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Multiple Endocrine Neoplasia type-1 (MEN1) is an autosomal dominant disorder with a combined occurrence of tumours of parathyroid glands, pancreatic islets, and anterior pituitary. About 90% of these patients carry mutations in the MEN1 gene, though the spectrum is not well defined in India. Forty clinically suspected cases of MEN1 were enrolled prospectively over six years; 32 patients (23 index-cases and nine affected relatives) with≥2 classical endocrine tumours of MEN1 were considered definite, and eight were categorised as ‘MEN1-like’. Details of their clinical presentation, treatment and mutational analysis including MEN1 gene, 3′ and 5′ untranslated regions (UTR) of MEN1, CDKN1B, and CaSR genes were collated. Asymptomatic first-degree relatives were also screened. Among the 32 definite MEN1 patients, all had primary hyperparathyroidism, 22 (68.7%) had gastroentero-pancreatic neuroendocrine tumours, and 21 (66%) had pituitary adenoma. Of the 23 definite index-cases, 13 (56.5%) carried mutations in the MEN1 gene. Five of nine affected first-degree relatives (55.5%), and four of 10 asymptomatic relatives (40%) also had MEN1 mutations. Seven of 10 MEN1 mutation-negative definite index-cases harboured p.V109G polymorphism in the CDKN1B gene. All eight MEN1-like cases were negative for mutations and large deletions in MEN1, mutations in 3′ and 5′ UTR of MEN1, CaSR and CDKN1B genes. The study has helped to clearly document the pattern of mutations among Indian MEN1 patients. However, the absence of MEN1 mutation in ~44% of cases and the presence of p.V109G polymorphism in CDKN1B gene raise the question whether such polymorphisms could independently contribute to pathogenesis.

OBJECTIVE This prospective study was carried out to assess trabecular bone score, bone mineral density (BMD), and bone biochemistry in Indian subjects with symptomatic primary hyperparathyroidism (PHPT), and to study the influence of baseline parathyroid hormone (PTH) on recovery of these parameters following curative surgery. METHODS This was a 2-year prospective study conducted at a tertiary care centre in southern India. Baseline assessment included demographic details, mode of presentation, bone mineral biochemistry, BMD, trabecular bone score (TBS), and bone turnover markers (BTMs). These parameters were reassessed at the end of the first and second years following curative parathyroid surgery. RESULTS Fifty-one subjects (32 men and 19 women) with PHPT who had undergone curative parathyroidectomy were included in this study. The mean (SD) age was 44.6 (13.7) years. The TBS, BTMs, and BMD at lumbar spine and forearm were significantly worse at baseline in subjects with higher baseline PTH (≥250 pg/mL) when compared to the group with lower baseline PTH (

Primary follicular lymphoma of the gut (PFL-GI) is a rare entity. This study aims to compare the clinicopathologic features of PFL-GI with cases of gastrointestinal involvement by disseminated nodal follicular lymphoma. This is a retrospective study with 6 cases of primary follicular lymphoma and 8 cases of secondary involvement of the gut, over a period of 9 years. The slides and blocks were retrieved and reviewed. Clinical data was obtained from hospital records. Clinicopathologic features were compared. PFL-GI cases had a slightly higher median age group (p value 0.23) and no gender predilection when compared to cases with secondary involvement which showed a female preponderance. Para-aortic lymphadenopathy was seen in all secondary cases whereas none of the primary cases showed significant lymphadenopathy. The only microscopic feature that was different was the presence of hollowed out pattern of immunostaining for follicular dendritic cells seen in all cases of PFL-GI but in none of the secondary cases.

