-Author name in bold denotes the presenting author
-Asterisk * with author name denotes a Non-ASH member
Clinically Relevant Abstract denotes an abstract that is clinically relevant.

PhD Trainee denotes that this is a recommended PHD Trainee Session.

Ticketed Session denotes that this is a ticketed session.

3026 Clinical Features and Outcomes of Localized Ocular Adnexal Mucosa-Associated Lymphoid Tissue Lymphoma: A Dual-Center Retrospective Observational Study with a Long-Term Follow-up

Program: Oral and Poster Abstracts
Session: 623. Mantle Cell, Follicular, Waldenstrom’s, and Other Indolent B Cell Lymphomas: Clinical and Epidemiological: Poster II
Hematology Disease Topics & Pathways:
Lymphomas, Diseases, Indolent lymphoma, Lymphoid Malignancies
Sunday, December 8, 2024, 6:00 PM-8:00 PM

Kikuaki Yoshida1*, Noriko Iwaki2*, Nobuhiko Yamauchi1*, Yusuke Koba1*, Yuko Shirouchi1*, Yuko Ishihara1*, Yuko Mishima1*, Shinichi Makita2*, Suguru Fukuhara2*, Wataru Munakata, MD, PhD2*, Norihito Inoue3,4*, Naoko Tsuyama3,4*, Senzo Taguchi, MD, PhD5*, Takashi Terauchi6*, Hideki Tsuji7*, Akiko Miyagi-Maeshima8*, Shigenobu Suzuki9*, Hiroshi Igaki10*, Kengo Takeuchi3,4,11*, Koji Izutsu, MD, PhD12 and Dai Maruyama, MD, PhD1

1Department of Hematology Oncology, Cancer Institute Hospital, Japanese Foundation for Cancer Research, Tokyo, Japan
2Department of Hematology, National Cancer Center Hospital, Tokyo, Japan
3Division of Pathology, Cancer Institute, Japanese Foundation for Cancer Research, Tokyo, Japan
4Clinical Pathology Center, Cancer Institute Hospital, Japanese Foundation for Cancer Research, Tokyo, Japan
5Department of Radiation Oncology, Cancer Institute Hospital, Japanese Foundation for Cancer Research, Tokyo, Japan
6Department of Nuclear Medicine, Cancer Institute Hospital, Japanese Foundation For Cancer Research, Tokyo, Japan
7Department of Ophthalmology, Cancer Institute Hospital, Japanese Foundation for Cancer Research, Tokyo, Japan
8Department of Diagnostic Pathology, National Cancer Center Hospital, Tokyo, Japan
9Department of Ophthalmologic Oncology, National Cancer Center Hospital, Tokyo, Japan
10Department of Radiation Oncology, National Cancer Center Hospital, Tokyo, Japan
11Pathology Project for Molecular Targets, Cancer Institute, Japanese Foundation for Cancer Research, Tokyo, Japan
12National Cancer Center Hospital, Tokyo, Japan

Background: Since ocular adnexal mucosa-associated lymphoid tissue lymphoma (OAML) is rare, several clinical questions remain unclear, including the optimal treatment strategy, particularly the validity of watchful waiting (WW), the incidence of systemic progression, histological transformation, and survival outcomes. Therefore, the present study investigated the clinical features and outcomes of patients (pts) with localized OAML.

Methods: We retrospectively analyzed consecutive pts with histopathologically diagnosed OAML in the Cancer Institute Hospital between 2000 and 2022 and the National Cancer Center Hospital between 1997 and 2022. WW was defined as observations without treatment after the initial diagnosis for more than 6 months, while treatment failure was defined as the commencement of second-line treatment, including systemic chemotherapy or radiation therapy (RT). This study was approved by the Institutional Review Board of each hospital.

Results: In total, 283 pts were identified as subjects. Median age was 62 years (range, 14–92), 134 pts were males, and 149 pts were females. The most frequently involved site was the conjunctiva (148 pts). RT was the most common initial treatment (237 pts, 84%), followed by WW (40 pts, 14%), immunochemotherapy (5 pts, 2%), and additional surgical resection (1 pt). WW pts were more likely to have had their tumors completely resected at the diagnosis. RT doses of 30-40 Gy were used for 149 pts, 16-24 Gy for 70, and 4-8 Gy for 18, with the selection of lower RT doses being more common in recent years. With a median follow-up of 7.9 years (range, 0.3-23.7), the cumulative incidence of treatment failure using death as a competing risk was 11.5% (95% CI, 7.7-16.1) at 5 years and 17.7% (95%CI, 12.5-23.6) at 10 years. Pts who initially received RT had a higher likelihood of not receiving second-line treatment in the first 5 years than those on WW (HR, 0.33; 95%CI, 0.15-0.71). Intra- and extra-ocular adnexal progression was observed in 28 (10%) and 21 pts (7%), respectively. The cumulative incidence of extra-ocular adnexal progression was 5.5% at 5 years and 10.7% at 10 years. Extra-ocular adnexal progression sites were the lymph nodes (12 pts), extranodal lesions (6 pts), and both (3 pts). Histological transformation was observed in 6 pts with a cumulative incidence of 1.4% at 5 years and 3.2% at 10 years, and all progressed to the lymph nodes. Progression-free survival (PFS) and overall survival (OS) in all 283 pts were 81.1 and 98.3% at 5 years and 72.2 and 91.9% at 10 years, respectively. PFS was worse in WW pts than in RT pts (HR, 2.62; 95% CI, 1.40-4.88), while no significant difference was observed in OS (HR 2.85; 95% CI, 0.97-8.39). In the RT group, the complete response rate was 94%. Twenty-seven pts received second-line treatment, which included RT (13 pts, 11 with contralateral ocular adnexa and 2 with lymph nodes) and immunochemotherapy (13 pts, including 10 with rituximab monotherapy or the R-CHOP(-like) regimen). In the WW group, 9 pts received second-line treatment, which mainly included RT (7 pts). Seventeen pts died during the study period, and the causes of death were as follows: other malignancies (5 pts), transformed lymphoma (1 pt), pneumonia (1 pt), and unknown (10 pts).

