Kindler syndrome complicated by invasive squamous cell carcinoma of the palate.

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.

An oral lesion as the primary clinical manifestation of sarcoidosis.

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.

Reconstruction of maxillary defect with musculo-adipose rectus free flap.

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/m2 . 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.

A case of hemangiopericytoma of the soft palate with articulate disorder and dysphagia.

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.

Rehabilitation of an extraoral and intraoral defect complicated with microstomia. A study case.

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.

T cell non-Hodgkin's lymphoma with colesional mucormycosis presenting as palatal perforation: a case report.

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.

Granulocytic sarcoma of palate. Case report and review of literature.

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.

Mushroom-shaped teratoma of the soft palate in a neonate: case report.

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.

Fibrolipoma associated with a mucus retention cyst in the palate: a case report.

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.

Bilateral cystic pulmonary glial heterotopia and palatinal teratoma causing respiratory distress in an infant.

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.

Solitary neurofibroma of the palate. A case report.

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.

Acute hearing loss, dysarthria, dysphagia, and a rubbery intraoral mass in an 18-year-old woman.

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.

Disseminated Kaposi's sarcoma and HCV infection: only a casual relationship? A case report.

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 .