Sheila Nair

Gastrointestinal lymphomas: Pattern of distribution and histological subtypes: 10 years experience in a tertiary centre in South India

BACKGROUND AND AIM Gastrointestinal tract (GIT) is one of the major sites of extra-nodal lymphomas constituting 10-15% of all non-Hodgkins lymphoma cases and about 30-40% of extra-nodal lymphomas. Considerable variation exists in the literature with respect to incidence of the various histological subtypes and sites of involvement. This study was undertaken to ascertain the anatomic distribution, histological subtypes and sites of all GIT lymphomas presenting to a tertiary referral hospital in southern India. MATERIALS AND METHODS The histological material of 361 patients over a period of 10 years (2001-2010), with histopathological diagnosis of lymphoma involving the GIT (both primary and secondary), was analyzed retrospectively. All lymphomas were reclassified according to the World Health Organization 2008 classification. RESULTS These 361 cases include 336 primary and 25 cases of lymphomas, where the involvement was secondary. Primary lymphomas consisted of 267 males (79.64%) and 68 females (20.24%) with a male:female ratio of 3.93:1. The mean age was 45 years (range 3-88). Diffuse large B-cell lymphoma (DLBCL) was the commonest subtype (222 cases; 66.71%), followed by low-grade marginal zone lymphoma of the mucosa associated lymphoid tissue (MALT) type (34 cases; 10.12%) and Burkitts lymphoma (35 cases; 10.48%). The commonest site was stomach (180 cases; 53.57%), followed by small intestine (79 cases; 23.51%) and large intestine (68 cases; 20.23%), respectively. There were some uncommon types of GIT lymphomas documented during the study. CONCLUSION In this largest retrospective single centre study from India, we establish that the pattern of distribution of primary GIT lymphomas (PGLs) in India is similar to the western literature in that the stomach is the commonest site of PGL and DLBCL is the commonest histological subtype. Immunoproliferative small intestinal disease cases were seen in this study, which is uncommon in the west.

Frequency and distribution of lymphoma types in a tertiary care hospital in South India: analysis of 5115 cases using the World Health Organization 2008 classification and comparison with world literature

Abstract This study aimed to analyze the distribution of lymphoid neoplasms in a single tertiary care center in India using the World Health Organization (WHO) 2008 classification. Histological material of 5115 patients with histopathological diagnosis of lymphoma, diagnosed over a period of 10 years (2001–2010), was analyzed retrospectively. Hodgkin lymphoma (HL) constituted 21.3% (n = 1089) and non-Hodgkin lymphomas (NHLs) constituted 78.7% (n = 4026). Of these, B-cell neoplasms accounted for 78.6% (n = 3166) and T-cell/natural killer (NK)-cell neoplasms 20.2% (n = 815) of the NHLs. The commonest subtype of NHL was diffuse large B-cell lymphoma (n = 1886, 46.9%). The frequency of peripheral T-cell/NK-cell lymphomas in this study was higher than in the Western literature but less than the frequency documented in some Asian countries. Similar to the Western literature but in contrast to previous Indian studies, peripheral T-cell lymphoma, not otherwise specified (PTCL NOS) (n = 238) was the commonest histological subtype of T-cell/NK-cell NHL in this study and constituted 5.9% of the total NHLs. Mixed cellularity (MC-CHL) (n = 488, 45%) was the major subtype of HL. Primary extranodal lymphoma (ENL) accounted for 32.8% (n = 1321) of all NHLs and most frequently involved the gastrointestinal tract. This study also documents the frequency of many rare types of lymphoma in South India.

Mast cell sarcoma of the small intestine: a case report

Mast cell sarcoma is an extremely rare mast cell proliferative disorder characterised by localised tumour, destructive growth and poor prognosis. Here we describe a case of mast cell sarcoma arising in the small intestine. To the best of our knowledge, so far only four cases have been reported in literature, presenting in the larynx, ascending colon, cerebrum and tibia.1–5 An elderly man presented with a 1.5-month history of abdominal pain. On examination, there was no lymphadenopathy or hepatosplenomegaly. Peripheral blood counts were normal except for mild anaemia. Serum lactate dehydrogenase was normal. CT abdomen showed a circumferential thickening of the wall of the ileum, with dilatation of bowel loops and a few enlarged mesenteric lymph nodes. The patient underwent ileal resection and anastomosis. Gross examination revealed a segment of the small intestine measuring 15 cm in length, 4 and 8 cm in circumference at either resection margin. The external surface showed a constriction about 3 cm in length. The lumen showed an annular constrictive …

Hodgkin’s lymphoma: immunohistochemical features and its association with EBV LMP-1. Experience from a South Indian hospital

