Cases reported "Neurofibromatosis 1"

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1/71. Malignant schwannoma of the stomach in a patient with von Recklinghausen's disease.

    A patient with von Recklinghausen's disease died with a malignant schwannoma of the stomach and was found at autopsy to have neurofibromatosis of the gastrointestinal tract, a plexiform neurofibroma of the myocardium and a phaechromocytoma. Malignancy of the gastrointestinal tract in von Recklinghausen's disease is rare, and this case highlights the difficulties in histological diagnosis of malignant nerve sheath tumours.
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keywords = schwann
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2/71. Malignant schwannoma with melanocytic differentiation arising in a patient with neurofibromatosis.

    A 50-year-old woman with von Recklinghausen's disease, but not Carney's complex, presented with a 1-year history of a hard subcutaneous mass on her right hip and right inguinal lymphadenopathy. Histological and immunohistochemical studies of the tumour revealed schwannian and melanocytic characteristics. Local recurrence without distant metastases was observed 5 years later. Although the diagnosis of malignant schwannoma with melanocytic differentiation, rather than neurotropic melanoma, was made for the primary tumour, based on the clinicohistopathological and ultrastructural findings, the overall clinical course in this case did not seem incompatible with malignant melanocytic schwannoma.
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keywords = schwann
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3/71. Malignant schwannoma of the sciatic nerve originating in a spinal plexiform neurofibroma associated with neurofibromatosis type 1--case report.

    A 26-year-old man with neurofibromatosis type 1 (NF1) presented with a giant malignant schwannoma of the sciatic nerve. The differential diagnosis of malignant peripheral nerve sheath tumor (MPNST) was based on clinical, radiological, and histological evidence. The tumor apparently originated in a spinal plexiform neurofibroma. The lesion was resected totally without neural damage to the sciatic nerve. However, the tumor recurred within 2 months. The patient died of unknown factors probably associated with the spinal involvement. MPNST associated with NF1 has a poor prognosis due to recurrence or metastasis despite complete macroscopic removal.
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keywords = schwann
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4/71. Pigmented (melanotic) neurofibroma. Report of an unusual case with immunohistochemical, ultrastructural and cytogenetic analyses.

    In the spectrum of neurofibromas, pigmented tumors are rare variants usually showing only faint, macroscopically obvious pigmentation. We report a case of a huge pigmented neurofibroma with extended, macroscopically striking pigmentation in a patient with stigmata of neurofibromatosis. The immunohistochemical and ultrastructural findings support a melanotic line of differentiation besides schwann cell differentiation and indicate a phenotypic neoplastic spectrum between tumorous schwann cells and melanocytes. Using comparative genomic hybridization, striking chromosomal aberrations were not detected. High level amplifications of the known chromosomal regions, including genes of major enzymes responsible for melanin synthesis, appear to be unlikely. However, smaller chromosomal defects might have been overlooked by the limited resolution of this screening method. Therefore, other mechanisms up-regulating melanogenesis, such as mutations in regulatory genes, have to be considered.
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ranking = 1.6540957720252
keywords = schwann cell, schwann
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5/71. Pancreatic schwannoma: an uncommon but important entity.

    The literature contains five single case reports of pancreatic schwannoma-two of the five occurred in patients with von Recklinghausen's disease, and three of the five proved malignant. Within a 3-month period, we resected benign pancreatic schwannomas in two patients without von Recklinghausen's disease. Both patients presented with pain that led to the discovery of a complex pancreatic mass on abdominal CT scan. Pancreatic schwannoma should be included in the differential diagnosis of cystic or solid pancreatic abnormalities on imaging studies.
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6/71. Malignant triton tumor of the right vagus.

    We report on the successful surgical resection of a mediastinal malignant triton tumor of the vagus, an exceedingly rare tumor in this location. Malignant triton tumor is a subtype of malignant peripheral nerve sheath tumors, in which the characteristic histologic finding is of rhabdomyoblastic differentiation among schwannoma cells. A 35-year-old man with associated neurofibromatosis type-1 underwent surgical resection and has been followed up for 18 months.
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keywords = schwann
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7/71. Recurrent malignant schwannoma of the parapharyngeal space in neurofibromatosis type 1.

