomas are rare, distinctive, benign, cutaneous soft tissue lesions with a predilection for the trunk, head and neck. Rarely it may involve lower extremities and genitalia [4]. Superficial angiomyxoma is an extremely rare lesion and only a few reports are available in the literature. It was first described by Allen et al. [6]. The term superficial angiomyxoma was first used in 1989 to designate a benign, myxoid-type neoplasm characterized by the presence of scattered cell nests and small
J. Maxillofac. Oral Surg. (2016) 15(Suppl 2):S371–S374
S373
Table 1 Previously reported cases of intraoral superficial angiomyxoma Reference
Site
Age
Gender
Race
Size (mm)
Presentation
Clinical impression
Chen et al. [1]
Right buccal mucosa
19 years
Male
Chinese
50 9 35 9 30
Slow growing painless lump since 2 years
Soft tissue tumor
Gardner [2]
Floor of mouth
69 years
Female
European
10 9 12 9 12
Slow growing painless lump since 3 years
Lipoma
Meer and Beavon [4]
Right buccal mucosa
37 years
Female
African
45 9 32 9 20
Slow growing painless lump since 2 years
Lipoma
Mokhtar et al. [3]
Floor of mouth
6 months
Male
Malaysian
50 9 36 9 26
Slow growing swelling noticed since he was 5 months old
Soft tissue tumor/cystic swelling
Ravindra et al. [5]
Upper posterior alveolar margin
30 years
Male
Indian
30 9 30
Slow growing painless swelling for past 1.5 years
Soft tissue tumor
Present case
Right lower buccal vestibule
32 years
Male
Indian
60 9 40
Slow growing painless swelling since 3 years
Cystic swelling
vessels. This entity was named ‘superficial angiomyxoma’ in order to distinguish it from ‘aggressive angiomyxoma’, which primarily affects the female genital region [9]. Calonje et al. [7] reported a 20 % recurrence rate locally after excision. This is mainly due to incomplete excision. Although extraoral superficial angiomyxomas and intraoral angiomyxomas are similar but intraoral angiomyxomas have not shown any recurrences [3]. There is slight male predilection and most cases present in the middle age [2]. They typically present as polypoid or papulonodular lesions that may be confused with a cyst, skin tag or neurofibroma [4]. Pathologically it may be difficult to differentiate myxoma from other tumors with myxoid stroma and is occasionally misinterpreted as malignant [8]. The distinctive histopathology of superficial angiomyxoma is multilobular growth pattern composed of spindle shaped to stellate cells in a copious myxoid stroma. Small thin walled vessels were prominent and there is presence of stromal inflammatory cells specially neutrophils which is an important clue in its diagnosis [5]. The main histologic differential diagnosis includes aggressive angiomyxoma, soft tissue myxoma, angiomyolipoma, myxoid nerve sheath tumor, myxoid neurofibroma, odontogenic myxoma, oral focal mucinosis, and myxofibrosarcoma. Superficial angiomyxoma can be distinguished from aggressive angiomyxoma by the presence of proliferation of spindle or satellite shaped cells that are widely separated by loose myxoid stroma in which there is prominent large vascular components [5]. A brief outline of previously reported oral superficial angiomyxoma is given above in Table 1.
Conclusion Superficial angiomyxomas are very rare neoplasms intraorally. Till date only five cases of intraoral superficial angiomyxoma has been reported. The additional features that were found in our case was firstly the involvement of mental nerve with in the tumor and secondly history of recurrence which is unlikely for intraoral superficial angiomyxomas according to literature. So although it can be readily treated by local excision regular review with long term follow up is required to rule out recurrence. Acknowledgments The authors thank Dr. Niranjan Kumar (MS, FRCS, Mch Plastic Suregry) Medical Director, SDM College of Medical Sciences & Hospital, Dr. Srinath Thakur, Principal, SDM College of Dental Sciences & Hospital for providing us with the opportunity to do the study. They also thank Dr. Ravikala Rao, Professor Department of General Pathology, SDM College of Medical Sciences & Hospital for providing us with a final diagnosis for this case.
