In conventional orthognathic surgery, Le Fort I osteotomy （L1）, sagittal splitting ramus osteotomy （SSRO）, and two-jaw surgery （L1+SSRO） have been used to correct severe reversed occlusion caused by maxillary hypoplasia in patients with cleft lip and palate. However, these methods produce a limited amount of movement for osteotomized jaw bone, and postoperative relapse has also been problematic. Maxillary distraction osteogenesis has attracted attention in recent years as a method that improves both the amount of jaw bone movement and postoperative relapse. We examined changes in maxillofacial condition and postoperative relapse in patients with unilateral cleft lip and palate. In addition, we compared patients who underwent L1+SSRO with those who underwent maxillary distraction osteogenesis. Materials and methods: The subjects were 14 patients with unilateral cleft lip and palate treated in the Maxillofacial Surgery of Tokyo Medical and Dental University from 1992 through 2005. Five patients underwent L1+SSRO conventional orthognathic surgery. Nine patients underwent maxillary distraction osteogenesis （4 RED cases and 5 Zürich cases）. Their maxillofacial conditions were examined on lateral cephalograms before surgery, immediately after surgery （just after distraction was completed）, and more than 6 months after L1+SSRO surgery （more than 12 months after distraction）. Results: Preoperatively, the SNA was less than -1 S.D. in all 14 patients （100 ％）, who all had maxillary hypoplasia. The SNB was less than -1 S.D. in 3 patients （21.4 ％）, within 1 S.D. in 10 patients （71.4 ％）, and more than 1 S.D. in 1 patients （7.1 ％）. In patients who underwent L1+SSRO, SNA averaged 72.3 ± 4.3 ° before surgery and increased to 75.2 ± 3.6 ° more than 6 months after surgery. However, no patient was included within 1 S.D. after surgery. In patients who received maxillary distraction osteogenesis, the SNA averaged 72.5 ± 5.0 ° before surgery and increased to 79.3 ± 5.1 ° more than 12 months after distraction. Five of these 9 patients were included within 1 S.D. after distraction. The mean distance of maxillary advancement was 4.8 mm in L1, 7.3 mm in the Zürich system, and 10.6 mm in the RED system. The relapse rate was 24.5 ％ of patients in L1, 12.7 ％ in the Zürich system, and 16.6 ％ in the RED system. Conclusion: In orthognathic surgery for severe maxillary hypoplasia in patients with cleft lip and palate, maxillary distraction osteogenesis is more effective than conventional orthognathic surgery.
Some cases of untreated cleft palate have been reported in elderly persons in Japan. Recently, such cases are rare because babies with a cleft palate can receive systematic treatment owing to the healthcare system in Japan. We report on a 63-year-old man with a large defect in the soft palate caused by cleft palate. The patient was referred to our clinic because of nasal leakage of water. The large defect, measuring 35 by 12mm, caused velopharyngeal insufficiency during speech. The patient had received primary palatoplasty once during his childhood, but the details were unclear because both of his parents had died while he was still a child. The patient underwent closure of the fistula in his soft palate by the repush back method. Eighteen months after surgery, the patient was able to blow and speak single words without nasal leakage. Fiberscopic examination revealed complete closure of the velopharyngeal space during speech.
We report a rare case of pleomorphic adenoma with dystrophic calcification and bone formation in the capsule of the submandibular gland. A 48-year-old man visited our hospital because of a mass in the submandibular gland. CT and FDG-PET images showed a well-defined round mass 20 mm in diameter in the right submandibular gland. It was covered with a calcified substance. The clinical diagnosis was a submandibular tumor, and the patient underwent extirpation of the right submandibular gland. The tumor was histopathologically diagnosed as a pleomorphic adenoma. Histopathologically, the tumor was composed of epithelial and mesenchymal structures including myxomatous and chondroid areas. Dystrophic calcification was observed in the entire periphery of the tumor, and bone formation was seen in contact with the calcified structure.
