• Tuberculosis and Respiratory Diseases
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• v.45 no.3
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• pp.604-608
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• 1998

Von Recklinghausen's disease is an autosomal dominant hereditary disease associated with characteristic cafe-au-lait spots of skin and multiple neurofibromatosis. It is complicated by malignancies, which in most cases is neurofibrosarcoma. The development of lung cancer in von Recklinghausen's disease is rare. A 61-year-old male was admitted for cough and sputum for 20 days. He had multiple cafe-au-lait spots and subcutaneous neurofibromas in whole body area and Lisch nodules in both iris and he had been diagnosed von Recklinghausen's disease 35 years ago. Chest radiography showed emphysematous bullae in both upper lung field and mass in right upper lung field. Chest CT scan revealed subcarinal lymph node enlargement. Bronchoscopic biopsy was done in mass in superior segment of right lower lobe and the results showed squamous cell carcinoma. The presence of von Recklinghausen's disease and lung cancer are noteworthy.

• Journal of Chest Surgery
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• v.21 no.4
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• pp.766-771
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• 1988

Von Recklinghausen`s neurofibromatosis, tuberous sclerosis and encephalotrigeminal angiomatosis[Sturge-Kalischer-Weber syndrome] are frequently classified under the heading of organic neurocutaneous syndromes. Both neurofibromatosis and tuberous sclerosis are believed to represent instances of simple autosomal dominant heredity. Multiple neurofibroma and cafe*-au-lait spots are the hallmarks of the van-Recklinghausen`s disease. The characteristic features of the fully developed syndrome are [1] pigmentation of the skin, including cafe*-au-lait spots, pigmented freckles and males, and occasionally a generalized darkening of the skin; [2] subcutaneous nodules and deep neurofibromatous tumors and diffuse plexiform growths of neural tissue; [3] skeletal anomalies, especially scoliosis; and [4] predilection to malignancy. In recent years cystic lung disease, usually of the so-called honeycomb lung variety, has been reported on several occasions in patients with tuberous sclerosis. This association has been shown to our sporadically as well as in members of a single family. Little attention has been paid to the presence of cystic lung disease in association with neurofibromatosis. Currently, most think of thoracic involvement in neurofibromatosis in terms of posterior mediastinal neuroma, pheochromocytoma, meningocele or, less commonly, parenchymal pulmonary neurofibromatosis. Author have experienced a case of von Recklinghausen`s disease. This case developed a huge neurofibroma in the both side thorax and invaded to the Lt. 7th rib.

• Journal of Chest Surgery
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• v.13 no.2
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• pp.149-153
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• 1980

Von Recklinghausen's disease is a systemic hereditary disorder with varied manifestations in bone, soft tissue, nervous system, and skin, the most common of which is the developement of multiple, small, cutaneous tumors with a characteristic histologic picture. Tumors develop after birth and before puberty in most cases, and they increase in number until old age. Malignant neoplasms that complicate multiple neurofibromatosis include gliomas of the optic nerve, astrocytomaas of the cerebral and cerebellar hemispheres, and sarcomas of peripheral nerves (femoral, tibial and intercostal nerves) and somatic soft tissues. Little attention has been paid to the presence of cystic lung disease in association with neurofibromatosis. Currently, most think of thoracic involvement in neurofibromatosis in terms of posterior mediastinal neuroma, pheochrocytoma, meningocele or, less commonly parenchymal pulmonary neurofibromas. Author have experienced 2 cases of Von Recklinghausen's disease. One case developed a hyge malignant Schwannoma in the parietal pleura of left 4th intercostal space and multiple benign neurofibromas (two in intercostal spacees and one in the neck) , and the other has several episodes of pneumothorax resulting from diffuse cystic lung disease which required closed thoracotomy drainage.

• Tuberculosis and Respiratory Diseases
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• v.47 no.4
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• pp.538-542
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• 1999

Spontaneous hemothorax may be developed as a complication of von Recklinghausen's disease. It is rare but fatal. A 60 year old man with von Recklinghausen's disease was admitted to our hospital because of left chest and shoulder pain. Radiograph of chest showed a massive left pleural effusion. Thoracentesis revealed gross blood. The peripheral angiography was done to determine the source of bleeding and its finding showed intercostal artery aneurysm in left 7th rib. No active bleeding from the aneurysm was seen. The source of the hemothorax was believed to be hemorrhage from rupture of intercostal artery aneurysm. He was inserted chest tube and treated embolization of intercostal artery aneurysm.

