Cases reported "Neurofibromatosis 1"

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11/86. gliosarcoma associated with neurofibromatosis type I: a case report.

    Neurofibromatosis type I (NF I) is the most common hereditary syndrome predisposing to neoplasia. We report the third case in the literature, documenting the combination of gliosarcoma with NF I. The patient's son was known at our center because of a history of pleomorphic xanthoastrocytoma (PXA) with NF I. A 48-year-old man who had a number of cafe-au-lait spots with neurofibroma since birth presented with severe headache. Neuroradiological studies revealed a cystic tumor of the right temporal lobe of high grade nature. Surgical excision was performed and the tumor was found to be located on the surface of the temporoparietal area with cystic formation and vascular and infiltrative features. Postoperative MRI Key words: gliosarcoma, neurofibromatosis I, temporal lobe. showed no detectable contrast enhancing tissue. Immunohistochemical examination evidenced the characteristics of typical gliosarcoma. The patient received radiation therapy but five months following surgery recurrence of the tumor was diagnosed. reoperation was performed and histopathological studies confirmed the diagnosis of gliosarcoma. We believe that the neurofibromatosis was inherited by the son with PXA from the father with gliosarcoma. The rarity of the combined occurrence of gliosarcoma and NF I, in addition to this uncommon family history, makes this case remarkable. Our findings suggest that NF I is a multifaceted disease associated with benign as well as malignant astrocytic tumors.
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12/86. Adjunctive tumescent technique in massive resections.

    Tumescent technique for liposuction is a widely used technique. Four cases are reported here in which this technique was used to aid in the resection of massive neuromatous lesions with good postoperative results and minimal morbidity. review of the relevant literature is done.
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13/86. paraparesis after excision of intrathoracic meningoceles in a patient with neurofibromatosis.

    Intrathoracic meningocele associated with neurofibromatosis is a rarity. We treated a 16-year-old boy with neurofibromatosis, marked kyphoscoliosis, and two right-sided intrathoracic meningoceles. Because his chief complaints of cough and chest pain were thought to be caused by the meningoceles, resection of these lesions was performed prior to correction of the spinal deformity. On the day after the resection, complete paraplegia developed, followed by recovery to paraparesis. Decompressive lumbar puncture was performed, but intraspinal pressure was normal. Postoperative spinal cord damage and consequent paresis may have resulted from a loss of pressure buffering by the meningocele, which rendered the cord vulnerable to injury. The possibility of a similar unusual complication should be borne in mind when treating patients with intrathoracic meningocele associated with neurofibromatosis.
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14/86. Management issues in massive pediatric facial plexiform neurofibroma with neurofibromatosis type 1.

    BACKGROUND: Plexiform neurofibroma is a relatively common but potentially devastating manifestation of neurofibromatosis type 1 (NF1). Surgical management is the mainstay of therapy, but within the head and neck region it is limited by the infiltrating nature of these tumors, inherent operative morbidity, and high rate of regrowth. METHOD: We describe a case of a 7-year-old girl with neurofibromatosis type 1 and a massive facial plexiform neurofibroma with the aim of emphasizing the treatment and timing issues involved in the management of this difficult problem. A medline search (1966 through December 2000) was carried out, and pertinent literature on the subject was reviewed. RESULT: The patient described in this case report was carefully observed for a period of 6 years from diagnosis before surgical excision of the tumor was undertaken with an uneventful recovery. CONCLUSION: Surgical management remains the mainstay of treatment for these locally invasive tumors, but functional disturbances are almost inevitable in resecting substantial tumors involving the head and neck region. The indication and timing of surgery in pediatric patients therefore needs to be carefully weighed against the physical and psychologic consequences of treatment.
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15/86. Hypertrophic scars after therapy with CO2 laser for treatment of multiple cutaneous neurofibromas.

    BACKGROUND: CO2 laser surgery is a treatment modality for cutaneous neurofibromas. OBJECTIVE: Hypertrophic and atrophic scars can result from treatment with CO2 laser surgery. We present a case of cutaneous neurofibromatosis that developed hypertrophic scars postoperatively. methods: Continuous wave CO2 laser surgery therapy was applied to the patient. RESULTS: Hypertrophic scars developed 2 months after therapy. CONCLUSION: With a preliminary test treatment the patient is able to see the expected result.
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16/86. Middle aortic syndrome: distal thoracic and abdominal coarctation, a disorder with multiple etiologies.