Stereotactic radiotherapy （SRT） has been used to treat brain tumors. In recent years, it has also been used to treat lung cancer, liver cancer, and cancer of the head and neck region. We report a case of carcinoma of the retromolar buccal mucosa that was treated by radiotherapy including SRT. An 85-year-old man was referred to our hospital because of discomfort on swallowing. The diagnosis on a biopsy was squamous cell carcinoma. It was difficult to perform surgery or chemotherapy because of his advanced age and poor general condition. Therefore, we decided to administer radiotherapy including SRT to this patient. He received a dose of 50 Gy by external irradiation after 20 Gy by SRT. After treatment, the tumor completely disappeared. Stomatitis and xerostomia developed. However, there were no other serious treatment-related complications. Three years after irradiation, there was no evidence of recurrence or distant metastasis. Therefore, radiotherapy including SRT may be an effective treatment for oral cancer.
Cystadenoma is a benign salivary gland tumor derived from glandular tissue. It occurs mainly in the ovary and occasionally in the testis and thyroid gland. We report a case of cystadenoma arising in the hard palate of a 40-year-old woman who presented with a swelling in the left side of the palate. The lesion was an elastic soft tumor measuring 20 × 20 mm with a clear border. The clinical diagnosis was a benign salivary gland tumor, and the tumor was resected under general anesthesia. The histopathological diagnosis was cystadenoma. There was no recurrence 1 year after the operation.
We report a case of spindle cell lipoma （SCL） of the tongue. A 65-year-old man was referred to our department because of a mass arising in the right margin of the tongue. Physical examination revealed 3 elastic soft masses of the right tongue margin, measuring 6mm, 8mm, and 12mm in diameter. Excisional biopsy of the largest mass was performed. The mass was histopathologically diagnosed as SCL, because it consisted of mature adipocytes and CD34 positive-spindle cells. There have been no signs of recurrence or an increase in size of the other 2 masses for 1 year 10 months.
We report a case of combined epithelial odontogenic tumor arising in the first premolar region of the mandible. A 21-year-old woman was referred for further evaluation of a radiolucent lesion of the mandible. Radiographic examination showed a monolocular radiolucent lesion including an impacted tooth and an irregular radiopacity. Surgical enucleation was performed under general anesthesia. Histopathological examination showed two distinct types of proliferation. In one, the tumor showed duct-like structures lined by a single layer of columnar epithelial cells, typical features of adenomatoid odontogenic tumor （AOT）. The other consisted of hyalinized tissue containing irregularly shaped calcified tissue and amyloid-like materials, features consistent with calcifying epithelial odontogenic tumor （CEOT）. The overall histopathological features confirmed the diagnosis of so-called combined epithelial odontogenic tumor. There have been no signs of recurrence as of 24 months after the operation.
Chondroblastoma is a rare benign tumor of the head and neck. We report a case of chondroblastoma of the mandibular condyle. A 56-year-old woman was referred to our department for the treatment of a left mandibular condyle tumor, which was detected on computed tomography （CT）. The patient had a 3-month history of intermittent pain in the left pre-auricular area, accompanied by episodic trismus and subjective swelling around the lesion. CT and magnetic resonance imaging （MRI） revealed an osteolytic defect in the anterior portion of the left mandibular condyle and a mass extending anterolaterally. The biopsy diagnosis was a chondroblastoma. We performed an en bloc resection that included part of the mandibular ramus. There has been no sign of recurrence for 26 months postoperatively.
We describe a rare case of small cell neuroendocrine carcinoma （SNEC） arising in the maxillary gingiva that responded to high-dose intra-arterial cisplatin and concurrent radiation therapy （RADPLAT）. A 58-year-old man was referred to our clinic because of a swelling in the right maxillary gingiva. The clinical diagnosis was a malignant tumor of the upper gingiva and a biopsy was performed. The histopathological diagnosis was SNEC. Four courses of intra-arterial infusion of CDDP （50 mg） were administered into the maxillary artery via the femoral artery by the Seldinger method, with concurrent external radiation （50 Gy）. As complete response （CR） was obtained. The patient subsequently received ten courses of systemic adjuvant chemotherapy （CBDCA: 500 mg, VP-16: 180 mg） in the outpatient clinic. He has remained free of disease for 27 months after RADPLAT. However, a PET-CT scan showed multiple systemic metastases. We planned to give the patient systemic chemotherapy again, but his renal function and liver function worsened considerably. Finally, the patient died of multiple organ failure within the same month.
We report a rare case of bilateral primary localized amyloidosis of the angle of the mouth. A 76-year-old woman presented with bilateral painless soft elastic masses arising in the angles of her mouth. An excisional biopsy was first performed for the right-side mass, followed by the left-side mass 6 months later. The histopathological diagnosis was amyloidosis, with a precursor of Aκ, in association with amyloid L protein deposition. We suspected amyloid deposition in other organs as well as the presence of amyloidosis in association with multiple myeloma. Comprehensive general physical examination revealed no problems, and bilateral primary localized amyloidosis of the angle of the mouth was confirmed.
