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Squamous cell carcinoma (Marjolin's ulcer) in an orocutaneous fistula of a large mandibular ameloblastoma: a case report
© Nthumba; licensee BioMed Central Ltd. 2011
Received: 13 August 2010
Accepted: 19 August 2011
Published: 19 August 2011
Ameloblastomas are rare lesions constituting 1% of all jaw tumors. Oral squamous cell carcinomas are common lesions; these constitute about 90% of all oral cancers. Concurrent tumors consisting of ameloblastoma and squamous cell carcinoma are extremely rare.
This case report describes a 35-year-old African man who presented with a large mandibular tumor with an orocutaneous fistula that was found to be an ameloblastoma on histopathological examination, with concurrent squamous cell carcinoma histology within the fistula. This presentation was consistent with a Marjolin's ulcer within an ameloblastoma.
Ameloblastomas and Marjolin's ulcers require different management strategies. Careful histopathological examination of surgical specimens is key to patient outcome, as treatment of these patients depends on an accurate diagnosis.
Ameloblastoma is a benign but locally aggressive odontogenic tumor of the mandible and maxilla. It represents about 1% of all jaw tumors, and 80% of ameloblastomas occur in the mandible . Ameloblastomas grow slowly and, if neglected, may grow to enormous sizes, causing severe facial deformities and functional impairment [1, 2]. Surgical resection with wide margins is the treatment of choice [3, 4]. Radiological investigations are useful, both as aids to diagnosis and for planning surgery, an orthopantogram may reveal a "soap bubble" appearance, and an axial computed tomography (CT) scan will reveal the extent of bony and/or soft tissue involvement. Ameloblastomas may rarely degenerate into ameloblastic carcinomas.
Squamous cell carcinoma, on the other hand, is the commonest malignancy of the oral cavity, constituting about 90% of all oral cancers . Most squamous cell carcinomas found in the jaws originate from lesions within the oral cavity; however, primary intra-osseous carcinoma may arise within the jaw, most likely developing from residues of odontogenic epithelium . Surgical excision of resectable lesions is the mainstay of treatment. The simultaneous occurrence of squamous cell carcinoma and ameloblastoma has previously been reported [6–9]. Herein the author presents an unusual case of squamous cell carcinoma that developed in an orocutaneous fistula through a large ameloblastoma of the mandible.
Tumors may grow to a size that outstrips their blood supply, leading to tumor necrosis and ulceration. If the tumor occurs in an anatomical area with two apposing epithelialized surfaces such as the oral cavity and skin, it is feasible that tumor necrosis and ulceration into both epithelia might lead to the formation of a fistula. Repeated attempts at epithelialization of the tract, with constant irritation by saliva, fluids, and oral bacteria, may lead to malignant degeneration into squamous cell carcinoma, also known as "Marjolin's ulcer." Marjolin's ulcers occur in scar tissue, classically in burn scars, but have also been described in numerous other conditions, including chronic sinuses and fistulas, such as those that occur in chronic osteomyelitis and urinary fistulas [10, 11]. The fact that squamous cell carcinoma was found only along the orocutaneous fistula in this patient provides a strong basis for the hypothesis that chronic inflammation along the fistula over time led to malignant degeneration and hence to Marjolin's ulcer (Figure 1). Because of the poor prognosis associated with Marjolin's ulcers [10, 11], the patient was encouraged to return for regular follow-up visits. His returns for follow-up were erratic, with no visits recorded between four months and twelve months post-operatively. The patient was noted to have gained weight, with no evidence of local or distant metastasis noted at the thirteen-month follow-up examination (Figure 5).
Demographics of patients reported with simultaneous ameloblastoma and squamous cell carcinoma of the mandible and/or maxilla
Hamakawa et al. 
Chemotherapy followed by mandibulectomy and neck dissection
No recurrence at four years
Tucker et al. 
Right and left mandibles
Ueta et al. 
Serial excisions leading to right mandibulectomy
Lung metastasis at one year
Nishimura et al. 
Radiotherapy for SCC followed by partial maxillectomy for ameloblastoma
No recurrence at 33 months
No recurrence at last visit 13 months after surgery
The occurrence of concurrent ameloblastoma and squamous cell carcinoma of the jaws, though previously reported, is extremely rare. Because the two lesions require different management strategies, careful histopathological examination of tumor specimens is crucial to surgical management and ultimately to clinical outcome. Marjolin's ulcers have not been previously reported to occur in tumors. This case report indicates that they can occur and that close follow-up, even in resource-poor environments, is important, because Marjolin's ulcers are generally associated with poor outcomes.
Written informed consent was obtained from the patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.
Dr Jerry M Grey, pathologist, is thanked for help with the slides.
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