• Journal of Korean Neurosurgical Society
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• v.59 no.2
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• pp.158-160
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• 2016

Ganglion cell tumors (GCT) are divided into two subtypes : gangliocytoma and ganglioglioma. Intramedullary gangliocytomas are extremely rare. A 20-year-old male patient with pain of neck, who also had a previously known neuroendocrine tumor of lung, was operated for mass found in the cervicomedullary junction with a presumptive diagnosis of metastases. Only partial resection could be performed. Pathological diagnosis had been reported as gangliocytoma. Only ten cases of intramedullary gangliocytoma have been reported in the literature. Although association with scoliosis and Von Recklinghausen;s disease were previously reported in the literature, no gangliocytoma case concomitant with endocrine tumor of lung have been published. Pathological study is the most important diagnostic method for gangliocytomas. Surgical excision is the primary treatment, but difficulty in total surgical tumor resection is the most important problem.

• Korean Journal of Head & Neck Oncology
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• v.9 no.1
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• pp.42-48
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• 1993

A group of 49 patients with neurogenic tumor in head and neck except intracranial tumor & Von-recklinghausen's disease was treated at the Department of Surgery. Presbyterian Medical Center during 12 years from January, 1980 to December, 1991. Of the 49 cases, 24 cases were neurilemmoma, 23 cases neurofibroma and 2 cases malignant schwannoma. The lateral cervical region was the commonest location of the neurogenic tumors, 24 cases (49%) arose from posterior triangle of neck and 12 cases from anterior triangle of neck. The origin of nerve was identified in 28 cases(57%). Cervical plexus(10 cases) and brachial plexus(7 cases) were most commonly affected. The most common site of neurogenic tumors arose from cranial nerve was anterior cervical triangle.

• Journal of Chest Surgery
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• v.28 no.10
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• pp.946-950
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• 1995

Neurosarcoma is relatively rare, but is seen frequently in association with stigmata of neurofibromatosis ie., Cafe au lait pigmentation and cutaneous neurofibromatosis. Both advanced age and association with neurofibromatosis increase the likelihood of malignant degeneration to neurosarcoma. It also may be dumb bell-shaped with intraspinal extension. Thoracic meningocele is rare and represents a protrusion of the dural sac through an abnormally large or malformed intervertebral foramen. The patient is usually asymptomatic, with evidence of paravertebral mass on the chest rentgenogram. In one operation we removed a "Dumb-bell" neurosarcoma and intrathoracic meningocele in a 52 year old woman using a cervical laminectomy with a trap-door incision. She was discharged at 17 days after the operation with a sequelae of excision of nerve roots C8 and T1. Postoperative radiation therapy was performed during the follow-up period.up period.

• Clinical and Experimental Pediatrics
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• v.48 no.4
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• pp.448-452
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• 2005

Neurofibromatosis(NF) is an autosomal dominant disorder, probably of neural crest origin that affects all three germinal layers. It is a group of heterogeneous multisystemic neurocutaneous disorders involving both neuroectodermal and mesenchymal derivatives. Type 1(von Recklinghausen disease) is the most common neurocutaneous disorder among the eight subtypes. Previous reports showed various involvements in the renal organ. Renovascular hypertension is the most common major manifestation of renal involvement in this disease. However, we experienced a case of ectopic kidney concurrent with neurofibromatosis type 1. The diagnosis of neurofibromatosis had been made by typical skin manifestation on physical examination, and ectopic kidney was discovered accidentally during routine abdominal sonography. The etiological basis of this association is not clear. We report a rare case of coexisting neurofibromatosis and ectopic kidney in a 7-year-old girl with a brief review.

• Korean Journal of Head & Neck Oncology
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• v.16 no.1
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• pp.87-91
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• 2000

Neurofibromatosis type I or Von Recklinghausen's disease can occur at any site in the body. It is characterized by multiple $c\'{a}fe\;\'{a}u\;l\'{a}it$ spots on the skin-more than six spots greater than 1.5cm-, neurofibromas of the peripheral and centarl nervous system, and variety of other dysplastic abnormalities of the skin, bones, endocrine organs, nervous systems, and blood vessels. It is an autosomal dominant trait disease with a frequency of 1 of 3000. Neurofibromatosis is known to be complicated by malignancies. Neurofibromatosis is progressive disease and shows a marked variations in expression in affected individuals. In this report we describe a male patient with neurofibromatosis type I developed in the parapharyngeal space. The patient had huge mass at left parapharyngeal space and inguinal area. We successfully treated the patient with surgery without complication.

