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  • 1
    Publication Date: 2009-11-20
    Description: Abstract 2496 Poster Board II-473 Background: HMG-CoA reductase inhibitors (statins) have been used to treat hypercholesterolemia and hyperlipidemia for over 20 years. Statins competitively inhibit HMG-CoA reductase thereby blocking the synthesis of mevalonate. They directly inhibit synthesis of steroid hormones and cholesterol but also indirectly inhibit prenylation and ubiqitination. As all currently marketed statins are lipophillic, with the exception of Pravachol (pravastatin), several therapeutic trials have attempted to exploit these indirect actions to treat both neoplastic (glioblastoma, anaplastic astrocytoma) and degenerative (Alzheimer's, Parkinson's) neurologic disease. We studied the impact of incidentally prescribed statins on high-risk patients with primary central nervous system diffuse large B cell lymphoma (PCNSL). Methods: We used an IRB-approved clinicopathologic database, derived from comprehensive tumor registry data at the Massachusetts General Hospital, to identify all patients diagnosed with primary central nervous system lymphoma (PCNSL) between 1991 and 2007 (n=118). We excluded pediatric patients, patients who did not receive curative treatment and patients who failed to achieve a CR with initial therapy. Automated analysis of billing and medication administration records was used to identify the administration of statins to these patients. We compared the outcome of patients who were receiving statins to controls with PCNSL who did not receive statins. We excluded patients who did not receive statins within the time interval from 6 months prior to 2 years after their PCNSL diagnosis. As only a single statin pt was less than 50 we excluded all pts below this age from the cases and controls. All patients received high dose MTX based therapy. Overall survival (OS) was calculated from the date of first methotrexate. Results: Median age of statin patients was 66 yrs (range 52 – 76); median age of controls was 66 years (range 50 - 86). Nine patients were on atorvastatin, 9 were on simvastatin, 1 each were on pravastatin, rosuvastatin and fluvastatin. At a median follow-up of 47.5 months, concurrent statin therapy was associated with improved OS (62% vs. 37%)(p=0.04 Log-rank test). The median OS for all statin patients 〉 50 years old (n=21) was 60 months versus 37 months for all other patients 〉 50 years old (n=67) (p=
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  • 2
    Publication Date: 2008-11-16
    Description: Burkitt lymphoma (BL) is a highly aggressive B-cell non-Hodgkin lymphoma (NHL) accounting for nearly 3% of all newly diagnosed NHLs. Treatment of adult non-HIV-related BL with the intensive CODOX-M/IVAC regimen (modified Magrath regimen) produces complete responses (CR) in 75 to 86% of patients, with lower CR rates reported in HIV-related BL. The CD20-directed monoclonal antibody rituximab has never been reported in combination with CODOX-M/IVAC, and here we report the first series in BL patients, with or without HIV infection. A total of 24 patients were identified at our institutions who received rituximab plus CODOX-M/IVAC with curative intent. Rituximab was administered at 375mg/m2 on day 3 of cycle 1, and then on day 1 of subsequent cycles. Twenty-three patients received 4 alternating cycles of R-CODOX-M/R-IVAC for high risk disease, while 1 patient received 3 cycles of R-CODOX-M alone for low risk disease, defined as a single focus less than 10cm with a normal LDH. All patients received white cell growth factor support, and pneumocystis prophylaxis was included for all HIV+ patients. The median age was 45 years (17–67), advanced Ann Arbor stage 80%, LDH 〉 upper limit of normal 80%, extra nodal involvement 80% and ECOG PS
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  • 3
    Publication Date: 2008-11-16
