Gorlin-Goltz Syndrome: A Case Series.

Gorlin-Goltz syndrome (GGS) among Indians is rarely reported. Since 1960, only 38 cases having 48 patients of Gorlin-Goltz syndrome have been identified in the Indian population. It is crucial to diagnose this illness early because it can be connected to a malignant lesion like fibrosarcoma, leiomyosarcoma or rhabdomyosarcoma. The four patients in this case series were identified and treated in our department between 2019 and 2023. The average patient age was around 20 years old. Jaw swelling and tooth movement were the two most typical presenting concerns. Odontogenic keratocysts (100%), palmer pits (100%), plantar pits (50%), calcification of falx cerebri (50%), and rib abnormalities (50%), were the most prevalent characteristics. None of the patients had basal cell cancer, cleft lip, or medulloblastoma. Multiple odontogenic keratocysts were present in three cases, whereas a single odontogenic keratocyst (OKC) was seen in one patient. Patients were managed with either marsupialization or enucleation, depending on the size of the cyst. Two cases with a large cyst size were marsupialized by using a modified obturator. Two cases with small cysts were managed with enucleation of the cyst followed by chemical cauterization. Recurrence was seen in two cases. In one patient, we noticed the formation of a new cyst. A GGS diagnosis can be made by having a systemic evaluation of the patient. A thorough examination of the patient should be performed in every histopathology-diagnosed case of OKC. This will help to miss the syndromic cases. The treatment part should be conservative, like marsupialization with an obturator in a large cyst. The obturator helps maintain patient hygiene and prevents regular visits for changing dressings. Small-sized cysts can be managed with enucleation and chemical cauterization. Radical resection should be avoided.

Pleomorphic Adenoma of the Palate With an Atypical Malignant Presentation: A Case Report.

A pleomorphic adenoma is a mixed salivary gland tumor. The parotid gland is the most common site. The intraoral palate is the most common site due to the abundance of accessory salivary glands in the palatal area. It has a very slow growth rate and is usually painless. Consequently, patients often have a lengthy history of presentation. Herein, we report a case of pleomorphic adenoma of the palate in a 53-year-old male patient. The mass was 5 cm by 4 cm in size. The growth rate was rapid, and it attained a large size in just nine months. On clinical examination, the overlying mucosa was ulcerated. On general examination, lymphadenopathy of the right side level 1 b lymph node was found. These clinical findings were consistent with malignancy. However, the histopathological report negated the clinical findings of malignant salivary gland tumors. The tumor was managed with a wide local excision of the tumor with a 1 cm clear margin. The postoperative course was uneventful. No recurrence was seen after two years of follow-up. A thorough cytological or histological examination is a prerequisite to defining the malignant nature of the lesion.

Solitary Neurofibroma of the Maxillary Soft Tissue Causing Bone Destruction: A Rare Presentation.

Neurofibroma is a benign tumor of the peripheral nerve. It usually presents as multiple tumors and as a part of von Recklinghausen's disease. However, rarely, it can occur in solitary form. The clinical diagnosis of solitary neurofibroma poses a challenge to clinicians as a plethora of benign and malignant lesions of the oral cavity have similar clinical presentations. We present a rare case of solitary neurofibroma of the maxillary soft tissue. A systemic clinical examination was performed to rule out neurofibromatosis. Computed tomography showed displaced teeth and bone destruction. Magnetic resonance imaging showed an ill-defined T2 hyperintense lesion involving the posterior aspect of the tooth-bearing alveolar process of the maxilla. The diagnosis of neurofibroma was confirmed by histological analysis and immunohistochemical studies. The tumor was successfully managed with excision. Three years of follow-up showed no recurrence. Solitary neurofibroma of the oral cavity is a very rare presentation. Neurofibroma in the maxilla with bone destruction is reported for the first time in the literature. This article will add to the current literature due to the atypical location and presentation of the tumor.

Rhino-Orbital Cerebral Mucormycosis Causing Temporomandibular Joint Ankylosis: A Case Series of Two Patients.

Temporomandibular joint ankylosis is caused by trauma, infection, autoimmune, inflammatory joint diseases, and several other minor causes. Mucormycosis causing temporomandibular joint ankylosis has not been reported. We report two cases of temporomandibular joint ankylosis caused by mucormycosis from 2018 to 2022. In both cases, the infection started in the maxilla, then progressed to orbit. After that, involvement of the mastoid process, styloid process, and base of the skull was observed in the first case, while in the second case, there was the involvement of the base of the skull and mandibular ramus. As the temporomandibular joint (TMJ) components are contiguous to the base of the skull, it got affected causing temporomandibular joint ankylosis. Mucormycosis was diagnosed by KOH mount. The smear examination showed aseptate hyphae at 900. Histopathology examination further confirmed mucormycosis. Glycemic control was done by infusing Insulin (both Lantus and regular). The case was managed with aggressive debridement and interpositional arthroplasty with a buccal fat pad. Liposomal amphotericin infusion was also started pre-operatively and continued in the post-operative phase. After 4 years of follow-up, the patient is well and had adequate mouth opening. Mucormycosis infection affecting the TMJ has been reported in the literature. However, this is the first report of a mucormycosis infection resulting in TMJ ankylosis in the literature. The infection should be aggressively managed. Reversals of an immunocompromised state, aggressive surgical management, and antifungal medication are the key factors for the success of the deadly fungal infection.

Mandibular Mucormycosis: A Report of Four Cases and a Discussion on Their Management.

Mucormycosis of the mandible (MOM) is a rare fungal infection, and only 23 cases had been reported during the last 50 years worldwide, from seven different countries. Most of the cases were reported in India (n=8, 34%), followed by the United States (n=5, 22%). It is usually associated with an immunocompromised state and generally occurs after tooth extraction. Radiographically, it presents with the characteristic sign of osteomyelitis. Most of the previous case reports/series on MOM described successful outcomes with the resection of the involved segment. However, our experience in managing these cases was quite different and it was observed that resection is seldom required. It was seen that MOM rarely causes cortical perforation. One of the probable reasons is the thicker cortical bone and well-confined boundary of the mandible. Another reason could be the fulminating nature of the disease that leads it to rapidly spread in less resistant medullary bone before perforating cortical bone. During surgery, a clear line was seen separating necrotic medullary bone from healthy cortical bone. The thicker cortical bone of the mandible was found to be resistant to fungal invasion; however, the medullary bone was rapidly invaded. Therefore, the healthy cortical bone could be saved. The preservation of the cortical parts thus helps in maintaining the continuity of the bone. Surgical curettage of necrotic medullary bone is usually the optimal method to manage MOM affecting the mandibular body or ramus region.