Jump to ContentJump to Main Navigation
Show Summary Details
More options …

Translational Neuroscience

Editor-in-Chief: David, Olivier

1 Issue per year


IMPACT FACTOR 2016: 0.922
5-year IMPACT FACTOR: 1.030

CiteScore 2016: 1.13

SCImago Journal Rank (SJR) 2015: 0.704
Source Normalized Impact per Paper (SNIP) 2015: 0.286

Open Access
Online
ISSN
2081-6936
See all formats and pricing
More options …

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

Helena Šarac
  • 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 Zagreb, University of Zagreb, Zagreb, Croatia
  • Email
  • Other articles by this author:
  • De Gruyter OnlineGoogle Scholar
/ Sanja Hajnšek
  • School of Medicine, University Hospital Centre Zagreb, Department of Neurology, University of Zagreb, Zagreb, Croatia
  • Email
  • Other articles by this author:
  • De Gruyter OnlineGoogle Scholar
/ Silvio Bašić / Srđana Telarović
  • School of Medicine, University Hospital Centre Zagreb, Department of Neurology, University of Zagreb, Zagreb, Croatia
  • Email
  • Other articles by this author:
  • De Gruyter OnlineGoogle Scholar
/ Jasenka Markeljević
  • School of Medicine, Departement of Immunology, Rheumatology and Pulmology, University Hospital Sestre Milosrdnice, University of Zagreb, Zagreb, Croatia
  • Email
  • Other articles by this author:
  • De Gruyter OnlineGoogle Scholar
/ Miroslav Vukić
  • School of Medicine, University Hospital Centre Zagreb, Department of Neursurgery, University of Zagreb, Zagreb, Croatia
  • Email
  • Other articles by this author:
  • De Gruyter OnlineGoogle Scholar
/ Marko Radoš
  • School of Medicine, University Hospital Centre Zagreb, Department of Interventional Radiology, University of Zagreb, Zagreb, Croatia
  • Email
  • Other articles by this author:
  • De Gruyter OnlineGoogle Scholar
/ Marija Bošnjak-Pašić
  • School of Medicine, University Hospital Centre Zagreb, Department of Neurology, University of Zagreb, Zagreb, Croatia
  • Email
  • Other articles by this author:
  • De Gruyter OnlineGoogle Scholar
/ Kamelija Žarković / Patrick Hof / Goran Šimić
  • School of Medicine, Croatian Institute for Brain Research, Department of Neuroscience, University of Zagreb, Zagreb, Croatia
  • Email
  • Other articles by this author:
  • De Gruyter OnlineGoogle Scholar
Published Online: 2011-09-24 | DOI: https://doi.org/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

  • [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, 878–891 Google Scholar

  • [2] Alexanian R., Weber D., Liu F., Differential diagnosis of monoclonal gammopathies, Arch. Pathol. Lab. Med., 1999, 123, 108–113 PubMedGoogle Scholar

  • [3] Heckl S., Aschoff A., Kunze S., Cavernomas of the skull: review of the literature 1975–2000, Neurosurg. Rev., 2002, 25, 56–62 http://dx.doi.org/10.1007/s101430100180CrossrefGoogle Scholar

  • [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, 63–67 http://dx.doi.org/10.1016/S0090-3019(00)00268-8CrossrefGoogle Scholar

  • [5] Maroon J. C., Haines S. J., Philips J. G., Calvarial hemangioendothelioma with intracranial hemorrhage: case report, Neurosurgery, 1979, 4, 178–180 http://dx.doi.org/10.1227/00006123-197902000-00013CrossrefGoogle Scholar

  • [6] Wyke B. D., Primary hemangioma of the skull, a rare cranial tumor, Am. J. Roentgenol., 1949, 61, 302–316 Google Scholar

  • [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, 206–212 http://dx.doi.org/10.1111/j.1600-0560.1999.tb01830.xCrossrefGoogle Scholar

  • [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, 1–9 http://dx.doi.org/10.1097/00006534-199901000-00001CrossrefGoogle Scholar

  • [9] Godanich I. F., Campanacci M., Vascular hamartomata and infantile angioectatic osteohyperplasia of the extremities, J. Bone Joint Surg., 1962, 44A, 815 Google Scholar

  • [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., 1990 Google Scholar

  • [11] Durie B. G., Salmon S. E., A clinical staging system for multiple myeloma, Cancer, 1975, 36, 842–854 http://dx.doi.org/10.1002/1097-0142(197509)36:3<842::AID-CNCR2820360303>3.0.CO;2-UCrossrefGoogle Scholar

  • [12] Clayer M., Skeletal angiomatosis in association with gastro-intestinal angiodysplasia and paraproteinemia: a case report, J. Orthop. Surg., 2002, 10, 85–88 PubMedGoogle Scholar

  • [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, 293–304 http://dx.doi.org/10.1016/S1470-2045(03)01077-5CrossrefGoogle Scholar

  • [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, 1219–1220 Google Scholar

  • [15] Kyle R. A., Rajkumar S. V., Monoclonal gammopathies of undetermined significance, Hematol. Oncol. Clin. North Am., 1999, 13, 1181–1202 http://dx.doi.org/10.1016/S0889-8588(05)70120-9CrossrefGoogle Scholar

  • [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, ECP1 Google Scholar

  • [17] Fornasier V., Protzner K., Radiation-induced tibial sarcoma in a treated case of hind foot angiomatosis, Skel. Radiol., 1998, 27, 164–168 http://dx.doi.org/10.1007/s002560050358CrossrefGoogle Scholar

About the article

Published Online: 2011-09-24

Published in Print: 2011-09-01


Citation Information: Translational Neuroscience, Volume 2, Issue 3, Pages 265–269, ISSN (Online) 2081-6936, ISSN (Print) 2081-3856, DOI: https://doi.org/10.2478/s13380-011-0032-3.

Export Citation

© 2011 Versita Warsaw. This work is licensed under the Creative Commons Attribution-NonCommercial-NoDerivatives 3.0 License. BY-NC-ND 3.0

Comments (0)

Please log in or register to comment.
Log in