腱鞘巨细胞瘤(手指或拇指上的肿块)
腱鞘巨细胞瘤是手指或拇指上常见的良性(非癌性)肿块。它是手部第二常见的肿块,仅次于腱鞘囊肿。它生长缓慢,不会扩散到身体其他部位,通常通过一个小手术切除,但有时可能会复发。本页解释了它的定义、诊断方法、切除过程以及复发频率。
它是什么
腱鞘巨细胞瘤起源于腱鞘或邻近关节(滑膜,即允许肌腱滑动的润滑组织)的衬里。名称中的“巨细胞”描述了其在显微镜下的外观,其中由多个细胞核组成的大型细胞簇与较小的细胞混合在一起。这并不表示其生物学行为:这是一种良性生长,局限于手部。医生有时将其称为局限性腱滑膜巨细胞瘤。
出现部位及感觉
它几乎总是出现在手指或拇指上,通常位于手掌面或侧面,靠近其中一个指关节。通常表现为:
- 质地坚韧且有弹性,而非柔软,且生长缓慢
- 通常无痛,但随着体积增大,可能会出现酸痛或卡顿感
- 实性,因此(与充满液体的腱鞘囊肿不同),用手电筒紧贴照射时不会透光
随着肿瘤增大,可能导致手指无法完全屈曲;偶尔会压迫细小神经并引起麻木感。极少数情况下,长期存在的肿瘤会在邻近骨骼表面压出光滑的凹陷,这在X线片上可见。
如何诊断
您的外科医生通常可以通过检查肿块来识别它。超声检查有助于确认其为实性而非腱鞘囊肿的液体。对于较大、较深或复发的肿块,MRI(磁共振成像)检查可显示其延伸范围,并具有较为特征性的表现(由于组织中含有含铁色素,在某些序列上呈暗色)。如果肿块似乎累及邻近骨骼,则需加拍X线片。当切除的肿块经显微镜下检查时,即可确诊。
治疗:切除肿块
常规治疗方法是进行一个小手术将其切除,称为边缘切除,通常在局部或全身麻醉下作为日间手术进行。通过一个小切口,外科医生会切除整个结节,以及任何较小的卫星结节和受累的腱鞘,并在邻近的神经、血管和肌腱周围小心操作。切除全部肿瘤,而不仅仅是明显的肿块,可以降低其复发的几率。没有任何口服药物或注射能使这种类型的肿块缩小。
复发频率
手术后,该肿块可能会复发。根据已发表的系列研究,大约 1/7 到 1/5 的患者会出现复发(即每 100 人中有 10 到 20 人)。当存在多个结节、肿瘤包裹肌腱或骨骼,或初次手术时有残留时,复发风险更高。大多数复发出现在 前两年内,因此外科医生会在此期间密切监测该区域,尽管在极少数情况下,肿块可能在多年后复发。如果复发,通常可以再次手术切除。
恢复
大多数人当天即可回家,并佩戴敷料,有时还会佩戴轻型夹板。早期轻柔活动手指有助于预防僵硬,如有需要,手部治疗师可提供指导。缝线通常在术后约两周拆除。在舒适度和外科医生指导的范围内,您可在几天内开始用手进行轻度活动,并在数周内逐步恢复完全使用。保护疤痕免受阳光照射,并在愈合后对其进行按摩,有助于疤痕恢复。
更深入探讨
本节内容更为深入,面向学生层次。理解或治疗肿块并非必需,但其解释了驱动该肿瘤的因素以及为何有时会复发。
驱动因素
腱鞘巨细胞瘤是称为腱鞘巨细胞肿瘤的一类肿瘤中的局部形式。其背后的驱动因素是CSF1(集落刺激因子1)这一信号蛋白的过度产生,通常由少数肿瘤细胞中染色体间一小段遗传物质的交换所触发。这些细胞释放CSF1,从而从血液中吸引大量免疫细胞(巨噬细胞)。实际上,大部分肿块由这些被招募的细胞构成,而非原始的异常细胞,这是一种不寻常的肿瘤自我构建方式。
显微镜下及影像学表现
在显微镜下,该肿块由较小的单核细胞、充满泡沫状脂肪的巨噬细胞以及赋予其名称的多核巨细胞混合组成。肿块内还含有含铁血黄素,这是组织内微小陈旧性出血后遗留的富含铁的色素。正是由于这种铁质,该肿块在某些MRI序列上常呈现暗色,这一特征有助于在手术前将其与充满液体的腱鞘囊肿区分开来。
局限性病变与弥漫性病变,以及为何会复发
常见的指和拇指肿块属于局限性类型,边界清晰,通常可被完整切除。较少见的弥漫性类型在关节滑膜内更广泛地扩散(在大关节中,同一病理过程称为色素沉着绒毛结节性滑膜炎)。弥漫性类型更难彻底切除,且复发率更高。即使是局限性类型,若残留微小的卫星结节,也可能复发。因此,外科医生会切除整个肿块及其周围受累的腱鞘,而不仅仅是切除可见部分。
片剂的适用场景
对于无法通过手术切除或持续复发的罕见弥漫性肿瘤,阻断CSF1信号(CSF1受体抑制剂)的药物可使其缩小。此类药物仅用于难治性病例,不用于可通过手术直接处理的普通、易切除的手指肿块。
何时就诊
如果您手指或拇指上出现一个不消退、缓慢增大、限制手指活动或引起麻木或刺痛感的硬块,请就诊于全科医生或手外科医生。术后,如果您在瘢痕内或附近发现新的肿块,请告知您的外科医生,因为这可能是肿瘤复发的迹象,值得尽早检查。
Evidence & references
Overview
- Giant cell tumour of tendon sheath is a common benign tumour of the hand [2].
