Extra Nodal Research Articles

275 (PB-099) Poster - Primary location of breast cancer and total tumor load of the sentinel lymph node

Background: Axillary lymph node staging is a determining factor in the treatment and prognosis of patients with breast cancer. One-step nucleic acid amplification (OSNA) is a molecular procedure that allows assessment of the total tumor load (TTL) in axillary sentinel lymph node (ASLN) and can be used to predict extra lymph node metastasis in early breast cancer. The primary location of the breast tumor seems to be closely related to ASLN positivity. The purpose of this study was to determine whether the primary location of the tumor could have an influence on TTL.

PSAT278 A Curious Case of Thyroglossal Duct Cancer

Abstract Introduction Thyroglossal duct (TGD) cyst is a common congenital anomaly. Thyroid cancer rarely occurs in TGD cysts, affecting less than 1% of cysts. Here we present a case of TGD cancer presenting as a central neck mass. Case Presentation 60 y.o female presented to her primary care physician with a neck mass without any compressive symptoms. She had no history of head/neck radiation exposure and no family history of thyroid cancer. She was noted to have a non-tender central neck mass with palpable cervical lymphadenopathy. Neck ultrasound revealed a 5.7 cm multilobulated cystic and solid mass anterior to the thyroid gland. CT scan of the neck revealed a lobulated mixed solid cystic mass at the level of the thyroid cartilage projecting anteriorly, with an abnormal right submandibular lymph node measuring 2.2×1.0 cm. She was evaluated by ENT and underwent resection of the neck mass and the hyoid bone (Sistrunk's procedure). Level 1b lymph node was also removed. Operative findings were concerning for a malignant process. This was confirmed on pathology with findings of TGD papillary thyroid cancer (PTC) and level 1b lymph node demonstrating metastatic PTC with cystic features. She was then referred to endocrine. Thyroid ultrasound revealed multiple bilateral intermediate suspicion nodules. Left inferior thyroid nodule was noted to have high suspicion sonographic features with abnormal right sided level 2 and 3 lymph nodes. She was referred for total thyroidectomy, pathology revealed multifocal PTC with lymph node involvement with extra nodal extension and metastasis to the left sternohyiod muscle, pT2, pN1b, AJCC stage 2. 6-week post-surgical labs revealed TSH of 1.69 mIU/L (ref range 0.40-4.50), thyroglobulin level of 0.8 ng/ml (ref range athyrotic <0.1), with undetectable thyroglobulin antibodies <1 IU/ml (ref range < or = 1 IU/mL). She underwent RAI with 104.6 mci. I-131 Metastatic survey revealed increased uptake in the thyroid bed, otherwise no increased activity elsewhere. Stimulated TSH and Thyroglobulin levels were done but not collected appropriately and therefore not mentioned here. Repeat labs 12 weeks after RAI revealed a TSH of 2.86 mIU/L, Thyroglobulin level of 0.2 ng/ml, and undetectable Thyroglobulin antibody. Conclusion There are no standardized guidelines for management of TGD cancer given the rarity of this condition. FNAB (Fine need aspiration biopsy) may be associated with high false negatives due to inadequate sampling, given the cystic nature of these lesions. In majority of these cases definitive diagnosis of cancer is made after excision of the TGD cyst. Some experts favor a conservative approach via Sistrunk's procedure and pursuing total thyroidectomy if there is high suspicion of thyroid gland involvement as well. Whereas other authors have suggested a more aggressive approach with total thyroidectomy even without evidence of concomitant involvement of the thyroid gland. Presentation: Saturday, June 11, 2022 1:00 p.m. - 3:00 p.m.

Impact of Depth of Invasion Tumor Budding and Worst Pattern of Invasion on Clinical Outcome in Post-Operative Oral Cavity Squamous Cell Carcinoma