• Journal of Korean Academy of Oral and Maxillofacial Radiology
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• v.24 no.1
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• pp.181-188
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• 1994

Neurofibromatosis, or Von Recklinghausen's disease is inhereted as an autosomal dominent neurocutaneous systemic disease. It is characterized by multiple Cafe-au-lait spots, generlaized cutaneous neurofibromas. It affects one in 3000 births. We observed the clinical, radiologic and histopathologic findings of 3 cases of neurofibromatosis and obtained following results. 1. All patients had multiple Cafe-au-lait spots and neufibromas. 2. Two patients had radiographic changes of pressive erosion and mesodermal dysplasia. 3. Two patients had plexiform neurofibromas and 1 patient had diffuse neurofibromas. Conclusively, we classified these 3 cases as NF-I.

• Radiation Oncology Journal
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• v.10 no.1
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• pp.77-83
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• 1992

The incidence of malignant schwannoma in the head and neck is extremely rare. Most tumors appear as a rapidly expanding nonpainful mass and the symptoms are usually attributable to local expansion of the mass. About one half occurs in association with von Recklinghausen's disease. Wide surgical excision is generally recommended as a primary treatment. Recently, there has been a trend to include postoperative radiation therapy as a primary modality. Prognosis of head and neck malignant schwannoma has been reported as particularly poor, However, recent authors advocate that prolonged survival is possible after adequate therapy including postoperative radiation therapy. We present our experience with these tumors on very rare locations such as the ethmoid sinus and the larynx, with the review of literatures.

• Journal of Yeungnam Medical Science
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• v.5 no.2
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• pp.195-200
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• 1988

The incidence of multiple meningiomas found prior to the introduction of CT was 1~2%, the incidence since the introduction of CT was 8.9%. The authous report a case of multiple meningiomas without evidence of von Recklinghausen's disease. The possible presence of more than 1 tumor in a patient with an intracranial meningiomas must always be kept in mind. The pathogenetic factors related to true multiple meningiomas is unknown. We report a patient with multiple meningiomas which was confirmed by operation.

• Advances in pediatric surgery
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• v.2 no.2
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• pp.88-93
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• 1996

A clinical analysis was made of 42 pilomatricoma in 34 children treated, from January 1980 to August 1996 at the Department of Pediatric Surgery, Hanyang University. The gender ratio was 13 to 21 with female preponderance. The average age at presentation was 7 years 3 months(range 8 months to 19 years old). The time average interval from onset to excision was 9.8 months. The predilection site was neck, upper extremities, face, trunk and lower extremities in the decreasing order of frequency. The masses were less than 3cm in diameter and there was no correlation between age at presentation and size of the mass. Of the six children with multiple lesions, two presented with multiple lesions initially and four presented with second lesion metachronously after excision of the primary lesions. Two girls had cysticercosis and von Recklinghausen's disease respectively in association with a pilomatricoma. All lesions were encapsulated subcutaneous masses which were clearly distinguished from surrounding tissue, and 4 of them had pigmentation, ulceration, skin disruption and hemorrhagic changes in the overlying skin. Microscopically the mass was composed of basophilic cells and eosinophilic shadow cells. Calcification, foreign body giant cell, ossification, focal cystic, inflammatory or necrotic changes were also seen.

• Journal of Korean Neurosurgical Society
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• v.30 no.9
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• pp.1127-1129
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• 2001

The vast majority of schwannomas occur on cranial nerves, and rarely in the retroperitoneum. Solitary schwannoma in the psoas muscle is extremely rare. The authors present a case of retroperitoneal neoplasm in the psoas muscle identified as schwannoma which is not associated with von Recklinghausen's disease. A 68 years old female patient was admitted because of low back pain and weakness at the left leg. CT and MRI revealed a large cystic mass with well-defined margin and multiple internal septation within the left psoas muscle. The tumor was totally extirpated and histologically confirmed as a schwannoma.