3rd Pannonia Congress of Pathology Bled, May 16, 2014 Slide seminar / Gastrointestinal pathology: Case Nr. 1. Lukáš Plank National Consultation Center for Haematopathology in Slovakia: Department of Pathology, Jessenius Faculty of Medicine, Comenius University & University Hospital and Martin s Biopsy Center, Ltd.
Case history
Case history Data given by clinician to pathologist in the actual time of the biopsy: 76 years old woman, enlargment of the left parotid gland tumor of the parotid gland Macroscopic appearance: ovoid excision yelowish-gray colour on the cut surface, max. 2 cm in diameter Consultation case from Dept. Pathol., Faculty Hospital Nitra
2 blocks - overview
the parotis CK7
The infiltrate: lobular arrangement, inapparent fibrosis
small cells, diffuse, monotonous / uniform
The infiltrate: epitheloid cells clusters
FH + epitheloid cells + LeL
Lymphoid (ccoid) < Pc differentiated cells
residual FH + epitheloid cells + Pc diff
inside the GC: epitheloid cells + Pc colonisation
outside the GC: Pc diff.
around the GC: Pc diff. GC
No Dutcher bodies, very few Russel bodies
?
IHC CD20
CD20 PAX5
k l
l GC
l GC
IHC MUM1/IRF4 Ki-67 CD138+ CD79a +
summary small B-cell predominant PC diff. NHL showing phenotype: CD20-, PAX5-, CD138+, CD79a+, MUM1+, l+, very low Ki-67 index Diff. Dx: small B-cell ML - marginal zone B-cell lymphoma of MALT-type, with massive /predominant Pc L+ differentiation, CD20 negat., PAX 5 negat. versus extraosseal plasmacytoma, G1 / L+?
MZBL of MALT-lymphoma type, extreme Pc diff., - patterns : predominant PC differentiated B-cell clonal ML: CD20-, PAX5-, CD138+, CD79a+, MUM1+, l+, very low Ki-67 index intermingled small cell Ly population + areas of residual FH: CD20+, PAX5+ and colonisation of bcl2 negative GCs isolated dispersed CD30+ B-(imuno-)blasts (+ CD3+/CD5+ T-cells) lymphoepithelial lesions present, - absence of classical LESA pattern
Ex post: the whole history of the patient 1998: RA, on treatment since 2003, incl. MTX + supportive care 2005: MGUS K+ diagnosed 02/2014: Tu of gl. parotis bilateral, however, classic Sjögren syndrome not present 2 BM biopsies - 06/2009-03/2014 - staging BM biopsy following left parotidectomy, - both showing : CD138+ mature Pcs, K+, < 10% (no progression) To hypothesize the clinical evolution?
MGUS (monoclonal gammopathy of undetermined significance) MGUS possible consequences and manifestations Amyloidosis MIDD organ damage CD138 smoldering myeloma active myeloma other NHL type organ damage k
MGUS (monoclonal gammopathy of undetermined significance) MGUS precursor lesion of myeloma however, it is more complicated... 3 MGUS types precursor lesion of non IgM MGUS (IgG, IgA) plasmacytic myeloma IgM MGUS lymphoplasmacytic lymphoma, M. Waldenström, other NHL with Pcoid differentiation light chain MGUS
salivary gland MALT-lymphoma pathogenesis salivary gland: physiologically no LT primary Ss secondary Ss: autoimmune SCTDs, e.g. RA, scleroderma, SLE, etc. acquired MALT: result of chronic inflammat. (autoimmune) disease
salivary gland MALT-lymphoma pathogenesis salivary gland: physiologically no LT primary Ss secondary Ss: autoimmune SCTDs, e.g. RA, scleroderma, SLE, etc. acquired MALT: result of chronic inflammat. (autoimmune) disease acquired MALT with LESA
salivary gland MALT-lymphoma pathogenesis salivary gland: physiologically no LT primary Ss secondary Ss: autoimmune SCTDs, e.g. RA, scleroderma, SLE, etc. acquired MALT: result of chronic inflammat. (autoimmune) disease acquired MALT with LESA MZBL of MALTtype multi-step process: - long-term stimulation of activating B-cells, - monocl. B-cell proliferation and expansion, - additional oncogenic events TP53, chr. translocations, etc. = onset of ML
Presented case - possible pathogenesis 1998: autoimmune SCTD of RA type + Tx (MTX)? 2005 MGUS, K+ 2014: MZBL of MALT-type, L+, with extreme Pc differentiation: loss of the B-cell diff. Ags gain of PCs diff. Ags 2014: MGUS, K+, No progression Chronic sialadenitis acquired MALT with LESA:
Encyclopedia of Pathology, Springer 2014
Thanks to my co-workers and for the attention Lymphoma Dx = puzzle Biopsy diagnosis: P. Szépe, T. Balhárek, J. Marcinek, J. Mičák, P. Vašeková IHC: Z. Kviatkovská, J. Cáliková, V. Šichtová FISH: T. Balhárek, A. Farkašová, M. Barthová, Ľ. Janáková,