3rd Pannonia Congress of Pathology. Slide seminar / Gastrointestinal pathology: Case Nr. 1. Lukáš Plank

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1 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.

2 Case history

3 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

4 2 blocks - overview

5 the parotis CK7

6 The infiltrate: lobular arrangement, inapparent fibrosis

7 small cells, diffuse, monotonous / uniform

8 The infiltrate: epitheloid cells clusters

9 FH + epitheloid cells + LeL

10 Lymphoid (ccoid) < Pc differentiated cells

11 residual FH + epitheloid cells + Pc diff

12 inside the GC: epitheloid cells + Pc colonisation

13 outside the GC: Pc diff.

14 around the GC: Pc diff. GC

15 No Dutcher bodies, very few Russel bodies

16 ?

17 IHC CD20

18 CD20 PAX5

19 k l

20 l GC

21 l GC

22 IHC MUM1/IRF4 Ki-67 CD138+ CD79a +

23 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+?

24 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

25 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/ / staging BM biopsy following left parotidectomy, - both showing : CD138+ mature Pcs, K+, < 10% (no progression) To hypothesize the clinical evolution?

26 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

27 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

28 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

29 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

30 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

31 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:

32 Encyclopedia of Pathology, Springer 2014

33

34 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,

Case: The patient is a 73 year old woman with vague complaints of dyspepsia and abdominal pain. Upper endoscopy showed features of gastritis and a

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