Stage IIE Research Articles

7008 Challenges in the Management of Thyroid Lymphoma - Case Report

Abstract Disclosure: F. Toloza Bonilla: None. P. Veeraraghavan: None. R. Lakhotia: None. M. Roschewski: None. N. Nilubol: None. S. Gubbi: None. J. Klubo-Gwiezdzinska: None. Introduction: Primary thyroid lymphoma is an extremely rare thyroid malignancy accounting for less than 5% thyroid malignancies. The management depends on the histological subtype and tumor staging, typically involving chemotherapy alone or combined with radiotherapy. Although, surgical intervention is generally not recommended, given the potential surgical risks and the absence of additional benefits compared to chemoradiation therapy, it is reserved to obtain tissue diagnosis and to treat compressive symptoms. Clinical Case: A 66-year-old Caucasian female presented with dysphagia to solid food and acute neck swelling for a few weeks. History was negative for fever, weight loss or nocturnal diaphoresis. The patient had a history of primary hypertension, pre-diabetes, and Hashimoto’s hypothyroidism. A neck computed tomography (CT) scan showed a thyromegaly (8.3 x 5.8 cm) and bilateral cervical lymphadenopathy. Thyroid ultrasound showed sub centimeter nodules in the right mid-gland and a heterogeneous and irregular left thyroid lobe. TSH was normal (0.87 mcIU/mL [0.27-4.2]). She was treated for suspected subacute thyroiditis with prednisone for two weeks, showing initial clinical improvement, but symptoms recurred after steroids were stopped. Subsequently, a CT neck and fludeoxyglucose F18 (FDG) positron emission tomography (PET) scan demonstrated FDG avid thyroid enlargement with local adenopathy and rightward tracheal deviation. Lymph node, FNA, core biopsies and molecular testing were consistent with stage IIE MYC/BCL6 rearranged diffuse large B-cell lymphoma as she had no bone marrow or central nervous system (CNS) involvement. The patient’s treatments included four cycles of etoposide, prednisone, vincristine, cyclophosphamide, doxorubicin, and rituximab with ibrutinib added after the first cycle and intrathecal methotrexate with cycles 3-6, to reduce the risk of CNS involvement. A 6-month post-therapy follow-up showed complete response (CR) on imaging with symptoms resolution. Four months later, she experienced worsening symptoms, including dysphagia with solids and liquids, and hoarseness despite CR to therapy and stable CT imaging. Laryngoscopic evaluation noted left vocal cord paralysis and extrinsic compression of the tracheoesophageal groove. Patient opted for a left hemithyroidectomy, experiencing excellent symptom relief. Surgical pathology showed findings consistent with a necrotic tumor and residual areas of lymphocytic thyroiditis. No active DLBCL was appreciated. The patient remains in remission and continues to be symptoms-free 24 months post-thyroid lymphoma treatment. Conclusion: While thyroidectomy is not typically recommended as the initial treatment for primary thyroid lymphoma, it emerges as a potentially effective option to manage recurrent or persistent compression symptoms following the completion of medical therapy. Presentation: 6/3/2024

Long-Term Clinical Efficacy of Radiotherapy for Patients with Stage I-II Gastric Extranodal Marginal Zone B-Cell Lymphoma of Mucosa-Associated Lymphoid Tissue: A Retrospective Multi-Institutional Study.

To evaluate long-term treatment outcomes in patients with localized gastric mucosa-associated lymphoid tissue (MALT) lymphoma treated with radiation therapy (RT). A total of 229 patients who received RT in ten tertiary hospitals between 2010 and 2019 were included in this multi-center analysis. Response after RT was based on esophagogastroduodenoscopy after RT. Locoregional relapse-free survival (LRFS) and disease-free survival (DFS) were evaluated. After a median follow-up time of 93.2 months, 5-year LRFS, DFS, and OS rates were 92.8%, 90.4%, and 96.1%, respectively. LRFS, DFS, and OS rates at 10 years were 90.3%, 87.7%, and 92.8%, respectively. Of 229 patients, 228 (99.6%) patients achieved complete remission after RT. Five-year LRFS was significantly lower in patients with stage IIE than in those with stage IE (77.4% vs. 94.2%, p=0.047). Patients with age ≥ 60 had significantly lower LRFS than patients with age < 60 (89.3% vs. 95.1%, p=0.003). In the multivariate analysis, old age (≥ 60 years) was a prognostic factor for LRFS [hazard ratio (HR) of 3.72 and confidence interval (CI), 1.38-10.03; p=0.009). Grade 2 or higher gastritis was reported in 69 (30.1%) patients. Secondary malignancies including gastric adenocarcinoma, malignant lymphoma, lung cancer, breast cancer, and prostate cancer were observed in 11 (4.8%) patients after RT. Patients treated with RT for localized gastric MALT lymphoma showed favorable 10-year outcomes. Radiation therapy is an effective treatment without an increased risk of secondary cancer. The toxicity for radiotherapy to the stomach is not high.

Two-year Outcomes of Ultra-low-dose Radiotherapy in the Treatment of Ocular Adnexal B-cell Lymphomas.