Aims: Immunohistochemistry (IHC) is known to aid in the diagnosis of Hodgkins lymphoma (HL). We studied HL using an antibody panel for Reed‐Sternberg cells (RSCs) to find which antibody would be most useful for identification of RSCs. We also studied the association of Epstein‐Barr virus (EBV) latent membrane protein‐1 (LMP‐1) with HL in South India. Methods: Lymph node biopsies of 100 cases of untreated HL were included in this study. Antibodies against CD15, CD30, CD3, CD20 (L26), CD45 (LCA), EMA and EBV LMP‐1 were used for paraffin section IHC. Results: Of the 100 cases of HL, the RSCs stained with CD30 (93%), CD15 (67%), CD20 (17%) and CD3 (2%). EBV LMP‐1 was positive in 82 (82%) cases, most often in the nodular sclerosis subtype, 43 (86%) cases. Conclusions: (1) Of the panel of antibodies, CD30 was the most useful in identifying RSCs in classical HL. (2) EBV LMP‐1 was demonstrated in 82% of all cases of HL and in 96% of childhood cases. Abbreviations: CHL, classical Hodgkins lymphoma; EBV, Epstein‐Barr virus; HL, Hodgkins lymphoma; IHC, immunohistochemistry; L&H, lymphocytic and histiocytic; LD, lymphocyte depletion; MC, mixed cellularity; NLPHL, nodular lymphocyte predominance Hodgkins lymphoma; NS, nodular sclerosis; RSCs, Reed‐Sternberg cells.

Immunomorphologic profile and Epstein-Barr virus status of a cohort of 35 cases of extranodal natural killer/T-cell lymphoma, nasal type of upper aerodigestive tract from a tertiary care center in South India.

Abstract Extranodal natural killer (NK)/T-cell lymphoma, nasal type (EN-NK/T-NT) is a lymphoma of NK-cell or cytotoxic T-cell origin, which occurs most commonly in the upper aerodigestive tract. We have studied the immunomorphologic profile and Epstein–Barr virus (EBV) status of a cohort of 35 cases of EN-NK/T-NT of the upper aerodigestive tract (UADT) diagnosed in our institution during the period May 2002 to April 2012. At our institution these lymphomas constitute 0.7% of total non-Hodgkin lymphomas (NHLs) and 5.1% of total peripheral T-cell lymphomas (PTCLs). The median age at diagnosis was 40 years with a male-to-female ratio of 4:1, and the most common site of involvement was the nasal cavity. Morphologically, the neoplastic cells were predominantly medium sized in most cases. Necrosis and angiocentricity were common histologic features. Thirty cases (85.7%) showed NK-cell immunophenotype, CD3+ (29/30, 96.7%), CD56+ (30/30, 100%) and TIA1/Granzyme B+ (30/30, 100%). Five cases (14.3%) showed cytotoxic T-cell immunophenotype, CD3+ (5/5, 100%), CD56− (5/5, 100%) and TIA1/Granzyme B+ (5/5, 100%). EBV-encoded RNA-in situ hybridization (EBER-ISH) was positive in all 35 cases, whereas EBV latent membrane protein-1 (EBVLMP1) was positive in only 19/35 (54.3%) of cases. In conclusion, this is the first large study from India to report the immunomorphologic profile and EBV association of EN-NK/T-NT, which is known to have geographic variation. The frequency of these lymphomas in our study is comparable to that of Western and European countries and much less than that of Far Eastern countries.

Non-Hodgkin lymphoma in childhood and adolescence: frequency and distribution of immunomorphological types from a tertiary care center in South India

BackgroundThere is a dearth of published literature on the frequency and distribution of pediatric and adolescent non-Hodgkin lymphoma (NHL) in India according to the 2001 WHO classification. The aim of this study was to record the distribution of the different subtypes, analyze the major subtypes, and compare it with the published data from other countries. A review of pediatric NHL statistics from population-based cancer registries was included in this study.MethodsThe study was conducted using information retrieved from the files of our institution. A total of 467 patients with lymphoma (excluding mycosis fungoides) were recorded in the under 20 years group over a period of 6 years, of which 252 patients suffered from NHL. The demographic characteristics, frequency and distribution of different subtypes were noted and compared with published reports from other parts of the world.ResultsT-lymphoblastic lymphoma/leukemia constituted the majority (32.1%) of all NHLs in children and adolescents in our study. The other major subgroups were Burkitt’s lymphoma, anaplastic large cell lymphoma, and diffuse large B cell lymphoma. Burkitt’s lymphoma in this study had clinical presentations similar to those seen in western countries.ConclusionsThe distribution of different subtypes of lymphoma in pediatric and adolescent NHL in India differs considerably from that in western countries and other eastern countries.

Kikuchi-Fujimoto disease: Clinical and laboratory characteristics and outcome

Introduction: Kikuchi-Fujimoto disease is an uncommon disorder with worldwide distribution, characterized by fever and benign enlargement of the lymph nodes, primarily affecting young adults. Awareness about this disorder may help prevent misdiagnosis and inappropriate investigations and treatment. The objective of the study was to evaluate the clinical and laboratory characteristics of histopathologically confirmed cases of Kikuchis disease from a tertiary care center in southern India. Materials and Methods: Retrospective analysis of all adult patients with histopathologically confirmed Kikuchis disease from January 2007 to December 2011 in a 2700-bed teaching hospital in South India was done. The clinical and laboratory characteristics and outcome were analyzed. Results: There were 22 histopathologically confirmed cases of Kikuchis disease over the 5-year period of this study. The mean age of the subjects’ was 29.7 years (SD 8.11) and majority were women (Male: female- 1:3.4). Apart from enlarged cervical lymph nodes, prolonged fever was the most common presenting complaint (77.3%). The major laboratory features included anemia (54.5%), increased erythrocyte sedimentation rate (31.8%), elevated alanine aminotransferase (27.2%) and elevated lactate dehydrogenase (LDH) (31.8%). Conclusion: Even though rare, Kikuchis disease should be considered in the differential diagnosis of young individuals, especially women, presenting with lymphadenopathy and prolonged fever. Establishing the diagnosis histopathologically is essential to avoid inappropriate investigations and therapy.

