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1.
J Oral Maxillofac Pathol ; 27(4): 685-692, 2023.
Article in English | MEDLINE | ID: mdl-38304494

ABSTRACT

Introduction: Odontogenic, non-inflammatory maxillofacial cysts and tumours vary greatly in their ability to grow and cause local tissue destruction. Despite their common embryologic origin, the biologic mechanisms responsible for this diverse array of clinical behaviour are largely unknown. Unfortunately, even with accurate tissue diagnosis and appropriate surgical management, these tumours have relatively high recurrence rates. While this may be related to surgical technique, it may also be due to intrinsic tumour biology. SOX2 is differentially expressed in odontogenic cysts and tumours, which has an impact over patient prognosis. This could be related to their diverse cells of origin or stages of histogenesis. SOX2 is expressed in OKC and ameloblastoma, and in this study, we look forward to find altered levels and intensity of SOX2 in the above-mentioned lesions. Aim and Objectives: To profile the expression of SOX2 in odontogenic keratocyst (OKC) and ameloblastomaTo compare the intensity of these lesions, analyse their intrinsic feature and predict their recurrence. Material and Methods: Histopathologically diagnosed cases of OKC and ameloblastoma will be selected (n = 40). Paraffin-embedded, formalin-fixed sections of these lesions will be stained for SOX2 marker using a standard immunohistochemical technique. Positive control will be taken as oral squamous cell carcinoma and negative control will be taken as normal oral mucosa. Results: A comparison between the stained cell types in odontogenic keratocyst and ameloblastoma revealed statistically significant differences. The immunoreactivity scores of SOX2 were analysed in both groups. The results indicated that 45% of OKC cases exhibited strongly positive reactivity, while 65% of ameloblastoma cases were negative. Statistical analysis demonstrated highly significant differences in the frequency of SOX2 expression between the two groups, with a higher frequency of negative expression in ameloblastoma. Conclusion: Stem cell markers have been observed in these lesions, suggesting the acquisition of stem-like properties by tumour cells, which can affect patient prognosis. Specifically, the marker SOX2 shows differential expression in odontogenic cysts and tumours. High expression of SOX2 in OKC indicates the presence of stem cells with significant self-renewal and proliferative properties, potentially signifying neoplastic behaviour. In contrast, weak or absent expression of SOX2 in ameloblastoma suggests different molecular pathways involved in its neoplastic behaviour.

2.
J Coll Physicians Surg Pak ; 29(4): 381-383, 2019 Apr.
Article in English | MEDLINE | ID: mdl-30925967

ABSTRACT

Hereditary ectodermal dysplasia (HED) is a rare genetic disorder chiefly affecting ectodermally derived structures including hair, nails, sweat glands etc. with pathognomic manifestations such as hypotrichosis, hypohidrosis, and hypodontia. Hypohidrotic ectodermal dysplaisa, being the most frequently encountered subtype and HED, being the rare subtype. HED is primarily transmitted through X-linked recessive trait in which the gene is carried by the female and manifested in male. Although rare, this disorder may be seen affecting lot of members of the same family. We hereby report a series of four cases with common classical manifestations accompanied with spoon shaped nails, hyperpigmentation, oligodontia and hypotrichosis. The patients were treated for prosthetic rehabilitation and were asked to wear cool clothing.


Subject(s)
Anodontia/complications , Ectodermal Dysplasia, Hypohidrotic, Autosomal Recessive/diagnosis , Ectodermal Dysplasia/complications , Hypohidrosis/complications , Abnormalities, Multiple , Adolescent , Asian People , Child , Child, Preschool , Female , Humans , India , Male
3.
Iran J Med Sci ; 42(6): 603-606, 2017 Nov.
Article in English | MEDLINE | ID: mdl-29184270

ABSTRACT

Juvenile ossifying fibroma (JOF) is a rare, benign, locally aggressive entity of the extragnathic craniofacial bones with a high tendency towards recurrence. Two distinctive microscopic patterns of juvenile ossifying fibroma have been described: a trabecular juvenile ossifying fibroma (TrJOF) and a psammomatoid juvenile ossifying fibroma (PJOF). Psammomatoid variant is predominantly a craniofacial lesion and occurs rarely in the jaws. The pathognomonic histopathologic feature is the presence of spherical ossicles, which are similar to psammoma bodies. Aneurysmal bone cyst exists as a secondary lesion arising from another osseous condition such as fibrous dysplasia, ossifying fibroma and giant cell granuloma. Very few cases of juvenile psammomatoid ossifying fibroma in association with the secondary aneurysmal bone cyst formation have been reported in the literature. Treatment consists of complete surgical removal; the incomplete excision has been associated with a high local recurrence rate. The authors report a case of recurrence of psammomatoid juvenile ossifying fibroma with aneurysmal bone cyst in an 8-year-old boy emphasizing the point that concomitant occurrence of these locally aggressive lesions requires adequate surgical removal and long-term follow-up.

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