Cases reported "Fibromatosis, Aggressive"

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1/23. Infantile fibromatosis of the neck with intracranial involvement: MR and CT findings.

    CT and MR imaging studies were performed in a 3-year-old boy with infantile fibromatosis arising from the infratemporal fossa and extending into the middle cranial fossa. On CT scans, the lesion was hyperattenuating (44-49 Hounsfield units [HU]), enhancing significantly after application of contrast material (63-66 HU). The MR images showed a multilobulated lesion of heterogeneous signal intensity. The tumor was markedly hypointense on T2-weighted images and slightly hypointense on T1-weighted images relative to brain tissue, iso- or slightly hyperintense relative to tongue muscle on both T2- and T1-weighted images, and enhanced strongly after administration of gadopentetate dimeglumine.
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2/23. Desmoid tumours of the neck: rare but problematic.

    Desmoids of the neck are histologically benign but locally aggressive fibrous tumours with a strong propensity to recur. Surgical excision is the treatment of choice, with patients requiring long-term follow-up. We present such a case and review the treatment of such tumours.
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3/23. Six cases of fibromatosis of the head and neck in children.

    Six cases of aggressive fibromatosis occurring in the head and neck in children are described. Fibromatosis is a rare, benign but locally infiltrative condition. Primary excision is not always possible in the head and neck areas. It is not always appropriate to treat children using the same modalities as adults due to associated growth problems. The case histories illustrate the management difficulties that can be encountered when treating the paediatric population.
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4/23. Gastric scirrhous carcinoma as a rare cause of aggressive fibromatosis in the neck.

    Aggressive fibromatosis is characterized by locally aggressive proliferation of fibroblasts and occasionally occurs in the head and neck. Although the etiology of this disease is still controversial, recent studies have shown that high levels of growth factors are found in these lesions. This article reports a case of 51-year-old woman with aggressive fibromatosis in the neck and inguinal region bilaterally. Gastric fiberscopic examination revealed a complication of advanced gastric scirrhous carcinoma. There were remarkably high levels of basic fibroblast growth factor (bFGF) and platelet-derived growth factor (PDGF) in the serum. Postmortem autopsy examination was performed and scattered gastric carcinoma cells were observed in the fibromatosis lesions. Immunohistological staining showed positive expression of bFGF in the fibromatoses and stomach. We concluded that this was a rare case in which bFGF and PDGF released from gastric carcinoma cells caused aggressive fibromatosis by promoting unregulated proliferation of fibroblasts and collagen production locally.
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5/23. Aggressive fibromatosis of the larynx: report of a new case in an adult patient and review of the literature.

    We describe a case of aggressive fibromatosis of the larynx occurring in a 75-year-old man. The lesion manifested with voice hoarseness and swallowing difficulty. A computerized tomographic scan of the neck revealed distortion of the glottic profile. A malignant tumor was suspected. Although a laryngoscopy-driven biopsy was non-diagnostic, total laryngectomy was done, since the lesion was not deemed amenable to conservative therapy. Grossly, the glottic rim was infiltrated by a hard, grey-white tissue showing a tentacular outline. Tissue sections featured a moderately cellular lesion composed of spindle cells with bland, tapered nuclei, enmeshed in a variably collagenized ground substance. Delicate spindle cell fascicles surrounded the native submucosal seromucous glands and had invaded the thyroid cartilage and the thyroid gland as well. The spindle cells were immunopositive for actins and vimentin, and negative for keratins, epithelial membrane antigen, desmin, and S-100 protein. No further therapy was administered. Periodic follow-up visits were negative. The patient died 5 years after surgery of myocardial infarction with no clinical evidence of lesion recurrence. Based on the available literature, our data confirm that laryngeal fibromatosis in adult patients is a locally infiltrating and progressive disease. Total laryngectomy with clear margins is needed as to avoid the high risk of local recurrence.
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6/23. Gardner-associated fibromas (GAF) in young patients: a distinct fibrous lesion that identifies unsuspected gardner syndrome and risk for fibromatosis.

