Epidermal nevus and ameloblastoma: a rare association.

Epidermal nevi are clinical manifestations of a malformation affecting the embryonic ectoderm. They appear as linear verrucous lesions and are more often located in the skin of the trunk or the extremities. Rarely, verrucous lesions affect the oral mucosa. Epidermal nevi can be a component of epidermal nevus syndrome (ENS), which is characterized by malformations involving several organs, such as the central nervous system. ENS is rarely associated with other solid tumors. We present the case of a patient affected by ENS who developed maxillary and mandibular ameloblastomas. Epidermal nevi were located in the left middle and lower third of the face and in the left cervical region, whereas the ameloblastomas arose within the left maxillary mucosa and within the left mandibular ramus. Features of the syndrome are described and the relevant literature is reviewed.

Congenital granular cell lesion in newborn mandible.

Congenital granular cell lesion (CGCL) is a rare non-neoplastic lesion found in newborns also known as Neumann's tumor. This benign lesion occurs predominantly in females mostly as a single mass. The histogenesis and natural history of the lesion remains obscure. It arises from the mucosa of the gingiva, either from the maxillary or mandibular alveolar ridge. The lesion is more common in the maxillary alveolar ridge than the mandibular.The present report describes a case of congenital granular cell lesion in an eight-day-old female child who was born with a mass on the anterior mandibular alveolar ridge. The mass was protruding from her mouth and compromised feeding. A clinical diagnosis of teratoma was suggested. Histologically, cells of this lesion are identical to granular cell tumor (neuroectodermal type) and show intense diastase-resistant Periodic Acid Schiff positivity. Immunohistochemically, cells are positive for vimentin but negative for S-100 and desmin, thus suggesting that CGCL is possibly derived from primitive gingival mesenchymal cells rather than having schwannian origin.

Intraosseous noninvoluting congenital hemangioma of the mandible in a neonate.

Hemangiomas are benign tumors of the capillary endothelium involved in varied clinical and pathologic entities. Congenital hemangioma is quite rare compared to infantile hemangioma. Hemangiomas rarely occur in bone. Intraosseous hemangiomas are most commonly found in the vertebral column and skull. The mandible is the most frequently affected skull bone. Intraosseous hemangiomas commonly present in the second decade of life. Here, we report a neonate with noninvoluting congenital hemangioma of the mandible. To our knowledge, she is the youngest patient reported in the literature.

Congenital granular cell lesion "congenital epulis"--report of a case.

Congenital granular cell lesion (CGCL) or "congenital epulis" is a rare lesion of newborn also known as Neuman's Tumor, which is benign in nature, mostly occurs as a single tumour but rarely as multiple. The histogenesis and natural clinical history of the lesion remains obscure. Even with the advent of modem histopathological techniques, it has not been possible to depict specific cellular features unique to this lesion. This lesion arises from mucosa of gingiva either from maxillary or mandibular alveolar ridge. The appearance of the lesion is most common in maxillary alveolar ridge than mandibular with predominance in females than in the males. The present report describes a case of congenital granular cell lesion (CGCL) in the incisor region of mandibular alveolar ridge. This lesion was causing feeding problem and was excised under local anaesthesia.

Gorham Stout syndrome (disappearing bone disease): two additional case reports and a review of the literature.

Gorham-Stout syndrome, or disappearing bone disease, is characterized by the proliferation of thin-walled vascular channels associated with regional osteolysis. There have been fewer than 150 cases reported in the literature. In this clinical report, we describe 2 additional cases of Gorham-Stout syndrome affecting the maxillofacial skeleton. We provide a review of the clinical diagnosis of this syndrome and describe treatment options.

Tuberous sclerosis: clinicopathologic features and review of the literature.

INTRODUCTION: Tuberous sclerosis is a hamartoneoplastic syndrome, which may involve multiple organ systems. Oral hard tissue manifestations of the syndrome have been described in the literature only as recently as 1955. Patients who presented with clinical manifestations of tuberous sclerosis did not routinely undergo oral surveys to rule out 'lesions', and consequently data on 'lesions' in the maxillofacial complex is scant. Ten cases have been found in the English language literature, which describe maxillofacial 'lesions', which may be tumours, new growths, neoplasms or overgrowths occurring in patients diagnosed with tuberous sclerosis. PURPOSE: To review the literature for all maxillofacial lesions associated with tuberous sclerosis and to present an eleventh case of a patient with a maxillofacial lesion diagnosed as having tuberous sclerosis. RESULTS: Eleven cases were found with maxillofacial fibroblastic lesions associated with tuberous sclerosis. These lesions were all fibrous benign neoplasms found in the maxillofacial bony complex. CONCLUSIONS: Maxillofacial fibroblastic lesions in tuberous sclerosis have various histopathological presentations, some of which may be difficult to differentiate. Consequently, close microscopic examination of these lesions is necessary so that adequate surgical treatment is provided.

