CASE REPORT

Unicystic Ameloblastoma: A Diagnostic Dilemma and Its Management Using Free Fibula Graft: An Unusual Case Report
Aniket Jain1, Satyajit Dandagi2, Amit Sangle3, Viquar Ahmed4, Akram Khan5
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ABSTRACT: A 20 year old patient reported with a swelling in the left posterior mandibular region since 4 months. On clinical examination, there was a hard, non-tender mass, measuring 8.5 cm by 5 cm arising from the left side of the mandible, involving the ramus,
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angle and body upto the leftt lower 1st premolar tooth. Radiographic picture and fluid aspiration of the pathology with protein analysis of 4.1 gm/dl, was suggestive of a keratinizing cyst or tumor. Hemimandibulectomy was performed with safe margins and an microvascular free fibula graft was placed for mandibular reconstruction. The final diagnosis after histopathological examination was given as Unicystic

Final Year P.G Student Professor 3 Professor 4 Senior Lecturer 5 First year P.G. Student Department and Institution Dept of Oral and Maxillofacial Surgery, M A Rangoonwala College of Dental Sciences and Research Centre, Pune. Article Info: Received: April 15, 2012; Review Completed: May, 14, 2012; Accepted: June 13, 2012 Published Online: August, 2012 (www. nacd. in) NAD, 2012 - All rights reserved Email for correspondence: draniketjain@gmail.com

Ameloblastoma. Facial Symmetry is well maintained with no recurrence after a systematic follow up of 18 months. Key words: Unicystic Ameloblastoma, Microvascular, Free Fibula Flap

INTRODUCTION: The most common tumour of odontogenic origin is ameloblastoma, which develops from epithelial cellular elements and dental tissues in their various phases of development. It is a slow-growing, persistent, and locally aggressive neoplasm of epithelial origin.1 Unicystic ameloblastoma is second important clinical type of ameloblastoma and accounts for 10-15% of all intraosseous ameloblastomas.2 They have been reported to occur in second and third decades of life as against its solid counterpart which occurs in fourth decade of life. Unicystic ameloblastoma most commonly occurs in posterior mandible followed by parasymphysis region, anterior maxilla and posterior maxilla.3 It is a classic example of a true neoplasm of enamel organ type tissue that lacks the potential to undergo differentiation, and hence has aptly been defined as unicentric, nonfunctional, intermittent in growth, anatomically benign and clinically persistent by Robinson.There are three forms of ameloblastomas, namely multicystic, peripheral, and unicystic tumors [4]. Multicystic ameloblastoma is the most common variety and represents 86% of cases. Peripheral tumors are odontogenic

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tumors, with the histological characteristics of intraosseous ameloblastoma that occur solely in the soft tissues covering the tooth-bearing parts of the jaws. Unicystic tumors include those that have been variously referred to as mural ameloblastomas, luminal ameloblastomas, and ameloblastomas arising in dentigerous cysts [5]. It refers to those cystic lesions that show clinical, radiographic or gross features of jaw cyst, but on histologic examination shows typical ameloblastomatous epithelium, lining part of the cystic cavity with or without luminal and/or mural tumor growth.6 We present a case of a large unicystic mandibular ameloblastoma in a young male. Case Report: A 20-year-old female presented to Department of Oral and Maxillofacial Surgery with the Chief complaint of painless swelling in left side of the face since 4 months which was gradually increasing in size. Patient was apparently alright 4 months back, suddenly developed a small swelling in lower left mandibular region which gradually increased in size to attain the present state.(Fig 1) There was no associated pain, difficulty in opening the mouth, chewing or articulating. On physical examination, there was a hard, non-tender mass, measuring 8.5 cm by 5 cm arising from the left side of the mandible, involving the ramus, angle and body upto the leftt lower 1st premolar tooth. Diffuse overgrowth seen in lower left posterior alveolar and vestibular region with normal overlying mucosa (Fig. 2). No neck nodes were palpable. Systemic examination was normal. An Orthopantomogram (OPG) was done, which showed large cystic lesion in the left side of mandible extending from lower left second premolar upto the ramus involving the condyle as well. Radiographic examination revealed a unilocular appearance and was suggestive of a cyst or tumour. (Fig 3 and 4) An aspirate of the fluid was obtained, and protein analysis revealed 4.1 gm/dl, which was suggestive of a keratinizing cyst or tumor. Fine needle aspiration cytology of the lesion as well as incisional biopsy was performed but both were not conclusive. Patient was taken up for surgery under general anesthesia. Exposure of the lesion was done via extended risdons incision followed by hemimandibulectomy along with dearticuation of the TM joint on the left side (Fig. 5 and 6).