Role of imatinib in the treatment of pediatric onset indolent systemic mastocytosis: a case report

Background: Mastocytosis is a sporadic disease characterized by an abnormal accumulation of mast cells (MCs) in single or multiple organs. It has a heterogeneous clinical picture which is a reflection of underlying tissue MC burden, mediators released and the organs involved. Here, the authors report significant symptomatic, cutaneous and systemic response to imatinib in a case of childhood onset indolent D816V KIT unmutated systemic mastocytosis (SM). Case report: A 19-year-old female presented with a history of itchy skin lesions over the face, trunk and extremities since 6 months of age associated with recurrent bouts of angioedema. The skin and bone marrow examination were consistent with mastocytosis. No pathogenic mutations were detected in exons 8 and 17. In view of the severity of cutaneous symptoms and evidence of bone marrow involvement, she was treated with imatinib which resulted in marked improvement. Conclusion: Imatinib has a therapeutic role in the presence of an imatinib-sensitive KIT mutation or in KIT816-unmutated patients with aggressive SM. Its role in the treatment of indolent and cutaneous mastocytosis is less well established. However, the authors have demonstrated the usefulness of imatinib in the treatment of c-KIT-negative indolent SM with extensive cutaneous involvement.

Our experience with papillary thyroid microcancer.

Background: Papillary thyroid microcarcinoma (PTMC) describes a focus of papillary thyroid cancer that is less than 1 cm in size. These tumors are frequently found on histopathological examination of thyroid specimens, operated upon for an indication other than suspected malignancy. Materials and Methods: From 2005 to 2012, 94 of 1300 thyroidectomy specimens in our institution were found to have PTMC. Of these, 77 were isolated PTMC while the others were associated with other differentiated cancers. We studied their clinicopathologic features, treatment and long-term outcome. Results: There were 18 men and 59 women (the male: female ratio was 1:3), their mean age was 44 ± 10.5 years (range: 18-72 years). Multinodular goiter was the most common indication for surgery. Malignancy was suspected in only 31.4% cases. The mean tumor size was 4.1 ± 2.3 mm. Nearly 17% cases had slightly larger tumors measuring >6 but <10 mm. Multifocal tumor was found in 44.1% of cases and among these, multifocal disease restricted to a single lobe was found in 19.5%. Eleven patients (14.2%) had cervical lymph node metastasis, 3 (3.9%) had extra thyroid tumor extension and 2 (2.6%) had evidence of vascular invasion. One patient (1.3%) presented with bone metastasis. Majority of the patients (79.2%) underwent total thyroidectomy with or without lymph node dissection. Sixteen patients (20.7%) who had initially undergone hemithyroidectomy went on to have completion thyroidectomy. Twenty nine patients (36.8%) also received radioactive iodine. The mean duration of follow-up was 20.2 ± 13.5 months. On follow-up one patient developed cervical lymph node recurrence and one died due to a second malignancy. Conclusions: PTMC is often found as an incidental finding on the thyroidectomy specimen. Sometimes they present with regional lymph node metastasis and very rarely with distant metastasis. They have a good prognosis similar to papillary thyroid carcinoma.

Clinicopathological features of hepatosplenic T cell lymphoma: a single centre experience from India

Abstract In a first series from India, we report 9 cases of hepatosplenic T cell lymphoma (HSTCL) seen in 23 months accounting for 4.2% of all mature T-non-Hodgkin lymphomas (NHLs) in our institution. All patients presented with organomegaly, cytopenias and had evidence of bone marrow involvement. The tumor cells had a blastic (55%) morphology with predominantly intrasinusoidal (33.3%) or intrasinusoidal with an additional interstitial component (33.3%). On flow cytometry, the classical phenotype (CD3+, CD7+, CD4−, CD8−, CD5−, CD56+/−) was seen only in 4 patients. Unusual variations included CD45 (overexpression), CD7 (dim expression), CD3 (overexpression, heterogeneous and dim), CD2 (overexpression), CD5 (heterogeneous), CD8 (heterogeneous or dim or overexpression) and aberrant expression of CD19. Fluoresvent in situ hybridisation (FISH) and karyotyping was abnormal in 5 out of 7 patients evaluated. All of the 5 cases showed abnormalities in chromosome 7 (ring chromosome or isochromosome 7q). Five patients died of disease and related complications in a span of 1–3 months after diagnosis whereas 4 were alive at their last follow up out of which 2 had documented a relapse. In our series, HSTCL was characterized by typical clinical and variable immunophenotypic features and a dismal clinical outcome.