Angiomyolipoma of the Nasal Cavity: A Distinct Entity. A Case Report and Literature Review
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CASE REPORTS
Angiomyolipoma of the Nasal Cavity: A Distinct Entity. A Case Report and Literature Review Liping Wang1 · Bing Leng1 · Lina Liu1 Received: 16 April 2020 / Revised: 5 July 2020 / Accepted: 5 September 2020 © Springer Science+Business Media, LLC, part of Springer Nature 2020
Abstract Angiomyolipoma (AML) is a mesenchymal tumor commonly found in the kidneys. Extra-renal AML is uncommon and especially rare in the nasal cavity. To our knowledge, fewer than five cases of nasal AML are reported in the United States. We present a case of nasal AML in a 65-year-old man with a history of rhinosinusitis and obstruction of the left nasal cavity. The lesion comprised of admixed spindled smooth muscle cells, mature adipose tissue, and thick-walled blood vessels. Smooth muscle differentiation of the spindled cells was confirmed by expression of smooth muscle actin. Surprisingly, melanocytic markers, such as HMB45, were negative. The histologic features and immunoprofile suggest that nasal AML is pathologically distinct from neoplastic AMLs/perivascular epithelioid cell tumors (PEComas) that typically occur in the kidney. We propose that nasal AML is a hamartomatous lesion rather than neoplastic. Although AML is rare in the nasal cavity, it should be considered in the differential diagnosis of clinically benign nasal masses. Keywords Angiomyolipoma · Perivascular epithelioid cell tumor · Smooth muscle cells · HMB-45
Introduction
Case Report
AML is a mesenchymal tumor composed of variable proportions of blood vessels, smooth muscle cells, and adipocytes [1]. AML occurs sporadically or in association with tuberous sclerosis. It is considered a perivascular epitheliod cell tumor (PEComa) and characteristically expresses both smooth muscle markers and melanocytic markers [1]. AML most commonly occurs in the kidney and rarely in the liver, skin, gastrointestinal tract, heart, lung, spinal cord, and oropharynx [2–6]. The majority of nasal AMLs have been described in Asia and the Middle East, with fewer than five cases reported in the United States [7, 8]. Here, we report a case of AML originating in the nasal vestibule.
A 65-year-old male presented with a two-year history of rhinosinusitis and nasal congestion/obstruction. He denied epistaxis. Neither a personal or family history of tuberous sclerosis was reported. Computed tomography (CT) revealed a 1.7 × 1.6 cm soft tissue mass with hypervascularity arising from the left lateral nasal vestibule (Fig. 1). Gross examination of the excisional biopsy specimen demonstrated a 1.2 × 1.0 × 0.2 cm unoriented ellipse of tan-pink skin and a loosely attached, ovoid, tan-pink, soft tissue mass that measured 1.2 × 1.1 × 0.8 cm and demonstrated focal yellow discoloration. Microscopically, the mass was separate from the skin ellipse and showed no surface epithelial lining. The tumor comprised three disorganized components: adipose tissue (approximately 30%) with no cytological atypia, mature, spindled smooth muscle cells (SMCs), and thick-walled blood vessels (Figs. 2, 3). Immuno
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