Bruce Patterson

Localized Mucosa-Associated Lymphoid Tissue Lymphoma Treated With Radiation Therapy Has Excellent Clinical Outcome

PURPOSE Extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue (MALT lymphoma) is a distinct lymphoma with unique clinicopathologic features. We report the clinical outcome of stage I and II MALT lymphoma treated with involved field radiation therapy (RT). PATIENTS AND METHODS From 1989 to 2000, 103 patients with stage IE and IIE disease were referred. Their median age was 60 years, with a 2:1 female predominance. Presenting sites were stomach (17 patients), orbital adnexa (31 patients), salivary glands (24 patients), thyroid gland (13 patients), and other sites (18 patients). Ninety-three patients received RT--85 received RT alone, and eight received chemotherapy and RT--with a median dose of 30 Gy. The median follow-up time was 5.1 years. RESULTS A complete response (CR) to RT alone was achieved in 84 of 85 patients. Among CR patients, 14 experienced relapse. Relapse sites were mostly contralateral paired-organ or distant MALT locations and, infrequently, lymph nodes. The crude local control rate with RT was 95.3% (81 of 85 patients). No relapses were observed in patients with stomach or thyroid lymphoma, whereas 14 of 63 patients (22%) experienced relapse in the other sites. The overall 5-year survival rate was 98%, and the disease-free survival rate was 77%. Transformed lymphoma was observed in 14% of patients (two of 14) experiencing relapse. CONCLUSION Moderate-dose RT achieved excellent local control in localized MALT lymphomas and had curative potential for three fourths of the patients. Gastric and thyroid MALT lymphomas had better outcome, whereas distant failures were common for other sites. Despite relapse, the disease often maintained an indolent course.

Prognostic relevance of immunophenotyping in 379 patients with acute myeloid leukemia

We investigated the prognostic relevance of immunophenotype and other clinical pathological features in 379 adult patients with de novo (acute myeloid leukemia) AML diagnosed and treated at our institution during an 8-year period. Acute promyelocytic leukemia (APL) cases were excluded because they received different treatment. The overall complete remission (CR) rate post-induction therapy with Ara-C and daunorubicin (DNR) was 60% with a median disease free survival (DFS) of 72 weeks, and a median overall survival (OS) of 54 weeks. At diagnosis, CD34, deoxynucleotidyl transferase (TdT), CD7, CD56, HLADR and CD19 were expressed in 65, 19, 32, 15, 87 and 5%, respectively, of 379 evaluable cases. CD34 positive patients had a significantly lower CR rate (P=0.0003) than CD34 negative patients and there was a trend to a lower remission rate in HLADR positive patients (P=0.067). In multi-variate analysis, co-expression of CD34 and HLADR was an independent adverse factor for achieving CR (P=0.0364). CD56 expression was associated with a significantly shorter overall survival (P=0.0262), but did not affect remission rate or disease free survival. Neither TdT nor CD7 expression was associated with treatment outcome. Age (60 years or older) and cytogenetic features (classified by favorable, intermediate and unfavorable groups) were associated with a lower CR rate, shorter disease free survival and shorter OS. Patients with higher white cell counts (WBC) also had a significantly lower remission rate (P=0.0064) and OS (P=0.0127). We propose a prognostic score for achieving CR in AML patients based on age, WBC, cytogenetics and CD34/HLADR status as four independent factors. Defined by number of factors, this score system may help to stratify AML patients to alternative treatment for better outcome.

Long-term outcome in stage I and II follicular lymphoma following treatment with involved field radiation therapy alone