    Description: The standard treatment for limited-stage Hodgkin lymphoma has been combined modality therapy, but late toxicities of radiation have prompted investigation of chemotherapy alone in low risk patients. Initial trials have demonstrated a small increased risk of relapse if radiation is omitted, but no difference in overall survival. We investigated the predictive value of interim FDG-PET (PET) scans in nonbulky limited stage patients, and asked whether PET may guide the use of consolidative radiotherapy for patients in complete remission after chemotherapy alone. A total of 68 patients with nonbulky limited stage disease were identified at our institutions with interim PET performed after 2–3 cycles of chemotherapy. All patients received anthracycline-based chemotherapy with curative intent. PET scan interpretations were extracted by chart review of radiology reports. The median age was 35 (range 18–77). Fifty-nine patients had disease in the neck and mediastinum, 6 had inguinal disease, and 2 in Waldeyer’s ring. Fifty-two patients were stage IIA, 4 were IIB, 10 were IA, and 1 was IB. Radiation was included at the discretion of the treating physician. Complete response required a negative PET scan. The complete response (CR) rate was 88%. Fifty-one patients (75%) had a negative interim PET, and 17 (25%) had a positive interim PET. Interim PET− patients were more likely to achieve a CR at the end of therapy compared to interim PET+ patients (98% vs. 59%; p=0.0001, Fisher’s exact test). At a median follow up of 32 months (range 3–70), the progression-free (PFS) and overall survival (OS) for the entire series were 85% and 100%, respectively. Interim PET− patients had an improved PFS compared to PET+ patients (90% vs. 71%; p=0.032, log rank test). Among the 60 patients who achieved a CR, 50 (83%) were interim PET−, and 10 (17%) were interim PET+. There was no difference in PFS between interim PET+ and PET− patients who achieved a CR. The most important predictor of PFS was achievement of CR at the end of therapy (92% vs. 37%; p
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  • 4
    Publication Date: 2010-11-19
    Description: Abstract 4150 Introduction: Primary extranodal pancreatic non-Hodgkin lymphoma (PPL) makes up no more than 0.16–4.9% of pancreatic malignancies and less than 0.7% of non-Hodgkin's lymphomas (NHL). Since lymphomas involving the pancreas may have a similar clinical presentation to primary pancreatic adenocarcinoma, and often have a similar radiographic appearance, pancreatic lymphoma is often not diagnosed until surgical exploration or definitive surgery for presumed pancreatic cancer. Preoperative distinction of adenocarcinoma and PPL is critical as the management and prognoses of these malignancies are mutually exclusive. The rarity of PPL has made epidemiologic studies difficult to conduct. Methods: We queried our IRB-approved clinicopathologic database, derived from comprehensive tumor registry data at the Massachusetts General Hospital, for all adult patients diagnosed with PPL between 2000 and 2010. The database contains 5821 patients with mature lymphoid malignancies. Cases were included in the analysis if they met clinicopathologic criteria for PPL, defined as dominant disease presentation within the body of the pancreas. Forty-five patients were found to have pancreatic involvement at initial presentation of whom 31 (68.9%) on further investigation had a pancreatic primary and are included in the analysis. For each patient, we collected complete demographic information, clinical presenting features, histology, chemotherapy regimens, use of radiotherapy, and type of surgical biopsy performed. We also collected outcome data including results of interim and final restaging scans. Results: PPL represented 0.5% of all mature lymphomas seen at our institution. The median age at diagnosis was 60 yrs (range 20–91). There were 21 male and 10 female patients. Eighteen patients had Diffuse Large B Cell Lymphoma (DLBCL) (one with a focus of follicular lymphoma (FL) grade 3), two each had Burkitts Lymphoma, FL Grade 1–2, and mantle cell lymphoma (MCL), and one each had small lymphocytic lymphoma (SLL), Hodgkin Lymphoma, Marginal zone lymphoma, and peripheral T cell Lymphoma not otherwise specified. Three patients had NHL not otherwise specified (NOS). Of the 31 patients, 13 patients were stage 4E, 5 were stage 3E, 8 were stage 2E, and 5 were Stage 1E. Seventeen patients presented with jaundice. In 2 cases clinical history at presentation was unavailable. Elevated lactate dehydrogenase (LDH) level was present in 18 of 26 patients (69%) for whom laboratory values were available. B symptoms were present at diagnosis in 13 patients, absent in 17 patients, and unavailable in 1. Diagnosis was made in sixteen patients by fine needle aspiration (FNA), in nine patients by core needle biopsy, in two by incisional biopsies, and four patients were diagnosed after a definitive pancreaticoduodenectomy (Whipple procedure). Information on