Clear cell carcinoma, not otherwise specified （NOS）, is extremely rare in the salivary gland. This tumor is one of the new additions to the 2005 World Health Organization （WHO） classification of salivary gland tumors. We report a case of clear cell carcinoma, NOS in the buccal mucosa. A 59-year-old woman visited a hospital because of a painless tumor in the left buccal mucosa. The tumor was elastic hard and was the size of the tip of the pinky. The clinical diagnosis was a fibroma, and the tumor was resected under local anesthesia. The histopathological diagnosis was clear cell carcinoma, NOS, and the surgical margin was positive. Half a month after the excision, the patient visited our hospital to treat the residual tumor. The tumor measured 8 mm in diameter, and was excised under general anesthesia. Histological examination revealed that the tumor consisted of small cellular nests with clear cells showing clear cytoplasm and polygonal nuclei. Tumor cells were negative for antibodies against S-100 protein, muscle actin, and Alcian blue. On the basis of these features, the tumor was diagnosed as a clear cell carcinoma, NOS. There has been no evidence of recurrence or metastasis as of 4 years after surgery.
Odontogenic fibroma is a rare benign mesenchymal tumor, originating from dental papillae, dental follicles, or periodontal ligaments. A 16-year-old boy was referred to our hospital because of swelling of the mandibular gingiva from the left second molar to the right canine. Panoramic and CT images revealed a well-circumscribed, extensive multilocular radiolucent lesion extending from the left third molar to the right second premolar. In addition, root resorption was found from the left second molar to the left canine. The clinical diagnosis was an ameloblastoma, and tumor extirpation was performed under general anesthesia. Histopathological examination of the extirpated specimen showed not the pattern of ameloblastoma, but proliferation of tumor cells with small spindle nuclei and cytoplasm. The lesion was composed mainly of fibroblastic tissue. Immunohistochemical staining was performed with cytokeratin 19, AE1/AE3, EMA, vimentin, CD 34, α-smooth muscle actin, and S-100 markers. Only vimentin was positive. The definitive diagnosis was a central odontogenic fibroma. No evidence of recurrence has been noted during 2 years 8 months of postoperative follow-up.
Specific target antigens have been identified in many autoimmune syndromes, and most of these diseases are caused by single target antigens. However, 2 or more target antigens are known to be involved in mucous membrane pemphigoid, which can exhibit various clinical characteristics. We report a case of anti-BP180-type mucous membrane pemphigoid that was difficult to distinguish from bullous pemphigoid on the basis of immunological analysis. The patient was a 76-year-old man who visited our department because of gum erosion and pain. Sore gums and ulcers were observed on both the upper and lower jaws, and mild pruritic erythema was noted on the posterior wall of the oral cavity. Direct immunohistological analysis of tissues obtained from the affected jaw showed deposition of IgG and C3 in the basal membrane. An enzymelinked immunosorbent assay（ELISA）for BP180 and BP230 showed positive results, and an immunoblot assay using the recombinant BP180 protein showed positive results. In addition, an immunoblot assay using the recombinant BP180 protein showed positive results for IgG antibodies against BP180 NC16a and negative results for IgG antibodies against the C-terminal portion of BP180. The patient was suspected to have bullous pemphigoid, but because the main clinical findings were pathologic changes of the oral mucosa, the patient was given a diagnosis of anti-BP180-type mucosal pemphigoid. The symptoms improved with an improvement in oral hygiene and the application of a steroid.
A 74-year-old man received radiotherapy and chemotherapy with vincristine, doxorubicin, and dexamethasone for a solitary plasmacytoma, which first arose in the ilium in 2006. In 2008, a solitary plasmacytoma of the rib was diagnosed, and the patient received radiotherapy and chemotherapy with melphalan, prednisolone, and thalidomide. In 2009, an extramedullary plasmacytoma of the stomach was diagnosed, and the patient underwent radiotherapy. Thereafter, plasmacytoma did not develop in any other part of the body. In 2010, the patient visited a local dental clinic because of mandibular gingival swelling. Panoramic radiography performed at the clinic showed a radiolucent image in the mandible. The patient was referred to our department and underwent a detailed medical examination to determine the treatment. During his first visit, a biopsy was performed, and the lesion was diagnosed as plasmacytoma. A general examination showed no evidence of multiple myeloma. He was transferred to the Internal Medicine Department of our hospital, where chemotherapy with melphalan and prednisolone was initiated. On completion of the second course of chemotherapy, the gingival swelling of the foretooth region of the mandible decreased, and the tumor disappeared. Approximately 1 year after the completion of chemotherapy, the patient did not show signs of plasmacytoma in the oral cavity or in any other part of the body.
Temporomandibular joint （TMJ） ankylosis sometimes occurs with re-adhesion postoperatively for various reasons. In patients with re-adhesion after surgery, bony adhesion at the TMJ advances rigidly leading to poor mobility of the mandible, making it difficult to treat TMJ ankylosis. We describe a patient with bilateral TMJ ankylosis in whom reconstruction was performed with the use of mandibular reconstruction plates with condylar heads during arthroplasty. A 56-year-old woman was referred to our hospital because of severe trismus. Bilateral TMJ ankylosis was diagnosed on CT examination. We performed bilateral arthroplasty. The bone of the mandibular ramus was resected, and the mandibular body was pulled forward and downward. Subsequently, the mandible was reconstructed using plates with condylar heads. The patient is making satisfactory progress without any postoperative signs of re-ankylosis.
