Background: Lacrimal gland adenoid cystic carcinoma (LGACC) has historically been associated with a poor prognosis even with localized disease, with a survival of 56% at 5 years. In 1988, we treated the first patient with neoadjuvant intra-arterial cytoreductive chemotherapy (IACC). Since then, we have used this protocol as the standard approach. We aim to analyze the outcomes of patients with LGACC treated with the protocol and compare them to a population-based cohort to assess if IACC can improve survival.
Methods: We prospectively assessed all non-metastatic patients with LGACC treated with IACC at a single institution between 1988 and 2021. For a comparison group, we identified all non-metastatic patients with LGACC treated with excision from the Surveillance, Epidemiology, and End Results (SEER) registry. We calculated disease-specific survival using the Kaplan-Meier and Cox proportional-hazards modeling methods.
Results: Thirty-five non-metastatic patients with LGACC treated with IACC were identified at a single institution, and 64 patients with non-metastatic LGACC treated with excision were identified in the SEER database. The 5- and 10-year disease-specific survival rates for patients treated with IACC were 84% (95%CI 71-97) and 76% (95%CI 60-92), respectively. While the 5- and 10-year disease-specific survival rates for the population-based cohort were 72% (95%CI 62-82) and 46% (95%CI 32-60). The survival analysis favored IACC, with a 60% lower risk of death (HR: 0.4; 95%CI 0.2-0.9).
Conclusion: IACC improves disease-specific survival in comparison to a population-based cohort treated with excision. Additional patients treated with IACC at multiple institutions are required to provide further external validity.
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http://www.ncbi.nlm.nih.gov/pmc/articles/PMC10911902 | PMC |
http://dx.doi.org/10.1093/oncolo/oyad346 | DOI Listing |
Heliyon
July 2024
Department of Ophthalmology, Beijing Chaoyang Hospital, Capital Medical University, 100027, Beijing, China.
Introduction: 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.
Case Presentation: A 34-year-old man presented with progressive left eye proptosis for 4 months.
Oncologist
March 2024
Department of Medicine, Division of Oncology, Sylvester Comprehensive Cancer Center, University of Miami Miller School of Medicine, Miami, FL, USA.
Background: Lacrimal gland adenoid cystic carcinoma (LGACC) has historically been associated with a poor prognosis even with localized disease, with a survival of 56% at 5 years. In 1988, we treated the first patient with neoadjuvant intra-arterial cytoreductive chemotherapy (IACC). Since then, we have used this protocol as the standard approach.
View Article and Find Full Text PDFOphthalmic Plast Reconstr Surg
March 2024
Department of Ophthalmology, Bascom Palmer Eye Institute, University of Miami, Miami, Florida.
Purpose: Lacrimal gland adenoid cystic carcinoma (LGACC) is a rare orbital malignancy with devastating lethality. Neoadjuvant intra-arterial chemotherapy (IACC) has demonstrated cytoreductive effects on LGACC macroscopically, but limited studies have examined cellular and molecular determinants of the cytoreductive effect. This post hoc study assessed apoptotic marker expression on excised tumor specimens after neoadjuvant IACC and globe-sparing resection, emphasizing the examination of tumor margins.
View Article and Find Full Text PDFData Brief
August 2023
Dr. Nasser Ibrahim Al-Rashid Orbital Vision Research Center, Bascom Palmer Eye Institute, University of Miami Miller School of Medicine, Miami, FL, USA.
Adenoid cystic carcinoma of the lacrimal gland (LGACC) is a slow-growing but aggressive orbital malignancy. Due to the rarity of LGACC, it is poorly understood, which makes diagnosing, treating, and monitoring disease progression difficult. The aim is to understand the molecular drivers of LGACC further to identify potential targets for treating this cancer.
View Article and Find Full Text PDFAm J Ophthalmol
August 2022
From Dr. Nasser Ibrahim Al-Rashid Orbital Vision Research Center. Bascom Palmer Eye Institute, Department of Ophthalmology (D.T.T, B.C.T, W.J.F), University of Miami Miller School of Medicine, Miami, FL, USA.
Purpose: To report the therapeutic efficacy of integrating neoadjuvant chemotherapy with conventional bimodal therapies for lacrimal gland adenoid cystic carcinoma by providing an additional 8 years of follow-up data on the same cohort of patients whose cumulative 10-year disease-free survival outcomes were reported in 2013.
Design: Non-randomized, retrospective, interventional case series.
Methods: Nineteen consecutive patients treated with neoadjuvant intra-arterial cytoreductive chemotherapy (IACC), orbital exenteration, chemoradiotherapy, and adjuvant intravenous chemotherapy at a single institution were included.
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