Stage IVE Research Articles

Primary testicular lymphoma: Clinical characteristics and oncological outcomes.

Primary testicular lymphoma (PTL) is a rare testicular malignancy, despite being considered the most common testicular tumor in patients older than 60 years. Primary testicular lymphoma represents only 1%-9% of testicular neoplasms. Few studies have been published regarding its clinical features and management. This study aimed to analyze the clinical characteristics and outcomes of PTL. Orchiectomy specimens of 15 patients with PTL diagnosed during 2000-2020 at our institution were retrospectively studied. We collected information on demographic data, clinical features, management aspects, and outcomes of PTL treatment. Kaplan-Meier survival curves and Cox regression analyses were used to study survival. The median patient age was 69 years (interquartile range, 61-72 years). The most prevalent clinical presentation was testicular swelling (80%), and only 13.33% of the patients presented with systemic symptoms. Central nervous system involvement was detected in 6 patients (40%). Of the 15 patients, 5 (33.33%) had stage IE and 10 (66.67%) had stage IVE lymphoma. Diffuse large B-cell lymphoma was the most common histological subtype. Twelve patients (80%) received chemotherapy. During follow-up, 4 patients (26.67%) relapsed. The recurrence rate in the contralateral testicle was 13.33%. The median cancer-specific survival was 21.58 months (95% confidence interval, 0-43.95 months). Univariate Cox regression analysis showed that central nervous system involvement and International Prognostic Index score were significantly associated with shorter cancer-specific survival. Primary testicular lymphoma has a high relapse rate and poor prognosis. Management strategies typically include radical orchiectomy and systemic chemotherapy. Central nervous system involvement and International Prognostic Index scores were associated with lymphoma-specific 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.

Chest Wall Mass as the Dominant Presentation of Low-Grade B-Cell Non-Hodgkin's Lymphoma: A Case Report.

Low-grade B cell non-Hodgkin's lymphoma with dominant presentation of chest wall mass is presented in this report. The patient, a 65-year-old woman, presented with pain, rising skin temperature and redness, and swelling on the right lower chest wall. The histopathological examination revealed non-Hodgkin's lymphoma; the staging fluorodeoxyglucose-positron emission tomography/computed tomography demonstrated stage IVE disease, with hypermetabolic active disease in the right anterolateral chest wall in the form of large soft tissue mass and subcutaneous tissue with underlying bony erosion with extension into right anterior cardiophrenic space and superiorly up to right second costosternal region along the right internal mammary vessels. This was along with hypermetabolic active right axillary, right supraclavicular and left inguinal lymphadenopathy, and thickened hypermetabolic posterior right pleura with ametabolic right-sided pleural effusion. Bone marrow biopsy revealed uninvolved bone marrow. On follow-up after eight cycles of R-CHOP chemotherapy, the mass had completely resolved on contrast-enhanced computed tomography.

Treatment Outcomes of Low-grade Lymphoma of the Orbit

AimsRadiation therapy can be used with curative intent in patients with low-grade orbital non-Hodgkin's lymphoma (NHL) stages IE and IVE (limited to the bilateral orbits). This study evaluated local control and survival outcomes of patients with unilateral or bilateral orbital lymphoma treated in a provincial population. Materials and methodsThe study subjects were 176 patients with low-grade orbital or conjunctival lymphoma referred for management from 1980 to 2016. Demographic, tumour and treatment characteristics were abstracted by chart review. Recurrence-free survival (RFS) and overall survival were assessed with competing risks analysis and Gray's test. ResultsThe median follow-up was 8.5 years (range 0.4–29.5 years). The median age at diagnosis was 65 years (range 20–97 years). The most common histological subtype was mucosa-associated lymphoid tissue (MALT) (73%). Stage IVE accounted for 20.5% of the cohort. Orbital radiation therapy was used in 122 patients with stage IE (87%) and 12 patients with stage IVE (28%). The median dose was 25 Gy (range 2–35 Gy). Other treatments were antibiotics (seven patients), chemotherapy (10 patients), radioimmunotherapy (six patients), surgery (three patients) and observation (16 patients). Within the group treated with orbital external beam radiation therapy (EBRT) there were no local recurrences. Among those with stage IE NHL, EBRT was associated with improved local RFS (P ≤ 0.001) but did not have an impact on contralateral or distant RFS. In patients with stage IVE NHL limited to the bilateral orbit, bilateral EBRT was associated with improved RFS (P = 0.012) but did not affect distant recurrences or overall survival. ConclusionThere were no local recurrences after EBRT for stage IE and IVE orbital low-grade NHL. The treatments offered over the study period varied, but only EBRT for stage IVE disease improved RFS. This supports EBRT as the preferred primary treatment for patients with localised orbital low-grade lymphoma, including those with bilateral disease.

