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Nasopharyngeal angiofibroma is an uncommon benign vascular tumor that occurs in adolescent boys and young men. We describe an unusual histological variant of this entity characterized by a prominent adipose tissue component. The patient was a 35-year-old man with a 20-year history of nasal obstruction. Results of physical examination and angiography demonstrated a nasal mass consistent with a nasopharyngeal angiofibroma. We discuss the differential diagnoses pathologically for this lesion.Nasopharyngeal angiofibroma is a benign vascular tumor that occurs almost exclusively in adolescent boys and young men. It is uncommon, accounting for only 0.5% of all head and neck tumors.Its benign histological appearance belies its potential local aggressiveness, tendency for local recurrence, and occasional malignant transformation.This tumor is composed of dense fibrous stroma with interspersed, variably sized slitlike or gaping vascular channels.Angiofibromas with a prominent mature adipose tissue component have not been reported. However, mature adipose tissue can be found in hamartomas, which are benign tumorlike malformations or inborn errors of tissue development, composed of an abnormal mixture of tissues indigenous to the affected region.We report a unique case of nasopharyngeal angiofibroma that contains a prominent adipose tissue component and resembles a hamartoma.REPORT OF A CASEA 35-year-old man presented with a 20-year history of left-sided nasal obstruction. Although the obstruction progressed to bilateral involvement during the past year, there were no associated symptoms such as epistaxis, pain, numbness, or visual disturbances.Physical examination demonstrated a left nasal mass with a pink surface and marked septal deviation to the right. The remainder of the examination results were unremarkable. Results of an arteriography showed a hypervascular lesion in the left nasal cavity compatible with an angiofibroma, supplied primarily via the sphenopalatine branch of the internal maxillary artery (Figure 1). The mass was then successfully embolized and excised endoscopically. The postoperative period was uneventful, and the patient was discharged the next day.Figure 1.Arteriography shows a hypervascular lesion in the left nasal cavity supplied primarily via the sphenopalatine branch of the internal maxillary artery.The lesion was a polypoid mass measuring 3.0 cm at its greatest dimension. The outer surface was smooth and pink. Sectioning revealed a firm, spongy, pink-gray, and yellow cut surface without hemorrhage or necrosis. Microscopic examination showed the following 3 components: blood vessels, fibrous stroma, and abundant mature adipose tissue (Figure 2). The blood vessels, varying in size and configuration, were thinly walled, lined by a single layer of endothelium, and surrounded by diminished elastic and smooth muscle layers (Figure 3). The fibrous stroma was cellular and dense, with focal hyalinization, but did not contain epithelial elements. The fatty component was composed of mature adipose tissue and constituted approximately 50% of the tumor volume (Figure 4). This fatty component was not compartmentalized but was diffuse throughout the lesion, blending with the fibrous stroma. The overlying mucosa had focal squamous metaplasia. We found no cytologic atypia or mitosis.Figure 2.Nasopharyngeal angiofibroma consisting of thin-walled blood vessels, varying in size and configuration, and surrounded by dense fibrous stroma and abundant mature adipose tissue (hematoxylin-eosin, original magnification ×50).Figure 3.Blood vessels lined by a single layer of endothelium and surrounded by diminished elastic and smooth muscle layers (hematoxylin-eosin, original magnification ×200).Figure 4.Intimate admixture of mature adipose tissue, fibrous stroma, and vasculature (hematoxylin-eosin, original magnification ×100).COMMENTAngiofibromas are distinctive fibrovascular tumors that occur almost exclusively in adolescent boys and young men. Most patients present with nasal obstruction and epistaxis. These tumors typically originate from a vascular nidus in the posterolateral wall of the nasal cavity in the vicinity of the sphenopalatine foramen.On roentgenographic examination, a nasopharyngeal soft tissue mass in a young man associated with anterior bowing of the posterior wall of the maxillary sinus (Holman-Miller sign) is practically pathognomonic of an angiofibroma. The blood supply is typically derived from the ascending pharyngeal or internal maxillary divisions of the external carotid artery, although branches of the internal carotid artery and others have also been implicated.Histologically, most angiofibromas are composed of dense fibrous tissue with interspersed blood vessels of varying size and configuration.The blood vessels are usually delicate and lined by a single layer of endothelium. Because their walls are devoid of an elastic layer and a complete smooth muscle layer,these vessels cannot undergo vasoconstriction when traumatized, causing profound hemorrhage. The fibrous component is usually dense and cellular. It may appear fascialike because of the parallel arrangement of the collagen fibersand may contain foci of hyalinization and/or myxoid degeneration. The stromal cells, which represent fibroblasts and/or myofibroblasts, have round to ovoid or stellate nuclei and, occasionally, prominent nucleoli. Mitoses are absent.Hamartomas are tumorlike but nonneoplastic malformations or inborn errors of tissue development, characterized by an abnormal mixture of tissues indigenous to that site. This feature distinguishes hamartomas from teratomas or dermoid cysts. The lesional tissues may be derived from any of the 3 germinal layers. Because they are spontaneous growths in nature and stop growing when the cells reach maturity, hamartomas are self-limiting.Nasopharyngeal hamartomas are extremely rare. Patients often present with obstructive or infectious symptoms early in life. Two histological types are found. One type is composed predominantly of specific mesodermal tissues and has been termed lipoma, fibrolipoma, chondroma, neurofibroma, or angioma.Whether they are true hamartomas is debatable. The other type contains a spectrum of squamous and cuboidal epithelial ducts, serous/mucinous exocrine glands, and various stromal elements. Depending on the predominating tissue type, it is designated as angiomatous, lipomatous, or lymphomatous hamartoma.In our case, the 35-year-old man appeared to have had a nasal mass since adolescence. The mass may have continued to grow, as suggested by progression of his nasal obstruction in the last year before surgery. Results of computed tomography and angiography showed a nasopharyngeal mass compatible with angiofibroma, but the adipose tissue component was not apparent. Angiographic findings demonstrated that the blood supply of this tumor was the sphenopalatine branch of the internal maxillary artery. Pathological examination showed a vascular lesion in a fibrous background admixed with abundant mature adipose tissue. Based on these clinical and pathological data, this lesion is consistent with a lipomatous variant of nasopharyngeal angiofibroma.Because this lesion was composed of vascular, fibrous, and adipose tissue components, we considered a differential diagnosis of nasopharyngeal hamartoma. However, most nasopharyngeal hamartomas become symptomatic early in life and cease to grow as patients mature, which was not consistent with our patient's history. In addition, the vessels in hamartomas have normal elastic and smooth muscle layers, which were not present in our case.We also considered angiolipoma in the differential diagnosis. However, angiolipoma is typically a small, painful subcutaneous nodule, and not a nasopharyngeal mass. 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G. Paulino, MD, Department of Pathology, University of Michigan Hospitals, 1500 E Medical Center Dr, Room 2G332, Ann Arbor, MI 48109 (e-mail: email@example.com).
JAMA Otolaryngology - Head & Neck Surgery – American Medical Association
Published: Apr 1, 2002
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