1,721,352 research outputs found
[The orodental findings in the Ehlers-Danlos syndrome. A report of 2 clinical cases]
No full textEhlers-Danlos syndrome (EDs) is a hereditary disorder of the connective tissue. Its clinical expression is variable and there are many difficulties in recognizing the disease because of the presence of eleven different forms. In this study the authors describe the oral manifestations in two cases of EDs. The children show hyperelasticity of the skin, easy bruisability, hyperextensibility of the joints, several dental manifestations: periodontitis, many caries, supernumerary teeth or hypodontia
KP1 (CD 68) staining of malignant melanomas
No full textThe monoclonal antibody KP1, which recognizes the CD 68 antigen on macrophages and myeloid precursors, was tested on 28 malignant (primary and metastatic) melanomas, 28 naevi, and 17 skin biopsies showing either normal (10) or hyperplastic melanocytes (7). Sixteen of 20 primary melanomas and six of eight metastatic melanomas showed variable numbers of KP1 positive tumour cells. All but five benign melanocytic proliferations (two Spitz naevi and three intradermal naevi), as well as normal and hyperplastic melanocytes were negative. These results indicate that difficulties may occur with the use of KP1 in the differential diagnosis between melanomas and neoplasms derived from histiocytes-macrophages, and that the expression of CD 68 antigen might be related to tumour progression in melanocytic cells
Follicular dendritic cell sarcoma
No full textFollicular dendritic cells (FDC) are mesenchymal-derived dendritic cells located in B-follicles where they play a pivotal role in triggering and maintaining B-cell adaptive immune response. In 1986 Dr. Juan Rosai first reported a series of neoplasms showing features of FDC and defined it as Follicular Dendritic Cell Tumor, subsequently renamed as “sarcoma” (FDCS). In its seminal and subsequent articles Rosai and colleagues highlighted the heterogeneous microscopic appearance of FDCS and its immunohistochemical and ultrastructural features. FDCS mostly occurs in extranodal sites (79.4% of cases) and lymph nodes (15.1%); in about 7%-10% of cases it is associated with hyaline-vascular Castleman disease. Given its significant growth pattern and cytological variability, FDCS can be confused with various neoplasms and even inflammatory processes. The diagnosis requires the use of a broad spectrum of FDC markers (e.g. CD21, CD23, CD35, clusterin, CXCL13, podoplanin), particularly considering that tumor antigen-loss is frequent. The inflammatory-pseudotumor-like (IPT-like) variant of FDCS, in addition to its peculiar histopathological and clinical features, is characterized by positivity of tumor cells for Epstein-Barr virus, representing a diagnostic requisite. No distinctive genetic and molecular anomalies have been identified in FDCS. It often carries an aberrant clonal karyotype and chromosomal structural alterations, frequently involving onco-suppressor genes. Direct or next generation sequencing showed alterations on genes belonging to the NF-κB regulatory pathway and cell-cycle regulators. In contrast to hematopoietic-derived histiocytic and dendritic cells tumors, FDCS typically lacks mutations in genes related to the MAPK pathway. FDCS recurs locally in 28% and metastasizes in 27% of cases. Extent of the disease, surgical resectability and histopathological features are significantly associated with the outcome. IPT-like FDCS behaves as an indolent tumor, even if it often recurs locally over years. Complete surgical excision is the gold standard of treatment. Data on targeted therapies (e.g.: tyrosine kinase inhibitors) or immune checkpoint inhibitors are very limited and responses are variable. A better understanding of the molecular drivers of this tumor may lead to potential new therapeutic strategies
Spindle cell haemangioendothelioma: further evidence against its proposed neoplastic nature
No full textInvolvement of epidermal Langerhans cells in porokeratosis of immunosuppressed renal transplant recipients
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Fine-needle aspiration cytologic findings in a case of lymph node tumor of plasmacytoid monocytes
No full textWe present a unique case of fine-needle aspiration (FNA) from a lymph node with subsequent histologic diagnosis of tumor of plasmacytoid monocytes (PMs). The patient had an associated myeloproliferative disease which terminated into an acute myelomonocytic leukemia. In a 95% ethanol-fixed, hematoxylin-eosin (H&E)-stained smear, the tumor cells appeared monomorphic, medium size, with oval to indented nuclei, finely stippled chromatin, and small nucleoli; the cytoplasm was scanty and slightly eccentric. The cytologic picture suggested some subtypes of small-cell non-Hodgkin's lymphomas or a leukemic infiltration. In the FNA specimen, the cells appeared immunocytochemically negative for some B- and T-cell markers (MB2, L26, LN1, and UCHL1) and strongly positive to KP1, a known histyocyte-macrophage and myeloid marker. The FNA differentiated diagnoses are discussed
The cryoglobulinemic vasculitis
No full textThirty-eight patients with essential mixed cryoglobulinemia (EMC: 16 type II and 22 type III) were evaluated. Almost all patients had clinical manifestations which could be related to a cutaneous and/or visceral vasculitic process. No significant clinical or laboratory differences were found between type II and type III EMC patients. Kidney biopsy performed in 9 patients showed membranoproliferative glomerulonephritis in 6 cases, diffuse proliferative glomerulonephritis in 2 cases and mesangioproliferative glomerulonephritis in one case. Skin biopsy performed in 11 patients showed typical leukocytoclastic vasculitis as the most common finding. A picture consistent with overlapping vasculitis was found in 2 cases (one type II and one type III) and thrombus-like deposits were observed in 3 cases (2 type II and one type III). These thrombotic lesions, not associated with vessel wall necrosis and inflammatory infiltrate, were indistinguishable from those observed in one case of type I cryoglobulinemia. Our data emphasize the complexity of blood vessel involvement in cryoglobulinemia and point out the possible existence of multiple pathogenetic mechanisms
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