    Malignant schwannoma is an aggressive tumor that carries a poor prognosis despite wide excision, chemotherapy, and radiotherapy. Malignant schwannoma of the parapharyngeal space is an uncommon finding; to our knowledge, only four cases have been described in the literature during the past 30 years, and only one of them involved a patient who had clinical evidence of neurofibromatosis type 1. In this article, we describe a new case of malignant schwannoma of the parapharyngeal space in a patient who had clinical evidence of neurofibromatosis type 1. Following resection of the tumor and a total parotidectomy, the diagnosis was made on the basis of histology and immunohistochemistry. The patient underwent postoperative chemotherapy with carboplatin and UP16. However, 5 months following surgery, the tumor recurred and metastasized. The patient was then placed on a different polychemotherapeutic regimen, which was made up of 3 g/m2 of ifosfamide, 1.5 mg/m2 of vincristine, and 1.5 mg/m2 of doxorubicin (IVA2 protocol). The IVA2 regimen slowed tumor growth, but 13 months after the initiation of therapy, the patient died of neoplastic cachexia. Although chemotherapy is generally ineffective in most cases of malignant schwannoma, we did experience some positive results with the IVA2 protocol. Therefore, we recommend that this combination be considered as a first-line adjuvant therapy following surgery or as a first-line therapy for patients with inoperable tumors.
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keywords = schwann
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8/71. Malignant Triton tumor. A case with protean histopathological patterns.

    A case of malignant Triton tumor occurring in the leg of a 48-year-old woman with neurofibromatosis (von Recklinghausen's disease) is presented. The neoplasm was composed mainly of spindle-shaped cells forming interlacing fascicles with areas of massive necrosis. The important feature in this neoplasm was the presence of rhabdomyoblastic cells admixed with other cellular components. In addition, variable histologic features including solid, myxomatous, hemangiomatous, hemangiopericytomatous, epithelioid, and lymphomatous areas were identified. This variety has been recognized in malignant schwannomas, but not in malignant Triton tumors. There were discrete foci with each predominant histologic feature shown in a map of the neoplasm in which the distribution of those components is demonstrated based on the histology and immunohistochemistry. Satisfactory biopsy specimens are necessary for accurate diagnosis of malignant Triton tumor.
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keywords = schwann
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9/71. Acute monocular blindness resulting from transformation of von Recklinghausen's neurofibromatosis to malignant melanocytic schwannomas.

    A 59-year-old male was transferred to our facility after initial workup for left eye blindness revealed multiple brain lesions. The patient presented with scant pedunculated skin lesions on his neck and arms, axillary freckling and bilateral subcutaneous ankle nodules suggestive of neurofibromatosis type 1 (NF-1). Neurological examination revealed left-sided blindness, diminished pupillary response to light, incomplete eyelid closure and facial droop. A CT series of the chest/abdomen/pelvis demonstrated several lesions in the lungs, liver and left kidney. CT-guided biopsy of the renal lesion was "consistent with metastatic melanoma" based on the presence of malignant cell staining for the melanocyte differentiation antigen MART-1, as well as the calcium binding protein s-100. A dermatology consultant confirmed neurofibromas and a cafe au lait macule. No dysplastic nevi or primary melanoma were identified. An ophthalmology consultant noted gray choroidal lesions on fundoscopic examination thought to be metastatic disease of unknown primary. Despite treatment with whole brain irradiation and intravenous steroids, the patient died on hospital day 10. A full autopsy showed diffuse neurofibromas and cafe au lait macules consistent with NF-1 and malignant melanocytic schwannomas with multiple metastases.
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keywords = schwann
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10/71. Spontaneous rupture of recurrent gastrointestinal stromal tumor associated with neurofibromatosis type 1.

    The incidence of gastrointestinal stromal tumor (GIST) among neurofibromatosis type 1 (NF-1) patients is approximately 3.9-25%, and this relationship is generally considered to be non-coincidental. We report a patient with NF-1 who underwent laparotomy 3 times due to recurrent intra-abdominal tumor rupture with internal bleeding in the space of 13 years. The pathologic diagnoses were schwannoma, malignant peripheral nerve sheath tumor and GIST. Because of the similar histologic features of these tumors, we considered them to be of the same nature. Immunohistochemical staining can help in the differential diagnosis. We suggest that NF-1 patients with gastrointestinal symptoms receive further survey to rule out GISTs.
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ranking = 0.2
keywords = schwann
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