Clinical and pathological analysis of ocular adnexal diffuse large B-cell lymphoma

Objective: To summarize the clinical and pathological characteristics of diffuse large B-cell lymphoma of ocular adnexal (OA-DLBCL). Methods: A retrospective case series study. Twenty-three cases of OA-DLBCL were collected in Tianjin Eye Hospital from January 2005 to December 2018. The clinical manifestations and imaging examination results were analyzed. The pathological characteristics and immune subtypes were analyzed with hematoxylin and eosin staining and immunohistochemistry. Eighteen patients completed the follow-up. Overall survival (OS) time was from the date of diagnosis to the date of death or the final follow-up (April 30, 2020). Clinical stages were analyzed by the Ann Arbor classification; Kaplan-Meier plots were used to visualize survival outcomes. The different risk groups were compared using Log-rank test. Results: There were 13 males and 10 females. The median age was 65 years (range, 43 to 82 years). Twenty-two patients had unilateral lymphoma (14 left eyes, 8 right eyes), and one patient had bilateral lymphoma. The OA-DLBCL was in the orbit in 14 patients, in the lacrimal gland in two patients, in both the lacrimal gland and the orbit in three patients, in both the lacrimal sac and the orbit in one patient, in the conjunctiva in one patient, in both the conjunctiva and the orbit in one patient, and in the skin of eyelids in one patient. Imaging examinations showed the tumors were of irregular soft tissue density. MRI showed the tumors were close to the extraocular muscles or ectocinerea. Centroblastic morphology was present in 21 specimens, and 2 specimens had immunoblastic morphology. Six patients were of germinal center B cell-like (GCB) type, and 17 were of non-GCB type. There were 2 patients with double expression of cellular-myelocytomatosis viral oncogene and B-cell lymphoma 2. The follow-up time ranged from 25 to 156 months, and the median follow-up time was 48 months. Five of them had primary OA-DLBCL, and 13 patients had secondary OA-DLBCL. Five patients were at the Ann Arbor clinical stage ⅠE, one was at stage ⅢE, and 12 were at stage ⅣE. During the follow-up, 8 patients survived, and 10 died. The 1-, 3-, and 5-year OS rates were 88.9%, 71.4% and 41.7%, respectively. Log-rank analysis indicated that the Ann Arbor clinical stage and age were related to the OS of OA-DLBCL (χ²=7.448, 8.804; both P<0.01). The gender, tumor size, molecular typing, Ki-67 index, and bone invasion were not related to the OS of OA-DLBCL (all P>0.05). Conclusions: OA-DLBCL mainly occurrs in the elderly population, unilaterally, and in the orbit. Most molecular types are non-GCB subtypes. The Ann Arbor clinical stage and age are associated with prognosis. (Chin J Ophthalmol, 2021, 57: 366-371).

Clinicopathological features and possible prognostic factors in parotid lymphomas

To investigate the clinicopathological features and prognostic factors in lymphoma of parotid origin. Clinicopathological data of the patients with parotid lymphoma who were initially diagnosed in Peking University Hospital of Stomatology from 2006 to 2016 were collected and analyzed retrospectively. The patients were followed-up for 5 to 149 months with a median period of 45 months, and the factors influencing the prognosis were evaluated. A total of 41 patients with primary parotid lymphoma were included in this retrospective study. The rate of male to female was 1:2.15. The median age was 57 years (ranging from 8 months to 91 years). According to WHO classification, 40 cases (97.1%) were diagnosed as non-Hodgkin lymphoma (NHL), including 15 cases of extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue (MALT), 14 cases of diffuse large B cell lymphoma (DLBCL) as well as 4 cases of follicular lymphoma, while other subtypes of NHL were rare. Only one case was diagnosed as Hodgkin lymphoma. Regarding the clinical staging at the initial diagnosis, 37 patients (90.2%) were diagnosed as stage IE or IIE of the disease, while 4 patients (9.8%) as stage IIIE or stage IVE. Seven patients (17.1%) had a history of Sjögren syndrome (SS), all of whom were MALT lymphoma. The mean ill duration of the 41 patients were 20.7 months. Thirty-two patients (78%) presented a slowly growing painless mass in the parotid gland. Treatment options included localized therapy and systemic therapy, all of whom had good curative effect. Nine patients (21.9%) died during the follow-up period. The overall survival rates of 2-year and 5-year were 84.5% and 81.3% respectively. The univariate analysis demonstrated statistically significant differences for accelerated growth of tumor (P=0.005), and presence of tumor capsule (P=0.011). The multi-univariate analysis demonstrated statistically significant differences for presence of tumor capsule (P=0.041). A large majority of primary parotid lymphoma were NHL, among which MALT lymphoma and DLBCL were common subtypes. Most patients presented an indolent mass in parotid gland, which should be distinguished from the parotid benign tumors. SS is closely related to the pathogenesis of MALT lymphoma. The prognosis is better than that of other malignant parotid tumors. Absence of tumor capsule may predict a poor prognosis in patients with parotid lymphoma.