therapeutic regimen was available for 24 patients. One of these 24 patients was treated with primary radiotherapy without chemotherapy for a grade 1–2 primary pancreatic FL. Twenty-three patients had initial chemotherapy: 8 with R-CHOP, 5 with CHOP, 2 with CVP, and 1 each with CHOP-14, CVP × 1 then CHOP, R-CVP, R-EPOCH, R-CODOX-M/R-IVAC, R-CDOP and R-CP then R-CHOP. One patient was treated with chemotherapy not otherwise specified in the medical record. Four of these 23 patients received consolidative radiotherapy after initial therapy. Chemotherapy had significant efficacy with an overall response rate (ORR) of 75% in all chemotherapy treated patients (10 CR, 8 PR). Therapy information was available on 13 patients with DLBCL with an ORR of 85% (7 CR, 4 PR) to R-CHOP in 8 patients, CHOP in 3, and 1 each R-EPOCH and R-CDOP. One patient died of meningeal relapse of DLBCL despite therapy with R-EPOCH. For the entire PPL group at median follow-up of 28.5 months, the progression free and overall survivals are 48.4% and 64.5%, respectively. At a median follow-up of 33.4 months, the progression free and overall survivals for the DLBCL-PPL group are 50.0% and 66.7%, respectively. Discussion: PPL is a rare extranodal presentation of lymphoma accounting for 0.5% of lymphomas seen at a tertiary referral center. Pre-surgical diagnosis of PPL is critical to avoidance of unnecessary major surgery. The outcome of PPL approximates the outcome of DLBCL in other sites. An increased rate of CNS relapse was not seen with presentations in this extranodal site. Disclosures: Hochberg: Biogen Idec: Speakers Bureau; Genentech: Speakers Bureau; Enzon Pharmaceuticals: Membership on an entity's Board of Directors or advisory committees, Speakers Bureau; Amgen: Speakers Bureau; WorldCare: Membership on an entity's Board of Directors or advisory committees; Proventys: Consultancy.
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  • 5
    Publication Date: 2010-11-19
    Description: Abstract 4042 Plasmacytomas are rare clonal proliferations of plasma cells that though cytologically identical to plasma cell myeloma, present with osseous or extraosseous growth pattern. Understanding their molecular characteristics can provide crucial insights into their pathogenesis and risk of progression to multiple myeloma (MM). To investigate the differences between extramedullary (EMP) and medullary plasmacytomas (MP) and MM without plasmacytomas, we sought to molecularly profile these tumors by tissue microarrays, gene expression, microRNA, and FISH. We identified 85 patients from our data base with a pathological diagnosis of plasmacytoma. Of the 85 patients, 13 patients presented with EMP, and 72 had MP. Among the patients with EMP (n=13), 2 patients presented with multiple lesions. Three of 13 (23%) patients progressed to develop MM at a median of 12 months. 72 patients presented with MP, of which 21 had solitary lesions and 27 (37%) progressed to MM at a median of 20.5months. There was a male preponderance (67% vs 33%) and the median age at diagnosis was 60.5 years (range 27.7–87.6). The mean overall survival for patients with EMP was 121 months (95% confidence interval[CI] 97–144 months) and for patients with MP was 102 months (95% CI 93–128 months) {p=0.025}. MicroRNA (miRNAs) profiling was performed on MP (n=19), EMP (n=7) and MM samples (n=66). Data was normalized using U6 endogenous control. Gene expression profiling was performed and correlated with the miRNA data to identify genes and transcripts of interest. miRNA 127, which regulates SET D8, was upregulated four fold in both MP and EMP compared to MM. miRNA 493, which regulates cadherin 11 and PTCH 1, both of which have been associated with metastatic potential in solid tumors, was similarly downregulated four fold in both MP and EMP compared to MM. A tissue microarray was created on 52 patients (8: EMP, 44: MP,) in whom paraffin-embedded tissue was available. Additional evaluation using SET 8, cadherin 11 antibodies and validation of additional functional targets is ongoing and will be reported. Differential expression patterns of factors involved in proliferation, survival, adhesion, and stroma-tumor cell interactions may help explain plasmacytoma biology and identify factors responsible for progression to MM. These insights may help identify new therapeutic approaches and targets in the treatment of these plasma cell disorders. Disclosures: No relevant conflicts of interest to declare.
