Summary
We present a rare case of temporal bone angiosarcoma diagnosed in a 26-year-old female patient at 36 week of pregnancy. The patient was referred with a 2 months history of left otalgia and tinnitus with a tender swelling above the mastoid. Cranial imaging studies showed a 7 × 5 × 4 cm hypervascularized mass located in the left middle fossa with lysis of the temporal bone and extension to the subcutis. After the baby was delivered by caesarean section, the patient entered the oncology protocol. Selective embolization of the feeding vessels was followed by gross total surgical resection using a combined supra- and infra-tentorial approach. Pathological findings were those of a poorly differentiated, highly malignant sarcoma with a large epitheloid component and immunohistochemical evidence of endothelial differentiation (CD31, Factor VIII related antigen, CD34), consistent with an angiosarcoma with epitheloid features. No extra-cranial tumor was found after extensive staging. The patient received adjuvant radiotherapy followed by a course of chemotherapy consisting of 6 cycles of paclitaxel. At 15 months follow-up, she developed multiple distant metastasis to a left postauricular lymph node and to the lungs and ribs. The patient was given a second line chemotherapy using doxorubicine and ifosfamide. Despite an initial good response, she died with metastatic disease 26 months after diagnosis. We present a rare case of primary temporal bone angiosarcoma and report our experience with a multimode therapeutic approach combining surgery, radiotherapy and chemotherapy.
Article PDF
Similar content being viewed by others
Avoid common mistakes on your manuscript.
References
FM Enzinger SW. Weiss (Eds) (1995) Soft Tissue Tumors EditionNumber3 Mosby St. Louis, MO 648–677
FG Fedok RJ Levin ME Maloney K Tipirneni (1999) ArticleTitleAngiosarcoma: current review Am J Otolaryngol 20 IssueID4 223–231 Occurrence Handle10.1016/S0196-0709(99)90004-2 Occurrence Handle10442774
N Naka M Ohsawa Y Tomita H Kanno A Uchida K Aozasa (1995) ArticleTitleAngiosarcoma in Japan: a Review of 99 Cases Cancer 75 IssueID4 989–996 Occurrence Handle7842420
M Cookston GW Cotter M Schlitt FO. Bastian (1991) ArticleTitlePrimary angiosarcoma of the brain South Med J. 84 IssueID4 517–520 Occurrence Handle2014445
IR Kirk R Dominguez M. Castillo (1992) ArticleTitleCongenital primary cerebral angiosarcoma: CT, US, and MR findings Pediatr Radiol. 22 IssueID2 134–135 Occurrence Handle10.1007/BF02011315 Occurrence Handle1501943
H Mena J Ribas F Enzinger J. Parisi (1991) ArticleTitlePrimary angiosarcoma of the central nervous system: study of eight cases and review of the literature J Neurosurg 75 73–76 Occurrence Handle2045922
W Paulus F Slowik K. Jellinger (1991) ArticleTitlePrimary intracranial sarcomas: histopathological features of 19 cases Histopathology 18 IssueID5 395–402 Occurrence Handle1715839
DS Russell LJ. Rubinstein (1989) Pathology of Tumours of the Nervous System EditionNumber5 Williams & Wilkins Baltimore 657–658
C Antoniadis P Selviaridis T Zaramboukas G. Fountzilas (1996) ArticleTitlePrimary angiosarcoma of the brain: case report Neurosurgery 38 583–586 Occurrence Handle10.1097/00006123-199603000-00033 Occurrence Handle8837814
JFR Flemming JHN Deck M. Bernstein (1985) Intracranial sarcomas RH Wilkins SS. Rengachary (Eds) Neurosurgery. McGraw-Hill New York 1030–1036
