55 yrs old male with lateral LINGUAL radix tumor and head and neck lymphadenopathy ("indurated salivary gland zone") one-sided ipsilaterally.  
Consultation case: original diagnosis NK/T NHL?  
No signs of acute leukaemia or blastemia in PB/flow.  
 
HISTO: "blastoid" and "histiocytoid" diffuse infiltration with reniform ovoid nuclei, eosinophilic cytoplasm. Focal BINARY pattern with clustered large eosinophilic cells (more positive CD1a and Langerin).  
IH FULL: HLA DR+; CD4+; Lysozyme+; CD56+; S100+; CD11c+; LCA+(silpnas); Pax5+ (silpnas); CD1a+/-; Langerin(-/+); Bcl6+; CD3/CD7/CD5/CD2/GranzymB/TIA1/Perforin/CD8/CD30(-); CD20/CD79a(-); CD138/CD38/Mum1(-); Ig kappa/lambda/IgM/IgD/IgA(-); CD68/CD123/CD163/CD14(-); MPO/CD117/CD34/Bcl2/TdT(-); CD23/CD21/CD35(-); EBER/HHV8(-); Ki67 prolif. index high 95%.  
 
Molecs: IgH and IgK clonal; BRAF no mutations in Ex15; NO DEFINITE AML mutations;  
SNP array: COMPLEX HYPERDIPLOID KARYOTYPE: +5, +8, +21 TRISOMIES, 7q, 12q, 9p DELETIONS ir 17q DUPLICATION.  
NOTE: NOT TYPICAL FOR AML/MDS or ALL. Despite HYPERDIPLOIDY, trisomies not typical for ALL. 7q deletion TYPICAL for AML, but, one specific ALL aberration was detected too: CDKN2AB homozygous deletion(9p del). GRAPH IS APPENDED BELOW.  
 
E-microscopy: NO definite Birbecks.  
Bone marrow: reactive follicular hyperplasia, no AML signs (will be appended afterwards).  
 
DIAGNOSTIC VERSION 1 WAS (at first look): Myeloid sarcoma (mono-differenctiation), but S100+ CD68/CD163(-) and B clone, no AML elsewhere, local not-life-threatening process.  
so DIAGNOSTIC VERSION 2 IS: Aggressive histiocytic tumor, probably Langerhans or indetermined cell sarcoma.  
DIAGNOSTIC VERSION 3: Combination of both above.  
 
ThankYou2018.  
 
NOTABENE: our similar published case with dr.Alex: APPENDED BELLOW: Unique composite hematolymphoid tumor consisting of a pro-T lymphoblastic lymphoma and an indeterminate dendritic cell tumor: evidence for divergent common progenitor cell differentiation.  
Buser L, Bihl M, Rufle A, Mickys U, Tavoriene I, Griskevicius L, Tzankov A.  
Pathobiology. 2014;81(4):199-205. doi: 10.1159/000365396. PMID: 25228298

tzankov 2018-12-03 07:38	would be so kind to add TCL1 and/or CD303?   would you be kind to ask the radiologists on the extension of the disease (size/destrutiveness) and the clinicians on whether there are skin lesions?   I think it is a dendritic tumor
ugnius 2018-12-03 07:56	Thanx. Unfortunatelly I still lack of 2 IH mentioned. SURGERY after PET scan will be performed (if hopefully local) and we will proceed later on...
ugnius 2018-12-03 13:16	BM added.
Juskevicius 2018-12-03 17:49	Although the morphology and S100 are suggestive of Langerhans tumor the phenotype is a unusual for mature Langerhans cells. The phenotype with no expression of CD123 and the presence of Lysozyme is also unusual for BPDCN (blastic plasmacytoid dendritic cell neoplasm). I agree with your first impression that this may be monocytic myeloid sarcoma, which at this point is localized and precedes the overt AML, which does happen. Karyotype/ FISH (especially for the core binding factor translocations) would be interesting. Have you done those?
ugnius 2018-12-04 08:48	Thanx. I have only paraffin now and no applicable FISHes for this. Ar resection moment fresh material appears and probably smth from NGS panel will be applied (outside my centre)(but if MONO- usually "silent" on AML mutation panel).   Still awaiting e-microscopy fun (quality from paraffin seems to be good).
Andrulis 2018-12-05 15:15	A difficult case. What sense makes SURGERY in this non-solid Tumor?
ugnius 2018-12-05 15:24	If local why not(?). BETTER (hopefully) DIAGNOSTICS and FRESH for molecs. Finally DECISSION not mine...
tzankov 2018-12-07 10:38	well, there is space for sugery in dendritic tumors, at least for IDSC/FDC and HS, R0 resections - if possible - are one of the very few curative options
ugnius 2018-12-13 15:52	Please find e-microscopy. A mass seems to be "blastic" ones with admixture of histiocytic ones. Plasma cells enriched with endoreticulum, some crystals, a lot af artifacts/vacuoles and SINGLE finding slightly similar to Birbecks... MORE EXPERIENCED "eyeballing" would be of value... Thanx.
tzankov 2018-12-17 18:18	well, I think at the moment the best working hypothesis would be that of an undetermined (immature/blastoid) dendritic cell sarcoma. has the surgical excision been performed?
ugnius 2018-12-18 09:16	PET is awaiting for staging. Be informed ASAP.
ugnius 2018-12-27 11:06	NEWS: Liver -ases (AST/ALT/gama) and bilirubinemija becooming high. Multiple focal lesions in liver and spleen and specific lymphadenopathy (abdominal) with little ascitis apear. PET awaiting, but seems facing with progression and no surgery will be applied...
tzankov 2019-01-02 07:59	behaves like a dendritic (langerhans?) sarcoma
ugnius 2019-01-09 16:52	The patient passed away under dexa/cyclophosphamide, antibiotics sue to Sepsis/multiple organ dysfunction s. Maybe NGS report will be issued later on. So it was NOT SO LOCAL as seems at first glance.
ugnius 2019-01-09 16:52	No definite AML mutations.
ugnius 2019-01-12 14:34	LAST POINT:   SNP array: COMPLEX HYPERDIPLOID KARYOTYPE: +5, +8, +21 TRISOMIES, 7q, 12q, 9p DELETIONS ir 17q DUPLICATION.   NOTE: NOT TYPICAL FOR AML/MDS or ALL. Despite HYPERDIPLOIDY, trosimes not typical for ALL. 7q deletion TYPICAL for AML, but, one specific ALL aberration was detected too: CDKN2AB homozygous deletion(9p del).
tzankov 2019-01-17 18:21	fits with the proposed diagnosis
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