Malignant Peripheral Nerve Sheath Tumor in Neurofibromatosis Type I : Unusual Presentation of Intraabdominal or Intrathoracic Mass

Kim, Jong Gwang; Sung, Woo Jin; Kim, Dong Hwan; Kim, Young Hwan; Sohn, Sang Kyun; Lee, Kyu Bo

doi:10.3904/kjim.2005.20.1.100

Cited by 10 publications

(10 citation statements)

References 13 publications

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“…MPNSTs have low attenuation on CT 9. On MR, MPNSTs are isointense on T1 and hyperintense on T2 8. Our findings for intrathoracic MPNST are similar, as 8/15 patients had hypoattenuating lesions on CT, and of the five patients with MR, all had isointense lesions on T1 and hyperintense lesions on T2.…”

Section: Discussionsupporting

confidence: 67%

“…Location also plays a role in outcome, with tumors in the trunk associated with poorer prognosis, likely attributable to difficulty in achieving negative surgical margins and consequent local or regional recurrence 1,5. While studying the impact of rhabdomyoblastic differentiation (MTT), presence of NF1 and location on the outcome of 84 consecutive patients with MPNST7, we noticed that the imaging literature on intrathoracic MPNST is extremely limited, consisting mostly of case reports with little information on imaging findings 4,8–14. Therefore, the purpose of this study was to assess the clinical and imaging characteristics of primary intrathoracic MPNST.…”

Section: Introductionmentioning

confidence: 99%

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Intrathoracic malignant peripheral nerve sheath tumors: imaging features and implications for management

Kamran

Shinagare

Howard

et al. 2013

Radiology and Oncology

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BackgroundThe aim of the study was to analyze the clinical and imaging characteristics of primary intrathoracic malignant peripheral nerve sheath tumors (MPNSTs).Patients and methods.In this institutional review board (IRB)-approved retrospective study, clinical and imaging features of 15 patients (eight men; mean age 50 years [range 18–83)] with pathologically proven malignant peripheral nerve sheath tumors seen from January 1999 to December 2011 were analyzed. Imaging features (CT in 15, MRI in 5 and PET/CT in 4) of primary tumors were evaluated by three radiologists and correlated with clinical management.ResultsOf the 15 tumors, six were located in the mediastinum (two each in anterior, middle and posterior mediastinum), four in chest wall, two were paraspinal, and three in the lung. Four patients had neurofibromatosis-1 (NF1); four tumors had heterologous rhabdomyoblastic differentiation (malignant triton tumor). Masses typically were elongated along the direction of nerves, with mean size of 11 cm. The masses were hypo- or isodense to muscles on CT, isointense on T1-weighted images, hyperintense on T2-weighted images and intensely fluorodeoxyglucose (FDG) avid (mean standardized uptake value [SUV]max of 10.5 [range 4.4–23.6]). Necrosis and calcification was seen in four tumors each. Finding of invasion of adjacent structures on imaging led to change in management in seven patients; patients with invasion received chemoradiation.ConclusionsIntrathoracic MPNSTs appear as large elongated masses involving mediastinum, lung or chest wall. Radiological identification of invasion of adjacent structures is crucial and alters therapy, with patients with invasion receiving neoadjuvant or adjuvant chemoradiation.

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Section: Discussionsupporting

confidence: 67%

Section: Introductionmentioning

confidence: 99%

Intrathoracic malignant peripheral nerve sheath tumors: imaging features and implications for management

Kamran

Shinagare

Howard

et al. 2013

Radiology and Oncology

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“…Lymphoma was not considered in our patient because she was 37 year old. Approximately 5% of patients with NF-1 develop MPNST, which usually arises from a plexiform neurofibroma [9]. MPNSTs are usually infilitrative and heterogenous, and have irreguler margins on CT and MRI.…”

Section: Discussionmentioning

confidence: 99%

Primitive Neuroectodermal Tumour: A Rare Association with Neurofibromatosis Type 1

