In this review, we highlight the complexities of the natural history, biology, and clinical management of three intermediate connective tissue tumors: desmoid tumor (DT) or aggressive fibromatosis, tenosynovial giant cell tumor (TGCT) or diffuse-type pigmented villonodular synovitis (dtPVNS), and giant cell tumor of bone (GCTB). Intermediate histologies include tumors of both soft tissue and bone origin and are locally aggressive and rarely metastatic. Some common aspects to these tumors are that they can be locally infiltrative and/or impinge on critical organs, which leads to disfigurement, pain, loss of function and mobility, neurovascular compromise, and occasionally life-threatening consequences, such as mesenteric, bowel, ureteral, and/or bladder obstruction. DT, PVNS, and GCTB have few and recurrent molecular aberrations but, paradoxically, can have variable natural histories. A multidisciplinary approach is recommended for optimal management. In DT and PVNS, a course of observation may be appropriate, and any intervention should be guided by symptoms and/or disease progression. A surgical approach should take into consideration the infiltrative nature, difficulty in obtaining wide margins, high recurrence rates, acute and chronic surgical morbidities, and impact on quality of life. There are similar concerns with radiation, which especially relate to optimal field and transformation to high-grade radiation-associated sarcomas. Systemic therapies must be considered carefully in light of acute and chronic toxicities. Although standard and novel therapies are promising, many unanswered questions, such as duration of therapy and optimal end points to evaluate efficacy of drugs in clinical practice and trials, exist. Predictive biomarkers and novel clinical trial end points, such as volumetric measurement, magnetic resonance imaging T2 weighted mapping, nuclear imaging, and patient-reported outcomes, are in development and will require validation in prospective trials.

中文翻译：

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局部侵袭性结缔组织肿瘤

在这篇综述中，我们强调了三种中间结缔组织肿瘤的自然病程、生物学和临床管理的复杂性：硬纤维瘤 (DT) 或侵袭性纤维瘤病、腱鞘巨细胞瘤 (TGCT) 或弥漫型色素沉着绒毛结节性滑膜炎 (dtPVNS) ）和骨巨细胞瘤（GCTB）。中间组织学包括软组织和骨来源的肿瘤，并且具有局部侵袭性且很少转移。这些肿瘤的一些常见方面是它们可以局部浸润和/或侵犯关键器官，导致毁容、疼痛、功能和活动能力丧失、神经血管受损，偶尔还会危及生命，如肠系膜、肠道、输尿管和/或膀胱梗阻。DT、PVNS 和 GCTB 很少有反复发生的分子畸变，但矛盾的是，可以有可变的自然历史。建议采用多学科方法进行优化管理。在 DT 和 PVNS 中，观察过程可能是合适的，任何干预都应以症状和/或疾病进展为指导。手术方法应考虑浸润性、切缘困难、复发率高、急性和慢性手术并发症以及对生活质量的影响。放疗也存在类似问题，尤其是与最佳视野和向高级别放疗相关肉瘤的转化有关。鉴于急性和慢性毒性，必须仔细考虑全身治疗。尽管标准和新颖的疗法很有希望，但许多悬而未决的问题，例如治疗持续时间和最佳终点，以在临床实践和试验中评估药物的疗效。预测性生物标志物和新的临床试验终点，如体积测量、磁共振成像 T2 加权映射、核成像和患者报告的结果，正在开发中，需要在前瞻性试验中进行验证。