Translational Neuroscience

, 2:265 | Cite as

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
  • 35 Downloads

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 

References

  1. [1]
    Devaney K., Vinh T.N., Sweet D. E., Skeletal-extraskeletal angiomatosis. A clinicopathological study of fourtheen patients and nosologic considerations, J. Bone Joint Surg. Am., 1994, 76A, 878–891Google Scholar
  2. [2]
    Alexanian R., Weber D., Liu F., Differential diagnosis of monoclonal gammopathies, Arch. Pathol. Lab. Med., 1999, 123, 108–113PubMedGoogle Scholar
  3. [3]
    Heckl S., Aschoff A., Kunze S., Cavernomas of the skull: review of the literature 1975–2000, Neurosurg. Rev., 2002, 25, 56–62PubMedCrossRefGoogle Scholar
  4. [4]
    Khanam H., Lipper M. H., Wolff C. L., Lopes, M. B., Calvarial hemangiomas: report of two cases and review of the literature, Surg. Neurol., 2001, 55, 63–67PubMedCrossRefGoogle Scholar
  5. [5]
    Maroon J. C., Haines S. J., Philips J. G., Calvarial hemangioendothelioma with intracranial hemorrhage: case report, Neurosurgery, 1979, 4, 178–180PubMedCrossRefGoogle Scholar
  6. [6]
    Wyke B. D., Primary hemangioma of the skull, a rare cranial tumor, Am. J. Roentgenol., 1949, 61, 302–316Google Scholar
  7. [7]
    Salama S., Jenkin P., Angiomatosis of skin with local intravascular immunoglobulin deposits, associated with monoclonal gammopathy. A potential cutaneous marker for B-chronic lymphocytic leukemia, J. Cutan. Pathol., 1999, 26, 206–212PubMedCrossRefGoogle Scholar
  8. [8]
    Chang J., Most D., Bresnick S., Mehrara B., Steinbrech D. S., Reinich J. et al., Proliferative hemangiomas: analysis of cytokine gene expression and angiogenesis, Plast. Reconstr. Surg., 1999, 103, 1–9PubMedCrossRefGoogle Scholar
  9. [9]
    Godanich I. F., Campanacci M., Vascular hamartomata and infantile angioectatic osteohyperplasia of the extremities, J. Bone Joint Surg., 1962, 44A, 815Google Scholar
  10. [10]
    Muliken J. B., Cutaneous vascular anomalies. In: McCarthy J. G., May J. W. Jr., Littler J. W, Plastic Surgery: Tumors of the head & neck and skin, Philadelphia: WB Saunders Co., 1990Google Scholar
  11. [11]
    Durie B. G., Salmon S. E., A clinical staging system for multiple myeloma, Cancer, 1975, 36, 842–854PubMedCrossRefGoogle Scholar
  12. [12]
    Clayer M., Skeletal angiomatosis in association with gastro-intestinal angiodysplasia and paraproteinemia: a case report, J. Orthop. Surg., 2002, 10, 85–88Google Scholar
  13. [13]
    Kumar A., Loughran T., Alsina M, Durie B. G., Djulbegovic B., Management of multiple myeloma: a systematic review and critical appraisal of published studies, Lancet Oncol., 2003, 4, 293–304PubMedCrossRefGoogle Scholar
  14. [14]
    Resnick D., Kyriakos M., Greenway G. D., Tumors and tumor-like lesions of soft tissues. In: Resnick D., Kransdorf N. J., eds., Bone and joint imaging, 3rd ed. USA, Elsevier Saunders, 2005, 1219–1220Google Scholar
  15. [15]
    Kyle R. A., Rajkumar S. V., Monoclonal gammopathies of undetermined significance, Hematol. Oncol. Clin. North Am., 1999, 13, 1181–1202PubMedCrossRefGoogle Scholar
  16. [16]
    Gottfried O. N., Gluf W. M., Schmidt H. M., Cavernous hemangioma of the skull presenting with subdural hematoma. Case report., Neurosurg. Focus, 2004, 17, ECP1Google Scholar
  17. [17]
    Fornasier V., Protzner K., Radiation-induced tibial sarcoma in a treated case of hind foot angiomatosis, Skel. Radiol., 1998, 27, 164–168CrossRefGoogle Scholar

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

Personalised recommendations