Ram Narayan Das

Primitive neuroectodermal tumor of adrenal: clinical presentation and outcomes.

Primitive neuroectodermal tumor (PNET) of adrenal is an extremely rare tumor of neural crest origin. A nonfunctional left adrenal mass (14.6 × 10.5 × 10.0 cm) on computed tomography (CT) was detected in a 40-year-old lady with abdominal pain, swelling, and left pleural effusion. She underwent left adrenalectomy and left nephrectomy with retroperitoneal resection. Histopathology revealed sheets and nest of oval tumor cells with hyperchromatic nuclei, prominent nucleoli, scanty cytoplasm, brisk mitotic activity, necrosis, lymphovascular invasion, capsular invasion, and extension to the surrounding muscles; staining positive for Mic-2 (CD-99 antigen), vimentin, synaptophysin, and Melan-A. Thoracocentesis, pleural fluid study, and pleural biopsy did not show metastasis. She responded well to vincristine, adriamycin, and cyclophosphamide followed by ifosfamide and etoposide (IE). This is the first report of adrenal peripheral PNET (pPNET) from India. This report intends to highlight that pPNET should be suspected in a patient presenting with huge nonfunctional adrenal mass which may be confused with adrenocortical carcinoma.

Cytokeratin 19 immunoreactivity in the diagnosis of papillary thyroid carcinoma

Context: The diagnosis of papillary thyroid carcinoma (PTC) is based on nuclear features. These features may be present in focal areas in benign thyroid diseases and follicular adenoma (FA), leading to diagnostic difficulty. Aims: To evaluate the expression and pattern of the distribution of cytokeratin 19 (CK19) in PTC and compare its reactivity with other neoplastic and non-neoplastic conditions to assess its potential as a useful marker for PTC. Materials and Methods: Twenty two cases of papillary carcinoma (usual type, follicular and diffuse sclerosing variant), eight follicular adenomas, eight multinodular goiters (MNG) were collected for a period of two years and six months. Sections were taken from thyroidectomy specimens fixed in 10% buffered neutral formalin. Hematoxylin and eosin staining and immunohistochemical staining for CK19 were done using standard protocol. Results were semiquantitatively scored as follows: 1+ (<5% positively stained cells), 2+ (5-25%), 3+ (25-75%) and 4+ (>75%), and then analyzed. Statistical Analysis and Results: All 22 (100%) papillary carcinomas showed diffuse and strong (3+ and 4+) CK19 expression. Six out of eight (75%) FAs and four out of eight (50%) MNG were positive for CK19, but it was of weaker intensity (1+ and 2+) and focal in distribution. Conclusion: Focal CK19 staining may be found in benign disease, but diffuse and strong positivity is characteristic of PTC, which can be used in the diagnosis of PTC in lesions of equivocal morphological appearances.

Collecting duct carcinoma: A rare malignancy

A 45-years old man complained of hematuria, and subsequent examination and ultrasonography revealed a mass in the left kidney. Nephrectomy was performed and macroscopically an ill-defined pale-cream, irregular mass was identified which occupied predominantly the renal medulla. Histopathologic examination showed slit like tubular ducts lined by atypical cuboidal to polygonal cells and a marked desmoplastic stromal reaction. The diagnosis of collecting duct carcinoma was made. Patient is now doing well after 11 months of follow up.

Study of histopathological features and proliferation markers in cases of Wilms' tumor

Context: The spectrum of pediatric renal tumors is different from adult renal tumors, and Wilms’ tumor (WT) forms the majority. The histological type and clinicopathological staging are the two important prognostic parameters. The role of newer prognostic factors is not clear. Aims: This study was performed to analyze the histopathological spectrum of pediatric renal tumors and to study the expression of proliferation markers (Ki-67 and p53) in WT and correlate its expression in epithelial and blastema components in different stages. Materials and Methods: Twenty-seven cases of pediatric renal tumors were collected over 2 years. Hematoxylin-eosin staining was used for diagnosis. Immunostaining was performed for Ki-67 and p53. Ki-67 proliferation index (PI) and p53 expression were determined in each case and for the epithelial and blastema components separately. Statistical Analysis and Results: We had 20 cases of WT (74.1%), three cases of mesoblastic nephroma (11.1%), three cases of clear cell sarcoma (11.1%) and one case of rhabdoid tumor (3.7%). It was observed that the PI of the epithelial component (57.2%) was significantly higher than that of blastema (39.53%) in all stages. The PI in Stage II is significantly higher than that in Stage I. Statistical analysis could not be performed in Stages III and IV due to the small number of cases. p53 expression did not show any significant difference in the epithelial and blastema components. There was also no significant difference between the stages. Conclusion: In this study, we found the differences between PI of different tissue components of WT, with the epithelial component having a higher PI, which correlated with the stage of advancement of the disease.

