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Introduction: Kindler syndrome is a very rare, autosomal recessive genodermatosis characterized by skin fragility and photosensitivity in infancy with progressive poikiloderma., Case Report: We report the case of a young woman with a history of Kindler syndrome predominantly characterized by extensive involvement of the oropharyngeal mucosa. The patient presented with an ulcerative lesion of the palate. Computed tomography and biopsy concluded on unresectable invasive squamous cell carcinoma of the hard palate. Neoadjuvant chemotherapy was proposed, but the patient died after the first course of chemotherapy in a context of severe gastrointestinal mucositis and generalized sepsis., Discussion: Mucosal manifestations of Kindler syndrome have been described in the literature, but very few cases of malignant transformation to squamous cell carcinoma have been reported, although it is a very well known, long-term complication of this disease. To our knowledge, this is the second reported case of Kindler syndrome complicated by invasive squamous cell carcinoma of the hard palate., (Copyright © 2017 Elsevier Masson SAS. All rights reserved.)

An oral lesion as the first clinical presentation of sarcoidosis is extremely rare. We present the case of a 39-year-old woman who was referred to the oral and maxillofacial surgery department with a persistent asymptomatic nodular lesion in the hard palate. This was located adjacent to a grossly carious upper first molar and a provisional diagnosis of chronic periapical granuloma was made. An incisional biopsy of the lesion was carried out, which unexpectedly revealed the presence of a non-caseating granulomatous inflammatory reaction. A referral was sent to the respiratory medicine team and a diagnosis was later confirmed of stage II sarcoidosis. This case report highlights the need for clinicians to be aware of all possible causes of oral lesions, including rare manifestations of underlying systemic disease.

Background: The rectus myocutaneous free flap (RMFF) is used for medium to large maxillectomy defects. However, in patients with central obesity the inset could be difficult due to the bulk from excessive layer of adipose tissue. We describe a modification of the RMFF for patients with excessive central obesity with a flap consisting of adipose tissue with minimal rectus muscle; the musculo-adipose rectus free flap (MARF)., Methods: Five cases of MARF reconstruction were performed between 2003 and 2013, with patients' body mass indexes ranging from 29.0 to 41.2 kg/m 2 . All patients had sinonasal tumor, of which three were adenoid cystic carcinoma, one squamous cell carcinoma, and one melanoma. Four patients had Codeiro IIIb defects and one had Codeiro II defect. Using the MARF technique, the maxillectomy defect was obliterated with vascularized adipose tissue overlying the rectus muscle and was trimmed to fit the maxillectomy defect. The adipose tissue was allowed to granulate and mucosalize., Results: The volume of adipose tissue harvested was between 120 and 160 mL. All flaps survived with no requirement for re-exploration. Complete oro-nasal separation was achieved in all patients. The time to commencement of oral intake ranges from 5 to 15 days. One patient developed seroma and one developed wound breakdown on the donor site. The length of stay at the hospital ranges from 9 to 22 days. On follow-up ranging 7.5-32.8 months, two patients died from their malignancies. The other three patients were able to tolerate oral soft diet., Conclusion: The MARF may be considered as an alternative to myocutaneous rectus free flap particularly for the reconstruction of maxillary defects in patients with central obesity. © 2015 Wiley Periodicals, Inc. Microsurgery 37:137-141, 2017., (© 2015 Wiley Periodicals, Inc.)

We report a case of hemangiopericytoma of the soft palate of 60-year-old patient, who noticed a mass of the soft palate and experienced difficulty in speaking. We found a pediculate, hard, elastic mass measuring 38 mm (cross-sectional diameter). Computed tomography (CT) scans and dynamic magnetic resonance imaging (MRI) confirmed irregularly shaped mass and revealed a heterogeneous internal composition, consistent with vascular tumors. We excised the tumor under general anesthesia. Histopathological diagnosis was based on positive immunoreactivity of CD99 and vimentin and weak, positive staining of CD34. Three and half years following tumor excision, there is no recurrence or metastasis.

