Abstract：
Neoplasms of the tongue are relatively common, and the vast majority are epithelial in phenotype. Although uncommon, a diverse and distinctive array of mesenchymal neoplasms arises in this anatomic site. To increase our understanding of these lesions, we reviewed our experience of MNs of the tongue and described their clinicopathologic features. The pathology archives from 2005-2021 and the consultation files of one of the authors were queried for all MNs of the tongue. We reviewed the histologic slides and ancillary studies and obtained clinical data from the available medical records. Ninety-three cases were identified, and they form the study cohort - to our knowledge, this is the largest series of mesenchymal neoplasms of the tongue. Forty-eight patients were female, and forty-five were male, with a mean age of 51 years (range: 1-94 years). The tumors included 43 (46.2%) hemangiomas, 14 (15%) granular cell tumors, 8 (9%) lipomas, 4 (4.3%) schwannomas, 4 (4.3%) solitary fibrous tumors - all with low risk of progression based on risk stratification criteria, 2 (2.2%) lymphangiomas, 3 (3.2%) Kaposi sarcomas, 2 (2.2%) chondromas, 2 (2.2%) myofibromas, 1 (1.1%) solitary circumscribed neuroma, 1 (1.1%) perineurioma, 1 (1.1%) neurofibroma, 1 (1.1%) ectomesenchymal chondromyxoid tumor, 1 (1.1%) atypical glomus tumor with a NOTCH2 rearrangement and TLL2 mutation, 1 (1.1%) spindle cell rhabdomyosarcoma, 1 (1.1%) pleomorphic fibroblastic sarcoma, 1 (1.1%) malignant rhabdoid tumor, 1 (1.1%) leiomyosarcoma, 1 (1.1%) angiosarcoma, and 1 (1.1%) alveolar soft part sarcoma. Most of the patients underwent surgical excision, and 1 patient (with hemangioma) underwent embolization. On follow-up, the patient with spindle cell rhabdomyosarcoma developed postoperative numbness at the surgical site and was disease-free through 17 months of follow-up. The patient with leiomyosarcoma declined adjuvant radiation and developed metastasis to the lung at 22 months. The patient with alveolar soft part sarcoma had metastases to the lung at the time of diagnosis and received adjuvant chemotherapy. The remaining patients had no local or distant recurrence. MNs of the tongue are usually benign and characterized by either endothelial, adipocytic, or schwannian differentiation. The mainstay of treatment is surgical excision with the extent of excision determined by tumor type. Adjuvant therapy is reserved for high-grade sarcomas.
摘要：
舌肿瘤比较常见，绝大多数是上皮表型。虽然不常见，在这个解剖部位出现了多样化和独特的间充质肿瘤。为了增加我们对这些病变的了解，我们回顾了我们的经验，并描述了他们的临床病理特征。从2005-2021年的病理档案和其中一位作者的咨询文件中查询了所有舌头的MN。我们回顾了组织学切片和辅助研究，并从可用的医疗记录中获得了临床数据。确认了93例病例,他们组成了研究队列——据我们所知，这是最大的舌间充质肿瘤系列。48名患者为女性,45人是男性，平均年龄51岁（范围：1-94岁）。肿瘤包括43例（46.2%）血管瘤，14（15%）个颗粒细胞瘤，8（9%）脂肪瘤，4例（4.3%）神经鞘瘤，4例（4.3％）孤立性纤维瘤-根据风险分层标准，均具有较低的进展风险，2例（2.2%）淋巴管瘤，3（3.2%）卡波西肉瘤，2（2.2%）软骨瘤，2（2.2％）肌纤维瘤，1例（1.1％）孤立性局限性神经瘤，1（1.1%）神经鞘瘤，1（1.1%）神经纤维瘤，1（1.1％）外胚间充质软骨粘液样肿瘤，1例（1.1%）具有NOTCH2重排和TLL2突变的非典型血管球瘤，1（1.1％）梭形细胞横纹肌肉瘤，1（1.1%）多形性成纤维细胞肉瘤，1例（1.1%）恶性横纹肌样瘤，1（1.1%）平滑肌肉瘤，1（1.1%）血管肉瘤，1例(1.1%)肺泡软组织肉瘤。大多数患者接受了手术切除，1例（血管瘤）患者行栓塞治疗。关于后续行动，梭形细胞横纹肌肉瘤患者在手术部位出现术后麻木,随访17个月后无病.患有平滑肌肉瘤的患者在22个月时拒绝辅助放疗并发生肺部转移。患有肺泡软组织肉瘤的患者在诊断时已转移到肺，并接受了辅助化疗。其余患者无局部或远处复发。舌头的MNs通常是良性的，其特征是内皮，脂肪细胞,或schwannian分化。治疗的主要方法是手术切除，切除程度由肿瘤类型决定。辅助治疗保留用于高级别肉瘤。