This study focuses on the efficacy and 2-year outcomes of ultra-low-dose radiotherapy (RT) in treating primary and secondary ocular adnexal lymphoma (OAL). A retrospective analysis was conducted on patients with OAL between 2017 and 2022, treated with 4 Gy of RT. The primary and secondary outcomes assessed were response rate, progression-free survival, and lymphoma-related death. Twenty-one patients with primary and secondary OAL of diverse, presentations, subtypes, and stages were included. The orbital tumors had an average size of 17 × 16 × 16 mm. Of the 14 primary OAL cases, 3 (14%) had T1N0M0 disease, 8 (38%) T2N0M0, and 3 (14%) T3N0M0 (AJCC 8th edition staging); of the 7 secondary OALs, 4 (19%) were stage IE, 2 (10%) stage IIE, and 1 (5%) stage IIIE (Ann Arbor staging). Ultra-low-dose RT yielded a 95% complete response rate and 100% progression-free survival rates, both locally and systemically at 2 years. Mild dry eyes were reported in 14% of patients as a late treatment toxicity. Ultra-low-dose RT emerges as an effective and well-tolerated treatment approach for OAL. Our findings support the use of 4 Gy, showcasing high complete response rates (95%) and durable disease control without significant local relapses over an average follow up of 27 months. Our results align with earlier investigations, validating the curative potential of ultra-low-dose RT and reinforcing the concept of achieving favorable outcomes with minimal intervention. This approach may potentially alleviate the burden of long-term ocular side effects associated with higher radiation doses, enhancing the overall quality of life for OAL patients.

Clinical characteristics and outcome of early-stage diffuse large B cell lymphoma of female genital track: A retrospective study of the Hellenic cooperative lymphoma group.

Involvement of female genital track (FGT) by diffuse large B cell lymphoma (DLBCL) represents an extremely rare diagnosis. Especially data regarding early-stage disease (i.e., IE, IIE) is very limited. Importantly, previous studies showed controversial results about the risk of central nervous system (CNS) relapse in this entity. Herein, we describe one of the largest reported real-world series of patients with early-stage FGT DLBCL aiming to investigate the clinicopathological characteristics, response to therapy and survival outcomes in the era of immunochemotherapy. We analyzed 21 consecutive patients with biopsy proven DLBCL from uterus or ovary classified as stage IE or IIE out of 1905 newly diagnosed DLBCL patients (1.1%). Uterine and ovarian localization was observed in 14 and seven patients, respectively. Median age was 66years (range 33-96); 9/21 (43%) were <55years. Regarding Cell of Origin DLBCL subtype, Germinal Center B-cell subtype was found in seven patients, non-GCB in 10 and non-classified in 4 patients. Median follow-up was 57months and 5-year overall survival, lymphoma specific survival and Freedom from Progression were 78%, 89% and 90%, respectively. There was no correlation of patients' characteristics with survival parameters. Interestingly, none of the patients experienced CNS relapse. Our results indicate that localized FGT DLBCL exhibits a good prognosis and may not increase the risk for secondary CNS involvement.

A Case of Diffuse Large B-cell Lymphoma Transformed from Primary Thyroid MALT Lymphoma

Diffuse large B cell lymphoma (DLBCL) is main subtype of primary thyroid lymphoma and can be histologically transformed from a low-grade B-cell lymphoma. The characteristics and treatment guidelines of these particular DLBCL have not been fully established. The mainstay of treatment of primary thyroid DLBCL is multimodality treatment with chemotherapy and radiotherapy. Meanwhile, surgery can be considered only for diagnosis or alleviation of airway compressive symptoms.A 57-year-old female visited our outpatient clinic for recently enlarged long-held anterior neck mass. A thyroid mass compressing the airway and esophagus was identified on imaging, which was diagnosed as MALT lymphoma by excisional biopsy. After staging, the patient underwent total thyroidectomy with regional lymph node dissection for treatment of stage IIE MALT lymphoma and relieving airway compromise symptoms. The final diagnosis was DLBCL transformed from MALT Lymphoma, and chemotherapy was additionally performed. We report this rare experience with a review of literature.

Targeting BCMA in Plasmablastic Lymphoma: Two Cases of Therapeutic Response to Teclistamab