Reconstruction was done using a microvascular free fibula graft. Shaping of the resected fibula was done according to the preoperative template. A miniplate with locking screws was used to secure the osteotomized fibula and the mandible (Fig. 7 and 8). Final diagnosis of unicystic ameloblastoma was confirmed with the help of histopathologic examination of the excised specimen (Fig. 9). Facial symmetry was well maintained postoperatively. One year systematic follow-up did not reveal any recurrence as well as showed excellent acceptance of graft in the region mimicking a lower jaw. (Fig 10 and 11) Discussion: Robinson and Martinez were the first persons to describe UA in 1977.7,8 It is most commonly seen in individuals who are 16 to 20 years of age. Occasionally, lesions occur in younger patients; rarely, they have been found in patients up to the age of 40.9 About 90% of the lesions are located in the mandible and between 50 to 80% of these cases are associated with an impacted tooth.10,11 As seen in the present case, the unusual aspect is that its not associated with an imapacted tooth. Facial asymmetry due to swelling is the regular presenting feature which infrequently relates with pain. Unilocular ameloblastoma (UA) is a rare type of ameloblastoma, accounting for about 6% of ameloblastomas. It refers to those cystic lesions that show clinical, radiographic or gross features of a mandibular cyst, but on histologic examination shows a typical ameloblastomatous epithelium lining part of the cyst cavity, with or without luminal and/or mural tumor growth hence, UA should be differentiated from odontogenic cysts and also should be recognized for the reason that the former has a higher rate of recurrence than the latter12. Ackermann et al have provided a histological subgrouping of the Unicystic Ameloblastoma as shown in (Table 1) and a diagrammatic representation of the same shown in (Fig. 12).2 The UAs diagnosed as subgroups 1 and 1.2 can be treated conservatively (careful enucleation), whereas subgroups 1.2.3 and 1.3 showing intramural growths require treated radical resection, as for a solid or multicystic ameloblastoma.13 Following enucleation, vigorous curettage of the bone should be avoided as it may implant foci of ameloblastoma more deeply

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into bone. Chemical cauterization with Carnoys solution is also advocated for subgroups 1 and 1.2. Subgroups 1.2.3 and 1.3 have a high risk for recurrence, requiring more aggressive surgical procedures. This is because the cystic wall in these cases has islands of ameloblastoma tumor cells and there may be penetration into the surrounding cancellous bone.14,15 Recurrence is always an aspect to look after resection specially in the case of unicystic ameloblastoma. Lau et al reported recurrence rates of 3.6% for resection, 30.5% for enucleation alone, 16% for enucleation followed by Carnoys solution application, and 18% by marsupialization followed by enucleation.16 Recurrence rates are also related to the histologic subtypes of UA, with those invading the fibrous wall having a rate of 35.7%, but others only 6.7%. 15 Vascularised fibula graft was first described by Taylor in 1975, and in 1989 Hidalgo first used free fibula flap in mandibular reconstruction.17 Later Chen and Yen incorporated an overlying skin paddle for composite reconstruction of the bone and soft tissue defect.18 After demonstrating that osteotomies can be performed in vascularised fibula grafts without compromising the viability of the bone segment, vascularised free fibula flap became the state of art reconstruction method after mandible ablation. Normally if the tumour is small (< 5cm) the defect can be repaired with a free bone graft. However, the tumour is often larger and a large defect reconstruction is challenging and may require a microsurgical flap either from fibula, iliac crest, scapula, radius or ribs. Since this patient has a large bony defect, a free fibula osteoseptocutaneous flap was chosen to address both the bony and soft tissue defect. Conclusion: The diagnosis of unicystic ameloblastoma was based on clinical, radiological and histopathologic features. Unicystic ameloblastoma is a tumor with a strong propensity for recurrence, hence the Pathologist should examine the tissue sections carefully for better prognosis of the treatment outcome.