Conclusion: The present results demonstrated that pts with localized OAML had favorable outcomes. Histological transformation and death associated with lymphoma were rare regardless of the initial treatment approach.

Disclosures: Yamauchi: Genmab, AbbVie, Takeda, Novartis, Chugai, Mundi, Daiichi-Sankyo, Kyowa-Kirin, Janssen: Honoraria; Ono, Roche, Genmab, Tanabe-Mitsubishi: Research Funding. Ishihara: Janssen Pharmaceutical K.K: Current Employment. Mishima: Bristol Myers Squibb: Research Funding; Eisai: Research Funding. Makita: Abbvie: Honoraria; Celgene/BMS: Honoraria; Chugai: Honoraria; Genmab: Honoraria; Gilead: Honoraria; Novartis: Honoraria; Takeda: Honoraria. Fukuhara: Genmab, Abbvie, Janssen, Chugai, Otsuka, Ono, Takeda, Astrazeneca, Amgen, Nippon shinyaku: Honoraria; Chugai, Loxo Oncology, Mitsubishi Tanabe Pharma, AbbVie: Research Funding; Genmab, Otsuka: Consultancy. Munakata: Mundipharma, Takeda Pharmaceutical, ONO PHARMACEUTICAL, Eisai, CHUGAI PHARMACEUTICAL, Bristol-Myers Squibb, AstraZeneca, NIPPON SHINYAKU, Gilead Sciences, Nippon Kayaku, MSD, Janssen Pharmaceutical, AbbVie GK, Amgen: Honoraria; CHUGAI PHARMACEUTICAL, Janssen Pharmaceutical, ONO PHARMACEUTICAL, Kyowa Kirin, Genmab, NIPPON SHINYAKU: Research Funding. Igaki: HIMEDIC: Honoraria; Itochu: Honoraria; ZAP Surgical Japan: Honoraria. Izutsu: Pfizer, Janssen, Gilead, Daiichi Sankyo: Honoraria, Research Funding; Beigene, Yakult, Otsuka: Consultancy, Research Funding; Incyte, Bayer, O Oncology, Regeneron: Research Funding; MSD, AstraZeneca, Genmab, Chugai, BMS, Kyowa Kirin, Novartis, AbbVie: Consultancy, Honoraria, Research Funding; AstraZeneca, Eli Lily, Astellas, Ono Pharmaceuticals, Eisai, Chugai, Janssen, Symbio, Bristol Myers Squibb, Daiichi Sankyo, Otsuka, Abbvie, Takeda, Eli Lilly, Genmab, Kyowa Kirin, MSD, Astellas, Pfizer, MeijiSeika Pharma, Novartis, Nihon Kayaku, Gilead,: Honoraria; MSD, AstraZeneca, Abbvie, Incyte, Bristol Myers Squibb, Novartis, Bayer, Pfizer, Janssen, Yakult, Kyowa Kirin, Daiichi Sankyo, Chugai, Beigene, Genmab, LOXO Oncology, Otsuka, Regeneron, Gilead: Research Funding; AstraZeneca, Zenyaku, Ono Pharmaceuticals, Mitsubishi Tanabe Pharma, Eisai, Chugai, Bristol Myers Squibb, Takeda, Otsuka, Abbvie, Zenyaku, Kyowa Kirin, MSD, Carna Biosciences, Novartis, Yakult, Nihon Shinyaku, Abe Pharma, Eisai,Beigene: Consultancy; Ono Pharma, Symbio, Takeda: Consultancy, Honoraria. Maruyama: Ono: Honoraria, Membership on an entity's Board of Directors or advisory committees, Research Funding; Janssen: Honoraria, Membership on an entity's Board of Directors or advisory committees, Research Funding; Eisai: Honoraria, Research Funding; Chugai: Honoraria, Membership on an entity's Board of Directors or advisory committees, Research Funding; Kyowa Kirin: Honoraria, Research Funding; MSD: Honoraria, Research Funding; Zenyaku: Honoraria, Research Funding; Sanofi: Honoraria, Membership on an entity's Board of Directors or advisory committees, Research Funding; Symbio: Honoraria, Research Funding; Takeda: Honoraria, Research Funding; AbbVie: Honoraria, Membership on an entity's Board of Directors or advisory committees, Research Funding; AstraZeneca: Honoraria, Membership on an entity's Board of Directors or advisory committees, Research Funding; BMS: Honoraria, Membership on an entity's Board of Directors or advisory committees, Research Funding; Genmab: Honoraria, Membership on an entity's Board of Directors or advisory committees, Research Funding; Novartis: Honoraria, Research Funding; Otsuka: Research Funding; Taiho: Research Funding; Pfizer: Membership on an entity's Board of Directors or advisory committees, Research Funding; Astellas: Research Funding; Nippon Shinyaku: Honoraria; Mundipharma: Honoraria.

*signifies non-member of ASH