- Reports of giant cell tumour of tendon sheath in the paediatric population are rare [2].
- Giant cell tumour of tendon sheath can be locally recurrent after excision [2].
- Imaging studies can be of little utility in distinguishing glomus tumors from other lesions like giant cell tumor of the tendon sheath [5].
- Vigilance for malignancy is encouraged for lesions such as giant cell tumors, as aggressive treatment such as wide excision or amputation may be necessary to prevent recurrence [1].
- Orthopaedic surgeons should be familiar with the spectrum of hand and wrist tumors, the work-up necessary to arrive at a precise diagnosis, and the treatment that will achieve the most favorable outcome [6].
Anatomy & Pathophysiology
- Giant cell tumors are classified as benign bony and soft tissue tumors of the hand [1].
- Giant cell tumors can exhibit aggressive behavior requiring wide excision or amputation to prevent recurrence [1].
- Giant cell tumors can recur at the third lumbar vertebra [22].
- If the vertebral body and posterior arch are affected in giant cell tumors, curettage is insufficient to prevent recurrence [22].
- Giant cell tumors can occur in the proximal phalanx [23].
- Giant cell tumors can recur in the second metacarpal [42].
- Primary parosteal osteosarcoma of the finger is a rare tumor with characteristic radiographic and microscopic appearance [36].
- Fibroma of tendon sheath (FTS) is an extremely rare condition in the hand of a 3-year-old child [39].
- Florid reactive periostitis presents as soft tissue swelling adjacent to hand bones with pseudomalignant or pseudoinflammatory features [45].
Classification
- Giant cell tumour of tendon sheath is a common benign tumour of the hand [2].
- Giant cell tumour of tendon sheath can be locally recurrent after excision [2].
- Reports of giant cell tumour of tendon sheath in the paediatric population are rare [2].
- Imaging studies can be of little utility in distinguishing glomus tumors from other lesions like giant cell tumor of the tendon sheath [5].
Clinical Presentation
- Giant cell tumour of tendon sheath is a common benign tumour of the hand [2].
- Reports of giant cell tumour of tendon sheath in the paediatric population are rare [2].
- Giant cell tumour of tendon sheath can be locally recurrent after excision [2].
- Imaging studies can be of little utility in distinguishing glomus tumors from other lesions like giant cell tumor of the tendon sheath [5].
- Prolonged and atypical swelling of soft tissue, even with a previous traumatic lesion, may indicate underlying malignancy, necessitating proper imaging before surgery [13].
- Primary bone and soft-tissue tumors that mimic common sports injuries are relatively rare but can be easily missed, leading to limb- and life-threatening consequences [20].
- Vigilance for malignancy is encouraged for lesions like giant cell tumors to prevent recurrence [1].
- Orthopaedic surgeons should be familiar with the spectrum of hand and wrist tumors, the work-up necessary to arrive at a precise diagnosis, and the treatment that will achieve the most favorable outcome [6].
- Soft tissue sarcomas of the hand may have better survival than those at other sites, but prognosis must be interpreted with caution due to the rarity of the condition [9].
- An unusual pathway for spread of squamous cell carcinoma from the thumb to the small finger is via the flexor tendon sheaths, which should be considered in the evaluation of patients with hand tumors [15].
- Local recurrence is seen in ≤20% of cases of giant cell tumor of bone [29].
- A second local intralesional procedure is typically sufficient in cases of giant cell tumor of bone detected early [29].
- Most recurrences of giant-cell tumor of bone can be expected within the first two years [12].
