a SciTechnol journal Case Report Crowson et al., J Otol Rhinol 2013, 2:4 http://dx.doi.org/10.4172/2324-8785.1000132 International Publisher of Science, Technology and Medicine Journal of Otology & Rhinology All articles published in Journal of Otology & Rhinology are the property of SciTechnol, and is protected by copyright laws. Copyright © 2013, SciTechnol, All Rights Reserved. Primary Angiosarcoma of the Temporal Bone in a 55-year- old Male with Audiovestibular Symptoms Matthew Gordon Crowson 1 , Kevin Higgins 1 , Vincent Lin 1 *, Robert Yeung 2 and Simon Raphael 3 Abstract We report a case of a 55-year-old man who presented with a history of new audiovestibular symptoms in context of chronic right ear and mastoid pain. Imaging demonstrated severe bony erosion of the temporal bone, and urgent mastoidectomy revealed a mass suspicious for spindle cell variant squamous cell carcinoma. An extensive en-bloc resection was completed, and inal pathology indicated high grade angiosarcoma. We detail the imaging and pathological techniques employed that led to this diagnosis. This is the eighth case of primary angiosarcoma of the temporal bone reported in literature, and possibly the irst ever recorded in an adult male. Keywords Angiosarcoma; Temporal bone; Skull base *Corresponding author: Vincent Lin, Department of Otolaryngology-Head & Neck Surgery, Sunnybrook Health Sciences Centre, 2075 Bayview Ave, Room M1-102, Toronto, ON M4N 3M5, Canada, Tel: 416- 480-6100 (ext. 7251); Fax: 416- 480-5761; E-mail: Vincent.Lin@Sunnybrook.ca Received: June 27, 2013 Accepted: September 15, 2013 Published: October 25, 2013 pain. He was treated with IV ceftazadime three weeks prior for presumed osteomyelitis of the temporal bone. His facial symptoms showed marginal improvement with a supplemental 1-week course of prednisone. A CT head & temporal bone scan showed severe bony erosion with soft tissue illing mastoid air cells with extension into the middle cranial fossa (Figure 1). One week later he was assessed in our clinic. He had an incomplete right facial paralysis with a House Brackmann score of III. His right external auditory canal was extremely edematous and illed with granulation tissue. However, a repeat CT scan was ordered and within a short time frame, the bony erosion had progressed so a right mastoidectomy was performed urgently. During surgery, some suspicious soft tissue was noted illing mastoid air cells and extending into the middle cranial fossa. Upon further exploration, this tissue was found adherent to the middle cranial fossa dura with extension onto the vertical segment of the facial nerve. The mass was biopsied and debulked with some residual mass remaining on the dura. Initial pathology frozen sections suggested spindle- cell variant of squamous cell carcinoma. A post-operative MRI of the head demonstrated enhancement of the right temporal lobe dura with extension right up to Meckel’s Cave (Figure 2). Posterior fossa dura also enhanced, and a large amount of extradural mass was detected in the infratentorial middle cranial fossa, the petrous apex, as well as deep to the bony labyrinth. His facial nerve enhanced up to the geniculate ganglion and into the internal auditory canal. An en-bloc resection was completed with middle craniectomy, translabyrinthine/transotic approach to the internal auditory canal and Meckel’s cave, facial nerve sacriice at the internal auditory canal, modiied radical neck dissection, parapharyngeal space dissection, parotidectomy and anterolateral thigh lap reconstruction. The extradural Introduction Angiosarcoma is a rare malignant sot-tissue sarcoma with a poor prognosis. he histological hallmarks under light microscopy include cytologically atypical cells arranged in iniltrating, anastomosing channels. Angiosarcomas represent 2% of all sot tissue sarcomas, and 50% of this sub-set are found in the head and neck [1,2]. hese tumors most oten present on the scalp as bruise-like cutaneous lesions with poorly deined borders [3]. At the time of writing, there have only been 17 prior cases of primary intracranial angiosarcomas reported in MEDLINE and other web databases [4,5]. We present an extremely rare case of a temporal bone angiosarcoma masquerading as osteomyelitis in a middle-age male. Case Report A 55-year-old insulin-dependent diabetic presented as an outpatient to the department of otolaryngology with a history of draining right ear, severe pain, vertigo, and acute facial palsy in context of long-standing chronic right ear and mastoid Figure 1: Axial (A) and coronal (B) CT images showing opaciication of the right mastoid air cells and middle ear with extensive bone erosions.