Cases reported "Neurofibroma"

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1/9. March 2002: 28-year-old woman with neck and back pain.

    Following a car accident a 28-year-old female, complained of a sharp pain of the anterior and posterior base of the neck on expiration and with exertion. Subsequently, she noticed a feeling of discomfort in her back when lifting her arm above her head. Imaging studies revealed a tumor mass involving the third intercostal nerve on the right side of T2. The differential diagnosis included neurofibroma and neurilemmoma. This was followed annually and five years later an increase in size warranted a transthoracic, transpleural removal en bloc of this lesion. At surgery, a 3 cm soft tissue tumor engulfed the third intercostal nerve and extended into the third intervertebral foramen where the proximal part of the nerve root was enlarged. The right third intercostal nerve was dissected and removed along with the tumor, after negative nerve stimulation. Histopathological examination showed multiple enlarged coalescent lymphoid follicles with an onion skin appearance of tight concentric layering of small, uniform mature lymphocytes at the periphery, arranged in a targetoid fashion with broad mantle zones and relatively small germinal centers. The germinal centers of variable size included hyalinized blood vessels. Lollipop follicles were seen. The interfollicular stroma showed numerous hyperplastic collagenized capillaries within an inflammatory background. However, the perinodal soft tissue was replaced by numerous inflammatory cells, primarily lymphocytes. The final diagnosis was Castleman's disease, hyaline vascular type. Castleman's disease can mimic various tumors and because Castleman's disease is a rare reactive entity, its diagnosis is generally overlooked by radiologists and clinicians. It is likely that this mass arose from one of the posterior intercostal lymph nodes, situated in the paravertebral region, however the capsule was not readily seen and the sinuses were not apparent. Almost all previous cases of Castleman's disease, hyaline vascular type were described in the anterior mediastinum. Hyaline vascular Castleman's disease usually does not invade and replace neighboring structures. This case is unique because of its location and the local invasion of adjacent structures.
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2/9. Isolated neurofibroma of the orbit with extensive myxoid changes: a clinicopathologic study including MRI and electron microscopic findings.

    Isolated orbital neurofibroma (INO) without systemic neurofibromatosis is rare. Previous reports including MRI with elaboration of the ultrastructural features of the cellular and extracellular components in INO do not exist. A 44-year-old male presented with painless, slowly progressive proptosis (6 mm) and downward displacement (3 mm) of the left eye of two years duration. MRI revealed a suprabulbar mass hypointense to fat, isointense to brain on T1-weighted images with small hyperintense areas following contrast enhancement. T2-weighted images showed a predominantly hyperintense mass with a few hypointense islands. The lesion was excised using an anterior superior transseptal orbitotomy. Histopathologically, large areas of a paucicellular myxoid stroma containing mucopolysaccharides made up approximately three-quarters of the mass. Focal hypercellular areas showed marked collagen deposition. blood vessels were distributed at the periphery of the lesion and in association with nodules of nerve sheath cells. Ultrastructurally, perineural and fibroblast-like cells were loosely arranged in a myxoid stroma. schwann cells were embedded in a dense collagenous matrix. Variable differentiation of schwann cells, perineural cells and fibroblast-like cells associated with a more solid (collagenous) or loose (myxoid) extracellular matrix may determine the characteristic, though not specific, MRI features in isolated neurofibromas of the orbit.
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3/9. Superficial femoral vein invasion by a benign neurofibroma in a non-neurofibromatosis patient: case report.

    OBJECTIVE AND IMPORTANCE: Neurofibromas are benign neural sheath tumors arising from intraneural supporting cells. Such tumors are characteristic of neurofibromatosis Type I (von Recklinghausen disease) but also occur sporadically. Vascular involvement by neurofibromata is rare, but has been described in the past in the context of neurofibromatosis. There is, to our knowledge, no description of vascular involvement by a neurofibroma in a non-neurofibromatosis patient. CLINICAL PRESENTATION: A 40-year-old woman presented with a 4 year history of a right thigh mass associated with diffuse lower extremity pain. She had no other clinical manifestations of neurofibromatosis and no known family member with neurofibromatosis Type I. magnetic resonance imaging scans revealed a well-defined solid mass in the anteromedial aspect of the right thigh closely associated with the superficial femoral vein. INTERVENTION: The vessel segment and encapsulated mass were resected "en bloc" after proximal and distal ligation of the vein. The pathological appearance of the mass was consistent with a benign neurofibroma that had infiltrated all layers of the vessel. CONCLUSION: Vessel invasion by a benign sporadic neurofibroma is a rare occurrence with potentially severe implications for the patient. It suggests that surgical removal of asymptomatic benign-appearing lesions of that type should be considered if they are adjacent to important anatomical structures.
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4/9. A clinicopathological study on soft tissue tumors of the head and neck.