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  • 6
    Publication Date: 2010-11-19
    Description: Abstract 4760 Background: Anaplastic large cell lymphoma (ALCL) is a rare disease, comprising 2–3% of all non-Hodgkin lymphomas. Case reports of seroma associated ALCL of the breast in association with silicone breast implants have appeared in the literature since 1997, but no data on the incidence of this complication has been reported. We use three case reports, including two previously published, in conjunction with data derived from three separate entities of Partners HealthCare (Brigham and Women's Hospital; Massachusetts General Hospital; Faulkner Hospital) to establish an incidence estimate for this rare entity. Methods: Individual cases were identified by pathologists, surgeons and medical oncologists. We compared a list of patients from the institutions’ Cancer Registries, with the results of a query we ran on an institution-internal query tool. For MGH patients only, we were also able to compare cancers revealed through a natural language processing search result of institutional pathology reports. Two of the cases were in the overlap of Cancer Registry data, and query results. One case was not contained within these results as it was omitted from the Cancer Registry. Case Presentations: Case 1 was surgically treated for breast cancer and reconstruction at New England Medical Center. At an unknown time relative to her breast cancer and tissue expander placement, she received a McGhan 210 cc textured silicone implant to her left breast. At time of rupture this implant was replaced with a 270 cc McGhan textured silicone implant filled to 295 cc at Newton Wellesley Hospital (NWH). Her surgical course was complicated by recurrent seroma, and she was eventually switched to Mentor smooth implant, with 275 cc implant on the right and 375 filled to 425 cc on the left. However, a biopsy of tissue at the time of this implant revealed ALK-negative ALCL in the left breast. Implants were removed at NWH and she was treated at Massachusetts General Hospital (MGH). After 3 cycles of chemotherapy (CHOP plus radiation) she remains in CR now at 18 months after treatment. Case 2 presented at Brigham and Women's Hospital (BWH) after a surgically treated right breast cancer with recurrence and reconstruction with a McGhan 270 cc textured saline implant. In 2000 the patient presented with erythema at surgical site of her cancer and a biopsy confirmed ALCL. Due to age the patient was treated with radiation alone and this induced a sustained remission of her ALCL. Case 3 originally had bilateral augmentation mammoplasty in 1974 with bilateral McGhan 270cc textured saline implants. She presented at Northwest Medical Center in 2007 with what appeared to be an abscess at her left implant site but was positive for ALCL when biopsied. She was treated with CHOP and radiation at that institution. She recurred in 2008 in the right breast and presented to BWH for treatment. She received ESHAP, then radiation, then gemcitabine, cisplatin, and dexamethasone; despite these treatments, her disease progressed and the patient died this year. Results: A query of the comprehensive electronic health database of the Partners hospitals (RPDR) revealed 9,941 patients at our institutions, who had undergone full or partial reconstruction of the breast, or removal of a breast implant or tissue expander from 1992–2009. Database queries revealed 5778 patients at MGH, 4,968 at BWH, and 4780 at Faulkner Hospital (FH) with non-Hodgkin lymphoma. Cancer Registry data revealed 18 ALCL patients (4 women) at MGH, 73 ALCL patients (24 women) at BWH and 2 ALCL patients at FH (1 woman). Of our three cases one was treated entirely within our core healthcare system, one was referred from another Partners Institution (NWH) and one was referred for tertiary care of her lymphoma. Incidence is established as 2 cases of implant-associated ALCL per 9941 patients or 0.02%. Implant-associated disease comprises 3.2% of all ALCL cases and 10% of ALCLs presenting among women. Conclusions: Incidence of breast implant-associated ALCL may be more common than the rare case reports suggest. Evaluation of late complications of breast implant such as chronic seroma or abscess with consideration of this disease may improve case recognition. The fatality as a result of systemic dissemination of this disease has not previously been reported. Disclosures: No relevant conflicts of interest to declare.
