Ramesh Dawar

Hodgkin's disease in Indian children: Outcome with chemotherapy alone

To assess the efficacy of chemotherapy alone, using four cycles of COPP alternating with four cycles of ABVD in all stages of childhood Hodgkins disease (HD).

Central nervous system metastases from primary epithelial ovarian cancer.

Epithelial ovarian carcinoma (EOC) is a common gynecologic malignancy in women.1 Approximately two thirds of patients present with advanced disease. Debulking surgery followed by platinum-based chemotherapy is the standard treatment approach, and 15% to 30% of patients achieve long-term survival.2 Relapse of disease in the pelvis, the abdomen, or both is the most common cause of failure.3 The central nervous system (CNS) is a rare site for metastasis. In this article, we report data on 18 such cases seen at our institution during the past 11 years. A systemic review of the English literature is also presented to assist the management of this interesting though still rare clinical problem.

Non-Hodgkin's lymphoma in Northern India. A retrospective analysis of 238 cases

A retrospective analysis was performed of 238 patients, aged 12 years and older, with non‐Hodgkins lymphoma presenting to the All India Institute of Medical Sciences, New Delhi, India, between September 1975 and December 1982. Pathologic material was reviewed and classified according to the modified Rappaport classification. The most common histologic type encountered was diffuse histiocytic lymphoma (39%), followed by diffuse poorly differentiated lymphocytic lymphoma (29%), and diffuse mixed histiocytic and lymphocytic lymphoma (9%). Nodular lymphomas constituted 9% of all non‐Hodgkins lymphomas. A lower frequency of nodular lymphomas, a lower median age of onset (45 years), and a higher male to female ratio (4.5:1) as compared with Western countries was observed. Survival information on 90 patients revealed no effect of age, sex, stage of disease, and “B” symptoms on survival, whereas histologic diagnosis had a significant influence on survival (P <0.05). A median survival of 24 months in 58 patients receiving chemotherapy is comparable to that reported by other investigators.

Non-Hodgkin's lymphoma presenting as a primary lesion in uterine cervix: case report.

Non‐Hodgkins lymphoma (NHL) arising from extra nodal tissue and staying localized to that region is rare. Rarer still is primary NHL of the uterine cervix. We describe a patient with primary extra nodal NHL of the uterine cervix and vagina who was treated successfully with chemotherapy and radiotherapy. She is free of disease 17 months after treatment. Data of 52 patients collected from the literature (Medline, National Library of Medicine, Bethesda, MD) is reviewed.

Proliferative index and CD15 expression in pediatric classical Hodgkin lymphoma

This study was conducted to assess the clinical and prognostic significance of lack of CD15 expression, proliferative index (PI), and expression of tumor suppressor protein p53 in pediatric classical Hodgkin lymphoma (CHL).

Fine Needle Aspiration Cytology Diagnosis of Lymphoproliferative Disease of the Breast

OBJECTIVE To evaluate the role of fine needle aspiration cytology (FNAC) in the diagnosis of lymphoproliferative disease (LPD) of the breast. STUDY DESIGN Over a period of 20 years (January 1982-December 2001), 13 diagnosed and/or suspected cases of LPD of the breast on FNAC were retrieved and reviewed from the files of the Cytology Laboratory, Department of Pathology, All India Institute of Medical Sciences. For each case, both May-Grünwald-Giemsa- and Papanicolaou-stained smears were reviewed along with hematoxylin and eosin-stained tissue sections and immunohistochemistry, when available. RESULTS Of the 13 cases, 1 aspirate was from a male breast and the rest were from female. Only 12 cases with documented histology were included in the study. Five of the 12 cases were diagnosed on FNAC as high grade lymphoma, 2 as low grade lymphoma, 2 as poorly differentiated malignant tumor/lymphoma, and 1 each as Hodgkins lymphoma, acute myeloid leukemia (AML) deposit and immature lymphoid cells, ?leukemia/lymphoma deposit. The tumors manifested mostly as an unilateral mass (10 cases), with 2 cases presenting with bilateral breast lumps. The lymph nodes were involved in 8 cases. Histologically, 2 of the 12 cases were poorly differentiated malignant tumors. Of the remaining 10 cases, 8 were documented as non-Hodgkins lymphoma and 1 each as Hodgkins lymphoma, mixed cellularity and AML. CONCLUSION FNAC is an inexpensive but highly useful diagnostic tool to distinguish between primary lymphoma and carcinoma of the breast. This helps with clinical management in avoiding unnecessary surgical procedures.

Superior vena cava syndrome.