Non-Hodgkin's lymphoma (NHL) is predominantly a disease of lymph nodes, but extranodal involvement is not very uncommon. Palatal involvement by NHL is rare. Mucormycosis is a devastating fungal infection commonly seen in immunocompromised individuals, including those with NHL, but it is affecting the same region has been reported very rarely. Simultaneous infiltration of hard palate by NHL and mucormycosis is extremely unusual. Herein we describe a patient who presented with palatal hole with histopathological examination revealing presence of lymphoma with colesional mucormycosis. The identification of mucor was vital because chemotherapy alone in the absence of antifungals would have had devastating consequences as the mortality of untreated mucormycosis is high.

A 72-year-old man was referred from the surgery department for rehabilitation following surgical resection of Basaloid carcinoma. The first surgical intervention involved the anterior palatal region and was restored with a simple obturator. Two years later further surgery was undertaken to excise a recurrent tumor in the nose and part of the cheek. This resulted in an exposed nasal cavity and maxillary sinus. In addition, there was a small oral aperture composed of thin tissue that stretched to its maximum due to scar formation. The defect was restored with a full thickness skin flap but it subsequently broke down leaving the midface exposed with limited mouth opening due to tissue contraction and scar formation after the flap operation. The defect was rehabilitated with Co-Cr obturator intraorally and a silicone nose retained to the naso-palatal extension of the obturator by a magnet extraorally. This resulted in practically good retention, placement, and adaptation of the two parts of the prosthesis.

A rare case of granulocytic sarcoma of the hand and palate, also known as chloroma, occurring in an adolescent patient is presented. Diagnostic clinical criteria, along with treatment pictures, are also reviewed.

Teratomas of the head and neck are uncommon congenital lesions, which are rarely seen in the soft palate. We here reported a male neonate with a soft palate teratoma associated with soft palate cleft. The most common presenting symptom of oral teratoma is feeding problems. The tumour looks like a mushroom, and was successfully treated with surgery. Feeding returned to normal by removing the mass, and the patient is under follow up for selective operation of the cleft palate. This case is unique because teratoma in a mushroom shape was not reported before., (Copyright 2010 The British Association of Oral and Maxillofacial Surgeons. Published by Elsevier Ltd. All rights reserved.)

Fibrolipomas are benign mesenchymal neoplasms of the fatty tissue rarely encountered in the oral cavity. They account for around 1% to 5% of all neoplasms affecting the mouth and occur as raised, slow-growing, painless lesions of normal or yellow coloration and uncertain etiology. In contrast, mucus retention cysts are epithelium-lined cavities originated from a salivary gland. They are also raised, asymptomatic, slow-growing lesions, located on the floor of the mouth, buccal mucosa and lips. This article reports a diagnostic and a surgical treatment of a rare fibrolipoma case associated with a mucus retention cyst located in the palate.

We report on a male infant with extensive, bilateral cystic and solid lung lesions who presented postnatally with respiratory distress caused by bilateral cystic lung lesions. Parenchyma-sparing resections were performed. Histology revealed the presence of neuroglial cell-lined cysts and glial nodules. In addition, a neural element containing palatinal teratoma was detected and excised. Based on previously published cases, the pathogenesis and clinical features of pulmonary neuroglial heterotopia are discussed.

The neurofibroma is a benign tumor of neuronal origin not frequently located in the oral cavity. The possible association of this neoplasia with systemic pathologies, such as Von Recklinghausen's disease and multiple endocrine neoplasia, makes its diagnosis fundamental. The diagnosis is, in most cases, quite complex, because of the neurofibroma's strong similarities with a great number of benign neoformations of the connective tissue, of the epithelium, and of the bone. The histology represents, therefore, the fundamental diagnostic criterion, in particular, through the use of appropriate immunohistochemical analysis. This articles presents the case of a solitary neurofibroma, subtype I (common Schwann cell type), detected on the left-hand side of the posterior region of the palate in a 56-year-old woman, which presented itself as an otherwise non-symptomatic ulcerated mass. After a careful anamnesis, an incisional biopsy was performed in order to establish the histological nature of the neoformation. The immunohistochemical test, which resulted positive for S-100 and negative for the epithelial membrane antigen and keratin, allowed the diagnosis of a presumably benign lesion deriving from a neural differentiation. The surgical excision of the entire mass was accompanied by the extraction of the upper left-hand second molar, which was attached to the mass through its palatal root.

