These findings suggest that mast cells may up regulate angiogenesis in OSCC. MCD and MVD may be used as indicators for disease progression.
Apert's syndrome (acrocephalosyndactyly) is a rare congenital disorder characterized by craniosynostosis, midfacial malformation and symmetrical syndactyly of hands and feet. Craniofacial deformities include cone-shaped calvarium, fat forehead, prop-tosis, hypertelorism and short nose with a bulbous tip. Intraoral findings include high arched palate with pseudocleft, maxillary transverse and sagittal hypoplasia with concomitant dental crowding, skeletal and dental anterior open bite and several retained primary teeth. We report one such case of 14-year-old boy having all the classical features of Apert's syndrome with particular emphasis on brief review of genetic features.How to cite this article: Kumar GR, Jyothsna M, Ahmed SB, Lakshmi KRS. Apert's Syndrome. Int J Clin Pediatr Dent 2014;7(1):69-72.
A 35-year-old female patient presented with a chief complaint of painless swelling on the left side of the face since eight months to the Department of Oral Medicine and Radiology, Malla Reddy Dental College for Women, Hyderabad. Telangana, India. Her history revealed that the swelling gradually grew to the present size. Her medical, dental, habitual and family history was not significant. On extra oral examination, inspection showed a swelling measuring approximately 4x3 cm in size, oval in shape, having well defined margins. The colour of the lesion was similar to the normal adjacent skin. The swelling extended superiorly to the line extending from the tragus of the ear to the corner of the mouth. Inferiorly to lower border of the mandible, posteriorly up to the ramus of the mandible and anteriorly up to the corner of the mouth [Table/ Fig-1]. No visible pus discharge was seen. On palpation, the inspectory findings were confirmed and the lesion was bony hard with central region showing variable consistency of hard and soft areas. The lesion was non tender. On intra oral examination, firm, smooth, non-fluctuant swelling was seen extending from 31 to the anterior border of the ramus. The mandibular left first molar and second premolar have been extracted 11 months back. First premolar was carious.Panaromic radiograph revealed an well defined mixed radioopaquelucent multilocular lesion extending from lower left central incisor to the ascending border of ramus [Table/ Fig-2]. Impacted second molar was seen at the inferior border of the mandible. Few septae were arranged in tennis raquet appearance. Root resorption was noticed in relation to 34. Over retained 85 was also seen. Radiological differential diagnosis of central hemangioma, ameloblastoma, odontogenic myxoma was considered.A provisional diagnosis of ameloblastoma and clinical differential diagnosis of dentigerous cyst and odontogenic keratocyst was made. After obtaining patient's consent, enucleation was done and multiple bits of the soft tissue specimen of approximately 4cm X 2cm X 1cm were sent to the Department of Oral Pathology.On microscopic examination, the tissue section revealed plexiform ameloblastoma with a prominent vascular component. It showed anastomosing cords and sheets of odontogenic epithelium in a loosely arranged vascular connective tissue stroma. The epithelium was surrounded by cubodial ameloblast like cells with central stellate reticulum like cells. The vascular component consisted of blood filled spaces of varying sizes lined by endothelial cells. At focal areas vascular spaces were large and compressing the odontogenic strands . A diagnosis of Hemangiomatous Amelobalstoma was made.Partial hemimandibulectomy was planned followed by immediate reconstruction with a nonvascularised illiac graft. But the patient was not cooperative and reluctant for surgical treatment and hence the procedure could not be carried out. The patient was recalled for follow up visits but did not return to the department. DisCussionAmeloblastoma is one of t...
The adenoid cystic carcinoma is a relatively rare epithelial tumor of the major and minor salivary glands, accounting for about 1% of all malignant tumor of the oral and maxillofacial regions. Peak incidence occurs between the 5th and 6th decades of life. The clinical and pathological findings typical of this tumor include slow growth, peri-neural invasion, multiple local recurrences and distant metastasis. Herein, we report a case of adenoid cystic carcinoma of oropharynx with unusual clinical presentation. The diagnosis of this case and importance of cytology in diagnosing such cases is discussed.
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