Angina bullosa hemorrhagica is a relatively rare lesion of uncertain etiology affecting the oral cavity and the oropharynx characterized by blood-filled blisters or bullae of varying size. The lesions are sudden in onset, usually asymptomatic and bullae often rupture leaving ulcers which heal without scarring. No specific treatment is usually advised since it is a self-limiting lesion. Rarely, larger lesions in the oropharynx may cause air obstruction and require immediate attention. The clinical presentation of this lesion may cause apprehension to the patient and diagnostic challenge to the clinician since they clinically mimic other serious disorders such as blood disorders and vesiculobullous lesions. An awareness among clinicians is needed to prevent misdiagnosis and unnecessary diagnostic procedures of this distinct entity.

Binder's syndrome, a rare congenital malformation of nasomaxillary complex, first described in 1962, has a hexad of characteristic clinical and radiographic features consisting of arhinoid face, intermaxillary hypoplasia with malocclusion, abnormal position of nasal bones, atrophy of nasal mucosa, reduced or absent anterior nasal spine and hypoplastic/absent frontal sinus. The typical facies due to mid-face hypoplasia may also be accompanied by other midline malformations such as cleft palate, spinal, skeletal and cardiac abnormalities. It is usually sporadic, of unknown etiology although various environmental and genetic mechanisms are implicated due to few familial cases predominantly in the Swedish population. A case of inherited Binder's syndrome is presented in an Indian female patient with an unusual finding of ankyloglossia (AG). The development of the anterior nasal spine and AG are chronologically related as they both occur during the 5^th–6^th weeks of gestation. The possible etiopathogenetic mechanisms for this rare association are reviewed.

Ectodermal dysplasia represents a group of inherited conditions in which two or more ectodermally derived anatomical structures fail to develop resulting in most notably anhidrosis/hypohidrosis, hypotrichosis and hypodontia. It is a xlinked recessive disorder with male predominance. We report a classical case in a 17-year-old male with emphasis on review of literature and latest updates.

Migratory stomatitis (MS) is an uncommon inflammatory condition with unclarified etiology, which despite its benign nature, may raise concerns for patients and diagnostic difficulties for professionals. This case report aims to describe the clinical features of a patient who presented with MS in conjunction with benign migratory glossitis (BMG), and its diagnostic process and management. The patient, a 25-year-old man, sought diagnosis of an oral condition, with cyclic behavior, which had been causing him great discomfort for a year. The patient presented erythematous patches on his lower lips and right side of the buccal mucosa, surrounded by a slightly elevated halo with a concomitant classical picture of BMG. After analysis of his entire symptomatology, the diagnosis of MS associated with BMG was concluded. The patient received clear explanations and symptomatic treatment. The diagnosis of MS may be challenging, even to oral medicine practitioners, especially if it occurs alone. MS with concurrent manifestation of BMG may make the conditions easier to diagnose, but it does not exclude the need to apply a complete process of differential diagnosis to rule out other similar possibilities.

Dental anomalies in human dentition consist of a considerable variation in size, number, position, shape and structure of the tooth. Microdontia is defined as a condition in which teeth are abnormally smaller in size. Hypodontia is defined as developmental absence of 1–5 teeth excluding third molars. Simultaneous occurrence of multiple dental anomalies is most commonly seen in patients with some abnormality or syndrome; it can also occur in nonsyndromic patients. This case report describes about an unusual simultaneous occurrence of dental anomalies such as localized microdontia of primary teeth and hypodontia in a nonsyndromic 10-year-old male patient. Simultaneous occurrence of multiple dental anomalies in a nonsyndromic patient is rare. As the treatment of such cases involves multiple phases, identification and proper timely management is important to help the patient lead a better quality life.

Adenoid cystic carcinoma (ADCC) is an uncommon tumor of head and neck, whose clinical patterns are characterized by slow growth, perineural invasion and distant metastasis. Cribriform, tubular and solid are the three recognized histopathological patterns which are seen in ADCC. We report a case of ADCC involving palate with an update on the current understanding of its clinical behavior, molecular biology, pathogenesis, histopathological aspect, treatment and prognosis.