- Some patients with giant-cell tumor of bone remain at risk for recurrence for a much longer period, with recurrences occurring nineteen to thirty years after initial treatment [12].
- Two cases of soft-tissue recurrence of giant-cell tumor within a surgical scar indicate that tumor cells may be implanted in a surgical wound [3].
- There are subsets of patients with giant cell tumor of bone who are at higher risk of recurrence and should be clinically followed more closely [7].
- Malignant transformation of a giant cell tumor of bone while receiving denosumab treatment is a rare but important possibility [16].
Investigations
- Imaging studies have limited utility in distinguishing glomus tumors from other lesions such as giant cell tumor of the tendon sheath [5].
- Prolonged and atypical soft tissue swelling, even in the presence of a previous traumatic lesion, may indicate underlying malignancy and necessitates proper imaging before surgery [13].
- The use of CT and whole body bone scans may be beneficial when indicated, particularly after the occurrence of a second tumor focus [38].
Treatment
- Vigilance for malignancy is encouraged, and aggressive treatment such as wide excision or amputation may be necessary for certain lesions like giant cell tumors to prevent recurrence [1].
- Giant cell tumour of tendon sheath is a common benign tumour of the hand that can be locally recurrent after excision [2].
- Two cases of soft-tissue recurrence of giant-cell tumor within a surgical scar are reported, indicating that tumor cells may be implanted in a surgical wound [3].
- Despite its benign histology, giant cell tumor of bone is an aggressive tumor that demands meticulous attention to surgical detail and close postoperative surveillance for successful local tumor control and durable, joint-preserving function [4].
- Both curettage and resection/amputation are acceptable treatment options for the rare condition of giant cell tumour of bone in the hand, with a need to individualize treatment decisions based on the site and extent of disease to minimize treatment morbidity while maximizing disease control [8].
- The transplantation of a toe phalanx for a recurrent giant-cell tumor in a skeletally immature patient resulted in a satisfactory outcome with full hand function and no tumor recurrence forty-one years later [10].
- Intralesional excision remains a viable, and likely the standard, mode of treatment for most giant cell tumors of the distal radius unless there is extensive bone loss [11].
- Although most recurrences of giant-cell tumor of bone can be expected within the first two years, some patients remain at risk for a much longer period, with recurrences occurring nineteen to thirty years after initial treatment [12].
- Wide excision should be considered in the presence of extensive soft tissue involvement, and early treatment yields good results [14].
- The available studies suffer from selection bias and are inadequate to answer questions regarding the appropriate use, duration, and efficacy of denosumab in giant cell tumors of bone definitively [17].
- The effectiveness of treatment of a recurrence with either an intralesional or a wide excisional procedure does not appear to be diminished by initial curettage and cementing [18].
- Treatment is directed at controlling the lesion locally, with curettage and adjuvant therapy being the primary goal for most lesions to preserve the articular surface [19].
- Giant-cell tumor of bone was effectively treated with megavoltage radiation in patients in whom operative resection would have been difficult or was not feasible, with a ten-year lack of progression rate of 85 percent [21].
- Radiation therapy is a safe and effective treatment option for benign giant cell tumors of bone [27].
- Reconstruction after wide excision by nonvascularized fibular graft is a viable alternative for giant cell tumors of the lower end of radius though it is a challenging procedure and may be accompanied by major complications [31].
- This tumor should be treated with conservative but thorough excision [32].
- The authors recommend intralesional surgery with polymethylmethacrylate for the majority of primary GCTs [33].
Complications
- Giant cell tumour of tendon sheath is a common benign tumour of the hand that can be locally recurrent after excision [2].
- Reports of giant cell tumour of tendon sheath in the paediatric population are rare [2].
- Soft-tissue recurrence of giant-cell tumor within a surgical scar indicates that tumor cells may be implanted in a surgical wound [3].
- Giant cell tumor of bone is an aggressive tumor that demands meticulous attention to surgical detail and close postoperative surveillance for successful local tumor control and durable, joint-preserving function [4].
- There are subsets of patients with giant cell tumor of bone who are at higher risk of recurrence and should be clinically followed more closely [7].
- Both curettage and resection/amputation are acceptable treatment options for giant cell tumour of bone in the hand, with treatment decisions needing to be individualized based on the site and extent of disease to minimize treatment morbidity while maximizing disease control [8].
- The transplantation of a toe phalanx for a recurrent giant-cell tumor in a skeletally immature patient resulted in a satisfactory outcome with full hand function and no tumor recurrence forty-one years later [10].
- Although most recurrences of giant-cell tumor of bone can be expected within the first two years, some patients remain at risk for a much longer period, with recurrences occurring nineteen to thirty years after initial treatment [12].