Context: Relative risk of non-Hodgkin lymphoma (NHL) in people living with HIV is 60–200 times that of normal population. This is the largest series from India on lymphomas arising in HIV-infected individuals including workup for Epstein–Barr virus (EBV) and human herpesvirus-8 (HHV-8). Aims: This study aims to ascertain the distribution and detailed clinicopathologic features of lymphoma arising in HIV-infected persons in India. Settings and Design: The study was done during the period of 2007–2011 in the pathology department of a tertiary care center in South India. Subjects and Methods: All cases diagnosed as lymphoma in the department of pathology during the study period were identified, and patients with HIV positive by serology were included in the study. Clinical details were obtained from electronic records, slides were reviewed and tissue blocks retrieved, and immunohistochemistry for HHV-8 and in situ hybridization for EBV-encoded RNA was done. Statistical Analysis Used: Descriptive statistics were done using SPSS software. Kaplan–Meier curves were used to do survival analysis. Results: Of 3346 patients diagnosed with lymphoma, 73 (2%) were diagnosed to be positive for HIV. About 87.6% of the cases were NHL, of which diffuse large B-cell lymphoma was the most common and plasmablastic lymphoma was the second common subtype. Survival was uniformly poor in 36% of the cases where follow-up was available. Conclusions: The striking differences from world literature included higher frequency of plasmablastic lymphomas, lack of primary central nervous system lymphomas, and low association with HHV8.

Background: Atypia of undetermined significance or follicular lesion of undetermined significance (AUS/FLUS) criterion in thyroid fine-needle aspirates (FNAs) has been a heterogeneous entity with much inter-observer variation. Sub-categorisation of AUS/FLUS has been observed to play an effective role in risk stratification. We aimed to validate AUS/FLUS sub-categorisation in correlation with the spectrum of malignancy. Study Design: Subjects included patients with AUS/FLUS diagnosed between January 2015 and December 2016. AUS/FLUS cases were sub-categorised into those exhibiting (1) architectural atypia, (2) cytological atypia, (3) architectural and cytological atypia, (4) AUS with Hürthle cells, and (5) AUS not otherwise specified (AUS-NOS). Each sub-category was correlated with their corresponding incidence of malignancy in surgical resections. Result: The overall incidence of AUS/FLUS in our centre was 13% (132/1,018). On retrospective review of 117 patients with AUS/FLUS, smears with cytological atypia showed a higher incidence of malignancy (78.3%) than those with architectural atypia (75.3%). AUS/FLUS cases with both cytological and architectural atypia had a malignancy rate of 71.4%. Conclusion: AUS/FLUS cases with cytological atypia had a higher risk of malignancy than those with architectural atypia. The sub-categorisation of AUS/FLUS is diagnostically important for the proper risk stratification of patients.

BACKGROUND Criteria for the pathological classification of adult adrenocortical tumours (ACTs) have been found to overestimate the malignant potential of childhood ACTs. We sought to evaluate the accuracy and utility of criteria developed for paediatric ACT compared to current criteria for adults. METHODS ACTs treated between January 2006 and December 2016 in two paediatric institutions were evaluated. Patients classified clinically as malignant (CM) had locally invasive disease at surgery requiring extensive en bloc resection to achieve clear margins, had local recurrence or distant metastasis. Slides were reviewed by pathologists blinded to the clinical outcome. A grade was assigned to each tumor according to the Weiss, Aubert, Wieneke and Dehner-Hill criteria. The pathological grade was compared to the clinical outcome. RESULTS The median follow-up was 60 months (interquartile range 25-80 months). Based on clinical criteria, of 22 patients 14 (64%) had a benign course and eight (34%) behaved malignant. The malignant potential was overestimated by Weiss criteria in 23% and Aubert criteria in 27%. Wieneke and Dehner-Hill criteria showed good clinicopathological correlation; no child who had a benign course was classified as malignant. The Dehner-Hill criteria, however, classified five (23%) children as intermediate risk of which three had a clinically benign and two a CM course. CONCLUSION The Wieneke criteria accurately predicts the clinical course in childhood ACTs and could be considered the gold standard in their pathological characterization.

