Sox10—A Marker for Not Only Schwannian and Melanocytic Neoplasms But Also Myoepithelial Cell Tumors of Soft Tissue

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Low-grade fibromyxoid sarcoma (LGFMS) is a deceptively bland malignancy with potential for late recurrence and metastasis, which usually occurs in the deep soft tissues of the extremities and trunk. Most LGFMSs harbor a characteristic gene fusion of FUS-CREB3L2, and recently MUC4 immunostaining has been found to be highly sensitive and specific for the diagnosis. We present a dedicated series of head and neck LGFMS, including the first reported laryngeal case, as well as a review of reported head and neck cases. The surgical pathology archives of our three institutions were searched for cases of LGFMS arising within the head and neck, and four cases were identified. The H&E slides were reviewed, and immunohistochemistry were performed for pancytokeratin, p63, p40, EMA, S100 protein, β-catenin, actin, CD34, and MUC4. The patients were 6, 43, 45, and 73 years old (mean 41.8 years) and included three males and one female. The tumors were located in the posterior cervical spine, facial skin, mandible, and larynx. The tumors were treated with surgical excision, and all four had histologic features typical for LGFMS including alternating myxoid and fibrous areas with prominent curvilinear vasculature. All tumors were MUC4 positive (100%), 2/4 (50%) were p63 positive, 1/4 (25%) showed focal EMA positivity; all 4 were negative for pancytokeratin, p40, S100 protein, β-catenin, actin, and CD34. LGFMS is a low grade sarcoma that rarely develops in the head and neck. Due to its rarity, a pathologist may not consider LGFMS in the differential diagnosis of spindle cell neoplasms within the head and neck. Immunohistochemical staining is helpful, but stains should be selected carefully to avoid misdiagnosis.

Section: Discussionmentioning

confidence: 92%

Low-Grade Fibromyxoid Sarcoma of the Head and Neck: A Clinicopathologic Series and Review of the Literature

Cowan

Thompson

Leon

et al. 2015

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“…MITF is also not specific for melanoma and has been expressed in some sarcomas, neurogenic tumors and other entities. SOX10 is a newer marker, showing a high sensitivity for MM (88-100%) [22,23]. However, SOX10 specificity in the wide range of neoplasms in the sinonasal region has only partially been studied, showing expression in non-melanomas, such as 8% of alveolar rhabdomyosarcomas [23].…”

Section: Melanocytic Markersmentioning

confidence: 99%

“…SOX10 is a newer marker, showing a high sensitivity for MM (88-100%) [22,23]. However, SOX10 specificity in the wide range of neoplasms in the sinonasal region has only partially been studied, showing expression in non-melanomas, such as 8% of alveolar rhabdomyosarcomas [23]. Thus, a panel of markers is employed to maximize both sensitivity and specificity in making a diagnosis of MM.…”

Section: Melanocytic Markersmentioning

confidence: 99%

Update from the 4th Edition of the World Health Organization Classification of Head and Neck Tumours: Mucosal Melanomas

Williams

2017

“…PEComas are rare in the sinonasal tract and are typically keratin negative. Also, though they often express various myoid and melanocytic markers, they are usually S100 and SOX-10 negative [18,19].…”

Section: Metastatic Clear Cell Lesionsmentioning

confidence: 99%

Sinonasal Renal Cell-Like Carcinoma: Case Report and Review of the Literature

Kubik

Barasch

Choby

et al. 2016

recent WHO classification of primary sinonasal malignancies contains 44 distinct histopathologic entities [3]. Understandably, these cancers present a formidable challenge for both the surgeon and pathologist.In this article we present an unusual case of clear cell neoplasia of the paranasal sinuses found to be consistent with sinonasal renal cell-like adenocarcinoma (SNRCLA). The finding of clear cell neoplasia in a sinonasal specimen is rare but associated with a surprisingly wide differential diagnosis. When evaluating the differential diagnosis for a clear cell neoplasm of the sinonasal tract, an underlying renal cell carcinoma (RCC) must first be ruled out. RCC is the most common metastatic lesion to the nasal cavity and paranasal sinuses [4] and approximately 6-15% of patients with RCC will have metastatic deposits in the head and neck [5,6]. In addition to metastatic RCC, other diagnostic considerations for clear cell neoplasia in the paranasal sinuses include a clear cell variant of squamous cell carcinoma, melanoma, primary salivary clear cell carcinoma, metastatic clear cell thyroid carcinoma, clear cell predominant mucoepidermoid carcinoma, and SNRCLA. Case ReportAn 80 year old female presented to our otolaryngology clinic with an asymptomatic right sinonasal mass. She reported a history of left sided acinic cell carcinoma treated at an outside institution with endonasal resection and adjuvant radiotherapy approximately eight years prior. Diagnostic nasal endoscopy revealed a large friable soft tissue mass arising from the lateral nasal wall near the axilla of the right middle turbinate. Imaging revealed a 3 cm tumor centered at the right nasolacrimal sac with extension superiorly to involve the nasal septum and dura of the anterior Abstract The differential diagnosis for clear cell neoplasms of the sinonasal tract is wide but critical to understand. In this paper, we describe a case of renal cell-like adenocarcinoma (SNRCLA) presenting as a paranasal sinus mass with clear cell morphology. The relevant literature is reviewed and the workup for clear cell neoplasms described. SNRCLA is a rarely encountered low grade glandular malignancy of the paranasal sinuses. Despite its morphologic mimicry of renal cell carcinoma, SNRCLA demonstrates a seromucinous phenotype and is associated with a favorable prognosis and low recurrence rates after surgical resection.