Shields Carol L, Silva Ana Maria Velasco, Laiton Andrea, Kalafatis Nicholas E, Schiller Emily, Lally Sara E, Grant-Kels Jane M, Shields Jerry A
Ocular Oncology Service, Wills Eye Hospital, Thomas Jefferson University, Philadelphia, Pennsylvania, USA.
Ocular Oncology Service, Wills Eye Hospital, Thomas Jefferson University, Philadelphia, Pennsylvania, USA.
Clin Dermatol. 2024 Jan-Feb;42(1):46-55. doi: 10.1016/j.clindermatol.2023.10.010. Epub 2023 Oct 18.
Conjunctival melanoma is quite rare, estimated at approximately 0.5 incidence per 1 million persons per year. This malignancy arises from a pre-existing nevus (7%), primary acquired melanosis (74%), or de novo without pre-existing condition (19%) and develops most often in patients with Fitzpatrick skin types I (23%) and II (62%). At initial presentation, the tumor size is approximately 13 mm in cross-sectional diameter and has 3-mm thickness, involving the bulbar (97%), forniceal (30%), tarsal (28%), or caruncular (11%) regions, often with corneal (54%) and rarely with orbital (4%) involvement. According to the eighth edition of the American Joint Committee on Cancer (AJCC), the tumor is classified as T1 (63%), T2 (18%), T3 (20%), and T4 (0%). Outcomes depend on several factors including patient age, AJCC classification, orbital invasion, and type of initial surgery, whereas tumor origin and Fitzpatrick skin type do not appear to impact outcomes. Older patients (≥70 years of age) demonstrate larger tumors, greater recurrence, and greater vision loss. Analysis of 425 patients by AJCC classification (T1 versus T2 versus T3) revealed increasing T category with greater lymph node metastasis (3% versus 13% versus 25%; P < .001), tumor-related systemic metastasis (13% versus 45% versus 40%; P < .001), and tumor-related death (8% versus 22% versus 37%; P < .001). Data of patients with orbital invasion revealed significantly greater 10-year rates of exenteration (P < .001), distant metastasis (P = .0005), and death (P = .001). Studies have demonstrated biomarkers related to conjunctival melanoma include mutations in BRAF, NRAS, ATRX, and NF1. Future therapies might be directed against these mutations or with small-molecule inhibitors and/or immunotherapy. In summary, conjunctival melanoma is a rare but ominous malignancy, imparting moderate risk for lymph node and systemic metastasis as well as death, depending on tumor features and classification. The first surgery is highly important in prevention of tumor seeding, recurrence, and metastasis.
结膜黑色素瘤相当罕见，估计每年每100万人中约有0.5例发病。这种恶性肿瘤起源于先前存在的痣（7%）、原发性后天性黑变病（74%）或无先前病变的新发情况（19%），最常发生在皮肤菲茨帕特里克分型为I型（23%）和II型（62%）的患者中。初诊时，肿瘤的横截面直径约为13毫米，厚度为3毫米，累及球结膜（97%）、穹窿结膜（30%）、睑结膜（28%）或泪阜（11%）区域，常累及角膜（54%），很少累及眼眶（4%）。根据美国癌症联合委员会（AJCC）第八版，肿瘤分为T1（63%）、T2（18%）、T3（20%）和T4（0%）。预后取决于几个因素，包括患者年龄、AJCC分类、眼眶侵犯和初次手术类型，而肿瘤起源和菲茨帕特里克皮肤类型似乎不影响预后。老年患者（≥70岁）的肿瘤更大，复发率更高，视力丧失更严重。根据AJCC分类（T1与T2与T3）对425例患者进行分析，结果显示T分类越高，淋巴结转移率越高（3%对13%对25%；P <.001）、肿瘤相关的全身转移率越高（13%对45%对40%；P <.001）和肿瘤相关死亡率越高（8%对22%对37%；P <.001）。眼眶侵犯患者的数据显示，眼球摘除术的10年发生率（P <.001）、远处转移率（P =.0005）和死亡率（P =.001）显著更高。研究表明，与结膜黑色素瘤相关的生物标志物包括BRAF、NRAS、ATRX和NF1的突变。未来的治疗可能针对这些突变，或使用小分子抑制剂和/或免疫疗法。总之，结膜黑色素瘤是一种罕见但凶险的恶性肿瘤，根据肿瘤特征和分类，发生淋巴结和全身转移以及死亡的风险中等。首次手术对于预防肿瘤种植、复发和转移非常重要。