Gümüş¹,

Gümüş²,

Firat³

et al. 2015

Ann Clin Anal Med

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ÖzetNörofibromatosis tip 1 (NF-1), benin tümörleri içeren yaygın herediter bir hastalıkdır. Omuriliğin primitive nöroektodermal tümörü (PNET) oldukça nadirdir. Biz burada NF-1'li bir kadın hastada paravertebral alandaki spinal epidural PNET'in US, BT ve MRG bulgularını sunuyoruz. 37 yaşındaki NF-1'li kadın hastanın bel ağ-rısı ve sağ alt ekstremitede ilerleyici güçsüzlüğü mevcuttu. US'da batında heterojen iyi sınırlı kitle lezyon izlendi. BT ve MRG'de L5-S1 düzeyinde sağ nöral foramenden kaynaklanan heterojen kontrastlanan kitle lezyon mevcuttu. Lezyon komşuluğunda posterolateral karın duvarında mezenter ve barsak anslarının bulunduğu büyük bir fıtık kesesi vardı. Tanı hastaya transabdominal tru-cut biyopsi yapı-larak konuldu. Nadir olmasına rağmen, spinal epidural lezyonlarda PNET ihtimali akılda tutulması gerekmektedir. Anahtar KelimelerSpinal Tümör; Primitive Nöroektodermal Tümör; Nörofibromatosis Tip 1 Abstract Neurofibromatosis type 1 (NF-1), is a common hereditary disease which often take the form of benign tumours. Spinal primitive neuroectodermal tumour (PNET) is very rare. Herein we report US, CT and MRI findings of a female patient with PNET involving the spinal epidural and paravertebral spaces with neurofibromatosis type 1. A 37-year-old woman has NF-1 presented with lower back pain and progressive weakness of the right lower extremities. US revealed a heterogenous well defined solid mass. CT and MRI revealed heterogenously enhanced mass originating from the right neural foramen at the level of L5-S1. Adjacent to the lesion, there was a large hernia sac, including bowel loops and mesentery, in the posterolateral abdominal wall. The definitive diagnosis was made by transabdominal percutaneous tru-cut biopsy. Although it is rare, the possibility of PNET should be kept in mind in assessing the spinal epidural lesions.

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“… 2 , 4 , 7 , 11 – 13 MPNSTs may arise from all peripheral nerves and frequently reported localizations are the extremities, trunk, head, and neck. 1 , 2 , 5 , 9 , 11 , 13 , 14 In patients with neurofibromatosis, MPNST most frequently occurs at ages of 30–40 years. When compared with benign tendon sheath tumors, MPNSTs more frequently occur at extremities.…”

Section: Discussionmentioning

confidence: 99%

Giant malignant peripheral nerve sheath tumor of thigh in an adolescent with neurofibromatosis type 1: a case report

Tosun¹,

Serbest²,

Türk³

et al. 2015

IMCRJ

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Malignant peripheral nerve sheath tumors (MPNSTs) are rare sarcomas of children and adolescents, and they are aggressive tumors with a high rate of local recurrence. We present a 15-year-old boy with neurofibromatosis type 1 (NF1), who had a giant MPNST on the right thigh taking into account the available literature. Diagnosis of MPNST may be delayed in NF1 patients due to confusion with a neurofibroma and/or a plexiform neurofibroma. Malignancy should be considered, especially in cases with big masses, with heterogeneous involvement, or in the presence of cysts or necrotic nodules. The aim of surgical treatment is complete surgical excision.

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Malignant Peripheral Nerve Sheath Tumor in Neurofibromatosis Type I : Unusual Presentation of Intraabdominal or Intrathoracic Mass

Cited by 10 publications

References 13 publications

Intrathoracic malignant peripheral nerve sheath tumors: imaging features and implications for management

Intrathoracic malignant peripheral nerve sheath tumors: imaging features and implications for management

Primitive Neuroectodermal Tumour: A Rare Association with Neurofibromatosis Type 1

Giant malignant peripheral nerve sheath tumor of thigh in an adolescent with neurofibromatosis type 1: a case report

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