Xanthoma of the Jaw Bones: Cases Series and Review of Literature.

BACKGROUND: Intraosseous xanthomas are rare benign lesions sometimes associated with excess lipid production. Xanthoma of the jaw bones (XJB) was first reported in 1964, and fewer than 50 cases have been reported in the English literature to date. The etiopathogenesis of XJB is highly suggestive of a reactive process or a metabolic condition. METHOD: Seven cases of XJBs were retrieved from the archives of 4 oral and maxillofacial pathology services. Clinical, radiographic and histopathologic features of all these cases were retrospectively analyzed. Immunohistochemical (IHC) stains for S100 and CD68 were performed. RESULTS: All seven cases involved the mandible. Patients' age ranged between 13 and 69 years with an evenly distributed female to male ratio. One patient had a medical history of hyperlipidemia, but the medical and dental histories of the others were unremarkable. For most cases, XJB was an incidental finding discovered during routine radiographic examination. Swelling and cortical expansion were noted in a few cases. Radiographically, cases typically presented as either well-defined multilocular or unilocular lesions, which were either radiolucent or mixed radiolucent/radiopaque. All the lesions were treated with surgical curettage and no recurrence was observed during subsequent follow-ups. Each of the seven cases exhibited sheets of foamy macrophages. The diagnosis is established by exclusion of entities with overlapping microscopic features and involved correlation with the clinical, histological, radiographic and IHC profiles. Immunohistochemically, all the cases expressed diffuse positivity for CD68 and were negative for S100. CONCLUSION: XJB is a rare lesion of unknown etiology, which may mimic other benign or reactive jaw lesions. Due to its rarity and the potential diagnostic challenges it presents, clinicians must remain vigilant and consider CXJ in their differential when assessing radiolucent jaw anomalies.

Secretory Carcinoma of the Oral Cavity: A Retrospective Case Series with Review of Literature.

Secretory carcinoma (SC) is an uncommon salivary gland neoplasm of the oral cavity that microscopically may mimic acinic cell carcinoma (ACC) and mucoepidermoid carcinoma (MEC). This study describes a series of SC in minor glands with a literature review. We performed a retrospective search for oral SC, within the archives of the University of Florida, Oral Pathology and Surgical Pathology Biopsy services from 2010 to 2018. A total of 10 SCs were identified in the oral and maxillofacial region, four of which were in the minor salivary glands. The demographic, clinical, histological, and molecular findings were aggregated for all 4 cases. Patient age varied from 30 to 60 years, with an average of 45 years. Two cases each were in female and male patients. Two cases presented on the labial mucosa, and one each on the hard and soft palate. Immunohistochemical (IHC) staining showed mammaglobin positivity in all cases, GATA3 positivity in two cases, S100 positivity in three cases, and SOX10 positivity in only one case. Fluorescence in situ hybridization demonstrated positivity for ETV6-NTRK3 fusion in 4 cases. Although oral SC is rare, pathologists should be aware of the histologic overlap between the SC and other salivary gland neoplasms such as ACC and MEC. A judicious application of IHC staining would aid in diagnosis. SC should be considered in the differential diagnosis for intraoral salivary gland tumors.

Comparison of outcomes between 2 week versus 4 week stenting in pediatric pyeloplasty-A single centre observational study.

