Wenzel C T, Halperin E C, Fisher S R
Division of Otolaryngology-Head and Neck Surgery, Department of Surgery, Duke University Medical Center, Durham, N.C., USA.
Ear Nose Throat J. 2001 Feb;80(2):106, 109-12.
Retinoblastoma is a malignant tumor of the embryonic retina. Although it is rare, it is the most common primary eye tumor of childhood. Life expectancy following treatment is now excellent, but survivors who have heritable retinoblastoma face an increased risk of a second malignant head or neck neoplasm. A second neoplasm, which often occurs in the irradiated field of the original tumor, has become the most significant threat to the survival of these patients. We report the case of a young girl who was cured of her retinoblastoma only to later develop a second nonocular tumor that metastasized to the superficial parotid gland. She underwent a superficial parotidectomy and neck dissection, but the malignancy eventually recurred and required further surgery and radiation therapy. In this article, we discuss the etiology, incidence, sites of occurrence, and management options for a second malignant neoplasm in retinoblastoma survivors. The head and neck surgeon must be vigilant in the diagnosis and management of second neoplasms in this patient population because they often occur in irradiated fields; surgical management is important to patient survival.
视网膜母细胞瘤是胚胎视网膜的一种恶性肿瘤。尽管它很罕见，但却是儿童期最常见的原发性眼肿瘤。现在治疗后的预期寿命很好，但患有遗传性视网膜母细胞瘤的幸存者面临着发生第二种头颈部恶性肿瘤的风险增加。第二种肿瘤常发生在原发肿瘤的放疗区域，已成为这些患者生存的最重大威胁。我们报告了一名年轻女孩的病例，她的视网膜母细胞瘤已治愈，但后来发展为第二种非眼肿瘤并转移至腮腺浅叶。她接受了腮腺浅叶切除术和颈部清扫术，但恶性肿瘤最终复发，需要进一步手术和放疗。在本文中，我们讨论了视网膜母细胞瘤幸存者发生第二种恶性肿瘤的病因、发病率、发生部位及治疗选择。头颈外科医生必须对该患者群体中第二种肿瘤的诊断和治疗保持警惕，因为它们常发生在放疗区域；手术治疗对患者生存很重要。
