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Branchio-oto-renal (BOR) syndrome is an autosomal dominant inherited disorder characterized by malformation of the external ear, cervical fistula, hearing loss, and renal anomalies. Branchio-oto (BO) syndrome is a part of BOR in which the phenotype shows only cervical fistula and hearing loss. We experienced two cases of BO syndrome in a Chinese family. Computed tomography (CT) with the injection of iodized oil through the external orifices revealed that these fistulas were originating from the 2nd pharyngeal arch remnants. Although both cases had Mondini type of anomaly, the shape of the kidneys as well as the renal function were normal. We excised the bilateral fistulas using nylon thread as a guide following a method previously reported. This is a simple and useful procedure involving only a small incision, which produces a favorable cosmetic result. The otoralyngologist - head and neck surgeon must be familiar with the embryology of the branchial apparatus, anatomy, and clinical manifestations as well as have knowledge of BOR and BO syndrome to establish an early diagnosis and appropriate therapy.

Primary malignant melanoma of the nasopharynx is a rare disease with poor prognosis. There has been no established treatment for the disease.We report herein a case of a 75-year old man, who was referred with hearing impairment and nasal obstruction. Clinical examination demonstrated left middle ear effusion and nasopharyngeal mass. The histological examination indicated malignant melanoma. Magnetic resonance imaging (MRI) showed a gadolinium-enhanced mass on the left side of nasopharynx. Imaging studies could not detect distant metastasis. The patient received combined chemotherapy with DAV treatment (DTIC+ACNU+VCR), but there was no response. Intratumoral administration of Interferon-beta inhibited tumor growth and X-knife was also effective in significantly decreasing tumor size. These therapies may be helpful in the local treatment of malignant melanoma, thus improving the patient's quality of life.

Mucoepidermoid carcinoma of the maxillary sinus is a rare neoplasm. We describe diagnostic difficulties in a case of low grade mucoepidermoid carcinoma of a cystic pattern in a 72-year-old man. The patient complained of double vision and nasal bleeding. CT findings indicated a shadow in the unilateral maxillary sinus as well as bony destruction of the maxilla on the same side. Although biopsies and maxillary operations were repeatedly performed over a period of 7 years, the histopathological diagnoses were chronic sinusitis with necrotic characteristics or squamous cell carcinoma.Several cases of mucoepidermoid carcinoma have been similarly misdiagnosed as squamous cell carcinoma because mucoepidermoid carcinomas contain epidermoid cell components similar to squamous cell carcinomas. Tumor necrosis in our case were extensive and the mucous discharge secreted by mucous cell components in this type of carcinoma may increase pressure inside the cystic tumor causing tumor necrosis. The significance of a differential diagnosis between mucoepidermoid carcinoma and chronic inflammation or squamous cell carcinoma in the maxillary sinus should be emphasized.

A case of cervical thymic cyst is presented, and the literature is reviewed. A 25-year-old-female was found to have a frontal neck mass. At surgery, the thyroid gland was found to be normal, but displaced by a cystic mass. The cyst was lobulated and contain clear fluid. It was removed from the carotid laterally and from the trachea medially.Thymic cysts are unusual neck masses that may occur along the embryologic pathway of migration of the thymus. The mass produces few symptoms, but can sometimes cause air way obstruction. Treatment consists of excision of the cyst and prognosis is usually good. This entity should be included in the differential diagnosis of neck masses.

Malignant fibrous histiocytoma (MFH) is the most common soft tissue sarcoma in adults. The head and neck are unusual sites for primary MFH, and MFH of the submental region is extremely rare. We encountered a case of MFH in a 60-year-old man who initially showed swelling in the submental region. He died of distant metastasis to lung 15 months after the radical surgery and postoperative chemotherapy.The clinical course and prognosis in this case were described, and the literature was reviewed and discussed.

It has been reported that lymphoepithelial lesions (LEL), including Sjogren syndrome and benign lymphoepithelial lesions (BLEL), often change to low-grade malignant B-cell mucosa associated lymphoid tissue (MALT) lymphomas. In the patient described here BLEL changed to malignant lymphoma in both parotid glands over a 2-year interval. Surgical removal was performed because this malignant lymphoma generally resists to both chemotherapy and radiation therapies, and the prognosis is poor when the lymphoma extends outside the gland.

Branchial cysts in the parotid gland are rare. Among 392 parotid masses which we examined and operated on during the past 15 years and 7 months in our hospital nine were branchial cysts. All were identified as second branchial cysts. Ultrasonography showed a septum structure in two of the cysts. Although ultrasonography and magnetic resonance imaging (MRI) provide useful information in the diagnosis of cysts, those which contain non-serous materials are sometimes misdiagnosed as solid tumor.

Isolated thyroid follicles of C57BL/6J mice were treated with anti-dendritic cell antibody plus complement in order to eliminate dendritic cells. The follicles were transplanted under the renal capsule of BALB/c mice. Acceptance of the grafts was verified by both histological study and the incorporation of 125I into the grafts 100 days after transplantation. Allografts treated with complement were rejected, whereas allografts treated with the antibody plus complement were accepted. These findings indicate that dendritic cells, but not other Ia positive cells, play a crucial role in the induction of rejection in mouse thyroid allografts, and that the deletion of dendritic cells permits allografts to be accepted.

A new method of thyroid allografts in mice was established. Thyroid follicles of C57BL/6J mice were isolated and treated with anti-Ia antibody plus complement in order to eliminate Ia-positive cells. The follicles were mixed with agarose and transplanted under the renal capsule of BALB/c mice. Acceptance of the grafts was verified by histological study 100 days after transplantation. Allografts treated with anti-Ia antibody plus complement were accepted. These findings indicate that Ia-positive cells play a crucial role in the rejection of mouse thyroid allografts, and that the deletion of Ia-positive cells permits allografts to be accepted. Our method presented here may be developed as a strategy for the treatment of patients with congenital or acquired hypothyroidism.