OBJECTIVE: To compare the surgical outcomes, improvement in renal function and complications between early stent removal (2 weeks) and late stent removal (4 weeks) after pediatric open pyeloplasty. METHODS: A total of 72 open pyeloplasty were included in the study. Forty-three underwent late stent removal (Group 1) and 29 underwent early stent removal (Group 2). Pre-operative and post-operative follow-up data were compared to see the effect of early stent removal on the postoperative drainage pattern at 6 months after surgery and improvement in split function of affected kidney. The complications between the two groups were also compared. RESULTS: Both the groups were matched with respect to age, sex, side and antero-posterior diameter of pelvis. Pre-operative mean split function in Group 1 was 42% (26%-54%) while it was 39% (19%-42%) in Group 2 (p=0.37). Postoperative improvement in drainage pattern was seen in 69 out of 72 (96%) patients, 41 out of 43 (95%) in Group 1 and 28 out of 29 (97%) in Group 2. Improvement in split function occurred in 35 of 38 (97%) in Group 1 and 23 of 26 (88%) patients in Group 2 (p=0.51). Complications were seen in nine out of 72 (12.5%) patients. Incidence of complication in Group 1 was 16% (7/43) and Group 2 was 7% (2/29), and relative risk was 2.36. CONCLUSION: A shorter duration of double J stenting is as effective as a longer stenting period in terms of surgical success outcomes and improvement in split renal function along with a decreased risk of stent related complications.

Bladder exstrophy-epispadias complex and the role of methylenetetrahydrofolate reductase C677T polymorphism: A case control study.

PURPOSE: The Bladder Exstrophy-Epispadias Complex (BEEC) is the most serious form of midline abdominal malformation. The etiology of BEEC is unknown and is thought to be multifactorial. Methylenetetrahydrofolate reductase (MTHFR) polymorphism C677T is strongly associated with other midline abnormalities such as neural tube defects. No proper case-control study existed comparing MTHFR polymorphism with BEEC. We sought to find an association with MTHFR polymorphism and patients with bladder exstrophy (BE). MATERIALS AND METHODS: The design of the study was a case-control study, involving 50 children with BEEC and 50 normal healthy school children. Genetic analysis for MTHFR 677 polymorphism was carried out after DNA extraction and polymerase chain reaction amplification. Epidemiological analysis was done by using the birth defect questionnaire on parents of BEEC. RESULTS: Forty-two classical BE, two cloacal exstrophies (CE), four epispadias, and two exstrophy variant patients were a part of this study. Severe variety of BE had a significant association with C667T MTHFR polymorphism as compared to the normal control population (P = 0.01). CONCLUSION: C677T MTHFR polymorphism has a strong association with severe variety (CE) of BEEC occurrence.

Correlation between functional outcomes and postoperative pelvic magnetic resonance imaging in children with anorectal malformation.

BACKGROUND: Though the outcomes in operated children with anorectal malformation (ARM) have greatly improved, postoperative soiling and constipation remain major issues. Among the various factors described for poor outcomes; misplaced bowel, hypoplastic sphincters and obtuse anorectal angle bear special mention. The aim of this study was to compare the stooling outcomes, type of anomalies and surgical procedure with postoperative pelvic magnetic resonance imaging (MRI). MATERIALS AND METHODS: This was a cross-sectional study involving operated children of ARM who had at least 2 years of follow-up, and who were at least 3 years of age. The subtypes of ARM, surgical procedures, and functional outcomes were documented using the Krickenbeck classification. All children were subjected to a pelvic MRI. RESULTS: Thirty-three eligible children were part of this study. Twenty-two patients underwent posterior sagittal anorectoplasty, seven patients underwent abdominoperineal pull-through (APPT) and four patients underwent perineal operations. Local abnormalities were present in 66% patients, and 34% had abnormalities of the spine detected on MRI. Poorer stooling outcomes were twice as common in children with local pelvic MRI abnormalities as compared to asymptomatic children. The highest incidence of local abnormalities were seen in patients treated with APPT (P = 0.0001). No significant difference in the pelvic MRI was seen among children who were constipated and those who had soiling. CONCLUSION: MRI is a useful imaging modality in operated children of ARM with poor stooling outcomes. Local abnormalities were the most common in children undergoing abdominoperineal pull-through procedure.

Annular pancreas, type I choledochal cyst and malrotation in a low-birth weight newborn: A case report.

We present a low-birth weight newborn who presented with bilious vomit and had a "double bubble" sign on plain abdominal X-ray. Intraoperatively, we found annular pancreas with malrotation and type I choledochal cyst. To the best of our knowledge, this is the first reported case with a combination of these anomalies.