• Nuclear Medicine and Molecular Imaging
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• v.43 no.4
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• pp.361-362
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• 2009

We present a patient with high $^{18}$F-fluorodeoxyglucose (FDG) uptake detected in a neurofibroma that was confused with sarcomatous transformation on a positron emission tomography/computed tomography (PET/CT) scan. A 39-year-old male patient with a 20-year history of neurofibromatosis-1 (NF-1) performed FDG PET/CT scan for the evaluation of lesions with sarcomatous transformation. The FDG PET/CT images demonstrated varying degrees of increased FDG uptake in the multiple nodules throughout whole body. The left pelvic mass with the highest FDG uptake had a maximum standardized uptake values (maxSUV) 5.0 and surgical resection was performed. Histological analysis confirmed the presence of a benign neurofibroma infiltrated with inflammatory cells.

• Clinical Pain
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• v.18 no.2
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• pp.97-101
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• 2019

Tardy ulnar nerve palsy is ulnar neuropathy at or around elbow and commonly evaluated in the electromyography laboratory. However, ulnar neuropathy at the elbow due to neurofibroma is rare. Neurofibromas are tumors that arise within nerve fasciculi and anywhere along a nerve from dorsal root ganglion to the terminal nerve branch. We report one case of ulnar neuropathy at the elbow due to neurofibroma. Patient had paresthesia on the left 5th finger and there had been left hypothenar atrophy since 2 months ago. Tinel's sign was positive at left elbow. As a result of electromyography, there were suggestive of right ulnar neuropathy at or around elbow, referred to as tardy ulnar nerve palsy. Ultrasonography showed a diffuse tortuous thickening with multiple neurofibromas arising from individual fascicles of the ulnar nerve in cubital tunnel area. Surgery was then performed to release cubital tunnel of left elbow, then the patient's symptoms improved.

• Journal of Chest Surgery
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• v.21 no.6
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• pp.1103-1110
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• 1988

We experienced 1 year old female patient with partial anomalous pulmonary venous return of the right lung into the inferior vena cava with atrial septal defect. Total anomalous venous drainage from the right lung is an uncommon form of congenital defect, and it may or may not be associated with an atrial septal defect. One patient having this venous anomaly is presented with a detailed description of the surgical reconstruction used. The postoperative result has been satisfactory.

• Maxillofacial Plastic and Reconstructive Surgery
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• v.14 no.4
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• pp.315-321
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• 1992

Tumors arising from nerve tissue are uncommon in the oral maxillofacial regions. Neurofibroma, a benign neurogenic tumor, can occur as circumscribed solitary or multiple lesions. A solitary neurofibroma is seldom undergo sarcomatous transformation, but fibromatosis is common. Therefore, it is important that we observe the oral and radiographic changes to help minimize the tremendous potential of the disease for facial disfigurement and altered function. The tumors were excised and there are no evidence of recurrence. The patients are satisfied with function and esthetic aspects.

• Journal of the korean academy of Pediatric Dentistry
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• v.37 no.2
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• pp.240-245
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• 2010

Neurofibromatosis type 1, an autosomal dominant disorder with prevalence of 1 per 3000 people, has clinical features of cafe au lait spots in skin, multiple neurofibroma and dysplasia in skeletal, endocrinal, and blood vessel systems. Actual intraoral neurofibromas are known to occur in 25% of patients. A 9 year-old girl diagnosed with type 1 neurofibromatosis visited our hospital with chief complaint of gingival swelling. Gingival enlargement in lower anterior region existed and cafe au lait spots were confirmed in patient' skin. Enlarged gingival tissue were excised under local anesthesia. Neurofibroma was confirmed with biopsy. Clinical examination after months showed fine recovery without any evidence of recurrence. Due to its possibility of recurrence, periodic follow-up will be needed.

• The Journal of the Korean bone and joint tumor society
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• v.11 no.1
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• pp.82-87
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• 2005

Both neurilemmoma and neurofibroma are originated from Schwann cell. Plexiform tumor was mostly neurofibroma but Neurilemmoma which has plexiform is extremely rare. So it is important to differentiate plexiform neurilemmoma from plexiform neurofibroma, because plexiform neurilemmoma appears to have neither a significant association with neurofibromatosis nor a propensity for malignant transformation. We report two cases of plexiform neurilemmoma involving the left arm and right foot.

• Journal of Chest Surgery
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• v.21 no.6
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• pp.1111-1116
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• 1988

Neurofibromatosis is very rare syndrome characterized by abnormal cutaneous pigmentation and numerous skin tumors was described by Smith in 1849, which is inherited as an autosomal dominant trait. Von Recklinghausen reported 2 cases of multiple skin and subcutaneous tumors in 1982. Malignant peripheral nerve tumors, although generally rare, are one of the most characteristic malignant tumors associated with Neurofibromatosis. We have experienced 3 cases of malignant Schwannoma in neurofibroma patients from 1982 to 1988 for 6 years at Thoracic and Cardiovascular surgery department, College of Medicine, Yonsei University, Seoul, Korea. l. One is 62 years old female who was taken total hysterectomy followed by irradiation treatment due to Uterine Carcinoma 21 years ago. She had a large bulging mass on left anterior chest wall and was taken enbloc resection of tumor including rib confirmed malignant Schwannoma. 2. Another is 18 years old female who had large bulging mass on right chest wall and pleural effusion in right thoracic cavity. Thoracentesis revealed a large amount of lymphocytes misdiagnosed of Tbc, pleurisy with Neurofibromatosis. We performed tissue biopsy on bulging mass and the specimen was confirmed malignant Schwannoma 2 months after first diagnosis of Tbc. pleurisy. She was not accessible to radical resection because of far advanced malignant Schwannoma at that time. 3. Third case is 28 years old male who was taken enbloc resection of tumor including rib due to Neurofibroma with Neurofibromatosis at M. hospital 6 months ago. But he had rapid growing mass at operation site again and taken tissue biopsy confirmed of malignant Schwannoma. He was not accessible to enbloc resection due to malignant Schwannoma extending to mediastinal structures.

• Journal of Chest Surgery
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• v.40 no.4 s.273
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• pp.317-320
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• 2007

Neurogenic tumors are the most common posterior mediastinal tumors and accounting for $19{\sim}39%$ of all mediastinal tumors and 75% of all posterior mediastinal tumors. Neurofibromatosis is an autosomal dominant disorder with variable expression of tumors, including neurologic tumors of the peripheral nerves, nrve roots, and plexi. A posterior mediastinal neufibroma in neurofibromatosis patients is rare. We report here a case of posterior mediastinal neurofibroma in a patient with type 1 neurofibromatosis.

• 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 the korean academy of Pediatric Dentistry
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• v.38 no.2
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• pp.187-193
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• 2011

Neurofibromatosis is known as an autosomal dominant disorder caused by a mutation of a tumor suppressor gene on the long arm of chromosome 17 that affects the bone, nervous system, soft tissue, skin, and endocrine system. The most characteristic finding, which is helpful for clinical diagnosis as well, is the neurofibroma. Likewise brown macules called Cafe-au-lait spots with color of caffee latte, and Lisch nodules found around iris are useful to diagnose the disease. As known, the possibility of the neurofibromatosis occurred in oral cavity is relatively rare, and in most of cases it is related to soft tissue changes with single or multiple neurofibromatosis. The purpose of this report is to present characteristic dental findings which were found in a 4-year-old male and his father, both diagnosed as neurofibromatosis at Chonbuk National University Hospital before visiting our department, pediatric dentistry.