PDF(Pubmed)
Abstract：
UNASSIGNED: Lacrimal gland adenoid cystic carcinoma (LGACC) is characterized by a high rate of recurrence, perineural invasion, and propensity for distant metastasis, resulting in poor prognosis. This case report aimed to highlight the diagnostic and therapeutic challenges of LGACC, underscore the importance of resectioning the tumor as completely as possible for the first time, adhere to postoperative adjuvant therapy, and provide detailed insights into its surgical and diagnostic management that may not be extensively covered in large case series and meta-analyses.
UNASSIGNED: A 34-year-old man presented with progressive left eye proptosis for 4 months. Initial evaluation and imaging led to a high suspicion of LGACC, which was confirmed after an eye-sparing excision of the left orbital tumor. The patient declined to undergo postoperative radiotherapy, which was recommended after the surgery. Thus, despite surgical intervention, the patient experienced tumor recurrence 3 months post-surgery, leading to orbital exenteration. Pathological examination confirmed the presence of poorly differentiated LGACC.This time the patient underwent postoperative radiotherapy, as recommended. However, despite local control, the patient developed an intracranial metastasis within a year.
UNASSIGNED: LGACC presents significant diagnostic and therapeutic challenges owing to its insidious onset, lack of specific symptoms, and high potential for recurrence and metastasis. Thus, this case emphasizes the need for early diagnosis, aggressive treatment, and adherence to postoperative adjuvant therapy to improve patient outcomes. Future research should focus on understanding the pathogenesis of LGACC and on developing standardized diagnostic and treatment protocols to enhance patient prognosis and survival.
UNASSIGNED: A 34-year-old man presented with progressive left eye proptosis for 4 months. Initial evaluation and imaging led to a high suspicion of LGACC, which was confirmed after an eye-sparing excision of the left orbital tumor. The patient declined to undergo postoperative radiotherapy, which was recommended after the surgery. Thus, despite surgical intervention, the patient experienced tumor recurrence 3 months post-surgery, leading to orbital exenteration. Pathological examination confirmed the presence of poorly differentiated LGACC.This time the patient underwent postoperative radiotherapy, as recommended. However, despite local control, the patient developed an intracranial metastasis within a year.
UNASSIGNED: LGACC presents significant diagnostic and therapeutic challenges owing to its insidious onset, lack of specific symptoms, and high potential for recurrence and metastasis. Thus, this case emphasizes the need for early diagnosis, aggressive treatment, and adherence to postoperative adjuvant therapy to improve patient outcomes. Future research should focus on understanding the pathogenesis of LGACC and on developing standardized diagnostic and treatment protocols to enhance patient prognosis and survival.
摘要：
■泪腺腺样囊性癌（LGACC）的特点是复发率高，神经周浸润,和远处转移的倾向，导致预后不良。本病例报告旨在强调LGACC的诊断和治疗挑战，强调第一次尽可能彻底切除肿瘤的重要性，坚持术后辅助治疗，并提供有关其手术和诊断管理的详细见解，这些见解可能未在大型病例系列和荟萃分析中广泛涵盖。
■一名34岁男子出现进行性左眼眼球突出4个月。初步评估和成像导致对LGACC的高度怀疑，这是在保留眼睛切除左眶肿瘤后证实的。病人拒绝接受术后放疗，这是手术后推荐的。因此，尽管有手术干预,患者在手术后3个月出现肿瘤复发,导致眼眶放血.病理检查证实存在低分化LGACC。这次病人接受了术后放疗,如推荐。然而,尽管有本地控制,患者在一年内出现颅内转移。
■LGACC由于其隐匿的发作而提出了重大的诊断和治疗挑战，缺乏特定的症状，复发和转移的可能性很高。因此，这个案例强调了早期诊断的必要性，积极治疗,并坚持术后辅助治疗以改善患者预后。未来的研究应该集中在了解LGACC的发病机制和发展标准化的诊断和治疗方案，以提高患者的预后和生存。
■一名34岁男子出现进行性左眼眼球突出4个月。初步评估和成像导致对LGACC的高度怀疑，这是在保留眼睛切除左眶肿瘤后证实的。病人拒绝接受术后放疗，这是手术后推荐的。因此，尽管有手术干预,患者在手术后3个月出现肿瘤复发,导致眼眶放血.病理检查证实存在低分化LGACC。这次病人接受了术后放疗,如推荐。然而,尽管有本地控制,患者在一年内出现颅内转移。
■LGACC由于其隐匿的发作而提出了重大的诊断和治疗挑战，缺乏特定的症状，复发和转移的可能性很高。因此，这个案例强调了早期诊断的必要性，积极治疗,并坚持术后辅助治疗以改善患者预后。未来的研究应该集中在了解LGACC的发病机制和发展标准化的诊断和治疗方案，以提高患者的预后和生存。
