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[Value of computed tomography for tumors of the epi- and oropharynx].

The results of computed tomography in 89 patients with tumours of the epipharynx and oropharynx involving the mouth are compared with the findings of other forms of clinical examination. In 26 patients, CT provided additional relevant information, in eight patients CT provided less information than clinical examination. The value of CT depended largely on the localisation of the tumour; the poorest results were found in tumours of the tonsils and the soft palate. CT provided additional information most commonly with large tumours, since these are frequently associated with bone destruction, local infiltration and occlusion of large vessels.

Carcinoma, Squamous Cell↗

Ameloblastoma: a clinical, radiographic, and histopathologic analysis of 71 cases.

OBJECTIVES: The purpose of this study was to compare the clinical, radiologic, and histopathologic features of 71 intraosseous ameloblastomas. STUDY DESIGN: Data with respect to the patients' ages, sex, tumor locations, and surgical treatment history, as well as the radiographic findings and number of recurrences, were analyzed. The histologic types of and radiologic findings regarding tumors with higher recurrence rates were also investigated. RESULTS: The patients' ages at biopsy ranged from 11 to 70 years (mean, 30.4 years). Thirty-nine (54.9%) of the 71 subjects were males, and 32 (45.1%) were females. Sixty-two (87.3%) of the 71 ameloblastomas were located in the mandible. Swelling was the most common symptom and was experienced by 27 (38.0%) patients. Radiographically, 42 (59.2%) of the 71 tumors were unilocular with a well-demarcated border. Of the remaining 29 cases, 14 were multilocular, 2 were of soap-bubble shape, and 13 were unknown in appearance. The most common histologic pattern was plexiform, rather than follicular or acanthomatous. Sixteen cases of ameloblastoma had developed in a cyst. The overall recurrence rate was 21.1%, and the average age of the patient at recurrence was 26.4 years. CONCLUSIONS: When the diagnosis of ameloblastoma in young people remains in doubt after clinical and radiologic examination, a biopsy is necessary. Long-term follow-up at regular intervals after surgery is also recommended.

Adolescent↗

Comparison of long-term results between different approaches to ameloblastoma.

OBJECTIVE: The long-term results of different surgical approaches to ameloblastoma were compared to develop a more rational surgical approach to this tumor. STUDY DESIGN: Seventy-eight primary ameloblastomas, including 27 unicystic, 21 multicystic, and 30 solid-type tumors, were examined in this study. The methods of treatment consisted of radical surgery (ie, resection-both segmental and marginal) and conservative treatments (ie, marsupialization alone, marsupialization followed by enucleation with sufficient bone curettage if necessary, and enucleation with bone curettage). The effect of marsupialization on recurrence data after a follow-up period of at least 5 years was evaluated with respect to clinical type and histologic pattern. RESULTS: Marsupialization was performed in 31 cystic ameloblastomas before surgery, and the effective rate of marsupialization was 74.2%. Recurrence was observed in 7.1% (3/42) after radical surgery and in 33.3% (12/36) in conservative treatments. Relatively higher tendencies of recurrence were observed in the multicystic type and follicular and/or plexiform pattern tumors. CONCLUSIONS: Conservative treatments including marsupialization and enucleation followed by sufficient bone curettage were thought to be useful, reducing the need for jaw resection. This result extends the indications for conservative treatment of ameloblastoma.

Adolescent↗

Hyperparathyroidism-jaw tumor syndrome: the HRPT2 locus is within a 0.7-cM region on chromosome 1q.

Hyperparathyroidism-jaw tumor syndrome (HPT-JT) is an autosomal dominant disease characterized by the development of multiple parathyroid adenomas and multiple fibro-osseous tumors of the maxilla and mandible. Some families have had affected members with involvement of the kidneys, variously reported as Wilms tumors, nephroblastomas, and hamartomas. The HPT-JT gene (HRPT2) maps to chromosome 1q25-q31. We describe further investigation of two HPT-JT families (K3304 and K3349) identified through the literature. These two expanded families and two previously reported families were investigated jointly for linkage with 21 new, closely linked markers. Multipoint linkage analysis resulted in a maximum LOD score of 7.83 (at recombination fraction 0) for markers D1S2848-D1S191. Recombination events in these families reduced the HRPT2 region to approximately 14.7 cM. In addition, two of these four study families (i.e., K3304 and K11687) share a 2.2-cM length of their (expanded) affected haplotype, indicating a possible common origin. Combining the linkage data and shared-haplotype data, we propose a 0.7-cM candidate region for HRPT2.

