Poster (Scientific congresses and symposiums)
Radiation-induced malignant peripheral nerve sheat tumors – a report of 2 cases
PHAN BA, Remy; BELACHEW, Shibeshih; JEDIDI, Zayd et al.
2011European Neurological Society 21st Meeting
 

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Keywords :
Malignant peripheral nerve sheath tumor; Radiation induced
Abstract :
[en] We discuss the case of two patients who developped delayed malignant peripheral nerve sheath tumor (MPNST) following radiotherapy. Case report: the first patient is a sixty year-old woman with a 2 years history of right cervicobrachial pain and weakness. The neurological examination depicted severe weakness, atrophy and loss of sensation in the right C5 and C6 territories. A subclavicular hardened and enlarged lymph node was noted. Her past medical history was notable for a Hodgkin’s disease (HD) treated with radiation therapy (>40Gy) 35 years earlier. Brachial plexus MRI revealed a tumoral mass arising from the right brachial plexus. Biopsy of the subclavicular mass revealed a poorly differentiated malignant tumour consisting of spindle cells showing moderate polymorphism and a high mitotic index. Immunohistochemistry showed positivity for the S-100 protein, the CD56 and for the epithelial membrane antigen (EMA) and a diagnosis of MPNST of the brachial plexus was proposed. The second case is a 36 year-old man referred for a history of right sciatic neuralgia that appeared 3 years earlier. The medical history of the patient was notable for a right seminoma, treated by orchidectomy and prophylactic radiotherapy (24 Gy) 5 years earlier. The neurologic examination revealed right L5 and S1 radicular territories involvement, and the CT of the pelvis demonstrated a nodular mass at the level of the greater sciatic foramen. A surgical biopsy was performed and the neuropathological findings were consistent with a diagnosis of low-grade MPSNT. Discussion: MPNSTs are rare tumors accounting for 3 to 10% of all tissues sarcomas. Half of the cases described are sporadic, while the other half tend to appear in patients suffering from tumor prone conditions, such as neurofibromatosis type 1. Although secondary neoplasms are known complications of radiotherapy, descriptions of peripheral nerve sheath tumors (PNST) are scarce. The exact pathophysiology of radiation-induced PNSTs remains unclear but vascular alterations, direct damages to axon or Schwann cell and nerve compression by soft tissue fibrosis are thought to play a role. Although surgical removal sometimes followed by chemotherapy is the mainstay of MPNSTs, they usually carry a poor prognosis. Our 2 cases emphasize that the possibility of radiation-induced MPNST has to be kept in mind when investigating a localized neuropathy in a previously irradiated area.
Disciplines :
Neurology
Author, co-author :
PHAN BA, Remy ;  Centre Hospitalier Universitaire de Liège - CHU > Neurologie Sart Tilman
BELACHEW, Shibeshih ;  Centre Hospitalier Universitaire de Liège - CHU > Neurologie Sart Tilman
JEDIDI, Zayd  ;  Centre Hospitalier Universitaire de Liège - CHU > Neurologie Sart Tilman
SAUTOIS, Brieuc  ;  Centre Hospitalier Universitaire de Liège - CHU > Oncologie médicale
DEPREZ, Manuel ;  Centre Hospitalier Universitaire de Liège - CHU > Anatomie pathologique
MOONEN, Gustave  ;  Centre Hospitalier Universitaire de Liège - CHU > Neurologie Sart Tilman
Language :
English
Title :
Radiation-induced malignant peripheral nerve sheat tumors – a report of 2 cases
Publication date :
May 2011
Number of pages :
Poster
Event name :
European Neurological Society 21st Meeting
Event place :
Lisbon, Portugal
Event date :
28-31st of May, 2011
Audience :
International
Available on ORBi :
since 14 October 2012

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