Plasmablastic lymphoma (PBL) is an aggressive subtype of B-cell Non-Hodgkin lymphoma (B-NHL) with poor outcomes. PBL occurs more commonly in HIV-positive or immunocompromised patients, but it can also occur in immunocompetent patients. Despite anthracycline-based induction therapy, PBL is associated with chemotherapy resistance and early disease relapse, yielding a median overall survival of 7 to 15 months (Castillo JJ, et al. Blood. 2015). The advent of novel therapies altered the therapeutic landscape for many common subtypes of B-NHL, but progress in PBL is lacking due to the rarity of this disease and the lack of effective therapeutic targets. While PBL does not express many common B-NHL markers (e.g. CD19 or CD20), it does highly express B-cell maturation antigen (BCMA). However, data regarding the efficacy of therapies targeting BCMA in PBL is limited. Herein, we report two cases of relapsed/refractory PBL treated with the BCMA-targeting bispecific antibody teclistamab. Patient 1 was a 33 year old male without significant comorbidities when he was diagnosed with Stage IIE PBL after presenting with a chest wall mass. He was treated with six cycles of dose-adjusted EPOCH-V, followed by radiation to the involved region and a BEAM autologous hematopoietic stem cell transplant. Post-transplant imaging showed him to be in a CR on day 30 PET, but he developed recurrent PBL on his day 100 PET exam with osseus lesions. He was then treated with daratumumab and ICE chemotherapy for four cycles, with an initial resolution of osseus lesions. However, he developed relapsed disease with perilymphatic lung nodules. His PBL was confirmed to express BCMA and he was started on teclistamab monotherapy. Following one cycle of weekly teclistamab (1.5mg/kg), his post-treatment PET imaging demonstrated a complete response. He then underwent a consolidative allogeneic hematopoietic stem cell transplant from a matched unrelated donor. As of this report, he remains in a complete response based on his 30 day PET restaging imaging after transplant and without clinical evidence of disease more than 90 days after transplant. Patient 2 was a 60 year old male with medical history of comorbid CAD, stage IV chronic kidney disease, chronic back pain requiring opioid treatment, and peripheral arterial disease when he was diagnosed with Stage IV PBL after he was found to have mass-like thickening of his descending colon on a CT scan. He underwent partial colectomy, with pathology revealing PBL and his post-operative staging PET/CT scan noting osseus, splenic, and hepatic lesions consistent with advanced stage disease. He was initially treated with DA-EPOCH for six cycles with intrathecal methotrexate, which yielded an initial complete response, though he relapsed four months after completing therapy. He was then treated with gemcitabine, oxaliplatin, and daratumumab but progressed after eight cycles on treatment with abdominal and perisplenic masses confirmed on biopsy to be PBL He was then initiated on teclistamab, which was continued weekly. His treatment course was complicated by a viral pneumonia from which he recovered with IVIG and supportive measures. Following 2 total cycles of therapy, his repeat PET/CT imaging was consistent with a complete response (Figure 1), and he remains on weekly teclistamab therapy with no evidence of disease to date. The encouraging responses noted in our two cases illustrate the therapeutic potential of targeting BCMA using teclistamab in PBL. In our cases, teclistamab was a well tolerated therapy, even in our patient with significant comorbidities. This report provides a foundation for future prospective, multicenter clinical trials to evaluate the efficacy and safety of teclistamab in the treatment of relapsed and refractory PBL.

Treatments and outcomes of patients with localized extranodal NK/T-cell lymphoma (ENKL) diagnosed between 2014 and 2021: A cooperative study in Japan.

7565 Background: A previous cooperative study of patients (pts) with ENKL who were diagnosed between 2000 and 2013 in 31 institutes in Japan reported that the most common first-line treatment for localized ENKL was radiotherapy with dexamethasone, etoposide, ifosfamide, and carboplatin (RT-DeVIC) (66%). The 2-year (yr) overall survival (OS) and progression-free survival (PFS) of pts with localized ENKL were 78% and 68%, respectively (JCO 2017). RT-DeVIC has been included as a preferred regimen of combined modality therapy in the NCCN guidelines. The treatments and outcomes of pts with localized ENKL in recent clinical practice are unknown. Methods: We conducted a cooperative study (NKEA-Next; UMIN000046300) by hemato-oncologists and radiation oncologists of pts with ENKL who were diagnosed between 2014 and 2021 in 45 institutes in Japan. The results were compared with those from the 2000-2013 cohort (JCO 2017). The primary endpoint was OS of pts with ENKL who received next-generation treatment in 2014-2021 at participating institutes. We analyzed the data of localized ENKL. Results: Of 351 pts in the 2014-2021 cohort, 235 (67%) had localized ENKL. First-line therapy was RT-DeVIC in 185 (79%) pts, sequential chemoradiotherapy in 17, RT alone in 15 pts, and chemotherapy alone in 12 pts. Six pts received no treatment due to poor performance status (PS). Only two pts (1%) received anthracycline-containing regimens. With a median follow-up of 3.4 yrs, the 2-yr OS and PFS of localized ENKL were 83% and 75%, respectively. Pts treated with RT-DeVIC showed the following clinical features: median age, 58 yrs (range, 13-82); age &gt; 60 yrs, 45%; stage IIE, 34%; B symptoms present, 27%; elevated serum lactate dehydrogenase, 23%; ECOG PS 2, 4%; and soluble interleukin-2 receptor (sIL-2R) level &gt; upper limit of normal (ULN), 31% (57/183). The complete response rate was 88% among the 179 pts who were evaluated for response. The 2-yr OS and PFS of RT-DeVIC were 87% and 79%, respectively. There was no treatment-related death due to RT-DeVIC. G3/4 mucositis was recorded in 32% (G3, n = 59; G4, n = 0). G3/4 febrile neutropenia occurred in 18% (G3, n = 32; G4, n = 2). sIL-2R &gt; ULN was associated with worse OS ( P &lt; 0.01). In comparison with pts with localized disease in the 2000-2013 cohort, pts in the 2014-2021 cohort received RT-DeVIC more frequently ( P &lt; .001). There was no significant difference in OS ( P = 0.24) or PFS ( P = 0.10) between the two cohorts. Conclusions: RT-DeVIC was more frequently used for first-line treatment of localized ENKL, and its efficacy has been maintained, with 87% 2-yr OS in recent clinical practice in Japan, providing further evidence of RT-DeVIC as a preferred first-line regimen for localized ENKL. No obvious improvement in OS and a reduction in G3 mucositis were observed, suggesting that more efficacious and less toxic regimens need to be introduced.

