A Case of Malignant Peripheral Sheath Tumor Arising from Neurofibromatosis Type 1

Kang, Min Ju; Kang, Hoon; Kim, Hyung Ok; Park, Young Min

doi:10.5021/ad.2008.20.1.32

Cited by 3 publications

(3 citation statements)

References 8 publications

(12 reference statements)

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“…NF1 associated MPNSTs tend to occur at a younger age than those without the syndrome, often metastasize in an early phase of the disease and have a poor prognosis. Thirty one MPNSTs including four cases of this study were reported from 21 articles [ 8 - 10 ]. The distribution of MPNSTs shows similar patterns with other studies: 5 head and neck, 9 trunk (including 2 cases of breast), 6 extremities, 3 intra-thoracic, 2 intra-abdominal, 5 retro-peritoneum, and 1 spine.…”

Section: Discussionmentioning

confidence: 99%

Oncologic manifestations of neurofibromatosis type 1 in Korea

et al. 2012

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PurposeThe aim of this study was to investigate the incidence and spectrum of malignant tumors in Korean neurofibromatosis type 1 (NF1) patients.MethodsWe retrospectively reviewed 125 patients who were diagnosed with NF1 at a single institution from 1995 to 2010. The incidence, location, histologic type, and radiologic findings of malignant tumors as well as development of multiple primary tumors were analyzed.ResultsEighteen malignant tumors occurred in 16 patients (12.8%) among 125 Korean NF1 patients; 9 carcinomas, 8 sarcomas and 1 central nervous system (CNS) tumor. Five tumors were of nervous system origin and 13 were non-nervous system tumors. The locations of the tumors were as follow: 1 CNS, 2 lung, 3 breast, 3 stomach, 3 small bowel, 1 colon, 1 liver, 1 uterus, 1 neck, and 2 in extremities. Three malignant peripheral nerve sheath tumors (MPNSTs) occurred at the neck and extremity, and one in the liver. All three gastrointestinal stromal tumors (GISTs) had multiple tumors in the jejunum, and one MPNST and one pheochromocytoma were accompanied in two GISTs. Multiple primary tumors, benign or malignant were reported in 4 patients (25.0%), synchronously or metachronously.ConclusionKorean NF1 patients had a high risk of developing malignant tumors. The common malignant tumors in Koreans such as breast, lung and stomach cancers developed frequently in addition to the NF1-related tumors such as MPNST or GIST.

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Section: Discussionmentioning

confidence: 99%

Oncologic manifestations of neurofibromatosis type 1 in Korea

et al. 2012

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“…Tumors seen in these group of patients are at worse prognosis due to early onset, central localization, large size, more frequent metastases and recurrences. There are few reports on this rare entity in literature [1][2][3][4][5][6][7]. In a case report, the authors present a typical case of MPNST in a 36-year-old man with NF type 1, which occurred on the left buttock.…”

Section: Case Reportmentioning

confidence: 99%

“…In a case report, the authors present a typical case of MPNST in a 36-year-old man with NF type 1, which occurred on the left buttock. A PET-CT showed findings of possible inguinal lymph node metastasis and a lymph node biopsy confirmed the diagnosis [1]. In an other report the authors presented a clinical case with Von Recklinghausen disease and recurrence of a left dorsal neurofibrosarcoma diagnosed by Positron Emission Tomography with fluorine-18-fluoroeoxyD-glucose [2].…”

Section: Case Reportmentioning

confidence: 99%