- Prolonged and atypical swelling of soft tissue, even with a previous traumatic lesion, may indicate underlying malignancy, necessitating proper imaging before surgery [13].
- The available studies regarding treatment with denosumab suffer from selection bias and are inadequate to answer questions regarding the appropriate use, duration, and efficacy of denosumab in giant cell tumors of bone definitively [17].
Recovery
- Giant cell tumour of tendon sheath is a common benign tumour of the hand that can be locally recurrent after excision [2].
- Reports of giant cell tumour of tendon sheath in the paediatric population are rare [2].
- Two cases of soft-tissue recurrence of giant-cell tumor within a surgical scar indicate that tumor cells may be implanted in a surgical wound [3].
- Despite its benign histology, giant cell tumor of bone is an aggressive tumor that demands meticulous attention to surgical detail and close postoperative surveillance for successful local tumor control and durable, joint-preserving function [4].
- There are subsets of patients with giant cell tumor of bone who are at higher risk of recurrence and should be clinically followed more closely [7].
- Although most recurrences of giant-cell tumor of bone can be expected within the first two years, some patients remain at risk for a much longer period, with recurrences occurring nineteen to thirty years after initial treatment [12].
- The transplantation of a toe phalanx for a recurrent giant-cell tumor in a skeletally immature patient resulted in a satisfactory outcome with full hand function and no tumor recurrence forty-one years later [10].
- The effectiveness of treatment of a recurrence with either an intralesional or a wide excisional procedure does not appear to be diminished by initial curettage and cementing [18].
Key Evidence
- [L5] Vigilance for malignancy is encouraged, and aggressive treatment such as wide excision or amputation may be necessary for certain lesions like giant cell tumors to prevent recurrence. [1] (10.1016/j.jhsa.2010.08.015)
- [L4] Giant cell tumour of tendon sheath is a common benign tumour of the hand that can be locally recurrent after excision, and reports in the paediatric population are rare, with this case believed to be the youngest reported. [2] (10.1177/1753193412455792)
- [L4] Two cases of soft-tissue recurrence of giant-cell tumor within a surgical scar are reported, indicating that tumor cells may be implanted in a surgical wound. [3] (10.2106/00004623-196749020-00016)
- [L3] Despite its benign histology, giant cell tumor of bone is an aggressive tumor that demands meticulous attention to surgical detail and close postoperative surveillance for successful local tumor control and durable, joint-preserving function. [4] (10.1097/01.blo.0000180055.76969.08)
- [Case_report] Imaging studies can be of little utility in distinguishing glomus tumors from other lesions like giant cell tumor of the tendon sheath. [5] (10.1016/j.jhsa.2012.02.035)
- [L5] Orthopaedic surgeons should be familiar with the spectrum of these tumors, the work-up necessary to arrive at a precise diagnosis, and the treatment that will achieve the most favorable outcome. [6] (10.5435/00124635-200611000-00013)
- [L4] Our observations suggest there are subsets of patients with giant cell tumor of bone who are at higher risk of recurrence and should be clinically followed more closely. [7] (10.1007/s11999-011-2172-8)
- [L4] Both curettage and resection/amputation are acceptable treatment options for the rare condition of giant cell tumour of bone in the hand, with a need to individualize treatment decisions based on the site and extent of disease to minimize treatment morbidity while maximizing disease control. [8] (10.1177/17531934211007820)
- [L5] It notes that while soft tissue sarcomas of the hand may have better survival than those at other sites, prognosis must be interpreted with caution due to the rarity of the condition. [9] (10.1016/j.jhsa.2010.08.014)
- [Case_report] The transplantation of a toe phalanx for a recurrent giant-cell tumor in a skeletally immature patient resulted in a satisfactory outcome with full hand function and no tumor recurrence forty-one years later. [10] (10.2106/00004623-199412000-00015)
- [L3] Intralesional excision remains a viable, and likely the standard, mode of treatment for most giant cell tumors of the distal radius unless there is extensive bone loss. [11] (10.1007/s11999-014-4054-3)
- [L4] Although most recurrences of giant-cell tumor of bone can be expected within the first two years, some patients remain at risk for a much longer period, with recurrences occurring nineteen to thirty years after initial treatment. [12] (10.2106/00004623-199408000-00013)
- [L5] Prolonged and atypical swelling of soft tissue, even with a previous traumatic lesion, may indicate underlying malignancy, necessitating proper imaging before surgery. [13] (10.1016/j.csm.2013.03.008)