Double tongue, intraoral anomalies, and cleft palate--case reports and a discussion of developmental pathology.

OBJECTIVE: Isolated cleft palate is the most common presentation of the nonsyndromic cleft lip/palate combinations and is multifactorial in etiology. We report two cases of children with clefts of the secondary palate coexistent with double tongue and in either case mandibular epulis or superiorly displaced salivary gland. RESULTS AND DISCUSSION: In each case, the palatal cleft correlated anatomically with the intraoral space-occupying lesion. The ratio of tongue volume to intraoral volume during palatogenesis is discussed with reference to the pathogenesis of cleft palate. These clinical cases propose the model of a unifying sequence of developmental events whereby deformation of palatal shelf elevation results in secondary palatal clefting.

Congenital ameloblastic fibroma in association with oculoauriculovertebral spectrum.

Although orodental anomalies have been reported to occur in patients with oculoauriculovertebral spectrum, no previous publications in the English literature have been located that describe an ameloblastic fibroma in a patient with this condition. This report presents a 15-month-old female monozygotic twin with concomitant oculoauriculovertebral spectrum and a congenital ameloblastic fibroma of the mandible. A theory linking the pathogenesis of the two conditions is also raised.

Congenital epulis.

Congenital epulis of the newborn is a rare gingival tumor that occurs along the alveolar ridge. We report the prenatal sonographic and postnatal MR imaging findings in an infant with maxillary and mandibular congenital epulides.

Congenital granular-cell myoblastoma.

Congenital granular-cell myoblastoma is a rare jaw tumor presenting in the neonatal period. The differentiation between congenital epulis and granular cell myoblastoma is a controversial issue amongst pathologists. It is a benign swelling in which simple excision is the treatment of choice. Here we report two cases with a brief review of literature.

[Congenital granular-cell tumor]. / Vrozhdennaia zernistokletochnaia opukhol'.

Tumour consisting of nodes (15 x 10 x 8 mm, 2 cm and 0.3 cm in size) on the alveolar processes of the maxilla and mandibula is found in a newborn. Histological diagnosis is granular-cell myoblastoma.

Granular cell lesions in head and neck: a clinicopathological study.

Twenty-five cases of granular cell lesions in the region of the head and neck are presented. The adult form (18 cases) was more frequent in women, and the most frequent sites were the tongue, the skin, and subcutaneous tissues, followed by the lip and buccal mucosa. One lesion occurred in the larynx. The congenital forms (7 cases) all occurred in female infants and involved the mucosa overlying either the anterior ridge of the maxilla or the mandible. Immunohistochemical studies revealed positive staining for S100 protein in all the granular cell tumors of the adult but in none of the congenital granular cell epulides. Embryonic antigen was present in the cells of all the congenital cases and in three-quarters of the adult cases. The significance of the histological and immunohistochemical findings is discussed. We conclude that the congenital and adult lesions are similar morphologically but are not necessarily of similar histogenesis. The findings with respect to S100 protein favor a neural (Schwann cell) origin of the adult granular cell tumor, but not the congenital form.

Bilateral mandibular hamartomas and familial natal teeth.

The case of a newborn girl with two mandibular incisor natal teeth and bilateral mandibular odontogenic harmartomas is presented. The hamartomas were evident as pedunculated masses on the posterior mandibular gum pads. The natal teeth were extracted at birth, and the hamartomas were excised at 5 months of age. Microscopic investigation of the hamartoma demonstrated the presence of all odontogenic tissues with the exception of an enamel organ. In addition, there was a strong family history of natal teeth, which may suggest a hereditary basis for the development of the odontogenic hamartoma.

Lymphangiomas of the alveolar ridge in a neonate: report of case.

Two alveolar ridge lymphangiomas in a black neonate were identified. No case of neonatal lymphangioma in a non-black has been reported. The majority of treatments for lymphangiomas have been either surgical removal or allowing for spontaneous regression. Both treatments were tried in this case with equal success. The treatment of choice may be to allow for spontaneous regression with periodic monitoring, if the lesion is not endangering the child, thus avoiding surgery.