    gardner syndrome (GS), caused by mutations in the adenomatous polyposis coli (APC) gene, is characterized by polyposis coli, osteomas, and various soft-tissue tumors. If undetected or untreated, virtually all patients develop colonic carcinoma at a young age. Early detection, while essential, can be difficult because of attenuated phenotypes or spontaneous mutations. We present the clinicopathologic features of 11 identical fibromatous lesions that we have termed Gardner-associated fibroma (GAF), which not only appear to be a part of the spectrum of lesions associated with GS but, in some cases, represent the sentinel event leading to its detection. The GAFs occurred in 11 patients (5 boys and 6 girls; age range, 3 months-14 years), were solitary (n = 7) or multiple (n = 4), and occurred in the superficial and deep soft tissues of the paraspinal region (n = 7), back (n = 3), face (n = 2), scalp (n = 2), chest wall (n = 2), thigh (n = 1), neck (n = 1), and flank (n = 1). Histologically, GAFs resemble nuchal-type fibromas (NFs), consisting of thick, haphazardly arranged collagen bundles between which are found occasional bland fibroblasts, and having margins that frequently engulf surrounding structures including adjacent fat, muscle and nerves. After surgical excision, four patients developed recurrences that were classic desmoid fibromatoses (DFs). In one patient with multiple GAFs, one lesion had the features of GAF and DF in the absence of surgical trauma. A family history of GS or polyposis (n = 6) or DF (n = 1) was known at the time of surgery in seven patients. In three patients, the diagnosis of GAF resulted in the diagnosis of unsuspected APC in older family members, with the detection of an occult colonic adenocarcinoma in one parent. In the family of the remaining patient, no stigmata of GS were present. Genetic analysis of this child was performed to investigate the presence of a spontaneous (new) mutation; however, no abnormalities were detected. The significance of GAF is that it serves as a sentinel event for identifying GS kindreds, including those with a high risk for the development of DF, and it may potentially identify children with spontaneous mutations of the APC gene. Because NFs and GAFs resemble one another, we suggest that a subset of NF occurring in multiple sites, unusual locations, or children may be GAF.
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7/23. Aggressive fibromatosis of the neck in a patient with Gardner's syndrome.

    We report a patient with Gardner's syndrome who, in addition to a total colectomy, had multiple excisions of desmoid tumors in both thighs. He presented with left-sided neck swelling and pain. MRI was highly suggestive of desmoid tumors in multiple neck muscles. To our knowledge this is the first description of diffuse fibromatosis of the neck in association with Gardner's syndrome.
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8/23. Aggressive fibromatosis of the head and neck (desmoid tumours).

    Desmoid tumours are histologically benign fibrous neoplasms arising from the musculoaponeurotic structures throughout the body. They are characterized as infiltrative, usually well-differentiated firm overgrowth of fibrous tissue and are locally aggressive. An aggressive clinical behaviour with tendency for recurrence makes the treatment of these relatively rare fibrous tumours difficult. Local recurrence rates are reported as high as 70 per cent of cases, the recurrence of desmoid fibromatosis in the head and neck is difficult to ascertain because of the different classification schemes used by different authors. A review of cases of desmoid tumours of the head and neck presented in the last 10 years is reported in this paper. Complete surgical excision of desmoid tumours is considered to be the only effective method of cure by most authorities.
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ranking = 1.5
keywords = neck
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9/23. Nuchal aggressive fibromatosis in childhood: two instructive case reports.

    Aggressive fibromatosis is a rare, benign tumour with a capacity for infiltration of surrounding structures and a propensity for local recurrence. The cornerstone of therapy is surgery, with various other treatment modalities having ill-defined roles. Assessment of the efficacy of these interventions is difficult. The natural history of the condition is variable and different treatment modalities are often used concurrently. Childhood cases pose particular management problems because of their tendency to occur in the head and neck region and the potential for treatment-related morbidity. Two children presented after surgery with recurrent disease threatening the airway. One remitted spontaneously and remains disease free at 20 years. The other achieved a complete remission with radiotherapy and toremifene. The role of non-surgical treatment, particularly radiotherapy, is reviewed.
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10/23. Desmoid tumour of the neck: complete regression following radiation therapy.

    Desmoid tumours are uncommon benign neoplasms. They arise from fascial and musculoaponeurotic tissues and are locally infiltrative, resulting in a high rate of local recurrence following surgical resection. They present difficult loco-regional control. The location and extent of the tumour, as well as the potential for significant morbidity and mortality, dictate the most appropriate therapeutic option. Complete surgical extirpation is the optimal treatment for primary and recurrent desmoid tumours. radiotherapy is indicated in incompletely excised or recurrent tumours. radiation as a primary treatment is seldom recommended. A case of desmoid tumour of the neck, primarily treated with radical radiation, is described and relevant literature reviewed.
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keywords = neck
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