- [Case_report] Wide excision should be considered in the presence of extensive soft tissue involvement, and early treatment yields good results. [14] (10.1016/j.jhsa.2014.01.004)
- [L5] This unusual pathway should be considered in the evaluation of patients with hand tumors. [15] (10.1016/j.jhsa.2009.06.012)
- [L4] Malignant transformation of a giant cell tumor of bone while receiving denosumab treatment is a rare but important possibility that physicians should be aware of, as denosumab is increasingly used for this condition. [16] (10.1007/s11999-015-4249-2)
- [L5] The available studies suffer from selection bias and are inadequate to answer questions regarding the appropriate use, duration, and efficacy of denosumab in giant cell tumors of bone definitively. [17] (10.1097/corr.0000000000001217)
- [L3] Furthermore, the effectiveness of treatment of a recurrence with either an intralesional or a wide excisional procedure does not appear to be diminished by initial curettage and cementing. [18] (10.2106/00004623-199412000-00009)
- [L4] Treatment is directed at controlling the lesion locally, with curettage and adjuvant therapy being the primary goal for most lesions to preserve the articular surface. [19] (10.1016/j.hcl.2004.03.016)
- [L5] Primary bone and soft-tissue tumors that mimic common sports injuries are relatively rare but can be easily missed, leading to limb- and life-threatening consequences. [20] (10.5435/jaaos-22-04-223)
- [L4] Giant-cell tumor of bone was effectively treated with megavoltage radiation in patients in whom operative resection would have been difficult or was not feasible, with a ten-year lack of progression rate of 85 percent. [21] (10.2106/00004623-199911000-00008)
- [L5] If the vertebral body and the posterior arch are affected, curettage of the lesion is insufficient to prevent tumor recurrence. [22] (10.1016/j.otsr.2010.05.009)
- [L4] The application of a temporary dorsal plaster backslab to unstable distal radius fractures causes insignificant further displacement. [23] (10.1177/1753193408097859)
- [L4] The authors conclude that radiation therapy is a safe and effective treatment option for benign giant cell tumors of bone. [27] (10.1097/01.blo.0000069890.31220.b4)
- [L5] Local recurrence is seen in ≤20% of cases, and a second local intralesional procedure is typically sufficient in cases that are detected early. [29] (10.5435/jaaos-21-02-118)
- [L4] Reconstruction after wide excision by nonvascularized fibular graft is a viable alternative for giant cell tumors of the lower end of radius though it is a challenging procedure and may be accompanied by major complications. [31] (10.1007/s00402-010-1059-6)
- [L4] This tumor should be treated with conservative but thorough excision. [32] (10.1097/01.blo.0000129555.37075.74)
- [L3] The authors recommend intralesional surgery with polymethylmethacrylate for the majority of primary GCTs. [33] (10.1007/s11999-010-1501-7)
- [L4] Primary parosteal osteosarcoma of the finger is a rare tumor with characteristic radiographic and microscopic appearance. [36] (10.1016/j.jhsa.2019.08.006)
- [L4] The use of CT and whole body bone scans may prove beneficial when indicated, particularly after the occurrence of a second tumor focus. [38] (10.1097/01.blo.0000063784.32430.b0)
- [Case_report] We experienced an extremely rare case of FTS in the hand of a 3-year-old child. [39] (10.1186/s12891-020-03728-x)
- [L4] Reversed vascularised toe joint transfer should be considered as an option for reconstruction of joint defects in a single finger, especially in a young active patient, and has shown good short- to medium-term results. [42] (10.1177/1753193408089048)
- [L4] Florid reactive periostitis should be considered in cases of soft tissue swelling adjacent to hand bones showing pseudomalignant or pseudoinflammatory features. [45] (10.1016/j.jhsa.2013.08.115)
References
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DOI: 10.1007/s11999-011-2172-8 [8] Giant cell tumour of hand bones: outcomes of treatment. Journal of Hand Surgery (European Volume). 2021. DOI: 10.1177/17531934211007820 [9] Malignant and Metastatic Tumors of the Hand. The Journal of Hand Surgery. 2010. DOI: 10.1016/j.jhsa.2010.08.014 [10] Phalangeal transfer for recurrent giant-cell tumor of the phalanx of a finger in a nine-year-old child. A case report with forty-one-year follow-up.. The Journal of Bone & Joint Surgery. 1994. DOI: 10.2106/00004623-199412000-00015 [11] Is Intralesional Treatment of Giant Cell Tumor of the Distal Radius Comparable to Resection With Respect to Local Control and Functional Outcome?. Clinical Orthopaedics & Related Research. 2015. DOI: 10.1007/s11999-014-4054-3 [12] Late recurrence of giant-cell tumor of bone. A report of four cases.. The Journal of Bone & Joint Surgery. 1994. 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