Intrathoracic Malignant Peripheral Nerve Sheath Tumor in von Recklinghausen`s Disease

Park, Ju Hyung; Choi, Koang Ho; Lee, Heung Bum; Rhee, Yang Keun; Lee, Yong Chul; Chung, Myung Ja

doi:10.3904/kjim.2001.16.3.201

Cited by 6 publications

(2 citation statements)

References 9 publications

(10 reference statements)

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“…Sordillo PP et al 7) and Guccion JG et al 8) reported that tumors associated with NF1 are located more centrally than without NF1. However, intraabdominal or intrathoracic manifestation of MPNST in patients with NF1 is quite rare 9 - 11) .…”

Section: Discussionmentioning

confidence: 99%

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

Kim

Sung²,

Kim³

et al. 2005

Korean J Intern Med

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A malignant peripheral nerve sheath tumor (MPNST) is an extremely rare soft tissue tumor in the general population. On the other hand, there is a higher incidence of MPNST in patients with neurofibromatosis type I (von Recklinghausen's disease). The common sites are the extremities, trunk, head and neck. However, an intraabdominal or intrathoracic manifestation is uncommon. This paper reports two patients, a 31 year-old woman with multiple neurofibromatosis presenting as an intraabdominal malignant peripheral nerve sheath tumor, and a 33 year-old woman with an intrathoracic malignant peripheral nerve sheath tumor. The patients were treated with chemotherapy followed by radiotherapy. However, one patient died as a result of disease progression 21 months after the diagnosis and the other patient is currently being treated with radiotherapy.

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Section: Discussionmentioning

confidence: 99%

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

Kim

Sung²,

Kim³

et al. 2005

Korean J Intern Med

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“…It usually arises in the extremities, trunk, head and neck. An intrathoracic localization is extremely rare, with only a few reports in the literature [2][3][4]. Diaphragm paralysis in von Recklinghausenʼs disease may represent the first manifestation in these patients [5].…”

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confidence: 99%

Regarding Neurogenic Malignant Tumors in the Mediastinum

Lentini¹

2011

Thorac cardiovasc Surg

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Unusual histopathological findings in a young Pekingese dog with intrathoracic malignant peripheral nerve sheath tumour

Dezfoulian¹,

Jamshidi

Ghaffari

et al. 2009

Comp Clin Pathol

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This report describes intrathoracic malignant peripheral nerve sheath tumour in a Pekingese dog with unusual histopathologic findings. A 2-year-old male Pekingese dog presented with a history of pain and chronic hind limb lameness. Thoracic radiographs revealed soft-tissue opacity ventral to the thoracic vertebrae and dorsal to the aorta and base of the heart. The echo texture of the mass ultrasonographically was heterogenic, including hypo-and hyper-echoic areas within the mass, which had a well-defined echogenic wall. The owner refused further diagnostic tests, and due to a poor prognosis, the dog was euthanized. On histopathological examination, ovoid structures, known as Verocay bodies in human schwannomas, together with the pseudo-rosette-like structures in which neoplastic cells were arranged around blood vessels, were found as the most pronounced histological feature. Immuno-histochemical examination revealed that neoplastic cells were extensively positive for both neuron-specific enolase and vimentin. To the authors' knowledge, such histopathological findings associated with malignant peripheral nerve sheath tumour in dogs have not been previously reported.Keywords Malignant peripheral nerve sheath tumour . Dog 0. Dezfoulian

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Intrathoracic Malignant Peripheral Nerve Sheath Tumor in von Recklinghausen`s Disease

Cited by 6 publications

References 9 publications

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

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

Regarding Neurogenic Malignant Tumors in the Mediastinum

Unusual histopathological findings in a young Pekingese dog with intrathoracic malignant peripheral nerve sheath tumour

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