Rosai-Dorfman disease of the central nervous system.

Rosai-Dorfman disease (RDD) is an idiopathic, non-neoplastic, lymphoproliferative disorder characterized by sinus histiocytosis and massive lymphadenopathy. When RDD involves the central nervous system the lesion simulates a meningioma. Histological and immunohistochemical confirmation is essential for a definitive diagnosis. In this paper, ten cases of RDD confined to the central nervous system are reported. Another case with orbital RDD was excluded. Nine cases involved the cranial cavity alone; in one, the cervical extradural region was also involved. Treatment consisted of surgical excision or biopsy. Histology and immunohistochemistry revealed a mixed cell population of predominantly mature histiocytes with evidence of emperipolesis and strong positivity for S100 protein in all cases. No recurrence was observed during follow up ranging from three months to eight years.

Desmoplastic infantile ganglioglioma -- a case report.

Desmoplastic infantile ganglioglioma is a very rare supratentorial tumor occurring in the first two years of life. A five-month-old female infant presented with recurrent seizures, large head and loss of acquired milestones. Computerized Tomographic Scan of brain showed a large subarachnoid cyst with a solid intensely contrast enhancing tumor in the right temporoparietal region with severe degree of mass effect. Craniotomy and total excision of the tumor followed subsequently by subduro-peritoneal shunt for the extracerebral fluid collection was done. The child made good recovery. Histopathology revealed features of desmoplastic infantile ganglioglioma, viz., marked desmoplastic component with glial and neuronal elements. Immunohistochemistry showed positive staining for glial fibrillary acidic protein (GFAP) with areas of synaptophysin and chromogranin positivity. Desmoplastic infantile ganglioglioma is a rare tumor of infancy, which has excellent prognosis after total excision. No adjuvant therapy is required. This is the first Indian report of desmoplastic infantile ganglioglioma out of less than fifty cases reported worldwide.

Persistent Müllerian Duct Syndrome (PMDS) with testicular seminoma.

Persistent Mullerian Duct Syndrome (PMDS) is characteristically associated with unilateral or bilateral cryptoorchidism. Like other undescended testis, these gonads are at an increased risk of malignant transformation. We report a case of intra abdominal seminoma in cryptorchid testis of a patient with the Persistent Mullerian Duct Syndrome, hitherto uncommonly reported in India.

Immunohistochemical profile of breast cancer patients at a tertiary care hospital in South India.

AIMS: 1) To evaluate the estrogen receptor(ER), progesterone receptor (PR) and Her-2 /neu expression in invasive breast carcinomas by immunohistochemistry and 2) to compare the pattern of expression with clinico-pathological parameters like patient's age, tumor size, mitotic index, histological type and grade and lymph node metastasis. METHODS: This is a retrospective study of 321 female invasive breast carcinomas diagnosed in the Department of Histopathology, Apollo Speciality Hospital, Chennai from January 2009 to June 2010. RESULTS: The age of the patients ranged from 24 to 99 years, with a mean of 53.8, and the majority of the tumors were T2 (83.8% in range of 2-5 cms), predominantly histological grade 2 (57.3%), followed by grade 3 (33.3%). ER, PR and Her-2/neu expression was seen in 59, 51 and 27% of cases respectively. Triple-negative breast cancers constituted 25 % of our cases. We also found characteristic associations between hormonal receptor and Her-2/neu expression and various clinico-pathological parameters. CONCLUSIONS: The hormonal receptor expression appears to be lower in the Indian population compared to the West. A significant proportion of tumors in our study with Her2/neu overexpression also showed ER and PR positivity. Triple-negative breast tumors were most commonly grade 3, in women aged more than 50 years.

Practical value of MIB-1 index in predicting behavior of astrocytomas.

BACKGROUND: The MIB-1 labeling index (LI) has proved to be useful in assigning grading and prognosis to astrocytomas. The purpose of our study was to analyze the utility of MIB-1 LI in differentiating astrocytomas of varying grades and the possible relationships of MIB-1 LI with clinical parameters like age and sex. We also wanted to study the prognostic role of MIB-1 index in predicting behavior of astrocytomas. MATERIALS AND METHODS: Our study included 145 patients with astrocytic tumors of varying grades. Immunolabeling for all patients was done using MIB-1 antibody. Survival data could be obtained for 64 patients. A Mann-Whitney U test was used to test the difference in MIB-1 LI between different histological grades. The univariate analysis was done by the Kaplan-Meier method, and the multivariate analysis for survival was performed using the Cox proportional hazard model. RESULTS: Significant differences were noted in mean MIB-1 LI of high-grade and low-grade diffuse astrocytomas. MIB-1 LI did not vary significantly with age and sex. Univariate analysis showed favorable prognostic factors for low histopathological grade, young patient age and low MIB-1 LI; however, multivariate analysis showed that only histopathological grade had independent prognostic significance. CONCLUSIONS: Our study proves that MIB-1 LI is not dependent on factors like age and sex and is solely dependent on histological grade. Though the average level of MIB-1 LI varies considerably in the different grades of astrocytomas, considerable overlap can be observed between them. MIB-1 LI is a very useful adjunct to the histopathological diagnosis and can be of great help in situations where the clinical and radiological findings do not correlate with histological diagnosis.

