Oral Juvenile Hyaline Fibromatosis: A Rare Entity

Juvenilna hijalina fibomatoza iznimno je rijedak poremećaj kod dojenčadi i djece, a javlja se prema zakonima autosomno recesivnog nasljeđivanja. Izgleda poput multiple kožne ili subkutane tumorne tvorbe, sporo se razvija i češća je u području glave i vrata te gornjeg dijela trupa. Često je povezana s gingivnom hipertrofijom, teškom fleksularnom kontrakturom udova i koštanom lezijom. Nema mentalne retardacije. Histološki se te lezije sastoje od obilne eozinofilne osnovne tvari s neravnomjerno raspršenim fibroblastima. Ekscidirane lezije u ranim stadijima bogatije su stanicama. Točno podrijetlo eozine hijaline tvari nije poznato. Nedavno je pronađen defekt kromosoma 4q21 povezan s lokusom gena – 2 za kapilarnu morfogenezu. Diferencijalna dijagnoza za juvenilnu hijalinu fibromatozu uključuje i infantilnu sistemsku hijalinozu, za koju se zna da je alelna. Trenutačno nema široko prihvaćene učinkovite terapije ni za juvenilnu hijalinu fibromatozu ni za infantilnu sistemsku hijalinozu. Juvenilna hijalina fibromatoza i infantilna sistemska hijalinoza ponekad se teško razlikuju jer su vrlo slične. Mi izvještavamo o slučaju juvenilne hijaline fibromatoze kod 10-godišnje djevojčice s pretežno gingivalnom hiperplazijom.Juvenile hyaline fibromatosis is an exceedingly rare disorder of infants and children which appears to have autosomal recessive inheritance. It is characterized by multiple, slowly growing dermal or subcutaneous tumors, especially in the head and neck region and upper trunk, often associated with gingival hypertrophy, severe flexural limb contractures and bone lesions. There is no mental retardation. Histologically, these lesions are composed of copious eosinophilic, homogenous ground substance with unevenly dispersed fibroblasts. Lesions excised in early stages are more cellular. The precise nature of the eosinophilic hyaline material is not known. Recently, a defect on chromosome 4q21 associated with the locus of the capillary morphogenesis gene – 2, has been demonstrated. The differential diagnosis of juvenile hyaline fibromatosis includes infantile systemic hyalinosis, which is now known to be allelic. Currently, no widely accepted effective treatment exists for juvenile hyaline fibromatosis or infantile systemic hyalinosis. Infantile systemic hyalinosis and juvenile hyaline fibromatosis are sometimes difficult to separate since they show significant overlap. We report one such unusual case of juvenile hyaline fibromatosis in a 10 year old female presenting mainly with gingival hyperplasia

Recurrent bilateral cystic hygroma of the submandibular space in an adult patient

Cystic hygroma is an uncommon type of benign neoplasm of lymphangioma commonly seen in children, rarely reported in adults. Most of the cases occur in head and neck region. The etiology and pathogenesis of this entity are still unclear. Clinically, they present as painless, soft, compressible, progressively enlarging mass. Various treatment methods have been described, but surgery remains the treatment of choice. Incomplete excision results in recurrence. We present a unique case of bilateral submandibular area cystic hygroma in an adult female patient with a history of recurrence twice

Lymphangioma of the buccal mucosa: A case report with a literature review

Lymphangioma is a benign, hamartomatous tumor of the lymphatic system. It is usually found in the head and neck region and is widely regarded as a developmental lesion rather than a true neoplasia. Most lymphangiomas are present at birth. In head and neck area, the most common location is the submandibular region, followed by the parotid gland. When lymphangioma occurs in the oral cavity, the anterior two third of the tongue is most commonly affected. The occurrence of lymphangioma in other parts of the oral cavity is very uncommon. Here, we report an unusual case of lymphangioma of buccal mucosa in a 45-year-old female

Histomorphometric analysis of nuclear and cellular volumetric alterations in oral lichen planus, lichenoid lesions and normal oral mucosa using image analysis software