Single Institution Experience of Gastrointestinal Involvement of Diffuse Large B- Cell Lymphoma: Presentation, Outcomes and Patterns of Treatment Failure

IntroductionExtranodal (EN) involvement of the gastrointestinal (GI) tract in diffuse large B-cell lymphoma (DLBCL) presents heterogeneously across different anatomic sites and often occurs at initial diagnosis. Rituximab, anthracycline-based combination chemotherapy, radiation, and surgery have all been studied separately and in various combinations. However, given the variability in how patients may present in these contexts, there remains no consistent standard of care or pattern of treatment failure. We hypothesized these patients have different outcomes than those lacking this EN site of involvement.MethodsWe conducted a single-institution, retrospective analysis of consecutive cases who presented at initial diagnosis with DLBCL involving the GI tract including the esophagus, stomach, small intestine, or portion of large intestine. We performed a search in the University of Virginia Pathology database to identify all patients age >/= 18 years with DLBCL involving the GI tract and excluded those without additional clinical information available for review. A total of 51 patients were identified and we retrospectively collected data on demographics, date of diagnosis, involvement of extranodal sites, stage, International Prognostic Index (IPI) score, treatment, response to treatment, relapse, and survival at last follow-up.ResultsOf the 51 DLBCL patients, 65% of patients were male and 82% were Caucasian. Median age was 65 years (range 22-92). Three patients had HIV infection and no patients had active hepatitis B or C. Sites included gastric (51%), colon (27%), small bowel (12%), esophagus (4%), and rectum (2%); 4% had multiple GI sites involved. Forty-nine percent of patients presented with abdominal pain. Other common presentations included gastric/bowel perforation (12%), GI bleed (12%), gastric/small bowel obstruction (12%). Three cases (6%) were discovered on a screening colonoscopy. Forty-three percent of patients presented as stages I-IIIE versus 57% presented as stage IVE. Fifty-five percent were IPI 0-2 while 45% were IPI 3-5. Six patients (12%) had CNS involvement at presentation. Median follow-up was 20 months (range 26 days to 17 years). Six patients were lost to follow-up prior to assessment of response, and 4 had treatment discontinued early (3 due to grade 5 infection, 1 for GI perforation leading to death). One patient was still undergoing treatment.Forty patients were available to assess for response to treatment. The majority were treated with R-CHOP (57%) or DA-R-EPOCH (29%). Sixty-eight percent (n=27) had a complete response (CR), 22.5% (n=9) a partial response (PR), and 10% (n=4) were primary refractory. Of the 27 CRs, 13 (48%) had presented as stage 1-3 and 14 (52%) had presented as stage 4; 16 (59%) had an IPI score of 0-2 and 11 (41%) had an IPI score of 3-5. Of the four patients who had primary refractory disease, 3 had multiple EN sites involved at diagnosis, including 2 with CNS involvement. Of those who had a response (including CR or PR), median duration of response was 1 year (range 4 months to 13 years). Eleven patients (28%) had a relapse: 3 in the CNS, 5 in the GI tract (3 at the same site of initial involvement, 1 with a different GI site involved, and 1 with a different GI site in addition to systemic disease), and 3 in lymph nodes alone. Time to relapse ranged 1 month to 13 years with a median of 8.4 months. Fifty-seven percent of patients were alive at time of last follow-up. Median Overall survival (OS) was 5.7 years and Median Progression Free Survival (PFS) was 5.6 years (Figure 1).ConclusionThere is limited data on how patients with DLBCL involving the GI tract initially present and respond to therapy. This study encompassing almost twenty years of experience at a single institution, suggests that though patients present at all stages and as low risk vs high risk IPI, outcomes are poor compared to prior studies, especially when additional extranodal sites are involved. Duration of response can be substantial, however relapses are common and may present years later indicating ongoing follow-up is imperative. [Display omitted] DisclosuresPortell:AbbVie: Research Funding; Amgen: Consultancy; Kite: Research Funding; TG therapeutics: Research Funding; BeiGene: Research Funding; Genentech/Roche: Consultancy, Research Funding; Acerta: Research Funding; Infinity: Research Funding. Williams:Sandoz: Consultancy; Pharmacyclics: Research Funding; Verastem: Consultancy; Seattle Genetics: Consultancy; TG Therapeutics: Consultancy; Abbvie: Consultancy; Novartis: Research Funding; Gilead: Consultancy, Research Funding; Astra-Zeneca: Consultancy; Takeda: Research Funding; Celgene: Consultancy, Research Funding; Janssen: Consultancy, Honoraria, Research Funding; Kite: Consultancy; Juno: Consultancy.

