Myxoid Solitary Fibrous Tumor of the Nasal Vestibule: An Unusual Histological Subtype in the Head and Neck Region.

IF 0.9 4区 医学 Q4 PATHOLOGY
Aswini Prabakaran, Aanchal Kakkar, Hitesh Verma, Deepali Jain
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引用次数: 0

Abstract

Solitary fibrous tumors (SFTs) are mesenchymal neoplasms associated with the characteristic NAB2::STAT6 gene fusion. They frequently occur in extra-thoracic sites and are not uncommon in the head and neck (HN) region. Myxoid SFT is a rare morphological subtype of SFT, the features of which overlap with those of other myxoid-appearing tumors, making the diagnosis challenging. We describe the distinctive histopathological and immunohistochemical features of myxoid SFT that occurred in a 32-year-old man with a recurrent swelling in the nasal vestibule. Histological examination showed a nodular tumor composed of short spindle-shaped cells in an abundant myxoid stroma. Nuclei were ovoid, with minimal pleomorphism. Occasional intervening slender vascular channels were present; staghorn vasculature was absent. Tumor cells were diffusely immunopositive for STAT6, CD34, and BCL2, while S100, SOX10, EMA, ER, and CD10 were negative, confirming the diagnosis of myxoid SFT. Thus, myxoid SFTs are unusual in the HN, with potential for misdiagnosis. Due to their propensity for local recurrence if incompletely excised, a high index of suspicion is required to include them in differential diagnosis of myxoid mesenchymal neoplasms occurring at this location. STAT6 is a reliable immunohistochemical marker that aids in diagnosis, reducing the necessity for molecular testing.

鼻前庭肌样孤立性纤维瘤:头颈部一种不寻常的组织学亚型。
孤立性纤维瘤(SFTs)是一种间叶肿瘤,与特征性的 NAB2::STAT6 基因融合有关。它们经常发生在胸腔外部位,在头颈部(HN)也不少见。肌样SFT是SFT的一种罕见形态亚型,其特征与其他肌样肿瘤重叠,因此诊断具有挑战性。我们描述了一名鼻前庭反复肿胀的 32 岁男性所患肌样 SFT 的独特组织病理学和免疫组化特征。组织学检查显示,肿瘤呈结节状,由短纺锤形细胞组成,基质为丰富的类肌瘤。细胞核呈卵圆形,多形性极小。间隙中偶见细长的血管通道,但没有鹿角状血管。肿瘤细胞STAT6、CD34和BCL2呈弥漫性免疫阳性,而S100、SOX10、EMA、ER和CD10呈阴性,确诊为肌样SFT。因此,肌样SFT在HN中并不常见,有可能被误诊。由于其在切除不彻底的情况下有局部复发的倾向,因此需要高度怀疑才能将其纳入该部位发生的肌样间充质肿瘤的鉴别诊断中。STAT6 是一种可靠的免疫组化标记物,有助于诊断,减少了分子检测的必要性。
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来源期刊
CiteScore
2.10
自引率
0.00%
发文量
198
审稿时长
1 months
期刊介绍: International Journal of Surgical Pathology (IJSP) is a peer-reviewed journal published eight times a year, which offers original research and observations covering all major organ systems, timely reviews of new techniques and procedures, discussions of controversies in surgical pathology, case reports, and images in pathology. This journal is a member of the Committee on Publication Ethics (COPE).
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