<h3>Purpose/Objective(s)</h3> Definitive treatment for oral cavity squamous cell carcinoma (OCSCC) is surgery followed by post-operative radiotherapy (PORT) with or without chemotherapy. Risk factors for PORT are T3/T4, node positive, lympho-vascular space invasion (LVI) or perineural invasion (PNI), close margin etc. Depth of invasion (DOI), tumor budding (TB) and worst pattern of invasion (WPOI) and tumor infiltrating lymphocytes (TILS) may have prognostic implications. We aim to report the incidence of these factors and its association with clinical outcome post-operative OCSCC patients treated at our institute. <h3>Materials/Methods</h3> 150 post-operative patients of OCSCC undergoing curative oncological resection were analyzed with median follow up time 18 months from year 2019 to 2021. WPOI (WPOI-5) was defined as tumor dispersion of ≥1mm between tumor satellites. High risk WPOI was defined as (WPOI 4/5). Tumor budding is defined as the presence of single cancer cell or cluster of <5 cancer cells at the invasive front. DOI and staging were used as per AJCC 8^th edition. Disease free survival (DFS) and overall survival (OS) were estimated with Kaplan- Meier method. <h3>Results</h3> Median age was 50 years with M:F ratio of 135:15. Tumor site was buccal mucosa: tongue:alveolus:others as 84 (56%):49 (32%):13 (8.6%):4 (2.6%). LVI:PNI: close/positive margin: extra nodal extension was seen in 57 (38%):52 (34%):35 (23%):33 (22%) patients respectively. DOI=>10, DOI<10, high risk WPOI, tumor budding and TILS were observed in 79(52.6%), 71(47.3%), 102(72.8%), 97(71.3%) and 30(21.5%) patients respectively. Association of these have been listed in Table 1. AJCC eighth stage was I:II:III:IV as 30 (20%):32 (21.3%):38 (25.3%):50 (33.3%). Median RT Dose was 60 Gray. Sixty-four patients received CTRT (Median 5 cycles). Median Overall Treatment Time (OTT) was 98 (95-120) days. 2-year OS and DFS for the entire cohort was 81% and 67% respectively. 2-year DFS for patient with TILS was 86% vs.64% without TILS (p=0.07). PNI (60% vs. 72%; p=0.06) and bone invasion (47% vs 84%; p=0.04) portended poorer DFS. TB, WPOI and DOI did not statistically impact DFS in our cohort. <h3>Conclusion</h3> TB, WPOI and DOI are often associated in post-operative OCSCC with other adverse risk features. Patients with TILS tend to have a better clinical outcome

Metastatic parotid gland malignancy: A preliminary study in an eastern Chinese population

ObjectiveThis study aimed to determine the incidence and clinicopathological patterns of metastatic carcinoma of the parotid gland. MethodNinety patients with parotid gland metastases admitted to our hospital between January 2003 and December 2018 were included in this study. Clinical and pathological data were obtained from the medical records and follow-ups. Kaplan-Meier analysis was used to assess overall survival of patients. ResultsAmong the 90 patients, parotid gland metastases originated from the head and neck in 86 (95.6%), from non-head and neck in 4 (4.4%), from the oral cavity in 30(33.3%), and from the eyelid in 21 (23.3%). Among the 85 cases with parotid gland lymph node metastasis, 45 (52.9%) were diagnosed with extra-lymph node metastasis. The capsule of the parotid lymph nodes was thinner than that of the cervical lymph nodes (P < 0.05). Hematogenous metastases to the parotid gland (only five cases) were rare, mainly from the non-head and neck malignancies. Patients with oral squamous cell carcinoma and meibomian adenocarcinoma with parotid metastatic disease had poorer overall survival (P < 0.05). ConclusionEastern China population analysis showed that parotid gland metastases usually arise from oral squamous cell carcinoma and eyelid, but rarely from cutaneous squamous cell carcinoma. Most cases metastasize to the parotid lymph nodes via the lymphatic system and are prone to extranodal extension with little or no facial nerve involvement. These findings have important implications for the treatment of metastatic parotid malignancies.

ROSAI-DORFMAN DISEASE WITH ISOLATED INVOLVEMENT OF THE FIFTH LUMBAR VERTEBRA: A CASE REPORT

Rossi-Dorfman Disease (RDD), or Sinus Histiocytosis with Massive Lymphadenopathy (SHML), is a rare benign disease of unknown etiology and self-limited character that typically presents as painless cervical lymphadenopathy and fever. To report a case of RDD with isolated involvement of the fifth lumbar vertebra. A 27-year-old male patient was admitted to the medical clinic with low back pain for six months, evolving with plegia of the lower limbs. Magnetic resonance imaging showed a collapse of the fifth lumbar vertebra, with a 50% reduction in height, associating the heterogeneity of the remaining bone matrix with an infiltrative pattern and posterior retropulsion promoting obliteration of the L5-S1 right lateral process. Surgical decompression and L5 arthrodesis were performed. The bone matter was sent for histopathological evaluation and immunohistochemical profile, revealing CD68+, S100+ and Vimentin+, aspects compatible with DRR. Laboratory tests were within normal limits. Chest and abdominal computed tomography did not reveal lymph node enlargement. It was decided to carry out 25 adjuvant radiotherapy sessions for local control and prevention of possible recurrences. RDD is a disease belonging to the group of primary histiocytosis histologically defined by the phenomenon of emperipolesis, engulfment of intact lymphocytes or plasma cells in the cytoplasm of histiocytes or giant cells. While the etiology remains unknown, infectious and autoimmune mechanisms are suspected to be involved. The natural history of the disease has a benign and self-limited course; in 70% of patients, the disease is permanent and stable, 20% have spontaneous remission, and 10% progress to generalization. It mainly affects children and young adults, the mean age being 19-year-olds, with a slight predilection for males. The clinical presentation is dependent on the location and size of the lesions. The involvement is classically confined to lymph nodes, however, in 23% of cases, the pathology can be present in extra-lymph node sites, such as skin, soft tissues, nasal cavity, paranasal sinuses, eye sockets, bones, salivary glands and the central nervous system. Of these cases, only 11% have presentation in the bones. Laboratory tests commonly show polyclonal hypergammaglobulinemia, increased red cell segmentation index, and anemia. Histopathological examination with immunohistochemical technique is essential to confirm the diagnosis. Treatments include steroids, chemotherapy and radiotherapy. RDD is a rare benign disease whose symptoms stem from the location and size of the neoplastic growth which can produce a variety of clinical syndromes. Without recognition and proper management, the disease is a diagnostic challenge that can evolve unfavorably.