M Lopes H Duffau G. Fleuridas (1999) ArticleTitlePrimary spheno-orbital angiosarcoma: case report and review of the literature Neurosurgery 44 IssueID2 405–407 Occurrence Handle10.1097/00006123-199902000-00102 Occurrence Handle9932897
E Bourekas M Cohen C Kamen R Tarr C Lanzieri J. Lewin (1996) ArticleTitleMaligant hemangioendothelioma (angioasarcoma) of the skull: plain film, CT and MR Appearance AJNR Am J Neuroradiol 17 1946–1948 Occurrence Handle8933884
WS Goldstein BC Bowen T. Balkany (1994) ArticleTitleMalignant hemangioendothelioma of the temporal bone masquerading as glomus tympanicum Ann Otol Rhinol Laryngol 103 IssueID2 156–159 Occurrence Handle8311392
RA Ibarra P Kesava KK Hallet C. Bogaev (2001) ArticleTitleHemangioendothelioma of the temporal bone with radiologic findings resembling hemangioma AJNR Am J Neuroradiol 22 IssueID4 755–758 Occurrence Handle11290494
R Volpe A. Mazabraud (1982) ArticleTitleHemangioendothelioma (angiosarcoma) of bone: a distinct pathologic entity with an unpredictable course? Cancer 15 49 IssueID4 727–736
YC Chou YL Chang T Harnod WF Chen CF Su SZ Lin YH Hsu PS Yen CC. Lee (2004) ArticleTitlePrimary angiosarcoma of the cranial vault: a case report and review of the literature Surg Neurol 61 IssueID6 575–579 Occurrence Handle10.1016/j.surneu.2003.07.002 Occurrence Handle15165804
R Eliashar D Saah P Osin JY. Sichel (1997) ArticleTitleHemangioendothelioma of the temporal bone in a child Int J Pediatr Otorhinolaryngol 40 IssueID1 67–71 Occurrence Handle10.1016/S0165-5876(96)01480-2 Occurrence Handle9184980
HZ Joachims Y. Cohen (1974) ArticleTitleHemangioendothelioma of the mastoid bone Laryngoscope 84 IssueID3 454–458 Occurrence Handle4814415
JM. Kinkade (1948) ArticleTitleAngiosarcoma of the petrous portion of the temporal bone Report of a case. Ann Otol Rhinol Laryngol 57 235–240
M. Masieh (1980) ArticleTitleHaemangiosarcoma of the petrous temporal bone J Laryngol Otol 94 IssueID2 205–210 Occurrence Handle7189541
M Muhlau J Schlegel HG Einsiedel ParticleVon B Conrad D. Sander (2003) ArticleTitleMultiple progressive intracerebral hemorrhages due to an angiosarcoma: a case report Eur J Neurol 10 IssueID6 741–742 Occurrence Handle10.1046/j.1468-1331.2003.00680.x Occurrence Handle14641524
KK Unni JC Ivins JW Beabout DC. Dahlin (1971) ArticleTitleHemangioma, hemangiopericytoma, and hemangioendothelioma (angiosarcoma) of bone Cancer 27 IssueID6 1403–1414 Occurrence Handle5088217
ES Casper RJ Waltzman GK Schwartz A Sugarman D Pfister D lson J Woodruff D Leung JR. Bertino (1998) ArticleTitlePhase II trial of paclitaxel in patients with soft-tissue sarcoma Cancer Invest 16 IssueID7 442–446 Occurrence Handle9774950
F Fata E O’Reilly D Ilson D Pfister D Leffel DP Kelsen GK Schwartz ES. Casper (1999) ArticleTitlePaclitaxel in the treatment of patients with angiosarcoma of the scalp or face Cancer 86 IssueID10 2034–2037 Occurrence Handle10.1002/(SICI)1097-0142(19991115)86:10<2034::AID-CNCR21>3.0.CO;2-P Occurrence Handle10570428
Author information
Authors and Affiliations
Corresponding author
Rights and permissions
About this article
Cite this article
Scholsem, M., Raket, D., Flandroy, P. et al. Primary temporal bone angiosarcoma: a case report. J Neurooncol 75, 121–125 (2005). https://doi.org/10.1007/s11060-005-0375-0
Issue Date:
DOI: https://doi.org/10.1007/s11060-005-0375-0