University of Miami Miller School of Medicine, Department of Pathology and Laboratory Medicine, Miami, FL, USA.
Departments of Pathology, Virginia Commonwealth University School of Medicine, Richmond, VA, USA.
Hum Pathol. 2024 Aug;150:42-50. doi: 10.1016/j.humpath.2024.06.005. Epub 2024 Jun 12.
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 to 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.
舌部肿瘤较为常见,绝大多数表现为上皮表型。尽管少见,但在该解剖部位也会出现多种独特的间叶性肿瘤。为了提高对这些病变的认识,我们回顾了我们在舌部间叶性肿瘤方面的经验,并描述了其临床病理特征。从 2005 年到 2021 年,我们查阅了病理档案和一位作者的会诊档案,以获取所有舌部间叶性肿瘤的信息。我们复习了组织学切片和辅助研究,并从可用的病历中获取了临床数据。共确定了 93 例病例,构成了研究队列-据我们所知,这是最大的一组舌部间叶性肿瘤。48 例为女性,45 例为男性,平均年龄为 51 岁(范围:1-94 岁)。肿瘤包括 43 例(46.2%)血管瘤、14 例(15%)颗粒细胞瘤、8 例(9%)脂肪瘤、4 例(4.3%)神经鞘瘤、4 例(4.3%)孤立性纤维性肿瘤-根据风险分层标准,所有这些肿瘤均为低度进展风险,2 例(2.2%)淋巴管瘤、3 例(3.2%)卡波西肉瘤、2 例(2.2%)软骨瘤、2 例(2.2%)肌纤维瘤、1 例(1.1%)孤立性局限性神经瘤、1 例(1.1%)周围神经鞘瘤、1 例(1.1%)神经纤维瘤、1 例(1.1%)间叶性软骨黏液样肿瘤、1 例(1.1%)伴有 NOTCH2 重排和 TLL2 突变的非典型血管球瘤、1 例(1.1%)梭形细胞横纹肌肉瘤、1 例(1.1%)多形性成纤维细胞肉瘤、1 例(1.1%)恶性横纹肌样瘤、1 例(1.1%)平滑肌肉瘤、1 例(1.1%)血管肉瘤和 1 例(1.1%)腺泡状软组织肉瘤。大多数患者接受了手术切除,1 例(血管瘤)患者接受了栓塞治疗。在随访中,接受梭形细胞横纹肌肉瘤治疗的患者在手术部位出现术后麻木,随访 17 个月后无疾病。接受平滑肌肉瘤治疗的患者拒绝辅助放疗,在 22 个月时出现肺转移。接受腺泡状软组织肉瘤治疗的患者在诊断时发生肺转移,并接受了辅助化疗。其余患者无局部或远处复发。舌部间叶性肿瘤通常为良性,表现为内皮、脂肪细胞或施万氏分化。主要治疗方法是手术切除,切除范围取决于肿瘤类型。高级别肉瘤保留辅助治疗。