Engle Joshua A, Dibb James T, Kundu Soumajit, Jakob John A
Department of Medicine, Summa Health, Akron, USA.
Department of Medical Oncology, Summa Health, Akron, USA.
Cureus. 2024 Sep 10;16(9):e69122. doi: 10.7759/cureus.69122. eCollection 2024 Sep.
Glomus tumors are typically benign, soft tissue neoplasms composed of thermoregulatory glomus bodies. The more common varieties, such as subungual, are treated surgically and typically have a very low mortality rate. Malignant glomus tumors are very rare, and their pathogenesis is poorly understood. As such, treatment options and prognosis are unclear. We present a 67-year-old female diagnosed with a chest wall glomus tumor with biopsy-proven metastases to her lungs. Her treatment course included neoadjuvant radiation therapy followed by immunotherapy with pembrolizumab. After completion of the initial radiation therapy, imaging showed disease regression. Interval imaging after seven months of immunotherapy showed the resolution of all lung nodules with no reported concerns for disease recurrence. Pembrolizumab was discontinued due to concerns for dermatologic and renal adverse events, and the patient continues to be monitored off therapy. The metastatic glomus tumor described in this case had several unique qualities, including its initial presentation on the chest wall as an aggressive lesion, as well as its spread to multiple locations in the lungs. Glomus tumors are not normally as aggressive as seen in this case, but the genetic profile with high tumor mutational burden allowed for guided treatment. Radiation is often used as neoadjuvant treatment in higher risk glomus tumors, but the addition of immunotherapy such as pembrolizumab represents a potential avenue to manage these patients when surgery is not an option. Malignant glomus tumors are exceptionally rare occurrences that, by nature of their rarity, require protocols or therapies that are not specifically designed for their treatment. The clinical course of these tumors is difficult to predict as most cases of metastatic spread have few examples from which to draw conclusions. This case provided encouraging results for treatment with radiation and, potentially, immunotherapy. Each instance of a malignant glomus tumor and its genetic profile should be closely examined and documented so that sufficient data can be accumulated to guide treatment for this rare cancer.
血管球瘤通常是由温度调节性血管球组成的良性软组织肿瘤。较常见的类型,如甲下血管球瘤,通过手术治疗,死亡率通常很低。恶性血管球瘤非常罕见,其发病机制尚不清楚。因此,治疗选择和预后也不明确。我们报告一例67岁女性,诊断为胸壁血管球瘤,活检证实已转移至肺部。她的治疗过程包括新辅助放疗,随后使用帕博利珠单抗进行免疫治疗。初始放疗完成后,影像学检查显示疾病有所缓解。免疫治疗7个月后的间隔期影像学检查显示所有肺结节均已消失,未报告疾病复发问题。由于担心出现皮肤和肾脏不良事件,停用了帕博利珠单抗,患者在停止治疗后仍在接受监测。本病例中描述的转移性血管球瘤有几个独特之处,包括最初在胸壁表现为侵袭性病变,以及扩散至肺部多个部位。血管球瘤通常不像本例中那样具有侵袭性,但高肿瘤突变负荷的基因特征有助于指导治疗。在高风险血管球瘤中,放疗常被用作新辅助治疗,但在无法进行手术时,添加帕博利珠单抗等免疫治疗可能是治疗这些患者的一种途径。恶性血管球瘤极为罕见,因其罕见性,需要采用并非专门为其治疗设计的方案或疗法。这些肿瘤的临床病程难以预测,因为大多数转移扩散病例几乎没有可供参考的实例。该病例为放疗以及可能的免疫治疗提供了令人鼓舞的结果。每一例恶性血管球瘤及其基因特征都应仔细检查并记录,以便积累足够的数据来指导这种罕见癌症的治疗。