    BACKGROUND: Congenital coarctation of the thoracic aorta at the ligamentum arteriosum or the aortic arch is well recognized. But a much less common variety (0.5% to 2.0%) of aortic coarctation is located in the distal thoracic aorta, or abdominal aorta, or both and is often called "middle aortic syndrome" or "mid-aortic dysplastic syndrome." These types of aortic coarctation are most often secondary to a form of granulomatis vasculitis commonly known as Takayasu's disease in this country or aortitis syndrome in japan. No single genesis explains every case and beside vasculitis as a cause, some are thought to be congenital in origin and others are associated with von Recklinghausen's disease. STUDY DESIGN: Eight patients with severe hypertension or claudication secondary to middle aortic coarctation were studied with aortograms and subsequently treated by vascular reconstruction procedures. RESULTS: Vascular reconstructions consisted of aortoaortic bypass, aortic resection with interposed grafting, reanastomotic resection of renal arteries into prosthetic grafts, and renal artery bypass with autogenous material. All eight patients' grafts have remained patent, with followups of 4 to 9 years, with relief of hypertension and claudication. Although Takayasu's disease can be progressive, aggressive surgical treatment in eight patients followed for 4 to 8 years postoperatively demonstrates that severe hypertension, claudication, or both are important indications for revascularization. CONCLUSIONS: Whatever the cause, assuming that active aortic inflammation has been medically treated and is in a burned-out state, patients with abdominal coarctation who have symptomatic renovascular hypertension, claudication, or both are good candidates for revascularization. Although surgical repair is more difficult than with congenital thoracic coarctation, because aortic walls are fibrotic and often also involve the renals, all eight of our patients had successful longterm correction of their hypertension and coarctation.
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17/86. Rapid expansion of benign scalp neurofibroma caused by massive intratumoral hemorrhage--case report.

    A 62-year-old man with neurofibromatosis type 1 presented with rapid growth of a scalp mass. Head computed tomography demonstrated a large extracranial tumor of soft tissue density with massive intratumoral hematoma. cerebral angiography demonstrated remarkable hypervascularity of the tumor. Preoperative embolization and total removal of the tumor was performed. The tumor contained a large amount of intratumoral hematoma (500 ml). The histological diagnosis was neurofibroma without malignant transformation. Benign scalp neurofibroma showing massive intratumoral hemorrhage is rare. Rapid growth or intratumoral hemorrhage in neurofibroma may be an indicator of malignant transformation.
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18/86. A neurofibromatosis type 1 patient with severe kyphoscoliosis and intrathoracic meningocele.

    The patient presented with neurofibromatosis and a dystrophic kyphoscoliosis around the cervico-thoracic junction. When the patient was 59 years old, he started to suffer from dyspnea caused by an intrathoracic meningocele in the upper left thoracic cavity. A wide laminectomy from T2 to T5 was performed and the meningocele was resected. Although the dyspnoea disappeared postoperatively, the patient started to neurologically deteriorate. laminectomy alone caused instability around the apex of the kyphosoliosis and spinal cord compression. Halo cast was applied and brought remarkable recovery of neurologic deficits. This result encouraged us to perform posterior fusion in situ from C3 to L2 with bone graft from the iliac crests and the Luque technique in conjunction with the Isola system. This resulted in the patient being able to walk again. The removal of the posterior element predisposes the patient to unstable postlaminectomy kyphosis and removes valuable bone stock required for posterior spinal fusion. For this reason, spinal fusion should have been conducted during surgery for the patient's meningocele.
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19/86. Pulmonary sarcoma complicated with neurofibromatosis type 1 (NF1).

    We report here a case of primary pulmonary sarcoma complicated with neurofibromatosis type 1 (NF1). A 60-year-old man was urgently admitted to Tokai University Hospital, japan, due to severe right chest pain. skin inspection showed subcutaneous tumor lesions and cafe-au-lait spots, and therefore a diagnosis of neurofibromatosis type 1 (NF1) was made. Chest X-ray showed marked fluid accumulation in the right pleural cavity, and he was diagnosed as having empyema by thoracic drainage. Chest X-ray showed a large round mass lesion (80 mm in diameter) in the right lower lung field. Percutaneous needle biopsy of the tumor revealed that the empyema was caused by actinomycotic infection, and then he underwent right middle and lower lobectomy. Histopathological examination of the surgical specimens revealed features of spindle cell sarcoma. Several postoperative systemic examinations showed no other lesions except in the right lung. We concluded that this was a complicated case of primary pulmonary sarcoma with NF1.
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20/86. Familial neurofibromatosis-1 and Gilbert syndrome.

    BACKGROUND: Neurofibromatosis (NF) is a genetic disorder of the neural sheet, affecting almost all organ systems. METHOD: A family that consisted of the father and his three children, two males and one female, having NF-1 is evaluated in detail for cosmetic purposes. The routine preoperative laboratory examinations, including complete blood counts, serum biochemistries, urinary analysis, extremity and chest roentgenograms, ultrasounds of the abdomen and the affected regions, and pelvic computed tomographies, revealed characteristics of NF-1 and unexpected hyperbilirubinemia. The patients are evaluated further for the cause of hyperbilirubinemia by the fasting test. Variously localized plexiform neurofibromas of the three children are totally excised. RESULTS: The father was affected as a result of a mutation, and his three children had inherited the disease. The father, having subcutaneous neurofibromas, differs from the children, as they have elephantiasis neurofibromatosa. The children had various rare clinical presentations besides disfiguring deformities. Two male patients had retroperitoneal neurofibromas, and one had splenomegaly and osseous changes. Gilbert's disease was diagnosed, interestingly, and coincidentally in the three patients with elephantiasis due to NF-1. DISCUSSION: An interesting diagnosis of NF occurring coincidentally with Gilbert's disease in a family is reported. This clinical study is the first report of Gilbert's disease accompanying familial NF.
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