Acquired hemophilia A is a rare disorder in which autoantibodies to factor VIII arise, and there is a sudden onset of a critical bleeding episode. Its incidence is reported to be 1.48 cases per million persons per year. We report the case of a patient who had a diagnosis of acquired hemophilia A with submucosal hematoma of the oral floor. A 75-year-old man presented with a swelling in the oral floor. Marked, dark red swelling was seen in the oral floor. On laboratory tests, the platelet count and prothrombin time were normal. However the activated partial thromboplastin time （APTT） was longer than normal. The patient was admitted on the same day. On hospital day 2, acquired hemophilia was suspected because of the presence of intrinsic pathway inhibitors of hemostasis. Then, hemostatic therapy and immunotherapy with corticosteroids were begun. At night of the same day, hypoxemia due to upper airway obstruction occurred, and intratracheal intubation was performed. After that, cyclophosphamide was administered. On hospital day 52, APTT returned to the normal range. The patient was discharged and is being treated as an outpatient in our hospital.
Epignathus is an extremely rare congenital teratoma that arises in the upper jaw, hard palate, or pharynx and causes life-threatening airway obstruction immediately after birth. We report a huge teratoma, detected prenatally on ultrasonography, arising in the oral cavity of a neonate. To save the child’s life, a Cesarean section EXIT (ex utero intrapartum treatment) and tracheotomy were performed. The neonatal patient was a girl and weighed 2,184 g after 35 weeks of gestation. The tumor developed in the floor of the nasal cavity through the cleft palate and adhered to the lingual side of the mandibular ramus, right buccal mucosa, and left hard palate. On postnatal day 15, the tumor was successfully removed under general anesthesia. The extirpated specimen was covered with skin and measured 86 × 53 × 40 mm. Histopathological examination revealed that the tumor was a mature teratoma containing two germ layers, including squamous epithelium, sweat glands, and tooth germ. The patient is now 29 months old and has a ventricular septal defect; the tracheostomy tube could not be removed. We have been working towards improvement of oral function with the use of palatal prostheses.
Cystadenocarcinoma is defined as a malignant salivary gland tumor lacking features of other specific types of salivary gland tumors. It is histologically characterized by cystic and papillary growth. Cystadenocarcinoma was classified as a malignant cystadenoma in the 2005 WHO classification. We report a case of synchronous double cancer involving cystadenocarcinoma in the left mandibular molar region and papillary thyroid carcinoma． A 82-year old man visited our hospital because of pain in the left lower jaw. Clinical examination showed a soft elastic tumor in the left mandibular molar region. An incisional biopsy yielded a pathological diagnosis of cystadenocarcinoma. In addition, papillary thyroid carcinoma was found on the preoperative PET-CT examination. Marginal resection of the mandible was performed under general anesthesia. The papillary thyroid carcinoma was treated by TSH suppressive therapy with thyroid hormone （Levothyroxine）. There was no evidence of recurrence for us to 6 months after the operation, but unfortunately the patient died of another disease in the 7th month.
Calcifying cystic odontogenic tumor is a comparatively rare tumor that often occurs in young people. Histopathologically, it is characterized by odontgenic epithelium and ghost cells with calcification. We report a case of calcifying cystic odontogenic tumor occurring in the mandible of an elderly woman, along with a discussion of the literature. A 92-year-old woman noticed swelling and pain at the left molar region of the mandible about 2 years before presentation. Because the swelling recurred several times after incisional drainage performed at a nearby dental clinic, she visited the department of oral and maxillofacial surgery of another hospital. Biopsy with histopathological examination revealed a calcifying cystic odontogenic tumor, and she was referred to our hospital. She was edentulous and had a bone-like firm bulge extending from the left molar region to the front of the ramus. Panoramic photography and computed tomography showed a radiolucent image with a clear border in the region. The tumor was surgically resected under sedation and local anesthesia. There has been no recurrence during the 15 months of follow-up.
We describe a descending necrotizing mediastinal abscess caused by post-extraction infection that was successfully treated by endoscopic-transthoracic drainage. The patient was 55-year-old man with an odontogenic infection of the oral floor and submandibular region after extraction of the lower second molar. We performed incisional drainage and administered broad-spectrum antibiotics. Despite treatment the inflammation persisted and formed a deep neck abscess that spread to the mediastinum, resulting in an abscess and subsequent sepsis. Surgical neck drainage for the deep-space neck abscess was performed by an otolaryngologist, and endoscopictransthoracic drainage for the descending necrotizing mediastinal abscess was performed by thoracic surgeons. On bacterial culture, abundant growth of Streptococcus constellatus was disclosed. We gave antibiotics against which the pathogen was sensitive to control the spread of inflammation. Systemic management was performed in an intensive care unit.