Adenoma↗

Malignant ameloblastoma with pulmonary metastasis and hypercalcemia. Report of an autopsy case and review of the literature.

A case of malignant ameloblastoma with hypercalcemia in a 67-year-old Japanese woman is presented. The tumor of the maxilla was removed and diagnosed as a follicular ameloblastoma. The tumor recurred in the lower orbita-zygoma region, and multiple tumors of the lungs and hypercalcemia were detected eight months after the second operation. The recurrent tumor resembled the primary tumor but was less well differentiated. Autopsy revealed widespread lung metastasis of the malignant ameloblastoma, nephrocalcinosis, and sigmoid colon cancer. Histologic examination showed the metastatic ameloblastoma to be composed of nests and strands of basaloid and spindle-shaped cells surrounded by columnar cells arranged in palisade formation, with focal areas of squamous differentiation and occasional cystic degeneration. Only two cases of malignant ameloblastoma with hypercalcemia have previously been reported. This is the first case of malignant ameloblastoma with hypercalcemia and sigmoid colon cancer. In addition, prostaglandin E2 assay revealed that ameloblastoma produces prostaglandin E2, which results in hypercalcemia.

Aged↗

Osteosarcoma of the facial bones.

Maxillo-facial osteosarcoma is a rare primary tumor in adults. Between 1980 and 1990, 11 patients were considered; 6 had primary tumors in mandible and 5 in the maxillo-paranasal region. All cases were treated with surgery as the primary modality. Resection was radical in 8 patients and palliative in the other 3. Adjuvant postoperative chemotherapy with adriamycin was administered for 6 months in the 8 patients treated with complete resection. After a median follow-up of 3 years, 7 patients are still alive and 4 died of progressive disease. In the group of patients treated with radical surgery and adjuvant chemotherapy only one died for distant metastases, and 7 are living free of disease. With complete surgical resection long term local tumor control was achieved in all patients. No patient treated with incomplete resection achieved local tumor control with subsequent radiotherapy. The possibility of performing a complete surgical resection of the primary appears to be an essential step to obtain long term local control and survival in maxillo-facial osteosarcoma. Our series is, however, too limited to evaluate the therapeutic benefit of adjuvant chemotherapy.

Adolescent↗

Osteosarcoma of bone and its important recognizable varieties.

Osteosarcoma of bone is a recognizable entity if the histopathologist designates tumors as such when their malignatn cells produce osteoid substance even if only in small foci. Such definition distinguishes this lesion from other sarcomas that arise in bone, especially chondrosarcoma and fibrosarcoma. There is a general tendency to consider that osteosarcomas represent a stereotyped form of disease for which new modalities of treatment can be applied and assessed. The question of whether a given osseous lesion is actually malignant and not a benign neoplasm or even a reactive non-neoplastic condition simulating a malignant tumor may be difficult for the histopathologist. Pathologists without considerable experience in the diagnosis of bone tumors find this question especially vexing. The establishment of a valid diagnosis of osteosarcoma introduces the additional problem that the 11 varieties considered in this paper may pose significant recognizable variations in the clinical capability of the disease. It is apparent that the physician must recognize the known clinicopathologic and prognostic factors of these subtypes in his assessment of the overall problem.

Adolescent↗

Benign osteoma with Gardner syndrome: review of the literature and report of a case.

Gardner syndrome, a variant of familial adenomatous polyposis, is an autosomal dominant disease characterized by gastrointestinal polyps that develop in the colon as well as in the stomach and upper intestine (duodenum), multiple osteomas, and skin and soft tissue tumors. Cutaneous findings include epidermoid cysts, desmoid tumors, and other benign tumors. Polyps have a 100% risk of undergoing malignant transformation; consequently, early identification and therapy of the disease are critical. Osteoma is a benign neoplasm of bone tissue that is characterized by slow continuous growth and is the most common accompanying bone lesion seen in Gardner syndrome. The authors report a case of Gardner syndrome that was operated on because of the mandibular osteoma.

