Sinonasal Angioleiomyoma With Adipocyte Differentiation: Clinicopathologic Study of 2 Cases and Review of the Literature

Abstract

Two patients presented to the otolaryngology department of our university with a history of left nasal obstruction. The first case, a 50-year-old man, presented with a 3-month progressively worsening history of left maxillary swelling with mild facial pain and left nasal obstruction. A previous diagnosis of AIDS was reported; tuberous sclerosis was excluded by clinical workup. No alcohol consumption or smoking was reported. Nasal endoscopy revealed the presence of a reddish mass in the middle meatus, arising from the left inferior turbinate (Figure 1A). Narrow band imaging evaluation showed a relatively regular arrangement of intraepithelial capillary; the microvascular network and submucosal veins are nearly invisible (Figure 1B). The patient underwent a maxillofacial computed tomography (CT) scan with contrast that confirmed the presence of a soft density tissue of 13 mm in diameter, occupying the posterior portion of the middle turbinate, without evidence of bony erosion in the medial wall ( Figure 1C). The patient underwent an endoscopic resection of the mass. No signs of recurrence of the disease were observed 1 year after surgery.The second case, a 63-year-old male, presented with left-side nasal obstruction and rhinorrhea. Anterior rhinoscopy revealed in the left nasal fossa a smooth spherical nasal mass arising from the head of the left inferior turbinate, without bleeding on touch. Contrast-enhanced CT scan showed a soft density tissue, arising from the head of the left inferior turbinate, without evidence of bony erosion (Figure 2). These clinical features were suggestive of a benign tumor; thus, a complete excision with nasal endoscopy was performed. No signs of recurrence of the disease were observed 5 months after surgery.Both excised tumors consisted of vascular channels surrounded by thick muscular coats associated with intersecting fascicles of spindle cells. Cavernous-like vessels were also recognized in case 1 and aggregates of lymphocytes in case 2. Of note, mature adipocytes were identified in both tumors. While in case 1 they formed large aggregates, in case 2 they were distributed either singularly or in very small clusters. In both tumors, mitosis, pleomorphism, and necrosis were absent, and immunohistochemical analysis performed as described previously 1 demonstrated positivity of the spindle cells for smooth muscle actin and musclespecific actin and negativity for S100, HMB45, MelanA, estrogen receptor (ER) and progesterone receptor (PR); the proliferative activity (Ki67) was less than 3%, and Epstein-Barr Encoding Region (EBER) in situ hybridization was negative (Figure 3). Based on these findings, the pathologic diagnosis of sinonasal-ALM with adipocytic differentiation 2 was made.The occurrence of mature adipocytes in an otherwise typical sinonasal-ALM is not uncommon. Indeed, the term angiomyolipoma has been inappropriately used to describe typical sinonasal-ALM. However, angiomyolipoma is a completely different entity, since lesional cells co-express melanocytic (mainly HMB45) and my...

Extraneural Sclerosing Perineurioma of the Tongue

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Naso-Ethmoidal Phosphaturic Mesenchymal Tumor: A Rare Tumor Site for an Uncommon Paraneoplastic Syndrome

“…6,7 At this level, PMTs often lack the distinctive (grungy) calcified matrix and multinucleated giant cells that characterize PMTs of soft tissues and bones and commonly present clusters of mature adipocytes and florid hemangiopericytoma-like vasculature associated with fascicles of myoid-appearing cells that may mimic other vascular and perivascular tumor. 5,8 To this regard, even though not specific, the immunoreactivity of the neoplastic cells for CD56 may be helpful because its expression in mesenchymal tumors is limited. 9 Production of the ''phosphatonin'' FGF23 by the neoplastic cells is the mechanism by which PMTs cause TIO.…”

Diagnosis, Treatment and Follow-Up of Sinonasal Leiomyomas: A Systematic Review

“…7 case reports included literature reviews, 3,35–40 and 8 studies were case series. 41–48 Among these studies, a total of 84 patients were described (Supplemental Table 1).…”