Sinus histiocytosis with massive lymphadenopathy (Rosai Dorfman Disease) - A rare case of isolated axillary lympadenopathy diagnosed on FNAC

Rosai-Dorfman disease (Sinus Histiocytosis with Massive Lymphadenopathy) is a rare disorder characterized by overproduction (proliferation) and accumulation of histiocytes in the lymph nodes of the body causing lymphadenopathy, most often involving neck. In some cases, abnormal accumulation of histiocytes may occur in other areas of the body besides the lymph nodes (extra nodal). These areas include the skin, central nervous system, kidney, and digestive tract. The case is being reported as a rare case involving isolated axillary lymph node and was diagnosed on FNAC.

Anaplastic large cell lymphoma of breast masquerading suppurative mastitis on cytology

Breast is an uncommon site of extra nodal lymphoma accounting for 2.2% of all extra nodal lymphomas. B cell lymphomas are more common than T cell types. The most common subtype is Diffuse Large B Cell Lymphoma. Breast lymphoma is very challenging to diagnose as it has nonspecific clinical and radiological features. We report a rare case of primary T cell lymphoma in a 20-year-old female. It was misdiagnosed as abscess on ultrasound and as suppurative mastitis on fine needle aspiration cytology (FNAC). However, excisional biopsy was suggestive of lymphoma and immunohistochemistry confirmed it as ALK positive, anaplastic large cell lymphoma. Even though FNAC is a part of the diagnostic triad for breast lesions, potential pitfalls cannot be overlooked and must be correlated with biopsy.

A CASE REPORT AND LITERATURE REVIEW: PRIMARY CARCINOMA OF LACRIMAL GLAND TREATED WITH ADJUVANT CHEMO- RADIATION.

Primary Sebaceous Carcinoma of Lacrimal Gland, possibly arising from heterotopic sebaceous tissue, is extremely rare and must be differentiated from secondary invasion of the Orbit by a primary Eyelid tumor/metastatic spread from other areas of the body. It is known to be aggressive So, delay in diagnosis can increase the chance of local recurrence and metastasis. In this article we report a case of Primary Sebaceous Carcinoma of the lacrimal gland, who underwent surgery with positive margin received Adjuvant radiotherapy in our Institute. A 55-year-OldCASE REPORT: Lady presented with Left eyelid swelling. MRI ORBITS &amp; PNS reveals malignant mass of approx. 4.3 X 3.8 cm size in superior &amp; temporal aspect of the left Orbit arising from the left upper eyelid compressing left globe and inseparable from the left lacrimal gland with approx. 2.3X 1.5 cm sized enlarged left Pre- auricular Node. No optic nerve encasement, Orbital wall Destruction &amp; Extension to adjacent paranasal sinuses is seen. CECT Thorax &amp; Whole abdomen – Normal. FNAC from eyelid swelling &amp; Pre- Auricular Node S/o Sebaceous Carcinoma with metastasis. Patient underwent left Orbital Exenteration + left supercial parotid split skin graft. HPE show the tumor mass of size 4.5 X 4X 3 cm, completely replacing tarsal plate of eyelid &amp; tumor mass show a highly inltrative malignant tumor with morphology consistent with sebaceous carcinoma with positive margin and tissue from left parotid show inltration by the sebaceous carcinoma (Possibly metastasis to the Intra-parotid node with extra nodal extension). Patient received Adjuvant/post op Chemoradiation 60 Gy/30#/ 6weeks/Rapid Arc technique with weekly 6 cycles chemotherapy Inj. Cisplatin. Patient requires close monitoring for any sign of recurrence / Distant metastasis. A Rare case report of sebaceousCONCLUSION: carcinoma of the Lacrimal Gland which requires appropriate evaluation &amp; management for better prognosis.

P1180: ARE EXTRANODAL LIMITED-STAGE DIFFUSE LARGE B-CELL LYMPHOMA MORE AGRESSIVE THAN NODAL FORMS?