Background: The t(8;14)(q24.1;q32) and its variants – the t(2;8)(p12;q24.1) and t(8;22)(q24.1;q11.2) are associated with B-cell neoplasia and result in MYC/immunoglobulin (IG) gene rearrangement. Patients and methods: We correlated the cytogenetic, molecular and clinico-pathological findings of patients with 8q24 translocations seen in the Department of Haematology, Christian Medical College, Vellore, from January 2003 to December 2015. Results: There were 34 patients with 8q24 translocations (31, ALL and three myeloma). The t(8;14) was seen in 25 patients, t(8;22) in seven and t(2;8) in two. The salient findings were as follows: 85% males; 79% adults, median age 37 years; L3 morphology in 61%; mature B immunophenotype in 77%; extra-medullary disease in 41%; additional abnormalities in 28 (85%), notably, structural abnormalities of chromosome 1q (41%) and 13q (9%) and monosomy 13 (15%); complex karyotypes in 68%.There were two double-hit lymphoma/leukemia, one with a t(14;18)(q32;q21) and the other with a t(3;14)(q27;q11.2), associated with nodal high grade B cell lymphoma and dermal leukemic infiltrates respectively.Only 13 samples were processed for DNA PCR and all these samples were positive for MYC-IgH (c-gamma type) rearrangement. Only in one patient, in addition to c-gamma, c-alpha rearrangement was also detected. Conclusion: The frequency (1.7%) and distribution of these translocations in our series and the association with 1q and 13q abnormalities is similar to the literature. Trisomies 7 and 12 were seen in less than 10% of our patients. Keywords: 8q24 translocations, Burkitt leukemia/lymphoma, Complex karyotype, Double-hit leukemia/lymphoma, MYC/immunoglobulin gene rearrangement

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.

Current treatment of Kaposi's sarcoma is reduction of immunosuppression with or without addition of mammalian target of rapamycin inhibitors (mTORi). Akt signalling plays a central role in oncogenesis of Kaposi's sarcoma. We describe a case of multifocal Kaposi's sarcoma in a renal allograft recipient, which showed unsatisfactory early response to immunosuppression reduction along with everolimus therapy but completely resolved after adding leflunomide. mTORi impair Kaposi's sarcoma oncogenesis by inhibiting mTOR downstream from the Akt signalling. Leflunomide inhibits Akt phosphorylation. This synergistic effect may be beneficial in treatment of Kaposi sarcoma and needs to be explored in trials.

There 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.The 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.T-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.The distribution of different subtypes of lymphoma in pediatric and adolescent NHL in India differs considerably from that in western countries and other eastern countries.

Context: Lymphomatoid granulomatosis (LYG) is a rare B-lymphoproliferative disorder characterised by an angiocentric and angiodestructive pattern along with Epstein - Barr virus (EBV) association. It is one of the diagnostic challenges in lymphoma pathology. Deregulation of EBV immune surveillance is one of the narrated hypotheses in the literature. Extrapulmonary manifestations are rare with LYG. Morphological grading is done based on the number of EBV-positive B cells, which is useful to strategize treatment protocol. Aims: We report here a series of nine cases of LYG to discuss the clinical, histological, and immunohistochemistry findings. Settings and Design: This is the first case series from India in published literature. Subjects and Methods: We reviewed cases of LYG diagnosed at our center for the past 11 years (2006-2016). A total of nine cases were included in this study. Histomorphology was studied in conjunction with immunohistochemistry and clinical details. Cases without classical morphology and negative for EBV immunostain were excluded from the study. Results: There were nine patients in our study (7 males and 2 female; M:F ratio 3.5:1). The age of these patients ranged from 4 years to 57 years (mean age: 30 years). The most common site involved was the lung (4, 44%), followed by the skin (2, 22%), central nervous system (2, 22%) and lymph node (1, 11%). One patient had primary immunodeficiency. Another patient had undergone renal transplant 11 years before the development of the lesion. Angiocentricity and angioinvasion were appreciated in all nine cases (9/9) with necrosis in four cases (44%) and ill-defined histiocytic aggregates in three cases (33%). The histological features were as follows: Grade 1(4 cases, 44%), Grade 2(2 cases, 22%), and Grade 3(3 cases, 33%). Conclusion: LYG is a rare EBV driven angiodestructive disease with predominantly lung involvement as well as isolated extrapulmonary sites as seen in our study. It is often progressive and ultimately fatal in the absence of appropriate treatment. Grading of the lesion helps to initiate the appropriate treatment of choice.