Objective : In order to study the clinical profile of children presenting with superior vena cava syndrome (SVCS), case records of all children presenting with SVCS over a 10-year period were retrospectively analyzed.Methods :Twenty one children (20 males and 1 female) with a median age of 10.0 years (range 5.0 to 12.0 years) were detected to have an underlying hematological malignancy.T-cell acute lymphoblastic leukemia accounted for a major share (57%) in the underlying etiology; 33% of the subjects had non-Hodgkin’s lymphoma (T-cell lymphoblastic lymphoma).Result : No Patient required radiotherapy or urgent thoracotomy. Among survivors (n=11), who were alive and well at the time of writing this report, the median follow-up was 6.5 years, (range 0.5–9.6 years). T-cell ALL and lymphoblastic lymphoma are common underlying causes of SVCS in Indian children.Conclusion : The value of diagnostic interventions performed under local anaesthesia and prebiopsy corticosteroids usage was found to be reassuring since long-term survival without disease is achievable.

Nucleolar organizer regions as a prognostic indicator in epithelial cancers of the ovary.

Recent studies have shown that quantification of silver-stained nucleolar organizer region associated proteins (AgNORs) is correlated with ploidy and proliferative activity in several neoplasms. The prognostic value of this marker, however, has not been well defined in epithelial ovarian carcinomas (EOCs). We studied 84 cases of EOCs in an attempt to assess the potential prognostic significance of AgNORs and to compare the results with other prognostic factors in EOCs. Clinical, operative and treatment details, and follow-up data were recorded for each case. In univariate analysis, a highly significant correlation of AgNOR count with clinical stage (p < 0.01), histologic grade (p < 0.05), response to chemotherapy (p < 0.05), and biologic behavior (p < 0.01) was found. High AgNOR counts were observed in late stage (III and IV) tumors, grade 3 carcinomas, tumors in patients with partial response to chemotherapy, progressive disease, recurrence, or death from tumor. In multivariate analysis, AgNOR count at cut off 3.5 was found to be the third prognostic variable after response to therapy and stage. These observations suggest that AgNOR count holds promise for prediction of tumor aggressiveness in EOCs. However, its role as an independent prognostic variable of patient survival remains questionable.

Myelofibrosis in children: experience at a single tertiary care center in India.

The authors describe 10 cases of myelofibrosis diagnosed and managed at their center over 16 years. There were 2 and 8 cases, respectively, of primary and secondary myelofibrosis. All patients presented with fever, pallor, hepatosplenomegaly, and/or lymphadenopathy. Hodgkins lymphoma (n = 4), neuroblastoma (n = 1), thrombasthenic thrombopathy (n = 1), and retroperitoneal-mass (n = 1) were causal in 7 patients, whereas the diagnosis could not be established in a sole case of secondary myelofibrosis. Patients were managed with chemotherapy and appropriate care. However, outcome was poor. The authors emphasize variable clinical-laboratory spectrum of myelofibrosis, highlight management concerns, and demonstrate that prognosis/outcome depends upon appropriate management of the underlying condition.

Unfavorable outcome in CD15 negative pediatric classical Hodgkin lymphoma

To the Editor: The letter of Barros et al. [1] raises interesting issues. In our study on pediatric Hodgkin lymphoma (HL), we found that lack of CD15 expression was independently associated with poor overall and poor failure-free survival (HL) [2]. Barros et al. reference to the study Petrella et al. [3] inwhich they have actually reported an unfavorable outcome of CD15 negative cases. Also, that study was done prior to when the distinction between classical HL and nodular lymphocyte predominant (LP) HL was described [4]. The latter is consistently CD15 negative, and has a significantly favorable outcome [5]. The report of Petrella et al. includes 11 cases of LP, which could have been either lymphocyte rich classical HL (one of the four classical HL subtypes) or nodular LP, and therefore cannot be compared with studies on CD15 negative classical HL. There are only two studies on classical HL which report CD15 negativity to be an independent prognostic factor for overall and disease-free/failure-free survival [2,6], while no prognostic significance is reported bymany studies, possibly due to the limited number of cases studied. TheGerman study included a large number of cases and is a good support for our findings, as they found a significant association between lack of CD15 expression and higher stages at diagnosis [6]. Other studies might confirm whether CD15 negative classical HL presents with unfavorable risk factors that would lead to a poorer survival. The significant association between CD15 negativity and p53 negativity reported in our paper refers to a total absence of p53 immunostaining, that is, absence of p53 overexpression. The same association was found between CD15 negativity and low p53 expression, with a cut-off value of 10% (P1⁄4 0.009; odds ratio 5.4, 95%confidence interval 1.4–22).Wild-type p53 protein is normally below the detection sensitivity of immunohistochemistry. P53 immunodetection in Reed-Sternberg cells is thought to be the result of accumulation of a non-functional p53 protein, due to mdm2 binding, rather than p53 mutation [7]. However, Chabay et al. have shown that p53 is functional as a transcriptional activator in more than half the pediatric HL cases studied [7]. In any case, the association between loss of CD15 antigen expression and lack of p53 overexpression is a purely statistical correlation, which might be verified in further studies. The significant cut-off value for p53 expression in Reed-Sternberg cells is still to be identified. Veronique Dinand, MD, PhD* Department of Pediatrics All India Institute of Medical Sciences New Delhi, India