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  • 7
    Publication Date: 2009-11-20
    Description: Abstract 1380 Poster Board I-402 Background: The impact of HMG-CoA reductase inhibitors (statins) on cardiovascular mortality is well established. The pleotropic effects of statins extend beyond inhibition of cholesterol synthesis and the mevalonate pathway, including secondary prevention of prenylation and ubiquitination. Independent of the effect on HMG-CoA reductase, increasing evidence suggests that their lactone moiety may independently block the chymotrypsin activity of the proteasome. Proteosome inhibition results in the accumulation of p21 and p27 with subsequent inhibition of cyclin-dependent kinases and G1 arrest. We investigated the impact of incidental statin use on patients with systemic Diffuse Large B Cell Lymphoma (DLBCL). Concurrently, we investigated the effect of statins on p27 expression in DLBCL. Methods: We used an IRB-approved clinicopathologic database, derived from comprehensive tumor registry data at the Massachusetts General Hospital to identify all patients 18 years or older diagnosed with DLBCL between 2000 and 2006 (n=411). Patients were considered eligible for the study if they had a pathology-confirmed diagnosis of systemic DLBCL without evidence of a prior low grade lymphoma, received therapy with curative intent and had routine follow-up care within the system. 178 patients who did not meet these criteria were excluded. Final analysis included 233 patients who were evaluated for statin use via automated review of electronic medial records and billing databases (n=93 with statin use). Immunohistochemical staining for p27 was performed on six existing tissue samples. p27 IHC staining was performed manually using clone 57 (BD Biosciences) at a 1:100 dilution. Two control patient samples could not be assessed for p27 due to high background signal. Samples of two statin patients were compared to two control patients. Results: A total of 233 patients met eligibility and are included in the analysis. Median age was 60 years (range 19-92 years). 50% of patients were above 60 years of age, and 5% had an IPI score of 4 or higher. 74% of patients received CHOP + rituximab (RCHOP). 23% received CHOP alone. 3% recieved R-EPOCH and one received ProMACE-CytaBOM. At a median follow-up of 51 months (range 0-166 months), PFS and OS for the entire cohort are 72% and 77%, respectively. On univariate analysis of outcome, the incidental use of statins did not improve PFS (66.7% vs. 69.3%,p=0.955, Logrank Test) or OS (72.4% vs. 73.6%, p=0.456, Logrank Test). In relapsed patients (n=30) statins prolonged disease free survival (DFS) (p=0.0515). Median DFS was 833 days for statin patients and 384 days for controls. Mean age of the relapsed patients on statins was 65 vs. 54 for controls. Tumor cells showed weak p27 staining in comparison to strong staining in background small lymphocytes in patients treated with statins, while patients not treated with statins showed no staining of tumor cells. Conclusions: Incidental use of statins did not impact PFS or OS in DLBCL. Statins may slow disease progression in patients with relapsed DLBCL. One potential mechanism is upregulation of p27 and G1 cell cycle arrest. Disclosures: Hochberg: Enzon: Consultancy, Speakers Bureau; Biogen-Idec: Speakers Bureau; Genentech: Speakers Bureau; Amgen: Speakers Bureau.