    The present study consists of 651 cases of soft tissue tumors originating in the head and neck area. Among these tumors 628 were benign (96%), while there were only 23 malignant cases (4%). In the benign group hemangiomas showed the highest incidence (47%) followed by tumors of adipose tissue (19%) and those of peripheral nerves (12%). Rare tumors consisted of nodular fascitis, hibernoma, lipoblastomatosis, glomus tumor, granular cell tumor, and chondroma. The majority of cases in the malignant group were either those of muscle origin or blood vessel origin. Excepting for two cases of rhabdomyosarcoma of the embryonal type and two cases of angiosarcoma, all showed recurrence and/or metastasis and terminated in death. Three cases of liposarcoma of either well-differentiated or myxoid type had a good prognosis with all three living for 3 or more years without any recurrence. Other malignant tumors consisted of malignant schwannoma, fibrosarcoma, malignant fibrous histiocytoma, alveolar soft part sarcoma, and chordoma. The age, sex, anatomical location, and differential diagnosis have also been described.
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5/9. Plexiform neurofibroma of the pelvis in neurofibromatosis: CT findings.

    Three cases of intrapelvic plexiform neurofibromas are presented in patients with neurofibromatosis. In all three cases computed tomography demonstrated widespread sheets of nodular tissue lying in an extraperitoneal location in front of the sacrum or extending along the pelvic sidewalls . Symptomatology in these patients related to compression of either nerve roots or adjacent vessels. It is important to differentiate these lesions from pelvic lymphadenopathy to prevent a mistaken diagnosis of malignant disease.
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6/9. Spinal neurilemmomas and neurofibromas: central dot sign in postgadolinium MRI.

    The MR studies of three histologically proven spinal neurilemmomas and neurofibromas were reviewed retrospectively. There were two benign neurilemmomas (schwannomas) and one neurofibroma. The common characteristic of these cases was a central low intensity focus ("dot") seen on postcontrast T1-weighted imaging. The low intensity foci corresponded histologically to a congeries of changes including edema, microcysts, foam cells, hyalinization of blood vessels, old hemorrhage, and dystrophic calcification.
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7/9. Diffuse neurofibroma obstructing the external auditory meatus.

    A case is presented of a 36-year-old male with narrowing of the external meatus due to a diffuse neurofibroma. This unusual variety of neurofibroma spreads superficially and has many ecstatic blood vessels. The size, vascularity, uncertain edges and a tendency to recur makes surgical removal difficult. The treatment options are discussed.
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8/9. magnetic resonance imaging of subcutaneous diffuse neurofibroma.

    A 31-year-old woman presented with increasing pain and tenderness of a long-standing soft tissue mass on her back. MRI showed a network of interconnecting tubular areas, which were T1 isointense and T2 hyperintense relative to skeletal muscle, and displayed marked Gd-DTPA enhancement. The lesion was situated within the subcutaneous fat. Clinically and radiologically, this mass was considered to be a subcutaneous venous haemangioma. Histological examination of the excised mass showed a diffuse neurofibroma with ectatic vessels and entrapped adipose tissue. Similar MRI appearances of subcutaneous haemangioma and diffuse neurofibroma may result in failure to make the correct diagnosis and in inappropriate management.
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9/9. Diffuse neurofibroma of the ankle.

    PURPOSE: To correlate the imaging and histological findings in diffuse neurofibroma. patients AND methods: Retrospective review of clinical, imaging and histological findings in two patients with diffuse neurofibroma. RESULTS: CT demonstrates diffuse infiltration of the deep and subcutaneous fat, isodense to muscle. magnetic resonance imaging shows extensive infiltration of the subcutaneous and deep fat that envelops tendons and vessels but does not involve bone. Superficial masses enhance homogeneously after intravenous gadolinium. The reticular nature of the deep infiltration is seen on all sequences but is most conspicuous on post-gadolinium T1-weighted images which show tumour enhancement and non-enhancing hypointense soft tissue strands. magnetic resonance angiography and Doppler ultrasound may show enlarged vessels, high blood flow and vascular pools. CONCLUSION: Diffuse neurofibroma has a characteristic appearance on magnetic resonance that is best shown on post-gadolinium T1-weighted images.
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