Rhabdomyosarcoma is the most common soft tissue tumor of childhood, frequently presenting in the head and neck, genitourinary tract, or extremities. We present a case of rhabdomyosarcoma in which an 18-year-old woman presented with abrupt onset unilateral hearing loss, tinnitus, dysarthria, dysphagia, and a new painless red bump on the palate. With an alveolar subtype and older age, both predictors of poor prognosis, early recognition of disease of these symptoms is vital.

The authors present a case of disseminated Kaposi's sarcoma in a male patient, HIV negative and Hepatitis C virus (HCV) positive. Although it is well-known that in HCV positive patients the onset of cutaneous diseases such as porphyria cutanea tarda, mixed essential cryoglobulinemia, lichen planus, polyarteritis nodosa, itch/prurigo, is possible, papers on its association with disseminated Kaposi's sarcoma in HIV negative patients are rare in the literature. Such an association is probably not a matter of chance: in fact, the changes to the immune system induced by the HCV virus, in synergy with those induced by the Human Herpetic virus-8, could likewise play a role in the development of Kaposi's sarcoma as happens in patients with immunodeficiency .

We report an unusual presentation of a salivary pleomorphic adenoma in the uvula. The adenoma caused otalgia and hypernasal speech in a patient without additional symptoms. The tumor was surgically excised with safe margins of the healthy tissue. After the excision of the mass, the new uvula was reconstructed through modified radiofrequency-assisted uvulopalatoplasty technique. After the treatment, the patient has remained disease-free for 1 year.

In this article we present 2 cases of necrotizing sialometaplasia (NS) associated with angiocentric lymphoma of the midline. Immunohistochemical analysis confirmed a T-cell origin, and in situ hybridization in one case revealed its relationship to Epstein-Barr virus. These findings suggest that vascular occlusion by the neoplastic cells produces ischemia, which leads to local infarction contributing to the salivary gland lesion. To our knowledge, the association between angiocentric lymphoma and NS has been previously reported in only one instance, and we suggest that this particular type of lymphoma should be added to the list of related conditions for NS.

Congenital teratoma is a rare malformation, and few papers have been published about it. We present a large teratoma that arose from the hard palate in a neonate. The obstructive mass caused maternal polyhydramnios and was identified prenatally by ultrasonography. The mother went into labour at 35 week's gestation at home. The child was in respiratory distress as a result of airway obstruction, and a tracheostomy was done when she was 4hours old. She also had major cardiac abnormalities. The palatal mass was removed successfully at 4 weeks of age. The typical components of a teratoma were identified including immature neural glial tissue.

Congenital tumors of the oral cavity are uncommon. Teratoid tumors account for most of the reported cases and are infrequently associated with a cleft of the soft palate. Of the remaining tumors associated with pediatric cleft palate, congenital lipoma is rare. Lipomas of the oral cavity may show cartilaginous or osseous changes. These changes have not been reported in lipoma associated with cleft palate. A rare case of congenital osteolipoma associated with cleft palate and showing osseous change is presented here. The pathogenesis of the lesion and the osseous metaplasia are described. The present case is the second case of congenital lipoma associated with cleft palate in the literature.

Mucosal haemangiomas are unusual and typically involve frequently traumatised areas such as the lip, buccal mucosa and lateral borders of the tongue. Uvular haemangioma is rare and to our knowledge, has never been reported to cause obstructive sleep apnoea (OSA). We report an unusual case of uvular haemangioma causing loud habitual snoring and symptoms suggestive of OSA. This case report illustrates a rare cause of OSA and demonstrates the efficacy of surgery for obvious obstructive lesions of the pharynx.

