Reecha Singh

Myelodysplastic syndromes/neoplasms: recent classification system based on World Health Organization Classification of Tumors - international Agency for Research on Cancer for Hematopoietic and Lymphoid Tissues

The myelodysplastic Syndromes (MDS) are a group of clonal hematopoietic stem cell diseases characterized by cytopenia(s), dysplasia in one or more of the major myeloid cell lines, ineffective hematopoiesis, and increased risk of development of acute myeloid leukemia. The classification and the diagnostic criteria have been redefined by the recent World Health Organization Classification of Tumors – International Agency for Research on Cancer for Hematopoietic and Lymphoid Tissues. The myelodysplastic syndromes are now classified into the following categories – refractory cytopenia with unilineage dysplasia, refractory anemia with ring sideroblasts, refractory cytopenia with multilineage dysplasia, refractory anemia with excess blasts, myelodysplastic syndrome associated with isolated del (5q), myelodysplastic syndrome – unclassifiable, and childhood myelodysplastic syndrome. The clinicopathologic features, morphology, differential diagnosis, immunophenotyping, cytogenetics, prognosis and predictive factors are presented in the light of recent World Health Organization Classification of Tumors – International Agency for Research on Cancer.

Xanthogranulomatous Pyelonephritis in a male child with renal vein thrombus extending into the inferior vena cava: a Case Report.

BackgroundWe present a case of Xanthogranulomatous pyelonephritis (XGPN) in a male child with renal vein thrombus extending into the inferior vena cava. This is a rare presentation. XGPN is a rare type of renal infection characterised by granulomatous inflammation with giant cells and foamy histiocytes. The peak incidence is in the sixth to seventh decade with a female predominance. XGPN is rare in children.Case presentationAn 11 year old male child presented with a history of high grade fever and chills, right flank pain and progressive pyuria for two months. He had a history of vesical calculus for which he was operated four years back. In our case, a subcapsular right nephrectomy was performed. The surgical specimens were formalin fixed and paraffin embedded. The sections were stained with routine Hematoxylin & Eosin stain. Grossly; the kidney was enlarged with adherent capsule and thickening of the perinephric tissue. The pelvicalyceal system was dilated and was filled with a cast of pus. Histological evaluation revealed diffuse necrosis of the renal parenchyma and perinephric fat. Neutrophils, plasma cells, sheets of foamy macrophages and occasional multinucleate giant cells were seen. The renal vein was partially occluded by an inflammatory thrombus with fibrin, platelets and mixed inflammatory cells. The thrombus was focally adherent to the vein wall with organization.ConclusionsThe clinical presentation and the macroscopic aspect, together with the histological pattern, the cytological characteristics addressed the diagnosis towards XGPN with a vena caval thrombus. Our case illustrates that the diagnosis of XGPN should be considered even in paediatric age group when renal vein and vena caval thrombi are present.

Basal Cell Adenocarcinoma in the Tongue: An Unusual Presentation

We present a case of basal cell adenocarcinoma (BCAC) in the tongue in a 65-year old male. This is an extremely rare presentation. BCAC generally occurs in the parotid gland and rarely involves the minor salivary glands. Few cases have been reported in literature with a variable presentation. The biopsy was formalin-fixed and paraffin-embedded. The sections were stained with routine Hematoxylin and Eosin. Immunohistochemistry was performed. Hematoxylin and eosin staining showed tumour composed of variable sized and shaped, nests and sheets of basaloid epithelial cells having hyperchromatic to vesicular nuclei. Immunohistochemistry was positive for Pancytokeratin, Epithelial membrane antigen and p53. The clinicopathological features and the cellular immunophenotype addressed the diagnosis towards BCAC of the tongue. The goal of this report is to increase awareness of this rare disease and to review and discuss the differential diagnosis and important considerations in treatment.

Mullerian Adenosarcoma of the Uterus with sarcomatous Overgrowth

Background and objectives Mullerian adenosarcoma is an uncommon variant of mixed mesodermal tumour of the uterus. This is a case report of a 65 year old post-menopausal lady who presented with complaints of passing tissue fragments per vaginum for 2 days followed by spotting. On examination, a polypoid mass protruding through the cervix was seen which was biopsied. Following a preliminary histologic diagnosis of poorly differentiated sarcoma on the biopsy; the patient underwent total abdominal hysterectomy with bilateral salpingo-oopherectomy. Method The surgical specimen was formalin fixed and paraffin embedded. Haematoxylin and eosin stained sections were studied. Result and conclusion Histopathological examination of the polypoid mass revealed a tumour comprising of an admixture of benign endometrial glandular component with overgrowth of sarcomatous stromal component and heterologous elements. This may pose a problem in diagnosis due to its rarity, and hence its distinctive morphological features merit attention as described here. In view of the rarity of this tumor, it is mandatory to do extensive histologic sampling to identify areas of sarcomatous overgrowth before arriving at a diagnosis of mullerian adenosarcoma as the clinical course and management vary.

Intussusception Caused by Yersinia enterocolitica Enterocolitis in a Patient with Sickle Cell Anemia.

Yersinia enterocolitica intussusception is rarely encountered in patients without an underlying susceptibility and is most frequently reported in iron-overloaded patients. This is thought to be related to the unusual use of iron by this microorganism. We present a case of a 5-year old child with intussusception of the terminal ileum caused by Y. enterocolitica whose past medical history was significant for sickle cell disease. This type of presentation is extremely rare. His monthly blood transfusions may have put him at risk for Y. enterocolitica enterocolitis. The pathogenesis of this disease relates to the role of iron as an essential growth factor for Y. enterocolitica, and this patients transfusions left him in an iron overloaded state despite treatment with Deferoxamine. Our patients unusual presentation of intusssuception was secondary to the mass effect caused by lymphoid hyperplasia, specifically hypertrophied Peyers patches in the ileum caused by the Y. enterocolitica infection. We believe that our case demonstrates that Y. enterocolitica should be considered a possible pathogen in patients with sickle cell disease, especially if symptoms occur shortly after blood transfusion.