References:
1. Gerzenshtein J, Zhang F, Caplan J, Anand V, Lineaweaver W: Immediate mandibular reconstruction with microsurgical fibula flap transfer following wide resection for ameloblastoma. J Craniofac Surg 2006;17(1):178-182. 2. Ackermann GL, Altini M, Shear M. The unicystic ameloblastoma: A clinicopathologic study of 57 cases. J Oral Pathol 1988; 17: 541-546 3. Philipsen HP, Reichart PA. Unicystic ameloblastoma: a review of 193 cases from the literature. Oral Oncol 1998; 34(5):317-325. 4. Philipsen HP, Reichart PA: Classification of odontogenic tumors and allied lesions. Odontogenic tumors and allied lesions Quintessence Pub. Co. Ltd 2004;21-23. 5. Chana, Jagdeep S, Yang-Ming Chang, Wei, Fu-Chan, Shen, Yu-Fen, Chan Chiu-Po, Lin Hsiu-Na, Tsai Chi-Ying, Jeng Seng-Feng: Segmental mandibulectomy and immediate free fibula osteoseptocutaneous flap reconstruction with endosteal implants: An ideal treatment method for mandibular ameloblastoma. Plast Reconstr Surg 2004; 113(1):80-87. 6. Philipsen HP, Reichart PA. Unicystic ameloblastoma. In: Odontogenic tumors and allied lesions. Quintessence Pub.Co.Ltd, 2004; 77-86. 7. Srinivasan H, Arathy Manohar. Unicystic ameloblastoma of the mandible: A case report. Annals and Essence of Dentistry 2010; 2(4):75-77. 8. Navarro CM, Principi SM, Massucato EM, Sposto MR. Maxillary unicystic ameloblastoma. Dentomaxillofac Radiol 2004;33:60-62. 9. Sapp JP. Contemporary Oral and Maxillofacial Pathology (2nd ed) USA: Mosby 2004. 10. Philipsen HP, Reichart PA. Unicystic ameloblastoma. Odontogenic tumors and allied lesions. London: Quintessence Pub. Co. Ltd 2004; 77-86. 11. Pizer ME, Page DG, Svirsky JA. Thirteen-year follow-up of large recurrent unicystic ameloblastoma of the mandible in a 15-year-old boy. J Oral Maxillofac Surg 2002; 60:211-215. 12. Ramesh Rakesh S, Manjunath Suraj, Ustad H Tanveer, et al. Unicystic ameloblatoma of the mandible-an unusual case report and review of literature. Head and Neck Oncology 2010; 2:1. 13. Philipsen HP, Reichart PA: Unicystic ameloblastoma. Odontogenic tumors and allied lesions London: Quintessence Pub. Co. Ltd 2004; 77-86. 14. Li TJ, Kitano M, Arimura K, Sugihara K: Recurrence of unicystic ameloblastoma: A case report and review of the literature. Arch Pathol Lab Med 1998;122:371-374. 15. Li T, Wu Y, Yu S, Yu G: Clinicopathological features of unicystic ameloblastoma with special reference to its recurrence. Zhonghua Kou Qiang Yi Xue Za Zhi 2002; 37:210212. 16. Lau SL, Samman N: Recurrence related to treatment modalities of unicystic ameloblastoma: A systematic review. Int J Oral Maxillofac Surg 2006; 35:681-690 17. Taylor GI, Miller GD, Ham FJ. The free vascularized bone graft: A clinical extension of microvascular techniques. Plast Reconstr Surg 1975; 55(5): 533-544. 18. Chen ZW, Yan W. The study and clinical application of the osteocutaneous flap of fibula. Microsurgery 1983; 4(1): 1116

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Figure 1: Facial Profile showing facial asymmetry on the left side

Figure 5: Exposure of the lesion via extended risdons incision

Figure 9: Histopathologic picture of the resected specimen showing typical ameloblastomatous epithelium lining part of the cystic cavity

Figure 2: Diffuse overgrowth seen in lower left posterior alveolar and vestibular region

Figure 6: Resected specimen after hemimandibulectomy

Figure 10: 18 months post operative radiographic picture showing graft in place, mimicking the lower jaw

Figure 3: OPG showing extent of lesion involving Figure 7: Retrieval and shaping of fibula graft with body, angle and ramus upto the condyle the help of osteotomies and mini plates and screws

Figure 11: 18 months post operative intraoral view showing well adaptation of graft

Figure 4: Lateral view of body of mandible showing the extent of lesion

Figure 8: Post operative radiograph showing free fibula Figure 12: Showing Ackermans grouping of UA as graft secured at the recipient site with plates and screws follows: 1) Luminal, 2) Intraluminal, 3) Intramural

Table 1: Showing Akermans grouping of UA and their interpretation Subgroup 1 1.2 1.2.3 1.3 Interpretation Luminal UA Luminal and Intraluminal UA Luminal, Intraluminal and Intramural UA Luminal and Intramural UA

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