Cases reported "Fibromatosis, Aggressive"

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1/10. Desmoid-type infantile fibromatosis in the mandible: a case report.

    A 3-year-old boy with desmoid-type infantile fibromatosis arose in the mandible was reported. He was referred to our hospital because of suspected malignant bone tumor of the mandible. Histological examination of an open biopsy specimen was performed followed by tumor resection with marginal mandibulectomy and reconstruction by iliac bone grafting, which caused no functional complications nor mandibular deformity. To treat tumors in the facial skeleton, the surgical procedure should be planned based on the histological diagnosis in order to determine the proper area of resection to prevent functional or cosmetic complications. Especially in children, attention should be taken for benign but clinically resembling malignant rare diseases such as desmoid-type infantile fibromatosis.
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2/10. Extra-abdominal fibromatosis invading the mandible: case report.

    Extra-abdominal fibromatosis (desmoid tumour) is a rare aggressive neoplasm with a tendency to infiltrate local structures but rarely metastasises or undergoes spontaneous malignant transformation. The treatment of choice is surgery, however, recurrences have been reported even after wide-field resection. This article presents a case of extra-abdominal fibromatosis that had extensively invaded the mandible.
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3/10. Desmoplastic fibroma of bone: case report.

    Desmoplastic fibroma of bone is an exceedingly rare tumor that was first described by Jaffe in 1958 [1]. It has a high incidence of local recurrence after surgical resection. It is usually seen in young patients and involves mandible and long bones. It has histological resemblance to the desmoid tumor of the abdominal wall. We report a surgically proven case of desmoplastic fibroma of bone with local aggressiveness and recurrence during follow-up. The radiograph, CT imaging features, radiological and pathological differential diagnosis of the case are described, and literature is briefly reviewed.
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4/10. Agressive fibromatosis involving the mandible--case report and review of the literature.

    BACKGROUND: Aggressive fibromatosis (AF) involving the mandible is rare, and surgery is often complicated by a high recurrence rate. CASE REPORT: A 4-year-old boy was referred because of a fast growing painless mass which involved the entire left angle of the mandible. Excisional biopsy revealed AF, and local excision of the tumor was performed. Six months after surgery a recurrence was detected. The tumor was determined to be unresectable and the boy was treated with low-dose chemotherapy including methotrexate and vinblastine for 1 year. With combined chemotherapy and surgical debulking, mutilating surgical resection will be delayed as long as possible or until completion of facial growth. CONCLUSION: According to the literature, surgery is the most common treatment of AF in the head and neck region. However, particularly in children, alternative modes of therapy must be considered because of the high recurrence rate and to avoid mutilating operations.
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5/10. Aggressive fibromatosis of the mandible: a case report.

    An extensive tumour in a 7-year-old girl, leading to severe disfigurement, proved to be an aggressive fibromatosis on histological examination. Eighteen months after surgery there was no evidence of recurrent disease. This suggests that tumour resection and reconstruction of the mandible had been successful. Contrary to some reports, tumour resection led to curative therapy whereas radiotherapy failed.
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6/10. Complications of mandibular reconstruction in childhood: Report of a case of Juvenile Aggressive Fibromatosis.

    Juvenile aggressive fibromatosis is an acquired disease affecting young children. There are two types: superficial and deep; the first is not aggressive whilst the second invades other tissues deeply. This is a case report of the deep variant of juvenile aggressive fibromatosis of the lateral mandible affecting a 24-month-old young female patient. The tumour has been treated surgically by resection of the mandible and reconstruction with a rib-graft. To by-passs resorption of the rib-graft and to re-establish the correct three-dimensional shape of the facial skeleton, osteodistraction of the reconstructed mandible was performed six months post-peratively. In this article the surgical techniques to reconstruct the mandible in young children are discussed.
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7/10. Pediatric fibromatosis requiring mandibular resection and reconstruction.

    Fibromatosis is the nonmalignant proliferation of fibroblasts that aggressively invade adjacent tissues. The cause of this abundant growth is unknown. When fibromatosis develops in the mandible or adjoining tissues, its aggressive growth can compress the trachea and cause death. Resection is the treatment of choice. mandibular reconstruction is performed to prevent displacement of the remaining mandibular segment or segments and consequent difficulties in eating and speaking. Perioperative nurses who care for children with oral or paraoral fibromatoses must understand the special physical and psychological needs of these patients to effectively plan their care.
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8/10. Gardner's syndrome. Case report and discussion of the manifestations of the disorder.

    Extracolonic manifestations of Gardner's syndrome are common and may precede the detection of colonic polyps. Tc-MDP bone scintigraphy performed on a patient with Gardner's syndrome demonstrated intense uptake of radiotracer within the maxilla and mandible as a result of the dental anomalies associated with this disorder. Nuclear scintigraphy has a role in the imaging of these patients for skeletal anomalies, the detection of thyroid carcinoma, and for skeletal metastases when colon carcinoma is detected.
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9/10. Muscular dystrophy associated with extra-abdominal desmoid tumor showing aberrant chromosome 1 [46,XX,add(1)(p36)].

    We report on a 2-year-old girl with probable limb-girdle muscular dystrophy associated with an extra-abdominal desmoid tumor of the right mandible. This association is previously undescribed. The tumor was totally removed. cytogenetic analysis of the tumor showed a clonal karyotypic abnormality: 46,XX,add(1)(p36) in 3 of 20 cells analyzed. Since an association of a neoplasm with limb-girdle muscular dystrophy has previously been reported in 3 cases, the two abnormalities are likely related causally. The chromosome abnormality in our patient may play a role in the occurrence of her desmoid tumor.
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keywords = mandible
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10/10. Fibromatosis of the mandible: case report and review of previous publications.

    The term 'fibromatosis' is used to denote two main pathological entities: juvenile fibromatosis and adult fibromatosis. It is also possible to distinguish between superficial fibromatosis of the aponeurosis and deep fibromatosis of the muscle-aponeurosis. Histopathological findings have indicated that fibromatosis is an invasive neoformation of fibromatous connective tissue involving adjacent structures. It does not metastasize, though recurrence rates vary. Treatment is based on either excision of the mass, or radiotherapy and chemotherapy if the condition is inoperable. We describe a case of fibromatosis of the mandible in a young girl. The growth was excised completely and she was still disease-free four years later.
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keywords = mandible
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