References 1. Chen YK, Lin LM, Lin CC, Yan YH (1998) Myxoid tumor of the oral cavity with features of superficial angiomyxoma: report of a case. J Oral Maxillofac Surg 56(3):379–382 2. Gardner AW (2007) Superficial angiomyxoma of the floor of the mouth—a case report. Br J Oral Maxillofac Surg 45(5):418–419 3. Mokhtar SM, Shahardin RZR, Abidin MZ, Rahman RA (2008) Superficial angiomyxoma of the floor of the mouth in an infant. Asian J Oral Maxillofac Surg 20(1):26–28 4. Meer S, Beavon I (2008) Intraoral superficial angiomyxoma. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 106(5):e20–e23 5. Ravindra SV, Srinivasa M, Sunitha JD, Taneja N, Chandra S, Mahajan S, Panwar E (2012) Intraoral superficial angiomyxoma of
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S374 the upper alveolus: report of a unique case. Case reports in medicine. Article ID 859021, 3 pp 6. Allen PW, Dymock RB, McCormac LB (1988) Superficial angiomyxoma with and without epithelial components: report of 30 tumors in 28 patients. Am J Surg Pathol 12:379–382 7. Calonje E, Guerin D, McCormick D (1999) Superficial angiomyxoma: clinicopathologic analysis of a series of distinctive but poorly
123
J. Maxillofac. Oral Surg. (2016) 15(Suppl 2):S371–S374 recognized tumors with tendency for recurrence. Am J Surg Pathol 23:910–917 8. Ramraj PN, Sah SP (2001) Myxoma of oral soft tissues. JNMA 40:274–276 9. Rodriguez PR, Vicente JD, Villalain LD, Blanco V (2012) Superficial angiomyxoma of the parotid region and review of the literature. Acta Otorrinolaringol Esp 63(2):147–149
CASE REPORT
Intraoral Superficial Angiomyxoma: A Case Report Venkatesh Anehosur1 • Sahana Adirajaiah1 • Rajarshi Ghosh1
Received: 1 September 2015 / Accepted: 21 March 2016 / Published online: 19 April 2016 Ó The Association of Oral and Maxillofacial Surgeons of India 2016
Abstract Angiomyxomas are a group of relatively uncommon myxoid mesenchymal tumors associated with a high risk of local recurrence without any metastatic potential. Till date only five cases of intraoral superficial angiomyxoma have been reported. This is a case report of a middle aged Indian male patient diagnosed with superficial angiomyxoma of lower left buccal vestibule. Keywords invasion
Myxoma Angiomyxoma Perineural
Case Report A 32 year old male visited our craniofacial unit, SDM College of Dental Sciences & Hospital, Dharwad with chief complaints of swelling on the left lower side of his face. On history patient revealed that the swelling first appeared 10 years back in 2006, in the same region and he got the lesion excised under local anesthesia in 2008 in a local hospital. The histopathological and FNAC reports of the lesion were not available. After this again 3 years back he noticed a similar swelling in the same region which was gradually increasing in size.
& Venkatesh Anehosur [email protected] 1
SDM College of Dental Sciences & Hospital, Sattur, Dharwad, Karnataka 580009, India
The extra oral swelling measured about 3 cm 9 2 cm in size. Soft in consistency, non-tender, skin over the swelling is pinchable and with no history of paresthesia. On intraoral examination a diffused swelling in left lower buccal vestibule was seen measuring around 5 cm 9 3 cm in size extending from 34 to 37 regions anterior-posteriorly. After clinical examination and FNAC reports provisional diagnosis of mucous retention cyst was achieved. Orthopantomogram and mandibular occlusal radiographs were taken but no evident changes were observed. The lesion was excised under general anesthesia. Before excision wide bore needle aspiration was done but no fluid or blood was aspirated. A vestibular incision was given over the mucosa covering the swelling. As the submucosal tissue was exposed part of the tumor bulged out as shown in Fig. 1. The tumor was encapsulated with no signs of local infiltration. The only distinctive feature noted was that the tumor was involving the mental nerve as shown in Fig. 2. The lesion was removed in toto and mental nerve and vessels were ligated and incised as shown in Fig. 3. Figure 4 shows the enucleated lesion. The lesion was then sent for histopathologic examination. There were no postoperative complications. Macroscopic examination showed lobulated, glossy cream in color, well capsulated lesion measuring about 6 cm 9 4 cm in size. Histologic examination showed a myxomatous stroma with focal bundles of collagen fibers consisting of spindle shaped to stellate shaped cells, numerous small to medium sized blood capillaries with thick walled vessels, mast cells, lymphocytes and extravasated RBCs. These features are quite evident in Fig. 5. The myxoid stroma infiltrating between muscle fibers and perineural infiltration is also evident.