Background: Limited-stage diffuse large B-cell lymphoma (DLBCL) is associated with favourable outcomes with overall survival of 70-80% at 10 years. Stage-modified IPI (sm-IPI) predicts the risk of relapse and death based on clinical factors that do not include extranodal (EN) involvement, although in some studies it has been associated with worse outcomes. Aims: To analyse the characteristics and outcomes of limited-stage DLBCL in a single center series and evaluate the prognostic impact of EN involvement. Methods: We conducted a retrospective analysis of consecutive patients with newly diagnosed stage I-II DLBCL treated with curative intent at a single centre from 2015 to 2019. Primary central nervous system (CNS) lymphoma, primary testicular lymphoma, primary mediastinal B-cell lymphoma and transformed lymphoma were excluded. Response to therapy was assessed according to Lugano criteria. Overall (OS) and progression free survival (PFS) were calculated by Kaplan-Meier method and comparisons done by the log rank test. Cumulative incidence of relapse was calculated using competing risk models and Gray test for group comparisons. Cox regression was used to identify prognostic factors for PFS. Results: Of 152 patients identified, 53 (35%) had EN disease and 99 (65%) were nodal (N). The most common EN sites were stomach (30%), sinus/nasal cavity (13%), skin and soft tissue (9%) and breast (9%). Seventy-two (47%) had low risk sm-IPI (45 N; 27 EN). Clinical characteristics were similar between both groups, except for stage (stage I in 50.9% of EN vs 32.3% of N cases) and bulky disease (EN 15.1% vs N 37.4%). All patients received R-CHOP (median 6 cycles; range 1-8). Eight patients, all with EN involvement (3 sinus/nasal cavity, 2 breast, 2 bone and 1 orbit), received CNS prophylaxis. Seventeen out of 152 patients received additional radiotherapy (RT), 71% of whom had nodal involvement; 16/17 were irradiated due to bulky disease and/or treatment with abbreviated immunochemotherapy. Globally, 89% patients had a complete response (CR) to treatment: 85% in the N and 96% in the EN group (p=0.212). Nine patients relapsed at a median of 18 (7-52) months after obtaining CR (6 from the N and 3 from the EN group). The 4-year cumulative incidence of relapse was similar: 11.2% (95%CI 9.0-13.9%) in EN and 6.7% (95%CI 5.9-7.5%) in N group (p=0.778). No gastric lymphomas relapsed. The most common sites of relapse were lymph nodes (3), soft tissue (3) and CNS (2). Nineteen (14 N; 5 EN) patients died at a median of 12 (0-47) months after starting treatment, mostly (52.6%) due to lymphoma progression. With a median follow-up of 43 months, 4-year OS and PFS were 87% and 82%, respectively. No significant survival differences were observed in patients with N or EN presentation with a 4-year PFS of 81.8% and 81.3% (p=0.428), respectively. On multivariable analysis adjusting for RT, sm-IPI and bulky disease, EN involvement was not associated with a decreased PFS compared with N involvement (HR 0.77; 95%CI 0.31-1.90; p=0.570). Image:Summary/Conclusion: Our study corroborates the very favourable outcomes of localized DLBCL. We could not find differences in PFS according to N and EN presentations when controlling for other prognostic factors. These results do not support different treatment strategies for EN disease; however, due to limited sample size the role of RT and the prognostic impact of specific locations cannot be fully ascertained.

Pathological characteristics and clinical prognosis of nodular sclerosis grade 2 of classic Hodgkin's lymphoma

Objective: To investigate the pathological characteristics and clinical prognosis of nodular sclerosis grade 2 of classic Hodgkin's lymphoma (cHL-NS2) in our cancer center. Methods: A retrospective collection of 23 cases of cHL-NS2 admitted in Cancer Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College from July 2008 to April 2019 was performed. Fifty-five cases of nodular sclerosis grade 1 of classical Hodgkin's lymphoma (cHL-NS1) during the same period were selected as control group. Survival curves were plotted using the Kaplan-Meier method, and Cox regression model was used to analyze the influencing factors for survival. Results: The median age of 23 cases of cHL-NS2 was 30 years old. Five cases had extra nodal invasion, and 19 cases were Ⅰ-Ⅱ stage based on Ann Arbor system. The pathological morphology of cHL-NS2 showed that the lymph node structure was completely destroyed and was divided into nodules by thick collagen. The tumor cells in the nodules were abundant and proliferated in sheets. The boundaries between the tumor cells were not clear. The incidence of tumor necrosis in cHL-NS2 was 43.5% (10/23), which was significantly higher than 18.2% (10/55) in cHL-NS1 (P=0.040). The 3-year progression-free survival (PFS) rate of patients in the cHL-NS2 group was 58.1%, which was significantly lower than 89.7% in the cHL-NS1 group (P=0.002). In all of 78 cases, the 3-year PFS rate of patients who did not obtain complete response (CR) was 67.1%, which was significantly lower than 92.2% in patients who achieved CR (P=0.030). Multivariate Cox regression analysis demonstrated that both cHL-NS2 and failure to obtain CR by first-line treatment were independent indicators for short PFS time (P<0.05). Conclusions: In cHL-NS2, the morphology of tumor cells are diverse, and tumor necrosis can be easily found. Under the current first-line treatments of doxorubicin, bleomycin, vinblastine, and dacarbazine (ABVD) or bleomycin, etoposide, doxorubicin, cyclophosphamide, vincristine, procarbazine, and prednisone (BEACOPP), cHL-NS2 is an independent indicator for worse PFS.

