Cases reported "Jaw Diseases"

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1/22. Cranio-facial fibrous dysplasia in a 38-year-old African woman: a case history.

    A 38-year-old woman sought treatment with cranio-facial dysplasia involving the cranium, maxilla and the mandible. Her chief complaint was a mandibular swelling, which had appeared about 2 years previously, had gradually enlarged, and was associated with spontaneous pain. x-ray film examination revealed a ground-glass opacity with blurred demarcation and a 99 Tc medronate bone scan disclosed an increase in tracer uptake in the cranium, maxilla and mandible. The rest of the skeleton was not affected. Histological examination of the lesions revealed solid proliferation of spindle-shaped cells associated with islands of osteid and bone trabecullae with Chinese letter pattern and numerous multinucleated giant cells consistent with fibrous dysplasia. The continued osteoblastic activity of involved bones, coupled with the medical condition of the patient, restricted the management of the patient to largely supportive and palliative care.
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2/22. cherubism and its charlatans.

    cherubism is a rare hereditary condition characterised by progressive cystic proliferation of the mandible and maxilla in childhood, followed by post-pubertal involution of the process and jaw remodelling in adulthood. Its name is derived from the cherubic appearance that results from the jaw hypertrophy. Here, we present the case of a young boy with cherubism, in the context of his pedigree, to illustrate the clinical characteristics and their variable expression. We also seek to distinguish cherubism from central giant cell granuloma and giant cell tumour of the jaws, with which it holds a false synonymity.
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keywords = giant
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3/22. Intralesional corticosteroids as an alternative treatment for central giant cell granuloma.

    Four cases of central giant cell granuloma were treated with intralesional infiltration of a solution of Kenacort-A (10 mg/mL, triamcinolone aqueous suspension SQUIBB) and either (1) lidocaine 2% with epinephrine 1:200,000 Marcaine or (2) bupivacaine, 50% mixture by volume. These cases were originally diagnosed by radiographic and histologic studies in 3 Guatemalan males--ages 31, 34, and 6 years old--and a 21/2-year-old Guatemalan girl. The average dosage of the aforementioned solution was 6 mL (equivalent to 30 mg of triamcinolone) for the adults and 5 mL (equivalent to 25 mg of triamcinolone) for the pediatric patients. Before treatment, an endocrinologist evaluated all of the patients to rule out hyperparathyroidism. Also before treatment, an incisional biopsy of the lesion was obtained from each patient for microscopic examination. Follow-up radiographs for all the cases showed progressive improvement and eventual resolution of the lesions.
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4/22. Multiple and synchronous peripheral giant cell granulomas of the gums.

    The presence of multiple giant cell lesions in the maxillofacial region is suggestive of hyperparathyroidism or neurofibromatosis. A case of synchronous, multiple peripheral giant cell granulomas is reported, bilaterally affecting the mandibular gums and also the upper right maxillary gingival mucosa, without concomitant systemic disease. Only 2 cases of central giant cell granulomas, and no cases of peripheral giant cell granulomas exhibiting similar characteristics, have been reported in the literature to date.
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5/22. Central giant cell granuloma.

    Central giant cell granuloma, a fibro-osseous lesion, is more commonly found in the mandible and mainly in children and young adults. The lesion, which has a greater incidence in females, may be uni or multilocular. On the basis of clinical, radiological and histologic features, central giant cell granulomas can be classified as "non-aggressive" or "aggressive". Management involves surgical removal and in most cases the dentition can be maintained. Three cases of central giant cell granuloma are reported and they illustrate clinical features of the lesion, how differential diagnosis can be assisted by ORAD (a special software program), treatment, and the importance of recall examinations.
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6/22. jaw claudication in primary amyloidosis: unusual presentation of a rare disease.

    We describe two patients with temporal artery biopsy-proven amyloidosis presenting with symptoms of jaw claudication, visual disturbance, and proximal muscle stiffness suggestive of giant cell arteritis (GCA) and polymyalgia rheumatica. At the onset of disease, neither patient had other characteristic symptoms to suggest primary amyloid. We point out similarities between GCA and primary amyloid that can lead to confusion in diagnosis.
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7/22. cherubism: diagnosis, treatment, and comparison with central giant cell granulomas and giant cell tumors.

