Reference : Radiation-induced malignant peripheral nerve sheat tumors – a report of 2 cases
Scientific congresses and symposiums : Poster
Human health sciences : Neurology
Radiation-induced malignant peripheral nerve sheat tumors – a report of 2 cases
PHAN BA, Remy mailto [Centre Hospitalier Universitaire de Liège - CHU > > Neurologie Sart Tilman >]
BELACHEW, Shibeshih mailto [Centre Hospitalier Universitaire de Liège - CHU > > Neurologie Sart Tilman >]
JEDIDI, Zayd mailto [Centre Hospitalier Universitaire de Liège - CHU > > Neurologie Sart Tilman >]
SAUTOIS, Brieuc mailto [Centre Hospitalier Universitaire de Liège - CHU > > Oncologie médicale >]
DEPREZ, Manuel [Centre Hospitalier Universitaire de Liège - CHU > > Anatomie pathologique >]
MOONEN, Gustave mailto [Centre Hospitalier Universitaire de Liège - CHU > > Neurologie Sart Tilman >]
European Neurological Society 21st Meeting
28-31st of May, 2011
[en] Malignant peripheral nerve sheath tumor ; Radiation induced
[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.
Researchers ; Professionals ; Students

File(s) associated to this reference

Fulltext file(s):

Open access
ENS2011-MPNST-P617.pdfPublisher postprint12.38 MBView/Open

Bookmark and Share SFX Query

All documents in ORBi are protected by a user license.