Clinicopathological Features of Ocular Adnexal Mantle-Cell Lymphoma in an International Multicenter Cohort

To our knowledge, the clinical features of ocular adnexal mantle-cell lymphoma (OA-MCL) have not previously been evaluated in a large multicenter cohort. To characterize the clinical features of OA-MCL. This retrospective multicenter study included patient data collected from January 1, 1980, through December 31, 2015, at 6 eye cancer centers in 4 countries. Medical records of 55 patients with OA-MCL were reviewed; the median length of follow-up was 33 months. Overall survival, disease-specific survival, and progression-free survival were the primary end points. Fifty-five patients were included; ocular adnexal MCL was found to be most common in older individuals (mean age, 70 years) and men (n = 42 of 55; 76%). Patients with OA-MCL frequently presented with disseminated lymphoma (n = 34 of 55; 62%), and were likely to experience stage IVE disease (n = 35 of 55; 64%), with bilateral involvement (n = 27 of 55; 47%), tumor masses (n = 27 of 36; 75%), and involvement of the orbit (n = 32 of 55; 58%). Chemotherapy with or without external beam radiation therapy was the most frequently used treatment. Overall survival rates for the entire cohort were 65% at 3 years (95% CI, 52%-78%) and 34% at 5 years (95% CI, 21%-47%). Disease-specific survival after 5 years was 38% for the entire cohort (95% CI, 25%-51%); the disease-specific survival adjusted by eye cancer center was better in patients who had received rituximab in addition to the chemotherapy regimen (hazard ratio, 3.3; 95% CI, 1.0-14.7; P = .06). The median progression-free survival was 2.3 years (95% CI, 1.8-2.7 years) in patients who experienced recurrence after primary treatment, and 4.1 years (95% CI, 3.9-4.3 years) in patients who presented with a relapse of systemic lymphoma in the ocular adnexal region. These results suggest that the distinctive features of OA-MCL are its appearance in older male individuals, advanced stage and bilateral manifestation at the time of diagnosis, and aggressive course. The prognosis of patients with OA-MCL might be improved by addition of rituximab to chemotherapy treatment.

Clinical features of 11 cases of primary bone lymphoma

目的探讨原发性骨淋巴瘤患者的临床特征、治疗及预后。方法回顾性分析11例原发性骨淋巴瘤患者的临床资料，对其临床特征，诊治过程及预后进行分析总结。结果11例患者中女7例，男4例，中位发病年龄45岁，仅1例患者以无痛性肿块起病，其余均以骨痛为首发症状，主要病理类型为弥漫大B细胞淋巴瘤和间变大细胞淋巴瘤。Ann Arbor临床分期ⅠE期3例，ⅡE期2例，ⅣE期6例。6例患者采取化疗联合放疗，2例行骨关节置换术联合化疗，3例选择单纯化疗。11例患者经初始治疗后5例达完全缓解，4例达部分缓解，2例疾病稳定。中位随访时间为21（6~58）个月，中位无进展生存期为17（5~58）个月。结论原发性骨淋巴瘤诊断时多为晚期，临床表现及影像学检查缺乏特异性，目前治疗主要采取以化疗为主的综合治疗，预后相对较好。

Bilateral hydrocele. Uncommon clinical presentation of primary testicular lymphoma in the elderly.

Primary testicular lymphoma (PLT) represents 5% of testis tumors, the incidence increases in patients older than 60 years of age. Bilateral hydrocele is an unusual clinical presentation. Relapse in the central nervous system and in the contralateral testis is often observed. The US shows hypoechoic nodular lesions with a complete structural involvement of didymus and hypervascularization at Color Doppler. Orchiectomy should be performed in all cases as it is indispensable for the histopathological diagnosis and to characterize the immunophenotypic features. The most common histotype is diffuse large-B cell lymphoma. Combined biological approach and chemotherapy with rituximab and doxorubicin has radically changed the prognosis of disease. The authors report two patients of 81 and 82 years-old who referred for evaluation of massive bilateral hydrocele causing severe limitation of deambulation. Negative cytological findings for neoplastic cells in the scrotal effusion made difficult the differential diagnosis between inflammatory and malignant disease. Histopathologic findings made a diagnosis of high grade diffuse large B-cell NHL, respectively stage IV-E and stage III-E. The 82 years old patient was treated with 6 chemotherapy cycles of rituximab, cyclophosphamide, vincristine, prednisone. The exitus was dued to the umbilical hernia complications. In the 81 years old patient, cognitive deficit and severe impairment of general conditions constituted an absolute contraindication to polychemotherapy treatment. Rapid tumor progression led the patient to exitus 2 months after diagnosis. In both patients the delayed diagnosis of PLT was probably due to the reduction of welfare protection in the elderly with adverse social conditions.