The clinicopathological characteristics of gastric cancer and precancerous conditions in gastric DLBCL and MALT lymphoma patients: a multi-center retrospective study

Objective The objective of this study is to explore the clinicopathological characteristics of gastric cancer and precancerous conditions in patients with primary gastric lymphoma. Methods We analyzed 474 cases of primary gastric lymphoma, mainly DLBCL and MALT, from three clinical centres retrospectively, and compared the clinicopathological parameters of primary gastric lymphoma patients complicated with gastric cancer, precancerous conditions, or with no complications. Results A total of 5.1% of the patients with primary gastric lymphoma were diagnosed with gastric cancer, including metachronous gastric adenocarcinoma (3.2%) and synchronous gastric adenocarcinoma (1.9%). Of the patients with gastric lymphoma, 14.6% had precancerous conditions including atrophy (14.6%), intestinal metaplasia (8.9%), and low-grade intraepithelial neoplasia (1.9%). Primary gastric lymphoma patients with an ulcerative type (p = 0.009) and Lugano classification stage IIE + IV (p < 0.001) lymphoma had a higher risk of complicating with gastric cancers or precancerous conditions. The rate of infection of Helicobacter pylori (Hp) was 68.4% in patients with primary gastric lymphoma, which was higher in patients with MALT lymphoma (p < 0.001), Lugano classification stage I + II (p < 0.001), and patients complicated with precancerous conditions and gastric cancer (p < 0.001), especially gastric cancer of the intestinal type (p = 0.04). Gastric cancer (95.8%) and precancerous conditions (91.3%) occurred mostly in Hp-infected primary gastric lymphoma patients, with a minor subset of Hp-eradicated patients. Primary gastric lymphoma patients had a higher detection rate of early gastric cancer (25.0%) and a five-year survival rate (40.0%) than the general Chinese population. Conclusions Patients with primary gastric lymphoma have a high risk of developing gastric cancer and precancerous conditions, and this risk may be related to Helicobacter pylori infection. Follow-up of primary gastric lymphoma provides an opportunity for the detection of early gastric cancer. Key messages 5.1% of the patients with primary gastric lymphoma were diagnosed with gastric cancer. 14.6% of the patients with gastric lymphoma had premalignant lesions including atrophy (14.6%), intestinal metaplasia (8.9%), and low-grade intraepithelial neoplasia (1.9%). Primary gastric lymphoma patients complicating with gastric cancer had a higher infection rate of Helicobacter pylori (100.0%), a detection rate of early gastric cancer (25.0%) and a five-year survival rate (40.0%) than the general Chinese population.

Role of Surgery in Patients with Stage IE Primary Thyroid MALT Lymphoma Staged by a Modified Classification System: The Tokyo Classification.

To establish the appropriate staging system and assess the role of curative thyroidectomy alone (Surgery) vs. involved-site radiation therapy after open biopsy (OB-ISRT) in stage IE mucosa-associated lymphoid tissue (MALT) lymphoma. We examined the Tokyo Classification as a modified classification. This retrospective cohort study included 256 patients with thyroid MALT lymphoma; 137 underwent standard therapy (i.e., OB-ISRT) and were enrolled for the Tokyo classification. Sixty stage IE patients with the same diagnosis were examined to compare Surgery with OB-ISRT. Overall survival (p = 0.0092) and relapse-free survival (0.00113) were significantly better in stage IE vs. stage IIE under the Tokyo classification. No OB-ISRT and Surgery patients died, but three OB-ISRT patients relapsed. The incidence of permanent complications was 28% in OB-ISRT (mainly dry mouth) and 0% in Surgery (p = 0.027). The number of painkiller prescription days was significantly greater in OB-ISRT (p < 0.001). During follow-up, the rate of the new appearance/change of the low-density area in the thyroid gland was significantly higher in OB-ISRT (p = 0.031). The Tokyo classification allows an appropriate discrimination between stages IE and IIE MALT lymphoma. Surgery can provide a good prognosis in stage IE cases; it also avoids complications, shortens painful periods during treatment, and simplifies ultrasound follow-up.

Anaplastic Large-cell Lymphoma Involving Gastrointestinal Tract: A Clinicopathologic Study of 25 Cases of a Rare Tumor at a Rare Site.