Mucoepidermoid carcinoma (MEC) is known to be a relatively common neoplasm of the major and minor salivary glands that can secondarily involve skin. The clinical manifestations, diagnostic cytology and histopathology of MEC presenting as a clinically benign periauricular cystic nodule have been analyzed. The challenge of accurate diagnosis can be illustrated by the fact that initially, on cytology, this tumor was misdiagnosed as a benign epidermal inclusion cyst due to the presence of mucin-filled cells which were misinterpreted as cyst macrophages. This case report emphasizes the need to include parotid tumors in the differential diagnosis of all periauricular cyst-like expansions. We also briefly discuss the reasons for false-positive cytology in this case. Delay in accurate diagnosis may result in larger surgical procedures, such as radical neck dissection, that may otherwise be avoided.

Epithelial myoepithelial carcinoma (EMC) is an uncommon low-to-intermediate grade salivary gland malignancy that accounts for 1% of all tumors arising in salivary glands. About 80% of these tumors affect the parotid gland. These lesions either arise de novo or from existing pleomorphic adenoma (PA). Histologically, these tumors reveal a biphasic cell population with inner ductal epithelial cells and peripheral myoepithelial cells. There are many histologic variants of EMC, but sebaceous, verocay-like differentiation and high-grade transformation is very rarely reported. This article describes a 48-year-old female patient diagnosed with EMC ex PA with unique histologic differentiation.

A 19-year-old male patient reported to dental OPD of our institution with a swelling in the posterior part of the mandible on the left side. The patient gave a history of gradual increase in the size of swelling for 7 years. The patient also had a radiograph and histopathology slides from his previous dental visit at another facility. The radiograph revealed a well-circumscribed radiolucency with an impacted tooth (38). Histopathology slides showed features of an ameloblastic fibroma (AF). The patient had deferred the treatment for 5 years since he was young and reported to our OPD due to increase in the size of the swelling over the past few weeks. The present radiographs revealed radiolucency with radiopacity. Excisional biopsy was performed and the histopathological examination revealed an Ameloblastic fibro odontoma. This case report is to document and highlight the possible progression of AF to Ameloblastic fibro-odontoma.

Benign mesenchymal odontogenic tumors are lesions derived from the mesenchymal components of the tooth-forming apparatus and are consequently found within the jawbone. Benign fibro-osseous tumors are part of this category of lesions in which normal bone is substituted, initially by fibrous tissue and within time become infiltrated by osteoid and cementoid elements. They are asymptomatic, slow-growing lesions and remain undiagnosed until swelling of the face becomes prominent and they share similar radiological characteristics. Herein, we report three cases of ossifying fibroma, cemento-osseous fibroma and periapical cemento-osseous dysplasia and analyze all the correlating factors, clinical history, radiological and histological features, intraoperative appearance, and treatment with a 3-year follow-up period. Despite the advances in the identification of these pathologies, clinicians still face difficulties in their classification and the diagnosis due to overlap in both histological and radiographic findings. An accurate final diagnosis is essential for appropriate treatment and an informative prognosis.

Odontogenic carcinosarcoma (OCS) is a rare malignant odontogenic tumor (OT) with only a few cases reported in the literature. Its synonyms are ameloblastic carcinosarcoma, malignant mixed OT. It is characterized by a true mixed tumor showing malignant cytology of both epithelial and mesenchymal components. The tumor invaded into adjacent tissues by destroying the bone. A 24-year-old patient visited the outpatient clinic of GITAM Dental College and Hospital, with a chief complaint of growth in the lower right back tooth region for 6 months. Based on clinical and radiographic features, it has been diagnosed as an aggressive central jaw lesion. The patient was further referred for histological examination for confirmatory diagnosis. It has been diagnosed as an adenomatoid OT. The OCS most commonly affects the posterior part of the mandible. A larger number of cases were reported recently, and prolonged follow-up is needed to further clarify the nature of OCS.

Odontogenic keratocyst (OKC), also known as keratocystic odontogenic tumor, is categorized as one of the developmental epithelial cysts, which accounts 10%–20% of all cystic lesions in jaws. Considering its high recurrence rate, combative treatment modalities such as enucleation and jaw resection are suggested. This case report aims to emphasize clinical, radiological and histological overview of OKC with conservative approach marsupialization using thermoform surgical splint in a 9-year-old female patient with no recurrence at 3-year follow-up.

