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Update on Select Benign Mesenchymal and Meningothelial Sinonasal Tract Lesions

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Update on Select Benign Mesenchymal and Meningothelial Sinonasal Tract Lesions Head and Neck Pathol DOI 10.1007/s12105-016-0697-6 SPECIAL ISSUE: SINONASAL TRACT PATHOLOGY. GUEST EDITORS: JUSTIN BISHOP, MD AND ALESSANDRO FRANCHI, MD Update on Select Benign Mesenchymal and Meningothelial Sinonasal Tract Lesions 1 2,3 Lester D. R. Thompson • Julie C. Fanburg-Smith Received: 6 July 2015 / Accepted: 10 August 2015 Ó Springer Science+Business Media New York (outside the USA) 2016 Abstract Several benign and malignant mesenchymal Nerve sheath neoplasms Á Diagnosis Á Differential Á and meningothelial lesions may preferentially affect or Meningioma Á Solitary fibrous tumor extend into the sinonasal tract. Glomangiopericytoma (GPC, formerly sinonasal-type hemangiopericytoma) is a specific tumor with a predilection to the sinonasal tract. Introduction Sinonasal tract polyps with stromal atypia (antrochoanal polyp) demonstrate unique histologic findings in the sino- Benign mesenchymal and meningothelial lesions uncom- nasal tract. Juvenile nasopharyngeal angiofibroma (JNA) monly affect the sinonasal tract, but are frequently a source arises from specialized tissue in this location. Meningioma of difficulty for pathologists. Epithelial lesions are much may develop as direct extension from its intracranial more common, but may take on a spindled, mesenchymal counterpart or as an ectopic tumor. Selected benign mes- or biphasic appearance, resulting in further difficulty. enchymal tumors may arise in the sinonasal tract and pose Malignant mesenchymal tumors may more commonly be a unique differential diagnostic consideration, such as identified in other head and neck locations. This paper solitary fibrous tumor and GPC or lobular capillary specifically discusses rare benign lesions that are unique to hemangioma and JNA. Although benign and malignant the sinonasal tract, such as a glomangiopericytoma (GPC, vascular, fibrous, fatty, skeletal muscle, and nerve sheath sinonasal tract hemangiopericytoma), antrochoanal polyp tumors may occur in this location, this paper focuses on a (ACP or inflammatory polyp with stromal atypia), and highly select group of rare benign sinonasal tract tumors juvenile nasopharyngeal angiofibroma (JNA), while soli- with their clinicopathological and molecular findings, and tary fibrous tumor (SFT), lobular capillary hemangioma differential diagnosis. (LCH) and meningioma may develop in this location, but are often unexpected or may have overlapping features Keywords Sinonasal tract Á Hemangiopericytoma Á with other lesions of this region. The following is a dis- Angiofibroma Á Polyps Á Granuloma Á Pyogenic Á cussion of these entities, along with their differential diagnosis. & Lester D. R. Thompson Glomangiopericytoma [email protected] Almost from its initial description by Stout and Murray as a 1 Department of Pathology, Woodland Hills Medical Center, Southern California Permanente Medical Group, 5601 De tumor primarily composed of pericytic cells [1], heman- Soto Avenue, Woodland Hills, CA 91365, USA giopericytoma (HPC) as a specific tumor type has been 2 Uniformed Services University of the Health Sciences debated. The histologic features of the sinonasal type HPC (USUHS), Bethesda, MD, USA appear unique, combined with a myoid phenotype (a site- 3 Department of Pathology, Sibley Memorial Hospital of Johns specific myofibroma), as a part of the myopericytic family Hopkins Medicine, Washington, DC, USA of tumors [2–5]. Although there are morphologic 123 Head and Neck Pathol similarities to other myopericytic entities, ‘‘GPC’’ of the prominent thick, acellular peritheliomatous hyalinization, a sinonasal tract is a relatively distinct tumor, defined as a feature uniquely characteristic of this tumor (Fig. 1a). tumor demonstrating composite features of perivascular Lacking distinct cell borders, the closely packed, uniform modified smooth muscle (myoid phenotype of glomus oval to elongated cells have a syncytial architecture. The tumor) arranged in a characteristic syncytium of ovoid to nuclei are round, oval to spindle-shaped, vesicular to spindle-shaped cells in a variably fibrotic stroma with hyperchromatic, surrounded by non-descript amphophilic, distinctive branching vessels (HPC) [6–10]. eosinophilic to cleared cytoplasm. Mitoses are limited (\3/ 10 high power fields) and nuclear pleomorphism is absent to Clinical Features mild. Necrosis and atypical mitoses are not identified. Three additional characteristic findings include mast cells, eosi- GPCs predilect to the nasal cavity and paranasal sinuses, nophils, and extravasated erythrocytes (Fig. 1b), with nearly where they comprise \0.5 % of all sinonasal tract neo- all tumors possessing these hematologic elements. In a few plasms [6, 11, 