Conjunctival Lymphoma--An International Multicenter Retrospective Study.

To date, the clinical features of the various subtypes of conjunctival lymphoma (CL) have not been previously evaluated in a large cohort. To characterize subtype-specific clinical features of CL and their effect on patient outcome. A retrospective multicenter study was performed. Patient data were collected from January 1, 1980, through December 31, 2010. The dates of the analysis were May 15, 2015, to August 20, 2015. The median follow-up period was 43 months. Seven eye cancer centers were involved in the study. In total, 268 patients with CL were identified, 5 of whom were excluded because of missing clinical data. Overall survival, disease-specific survival, and progression-free survival were the primary end points. Two hundred sixty-three patients with CL were included in the study. Their mean age was 61.3 years, and 55.1% (145 of 263) were female. All lymphomas were of B-cell type. The most frequent subtype was extranodal marginal zone lymphoma (EMZL) (68.4% [180 of 263]), followed by follicular lymphoma (FL) (16.3% [43 of 263]), mantle cell lymphoma (MCL) (6.8% [18 of 263]), and diffuse large B-cell lymphoma (DLBCL) (4.6% [12 of 263). Conjunctival lymphoma commonly manifested in elderly individuals (age range, 60-70 years old), with EMZL having a female predilection (57.8% [104 of 180]) and MCL having a marked male predominance (77.8% [14 of 18]). Unlike EMZL and FL, DLBCL and MCL were frequently secondary diseases (41.7% [5 of 12] and 88.9% [16 of 18], respectively), with MCL showing a frequent occurrence of stage IVE lymphoma (61.1% [11 of 18]) and bilateral manifestation (77.8% [14 of 18]). Localized disease (stage IE or IIE) was commonly treated with external beam radiation therapy (EBRT) with or without chemotherapy, while widespread lymphoma (stage IIIE or IVE) and MCL of any stage were managed with chemotherapy with or without EBRT. Diffuse large B-cell lymphoma and MCL had a poor prognosis, with 5-year disease-specific survival of 55.0% and 9.0%, respectively, in contrast to EMZL (97.0%) and FL (82.0%). Further survival predictors included age (EMZL), sex (FL), and Ann Arbor staging classification (EMZL and FL). The American Joint Committee on Cancer TNM staging showed limited prognostic usefulness, only being able to predict survival for patients with DLBCL. Conjunctival lymphoma consists of mainly 4 subtypes of B-cell non-Hodgkin lymphoma: EMZL, FL, MCL, and DLBCL. Mantle cell lymphoma is characterized by a particularly high frequency of secondary disease of stage IVE and bilateral manifestation. The histological subtype is the main outcome predictor, with MCL and DLBCL having a markedly poorer prognosis than EMZL and FL.

