Essay Example on Lymph Node Sarcomas are very Uncommon Tumours

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INTRODUCTION Lymph node sarcomas are very uncommon tumours arising either from Interdigitating dendritic cells present in the paracortical region or from Follicular dendritic cells present in follicles and rarely from fibroblastic reticular cells ensheathing post capillary venules in lymphnodes Interdigitating dendritic cells arise from hematopoietic stem cell expressing high levels of MHC II and are strongly S100 positive Follicular dendritic cells arise from mesenchymal stem cells and are present in the follicles in the lymph node They present antigen to B lymphocytes and are positive for CD 21 CD23 and CD35 but negative for CD45 Fibroblastic reticular cells are also mesenchymal in origin and are involved in transport of cytokines and other mediators 3 Only nineteen cases have been reported till now thus the case is being reported because of its rarity Case Report 37 year old female presented with mass in left axilla in the Surgery OPD of a tertiary care center Breast examination did not show any lump On ultrasonomammography both breasts were normal Multiple enlarged hypoechoic lymph nodes measuring 40X20mm in left axillary region were present FNAC from outside reported the presence of atypical suspicious cells So excision biopsy for the same was done for histopathological evaluation Grossly a nodular grey white and firm tissue piece measuring 4 X 2 cm in size was received and tissue sections were processed Histopathological examination showed round to oval cells with vesicular nuclei having prominent nucleoli along with mature lymphocytes interspersed amongst which were seen elongated tumour cells with moderate amount of eosinophilic cytoplasm forming fascicles and sheets Histopathologically a diagnosis of Lymphoproliferative disorder Non Hodgkin's Lymphoma was made and IHC was advised Immunohistochemistry was done first using CD45 leukocyte common antigen which showed positive staining for lymphocytes only whereas the other population of cells were negative After this the B cell markers CD20 andPAX5 were used to rule out B cell lymphomas along with the T cell markers CD2 CD3 CD4 and CD8 to rule out Tcell lymphomas

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All of these markers showed negative results CD56 and CD57 were also negative and thus ruled out neuroendocrine tumours Further CD1a CD34 and desmin negativity ruled out Langerhans cell histiocytosis Langerhans cell sarcoma angiosarcomas and rhabdomyosarcoma MPO stain CD 15 and CD 30 negativity ruled out the myeloid origin as well as hodgkins and anaplastic large cell lymphoma As CD1a was negative with focal CD68 positivity possibility of Interdigitating dendritic cell sarcoma was ruled out Then Follicular dendritic cell markers were used CD21 the most sensitive marker for FDCs came out negative but pancytokeratin showed very strong positivity proving epithelial origin of neoplastic cells CK8 and CK18 were also positive Smooth muscle actin SMA showed focal positivity thus showing fibroblastic nature of the tumour So it was diagnosed as Fibroblastic reticulum cell sarcoma

These findings suggested that CIRCs originate from mesenchymal stem cells that show bi directional differentiation towards epithelioid and myoid myofibroblastic cells 3 DISCUSSION Lymph node consists of heterogenous population of stromal cells showing reticular morphology and this group is formed by follicular dendritic cells FDCs interdigitating dendritic cells IDCs Langerhan s cells LCs and fibroblastic reticular cells FRCs FRCs plays a role in maintaining the integrity of lymph nodes production and transport of cytokines and other mediators Franke and Moll in the year 1987 were the first to identify and label these cells as Cytokeratin positive interstitial reticulum cells 2 These interstitial reticulum cells are considered to be indigenous to lymphoid organs originating from mesenchymal stem cells They have long slender cytoplasmic processes extending between lymphocytes and are found in the extrafollicular compartment paracortex and medulla of lymph nodes spleen and tonsils They express cytokeratin 8 and 18 with some of them co expressing smooth muscle actin SMA 20 60 and desmin 1 10 Dendritic cell markers are negative Lymph node sarcomas are very uncommon tumours Arising either from Interdigitating dendritic cells present in the paracortical region or from Follicular dendritic cells present in follicles and rarest from fibroblastic reticular cells ensheathing post capillary venules in lymph nodes Interdigitating dendritic cells arise from hematopoietic stem cell expressing high levels of MHC II and are strongly S100 positive Whereas Follicular dendritic cells arise from mesenchymal stem cells and represent in the follicles in the lymph node They present antigen to B lymphocytes and are positive for CD 21 CD23 and CD35 but negative for CD45 Fibroblastic reticular cells are also mesenchymal in origin and are involved in transport of cytokines and other mediators 3 IDC sarcomas typically have branching and interdigitating processes with an absence of cell junctions 4 5 FDC sarcomas are characterized by interdigitating spindle cells with well developed desmosomes and a lack of cytoplasmic filaments 6 7 8 On the other hand the keratin positive subset of FRC sarcoma can apparently show both desmosomes and tonofilaments along with variable myofibroblastic features and dense bodies 9 Immunophenotypically FDC sarcomas have shown strong uniform positivity for CD21 CD23 and CD35 in nearly all reported cases 6 7 Some investigators have reported expression of CD45 weak B cell markers CD19 CD20 CD22 EMA or CD4 on cases of FDC sarcoma Nodal FRC show uniform positivity for vimentin with subsets positive for actin desmin CD68 and keratin with focal SMA positivity 10

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