Abstract 3480: Quantitative assessment of the evolution of therapeutic target antigen expression in diffuse large b-cell lymphoma in response to treatment

Abstract Introduction: Variability in expression of B-cell surface antigens could be an important mechanism of treatment failure after immunotherapy in patients with relapsed or refractory (R/R) diffuse large B-cell lymphoma (DLBCL). Understanding the evolution of target antigen expression can guide selection and sequencing of newer therapies targeting CD19 and CD20. We sought to quantitate the change in expression of CD19 and CD20 in R/R DLBCL after Rituximab (R)-based chemo immunotherapy (CIT) relative to pretreatment levels. Methods: Pts. diagnosed with DLBCL after January 2014 who were R/R to R-based CIT (R-CHOP or R-EPOCH), were identified and clinical variables at diagnosis (Dx) and first R/R were recorded. Data from archival flow cytometry assays on Dx and R/R biopsies were accessed. Using FCS Express® software, neoplastic cells were gated, and fluorescence intensity (FI) of CD19 and CD20 expression were reported. Multivariate linear mixed model was used to compare median and geometric mean FI of CD19 and CD20 between Dx and R/R. Results: A total of 51 flow cytometry assays (26 Dx and 25 R/R) were analyzed for 33 pts., of whom paired assays (at both Dx and R/R) were available for 18 pts. Median age at Dx was 64 (range, 41-76) yrs, 24 pts. (73%) were male and 29 (88%) had IPI ≥ 2. Cell of origin at Dx was GCB in 16 (49%), non-GCB in 12 (36%) pts., while two had a double-hit rearrangement. Median time to R/R was 10.4 months. There was a significant reduction in median FI of CD20 from Dx [median: 40,610 (range: 167 - 259,962)] to R/R [median: 11,596 (range: 63 - 79,592)], representing a reduction of 63% at R/R relative to Dx (P= 0.01; 95% CI: 20-73%). Similar change was observed in geometric mean FI of CD20, which was reduced 65% at R/R relative to Dx (P&amp;lt; 0.01; 95%CI: 31-82%). Median and geometric mean FI of CD19 at R/R were 38% and 20% lower compared to Dx, respectively, but these differences were not statistically significant (P= 0.08 and 0.39, respectively). Relative change in FI at R/R in individual cases, compared to the mean FI of all Dx cases, showed that 21 out 25 R/R cases (84%) had reduction of CD20 whereas only 14/25 (56%) had reduction of CD19. Interestingly, 7/25 (28%) R/R cases had an increase in CD19 expression by &amp;gt;80%. Reduction in CD20 geometric mean FI from Dx to R/R was significantly associated with age &amp;gt;60 years (p=0.04), bone marrow involvement (p &amp;lt;0.01) and &amp;gt;1 site of extra nodal involvement (p= 0.03) at Dx. Conclusions: Quantitative assessment by flow cytometry revealed a significant decline in expression of CD20 at R/R compared to Dx in patients with DLBCL treated with R-based CIT. CD19 expression was largely unchanged, although dramatically upregulated in a subset of R/R cases. Given the role of CD19 mediated pathways in B-cell NHL and its association with PI3K pro-survival signaling, these data merit further exploration as a potential mechanism of treatment resistance. Citation Format: Agrima Mian, Narendra Bhattarai, Wei Wei, Manishkumar S. Patel, Paolo F. Caimi, Sarah L. Ondrejka, Brian T. Hill. Quantitative assessment of the evolution of therapeutic target antigen expression in diffuse large b-cell lymphoma in response to treatment [abstract]. In: Proceedings of the American Association for Cancer Research Annual Meeting 2022; 2022 Apr 8-13. Philadelphia (PA): AACR; Cancer Res 2022;82(12_Suppl):Abstract nr 3480.