Malignant odontogenic neoplasms are extremely challenging to study due to their rarity and variable clinical presentations. Ameloblastic carcinoma (AC) is one such odontogenic tumor which has been the subject of controversy, in part because of its scarcity, complicated by confusion in terminology along with complexity in classification. Histologic features of AC resemble tumor cells of ameloblastoma but exhibit cellular atypia. Surgical resection for this kind of lesion, leaving at least a 2 cm free margin coupled with neoadjuvant radiotherapy, might prove fruitful results. The current paper reports a case of an extraosseous variant of AC which posed a diagnostic challenge due to variable presentations histopathologically, suggesting the need for evidence-based case studies and molecular workup for a better therapeutic and prognostic insight.

The dentinogenic ghost cell tumor (DGCT), a solid variant of the calcifying odontogenic cysts, is an uncommon odontogenic neoplasm which is aggressive and has a propensity for recurrence. It accounts for <0.5% of all odontogenic tumors which can exhibit intraosseous (central) or extraosseous (peripheral) localization. Till today, only 39 cases of central DGCT have been reported in English literature according to WHO 2017 Classification. Therapeutic intervention of central variant should be aggressive, local resection with adequate safety margins and monitoring the patient for recurrence as the lesions show recurrence rate up to 71%. The purpose of this paper is to describe a rare case report of central DGCT in a 57-year-old female patient with a brief review of literature which provides an update on the epidemiology, diagnostic and clinicopathological characteristics of the published cases.

Inflammatory myofibroblastic tumor (IMT) is a rare benign neoplasm with variable clinical presentation. We hereby present a case of a 27-year-old female who presented to us with an unsuspecting gingival overgrowth in her anterior gingiva. This article aims to describe IMT as a rare intraoral entity which may involve the anterior maxillary gingiva, involving or sparing the underlying bone, as only a handful of such cases have been reported so far. Surgical excision is the mainstay of treatment. Biopsy and histopathological examination of even the smallest lesions is a must. Timely diagnosis and intervention can prevent severe morbidity that can arise if these lesions are left undiagnosed as aggressive management of lesions that become extensive is mandatory.

Congenital epulis (CE) is a rare condition in newborns, whose histogenesis remains unclear. It mostly presents as a circumscribed mass in the maxilla alveolar ridge of female neonates and can interfere with their normal breathing and breastfeeding. This benign oral lesion usually appears as a pedunculated nodule covered with normal mucosa that extends from the anterior vestibular alveolar ridge up to the posterior region. There are some pathological conditions that show clinical and histopathological features similar to those of CE, such as granular cell tumor, gingival cyst of the newborn, vascular malformations and others. This case report aimed to describe the clinical and pathological features of a newborn patient with a clinical and histopathological diagnosis of CE on the right side of the maxillary alveolar ridge, treated with excision by laser surgery, with a follow-up of 5 months without any sign of recurrence.

Inflammatory myofibroblastic tumor (IMT) of the oral cavity is an extremely rare finding. The etiology and pathogenesis of IMT is controversial and unclear. The tumor requires complete surgical excision and continuous monitoring of clinical consequences. The present article describes the clinical, histological, operative and immunohistochemical features of a case of IMT in the mandibular retromolar region of an 8-year-old male. Histologically, the lesion shows myofibroblastic spindle cell proliferations with infiltrative margins in an inflammatory background. Immunohistochemically, the myofibroblastic spindle cells in the present case were positive for α-smooth muscle actin and CD68 due to which the diagnosis of IMT was confirmed.

Etopic tooth eruption is the occurrence of the tooth germ in a nonanatomical position. It can be associated with dentigerous cyst, which is the second most seen in the development of odontogenic cyst commonly presented in mandibular region and seen in single form. It is usually accompanied with mandibular third molar followed by canine. Bilateral and multiple dentigerous cysts in nonsyndromic patients are fairly rare phenomenon and barely documented in the literatüre. We report a rare case of bilateral dentigerous cysts associated with ectopic third molars in the maxillary sinuses. Computerized tomography scan confirmed the diagnosis. A 32-year-old female patient manifesting as postnasal discharge accompanied with chief complaint of nasal obstruction, pain at blinking right eye from pressure of cyst at the inferior orbital rim, and headache for the last 1 year. This report presented our management of rarely seen case of bilateral dentigerous cysts related with ectopic third molars in maxillary sinuses. Ectopic teeth eruption in maxillary sinus should be treated as early as possible once it is diagnosed, in consideration of related complication of orbital and nasal involvement. The symptoms totally recovered with the suitable management method for our patient. The prevalence of maxillary third molars ectopic eruption in the maxillary sinus (Antrum of Highmore) is extremely rare in the literature. Lack of treatment protocol for such ectopic eruptions in the maxillary sinus and its rareness deserved to be added to the literature.