Primary Ovarian Non-Hodgkin’s Lymphoma: Retrospective Study of 16 Patients

Background: Primary non-Hodgkin’s lymphoma (PONHL) of the ovary is rare disease. Ovarian involvement by NHL is usually secondary, as a part of systemic disease. It is frequently diagnosed as ovarian carcinoma which causes a significant delay in diagnosis and management. Objective: To analyze, to report and to better understand the clinico-pathologic features and results of treatment, and prognostic factors of these tumors. Material & methods: This was a retrospective single institutional work that included 16 cases of primary ovarian non-Hodgkin lymphoma (PONHL) treated in National Cancer Institute-Cairo University from January 2010 till January 2015. All available medical data including the clinical and pathological characteristics, treatment, and outcomes of patients with PONHL are analyzed. Results: Data from 16 patients are obtained. The patient’s age ranges from 14 to 55 years (mean 28 years). Ascites is the most common manifestation (75%). Tumor size ranges from 5 to 24 cm (mean 13.1). LDH is elevated in all cases (mean 644 U/L) and CA-125 is elevated in only 4 cases (25%) especially when there is an extensive peritoneal irritation. Ten cases (62.5%) are bilateral with stage IV-E according to the Ann Arbor staging system. The remaining six cases (37.5%) are unilateral with Ann Arbor stage I-E. There are no stage II-E or III-E in the current study. Tumors are classified according to the World Health Organization as follows: diffuse large B-cell lymphoma (10 cases) (62.5%), Burkitt’s lymphoma (5 cases) (31%) and only one case of B-lymphoblastic lymphoma/leukemia. All the tumors are of B-cell lineage and are all CD20 positive. All Burkitt’s lymphoma cases show higher Ki67 index (4 cases are 100% and one is 88%). The case of B-lymphoblastic lymphoma/leukemia is positive for TDT & CD 10. Surgery is the main treatment modality for primary diagnosis and for staging, although chemotherapy should have been the primary treatment because it is one of the most chemosensitive tumors. Follow-up period ranges from 3 months to 5 years (mean 33 months). Ten patients are alive without disease. Two cases experienced relapse and one case died during chemotherapy treatment. The remaining three cases died from other causes than disease. The median overall survival time was not reached yet; however, the mean overall survival was 46.8 monthes; median progression free survival was 36 months. Conclusion: Most patients with PONHL present with symptoms attributable to an ovarian mass which necessitates extensive surgical staging that is not mandatory for lymphomas. More studies will be needed to better define and treat this rare entity.

PET/CT Findings in a Case With FDG-Avid Disseminated Lacrimal Gland MALToma With Sequential Development of Large B-Cell Lymphoma and Gastric MALToma

Primary orbital lymphomas are rare; the major histopathologic type is extranodal marginal-zone B-cell lymphoma of mucosa-associated lymphoid tissue (MALT) or MALToma. We present a case of a 79-year-old female patient with FDG-avid lacrimal gland MALToma with nodal and pulmonary involvement (stage IVE). Nasopharynx and gastric involvement were detected on restaging F-FDG PET/CT after rituximab therapy, and it was found to have a site of transformation to diffuse large B-cell lymphoma (DLBCL) in the nasopharynx. Gastric MALT lymphoma was also confirmed histopathologically, and she then underwent combination chemotherapy regimen, resulting in regression after 3 courses.

Clinical features, treatment and outcome of mucosa-associated lymphoid tissue (MALT) lymphoma of the ocular adnexa: single center experience of 60 patients.

BackgroundOrbital marginal zone B-cell lymphoma (OAML) constitutes for the most frequent diagnosis in orbital lymphoma. Relatively little data, however, have been reported in larger cohorts of patients staged in a uniform way and no therapy standard exists to date.Material and MethodsWe have retrospectively analyzed 60 patients diagnosed and treated at our institution 1999–2012. Median age at diagnosis was 64 years (IQR 51–75) and follow-up time 43 months (IQR 16–92). All patients had undergone uniform extensive staging and histological diagnosis was made by a reference pathologist according to the WHO classification.ResultsThe majority of patients presented with stage IE (n = 40/60, 67%), three had IIE/IIIE and the remaining 17 stage IVE. Seven patients with IVE had bilateral orbital disease whereas the others showed involvement of further organs. Treatment data were available in 58 patients. Local treatment with radiotherapy (14/58, 24%) or surgery (3/58, 5%) resulted in response in 82% of patients. A total of 26 patients (45%) received systemic treatment with a response rate of 85%. Nine patients received antibiotics as initial therapy; response rate was 38%. Watchful-waiting was the initial approach in 6/58 patients. In total 28/58 patients (48%) progressed and were given further therapy. Median time-to-progression in this cohort was 20 months (IQR 9–39). There was no difference in time-to-progression after first-line therapy between the different therapy arms (p = 0.14). Elevated beta-2-microglobulin, plasmacytic differentiation, autoimmune disorder and site of lymphoma were not associated with a higher risk for progress.ConclusionOur data underscore the excellent prognosis of OAML irrespective of initial therapy, as there was no significant difference in time-to-progression and response between local or systemic therapy. In the absence of randomized trials, the least toxic individual approach should be chosen for OAML.

Refractory Classical Hodgkin Lymphoma Presenting with Atypical Cutaneous Involvement and Diagnosis of ZZ Phenotype Alpha-1 Antitrypsin Deficiency.