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  • 8
    Publication Date: 2009-11-20
    Description: Abstract 1667 Poster Board I-693 Burkitt lymphoma is a highly aggressive B-cell non-Hodgkin lymphoma (NHL) accounting for nearly 3% of all newly diagnosed NHLs. It is unclear whether the addition of the anti-CD20 monoclonal antibody rituximab to CODOX-M/IVAC chemotherapy improves response rates or outcomes. We used an IRB-approved comprehensive clinicopathologic database to identify all adult Burkitt lymphoma patients treated at our institutions from 12/1/1992 through 2/1/2009 with CODOX-M/IVAC for curative intent. Patients who received CODOX-M/IVAC with rituximab were compared to patients treated with CODOX/M-IVAC without rituximab. Primary endpoints were objective response rate (ORR), overall survival (OS) and progression free survival (PFS). Multivariable Cox regression models of OS and PFS were utilized to provide adjusted treatment comparisons and identify simultaneous significant prognostic factors. Forty patients received R-CODOX-M/R-IVAC, compared to 47 patients treated with CODOX-M/IVAC alone. All patients received white cell growth factor support. The median age was 45 years (17-78), advanced Ann Arbor stage 70%, LDH 〉 upper limit of normal 72%, extra nodal involvement 80% and ECOG PS '2 in 78% of patients. Twelve patients (14%) were low risk defined as a single focus less than 10cm with a normal LDH and 75 (86%) patients were high risk. Fourteen patients (16%) were HIV positive, all of whom received concurrent HAART therapy. Mean CD4 count in HIV positive patients was 237. Seventeen patients (20%) had CNS involvement at diagnosis. Overall response rate was 90% in patients who received rituximab, and 85% in those who did not (P=0.54, Fisher's exact test). Thirty-six patients (90%) achieved complete response (CR) in the R-CODOX-M/R-IVAC group compared to 38 (81%) in the CODOX-M/IVAC group (P=0.37, Fisher's exact test). At a median follow-up among all patients alive and well (n=56) of 30.4 months (range 1.0-127.8), the progression-free (PFS) and overall survival (OS) for the entire cohort are 66% and 68%, respectively. Median follow-up among patients alive and well (n=29) for the R-CODOX-M/R-IVAC group is 23 months. At 23 months, the PFS and OS for rituximab treated patients are 70% and 73%, respectively, compared to CODOX-M/IVAC patients whose 23 month PFS and OS are 61% and 68% respectively. On univariate analysis of both OS and PFS, neither inclusion of rituximab nor HIV status was found to be statistically significant. Patients with high-risk disease, older than 60 years, or CNS involvement had an inferior overall survival on univariate analysis (P
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  • 9
    Publication Date: 2011-11-18
    Description: Abstract 1601 Introduction: Primary Mediastinal B-cell Lymphoma (PMBCL) is an uncommon variant of Diffuse Large B-cell Lymphoma (DLBCL). Given the rarity of this disease, data guiding management is extrapolated from DLBCL trials, or from small retrospective analyses limited to PMBCL. Prospective evaluation of R-CHOP in the MiNT trial showed excellent results in PMBCL, but this trial was limited to young low risk patients. We present the largest retrospective series to date of R-CHOP for PMBCL in all risk groups. Methods: We identified cases of PMBCL at our institution using a comprehensive clinicopathologic database derived from tumor registry data. Natural language processing software was used to search pathology reports for terms of “mediastinal lymphoma,” “mediastinal large cell lymphoma,” “mediastinal large B-cell lymphoma,” as well as “lymphoma” in mediastinal biopsy specimens. Cases were included if they met clinicopathologic criteria for PMBCL, defined as a large B-cell lymphoma with typical features for PMBCL presenting with a dominant anterior mediastinal mass. All patients had to have been treated with R-CHOP. Progression-free survival (PFS) and overall survival (OS) are calculated by the Kaplan-Meier method and univariate analysis is performed to assess predictors of outcome. Results: Fifty-eight cases from 2000–2011 met inclusion criteria and are included in the analysis. The median age was 38 years (range 20–82) and 60% were male. Forty-four patients (76%) presented at limited Ann Arbor stage and 12 patients (21%) at advanced stage; presenting stage could not be discerned in 2 patients. Fifty-five percent of patients presented with mediastinal bulk ≥10cm in size; median size was 