Szymanski Linda J, Sibug Saber Maria E, Kim Jonathan W, Go John L, Zada Gabriel, Rao Narsing, Hurth Kyle M
Department of Pathology, Keck School of Medicine of the University of Southern California, Los Angeles, CA, USA.
Department of Opthalmology, Keck School of Medicine of the University of Southern California, Los Angeles, CA, USA.
Ocul Oncol Pathol. 2017 Dec;4(1):33-37. doi: 10.1159/000477410. Epub 2017 Jul 6.
The aim of this study was to describe a 34-year-old male with hereditary bilateral retinoblastoma treated with radiotherapy as a child who developed 4 distinct tumors within the radiation field.
A 34-year-old male with bilateral retinoblastoma status postradiation therapy and recurrence requiring enucleation presented with left-eye visual acuity changes. Magnetic resonance imaging demonstrated a left orbital mass and a right parasellar complex lobulated mass (right sphenoid and right cavernous sinus). Two weeks later, the patient underwent excision of the orbital mass and biopsy of an upper-lid nodule. This was followed by craniotomy for removal of the complex mass.
Histology revealed 4 distinct tumors, including an undifferentiated pleomorphic sarcoma (left orbit), a radiation-induced meningioma (right sphenoid), a schwannoma (right cavernous sinus), and a basal-cell carcinoma (left lid).
Although occurrence of a second neoplasm is a well-known outcome following radiation treatment in patients with hereditary retinoblastoma, the diagnosis of 4 additional neoplasms is rare. Pleomorphic sarcoma, radiation-induced meningioma, and schwannoma are uncommon tumors and not well represented in the literature describing irradiated retinoblastoma patients. Secondary malignancies are a leading cause of early death in retinoblastoma survivors, and long-term follow-up is crucial for patient care.
本研究旨在描述一名34岁男性，他儿童时期患遗传性双侧视网膜母细胞瘤并接受了放射治疗，之后在放射野内出现了4种不同的肿瘤。
一名34岁男性，双侧视网膜母细胞瘤放疗后复发并需要摘除眼球，出现左眼视力变化。磁共振成像显示左眼眶有肿物，右侧鞍旁有分叶状肿物（右蝶骨和右海绵窦）。两周后，患者接受了眼眶肿物切除术和上睑结节活检。随后进行开颅手术以切除复杂肿物。
组织学检查发现4种不同的肿瘤，包括未分化多形性肉瘤（左眼眶）、放射诱导的脑膜瘤（右蝶骨）、神经鞘瘤（右海绵窦）和基底细胞癌（左眼睑）。
虽然在遗传性视网膜母细胞瘤患者接受放射治疗后出现第二种肿瘤是一个众所周知的结果，但额外诊断出4种肿瘤的情况很少见。多形性肉瘤、放射诱导的脑膜瘤和神经鞘瘤是不常见的肿瘤，在描述接受放射治疗的视网膜母细胞瘤患者的文献中没有很好的体现。继发性恶性肿瘤是视网膜母细胞瘤幸存者早期死亡的主要原因，长期随访对患者护理至关重要。