Breast cancer oncotype recurrence score versus traditional tumor characteristics: Strong association with tumor grade and degree of hormone receptor (HR) expression but no correlation to tumor size, histologic subtype, lymph node status or patient age—A single center three-year retrospective study.

e12506 Background: Breast cancer accounts for a million new cases annually worldwide with 40,000 deaths reported in 2020 in the United States. Treatment includes surgery, adjuvant chemotherapy, radiotherapy and endocrine therapy. Oncotype recurrence score (RS), a 21-gene expression assay, is a tool to predict the benefit of adjuvant chemotherapy in hormone receptor (HR) positive breast cancer. High RS suggests benefit with chemotherapy. The goal of this study is to evaluate the relationship of the Oncotype RS with traditional prognostic/predictive factors. Methods: Retrospective IRB approved study of all patients from January 1, 2017, to December 31, 2019 with invasive breast cancer and an Oncotype RS in a community hospital setting. Study parameters included patient age, grade, tumor size, histologic subtype, HR status, number positive (0-4) lymph nodes (LN), extra nodal extension (ENE), lymphovascular invasion (LVI), and RS. Statistical analysis was done using linear regression and one-way ANOVA followed up with Tukey’s procedure. Results: A total of 470 patient charts evaluated and 22 patients excluded due to incomplete data with the following breakdown. LN positive = 14% (61/450). Oncotype score low (RS &lt; 11) = 25% (114/448), intermediate (RS 11-25) = 59% (265/448) and high (RS &gt; 25) = 15% (69/448). Grade 1 = 34% (153/448), Grade 2 = 50% (225/448) and Grade 3 = 15% (70/448). There was statistical significance in the mean difference in RS between the levels of grade and the levels of HR. The mean differences in RS between Grades 3 versus 1 was 10.9 (p &lt; 0.001); between Grades 3 versus 2 was 8.3 (p &lt; 0.001); and between Grades 2 versus 1 was 2.6 (p = 0.026). The mean Oncotype RS for grades 1,2,3 was 13.87 vs 16.33 vs 28.24 respectively. HR status was categorized as 51-100% (strong HR+), 11-50% (intermediate), or 1-10% (weak). Comparing strong HR+ versus intermediate/weak, the mean differences in RS were -41.4 and -35.7, respectively (p &lt; 0.001). There were no significant differences in RS evaluated against the following parameters, patient age, tumor size, number of positive lymph nodes (0-4), LVI, or ENE. Conclusions: Tumor grade and HR status had strong association with the Oncotype RS as expected. Grade 3 tumor associated with high RS (mean 28). Intermediate HR (11-50%) associated with much higher RS = 52 similar to weak HR (1-10%) = 58. We report that RS had no correlation to patient age, tumor size or even LN status. The LN and RS association may have been impacted by low (14%) LN positive status (reportable in the modern era). Our study adds to the data that biology trumps size (traditional risk factors) while posing the question does intermediate HR status (11-50%) deserve recognition as a more substantial risk factor than previously considered.

Malignant lymphoma of the lower urinary tract: A single institutional experience

Lymphoma of the urinary tract is relatively rare and comprises of < 5% of all primary extra nodal lymphoma. Diagnoses of these lesions at anearly stage is important as they can disseminate or transform into high grade lesion if there is a delay in the diagnoses. There are only few case series and case reports on the malignant lymphoma of the urinary tract. The aim of this study was to characterize lymphoma involving the urinary bladder and prostate. We retrospectively reviewed the clinical data and histologic findings of the malignant lymphoma involving urinary bladder and prostate at our institution. Lymphoma involving the lower urinary tract clinically presented with lower urinary tract symptoms and usually with concurrent associated urinary bladder cancer or prostatic cancer in our series. Lymphoma should be included in the differential diagnoses especially in patients with prior history of lymphoid disorders. There should be a high index of suspicion when there is any atypical lymphoid infiltrate in routine urinary bladder and prostate surgical specimens.

The false alarm: Rosai\u2013Dorfman disease of breast: case report

IntroductionRosai–Dorfman disease is a rare disorder caused by benign proliferation of histiocytes and thus also called sinus histiocytosis with massive lymphadenopathy. It is typically known to involve lymph nodes but can also affect extra nodal sites with breast involvement being extremely rare. This disease is known to mimic malignancy clinically and on imaging. The final verdict is through histopathological evaluation.Case presentationWe present a case of a 21-year-old female who presented with a palpable suspicious lump in right breast. Ultrasound, mammogram and MRI breast were done followed by fine needle aspiration, which showed lymphocytes and occasional histiocytes, which is suggestive of a lymphoid process. Surgical excision was done due to high suspicion of low-grade angiosarcoma in spite of a negative cytology. Diagnosis of Rosai–Dorfman disease was concluded after histopathological evaluation and was further confirmed by immunohistochemical staining by immunohistochemical markers S-100 and CD68.ConclusionRosai–Dorfman disease can present as low-grade angiosarcoma clinically as well as on imaging. There is a need for high level of suspicion to rule out benign mimics of malignancy to avoid radical surgery. Final diagnosis is by histopathology along with immunohistochemical staining.