Epignathus is an extremely rare, benign, congenital teratoma that arises from within the oral cavity and may be attached to the mandible, palate, or base of the skull. Because of its location, it can cause airway and feeding problems, as well as secondary defects due to the presence of an intraoral tumor. This tumor usually causes death in neonates, due to airway obstruction. Surgical removal is usually impossible, especially in large tumors (Stone, 1951; Bennett, 1970). A patient with a neonatally diagnosed nonobstructive palatopharyngeal epignathus is described. It was associated with a shortened palate, cleft uvula, and velopharyngeal insufficiency, and was successfully excised surgically.

A case of epignathus combined with cleft palate, lobulated tongue, and lingual hamartoma is reported. A newborn female presented with a large skin-covered mass arising from the palate, cleft palate, and lobulated tongue with a soybean-sized lingual lesion. The palatal and lingual masses were removed on the 9th day after birth. A cystic lesion was also found on the nasal septum after removal of the palatal mass; this lesion was resected at its base. Glossoplasty was performed after excision of the lingual lesion. Histologically, the palatal mass was diagnosed as epignathus. The cystic lesion was suspected to be meningothelial tissues on the basis of hematoxylin-eosin (HE) and immunohistochemical staining: Vimentin and epithelial membrane antigen were positive, and factor VIII was negative. The mass of the tongue dorsum was diagnosed as hamartoma. No recurrence of epignathus or evidence of meningocele have been observed after 5 years of follow up.

Background: Neurofibromatosis type 1 (NF1) is the most common form of neurofibromatosis. While typically considered a dermatologic disorder, intraoral signs of neurofibromatosis occur quite commonly. This clinical entity can be confused with periodontitis because of the presence of periodontal pockets. In this report, we present the case of a palatal neurofibroma with radiographic involvement in a patient with NF1., Methods: A 40-year-old female patient was referred from her general dentist to evaluate advanced periodontitis in the maxillary left quadrant. The patient's medical history was significant for a soft tissue lesion excised from her back 11 years previously and diagnosed as a neurofibroma. Subsequent medical examination at that time confirmed a systemic diagnosis of NF1. A comprehensive periodontal evaluation was performed, and panoramic and periapical radiographs were taken. Teeth were tested for vitality. An incisional biopsy was completed for histopathologic examination., Results: The periodontal evaluation revealed the presence of 6 to 9 mm probing depths adjacent to teeth #14 and #15. Panoramic and periapical radiographs showed a circumscribed 0.8x0.9-cm unilocular radiolucency superimposed over the root of tooth #13 and extensive horizontal bone loss on the distal side of #15. Incisional biopsy confirmed the presence of a neurofibroma, and because of the extent of the lesion, the patient was referred to the Oral and Maxillofacial Surgery service for complete excision., Conclusions: Neurofibromas can cause extensive destruction of alveolar bone, mimicking periodontitis. Due to the potential systemic and genetic implications, the diagnosis of neurofibroma requires appropriate medical referral.

Oral vascular lesions are of clinical importance to the dental profession because they pose serious bleeding risks. A case report is presented here where a patient with a complex palatal vascular malformation was successfully treated with fixed appliances. The problems encountered during treatment are discussed.

Pleomorphic adenoma of soft palate is extremely rare. A large tumour in soft palate is reported with sleep apnea syndrome. Diagnosis was made by fine needle aspiration cytology and intraoperative consultation by imprint smears, which were confirmed by histopathological and immunohistochemical studies.

