13Pathology 1. Partial excision of Left Orbital tumor: Marginal zone lymphoma, most likelyMALT lymphoma (Extra-nodal Marginalzone lymphoma, Indolent lymphoma)2. Left inguinal LN biopsy:lymphoid hyperplasia3. PES with biopsy :ulcer at gastric antrum
20Treatment & Follow-upChemotherapy as low grade (indolent) lymphoma with Cyclophosphamide : 2008/1/11~4/18Partial RemissionLeft vision improves, Neck and axillary LN become not palpable. But bilateral inguinal LNs palpable
35Marginal zone B-cell lymphoma The marginal zone lymphomas are so named because of their involvement of the marginal zone surrounding normal lymphoid follicles.Indolent lymphomaThree subtypes:1. Extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue (MALT) or MALT lymphoma (or Maltoma)2. Splenic marginal zone B-cell lymphoma3. Nodal marginal zone B-cell lymphoma
36MALT lymphoma Extranodal marginal zone B-cell lymphoma May occur in the stomach (most often), orbit, intestine, lung, thyroid, salivary gland, skin, soft tissues, bladder, kidney, and CNS.May present as a new mass, found on routine imaging studies, or be associated with local symptoms.
37MALT lymphomaPathology pattern: infiltration of small lymphocyte that are monoclonal B cell and CD5(-).The majority present with localized stage I or II extranodal disease, involving glandular epithelial tissues of various sites.In some cases, transformation to diffuse large B cell lymphoma (aggressive lymphoma, worse prognosis) occurs, and both diagnoses may be made in the same biopsy.
39MALT lymphomaThese lymphomas can disseminate to other MALT sites, lymph nodes, or marrow in about 30 percent of cases.In a series of 36 pts presenting with non-GI MALT lymphoma, 12 (33%) were found to have gastric involvement at the time of initial workup.Some suggest that routine evaluation of the stomach should be a part of the initial staging workup, and at relapse, of non-GI MALT lymphomasClinical manifestations, pathologic features, and diagnosis of extranodal (MALT) and nodal marginal zone lymphomas. UpToDate
40MALT lymphomaThey often arise within tissues involved by chronic inflammatory disorders of autoimmune or infectious etiology:1. Sjogren syndrome (salivary gland MALT)2. Helicobacter gastritis (gastric MALT)3. Chlamydophila psittaci conjunctivitis (ocularMALT)4. Borelia skin infection (cutaneous MALT)★ This neoplasm may lie on a continuum between reactive lymphoid hyperplasia and full-blown B-cell lymphoma.
41Treatment of MALT Lymphoma Localized disease:Local therapy such as radiation or surgeryMore extensive disease:Single-agent chemotherapyCoexistent diffuse large B cell lymphoma:Combination chemotherapy
42FDG Uptake Varies Among Different Types of Lymphoma ★ Intensity of FDG uptake determined by:HistologyGradeViable tumor cell fractionTumor cell proliferationUp-regulation of glucose meyabolismLocal perfusionPresence of hypoxiaPET Imaging for Response Assessment in Lymphoma: Potential and Limitation. Radio Clin N Am 46(2008)
43FDG Uptake Varies Among Different Types of Lymphoma Indolent lymphoma exhibit lower glucose metabolic activity and hence FDG uptake than the more aggressive ones.Using an SUV of 10 as a cutoff, FDG-PET seperated aggressive from indolent lymphoma with a sensitivity of 71% and a specificity of 81%.There is (sometimes large) heterogeneity between lesions of the same histologic entitiy and sometimes overlap between tumor grades.The Impact of Fluorodeoxyglucose-Positron Emmision Tomography in Primary Staging and Patient management in Lymphoma Patients. Radiol Clin N Am 46(2008)
44FDG Uptake Varies Among Different Types of Lymphoma AggressiveIndolentIndolentIndolentThe Impact of Fluorodeoxyglucose-Positron Emmision Tomography in Primary Staging and Patient management in Lymphoma Patients. Radiol Clin N Am 46(2008)