Site-Specific Survival of Extra Nodal Diffuse Large B-Cell Lymphoma and Comparison With Gastrointestinal Diffuse Large B-Cell Lymphoma.

BackgroundDiffuse large B-cell lymphoma (DLBCL) constitutes 30% of all non-Hodgkin’s lymphomas. It can present as a nodal disease or as an extra nodal disease. Based on the site of origin, extra nodal DLBCL (EN-DLBCL) may have a distinct clinical outcome. Apart from the site of origin, factors including demographics, stage, and presence of any other primary malignancy also affect the outcome. The purpose of our study was to characterize prognostically distinct groups based on the site of presentation of EN-DLBCL.MethodsWe used 18 registries in Surveillance, Epidemiology, and End Results database to identify the patients with EN-DLBCL for 2000 - 2015 with last follow-up till December 31, 2018. A total of 30,290 EN-DLBCL patients were selected and categorized based on 13 broad sites grouping. Demographic variables were summarized. We did overall survival analysis with univariate and multivariate Cox-proportional hazard modeling. Short-term survival trend was calculated as well.ResultsThe percentage of EN-DLBCL of all DLBCLs is 34.48%. EN-DLBCL was comparatively seen more in males (54.94%) and non-Hispanic whites (71.52%). In terms of clinical characteristics, patients with EN-DLBCL were mostly diagnosed at age ≥ 60 years (66.11%), early stage (69.33%), and presentation as first primary cancer (81.89%). A higher risk of mortality was seen in non-Hispanic black (hazard ratio (HR) 1.36), with late age of onset (HR 2.69), late stage at presentation (HR 1.42), and with history of other malignancy (HR 1.29). Compared to the intestinal tract, the risk of overall mortality was higher in individuals with involvement of nervous system (HR 1.85), pancreas and hepatobiliary system (HR 1.22), and respiratory system (HR 1.18) and the best outcomes were seen in heart and mediastinal site (HR 0.58) of DLBCL.ConclusionBased upon our population-based study, we conclude that primary site of presentation of EN-DLBCL is an important prognostic factor with significant difference in survival based on histological and epidemiological characteristics.

Primary Gastric Lymphoma: A Silent Assassin

The gastrointestinal (GI) tract is the predominant site of extra nodal lymphoma involvement. Gastric lymphoma is the most common extra nodal site of lymphoma. Most of these lesions are either extra nodal marginal zone B cell lymphoma of mucosa associated lymphoid tissue (MALT) type or diffuse large B cell lymphoma (DLBCL). Primary gastric lymphoma accounts for less than 5% of gastric cancers. They are usually non-Hodgkin’s lymphomas (NHL), but have been considered as a separate entity from NHLs of peripheral nodes. The diagnosis of primary lymphoma of stomach requires histological confirmation without any evidence of peripheral lymphadenopathy or organomegaly. Secondary gastric lymphoma indicates the involvement of the stomach by a diffuse lymphoma developed elsewhere. We report a 60 years old male presented with intermittent vomiting and reflux symptoms. Upper GI endoscopy showed large hard mass with superficial ulceration in the fundus of stomach. Histopathology showed lymphoproliferative disorder. Immunohistochemistry confirmed positive CD 20 and BCL 2 suggestive of extranodal marginal zone B cell lymphoma. Subsequently, he was referred to surgery and oncology department for further management.

An uncommon association of L-asparaginase with gastrointestinal vascular congestion

A 71-year-old Chinese female was admitted with nasal symptoms and biopsy of her right nasal mass confirmed extra-nodal Natural Killer/T (NK/T) cell lymphoma. She was commenced on combination chemotherapy regime consisting of Prednisolone, Vincristine and L-asparaginase. Six days after initiation of chemotherapy, she developed an acute onset of generalized 2 abdominal distension with large amount of ascites. A Computed Tomography scan of her abdomen and pelvis showed stomach and proximal small bowel oedema and non-specific hyper densities within her duodenum. A pushed enteroscopy and colonoscopy subsequently showed a highly oedematous gastrointestinal tract, making it extremely challenging to scope. Multiple biopsies of her stomach corpus and fundus were acquired which revealed vascular congestion of the gastric body-type mucosa, with no malignancy involved, demonstrating a rare association of gastrointestinal vascular congestion with L-Asparaginase. Keywords: L-Asparaginase; extra nodal nk/t cell lymphoma; vascular congestion; hypercoagulable state.