Gardner Syndrome↗

Odontogenic keratocysts: a clinicopathological study in Hong Kong Chinese.

OBJECTIVE: To analyze the clinicopathological features of odontogenic keratocysts in Chinese patients. STUDY DESIGN: Retrospective analysis. METHODS: The clinical records and pathological features of odontogenic keratocysts from 69 ethnic Hong Kong Chinese patients (40 male and 29 female patients) were reviewed. RESULTS: The male-to-female ratio was 1.4:1; patient age ranged from 6 to 69 years with a modal peak in the third decade (mean age, 28 y; median age, 23 y). The preoperative diagnosis was correct in 78% of the cases and the most common misdiagnosis was dentigerous cyst. Sixty-two percent of the cysts were found in the mandible, and 38% in the maxilla. Multiple cysts occurred in 9% (including three cases of basal cell nevus syndrome). Histologically, 82.6%, 5.8%, and 11.6% of the cysts were parakeratinized, orthokeratinized, and mixed types, respectively. Patients with multiple cysts all showed parakeratinization. The majority (80%) of the cysts were lined by epithelia with a thickness of five to eight cells. Surface corrugation, subepithelial split, suprabasal split, satellite microcysts, epithelial islands, significant inflammation, hyaline bodies, and dystrophic calcification were present in 10%, 81%, 25%, 38%, 42%, 46%, 7%, and 10% of cysts, respectively. The overall recurrence rate was 240%, with a median time for first recurrence of 19 months (range, 1 mo-22 y). Cysts showing orthokeratinization or mixed types of keratinization recurred less often than parakeratinized cysts. Recurrence had the same type of keratinization as the initial cyst. CONCLUSIONS: Pathological examination of keratocysts is important, because keratocysts have different clinicopathological features and carry a risk for clinical misdiagnosis.

Adolescent↗

Recurrent ameloblastoma of the jaws--a review of 22 cases.

A retrospective study of 22 patients who had surgical treatment for recurrent ameloblastoma of the jaws at our clinic, within a period of 16 years, was carried out. (1) The recurrent lesion in 18 patients was due to an inadequate previous operation; (2) massive soft-tissue recurrences even after wide mandibular resection occurred in 4 patients; and (3) a malignant transformation was noted in 1 patient.

Adolescent↗

The lateral pectoral composite flap in one-stage reconstruction of the irradiated mandible.

We present a composite flap based on the musculature of the anterolateral thorax that allows immediate reconstruction of the mandible through the interposition of vascularized bony fifth rib. While the vascular pedicle remains the pectoral branch of the thoracoacromial artery, the pectoralis major muscle can be spared largely intact. The lateral position of the composite rib segment ensures a purely bony composition, while the lateral skin island remains largely hairless. Despite the unfavorable conditions of massive tumor size and prior irradiation attending the clinical cases presented, the complication rate was acceptable, with only one flap loss, and this followed technical error.

Adult↗

Functional reconstruction after a total maxillectomy using a fibula osteocutaneous flap with osseointegrated implants.

The fibula osteocutaneous flap with osseointegrated implants was used for reconstruction of a total maxillectomy defect. We have achieved satisfactory reconstruction of three-dimensional facial structure, orbit support, and a functional prosthesis. Our procedure restored the patient's masticatory function of the maxilla and enabled good speech and a natural facial appearance. A very high quality of function was obtained without any complications, but long-term follow-up is necessary for maintenance of the implants.

Aged↗

Diagnosis and treatment of metastatic ameloblastoma.

Although there are several theoretic mechanisms for metastasis of ameloblastoma, hematogenous and lymphangitic spread are the most likely. Metastatic ameloblastoma in the lung can be accurately diagnosed by transbronchial biopsy. Surgical excision of metastatic ameloblastoma is one choice for treatment, but there is no good evidence that this benefits the patient. Treatment of metastatic ameloblastoma with previously used chemotherapeutic regimens has shown little clinical response and has always been followed by disease progression. There may be a role for our chemotherapy regimen for local control in dangerous locations, thereby facilitating further surgical removal and minimizing postoperative sequelae.

Adult↗