Angiolymphoid hyperplasia with eosinophilia (ALHE) is an uncommon benign vascular lesion that is clinically characterized as papule or nodule and commonly found in the head-and-neck areas although rarely involve oral cavity. The etiology of ALHE is currently unknown, and various hypotheses include a reactive vascular proliferation, vascular malformation or neoplasm. ALHE is mostly occurs in middle-aged females and rare in children. Here, we are presenting a case of ALHE on lip in a 10-year-old female child.

Small cell neuroendocrine carcinoma (SNEC) was first described in the 19^th century which is usually seen in association with lung cancer and is very rare in the oral cavity. Here, we report one such case in a 39-year-old male patient who presented with swelling on the left middle third of the face for 6 months. This is the 12^th SNEC of oral cavity case so far reported in the English literature and the third case to be present on buccal mucosa. It presented as a nodular swelling over the buccal mucosa and was clinically diagnosed as a traumatic fibroma. The histopathological sections showed diffuse sheets of round cells with granular chromatin. Immunohistochemical findings helped us arrive at a final diagnosis. SNECs are highly proliferative tumors characterized by early and widespread metastases through the vascular channels. Hence, the diagnosis and appropriate management of these lesions is important to decrease the morbidity and mortality rate.

Tumors of the parapharyngeal space (PPS) are extremely rare in pediatric age group. Out of all head-and-neck neoplasms, PPS tumors comprise only 0.5%. Majority of neoplasms in poststyloid PPS are neurogenic tumors. Conventional benign peripheral nerve sheath tumor (PNST) includes neurofibromas, schwannomas and perineuriomas. Hybrid PNSTs are rare entities having combined features of more than one histologic type, and the World Health Organization Classification of Tumors of the Central Nervous System, in the latest 2016, 4^th edition, has recently recognized and published it. The most common hybrid tumor is schwannoma/perineuroma followed by neurofibroma/schwannoma and neurofibroma/perineuroma. Here, we are reporting a hybrid PNST which was completely excised and having combined features of neurofibroma and schwannoma, confirmed by both histopathologically and immunohistochemically, in an 8-year-old child, in PPS.

Spindle cell/sclerosing rhabdomyosarcoma (RMS) is an uncommon type of RMS and has been classified as a separate entity by the WHO in 2013. It affects both children and adults with a greater incidence in males. These tumors can pose a diagnostic challenge and can be difficult to differentiate from other spindle cell malignant tumors in the head and neck. Here, we report a case of spindle cell/sclerosing RMS in a young woman presenting with a swelling on the left side of the face of 3 months duration. A careful correlation with the radiographic images, histopathological findings and immunohistochemistry helped to arrive at a diagnosis.

Ewing's sarcoma (ES) is a small blue round cell tumor, malignant in nature typically affecting long bones and pelvis. Occurrence of ES in the head and neck region is rare and is reported to be 2%–3%, of which the chances of having primary lesion are rare. In the head and neck region, it has been reported to strike skull, supraclavicular region, parotid region, orbital floor, nasal cavity, maxilla, mandible and zygoma. We present a case of primary ES of the mandible in a 22-year-old female who reported with a palpable swelling on the lower left part of the face; and intraorally, there was a growth in the molar region. Orthopantomogram showed multilocular radiolucency in the molar–ramus region. The diagnosis was made after surgical resection, histopathology and immunohistochemistry profiling. The patient was treated by cortical segmental resection of mandible combined with chemotherapy and a follow-up was done for 2 years.

