Translational Neuroscience

, 2:265

Giant cavernoma of the skull and skeletal-extraskeletal angiomatosis associated with paraproteinemia

  • Helena Šarac
  • Sanja Hajnšek
  • Silvio Bašić
  • Srđana Telarović
  • Jasenka Markeljević
  • Miroslav Vukić
  • Marko Radoš
  • Marija Bošnjak-Pašić
  • Kamelija Žarković
  • Patrick R. Hof
  • Goran Šimić
Case Report

DOI: 10.2478/s13380-011-0032-3

Cite this article as:
Šarac, H., Hajnšek, S., Bašić, S. et al. Translat.Neurosci. (2011) 2: 265. doi:10.2478/s13380-011-0032-3

Abstract

Skeletal-extraskeletal angiomatosis is defined as a benign vascular proliferation affecting the medullar cavity of the bone and at least one other type of tissue, including skin, subcutaneous tissue, viscera, muscle, or synovium, and which does not spread to avascular tissue such as cartilage. Primary hemangiomas/cavernous hemangiomas (cavernomas) are exceedingly rare in the skull, accounting for 0.2% of all osseous neoplasms and are usually located in frontal and parietal bones. The authors present the case of a 66-year-old man who was admitted with right-side hemiparesis. MRI revealed a destructive bone lesion of the left frontal bone. Digital subtraction angiography of the brain did not reveal pathological vascularization, but a minor submucous hemangioma was seen in the nasal airway. Urine test for Bence-Jones proteins was positive for IgG λ light chain. Bone marrow aspiration and CSF analysis revealed no evidence of systemic myelomatosis suggesting a monoclonal gammopathy of undetermined significance. A highly vascular tumor was surgically removed. The histopathology verified cavernous hemangioma of the skull and the nasal submucous hemangioma. We discuss the diagnostic procedure, possible pathophysiological mechanisms and treatment implementation. It is possible that immunoglobulins from monoclonal gammopathies have an etiologic role in the development of the bone and skin changes in older patients, as an acquired condition, by producing a vascular injury that could lead to the multiple hemangiomas in skeletal-extraskeletal angiomatosis. To prevent misdiagnosis with lesions of other origins, multiple lesions of the head must be resected and histopathologically verified. In conclusion, to the best of our knowledge, this is the first case of giant cavernous hemangioma of the skull associated with paraproteinemia and skeletalextraskeletal angiomatosis limited to the head.

Keywords

Skull Cavernoma Hemangioma Monoclonal Gammopathy Skeletal-extraskeletal angiomatosis Paraproteinemia 

Copyright information

© © Versita Warsaw and Springer-Verlag Wien 2011

Authors and Affiliations

  • Helena Šarac
    • 1
  • Sanja Hajnšek
    • 2
  • Silvio Bašić
    • 3
  • Srđana Telarović
    • 2
  • Jasenka Markeljević
    • 4
  • Miroslav Vukić
    • 5
  • Marko Radoš
    • 6
  • Marija Bošnjak-Pašić
    • 2
  • Kamelija Žarković
    • 7
  • Patrick R. Hof
    • 8
  • Goran Šimić
    • 9
  1. 1.School of Medicine, University Hospital Centre Zagreb, Department of Neurology and Croatian Institute for Brain Research, Diagnostic Centre „Neuron“, Department of Neurology, School of Medicine, University of ZagrebUniversity of ZagrebZagrebCroatia
  2. 2.School of Medicine, University Hospital Centre Zagreb, Department of NeurologyUniversity of ZagrebZagrebCroatia
  3. 3.Department of NeurologyUniversity Hospital DubravaZagrebCroatia
  4. 4.School of Medicine, Departement of Immunology, Rheumatology and Pulmology, University Hospital Sestre MilosrdniceUniversity of ZagrebZagrebCroatia
  5. 5.School of Medicine, University Hospital Centre Zagreb, Department of NeursurgeryUniversity of ZagrebZagrebCroatia
  6. 6.School of Medicine, University Hospital Centre Zagreb, Department of Interventional RadiologyUniversity of ZagrebZagrebCroatia
  7. 7.Department of NeuropathologyUniversity Hospital Centre ZagrebZagrebCroatia
  8. 8.Department of NeuroscienceMount Sinai School of MedicineNew YorkUSA
  9. 9.School of Medicine, Croatian Institute for Brain Research, Department of NeuroscienceUniversity of ZagrebZagrebCroatia

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