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Pachymeningeal involvement in POEMS syndrome: MRI and histopathological study
  1. Chiara Briani1,
  2. Marny Fedrigo2,
  3. Renzo Manara3,
  4. Chiara Castellani2,
  5. Renato Zambello4,
  6. Valentina Citton3,
  7. Marta Campagnolo1,
  8. Chiara Dalla Torre1,
  9. Marta Lucchetta1,
  10. Enrico Orvieto5,
  11. Antonino Rotilio6,
  12. Sabrina Marangoni7,
  13. Stefania Magi8,
  14. Davide Pareyson9,
  15. Igor Florio10,
  16. Elena Pegoraro1,
  17. Gaetano Thiene2,
  18. Leontino Battistin1,11,
  19. Fausto Adami4,
  20. Annalisa Angelini2
  1. 1Department of Neurosciences, University of Padova, Padova, Italy
  2. 2Department of Medical-Diagnostic Sciences and Special Therapies, University of Padova, Padova, Italy
  3. 3Department of Neuroradiology, University of Padova, Padova, Italy
  4. 4Department of Clinical and Experimental Medicine, University of Padova, Padova, Italy
  5. 5Department of Oncological and Surgical Sciences, Division of Pathology, University of Padova, Padova, Italy
  6. 6Department of Neurosurgery, University of Padova, Padova, Italy
  7. 7Department of Neurology, Santa Chiara Hospital, Trento, Italy
  8. 8Department of Neurology, San Donato Hospital, Arezzo, Italy
  9. 9Department of Clinical Neurosciences, IRCCS Foundation, C. Besta Neurological Institute, Milan, Italy
  10. 10Department of Neurology, Bolzano Hospital, Bolzano, Italy
  11. 11IRCCS San Camillo Hospital, Venice, Italy
  1. Correspondence to Chiara Briani, Department of Neurosciences, University of Padova, Via Giustiniani 5, 35128 Padova, Italy; chiara.briani{at}unipd.it

Abstract

Polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, skin changes (POEMS) syndrome is a rare plasma cell disease. Vascular endothelial growth factor (VEGF) seems to play a pathogenic role. Peripheral neuropathy is the main neurological feature. Cranial pachymeningitis has occasionally been reported, but no histopathological studies have been performed. The authors extensively evaluated the central nervous system MRI in 11 patients (seven men, four women; mean age at diagnosis 54.45 years) with POEMS syndrome. In two patients, meningeal histopathology with staining for VEGF and VEGF receptor was performed, and pachymeningeal involvement characterised at histopathological, immunohistochemical and confocal microscopy levels. Nine patients presented with cranial pachymeningitis. One patient suffered from migraine, and none complained of cranial nerve palsies or visual loss. None showed any MRI signs of spinal pachymeningitis. No correlation was found with disease duration and VEGF serum level. Histopathology showed hyperplasia of meningothelial cells, neovascularisation and obstructive vessel remodelling, without inflammation. VEGF and VEGF receptor were strongly coexpressed on endothelium, smooth-muscle cells of arterioles and meningothelial cells. In conclusion, POEMS patients present a high prevalence of meningeal involvement. The histological changes, different from those present in chronic pachymeningitis of other aetiology, suggest a possible VEGF role in the pathogenesis of the meningeal remodelling.

  • POEMS syndrome
  • pachymeningitis
  • MRI
  • VEGF

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Footnotes

  • Competing interests None.

  • Patient consent Obtained.

  • Provenance and peer review Commissioned; externally peer reviewed.

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