Cutaneous Hodgkin lymphoma is a rare condition. Specific neoplastic involvement can be primary (confined to the skin) or secondary to systemic involvement (metastatic). Cutaneous involvement by HL usually occurs late in the course and is associated with poor prognosis; however in some cases it can exhibit indolent behavior. Skin involvement with nonspecific cutaneous findings may represent a paraneoplastic syndrome. We describe a case of 46-year-old white male patient presented with rash and lymphadenopathy which led to the diagnosis of stage IVE mixed cellularity classical Hodgkin lymphoma with skin involvement. His disease was refractory to multiple lines of chemotherapy including (1) AVD (doxorubicin/bleomycin/dacarbazine), (2) brentuximab, and (3) bendamustine, he later achieved complete remission with (4) GCD (gemcitabine/carboplatin/dexamethasone) salvage regimen. Bleomycin was not given secondary to poor pulmonary function tests. His treatment was complicated after AVD with multiple pneumothoraces which unmasked the diagnosis of ZZ phenotype alpha-1 antitrypsin (ATT) deficiency. Simultaneous existence of Hodgkin lymphoma and ATT is rarely reported.

Intrathecal Prophylaxis Might Be Required For Primary and Secondary Breast Lymphoma to prevent Central Nervous System Relapse

BackgroundBreast lymphomas is a rare type of malignant lymphoma. The major histopathological type of breast lymphoma is diffuse large B-cell lymphomas (DLBCL). Although conventional treatment regimen is cyclophosphamide, doxorubicin, vincristine, and predonisolone with rituximab (R-CHOP), the optimal cycles of chemotherapy, role of additional radiotherapy, and efficacy of intrathecal (IT) prophylaxis are still unclear. MethodWe retrospectively analyzed the clinical features and treatment outcomes of 22 patients with newly diagnosed primary breast lymphoma (PBL) of stage IE and IIE and secondary breast lymphoma of stage IIIE and IVE, and evaluated details of 20 DLBCL patients. All patients were treated at our institution between May 2002 and July 2013. Patients of stage IE were treated by 8 cycles of rituximab plus three cycles of CHOP-21 with radiotherapy, and patients of stage IIE-IVE were treated by 8 cycles of rituximab plus 6-8 cycles of CHOP-21 with or without radiotherapy. All patients were considered to receive intrathecal prophylaxis unless clinical study setting or patients' denial. Patients were divided into three groups; primary breast lymphoma (PBL) of stage IE, PBL of stage IIE, and secondary breast lymphoma of stage IIIE and IVE. ResultThere were 22 malignant lymphoma patients of breast such as one follicular lymphoma patient, one Burkitt lymphoma patient, and 20 DLBCL patients with no bilateral breast involvement. In patients with DLBCL of the breast, the median age was 60 years (range 32–69 years), with all female. The median largest tumor diameter was 5 cm, and eight patients had bulky disease (>5 cm). Nine of 20 patients (45%) received at least once of IT prophylaxis during one to two cycles of R-CHOP therapy. Six patients were stage IE, nine patients were stage IIE, and five patients were stage III and IVE, who had another invasion including liver, bone, and bone marrow. CR rate after initial therapy was 90% (18/20). Five of 20 patients (25%) developed central nervous system (CNS) relapse with the median time to CNS relapse of 28 months. Although four of 11 (36.4%) patients without IT prophylaxis versus one of nine (11.1%) patients with IT prophylaxis developed CNS relapse with no significant difference between two groups (p=0.363), and IT prophylaxis tends to prevent CNS relapse. The median follow-up time was 48.5 months, and the 3-year progression free survival (PFS) and overall survival (OS) in each group were 75%, 87.5%, and 40.0% (p=0.154), and 100%, 85.7%, and 53.3% (p=0.244), respectively. ConclusionsPrimary and secondary breast lymphoma is still considered high-risk for CNS relapse in Rituximab era. IT prophylaxis might prevent CNS relapse in breast lymphoma patients, and, further study is needed to evaluate the efficacy of IT prophylaxis or another appropriate approach for CNS relapse. Disclosures:Nishimura:Chugai Pharmaceutical CO., LTD.: Consultancy. Yokoyama:Chugai Pharmaceutical CO., LTD.: Consultancy.

Diagnosis and treatment of primary colorectal non-Hodgkin's lymphoma: analysis of 52 cases

