Acquired Vascular Tumors of the Head and Neck

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Key points

  • •

    Acquired head and neck vascular tumors are rare and account for less than 5% of head and neck neoplasms.

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    Management of head and neck paragangliomas has evolved from primarily surgical to more conservative treatment consisting of observation and nonsurgical therapy.

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    The mainstay treatment of juvenile nasopharyngeal angiofibroma and hemangiopericytoma is surgery, with radiation reserved for adjuvant therapy and recurrent tumors.

Natural History and Physical Findings

Paragangliomas are vascular neoplasms that arise from the extraadrenal paraganglia derived from the neural crest and most commonly occur in the head and neck region. These tumors are closely associated with either blood vessels (carotid artery, jugular bulb) or nerves (vagus, tympanic plexus). Paragangliomas are usually slow-growing tumors with an average growth rate of 1 to 2 mm per year and a median doubling time of 4.2 years. Their growth pattern may be described as biphasic because very

Natural History and Physical Findings

Juvenile nasopharyngeal angiofibroma (JNA) is a highly vascular, histologically benign but locally aggressive and destructive tumor that exclusively affects adolescent boys. It accounts for approximately 0.5% of all head and neck neoplasms.57, 58 The etiologic factors and pathogenesis of the disease remain to be elucidated. The tumor seems to originate in the posterior nasal cavity instead of the nasopharynx, specifically in the posterolateral wall of the superior aspect of the nasal cavity, at

Treatment

The mainstay treatment of JNA is surgery, especially for the early-stage disease process. Previously, the various surgical approaches were: transpalatal, transnasal, transantral, transmandibular, transzygomatic, combined craniotomy and rhinotomy, lateral rhinotomy, and midface degloving. With increasing experience using endoscopic nasal techniques, excellent results have been achieved with this more minimally invasive approach. Ultimately, the approach chosen is determined by tumor location,

Natural History and Physical Findings

Hemangiopericytoma of the head and neck is a rare neoplasm that originates from the pericytes or cells of Zimmerman surrounding normal vascular channels.87 It is considered by some to be a lesion with low risk of malignant potential and by others a malignant lesion of high metastatic potential.87, 88 Thus, it is known as a tumor that varies greatly in appearance and biologic behavior.89 Hemangiopericytomas represent 3% to 5% of all soft-tissue sarcomas90 and 1% of all vascular tumors.91

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    Disclosure: The authors have nothing to disclose.

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