Background. Anaplastic large-cell lymphoma (ALCL) is an uncommon lymphoma divided into anaplastic lymphoma kinase (ALK) positive, ALK negative, and breast implant-associated (BIA) ALCL. Gastrointestinal tract involvement is very rare and may be difficult to diagnose. Its recognition is crucial as prognostic ramifications are different. Objectives. To describe clinicopathological features of ALCL involving the gastrointestinal tract. Materials and Methods. Slides were reviewed. Diagnosis was confirmed. Histological and immunohistochemical features were described. Results.Twenty-five tumors were diagnosed during the study period. Ages ranged from 14 to 65 years (mean 41 years). Mean age for ALK-negative and ALK-positive patients were 49 and 17 years, respectively. Twenty-one were males and 4 were females. Eighteen involved small intestine. Mean tumor size was 4.2 cm. All showed diffuse sheets of large anaplastic cells with pleomorphic nuclei, abundant pink cytoplasm, and strong positivity for CD30. Epithelial membrane antigen was positive in 17 tumors and keratin was negative in all. Eighteen tumors were ALK negative. Out of 14 patients with follow-up, 12 died within a few months of diagnosis. Seven had stage IE, 5 had stage IIE, and 2 had stage IV disease. Two patients were alive at 35 and 60 months. Twelve received chemotherapy. Conclusion. A marked male predominance was noted. Small intestine was the commonest site of involvement. Majority were ALK negative. ALK-negative tumors occurred in older patients and ALK positive in younger patients. Prognosis was poor. ALCL should be included in the differential diagnosis of anaplastic epithelioid cell neoplasms in the gastrointestinal tract.

P090: Staging of Lung Lesions Survey Demonstrates Continued Need for Harmonization

Figure 1: Case vignettes and survey responses. Background: Staging of lung disease in Hodgkin lymphoma (HL) has been a longstanding and controversial challenge. In 1984, a survey of 14 experts was published in the Journal of Clinical Oncology. Four case vignettes were distributed, and experts designated each vignette as either Stage IIE or Stage IV disease. Results were very divided for 2 of the 4 case vignettes, and the conclusion was a call for clear guidance and group consensus for staging of lung lesions. Despite updates to the HL staging systems at both the Cotswold (1989) and Lugano (2014) meetings, there remains today, nearly 30 years later, a perceived lack of uniformity in the approach to the staging of lung lesions even among experts in the field. The approach to each of these issues not only varies across consortium groups but among individuals within the same consortium groups. When reviewing the most recent frontline HL trials from St. Jude-Stanford-Dana Farber, EuroNet PHL, and the Children’s Oncology Group, lung lesions were defined differently for each. This creates difficulty in comparing outcomes across trials and highlights the need for consensus and uniformity. Methods: The SEARCH for CAYAHL (Staging, Evaluation and Response Criteria Harmonization for Childhood, Adolescent, and Young Adult HL) group recreated a similar survey using 7 case vignettes and distributed it to 26 internationally recognized experts in pediatric HL to establish how current world experts are staging lung lesions (see Figure 1). Of the 21 survey respondents (81% response rate), 7 were radiologists or nuclear medicine radiologists, and 14 were pediatric oncologists. While there was near consensus for some of the cases, several remained very divided, namely around the role of PET avidity in lung lesions, the number and location of lung lesions, and the concept of contiguous disease. Conclusions: After reviewing the results of our survey, the SEARCH group acknowledges the controversy and inconsistency with which we are currently staging lung disease around the globe. We are now actively working to establish clear criteria, based on available data, and expert consensus through Delphi survey methods, and plan to have this completed in the fall of 2022.

ABCL-398 Primary Breast Diffuse Large B-Cell Lymphoma: An Indian Experience

Primary breast diffuse large B-cell lymphoma (PB-DLBCL) is a rare site of localized extranodal DLBCL. It represents 0.5% of all primary malignant neoplasms of the breast and around 2% of extranodal lymphomas. There is a paucity of data regarding the clinicopathologic characteristics, cell of origin, response, and outcome of treatment with uniform treatment protocol in patients from India diagnosed with PB-DLBCL. This was a retrospective study of 18 consecutive cases of PB-DLBCL registered at All India Institute of Medical Sciences between 2006 and 2021 treated with uniform 3x weekly cyclophosphamide, doxorubicin, vincristine, and prednisolone (CHOP) ±rituximab (R) with involved-field radiotherapy. The patients were classified as germinal center B-cell-like or activated B-cell (ABC) type using the Hans classification. The median age was 43 (21-67) years with a male:female ratio of 1:17. All the patients presented with a breast lump with a median tumor size of 5 cm. The right side was involved in 11 (61.1%) cases. Eight patients were diagnosed with lumpectomy, 9 patients with biopsy, and one patient with mastectomy specimen. Out of 18 patients, Ann Arbor: Stage I was seen in 6 (33.3%) patients, Stage IIE in 11 (61.1%) patients, and Stage 1V in 1 (5.5%) patient. B-symptoms and bulky disease were seen in 6 (33.3%) and 7 (38.8%) patients, respectively. Cell of origin data were available in 14 patients, and 11 (71.4%) were ABC in origin. Low- and intermediate-risk International Prognostic Index was seen in 42% and 48% of patients, respectively. The median number of chemotherapy cycles was 4 (3-6), rituximab was used in 10 (55.5%) cases, and CNS prophylaxis with intrathecal methotrexate was used in 10 (55.5%) cases. The complete response rate was 83.3%. After a median follow-up of 42 months, the 3-year progression-free survival and overall survival were 75% and 88%, respectively. The most common site of relapse (n=3) was parenchymal brain disease. Multimodality combination therapy (chemotherapy, immunotherapy, and radiation) can give good survival with PB-DLBCL but adding high-dose methotrexate to existing protocol needs further exploration.

