Cases reported "Fibroma"

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1/91. Cicatricial fibromatosis mimics metastatic medulloblastoma.

    Cicatricial fibromatoses usually occur in the anterior abdominal wall or in the extremities, but rarely in the scalp or the soft tissues of the neck. We report a case of desmoid fibromatosis that developed in a 15-year-old boy 8 months after surgery for cerebellar medulloblastoma.
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keywords = neck
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2/91. 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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keywords = neck
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3/91. Nuchal fibroma: a clinicopathological review.

    Nuchal fibroma, or collagenosis nuchae, is a benign soft tissue tumor that arises from the posterior cervical subcutaneous tissue, with a predilection for the interscapular and paraspinal regions. Because of its benign clinical course and its close histopathologic similarity to other benign head and neck lesions, this lesion may be misdiagnosed and underreported. The purpose of this paper is to review the histopathologic and radiologic findings unique to nuchal fibroma, and compare and contrast it to the other soft tissue neoplasms within the clinical differential diagnosis. These include several benign (elastofibroma, lipoma, fibrolipoma, nodular fasciitis) and rare malignant entities (fibrosarcoma, liposarcoma, fibromatosis).
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keywords = neck
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4/91. Calcifying fibrous pseudotumor of the neck.

    Calcifying fibrous pseudotumor is a rare lesion of uncertain histogenesis that has a unique histologic appearance. We report herein a case of a 24-year-old woman with a mass on the right posterior side of the neck. magnetic resonance imaging with contrast showed a well-circumscribed mass between the right splenius and semispinalis cervicis muscles; the study suggested high collagen content. Simple excision was performed. The histologic findings were diagnostic of calcifying fibrous pseudotumor. Our review of 19 previously reported cases suggests that a good outcome is expected when a diagnosis of calcifying fibrous pseudotumor is made.
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keywords = neck
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5/91. Nuchal fibroma associated with scleredema, diabetes mellitus and organic solvent exposure.

    A case of scleredema diabeticorum of Buschke associated with nuchal fibroma and organic solvent exposure is reported. The patient presented with a neck mass causing discomfort and restriction of movement. Histological examination showed this to be a nuchal fibroma. Additionally, there was widespread induration of the skin of his trunk which was asymptomatic. A biopsy showed features of scleredema. This is the first reported association of these two conditions, both of which show increased and thickened collagen bundles without significant fibroblast proliferation. They differ by the occurrence of mucin in scleredema, although this is not always demonstrable, particularly in late lesions. The possibility that nuchal fibroma is an end stage, localized form of scleredema is canvassed. The patient's medical history included insulin-dependent diabetes mellitus with complications of retinal vessel thrombosis and peripheral neuropathy. The patient also had significant past exposure to a wide variety of chemicals, including organic solvents.
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6/91. Elastofibroma of the neck.

    Elastofibromas are benign lesions of the chest wall. We describe the first reported case of elastofibroma in the neck. Imaging features as well as location of the lesion were atypical. On computed tomography and magnetic resonance imaging the lesion contained a marked preponderance of fat, because the lesion arose within fat.
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keywords = neck
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7/91. Localized birt-hogg-dube syndrome with prominent perivascular fibromas.

    The autosomal dominant birt-hogg-dube syndrome is a cutaneo-intestinal condition that manifests on the skin in the form of multiple, skin-colored small papules that, histologically, prove to be mantleomas (fibrofolliculomas and trichodiscomas). These cutaneous lesions usually appear in the region of the head, neck, and upper part of the trunk. To date, only a single report in the literature describes the localized occurrence of this syndrome. We now describe a localized form of the birt-hogg-dube syndrome in a man with multiple mantleomas that were confined to the left half of the face, and which, in part, were arranged in the form of plaques. Another striking finding in this patient was a conspicuous vascular component in the lesions, characterized by a pronounced, well-demarcated fibrosis in the region of cutaneous blood vessel proliferations. Because perivascular fibromas have already been observed in other patients with Birt-Hogg-Dube-syndrome, the perivascular fibroma, with fibrofolliculoma and trichodiscoma, must be included within this syndrome's spectrum of skin changes.
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8/91. 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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ranking = 1
keywords = neck
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9/91. Fibromatosis of the head and neck region.

    OBJECTIVE: Five cases of head and neck fibromatosis were analyzed. The imaging and pathologic findings, surgical management, and clinical outcome were discussed. STUDY DESIGN AND SETTING: A retrospective study of 5 adult head and neck fibromatosis cases, evaluating long-term follow-up results of conservative treatment. RESULTS: The 5 patients (2 male and 3 female) ranged in age from 16 to 51 years. The lesion size ranged from 1 to 8 cm. Four cases had limited surgical resection; 1 case was followed only. One of the surgically treated cases had a recurrence that was irradiated. All patients were well for a follow-up period of 2 to 8 years. CONCLUSION: Aggressive excision of head and neck fibromatosis cannot be achieved easily. Vigilant follow-up with or without conservative surgical excision achieves good disease control. Low dose radiotherapy can be used for inoperable cases. SIGNIFICANCE: Judicious conservative treatment should be attempted for head and neck fibromatosis to achieve optimal functional preservation.
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ranking = 8
keywords = neck
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10/91. A case of head and neck kaposiform hemangioendothelioma simulating a malignancy on imaging.

    Kaposiform hemangioendothelioma (KH) is an endothelial-derived spindle cell neoplasm often associated with kasabach-merritt syndrome. Most cases arise in infancy and childhood and are soft-tissue tumors. The tumor displays an appearance between capillary hemangioma and Kaposi's sarcoma. We report a case of KH in a 1-year-old girl involving a mass that showed abnormal enhancement of soft tissue superficial to the right temporal bone with partial destruction of the temporal bone, the temporomandibular joint, mandibular condyle, and occipital bone. The physical finding of a discolored mass led clinicians to consider a hemangiomatous lesion, whereas the radiological picture suggested a more aggressive diagnosis of rhabdomyosarcoma and aggressive fibromatosis.
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ranking = 4
keywords = neck
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