• Tuberculosis and Respiratory Diseases
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• 제45권3호
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• pp.604-608
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• 1998

Von Recklinghausen병에 동반되는 악성종양은 주로 신경섬유육종이지만 드물게는 폐암이 동반되는 경우가 보고되고 있고 ras 유전자가 양 질환의 발생에 영향이 있음이 알려져 있어 앞으로 이에 관한 연구가 필요하리라 생각된다.

• Journal of Chest Surgery
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• 제21권4호
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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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• 제13권2호
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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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• 제47권4호
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• pp.538-542
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• 1999

von Recklinghausen 병을 진단 받았거나 임상적으로 의심되는 환자에서 외상력없이 갑자기 시작된 흉통을 주소로 내원하여 흉부 X-선 소견상 흉수 소견이 관찰될 때 동맥파열에 의한 자발성 혈흉의 가능성을 의심할 수 있다. 저자들은 von Recklinghausen 병에 드물게 동반되는 늑간동맥류 파열에 의한 자발성 혈흉 1예를 경험하였기에 문헌고찰과 함께 보고하는 바이다.

• Journal of Chest Surgery
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• 제1권1호
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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.

• 대한기관식도과학회:학술대회논문집
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• 대한기관식도과학회 1979년도 제13차 학술대회 연제순서 및 초록
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• pp.9.5-9
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• 1979

후두에 발생한 신경성종양은 극히 드물 질환이며 신경초종 혹은 신경섬유종이 나타난다. 현재까지 약 100례 이하의 신경성종양의 보고가 있으며 이들중 신경섬유종양과 동반된 예는 20례에 불과하다. 주 증상으로는 호흡곤란, 발성장애와 언어장애등이 있으며 어느 연령에나 발생할 수 있다. 저자들은 2례의 후두의 신경섬유종을 보고하고자 한다. 증례 1은 선천성후두신경섬유종으로 2개월된 남아는 생후즉시로 부터 심한 호흡곤란과 발성장애가 있었으며, 증례 2는 28세의 여자에서 다발성신경섬유종증(von Recklinghausen's disease)에 동반하여 후두에 신경섬유종이 발생한 예이다. 본 논문에서 자자들은 증례보고와 함께 문헌고찰을 하고자 한다.

• Journal of Chest Surgery
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• 제29권1호
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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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• 제12권3호
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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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• 제30권7호
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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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• 제11권2호
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• pp.81-86
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• 1989

말초 신경 조직에서 발생되는 종양은 신경섬유종, 신경초종, 파립세포종, 악성신경초종 등이 있다. 신경섬유종은 2가지 형태로 분류될 수 있다. 첫째 형태는 단독형 신경섬유종이고, 둘째는 신경섬유종 혹은 Von Recklinghausen's병으로 불리우는데 이는 주로 피부, 신경조직, 뼈, 연조직에 기형을 유발할 수 있고, 선천적이며, 가족력을 나타낸다. 단독형 신경섬유종은 양성이며, 경계는 명확하나 capsule에 의해 싸여 있지 않으며, 피부나 피하조직에 sessile 혹은 pedunculated 형태로 나타난다. 두경부의 어느 부위에나 나타날 수 있으며, 구강내의 호발 부위는 혀, 협점막, 구개 순이다. 단독형 신경섬유종은 방사선에 의한 치료에 효과가 적으며, 재발율이 낮고, 치료는 주로 외과적 절제술로 제거를 하고, 결손부를 삼각흉피판(D-P flap)과 설피판(tongue flap)으로 수복하여 현재까지 합병증 없이 양호한 결과를 얻었다.