Head and Neck Pathol (2015) 9:463–473 DOI 10.1007/s12105-015-0636-y ORIGINAL PAPER Angioleiomyoma of the Sinonasal Tract: Analysis of 16 Cases and Review of the Literature 1 2 3 2 Abbas Agaimy • Michael Michal • Lester D. R. Thompson • Michal Michal Received: 7 April 2015 / Accepted: 30 May 2015 / Published online: 6 June 2015 Ó Springer Science+Business Media New York 2015 Abstract Angioleiomyoma (ALM; synonyms: angiomy- The covering mucosa was ulcerated in 6 cases and showed oma, vascular leiomyoma) is an uncommon benign tumor squamous metaplasia in one case. There were no recur- of skin and subcutaneous tissue. Most arise in the rences after local excision. Submucosal sinonasal ALMs extremities (90 %). Head and neck ALMs are uncommon are rare benign tumors similar to their reported cutaneous (*10 % of all ALMs) and those arising beneath the counterparts with frequent adipocytic differentiation. They sinonasal tract mucosa are very rare (\1 %) with 38 cases should be distinguished from renal-type angiomyolipoma. reported so far. We herein analyzed 16 cases identified Simple excision is curative. from our routine and consultation files. Patients included seven females and nine males aged 25–82 years (mean 58; Keywords Angioleiomyoma Á Sinonasal tract Á median 62). Symptoms were intermittent nasal obstruction, Angiomyolipoma Á Vascular leiomyoma Á Angiomyoma Á sinusitis, recurrent epistaxis, and a slow-growing mass. PEComa Á Nasal Fifteen lesions originated within different regions of the nasal cavity and one lesion was detected incidentally in an ethmoid sinus sample. Size range was 6–25 mm (mean 11). Introduction Histologically, all lesions were well circumscribed but non- encapsulated and most (12/16) were of the compact solid Mesenchymal tumors of the sinonasal tract are rare. They type superficially mimicking conventional leiomyoma but encompass benign tumors (benign peripheral nerve sheath contained numerous compressed muscular veins. The tumors, angioleiomyoma and hemangiomas), lesions of remainder were of venous (2) and cavernous (2) type. low-grade or uncertain biological potential (sinonasal Variable amounts of mature fat were observed in four cases hemangio/glomangiopericytoma, solitary fibrous tumor, (25 %). Atypia, necrosis, and mitotic activity were absent. desmoid fibromatosis, low-grade malignant peripheral Immunohistochemistry showed consistent expression of nerve sheath tumors and low-grade sinonasal sarcoma with smooth muscle actin (12/12), h-caldesmon (9/9), muscle- neural and myogenic features) and frankly malignant specific actin (4/4), variable expression of desmin (11/14) aggressive neoplasms (conventional malignant peripheral and CD56 (4/6), and absence of HMB45 expression (0/11). nerve sheath tumors, leiomyosarcoma, rhabdomyosarcoma and other rare sarcoma types) [1–5]. Due to the rarity of sinonasal mesenchymal neoplasms, many pathologists are & Abbas Agaimy not familiar with their broad phenotypic spectrum. [email protected] Angioleiomyoma (ALM; synonyms: angiomyoma, vas- 1 Pathologisches Institut, Universita¨tsklinikum Erlangen, cular leiomyoma) is an uncommon benign tumor of skin Krankenhausstrasse 8-10, 91054 Erlangen, Germany and subcutaneous tissue composed of well differentiated 2 Sikl’s Department of Pathology, Faculty of Medicine, smooth muscle proliferations associated with variable but Charles University, Pilsen, Czech Republic usually prominent vascular component [6]. The latter may 3 Department of Pathology, Woodland Hills Medical Center, consist of thick-walled collapsed vascular channels (solid 5601 De Soto Avenue, Woodland Hills, CA 91367, USA type), predominant thick-walled venous vessels with well 123 464 Head and Neck Pathol (2015) 9:463–473 recognizable lumens within smooth muscle background symptoms in eight patients with detailed data, respectively. (venous type), or display ectatic muscular venous channels A painful mass was stated in one case. Other cases either mimicking venous hemangioma but with variable smooth lacked detailed history or a slowly growing mass or nodule muscle component in-between (cavernous type) [6]. The was the presenting symptom of the disease. The site was majority of lesions originate in the extremities (*90 %), stated within different compartments of the nasal cavity mainly in the lower limbs while ALM of the head and neck (five in turbinates, four in the nasal cavity unspecified, region is uncommon (*10 %) [7, 8]. Submucosal ALMs three from the nasal septum, two in the nasal orifices, and of the sinonasal tract are exceptionally rare. To date, 38 one in the lateral nasal wall). One case (the only sinus- cases have been reported, mostly as single case reports [9– based lesion) was found incidentally in a polyposis speci- 41] (Table 1). In this