A number of oral lesions have been reported in association with HIV, including lesions caused by other viruses such as the epitheliotropic human papillomavirus (HPV). More than 90 types of HPV have been identified, with the less commonly encountered strains of HPV tending to show association with immunodeficiency states. In addition, HIV-infected patients may have Kaposi's sarcoma develop, a malignancy thought to be caused by human herpes virus, type 8. Recent evidence suggests a sexual mode of transmission for this virus. We report an HIV-positive man with a large, HPV type 40-associated papilloma of the anterior palate and a previously undiagnosed focus of Kaposi's sarcoma.

This paper illustrates the clinical findings and aims at report surgical matters observed in 2 patients affected by neurofibromatosis type I with a rare palatal localization. By means of retrospective analysis 2 cases of neurofibromatosis type I with a palatal mass are selected. Both cases were surgically treated and underwent 12 months follow-up. Patients underwent surgical removal of the palatal neurofibroma. At 12 months follow-up a good local condition without any recurrence was observed. Malignant transformation was very rarely reported in oral neurofibromata and can follow incomplete removal. Surgical treatment of neurofibromata requires the sacrifice of the nerve trunk from where they originate, together with the complete removal of the mass with security margins. Therefore, risks and benefits from surgery should be carefully weighted in each patient and where surgery is not performed clinical and radiographic trials are advisable.

We present a rare case of an infant's palatal polyp associated with an impacted supernumerary tooth. We have previously reported three cases of palatal polyps in infants [1]. In one case, after surgical removal of the palatal polyp at the age of 1 year and 8 months, the lesion subsequently began swelling. A periapical radiograph at the age of 2 years and 9 months revealed a small calcified mass in the maxillary left incisor region. The swelling was kept under observation, the calcified mass developed gradually and was removed surgically at the age of 5 years and 5 months. The removed calcified mass was clinically diagnosed as an impacted supernumerary tooth and this was confirmed histologically. Histological findings did not indicate any relationship between the palatal polyp and the impacted supernumerary tooth.

Objective: Congenital tumors of the oral cavity are extremely uncommon. The teratoid tumors (epiganthi, dermoid, and hairy polyps) account for almost all of the reported cases and are not infrequently associated with cleft of the soft palate. Of the remaining tumors in the pediatric cleft palate population, infantile lipoma of the oral cavity is exceedingly rare. A case of a congenital lipoma associated with a cleft of the soft palate is presented. The review of the literature, the description of the lesion, the diagnosis, and the management of this finding are outlined.

8 patients (7 male and 1 female) treated between 1991-1998 in Szczecin centre due to the tumours different than carcinoma planoepithelial of the palate were presented. Their age ranged from 30 to 81 years. The dimension of the tumour ranged from 1 to 5 cm. The diagnosis of those tumours was: adenoid cystic carcinoma--2 cases, rare (unnamed) tumour of low malignancy probably of salivary gland origin--1 case, cystis lined with cylindrical epithelium--1 case. 7/8 patients were treated by surgery and in 3 cases RTG therapy followed surgery. One patient refused the treatment. 6/8 patients are alive. One patient died 2 years after surgery. There is no information about the one, who wasn't operated on. 3 patients remain without complains. 3 developed rhinolalia aperta and one from them (following RTG therapy) complain of dryness in the oral cavity. None from the operated on reports any swallowing problems.

An 1-day-old female newborn who had typical clinical features of Aicardi syndrome, such as agenesis of the corpus callosum, ocular abnormalities and infantile spasm associated with a palatal hemangioma is reported. The intraoral mass, which occluded incompletely the oropharynx and right side of the nasopharynx, was partially excised under general anesthesia. This is the first reported patient with Aicardi syndrome with palatal hemangioma, according to the med-line search.

We present a case of duplication of the pituitary gland with associated clefts of the hard palate and body of the sphenoid and a midline palatine dermoid. This is an extremely rare malformation with only six previous case reports in the literature. Imaging findings are discussed and literature reviewed for possible morphogenesis, including its association with partial twinning and midline cleft face syndrome.

