Neural tumors located centrally in jaw bones are relatively rare compared with soft tissue neurofibromas. Less than 50 cases have been reported in the literature with a predilection for mandible. This article aims to elucidate a unique case of intraosseous neurofibroma of mandible in a 62-year-old edentulous female patient associated with facial asymmetry due to the swelling extending from the right body of mandible to left body of mandible. The uniqueness of this case is related to the age and extensiveness of this lesion. A review of clinical, radiographic, histological, and immunohistochemical features, and the surgical management pertaining to this case are discussed along with a review of the literature.
Background Desmoplastic Ameloblastoma (DA) is a rare, true neoplasm of jaws with reported incidence of 4-13% among other variants of Ameloblastoma, however this appears distinct than the classic Ameloblastoma in anatomical distribution and clinical presentation. This is often mistaken as a fibro-osseous lesion because of its similar radiological appearance. Material and Methods To describe the clinical, radiographic and histopathological characteristics through a series of new cases of histologically proven DA including a case of an exceptionally large, recurrent lesion along with retrospective analysis of cases from literature available for an improved understanding of the behaviour and prognosis of DA. A total of 50 cases were analysed for the anatomical distribution, radiographic presentation and management. Out of the 50 cases, 47 cases were from the English literature reported from 2011 to 2019 and 3 were new cases. Results DA showed a slight male predilection (male: female=1.17:1) with a predominance in the fourth and fifth decade of life. Mandibular involvement (52%) was more commonly seen with a marked tendency for the anterior region. Radiographically, most of the lesions presented mixed radiopacity with radiolucency(80%) and root displacement was observed in only 70.27 % cases. Recurrence rate of 26 .47 % was observed. Cases treated with resection resulted in lesser recurrence as compared to those treated with enucleation and curettage. Conclusions DA is distinguished by a peculiar display of clinicalopathological parameters. DA has tendency of local disposition and propensity of recurrence, which thus necessitates its aggressive management. It is not possible to conclude or report on the aggressive/recurrent nature and appropriate treatment modality for DA due to inadequate follow-up results. Key words: Desmoplastic ameloblastoma, mixed radiopaque – radiolucent, odontogenic tumors, recurrence, enucleation, curettage, resection.
Osteopetrosis (OP) is a group of rare genetic bone disorders. Osteoclast-poor form of osteopetrosis is much rarer in humans and represents a small percentage of the total cases of autosomal recessive osteopetrosis presenting with impaired bone remodeling due to defective osteoclastic activity and is characterized by distinctive increase in bone density and high bone fragility. Reduction in marrow spaces with decreased vasculature to the bone owing to increased bone mass makes the bones vulnerable for varied infections resulting in osteomyelitis. This case report discusses challenges in management of recalcitrant osteomyelitis of mandible developed as a complication in an 8-year-old girl child identified with rare, dual heterozygous mutations in RANKL ( TNFSF11 ) gene and COL5A1 gene with uncertain significance responsible for osteoclast-poor osteopetrosis and Classic Ehlers-Danlos, respectively. How to cite this article Sharma A, Ingole SN, Deshpande MD, et al. A Rare Case of Osteoclast-poor Osteopetrosis (RANKL Mutation) with Recurrent Osteomyelitis of Mandible: A Case Report. Int J Clin Pediatr Dent 2020;13(6):717–721.
Rationale: Presenting a rare case report of a giant multilobular lipoma in submandibular and submental spaces of anterior and lateral aspect of neck. Patient's Concerns: The patient's main concern was persistent diffuse swelling in right lower face and neck region for 5–6 years. Diagnosis: The lesion was diagnosed as multiple septate lipoma measuring 11.5 cm × 10.5 cm × 6.5 cm involving submandibular region and anterior triangle of the right neck following fine-needle aspiration cytology and radiological imaging. Treatment: Extraorally complete surgical excision was carried out through submandibular approach under general anesthesia. Outcomes: The patient's postoperative recovery was uneventful. The patient was followed up on a monthly basis for 6 months. No recurrence was observed. The patient was satisfied with the treatment. Lessons: Lipomas should be considered as a rare differential diagnosis for anterior neck swelling. Biopsy is not necessary to confirm the diagnosis. Surgical excision remains the mainstay of treatment following final diagnosis through imaging modalities.
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