Primary Renal Rhabdomyosarcoma: An Unusual Bone Metastasizing Tumor of Kidney

ABSTRACT Rhabdomyosarcoma (RMS) is one of the common malignant tumors in the pediatric age group. There is only a single case report of primary renal alveolar RMS. Fine needle aspiration (FNA) findings of primary renal RMS has not been reported so far. Hence we present an unusual case of primary alveolar RMS of the kidney. An 11 year old boy presented with an abdominal mass. On FNA a diagnosis of undifferentiated sarcoma and anaplastic Wilms tumor were considered. The tumor was resected and showed histopathological features of alveolar rhabdomyosarcoma. He developed multiple bony metastases and succumbed to the illness despite aggressive chemotherapy. RMS of the kidney should be considered in the differential diagnosis of children with a renal mass, and may have an aggressive clinical course with bone metastases.

Lipoblastoma and lipoblastomatosis: A clinicopathological study of six cases

Lipoblastoma and lipoblastomatosis are benign adipocytic tumors seen in children less than 3 years of age. Awareness amongst surgeons of this fast growing lipomatous tumor in young children is important to avoid mutilating surgery and chemotherapy. Histological diagnosis of lipoblastoma is sometimes difficult because of close resemblance with myxoid, round cell, and well-differentiated liposarcoma. We came across a series of six cases of lipoblastoma/lipoblastomatosis, over the last 10 years (2002-2012). Of these, we had three cases of lipoblastomatosis, one of which recurred after 2 months. All others were resected completely and successfully.

Diffuse large B-cell lymphoma of the kidney: a rare neoplasm.

Primary renal lymphoma is a rare neoplasm, but it should be kept in mind in the differential diagnosis of renal neoplasms. A middle aged man presented with symptoms of weight loss, anorexia and fullness of the abdomen after meals. On clinical and radiological examination, a renal mass was revealed and operated upon. A diagnosis of primary high grade renal lymphoma was made on histopathological examination and immunohistochemically it was further classified as diffuse large B-cell lymphoma. Unfortunately, the patient died after 5 months of diagnosis in spite of three cycles of chemotherapy following surgery. The pathological details of rare tumor are presented here.

Sirenomelia associated with discoid adrenal and lumbar meningocoele: An autopsy report

Mermaid syndrome or Sirenomelia is a rare congenital deformity in which the legs are fused and bears resemblance to mermaids tail. It carries a poor prognosis, due to associated urogenital and gastrointestinal abnormalities. An early antenatal diagnosis using Magnetic Resonance Imaging (MRI) can help in termination of pregnancy. Embryologically, it is considered as the extreme form of caudal regression syndrome due to the persistence of vitelline artery. Here, we report a case of Sirenomelia associated with bilateral renal agenesis along with the rare findings of discoid adrenal, lumbar meningocoele and abnormalities of the hand.

FNAC Aided Diagnosis and Categorization of Hepatoblastoma:: A Report of Three Cases.

Hepatoblastoma is the most common primary malignant hepatic tumour of infancy and early childhood. Histologically hepatoblastomas are categorized into pure epithelial and mixed epithelial‐mesenchymal types and epithelial type is further subcategorized into pure fetal type, fetal and embryonal type, pure embryonal, and small cell types. This categorization has been shown to have prognostic and therapeutic implication. Fine needle aspiration cytology (FNAC) is useful in pre‐operative diagnosis and categorization in most cases of hepatoblastomas. Periodic acid–Schiff (PAS) stain can be helpful to differentiate fetal subtype from embryonal subtype of hepatoblastoma. Here we describe three cases of hepatoblastomas diagnosed and categorized on cytology with subsequent confirmation on histological examination. Diagn. Cytopathol. 2017;45:77–82.

Cytological diagnosis of mesoblastic nephroma: A report of three cases with summary of prior published cases.

Congenital mesoblastic nephroma (CMN) is a rare pediatric renal neoplasm, occurring most commonly in the first few months of life, with a favourable clinical outcome. Accurate pre‐operative cytological diagnosis of this entity is important as pre‐operative chemotherapy is not recommended and surgery is the treatment of choice. Cytodiagnosis of this rare tumor is discussed in only a few case reports. Here two cases of CMN and one case of cellular congenital mesoblastic nephroma (CCMN) diagnosed on FNAC along with their morphological differential diagnoses has been reported. They also take this opportunity to compare the cytological features of CMN with cellular CMN. Diagn. Cytopathol. 2016;44:823–827.