Brown tumors are rare focal giant-cell lesions that arise as a direct result of the effect of parathyroid hormone (PTH) on bone tissue in some patients with hyperparathyroidism. Browns tumor is a syndrome associated with an increase in PTH levels by parathyroid glands resulting in hypercalcemia. In the present case report, a 44-year-old female patient presented with a rare case of brown tumor with multiple lesions in the head-and-neck region. The recent advance in various diagnostic and biochemical tests helps in early diagnosis of hyperparathyroidism cases. The dentist should be aware of oral manifestations associated with this type of systemic disease.

Maxillary sinus is the common site for the nose and paranasal sinus tumors with diverse histopathological types and the treatment for each may differ. Making a histopathological diagnosis on occasion can be challenging. We had two patients presenting with upper alveolus growth in whom establishing the histopathological diagnosis was challenging. Through clinical evaluation, imaging (computed tomography and/or magnetic resonance imaging) and identification of key histopathological features helped in the management of these patients.

Pyogenic granuloma (PG) is a benign nonneoplastic mucocutaneous lesion. It occurs as a result of chronic irritation or due to hormonal changes. The most favorable site for this fairly common lesion is gingiva, but rarely, it can occur outside the oral cavity, later often difficult to diagnose, as a diverse group of the pathologic process can produce such lesions outside the oral cavity. The diagnosis is also challenging as the lesions appear as smooth or lobulated red nodules with easy bleeding, occasionally ulcerated mimicking malignancies. The purpose of this article is to report a rare case of extragingival PG of the lower lip simulating as a vascular lesion in young male of 30 years old diagnosed by ultrasound followed by histopathological examination.

Chewing coca leaves is a habit still practiced in Bolivia, Peru and Argentina. There is scarce evidence in the relationship between this habit and development of oral squamous cell carcinoma (OSCC), some authors have found oral epithelial changes in coca chewers. This study aimed to present a case series of patients with a coca chewing habit that developed OSCC, in the absence of risk factors for oral cancer. Patients were evaluated in Hospital Señor del Milagro, Salta, Argentina. None of them had a relevant morbid history and presented intraoral tumors, with an ulcerated surface on the gingivobuccal complex. Coca chewing habit was recorded in all cases. Present cases could start discussions and new lines of researches focusing on the habit of coca leaves chewing as a risk factor for OSCC. It would be very useful to know the underlying mechanisms between this habit and a possible role in oral carcinogenesis.

Amelogenesis imperfecta (AI) is a developmental, inherited disorder affecting dental enamel. Preterm and low birth weight children are prone to many serious medical problems during the neonatal period, which may affect the development of oral tissues. We report a case of AI presenting with renal disease and thereby highlighting the importance of early diagnosis of this possible association to prevent renal failure and death of the patient.

Fanconi anemia (FA) is a rare genetic disease involving an increased risk of developing acute myeloid leukemia and solid tumors, especially head-and-neck squamous cell carcinomas, for which the oral cavity is the most frequent site of occurrence. The patient presented in this study underwent allogeneic hematopoietic stem cell transplantation (HSCT) and developed nonhomogeneous oral leukoplakia after 7 years, which was promptly removed and diagnosed with high-grade epithelial dysplasia. Many risk conditions for oral squamous cell carcinoma were featured in the present case including FA, allogeneic HSCT, graft-versus-host disease, immunosuppressive therapy, female gender, nonsmoker, tongue location and nonhomogeneous type of leukoplakia. Close follow-up of the entire upper aerodigestive tract mucosa and early removal of all suspected lesions are highly recommended in the management of such patients.

Oral lesions are often the first tell-tale sign for human immunodeficiency virus infections (HIV). Numerous oral lesions have been associated with HIV infections, some lesions such as candidiasis being more common than others. Regular oral screening can aid in identifying such lesions allowing for the early diagnosis of HIV and help in monitoring the progression of HIV in such individuals. We report a case of a family who manifested with oral lesions consistent with HIV. A review of literature on diagnosing immunocompromised individuals in clinical practice has also been summarized.

Salivary sialolithiasis is a well-known cause for obstructive disease of the submandibular and parotid glands. However, the condition frequently occurs unilaterally, and it is uncommon to find a patient reporting with stones in both submandibular or parotid glands. Children below the age of 16 years rarely suffer from salivary stones. Thus, bilateral sialoliths in a child are extremely rare, with only four previous cases been reported in the literature. This is an additional case report of bilateral submandibular sialolithiasis occurring in the hilar area in a 13-year-old boy.