6521 Background: Patients with localized follicular lymphoma (FL) are usually treated with radiation therapy (RT). The aim of this study was to assess the long-term overall survival (OS), local disease control and pattern of relapse after involved-field RT in stage I/II FL Methods: 669 patients with stage I and II FL were seen between 1968 and 1999. The records on these patients were systematically collected in an institutional database. The analysis focused on the outcome of treatment in terms of OS, relapse and local disease control in those initially treated with involved-field RT alone (n=460). Median RT dose was 35 Gy (16.0 -47.5 Gy) given over 3-4 wks Results: In 460 patients treated with RT alone, the median age at diagnosis was 59 yrs (17 - 86 yrs); median follow-up duration was 12.5 yrs (range 1.1 -32.2 yrs). The clinical stage was: stage IA: 337 pts, stage IIA: 115 pts, IB: 2 pts and IIB: 5 pts. Histology was: follicular grade 1: 35%, grade 2: 38%, and grade 3: 27%. 75% had nodal disease alone, and 25% presented with extranodal disease. 43% had Karnofsky performance status (KPS) of 100% and 55% KPS of 90%. OS at 5 and 10 yrs were 79% and 62% and DFS 56% and 41%, respectively. Local disease control was excellent; 450 patients achieved complete response and only 5.5% relapsed in radiation fields. Most relapses occurred in distant sites. The cumulative incidence rates for relapse at 5, 10, 15, 20, and 25 yrs were 38%, 48%, 54%, 56%, and 56% respectively. For those at risk of relapse, the probability of relapse between 5 and 10 yrs was only 11%, for 10-15 yrs 6%, and beyond 15 yrs, 2%. CONCLUSIONS Involved-field radiation therapy provides excellent local control in stage I and II follicular lymphomas. Long-term clinical disease control is observed in over 40% of pts. While 30% of all relapses occur after 5 years, risk of very late relapse is low. Long-term follow-up is recommended. No significant financial relationships to disclose.

Outcome analysis of localized gastrointestinal lymphoma treated with surgery and postoperative irradiation.

One hundred thirteen patients with localized gastrointestinal lymphoma treated by surgery and postoperative irradiation between 1967 and 1985 were reviewed. At 15 years, actuarial survival of this group was 40.6%, with a cause-specific survival of 69.2% and a relapse-free rate of 64%. Two-thirds of relapses occurred at distant sites. In Stage IA and IIA patients with no residuum or with positive resection margins, (N = 90) only site of involvement and stage predicted for relapse. Age, histologic subtype group, and depth of bowel wall invasion did not affect relapse risk. In the very favorable group (Stage IA, IIA, no residuum or microscopic residuum), 8.4% of patients with stomach lymphoma relapsed compared to 25% of patients with small bowel lymphoma. The risk of early relapse was higher in those with Stage IIA small bowel lymphoma than those with Stage IA small bowel lymphoma. We continue to recommend adjuvant abdominal irradiation for patients with Stage IA, IIA completely resected stomach lymphoma and Stage IA completely resected small bowel lymphoma. We recommend combined modality therapy for patients with completely resected Stage IIA small bowel lymphoma and all other localized gastrointestinal lymphoma where visible residual disease is present.

Significant increase of CKS1B amplification from monoclonal gammopathy of undetermined significance to multiple myeloma and plasma cell leukaemia as demonstrated by interphase fluorescence in situ hybridisation

The genetic events that lead to tumour progression in plasma cell dyscrasia are not well understood. Interphase cytoplasmic fluorescence in situ hybridisation was used to investigate the CKS1B amplification status (at 1q21) in clonal plasma cells from 123 patients: 23 monoclonal gammopathy of undetermined significance (MGUS), 75 multiple myeloma (MM) and 26 plasma cell leukaemia (PCL). While CKS1B amplification was absent in MGUS patients, such amplification (3–8 copies) was detected in 36% of newly diagnosed MM, 52% relapsed MM and 62% PCL (P < 0·001). Our results suggest that CKS1B amplification is associated with transformation from MGUS to MM and progression to PCL.

Primary Pituitary Lymphoma: A Histological, Immunohistochemical, and Ultrastructural Study with Literature Review

We report the case of a 62-year-old man with headache and left sixth cranial nerve palsy. A computerized tomography scan revealed an osteolytic process involving the sella turcica and clivus. A partial tumor resection was achieved via an endoscopic transsphenoidal approach. Morphologic investigation revealed a diffuse large B cell lymphoma involving pituitary parenchyma. No systemic disease was found upon staging. Primary pituitary lymphoma is extremely rare. An accurate histologic diagnosis is key to successful treatment and a favorable prognosis. The literature is reviewed.