RESUMO
Juvenile angiofibroma (JNA) is a relatively uncommon, highly vascular and benign tumor that presents most commonly in adolescent males. Symptoms may persist from months to years and often times, these tumors are asymptomatic until they increase and encroach on critical structures. Because of technological advances both in surgery and radiology, management of JNA patients has been refined. With the advent of more sophisticated capabilities such as CT, MRI, intensity-modulated radiation therapy (IMRT), stereotactic guidance systems as well as advanced embolization techniques, these tumors can be diagnosed and managed more effectively.Patients with juvenile angiofibroma (JNA) are typically silent for years and often present with epistaxis, nasal obstruction, facial numbness, rhinorrhea, ear popping, sinusitis, cheek swelling, visual changes and headaches. In addition to these symptoms, up to one-third of patients with this condition may present with proptosis or other orbital involvement, which are late symptoms and findings.Most physicians agree that surgery is the primary treatment modality for the early-stage disease process. However, controversy arises regarding the best treatment when a patient presents with more locally advanced disease involving widespread cranial-based extension or intracranial involvement which may necessitate a combination of treatment modalities including surgery and postoperative radiation.With the advancement of endoscopic surgery, there have been a number of cases reporting the value of its use. The purpose of this review, however, will address not only endoscopic alternatives, but will discuss other treatment options as reported in the literature. Robotic surgery of the skull base for JNA is something to expect for the future.Finally, with the advent of IMRT and an image-guided robotic radiotherapy delivery system, some researchers speculate that this will result in less objections for radiation in general and certainly less reservations for the use radiotherapy in certain circumstances, i.e. patient refusal of surgery or extensive non-resectable or recurrent JNA tumors.
RESUMO
Superficial leiomyosarcomas are rare in the head and neck region. Because of the infrequent nature of soft tissue sarcomas in general, superficial leiomyosarcomas are often misdiagnosed on clinical grounds. Immunohistochemistry is essential for an accurate histologic diagnosis, and it should include a broad panel of antibody studies. With respect to differences in clinical appearance and biologic behavior, superficial leiomyosarcomas can be broadly classified as either cutaneous or subcutaneous; local control and overall survival are significantly more favorable in patients with the former. The primary treatment of a leiomyosarcoma is a wide surgical excision with an emphasis on negative margins. Treatment failures are usually attributable to a local recurrence. Systemic metastasis occurs in about one-third of patients with subcutaneous involvement. Although cutaneous leiomyosarcoma is considered a relatively more benign process with minimal metastatic potential, systemic metastasis is still possible. This was demonstrated in our case, as a recurrent cutaneous leiomyosarcoma metastasized to the lung. Proper management requires inclusion of this entity in the differential diagnosis, as well as familiarity with its clinical behavior. In this article, we review the literature on superficial leiomyosarcoma and discuss its epidemiology, presentation, clinical behavior, evaluation, tissue diagnosis, staging, and treatment.