ABCL-178 Primary Gastric Lymphoma Mimicking Gastric Carcinoma: The Clinicopathological Features and Treatment

The stomach is the most common site of gastrointestinal lymphoma and accounts for fewer than 5% of primary gastric tumors. Gastric lymphomas can be primary or secondary to systemic lymphomas. Primary gastric lymphomas (PGL) are defined as the involvement of the stomach mainly without systemic disease or those with gastrointestinal symptoms on presentation. This study reports a single-center experience with PGL simulating carcinoma with analysis of clinicopathological presentations and lines of treatment. A retrospective study was performed between September 2006 and May 2021. The study was carried out in the Oncology Center, Mansoura University, Mansoura, Egypt. The study included 66 PGL patients who were F/M (50%/50%) with a mean age of 58.26±12.8 years. Twenty-nine patients underwent gastrectomy, and 37 patients were treated with rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisolone (R-CHOP). The patients' clinical, endoscopic, and pathologic data were analyzed. Demonstration of the clinicopathological features of PGL patients and assessment of the role of surgery in PGL in the era of rituximab. The patients' presentations were dyspepsia (65.2%), vomiting (21.2%), gastrointestinal bleeding (10.6%), and dysphagia (3%). The endoscopic appearances were ulcerating lesions (60.6%), exophytic masses (24.2%), hypertrophic lesions (10.6%), and hyperemic lesions (4.5%). The median tumor size was 4.9 cm (range 1.5-17 cm). The tumor locations were the lower part of the stomach (n=40 cases), the middle part (n=11 cases), the upper part (n=14 cases), and diffuse (n=1 case). Patients were classified by Lugano staging into stage I (18.2%), stage II (53%), stage IIE (16.7%), and stage IV (12.1%). Twenty-nine patients underwent surgical exploration in the form of distal gastrectomy (79.3%), total gastrectomy (13.8%), and gastro-jejunostomy (6.9%). Indications of surgery were PGL mimicking carcinoma in 25 patients and 4 patients with gastric outlet obstruction. Pathologically, PGL was diffuse large B-cell lymphoma (DLBCL) (87.9%), mucosa-associated lymphoid tissue lymphoma (7.6%), follicular lymphoma (3%), and T-cell-rich non-Hodgkin lymphoma (1.5%). The median overall survival was 27.5 months (2-146 months). PGL is simulating gastric carcinoma in clinical and radiological presentations, and surgery may be a therapeutic option, especially in DLBCL.

Clinical Characteristics and Prognostic Factors in Primary Breast Diffuse Large B-Cell Lymphoma.

BackgroundPrimary breast diffuse large B-cell lymphoma (PB-DLBCL) is a rare subtype of non-Hodgkin lymphoma (NHL) with limited data on the clinical features and prognostic factors.Patients and MethodsA consecutive cohort of patients with PB-DLBCL was retrospectively analyzed in our hospital from February 1997 through July 2018. The primary endpoint is overall survival (OS) contributing to any cause.ResultsA total of 76 patients were diagnosed with PB-DLBCL. The median age at diagnosis was 51 years (range: 25–80 years), with female prevalence (98.7%). Forty (52.6%) patients had right-sided breast involvement but no bilateral breast involvement at diagnosis. Overall, disease stages IE and IIE were seen in 55 (72.4%) and 21 (27.6%) patients, respectively. According to the stage-modified International Prognostic Index (IPI), 37 (48.7%) patients were classified in the very good risk group (IPI 0). Of the 72 patients available, the non-germinal center B-cell (non-GCB) subtype of DLBCL was observed in 66 (91.6%) patients. All patients received anthracycline-based chemotherapy, 56 (73.7%) with rituximab, 31 (40.8%) also with additional radiation therapy, and 14 (18.4%) patients received a prophylactic intrathecal injection. Seven (9.2%) patients had refractory disease. With a median follow-up of 6.8 years (range 0.4–25.0 years), 10 (13.2%) patients had a relapse in the central nervous system (CNS) site. The 5-year and 10-year OS of all the patients was 97.2% (95% CI: 99.3–89.5) and 84.8% (95% CI: 70.0–93.5), respectively. The median OS was not reached. The median progression-free survival (PFS) was 10.3 years for patients with PB-DLBCL. The 5-year PFS of all the patients was 76.3% (95% CI: 64.6–84.6). Univariate analysis revealed several prognostic factors, including stage-modified IPI, breast surgery, refractory disease, and CNS relapse. Multivariate analyses produced two independent prognostic factors for patients with PB-DLBCL, including stage-modified IPI score (2–3 versus 0) (hazard ratio: 19.114, 95% CI 1.841 to 198.451, p=0.013) and CNS relapse (hazard ratio: 5.522, 95% CI 1.059 to 28.788, p=0.043).ConclusionIn our cohort, PB-DLBCL clinical features are similar to prior literature reports. Stage-modified IPI score and CNS relapse were associated with overall survival.