Plasma cell orificial mucositis is a relatively rare condition which may occur on mucous membrane. Plasmoacanthoma is a verrucous tumor with plasmacytic infiltration and has been described as a separate disease entity. Such a tumor is supposed to occur without lesions which could be compatible with plasma cell orificial mucositis. The present case report documents the coexistence of both conditions in one single patient. This observation suggests that both conditions are part of one single disease entity.

The case of a 55-year old man who was affected simultaneously by acute myeloblastic leukaemia (AML) and adenoid cystic carcinoma of the palate is reported. The carcinoma was removed after induction chemotherapy for AML and the patient subjected to consolidation therapy. No reports are evident of other cases in which acute leukaemia and other malignancies were treated simultaneously, and in which the patient was cured of both. It is suggested that this case may serve as a reference for future similar cases.

Garcin syndrome is an ipsilateral step-by-step deterioration of all 12 brain cranial nerves, first described in 1927. The underlying cause is usually a sarcoma or carcinoma of the skull base. The pathogenesis of the Garcin syndrome is chiefly determined by the slow localized growth of these tumors, rather than by their histology and primary localisation. The prognosis is as a rule unfavourable. The findings on computed and magnetic resonance tomography are important for an early diagnosis. Two cases of Garcin syndrome are reported, one of which was probably due to a tonsillar carcinoma, while the other was caused by a mucinous adenocarcinoma of the nasopharynx.

Extracutaneous squamous cell carcinoma is an unusual complication of epidermolysis bullosa (EB). We present a patient with long-standing EB who developed squamous cell carcinoma involving the palate and the maxillary sinus, and we describe the findings on CT.

We report an example of sacrococcygeal teratoma presenting as a "supernumerary limb" in the left gluteal area with a rudimentary hemipelvis, femur, tibia, fibula, and tarsal bones. This monocephalus tripus dibrachius-appearing complex with associated with a deep seated but separate pararectal mature teratoma. This combination of a well-developed external limb with a deep but separate teratoma has been described.

An 8-year-old Quarter Horse mare was examined for chronic nasal discharge and obstruction of both nasal passages. A solid mass lesion was identified in the maxillary sinuses, soft palate, nasal and pharyngeal cavities. Palliative surgery was used to debulk the lesion and facilitate nasal airflow. Squamous cell carcinoma was diagnosed from surgical biopsies. Approximately 7-8 weeks after surgery, the mare was observed to be acutely blind. Ophthalmologic examination revealed central origin blindness and active retinitis. The squamous cell carcinoma had reobstructed the nasal passages. Pressure by the expanding tumor deformed the ethmoid and sphenoid bones resulting in compression of the optic tracts. No bony invasion by the tumor was present.

Six cases are reported of an unusual synchronous association between aerodigestive squamous cell carcinoma and papillary carcinoma of the thyroid. Six males (47-62 years) were observed until 1975 at the Department of Head and Neck Surgical Oncology of the Istituto Nazionale Tumori, Milan. All were affected by aerodigestive cancer: oral cavity (1), pyriform sinuses (1), larynx (2), lip (1) and oropharynx (1). Clinical examination of the thyroid was always negative. For this reason they underwent surgery, complete surgical approach to aerodigestive neoplasm; one hemithyroidectomy and one total thyroidectomy were also performed. Unexpectedly pathological examination of the dissected lymph nodes indicated the presence of metastases of papillary carcinoma of the thyroid. Three of the six cases had only metastases of thyroid papillary carcinoma while the others presented both squamous cell and papillary carcinoma metastases. Excluding the 2 patients who had undergone thyroid surgery during aerodigestive cancer therapy, in 3 of the 6 patients, two total thyroidectomies and one hemithyroidectomy were performed after the metastases were discovered. In all cases, primary thyroid cancers were demonstrated. One patient did not undergo any subsequent surgery on the thyroid gland because of negative thyroid scanning and bad prognosis due to aerodigestive cancer. Two of the 6 patients died of aerodigestive cancer at 54 and 34 months, 2 are still alive and free of both diseases while one was lost after a 43 month follow-up.