Extranodal testicular anaplastic versus plasmablastic plasma cell tumor: A rare case with diagnostic dilemma in a developing country.

Primary testicular plasmacytoma is rare, especially when occurring in the absence of a previous or concurrent diagnosis of multiple myeloma. An 84-year-old gentleman was admitted with complaints of swelling over the right side of scrotum for the past 2 months. Local examination revealed a 10×15 cm tense and tender swelling involving the right scrotum. Therefore, high orchidectomy with excision of hemiscrotum was done. The histopathology revealed testicular plasmacytoma, which was positive for CD 138.

The role of immunohistochemistry in predicting behavior of astrocytic tumors.

The purpose of this study was to analyze the significance of p53, bcl-2 and EGFR expression in the grading and biological behavior of astrocytic tumors, especially in the Indian population. A total of 117 cases of astrocytomas graded using the WHO grading system published in 2007 were immunolabeled using p53, EGFR and bcl-2 monoclonal antibodies and analyzed with respect to grade and other relevant parameters. The 117 cases included 16 cases of pilocytic astrocytomas and 25, 15 and 61 cases of diffuse fibrillary astrocytomas WHO grade II, anaplastic astrocytomas WHO grade III and glioblastomas (GBM), respectively. Our results showed that p53 alterations is an early event in astrocytic gliomagenesis, but is not significant in the evolution of pilocytic astrocytomas. Bcl-2 expression did not correlate with grade and no statistical correlation was seen with p53 expression. EGFR protein expression correlated with the severity of tumor grade. Of the GBM cases, 47.5% were p53 positive only, 18% were EGFR positive only, 16.5% were negative for both and 18% were positive for both. The mean age in the dual positive category was significantly higher when compared to the others. EGFR and p53 alterations are not mutually exclusive and might act synergistically to promote progression. We also noted a significantly higher p53 expression in females in GBMs. Though most of our findings correlated with those of previous studies, some differences were noted, especially in the pattern of immunoexpression in GBMs, perhaps because of ethnicity.

Inguinal pick in invasive penile carcinoma: can it stage node negative patients?

In node negative patients of invasive penile carcinoma, prophylactic lymphadenectomy is associated with considerable morbidity, while a "wait and watch" policy is associated with up to 20% cancer related mortality in many series. In between, selective node biopsies, including sentinel node biopsies, have been suggested to stage these patients. However, these procedures are unreliable and associated with high false negative rates. The inguinal pick procedure was devised by us to stage these patients more accurately. It is more elaborate that the previously described selective biopsies and includes biopsy of all identifiable nodes in the inguinal region, including the sentinel node area. In our experience of the procedure in 52 patients with invasive penile carcinoma, it was positive in 5 patients (9.6%). However, 7 of the 47 patients with negative result developed inguinal recurrences and 3 other patients developed distant metastases on follow up. The 5-year disease free survival of inguinal pick positive and negative patients was 100% and 82.9%, respectively. The sensitivity of the procedure in detecting regional spread in these patients was only 72%. Thus, the inguinal pick, though associated with no morbidity, can be meaningful only if it is positive and a negative result does not guarantee absence of regional metastases in node negative patients of invasive penile carcinoma.

Rhinosporidiosis mimicking penile malignancy.

Rhinosporidiosis is a chronic infection which is endemic in India and Sri Lanka. Penile involvement in rhinosporidiosis is rare and we report one such case. The pathology, treatment and possible etiology of the condition are reviewed.

Adenocarcinoma of the urachus.

During the past 5 years, three patients with adenocarcinoma of the urachus were treated at our hospital. Abdominal ultrasonography helped clinch the diagnosis in all cases. All patients underwent surgical excision of the neoplasm with partial cystectomy. The clinical, radiological, and histological findings, as well as the pathogenesis of this rare neoplasm are discussed along with the surgical management.