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Fig. 4 Macroscopic view of the enucleated lesion Fig. 1 Lesion bulging out from the left lower buccal vestibule
Fig. 2 Lesion involving the mental nerve
Fig. 5 Microscopic view showing a myxomatous stroma consisting of spindle shaped cells and numerous small to medium sized blood capillaries
Discussion
Fig. 3 Post removal of the lesion showing ligated mental nerve
123
Superficial angiomyxomas are rare, distinctive, benign, cutaneous soft tissue lesions with a predilection for the trunk, head and neck. Rarely it may involve lower extremities and genitalia [4]. Superficial angiomyxoma is an extremely rare lesion and only a few reports are available in the literature. It was first described by Allen et al. [6]. The term superficial angiomyxoma was first used in 1989 to designate a benign, myxoid-type neoplasm characterized by the presence of scattered cell nests and small
J. Maxillofac. Oral Surg. (2016) 15(Suppl 2):S371–S374
S373
Table 1 Previously reported cases of intraoral superficial angiomyxoma Reference
Site
Age
Gender
Race
Size (mm)
Presentation
Clinical impression
Chen et al. [1]
Right buccal mucosa
19 years
Male
Chinese
50 9 35 9 30
Slow growing painless lump since 2 years
Soft tissue tumor
Gardner [2]
Floor of mouth
69 years
Female
European
10 9 12 9 12
Slow growing painless lump since 3 years
Lipoma
Meer and Beavon [4]
Right buccal mucosa
37 years
Female
African
45 9 32 9 20
Slow growing painless lump since 2 years
Lipoma
Mokhtar et al. [3]
Floor of mouth
6 months
Male
Malaysian
50 9 36 9 26
Slow growing swelling noticed since he was 5 months old
Soft tissue tumor/cystic swelling
Ravindra et al. [5]
Upper posterior alveolar margin
30 years
Male
Indian
30 9 30
Slow growing painless swelling for past 1.5 years
Soft tissue tumor
Present case
Right lower buccal vestibule
32 years
Male
Indian
60 9 40
Slow growing painless swelling since 3 years
Cystic swelling
vessels. This entity was named ‘superficial angiomyxoma’ in order to distinguish it from ‘aggressive angiomyxoma’, which primarily affects the female genital region [9]. Calonje et al. [7] reported a 20 % recurrence rate locally after excision. This is mainly due to incomplete excision. Although extraoral superficial angiomyxomas and intraoral angiomyxomas are similar but intraoral angiomyxomas have not shown any recurrences [3]. There is slight male predilection and most cases present in the middle age [2]. They typically present as polypoid or papulonodular lesions that may be confused with a cyst, skin tag or neurofibroma [4]. Pathologically it may be difficult to differentiate myxoma from other tumors with myxoid stroma and is occasionally misinterpreted as malignant [8]. The distinctive histopathology of superficial angiomyxoma is multilobular growth pattern composed of spindle shaped to stellate cells in a copious myxoid stroma. Small thin walled vessels were prominent and there is presence of stromal inflammatory cells specially neutrophils which is an important clue in its diagnosis [5]. The main histologic differential diagnosis includes aggressive angiomyxoma, soft tissue myxoma, angiomyolipoma, myxoid nerve sheath tumor, myxoid neurofibroma, odontogenic myxoma, oral focal mucinosis, and myxofibrosarcoma. Superficial angiomyxoma can be distinguished from aggressive angiomyxoma by the presence of proliferation of spindle or satellite shaped cells that are widely separated by loose myxoid stroma in which there is prominent large vascular components [5]. A brief outline of previously reported oral superficial angiomyxoma is given above in Table 1.
Conclusion Superficial angiomyxomas are very rare neoplasms intraorally. Till date only five cases of intraoral superficial angiomyxoma has been reported. The additional features that were found in our case was firstly the involvement of mental nerve with in the tumor and secondly history of recurrence which is unlikely for intraoral superficial angiomyxomas according to literature. So although it can be readily treated by local excision regular review with long term follow up is required to rule out recurrence. Acknowledgments The authors thank Dr. Niranjan Kumar (MS, FRCS, Mch Plastic Suregry) Medical Director, SDM College of Medical Sciences & Hospital, Dr. Srinath Thakur, Principal, SDM College of Dental Sciences & Hospital for providing us with the opportunity to do the study. They also thank Dr. Ravikala Rao, Professor Department of General Pathology, SDM College of Medical Sciences & Hospital for providing us with a final diagnosis for this case.
References 1. Chen YK, Lin LM, Lin CC, Yan YH (1998) Myxoid tumor of the oral cavity with features of superficial angiomyxoma: report of a case. J Oral Maxillofac Surg 56(3):379–382 2. Gardner AW (2007) Superficial angiomyxoma of the floor of the mouth—a case report. Br J Oral Maxillofac Surg 45(5):418–419 3. Mokhtar SM, Shahardin RZR, Abidin MZ, Rahman RA (2008) Superficial angiomyxoma of the floor of the mouth in an infant. Asian J Oral Maxillofac Surg 20(1):26–28 4. Meer S, Beavon I (2008) Intraoral superficial angiomyxoma. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 106(5):e20–e23 5. Ravindra SV, Srinivasa M, Sunitha JD, Taneja N, Chandra S, Mahajan S, Panwar E (2012) Intraoral superficial angiomyxoma of
123
S374 the upper alveolus: report of a unique case. Case reports in medicine. Article ID 859021, 3 pp 6. Allen PW, Dymock RB, McCormac LB (1988) Superficial angiomyxoma with and without epithelial components: report of 30 tumors in 28 patients. Am J Surg Pathol 12:379–382 7. Calonje E, Guerin D, McCormick D (1999) Superficial angiomyxoma: clinicopathologic analysis of a series of distinctive but poorly
123
J. Maxillofac. Oral Surg. (2016) 15(Suppl 2):S371–S374 recognized tumors with tendency for recurrence. Am J Surg Pathol 23:910–917 8. Ramraj PN, Sah SP (2001) Myxoma of oral soft tissues. JNMA 40:274–276 9. Rodriguez PR, Vicente JD, Villalain LD, Blanco V (2012) Superficial angiomyxoma of the parotid region and review of the literature. Acta Otorrinolaringol Esp 63(2):147–149
