• Title/Summary/Keyword: 신경섬유종증

Search Result 51, Processing Time 0.035 seconds

Lethal Hemomediastinum due to Spontaneous Rupture of an Aberrant Bronchial Artery in a Patient with Neurofibromatosis Type 1: Successful Treatment with Embolization (신경섬유종증 1형 환자에서 발생한 이소성 기관지동맥의 자발성 파열에 의한 치명적 종격동혈종: 색전술을 이용한 성공적 치료)

  • Yun Jin Im;Min Jeong Choi;Bong Man Kim
    • Journal of the Korean Society of Radiology
    • /
    • v.81 no.4
    • /
    • pp.958-964
    • /
    • 2020
  • Spontaneous bleeding due to vascular involvement of neurofibromatosis type 1 is rare but potentially fatal. Herein, we report a case of a lethal spontaneous hemomediastinum in a patient with neurofibromatosis type 1. The bleeding was caused by rupture of an aberrant bronchial artery arising from the ipsilateral subclavian artery, which was successfully treated using transarterial embolization with coils and N-butyl-2-cyanoacrylate.

Recurrent Shoulder Dislocation and Proximal Humerus Fracture in Neurofibromatosis Type I - A Case Report - (제 1형 신경 섬유종증 환자에서 발생한 재발성 견관절 탈구 및 근위 상완골 골절 - 1예 보고 -)

  • Hwang, Tae Hyok;Cho, Hyung Lae;Wang, Tae Hyun;Yang, Hui Sun
    • Clinics in Shoulder and Elbow
    • /
    • v.16 no.2
    • /
    • pp.135-140
    • /
    • 2013
  • We report a case of recurrent shoulder dislocation and combined proximal humerus fracture in neurofibromatosis type I. A twenty-year-old male patient with known neurofibromatosis type I presented with right shoulder dislocation and proximal humerus fracture following a minor trauma. His injured arm also had large plexiform neurofibromas on the elbow, and the dislocation of the shoulder joint was proven to be recurrent. Bony deformation of the humerus and scapula were revealed on X-ray and computed tomography, as well as multiple neurofibromatic changes of the deltoid and periscapular muscles were noted on magnetic resonance imaging. Open reduction and capsular reconstruction were performed and humeral fracture was stabilized with a reconstruction plate. Satisfactory union and functional result were achieved and the dislocation did not recur until the 7-month followup after the procedure. Recurrent shoulder dislocation and combined proximal humerus fracture in neurofibromatosis type I are rare complications. However, it should still be considered in the orthopaedic evaluation process of the diseases.

GINGIVAL NEUROFIBROMAS OF NEUROFIBROMATOSIS TYPE 1: CASE REPORT (치은에 발생한 신경섬유종의 외과적 치험례)

  • Park, Seung-Hyo;Lee, Nan-Young;Lee, Sang-Ho
    • Journal of the korean academy of Pediatric Dentistry
    • /
    • v.37 no.2
    • /
    • pp.240-245
    • /
    • 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.

A Case of Neurofibromatosis with Invasion of Bladder (방광을 침범한 신경섬유종증 1례)

  • Kim, Mi-Kyung;Park, Cheol;Kim, Min-Sang;Yim, Hyung-Eun;Yoo, Kee-Hwan;Hong, Young-Sook;Lee, Joo-Won
    • Childhood Kidney Diseases
    • /
    • v.16 no.1
    • /
    • pp.68-71
    • /
    • 2012
  • Neurofibromatosis is a rare systemic disease, and genitourinary tract involvement is especially uncommon. Bladder is the most frequently involved organ in the genitourinary tract. Bladder neurofibromatosis may present as a diffuse infiltrative process or an isolated neurofibroma. The symptoms vary, ranging from urinary incontinence to retention. Treatment is usually conservative. The patient should be worked up to rule out other manifestation of tumor enlargement and followed to evaluate the development of new lesion. We report a case of the development of invasion of bladder in a patient with neurofibromatosis.

