Abstract

Background Adenoid cystic carcinoma (ACC) is a rare and relentless malignancy, which accounts for less than 1% of all head and neck carcinomas. The minor salivary glands of the hard palate, as in this case, are most commonly affected. ACC's characteristic perineural invasion and subsequent proximity to vital neural and vascular structures often result in an unresectable lesion at the time of diagnosis. The frequency of recurrence is high. The goal of this report is to present some of the case findings regarding disease progression and treatment sequelae over a 7-year time span. Materials and Methods Clinical and radiologic findings: A 37-year-old male presented with an ulcerated lesion of the left palate. Pantomography revealed an ill-defined relative radiolucency of the left posterior maxilla, with crestal bone involvement. The inferior border of the maxillary sinus was untraceable. Result Differential or definitive interpretation: Biopsy and subsequent analysis of the lesion demonstrated epithelial islands and a cribriform structure consistent with adenoid cystic carcinoma. Discussion The initial multidetector computed tomography (MDCT) and magnetic resonance imaging (MRI), performed after analysis of the biopsy specimen confirmed the diagnosis, revealed invasion of ACC along the V2 distribution to the foramen rotundum, with involvement of the pterygopalatine fossa. A left partial maxillectomy was performed, followed by radiation therapy. Subsequent findings of disease invasion and treatment sequela demonstrated were skull base radiation necrosis; metastasis to the left frontal lobe after left frontal lobe resection, which was complicated by brain abscess; ventriculitis; hydrocephalus; perforation of the cartilaginous nasal septum; infiltration of the left masticator space, vidian canal, superior orbital fissure, and eustachian tube; and osteoradionecrosis of the greater sphenoid wing, clivus, and left petrous apex. Adenoid cystic carcinoma (ACC) is a rare and relentless malignancy, which accounts for less than 1% of all head and neck carcinomas. The minor salivary glands of the hard palate, as in this case, are most commonly affected. ACC's characteristic perineural invasion and subsequent proximity to vital neural and vascular structures often result in an unresectable lesion at the time of diagnosis. The frequency of recurrence is high. The goal of this report is to present some of the case findings regarding disease progression and treatment sequelae over a 7-year time span. Clinical and radiologic findings: A 37-year-old male presented with an ulcerated lesion of the left palate. Pantomography revealed an ill-defined relative radiolucency of the left posterior maxilla, with crestal bone involvement. The inferior border of the maxillary sinus was untraceable. Differential or definitive interpretation: Biopsy and subsequent analysis of the lesion demonstrated epithelial islands and a cribriform structure consistent with adenoid cystic carcinoma. The initial multidetector computed tomography (MDCT) and magnetic resonance imaging (MRI), performed after analysis of the biopsy specimen confirmed the diagnosis, revealed invasion of ACC along the V2 distribution to the foramen rotundum, with involvement of the pterygopalatine fossa. A left partial maxillectomy was performed, followed by radiation therapy. Subsequent findings of disease invasion and treatment sequela demonstrated were skull base radiation necrosis; metastasis to the left frontal lobe after left frontal lobe resection, which was complicated by brain abscess; ventriculitis; hydrocephalus; perforation of the cartilaginous nasal septum; infiltration of the left masticator space, vidian canal, superior orbital fissure, and eustachian tube; and osteoradionecrosis of the greater sphenoid wing, clivus, and left petrous apex.

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