Clinical and Survival Outcomes in Patients with Supra-Diaphragmatic Vs İnfra-Diaphragmatic Diffuse Large B Cell Lymphoma

Limited-stage diffuse large B-cell lymphoma (DLBCL) accounts for approximately 30% of all DLBCL cases. This study aimed to investigate the impact of the lymphoma involvement side relative to the diaphragm on clinical and survival outcomes in patients with limited-stage DLBCL treated with first-line rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone (R-CHOP). Data from 93 patients with limited-stage DLBCL between 2010 and 2019 receiving R-CHOP were retrospectively analyzed. Patients were divided into two subgroups according to the side of the diaphragm: 29 patients with infradiaphragmatic (InD) and 64 patients with supradiaphragmatic (SpD). There were no significant differences in survival outcomes [5-year PFS rate of SpD and InD groups, 76.7% and 85.7%, respectively (P =0.553); 5-year OS rates of SpD and InD groups, 82.1% and 89.1%, respectively (P = 0,524)] and clinical characteristics, except that extra nodal involvement was dominant in the InD group and the SpD group had a higher IPI. In conclusion, in early-stage DLBCL, extra nodal involvement is expected more if the primary involvement area is below the diaphragm, however whether the primary involvement area is below or above the diaphragm has no effect on survival outcomes. The results of this study need to be confirmed by further studies with a larger case group.

A258 ROSAI-DORFMAN0-DESTOMBES DISEASE: A RARE CAUSE OF OBSTRUCTIVE JAUNDICE

BackgroundRosai-Dorfman-Destombes Disease (RDD) is rare histiocytic disorder that is most frequently seen in children and young adults. Gastrointestinal involvement is reported in <1% of cases and typically involves the small bowel and colon. Pancreatic and hepatic involvement has been previously reported but is extremely rare.AimsTo describe a case of obstructive jaundice in the setting of a very rare histiocytic disorder known as RDD.MethodsCase ReportResultsA 59-year old previously healthy male of Asian descent presented with obstructive jaundice. Initial imaging demonstrated intra and extrahepatic biliary duct dilation with concurrent diffuse enlargement of the pancreas compatible with autoimmune pancreatitis. Endoscopic retrograde cholangiopancreatography (ERCP) was performed with stenting and biopsy. ERCP demonstrated a distal common bile duct stricture with biopsies suggestive of low grade reactive changes and inflammation. Subsequent endoscopic ultrasound (EUS) guided biopsy of the pancreas showed active and chronic inflammation, necrosis, and atrophic pancreatic tissue, with no definitive evidence of autoimmune pancreatitis (Figure 1). Ca-19-9 and IgG4 were normal. He was treated with a course of prednisone and responded well, with repeat CT imaging showing almost complete resolution of the previously demonstrated pancreatic changes. A diagnosis of autoimmune pancreatitis was made. He re-presented 6-months later, however, with fatigue and repeat imaging now displayed lymphadenopathy in the neck, chest, and abdomen, and a bulky pancreatic head with associated hepatomegaly. Lymph node excisional biopsy confirmed the diagnosis of RDD with the presence of scattered histiocytic cells showing emperipolesis with a low number of IgG4 positive cells (Figure 1). The patient was promptly initiated on prednisone and rituximab and has since then had excellent clinical response.ConclusionsRDD is a rare non-Langerhans cell histiocytosis of unknown etiology that has a prevalence of 1:200 000. RDD clinically presents with painless bilateral cervical lymphadenopathy and can manifest with both nodal and extra nodal involvement. The most common sites of extra nodal disease are the skin and central nervous system, but rarely, can also present with pancreatic involvement. The use of fine needle guided biopsy in diagnosing RDD with extra nodal disease can be limited by low yield, sclerotic tissue, or non-diagnostic findings. For this reason, RDD with pancreatic involvement can masquerade as autoimmune pancreatitis, pancreatic malignancy and IgG4-related disease.This case report raises awareness about RDD with pancreatic and biliary involvement, a rare entity, that can present with obstructive jaundice. Figure 1: Histological sections of lymph node(left) with hystiocytic cells showing emperipolesis(arrow) and pancreas(right) showing active and chronic inflamation, necrosis and atrophyFunding AgenciesNone

Primary testicular T cell Non Hodgkin Lymphoma with unusual histomorphology: A rare case

Primary testicular non-Hodgkin lymphoma (NHL) is an uncommon extra nodal lymphoma presentation, constituting 1-2% of all lymphomas. Majority are of B-Cell type, with very few cases being primary T-Cell phenotype. A 28 years male presented with painless, unilateral testicular swelling with elevated serum LDH levels, beta HCG and AFP serum levels were within normal limits. Gross evaluation of high inguinal orchidectomy specimen showed an enlarged homogenous, grey white lobulated mass with adjacent entrapped testicular parenchyma. Microscopy showed diffuse sheets of medium sized, monomorphic atypical lymphoid cells with sparing of adjacent seminiferous tubules. A panel of Immunohistochemistry markers done were suggestive of T-Cell Non-Hodgkin Lymphoma. Testicular T cell NHL are extremely rare amongst the testicular lymphomas, constituting for 5% of all testicular tumours. This case is being presented for its rarity in literature, especially in young age group and the unusual histomorphology.