12]. With a peak in the 7th decade (range, in cases, tumor giant cells may be identified, interpreted to utero to 86 years), there is a slight female predominance. represent aggregation of cells as part of a degenerative This tumor is mostly unilateral, affecting the nasal cavity change, similar to symplastic glomus tumor [19]. Fibrosis or alone, occasionally extending into the paranasal sinuses, myxoid change may be present in a few areas, but only in a with about 5 % bilateral [6, 11–14]. The majority of small percentage of cases. Rare examples of gloman- patients experience nasal obstruction and epistaxis, with a giopericytoma may contain mature adipose tissue (lipoma- wide range of other non-specific findings (polyps, sinusitis, tous GPC) or reveal extramedullary hematopoiesis [6, 20– headaches, difficulty breathing, congestion), usually pre- 22]. Concurrent, collision tumors may be found, with SFT sent for less than 1 year on average. Severe oncogenic noted as a distinctly separate and immunophenotypically osteomalacia occurring in association with GPC is reported different neoplasm [6, 23, 24]. Rarely, severe pleomor- [11, 15]. GPCs are indolent tumors, with an overall phism, necrosis and increased mitoses may be identified, excellent survival ([90 % 5-year survival) achieved with with a malignant designation applied to these cases, which complete surgical excision. Recurrences, in up to 40 % of have a much higher chance of developing recurrence or cases, are usually a result of inadequate resection, with the rarely metastasis with death due to disease [6]. recurrence affecting the same site, occurring from a few months up to two decades after the initial surgery [6, 11, Ancillary Techniques 13, 14, 16, 17]. Aggressive behavior (malignant GPC) is uncommon, suggested by large tumors ([5 cm), bone GPC show a distinct immunohistochemistry profile, invasion, profound nuclear pleomorphism, increased showing a diffuse reactivity with actins (smooth mus- mitotic activity ([4/10 high power fields; Ki-67 prolifera- cle [ muscle specific, Fig. 1c), vimentin, nuclear b-catenin tion index of [10 %), and necrosis, with rare metastasis (Fig. 1d), cyclin-D1 and factor XIIIA, while lacking any reported [6, 11–13, 18]. significant expression of CD34, CD31, FVIII-R Ag, CD117, STAT6, bcl-2, AE1/AE3, CK7, EMA, desmin, Pathology Findings S100 protein, GFAP, CD68, CD99, and NSE. Individual cells are highlighted with laminin, similar to argyrophilic The tumors are generally polypoid, with an average size of stains that envelop individual pericytes by a silver 3.0 cm, ranging up to 8.0 cm. They are non-translucent, impregnated matrix representing basal laminar material beefy red to grayish pink, soft, edematous and fleshy to [25]. Rare cases have demonstrated focal bcl-2, S100 friable tumors. It is remarkable how similar these tumors are protein, CD34, GFAP and CD68 [6, 8, 11, 17, 18, 26]. The one to another. These unencapsulated tumors are identified intensity of immunoreactivity may vary both within and below an intact respiratory or metaplastic squamous between cases, ranging from diffuse and strong to focal and epithelium. Sometimes surface erosion is observed due to weak, but in general, the SMA, b-catenin and cyclin-D1 are friction of a large polypoid mass within the nasal cavity. The strong and diffuse reactions. The tumor giant cells characteristic appearance is that of a ‘‘patternless’’ diffuse demonstrate an identical immunohistochemical antigenic architecture, frequently effacing or surrounding normal profile to the tumor cells, representing true neoplastic giant tissue (Fig. 1a). The cells may be arranged in short fascicles, cells not macrophage-type giant cells. storiform, whorled, ‘‘meningothelial’’, reticular, or short b-catenin, a cadherin-associated membrane protein, palisades of closely packed cells. The cells are separated by participates in the regulation of cell-to-cell adhesion, a ter- a rich vascularity, revealing capillaries to large patulous minal component of the Wnt-signaling pathway. Aberrant spaces giving the characteristic ‘‘staghorn’’ or ‘‘antler-like’’ expression of b-catenin is a well-known event in tumori- configuration. Sometimes focal, there is usually a very genesis and tumor progression [27, 28]. Strong nuclear b- 123 Head and Neck Pathol Fig. 1 Glomangiopericytoma (GPC). a Intact surface epithelium Extravasated erythrocytes and eosinophils are noted. c Strong and overlying a patternless proliferation with well developed perithe- diffuse smooth muscle actin reaction. d Nuclear b-catenin reaction in liomatous hyalinization. b Oval to elongated nuclei in syncytial cells. the neoplastic cells catenin and cyclin D1 immunoexpression is observed in melanoma, leiomyosarcoma, angiosarcoma, spindled cell nearly all cases of GPC. Somatic, single nucleotide substi- ‘‘sarcomatoid’’ squamous cell carcinoma, synovial sarcoma,
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