Thyroid Lymphoma: Case Series Review.

Primary thyroid lymphoma (PTL) is a rare malignancy accounting for about 5% of all thyroid malignancies and less than 2% of extra-nodal lymphomas. Diffuse B-cell lymphoma is the most common pathological subtype of PTL. This is a retrospective review of all patients diagnosed with thyroid lymphoma in our hospital during the period from 2011 to 2021. All patients had clinical, radiological, and pathological evaluation. We have followed up these patients since diagnosis till now. Eight patients were included in our review. Four patients were males and four were females. Mean age at diagnosis was 63 ± 32years of age. Diagnosis was confirmed only with tissue histology. Six patients had stage IIE disease and two had stage IVE disease. Four patients had disease related mortality. Of the other four patients, two had chemotherapy (RCHOP) and one had local radiotherapy; all achieved complete remission, and the last one had hemithyroidectomy to relieve airway compression and still under treatment. Primary thyroid lymphoma is rare malignancy of the thyroid gland. Advanced stage and age at the time of diagnosis, the presence of compression manifestations, large tumor mass, and presence of distant metastasis, all are poor prognostic criteria. Our knowledge of the disease pathology, diagnosis, and management is still limited and more research studies are required.

The Relationship Between Primary Thyroid Lymphoma and Various Types of Thyroid Autoimmunity: A Retrospective Cohort Study of 498 Cases, Including 9 Cases with Graves' Disease.

Background: Primary thyroid lymphoma (PTL) is known to develop mostly in patients with Hashimoto's thyroiditis (HT), and it is rare for it to develop in patients with Graves' disease (GD). The objective of this study was to investigate the clinical features, pathological findings, and long-term outcomes of PTL patients, grouped according to the presence of GD, HT, or no autoimmune thyroid disease (AITDs). The GD group was of major interest due to limited knowledge of the relationship with PTL. Methods: In this single-center retrospective cohort study, we reviewed the medical records of all patients diagnosed with PTL between August 1979 and October 2021, and we characterized the patients according to the presence of HT, GD, or no AITDs. Pathological specimens were classified according to the World Health Organization classification. Staging was performed in accordance with the Ann Arbor classification. Results: During the 42-year period, 498 participants were diagnosed with PTL. The median age was 68 (interquartile range 61-76) years, and 221 patients were stage IE, whereas the remaining 277 patients were stage IIE. Of the PTL patients, 431 (86.6%) were diagnosed with HT, 9 (1.8%) were diagnosed with GD, and 58 (11.6%) did not have AITDs. All nine patients with GD were positive for anti-thyroglobulin antibody and/or anti-thyroid peroxidase antibody. All patients with GD were treated with anti-thyroid medication. There were no significant differences in the proportions of each subtype of PTL between the PTL patients with GD and all subjects with PTL (p = 0.51), PTL patients with HT (p = 0.51), or PTL patients without AITDs (p = 0.48). The median follow-up time was 6.2 (interquartile range 3.0-10.7) years after the diagnosis of PTL. The Kaplan-Meier curve analyses showed no significant differences in overall survival and event-free survival between PTL patients with GD and those with HT (p = 0.37), or between PTL patients with GD and those without AITDs (p = 0.43). Conclusions: The PTL was observed with HT in a majority of cases, and rarely with GD (1.8%). The proportions of each pathological subtype of PTL and the prognosis of PTL were not different between the patients with GD and those with HT or those without AITDs.

Role of local treatment in primary breast B-cell non-Hodgkin's lymphoma: a propensity score matching-based analysis from SEER database.

Primary breast lymphoma (PBL) has been defined as disease localized to breast with or without ipsilateral axillary nodal involvement. Primary breast B-cell non-Hodgkin's lymphoma is rare to be diagnosed clinically. The role of surgery and radiotherapy (RT) as local treatment is unclear. The aim of this study was to evaluate the prognostic factors and investigate the effect of local treatment in patients with primary breast B-cell non-Hodgkin's lymphoma. We identified patients with primary breast B-cell non-Hodgkin's lymphoma diagnosed between 1998 and 2015 in the Surveillance, Epidemiology, and End Results (SEER) database. Propensity score matching (PSM) was performed to reduce possible bias between groups. The overall survival (OS) and disease-specific survival (DSS) were calculated using the Kaplan-Meier method. Multivariate Cox regression analysis was used to identify independent prognostic factors. Altogether 956 patients with primary breast B-cell non-Hodgkin's lymphoma were included. Most patients were white women over the age of 60. The most common histological type was diffuse large B cell lymphoma (DLBCL), and most patients present with stage I disease. Furthermore, old age (>60 years), DLBCL histology and stage IIE disease were the statistically significant factors associated with worse OS and DSS. Surgery did not improve survival of patients, and surgery combined with RT did not achieve a better prognosis than RT alone. RT was associated with better survival in patients with stage IE DLBCL, but patients with stage IE MZL and FL and stage IIE primary breast B-cell non-Hodgkin's lymphoma could not benefit from RT. In local treatment, surgery offered no survival benefit for patients with primary breast B-cell non-Hodgkin's lymphoma, while RT is an effective choice because it can improve both OS and DSS in the stage IE DLBCL subgroup.