Introduction: Lichen planus is a chronic inflammatory mucocutaneous disease that clinically and histologically resembles lichenoid lesions, although the latter has a different etiology. Though criteria have been suggested for differentiating oral lichen planus from lichenoid lesions, confusion still prevails. Aims: To study the cellular and nuclear volumetric features in the epithelium of normal mucosa, lichen planus, and lichenoid lesions to determine variations if any. Materials and Methods: A retrospective study was done on 25 histologically diagnosed cases each of oral lichen planus, oral lichenoid lesions, and normal oral mucosa. Cellular and nuclear morphometric measurements were assessed on hematoxylin and eosin sections using image analysis software. Statistical Analysis: Analysis of variance test (ANOVA) and Tukey′s post-hoc test. Results: The basal cells of oral lichen planus showed a significant increase in the mean nuclear and cellular areas, and in nuclear volume; there was a significant decrease in the nuclear-cytoplasmic ratio as compared to normal mucosa. The suprabasal cells showed a significant increase in nuclear and cellular areas, nuclear diameter, and nuclear and cellular volumes as compared to normal mucosa. The basal cells of oral lichenoid lesions showed significant difference in the mean cellular area and the mean nuclear-cytoplasmic ratio as compared to normal mucosa, whereas the suprabasal cells differed significantly from normal mucosa in the mean nuclear area and the nuclear and cellular volumes. Conclusions: Morphometry can differentiate lesions of oral lichen planus and oral lichenoid lesions from normal oral mucosa. Thus, morphometry may serve to discriminate between normal and premalignant lichen planus and lichenoid lesions. These lesions might have a high risk for malignant transformation and may behave in a similar manner with respect to malignant transformation

Culture-based identification of pigmented Porphyromonas and Prevotella species in primary endodontic infections

Background. The most common species isolated from primary endodontic infections are black-pigmented bacteria. These species are implicated in apical abscess formation due to their proteolytic activity and are fastidious in nature. Therefore, the present study was carried out to evaluate the presence and identification of various pigmented Porphyromonas and Prevotella species in the infected root canal through culture-based techniques. Methods. Thirty-one patients with primary endodontic infections were selected. Using sterile paper points, samples were collected from the root canals after access opening and prior to obturation, which were cultured using blood and kanamycin blood agar. Subsequently, biochemical test was used to identify the species and the results were analyzed using percentage comparison analysis, McNemar and chi-squared tests, Wilcoxon match pair test and paired t-test. Results. Out of 31 samples 26 were positive for black-pigmented organisms; the predominantly isolated species were Prevotella followed by Porphyromonas. In Porphyromonas only P. gingivalis was isolated. One of the interesting features was isolation of P. gingivalis through culture, which is otherwise very difficult to isolate through culture. Conclusion. The presence of Prevotella and Porphyromonas species suggests that a significant role is played by these organisms in the pathogenesis of endodontic infections

Development of Automated Risk Stratification for Sporadic Odontogenic Keratocyst Whole Slide Images with an Attention-Based Image Sequence Analyzer

(1) Background: The categorization of recurrent and non-recurrent odontogenic keratocyst is complex and challenging for both clinicians and pathologists. What sets this cyst apart is its aggressive nature and high likelihood of recurrence. Despite identifying various predictive clinical/radiological/histopathological parameters, clinicians still face difficulties in therapeutic management due to its inherent aggressive nature. This research aims to build a pipeline system that accurately detects recurring and non-recurring OKC. (2) Objective: To automate the risk stratification of OKCs as recurring or non-recurring based on whole slide images (WSIs) using an attention-based image sequence analyzer (ABISA). (3) Materials and methods: The presented architecture combines transformer-based self-attention mechanisms with sequential modeling using LSTM (long short-term memory) to predict the class label. This architecture leverages self-attention to capture spatial dependencies in image patches and LSTM to capture sequential dependencies across patches or frames, making it suitable for this image analysis. These two powerful combinations were integrated and applied on a custom dataset of 48 labeled WSIs (508 tiled images) generated from the highest zoom level WSI. (4) Results: The proposed ABISA algorithm attained 0.98, 1.0, and 0.98 testing accuracy, recall, and area under the curve, respectively, whereas VGG16, VGG19, and Inception V3, standard vision transformer attained testing accuracies of 0.80, 0.73, 0.82, 0.91, respectively. ABISA used 58% fewer trainable parameters than the standard vision transformer. (5) Conclusions: The proposed novel ABISA algorithm was integrated into a risk stratification pipeline to automate the detection of recurring OKC significantly faster, thus allowing the pathologist to define risk stratification faster