• Journal of Chest Surgery
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• v.1 no.1
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• pp.31-36
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• 1968

A case of funnel chest associated with von-Recklinghousen`s disease of the skin who was treated surgically under the method of sternoturn-over at the 63rd Army Hospital, Republic of Korea Army, is presented with review of the literature.

• Proceedings of the KOR-BRONCHOESO Conference
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• 1979.05a
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• pp.9.5-9
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• 1979

Somewhat less than 100 cases of neurogenic tumors of the larynx are reported in the literature. This is slowly growing tumor and is mostly schwannomas and neurofibromas, and could affect any age group. Since the first description of Von Recklinghausen's disease in 1882, about 20 cases of laryngeal involvement have been reported in the world literature. In this paper two cases of laryngeal neurofibroma will be presented: one congenital laryngeal neurofibroma in a 2-month-old boy, and one large laryngeal neurofibroma in Von Recklinghausen's disease in a 28 year old woman. The importance of conservative surgical procedure is discussed with. case presentation and literature review.

• Journal of Chest Surgery
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• v.29 no.1
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• pp.120-124
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• 1996

We report three cases of posterior mediastinal tumor in Von Recklinghausen's disease on 12-yearsy old boy, 8-years-old girl and 6-years-old boy. Especially the last case was combinded with "Dumbbell" neurilemmoma of cervical spine. In the first case, preoperative CT scan showed a homogeneous large mass In the posterior mediastinum. But the mass did not show intraspinal invasion on the chest MRI scan. In the last case, cervical spine CT with thecal enhancement shows dumbbell shaped neurofibroma, compressed spinal cord and thecal sac In three cases we removed all the masses successfully and the patients were discharged safely without significant problem.t problem.

• Journal of Chest Surgery
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• v.12 no.3
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• pp.165-169
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• 1979

Neurofibromatosis [Von Recklinghausen`s disease] is a rare Mendelian dominant disease, which shows multiple generalized symptoms and signs at various sites [Ex Skin, Bone, Nerve, Endocrine, Mediastinum rarely Lung, etc.]. We experienced one case of neurofibromatosis which has typical skin lesions [cafe-au-lait, multiple nodules, axillary freckling] with neurofibrosarcoma [malignant change from mediastinal lesion]. Patient was admitted our department because of recently developed severe dyspnea which was probably due to main tracheal compression by mediastinal neurofibrosarcoma. After successful removal of mediastinal mass dyspnea disappeared completely. Patient`s postoperative course was uneventful, and the patient was discharged 14 days after operation.

• Journal of Korean Neurosurgical Society
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• v.30 no.7
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• pp.925-927
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• 2001

Multiple intracranial tumors of different cell types are rare. We report a case of double primary intracranial tumors of different histologic types : oligodendroglioma, and pituitary adenoma. Von Recklinghausen's disease or other etiologic factors supposedly associated with multiple brain tumors were not recognized in this 36-year-old male patient.

• Maxillofacial Plastic and Reconstructive Surgery
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• v.11 no.2
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• pp.81-86
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• 1989

Tumors originated from peripheral nerve tissues are neurofibroma, neurilemoma, plexiform neurofibroma, malignant schwannoma, and granular cell tumor. Neurofibromas seem to occur in two forms : The first one is circumscribed solitary neurofibroma and the second group is neurofibromatosis or Von Recklinghausen's disease, which is a congenital and familial disease, presenting abnormalities of the skin, nerve system, bones and soft tissue. A solitary neurofibroma is a benign, relatively circumscribed, noncapsulated tumor which often presents in the skin and subcutaneous tissue as a soft sessile or pedunculated mass. It may occur anywhere in the head and neck, but the common site of the occurrence is the tongue, buccal mucosa, palate in frequency. Since solitary neurofibroma is a relatively radioresistant and its recurrence rate seem to be low, the treatment of choice is surgical excision. The author would like to present a case of unusually large solitary neurofibroma occured in the chin, which was successfully treated with surgical excision and reconstructed using deltopectoral flap and tongue flap.