Long-Term Clinical Outcomes and Sequelae of Therapy in Early-Stage Orbital Mucosa-Associated Lymphoid Tissue Lymphoma

IntroductionOrbital mucosa-associated lymphoid tissue (MALT) lymphoma, which are rare and indolent, often present at an early stage. We report the efficacy and safety outcomes of treatment in these patients. MethodsWe analyzed adult patients with stage IE or IIE orbital MALT lymphoma between 1999 and 2017 treated at our institute. We assessed local control (LC) rates, overall survival (OS), relapse-free survival (RFS) using Kaplan Meier method and the incidence of late toxicities. ResultsSeventy patients were analyzed for clinical outcomes. The median age at diagnosis was 52 years (IQR-45-62 years). Radiotherapy was offered to 97% of patients and the dose ranged from 36 to 45 Gy. Chemotherapy was administered in 5(7.1%) patients. Relapse occurred in 8 patients (local: 2, distant: 6). At a median follow-up of 101 months (IQR-47-146 months), the median OS and RFS was not reached. 8-year OS, RFS and LC rates were 96.5%, 88.5%, 96.7% respectively. Univariate analysis showed age ≤60 years and lacrimal involvement significantly correlated with better OS (P = .01 and .04, respectively). Cataract was the most common sequelae observed in 31 patients (44.3%). ConclusionModerate doses of radiotherapy are curative in early-stage orbital MALT lymphoma with favorable clinical outcomes. Lower doses of radiation can reduce the toxicity further, without compromising efficacy.

Clinicopathological features and prognosis of the skeletal-muscle cytotoxic T-cell lymphoma

Objective: To investigate the clinicopathological features and prognosis of skeletal-muscle cytotoxic T-cell lymphoma (CTL). Methods: The clinical data of 14 cases of skeletal muscle CTL and 47 cases of non-skeletal muscle extranodal CTL patients in Beijing Friendship Hospital Affiliated to Capital Medical University and the 989 Hospital of the joint logistics support force of the people's Liberation Army (the former 152 hospital) from 2008 to 2019 were collected retrospectively. Immunophenotype, EBV infection status and T-cell receptor (TCR) clonality of tumor cells were evaluated. The clinicopathological features and prognosis of the two groups were compared. Results: Skeletal-muscle CTL accounted for 23.0% (14/61) of extranodal CTL in the same period. The median age of the patients was 42.3 years (range 11-76 years), including six males and eight females. The main clinical manifestations were painless masses. Two patients (2/14) had B symptoms. The tumors occurred in the cheek (7 cases), the tongue (4 cases), the lower lip (3 cases) and the left upper arm (2 cases), and in two cases had two sites. Ten cases were of stage ⅠE and four cases stage ⅡE. Compared with non-skeletal-muscle extranodal CTL, many patients of skeletal-muscle CTL did not have B symptoms, the clinical stage was lower, and the tumor mainly involved the oral cavity (cheek, tongue and lip, P<0.05). Morphologically, the tumor showed diffuse infiltration of heterotypic lymphocytes in skeletal muscle. Immunohistochemistry showed that in 11/14 cases, there were reduced or loss of expression of some the T cell antigens (CD2, CD3, CD5, CD7). TIA-1, Gr B and CD8 (CD8+>CD4+) were expressed in all cases, and CD56 was negative. The median Ki-67 proliferation index was 35.0% (range 5%-60%). EBER in situ hybridization was negative in all cases. The results of TCR clonality analysis showed clonal TCR gene rearrangement were detected in eight cases. The median follow-up time was 40 months (range 10-67 months). Ten patients were tumor free; the 5-year survival rate of skeletal-muscle CTL was 100%. Compared with non-skeletal-muscle extranodal CTL (5-year overall survival rate was 35.9%), the difference was statistically significant (χ²=8.277, P=0.004). Conclusions: Skeletal-muscle CTL mostly occurs in the skeletal muscle of cheek and mouth. Tumor cells show morphologic characteristics of muscle invasion and myositis-like feature. It also shows CD8+>CD4+immunophenotype, cytotoxic molecular pattern and is associated with low clinical stage and good prognosis.

Favorable Outcomes of Patients with Limited-Stage Ocular Adnexal DLBCL Treated in the Rituximab Era: Long-Term Follow-up of a Single Center Experience

Favorable Outcomes of Patients with Limited-Stage Ocular Adnexal DLBCL Treated in the Rituximab Era: Long-Term Follow-up of a Single Center Experience