study, we describe our experience men from the ethmoid sinus. None of the lesions was with submucosal ALMs of sinonasal tract and discuss their multifocal or involved more than one subregion of the clinicopathological and immunohistochemical characteris- sinonasal tract. There was no evidence of associated dis- tics in light of previously reported cases with special eases or similar tumors elsewhere in the body. All lesions emphasis on the frequent presence of adipocytic differen- were removed via simple complete local excision with free tiation and similarities and differences compared to their albeit close margins. cutaneous and soft tissue counterparts. At last follow-up (range 9–211 months; mean 58 months), no recurrences were recorded. Materials and Methods Pathological Findings We reviewed our routine and consultation files for lesions Tumor size ranged from 6 to 25 mm (mean 11) in cases coded as angioleiomyoma, angiomyoma, angiomyolipoma with gross description or as measured from glass slides. and vascular leiomyoma originating in the nose, nasal Grossly, the lesions were described as tan-whitish with cavity, or paranasal sinuses. Diagnosis was based on cri- solid whorled cut-surface and firm consistency. Histologi- teria defined for similar lesions in the most recent World cally, all lesions were non-encapsulated but well circum- Health organization (WHO) classification of tumors of the scribed (Fig. 1a, b). The tumors were covered by sinonasal head and neck and tumors of soft tissue and bone [1, 6]. mucosa with variable reactive or metaplastic changes After review, only tumors arising from the mucosa-lined (Fig. 1c). Six tumors showed mucosal ulceration/erosion sinonasal sites (excluding cutaneous lesions) were included with variable inflammation between the tumor and mucosal in this series. The tumor specimens were fixed in buffered surface associated with variable degree of fibromyxoid formalin and embedded routinely for light microscopic vascular obliteration (Fig. 1d). The tumors were composed examination. Immunohistochemical studies were per- of well differentiated smooth muscle cells having elon- formed on 3–4-lm sections cut from paraffin blocks using gated vesicular blunt-ended nuclei with inconspicuous a fully automated system (‘‘Benchmark XT System’’, nucleoli and brightly eosinophilic fibrillary cytoplasm Ventana Medical Systems Inc, Tucson, Arizona, USA) without atypia. The cells were arranged in intersecting using the following antibodies: a-smooth muscle actin bundles and whorls encasing or surrounding numerous (clone 1A4, 1:200, Dako), h-caldesmon (clone h-CD, vascular channels. The smooth musculature of the vessel 1:100, Dako), desmin (clone D33, 1:250, Dako), CD34 walls seemed to merge gradually and imperceptibly with (clone QBEnd10, 1:200, Immunotech), CD56 (clone MRQ- the surrounding smooth muscle bundles. There was no 42, 1:100, CELL MARQUE), HMB45 (clone HMB45, cellular pleomorphism and mitotic figures were absent. 1:50, Loxo), and podoplanin (clone D2-40, 1:50, Twelve of 16 cases showed compact arrangement of the Zytomed). smooth muscle occasionally mimicking leiomyoma but careful assessment revealed the characteristic thick-walled collapsed vascular channels (solid type) (Fig. 2a). Two Results lesions showed convolutes of thick-walled venous channels closely mimicking venous hemangioma (venous type) Clinical Features (Fig. 2b). Another two cases showed prominent dilated venous vessels within the smooth muscle proliferation Sixteen cases were retrieved from our files (Table 1, Cases corresponding to the cavernous type (Fig. 2c). A variable 39–54). Patients included seven females and nine males but generally prominent adipocytic component was aged 25–82 years (mean 58; median 62). Variable combi- appreciated in four cases (25 %). Mature macrovesicular nations of intermittent nasal obstruction, chronic sinusitis- adipocytes were scattered either singly or forming small like symptoms and recurrent epistaxis were the presenting aggregates and lobules within the lesions. The fatty 123 Head and Neck Pathol (2015) 9:463–473 Table 1 Clinicopathological features of previously reported sinonasal angioleiomyomas including current series (n = 54) No. Author Age/gender Site, size cm Symptoms, duration Histological IHC positive IHC negative Associated Follow pattern markers markers mucosal up changes (months) 1 Maesaka et al. 49 F Vestibule Facial pain Vascular ND ND NS NR [9] 2 Ram [10] 40 M Right inferior Nasal obstruction, few mo. Solid (reported ND ND NS NA turbinate, 2.5 cm as fibromyoma) 3 Wolfowitz 42 F Inferior turbinate, Recurrent epistaxis Vascular ND ND Ulceration NR (30 et al. [11] 1cm leiomyoma mo.) 4 Schwartzman 57 M Sinuses Obstruction, headache Vascular ND ND NS NR (36 et al. [12] mo.)