    An extensive case of cherubism in a 17-year-old girl with no apparent familial history is presented. The rationale for treatment is presented and is compared with previous reports that have advocated nontreatment. The clinical and histologic similarities of cherubism to central giant cell granuloma and the giant cell tumor of bone are also discussed.
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keywords = giant
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8/22. cherubism - new hypotheses on pathogenesis and therapeutic consequences.

    AIMS: The hereditary occurrence of cherubism indicates a probable genetic aetiology: a correlation with a mutation in the gene SH3BP2 has been demonstrated. A convincing concept of formal pathogenesis is not yet available. The study was aimed at advancing the understanding of the pathogenesis of cherubism by presenting a case study including genetic findings and an evaluation of the literature. RESULTS AND CONCLUSION: Because of its association with the development of the second and third molars, cherubism could be defined as a genetically determined alteration of tooth development. In this context, disturbed PTHrP - PTHrP receptor interaction induced by the mutation in SH3BP2 is discussed. The temporal and spatial determination of the clinical symptoms is explained by an interaction of SH3BP2-dependent signal transduction pathways with jaw morphogenesis (e.g. Hox-gene Msx-1). Because of the disease-induced lack of determination of the cap phase of the second and third molar, a spatial compartmentation, which is necessary for normal dental development, does not take place. This leads to dysregulation of mesenchymal bone building tissue areas, and to the development of giant cell granulomas with high osteoclastic activity. Because of the genetic determination of cherubism and the associated dedifferentiation of the diseased tissue, a surgical removal should be exclusively restricted to specific indications. Therefore an attitude of wait and see is preferred.
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9/22. erdheim-chester disease in a child presenting with multiple jaw lesions.

    BACKGROUND: erdheim-chester disease is a rare histiocytic disease entity related to juvenile xanthogranuloma. It is a systemic condition, usually occurs in adult, characterized by infiltration of foamy histiocytes within the bone and soft tissues. methods AND RESULTS: We report a case of 13-year-old female patient who first presented with multiple osteolytic lesions of the jaws followed by bilateral symmetrical bone lesions affecting the lower extremities, as well as brain and abdominal involvement. Histological findings of the jaw lesions showed lipid-storing CD68 ( ), CD1a (-) histiocytes with Touton type giant cells. CONCLUSION: To the best of our knowledge, this is the first case of erdheim-chester disease with jaw bone lesions occurring as initial presenting symptom.
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keywords = giant
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10/22. Brown tumors of the jaws associated with primary or secondary hyperparathyroidism. A clinical study and review of the literature.

    PURPOSE: The aim of this article is to present the development of brown tumors in the jaws as a definite feature of hyperparathyroidism (HPT), whether primary or secondary. patients AND methods: The study included 2 cases of primary HPT and 3 cases of secondary HPT associated with brown tumors in the jaws. The patients presented in our clinic at the "G. Papanikolaou" Hospital in Thessaloniki for treatment of their disease. The patients were 4 women and 1 man, and patient's ages ranged from 21 to 76 years. Four cases of the brown tumors involved the mandible, and 1 case involved the maxilla and the maxillary sinus. The surgical excision of bone lesions and treatment of primary or secondary HPT were performed on the patients in our study. RESULTS: Patient 1 (primary HPT, caused by an adenoma of the right parathyroid glands) was treated with surgical removal of the maxillary lesion and excision of the adenoma. Three years later, the patient is free of the disease, with no recurrence of the brown tumor. Patient 2 (primary HPT, caused by an adenoma of the right parathyroid glands) was treated with excision of the adenoma. One year later, a second bone lesion developed in the mandible, and 3(1/2) of the fourth parathyroid (right) glands was removed. The patient is being observed. The other 3 patients with brown tumors associated with secondary HPT were treated with excision of the bone lesions; HPT was treated with hemodialysis (2 patients) and renal transplantation (1 patient). For the patients treated with hemodialysis, the disease is under control and there is no recurrence of the brown tumors. The patient who underwent transplantation is free of the disease 7 years later. CONCLUSIONS: Primary or secondary HPT may be recognized by the presence of an osteolytic lesion with giant cells, a condition referred as "brown tumor." The most useful therapy for patients with brown tumors is surgical excision of bone lesions and therapy (surgical or medical) for primary or secondary HPT.
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