45Diagnostic accuracy of FDG-PET in patients with MALT lymphoma Overall disease detection sensitivity:In 5 studies (132 pts),54.4%(18/33) to 81%(21/26, 34/42)Site dependent:gastric MALT : 38.9%1 & 60%2non-gastric MALT : 75%1 & 88%2Grade dependent:early stage disease (I-II): 42.3%1 & 79%2advanced disease (stage III-IV): 100% 1,2Diagnostic accuracy of PET/CT in patients with extranodal marginal zone MALT lymphoma .Eur J Haematol Sep; 79(3): Epub 2007 Jul 27FDG-PET scanning for detection and staging of extranodal marginal zone lymphomas of the MALT type: a report of 42 cases. Annals of Oncology 16: 473–480, 2005
46Diagnostic accuracy of FDG-PET in patients with MALT lymphoma Large cell transformation3:Non-transformed SUV: 3.7 (SD 1.4)Transfromed SUV: 11.3 (SD 5.5)3. Role of Fluorine-18 Fluoro-Deoxyglucose Positron Emission Tomography Scan in the Evaluation and Follow-Up of Patients With Low-Grade Lymphomas. CANCER July 1, 2006 / Volume 107 / Number 1
47Usefulness of FDG-PET in low grade lymphomas F/U of MALT lymphoma: specificity PET<CT 3 False positivie: one gastritis, two beingn lung processRole of Fluorine-18 Fluoro-Deoxyglucose Positron Emission Tomography Scan in the Evaluation and Follow-Up of Patients With Low-Grade Lymphomas. CANCER July 1, 2006 / Volume 107 / Number 1
48Large cell transformation (LCT) Some of indolent lymphomas (about 3% per year) will undergo large cell transformation (histologic transformation) during the course of the disease, an event that dictates a different management strategy and alters survival signicantly.Although LCT can be suspected on clinical grounds alone, it may also go unnoticed or may be difficult to prove.Role of Fluorine-18 Fluoro-Deoxyglucose Positron Emission Tomography Scan in the Evaluation and Follow-Up of Patients With Low-Grade Lymphomas. CANCER July 1, 2006 / Volume 107 / Number 1
50Large cell transformation (LCT) Significant difference between FDG uptake of nontransformed and transformed lymphomas.When during the course of an otherwise indolent disease, there are FDG avid foci with much higher uptake than noted on the baseline study, suspicion should be raised regarding LCT.Role of Fluorine-18 Fluoro-Deoxyglucose Positron Emission Tomography Scan in the Evaluation and Follow-Up of Patients With Low-Grade Lymphomas. CANCER July 1, 2006 / Volume 107 / Number 1
51FDG-PET v.s. Bone Marrow Biopsy Patients of Lymphoma in SKH533245Low grade lymphomaHigh grade lymphoma
5267y,M, Malignant lymphoma, small lymphocytic type, PET bone- : Bone, iliac crest, side not specified, biopsy--- Malignant lymphoma with marrow involvement, B cell:Lymph node, left neck, excisional biopsy--- Malignant lymphoma, small lymphocytic type67y,M, Malignant lymphoma, small lymphocytic type, PET bone-
53ConclusionPET usefulness in staging low-grade(indolent) lymphomas varies depending on histology.PET sensitivity is excellent in follicular lymphoma and moderate in marginal zone lymphoma.Detectability of extra-nodal marginzal zone lymphoma (MALT lymphoma) is site and grade dependent:★Non-gastric > Gastric★Advanced > Early
54ConclusionPET is more specific than CT for follow-up in follicular lymphoma, marginal zone lymphoma, and B-cell small-cell lymphocytic lymphoma (SLL/CLL) .PET has limited usefulness for B-cell small-cell lymphocytic lymphoma (SLL/CLL) staging.
55ConclusionIn low-grade lymphomas, the emergence of foci of intense uptake should raise suspicion of conversion to high-grade disease.FDG-PET scan may become the test of choice for early detection of LCT and/or selection of the optimal biopsy site when transformation is suspected.
56ConclusionFDG-PET cannnot substitute for bone marrow biopsy, and is less sensitive in detecting bone marrow involvement of low grade(indolent) lymphoma.