• Nuclear Medicine and Molecular Imaging
• /
• 제43권4호
• /
• pp.361-362
• /
• 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
• /
• 제18권2호
• /
• pp.97-101
• /
• 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
• /
• 제21권6호
• /
• pp.1103-1110
• /
• 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
• /
• 제14권4호
• /
• pp.315-321
• /
• 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.

• 대한소아치과학회지
• /
• 제37권2호
• /
• pp.240-245
• /
• 2010

제1형 신경섬유종증은 약 3000명당 1명 꼴로 발생되며 상염색체 우성유전질환이다. 피부의 카페오레반점 및 다발성 신경 섬유종과 피부, 신경계, 골격계, 내분비계, 혈관계의 다양한 이형성이 특징이다. 실제적인 구강 내 신경섬유종은 25%의 환자에서 발생한다고 알려져 있다. 제1형 신경섬유종증으로 진단된 만9세의 여아가 치은의 부종을 주소로 본원에 내원하였다. 하악 전치부 설측에 치은비대가 존재하였고, 환자의 사지와 몸통에서 갈색반점인 카페오레 반점을 확인할 수 있었다. 비대된 치은조직을 국소마취 하에 제거하였고, 조직검사를 시행한 결과 신경섬유종으로 확진하였다. 7개월 후 검사 결과 재발의 증거없이 양호한 치유를 보였다. 재발의 가능성이 있으므로 정기적인 검사가 필요하다.

• 대한골관절종양학회지
• /
• 제11권1호
• /
• pp.82-87
• /
• 2005

신경초 세포에서 기원하는 양성 종양에는 신경초종과 신경섬유종이 있다. 이 중 총상의 형태를 가지는 경우는 대부분 신경섬유종이며, 신경초종의 경우는 매우 드물다. 총상 신경초종은 신경섬유종증을 동반하지 않고, 악성화하지 않는다는 점에서 반드시 신경섬유종과 감별이 필요하다. 저자들은 각 각 좌측 상완부와 우측 족부에 발생한 총상 신경초종 2례를 치험하였기에 보고하는 바이다.

• Journal of Chest Surgery
• /
• 제21권6호
• /
• pp.1111-1116
• /
• 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
• /
• 제40권4호
• /
• pp.317-320
• /
• 2007

신경기원의 종양은 전체 종격동 종양의 $19{\sim}39%$를 차지하며 후종격동 종양의 75%를 차지하는 가장 흔한 종양이다. 신경섬유종증은 상염색체 우성으로 유전되는 질환으로 임상 양상은 다양하며, 신경계에서는 말초신경, 신경근, 신경총 등에서 종양을 형성한다. 신경섬유종증 환자에서의 후종격동 신경섬유종은 드물어, 이에 1형(type 1) 신경섬유종증 환자에서 후종격동에 발생한 신경섬유종을 경험하게 되어 보고한다.

• Journal of Chest Surgery
• /
• 제21권4호
• /
• pp.766-771
• /
• 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.

• 대한소아치과학회지
• /
• 제38권2호
• /
• pp.187-193
• /
• 2011

신경섬유종증(neurofibromatosis)은 골조직, 신경계, 연부조직, 피부 및 내분비계 장애를 수반하며 상염색체 우성을 보이는 유전질환으로 제17번 염색체의 장완에 위치한 종양 억제 유전자의 변성에 의해 발생한다. 임상적으로 진단에 도움을 주는 특정적인 소견들 중 가장 중요한 것은 신경섬유종(neurofibroma)이고 그 외에 밀크 커피색을 띠는 밀크 커피색 반점(cafe-au-lait spot)과, 홍채결절(Lisch nodule)이 있다. 신경섬유종증이 비교적 구강 내에서 발현하는 비율은 비교적 낮은 것으로 알려져 있고, 대부분의 경우 단독 혹은 다수의 신경섬유종이 존재하는 연조직 변화와 관련되어 있다. 최근 전북대학교 병원에서 제1 형 신경섬유종증으로 진단받고, 치아우식증 치료를 위해 본원 소아치과에 내원한 만 4세의 남아와 그의 아버지에게서 제1형 선경섬유종증의 특징적인 치과적 소견을 발견하였기에 치료 경과와 함께 보고하는 바이다.