To summarize and analyze the diagnosis, clinical features and therapy of primary colorectal non-Hodgkin's lymphoma (NHL). The clinicopathological data of 52 patients with primary colorectal NHL diagnosed and treated in our department from January 2000 to January 2010 were reviewed and analyzed retrospectively in this study. This group of patients was composed of 45 cases of B cell and 7 T cell lymphomas, including 33 males and 19 females, with a male to female ratio of 1.7:1, and the age at diagnosis was 16 - 74 years old, with a median age of 50 years. The ileocecal region was most frequently involved site, acounted for 48.1%. The common symptoms encountered were abdominal pain (66.7%), diarrhea (15.6%), blood stool (24.4%), and body weight loss (8.9%). All patients were eventually diagnosed by histopathology, and the DLBCL subtype took up 64.4%. Among the 45 cases of B cell subtype, 33 cases (73.3%) were of early stage (IE and IIE confirmed), and the 5-year survival rate was 78.1%, while those of stage IIIE and IVE comprised 26.7%, with a 5-year survival rate of 45.5% (P < 0.05). The 5-year survival rate of all patients was 71.1%. Surgery was employed in 36 cases, and 9 patients received chemotherapy alone. Radical surgery could significantly increase the patients' overall survival rate, as compared with the chemotherapy alone group and palliative surgery group (P < 0.05). Colorectal non-Hodgkin's lymphoma is a rare malignancy of the gastrointestinal tract. B cell type, male predominance and DLBCL subtype are most encountered manifestations in clinics. Multi-modality management with radical surgical resection of the primary lesion followed by standard chemotherapy, affords better local disease control, and a better survival outcome. Early detection and tailored immunotherapy can obviously prolong the long-term survival time.

Stage IVE Non-Hodgkin Lymphoma Presenting Solely as Swelling of the Lids and Conjunctiva

Stage IVE Non-Hodgkin Lymphoma Presenting Solely as Swelling of the Lids and Conjunctiva

Nasopharyngeal/nasal type NK/T lymphoma: Analysis of 23 cases and current review of the literature

This study aims to identify the presenting symptoms, treatment and outcome of patients with nasal natural killer T (NK/T)-cell lymphoma and to find possible differences in survival based on Ann-Arbor stage and international prognostic index (IPI). Computed tomography and biopsy results of 23 patients (15 males, 8 females; mean age 41 years; range 22 to 72 years) with extranodal NK/T-cell lymphoma who were treated at the department of clinical hematology between 1995 and 2011 were retrospectively analyzed. The median time from onset of clinical symptoms to histological diagnosis was five months. Most patients presented with nasal obstruction (69%) and rhinism (52%). The site of extranodal NK/T-cell lymphoma primarily involved nasal cavity in 39%. Orbital extension was observed in 26%. Lymphomas were classified as stage IE in 30.4%, stage IIE in 47.8% and stage IVE in 21.7%. Nineteen patients received treatment: 10 received chemotherapy plus radiotherapy, nine received chemotherapy only. We used several regimens of chemotherapy including some protocols containing etoposid, L-asparaginase and others without this drugs. Univariate analysis showed that lower IPI score, low Ann-Arbor stage and responsiveness to treatment with both chemotherapy and radiotherapy were significant factors influencing both OS and PFS. Nasal type NK/T-cell lymphoma showed a poor response to the conventional anthracycline-based chemotherapy, thereby an investigation for a novel therapy is urgently needed to improve survival.

P-0223 Primary Colorectal Lymphoma: 23 Case Reports and Literature Review

ABSTRACT Introduction Primary colorectal lymphoma (PCRL) is a rare disease and it comprises only 0.2 – 1.2% of all colonic malignancies. We present in this study the experience of our institute during nine years concerning this disease. Methods All patients who were treated for a primary colorectal lymphoma between 2000 and 2009 in the national institute of oncology in Rabat were analyzed retrospectively. Patient's medical records were used to identify demographic variables, symptoms, histological type, treatment used and evolution. Results 23 cases of PCRL were identified. There were 17 men and 6 women. The median age was 41 years (20-76). The most common signs at presentation were pain in 17 cases (74%), diarrhea in 15 cases (65%) and bleeding in 12 cases (52%). The sigmoid colon/rectum was the most invaded site (11 cases) followed by the caecum (4 cases) and the left colon (3 cases). The diagnosis was established through laparotomy in 12 cases (52%) and with endoscopy in 11 cases (48%). Most of cases (20 patients) were diffuse large B-cell lymphomas, 3 cases were T-cell lymphomas and one was follicular lymphoma. According to Ann Arbor classification, 13 patients were stage IE, 4 were stage IIE, 4 were stage IIIE and 2 were stage IVE. In the early stages, 9 patients (53%) underwent colectomy followed by chemotherapy while the 8 others were treated by chemotherapy alone. Concerning diffuse disease, patients were treated by chemotherapy exclusively. The CHOP regimen was used in all patients. Rituximab was used in only 4 cases. Median overall survival was 56 months (1-84). The 5-years survival was 64,7 % in stages IE- IIE versus 16.7 % in stages IIIE- IVE. Conclusion PCRL is a rare disease whose treatment is not well defined. In our experience, chemotherapy with CHOP or R-CHOP seems to give good results.