Fanconi Anemia is a rare autosomal recessive disorder characterized by various congenital malformations, progressive bone marrow failure at a very young age and of solid tumors development. The authors present a rare case of a squamous cell carcinoma of the hard palate in a Fanconi Anaemia patient. The atypical clinical manifestation rendered the diagnosis more difficult. This case, for age of appearance, sex and localization, is unique in international literature. We recommend a quarterly follow up of the oral-rhino-pharynx complex in FA patients and to consider as carcinomas, all oral lesions that last more than two weeks.
Both internal and external devices are effective in distraction craniofacial advancement. However, significant differences do exist concerning the surgical aspects and the practical difficulties experienced by the patients.
The metastases to the TMJ from a primary lung tumour is a very rare occurrence.This case is unusual in several aspects, as the non-reducible dislocation of the TMJ was the first clinical manifestation of the tumour. CT staging showed that this secondary tumour in the condyle was the only bone metastasis.
The purpose of this article was to report a pediatric case of secondary cervical esthesioneuroblastoma involving the parapharyngeal lymph nodes. A 3-year-old boy came to our clinical observation because of a right lymphonodal mass evidenced by nuclear magnetic resonance and a diagnosis of Cushing syndrome associated with ectopic adrenocorticotropic hormone secretion, moon face, central obesity, asthenia, and hirsutism. At the age of 10 months, the patient underwent endoscopic surgery for asportation of the World Health Organization stage IV esthesioneuroblastoma. At 38 months of age, the patient underwent right parapharyngeal lymphadenectomy with surgical access by a double mandibulectomy. After surgery, serum ACTH, cortisolemia, and urinary excretion of cortisol were within the reference range. Blood pressure was recorded at 110/70 mm Hg. Moon face disappeared, as well as central obesity and hirsutism. Clinical report is presented together with brief review of literature.
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