Pancreatic Collision Tumor of Desmoid-Type Fibromatosis and Mucinous Cystic Neoplasm: A Case Report (데스모이드 섬유종증과 점액성 낭성 종양으로 이루어진 췌장의 충돌 종양: 증례 보고)

  • Min Jung Ryu;Jae Woon Kim;Seung Eun Lee;Joon Hyuk Choi
    • Journal of the Korean Society of Radiology
    • /
    • v.82 no.5
    • /
    • pp.1297-1303
    • /
    • 2021
  • Pancreatic collision tumors are rare neoplasm, and cases consisting of ductal adenocarcinoma with a neuroendocrine tumor, intraductal papillary mucinous neoplasm with a neuroendocrine tumor, and solid pseudopapillary neoplasm with a neuroendocrine tumor have been reported. We report a case of a rapidly growing pancreatic collision tumor consisting of desmoid-type fibromatosis and mucinous cystic neoplasm in a 30-year-old pregnant female. To the best of our knowledge, this is the first reported case of a pancreatic collision tumor consisting of desmoid-type fibromatosis and mucinous cystic neoplasm.

Congenital Pseudoarthrosis of the Clavicle Related with Neurofibromatosis - A Case Report - (신경섬유종증과 연관되어 발생한 선천성 쇄골 가관절증 - 증례 보고 -)

  • Yun, Ho-Hyun;Ahn, Gil-Yeong;Nam, Il-Hyun;Moon, Gi-Huk;Lee, Jung-Ik;Yoo, Yon-Sik
    • Clinics in Shoulder and Elbow
    • /
    • v.10 no.2
    • /
    • pp.236-240
    • /
    • 2007
  • Reports of bowing and pseudarthrosis of the humerus and clavicle are rare. Most patients with congenital pseudoarthrosis of the clavicle involving the right side and midportion of clavicle tended to heal better than congenital pseudoarthrosis of the tibia. We experienced a patient who had special features in terms of location, pseudoarthrosis pattern, and neurofibromatosis, and report on this case here.

Imaging Findings of Breast Metastasis from Malignant Peripheral Nerve Sheath Tumor of Mediastinum: A Case Report (종격동 악성 말초 신경 초종으로부터 유방 전이의 영상 소견: 증례 보고)

  • So Hyeong Park;Ji Yeon Park;Mee Joo
    • Journal of the Korean Society of Radiology
    • /
    • v.84 no.3
    • /
    • pp.770-775
    • /
    • 2023
  • Malignant peripheral nerve sheath tumor (MPNST), a rare soft-tissue sarcoma, is most commonly located in the trunk, extremities, and head and neck, but rare in the breast. We report a metastatic breast MPNST in a 27-year-old woman with neurofibromatosis type 1 (NF-1). Chest computed tomography revealed a well-defined, oval, mildly enhancing nodule in the right breast. US revealed a circumscribed, oval, heterogeneous echoic mass with vascularity and intermediate elasticity in the right upper outer breast. The breast mass was excised and diagnosed as MPNST on histopathology evaluation. Although rare, it should be included in the differential diagnosis of breast mass in NF-1 patient.

Distribution of Benign Soft Tissue Tumors Occurred in Extremities and Trunk (사지 및 체간에 발생한 양성 연부조직 종양의 발생 분포)

  • Kim, Tai-Seung;Seo, Kyoung-Won;Kim, Tae-Jin
    • The Journal of the Korean bone and joint tumor society
    • /
    • v.5 no.1
    • /
    • pp.35-43
    • /
    • 1999
  • The authors reviewed and analyzed the pathologically confirmed 230 cases of benign soft tissue tumors which had been treated at Department of Orthopaedic Surgery in Seoul Hospital, Hanyang University College of Medicine from February 1984 to November 1997. The following results were obtained. 1. The most common benign soft tissue tumors was ganglion(26.5%) followed by hemangioma(19.6%), lipoma(17.0%), Baker's cyst(13.0%) and neurilemmoma(7.0%) in decreasing order of incidence. 2. Benign soft tissue tumors were found evenly distributed over all age group. Hemangioma and lymphangioma were usually found to occur before 20 years old. 3. Female was affected about 1.3 times more common. But, Baker's cyst and neurilemmoma occurred most frequently in male. 4. In children, the most common benign soft tissue tumors was hemangioma followed by ganglion, lymphangioma, and lipoma. In adult, ganglion, lipoma, Baker's cyst, hemangioma were common. 5. The lower extremity was the predominant site of occurrence(60.0%). 6. Benign soft tissue tumors were taken by excision. Local recurrences were developed in 22(9.6%) out of 230 cases, especially in hemangioma.

  • PDF