11cm (range 5–17cm). LDH was elevated at diagnosis in 60% of patients, normal in 21%, and unknown in 19%. By revised IPI score, 19% were low-risk (0 risk factors), 60% were intermediate risk (1–2 risk factors) and 12% were high-risk (≥3 risk factors). R-CHOP was given for a median of 6 cycles (range 1–8); 51 of 58 patients received 6 or 8 cycles. Among patients who achieved initial remission, 78% underwent consolidative radiotherapy and the remainder were observed after chemotherapy alone. The overall response rate was 81% (90%CI [71%–89%]) with 72% complete responses and 9% partial responses. Ten patients (17%) had primary refractory disease defined as progression on treatment or within 3 months of completion of therapy. Among 46 patients who achieved a response, 5 (11%) subsequently relapsed. Two patients, both elderly, died during treatment. Among the 10 patients with primary refractory disease, 6 have died from progressive lymphoma, 2 patients are alive with active disease undergoing salvage therapy, 1 is alive and free of disease greater than 8 years from diagnosis, and 1 was lost to follow-up. Among 5 patients with relapsed disease, 2 are alive without disease at last follow-up, while 3 have died of progressive lymphoma. Median follow-up for the entire series is 58 months. Five-year PFS is 68% (95% CI, 55% to 80%) and 5-year OS is 76% (95% CI, 65% to 88%). On univariate analysis, advanced Ann Arbor stage and high R-IPI score were associated with inferior PFS and OS. (p=0.006 and p
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  • 10
    Publication Date: 2007-11-16
    Description: FDG-PET scanning has demonstrated utility in the staging and prognostication of Hodgkin lymphoma, but PET characteristics of discrete histologic subsets of Hodgkin lymphoma have never been reported. Further, while studies in this disease have focused on classical Hodgkin lymphoma, the PET features of nodular lymphocyte predominant Hodgkin lymphoma (NLPHL) have not been addressed to date. NLPHL represents less than 5% of all cases of Hodgkin lymphoma and has distinct clinicopathologic features, natural history, and treatment from classical subtypes. Diagnosis may be difficult as it exists in a “gray zone” between Hodgkin and non-Hodgkin lymphomas (NHL), and shares immunophenotypic and histologic features with the T-cell rich variant of diffuse large B-cell lymphoma. Clinically, NLPHL follows a natural history more akin to indolent B-cell NHLs than to classical Hodgkin lymphomas. We retrospectively identified 7 cases of NLPHL diagnosed at our institution since 2003 with available PET staging data. Consistent with prior series of this disease, the majority of patients were male (6:1) and the median age was 32 years. All 7 patients presented with limited stage disease (3 stage I, 4 stage II) and without “B” symptoms. All 7 cases demonstrated increased FDG avidity on PET scanning. The mean Standard Uptake Value (SUV) max was 6.1 (range 1.1–8.8), and mean SUV mean was 5.6 (range 1.0–8.3). We compared these results to recently diagnosed patients with nodular sclerosis and mixed cellularity subtypes of classical Hodgkin lymphoma. Among 13 patients with nodular sclerosis Hodgkin lymphoma (NSHL), the mean SUV max was 10.1 (range 4.8–15.8), while among the 7 patients with mixed cellularity histology (MCHL), the mean SUV max was 5.6 (range 4.0–9.8). Patients with NLPHL had significantly decreased SUV max compared to NSHL patients (p=0.022), but were similar to patients with MCHL (Figure 1). Mixed cellularity histology similarly demonstrated significantly decreased SUV max compared to patients with nodular sclerosing disease (p=0.005). PET intensity therefore highlights differences between classical subtypes of Hodgkin lymphoma, as well as between classical and non-classical variants. When compared to published PET SUV data in aggressive B-cell lymphomas, the maximal SUVs we observe in NLPHL fall well below those reported in aggressive B-cell lymphomas, which may aid in the diagnosis of these occasionally similar appearing neoplasms. We conclude that PET scans identify differences in FDG avidity across classical and non-classical Hodgkin lymphoma subtypes, likely reflecting underlying biological differences. We also demonstrate for the first time that despite its indolent behavior, NLPHL is a uniformly FDG-avid neoplasm, and so PET scans may have a role in the staging and surveillance of this uncommon disease variant. Figure Figure
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