Hereditary hemorrhagic telangiectasia （HHT） is an autosomal dominant inherited disorder affecting blood vessels of the skin, mucous membranes and viscera. A definite diagnosis of HHT is made in the presence of at least three of the Curaçao criteria: （1） epistaxis, （2） telangiectasias, （3） visceral lesions, and （4） a family history. We describe a case of upper gingival carcinoma in a patient with HHT. A 79-year-old woman visited our department because of an upper gingival mass. A biopsy specimen revealed squamous cell carcinoma. Preoperative examination revealed consistency with three of the above criteria: （2） skin and mucosal telangiectasias, （3） gastrointestinal telangiectasia and pulmonary and hepatic arteriovenous malformations （AVMs）, and （4） mucosal telangiectasia and pulmonary AVM in her daughter. HHT was definitely diagnosed. Tumorectomy was scheduled; however, HHT-associated complications such as bleeding, hypoxemia, cerebral thromboembolism, and AVM rupture were expected. Tumor resection by partial maxillectomy and reconstruction with a buccal fat pad were performed under general anesthesia. The postoperative course was uneventful, and follow-up at 1.5 years showed no evidence of tumor recurrence or metastasis. We conclude that recognition of hemorrhagic tendencies and evaluation of systemic AVMs are critical before surgical treatment in patients with HHT.
Polymorphous low-grade adenocarcinoma （PLGA） is a rare malignant salivary gland tumor arising most commonly in the palate. We report a case of PLGA in a 72-year-old woman. The patient had a protrusive tumor, measuring 18 × 18 mm, in the left retromolar region. Radiographically, it was located in the submucosa with smooth and shallow resorption of the mandibular bone cortex. A biopsy suggested a benign salivary gland tumor, although a definitive diagnosis was not obtained. The tumor was surgically removed under general anesthesia. It was easily removed from the surface of the mandibular bone. Histopathological examination of the surgical specimen showed a relatively well-demarcated solid epithelial tumor with focal lack of a fibrous capsule. Tumor cells showed a monotonously basaloid appearance characterized by various architectural patterns, including solid, tubular, trabecular, and cribriform ones. Immunohistochemically, the tumor cells were positive for keratin 7, vimentin, and S-100 protein, which indicated myoepithelial characteristics. It was finally diagnosed as PLGA. The postoperative course has been uneventful as of 2 years after surgery.
Supernumerary teeth and odontomas are relatively common conditions of the maxillofacial region, but it is rare for both to be present in a multiple metachronous form. We report a case of metachronous multiple supernumerary teeth with multiple odontomas in a 10-year-old boy. Because impacted supernumerary teeth were present in the median maxillary region between the right mandibular canines and first premolar and in the left mandibular first premolar region on a panoramic X-ray film, the teeth were extracted. On an X-ray film obtained approximately 3 years after the first examination, impacted supernumerary teeth were seen between the left maxillary incisors and canines, as well as between the right mandibular second premolar and first molar. In addition, an irregular opaque image was observed near the left mandibular second premolar region and right maxillary second premolar region. Tooth extraction and surgical removal were performed. The lesions in the left mandibular second premolar region and the right maxillary second premolar region consisted of tooth-like hard tissue encapsulated in white elastic soft tissue, and a compound odontoma was diagnosed. The disease course was good thereafter, and he is currently under observation as an outpatient.
Osteoma is a benign tumor consisting of mature bone tissue. It often occurs in the skull and facial bones; however, peripheral osteoma arising in the inferior border of the mandible is relatively rare. We describe a case of peripheral osteoma arising in the inferior border of the mandible in a 17-year-old girl. She had been aware of a swelling at the left side of the mandible for 1 year. Radiographic and computed tomographic examinations revealed a bony mass located in the inferior border of the mandible. A clinical diagnosis of peripheral osteoma was made, because the tumor showed evidence of slow progression and arose unilaterally in a young woman. We surgically excised the tumor via an intraoral approach. The tumor measured 23 × 18 × 15 mm. The histopathological diagnosis was a compact osteoma. The patient has remained free of recurrence for 6 months after surgery.