To identify factors predicting the risk of inguinal metastasis in squamous cell carcinoma of the penis. The therapeutic advantages of early lymphadenectomy in squamous cell carcinoma of the penis must be counterbalanced against its post-operative morbidity. Loss to follow up is a major problem in developing countries. Generating a nomogram based on clinical lymph node status and histopathological findings in the primary tumor could facilitate clinical decision making in the management of penile cancer. We prospectively studied 106 patients with penile squamous cell cancer treated from September 2001 to August 2007 at our institution. All patients were offered lymphadenectomy (LAD). A multivariate logistic regression model was used to develop a nomogram. We highlight the problems of loss to follow up in these patients. Of 53 who opted for LAD, 22 had nodal metastasis. The presence of high grade (P = 0.004), lymphovascular invasion (LVI) (P = 0.01) and palpable inguinal lymph nodes (P = 0.05) were the strongest predictors of metastasis. Of 51 who refused LAD, 22 were lost to follow up. Out of these, 16 were at high risk and 9 of them came back with inoperable fungating nodes. A nomogram predicting the risk of lymph node metastasis showed a bias corrected good concordance index (0.74) and good calibration. High grade and LVI in the tumor along with clinical stage of the inguinal nodes were the strongest predictors of metastasis. These features helped us to develop a nomogram to predict and to identify patients at risk of nodal metastasis.

Primary pleural thymomas are rare tumors often mistaken for malignant mesothelioma clinically and radiologically. An autopsy case report of primary pleural thymoma associated with a coincidental small hepatocellular carcinoma is presented. This case is reported because of the rarity of pleural thymoma and the coincidental finding of a small hepatocellular carcinoma in a non-cirrhotic background. The literature on these two tumors has been reviewed.

We present a case of extracavitary primary effusion lymphoma presenting, as jejunal polyps in a 38-year-old man. This is the first report of this entity from India. Although rare in our country, the diagnosis should be suspected in cases of CD20 negative large cell lymphoma with plasmablastic or immunoblastic differentiation in seropositive patients. Immunostaining for latency-associated nuclear antigen-1 and in situ hybridization for Epstein-Barr virus-associated RNA will confirm the diagnosis.

There is a wide range of plasma cell abnormalities in people living with HIV (PLHIV). Extramedullary plasmacytomas are not common in HIV infection, unlike plasmablastic lymphomas. An HIV-positive 44-year-old man on antiretroviral therapy presented with a rapidly progressing swelling on the face. Imaging revealed underlying bone destruction. Histologically, there was a tumour composed of small to medium-sized plasmacytoid cells admixed with many mature plasma cells and plasmablasts. These were positive for CD138 and MUM 1. Extramedullary multiple myeloma was ruled out as CD56 and cyclin D-1 were negative. EBV was negative. As the tumour cells were mostly mature, plasmablastic lymphoma was also excluded. The presence of a monoclonal protein (1 g%), IgG kappa type, was detected. Neoplasia of plasma cells acquires special clinical characteristics in PLHIV. These patients are younger, with a greater tendency to develop solitary extramedullary plasmacytomas with atypical clinical evolution and greater aggressiveness of the neoplastic process. All of these features, along with a high proliferation index (MIB1 60%) was found in our patient. We report this case for its rarity, histopathological dilemma and its atypical features in HIV infection.

An 18-year-old man presented with a large (12 x 10 cm) cystic mass involving the lower lobe and lingula of the left lung on computed tomography. Intraoperatively a large cystic mass was seen densely adherent to the left lung and the chest wall. A left pneumonectomy was performed because of the dense adhesions and extreme vascularity. Pathologic examination revealed a benign sugar cell tumor of the lung. We believe this is the first case report of such a large, clear cell tumor of the lung, mimicking malignant behavior in terms of vascularity and local invasion and requiring pneumonectomy.

Background and Aim: Lennert's lymphoma is a rare variant of peripheral T-cell lymphoma (PTCL) not otherwise specified (NOS) rich in epithelioid histiocytes. This study aims to analyze the clinical, morphologic, and immunophenotypic profile of cases of Lennert's lymphoma from our country and determines the utility of T-cell receptor (TCR) gene rearrangement in the diagnosis. Materials and Methods: All cases diagnosed as Lennert's lymphoma during the period of January 2001 to August 2011 were included in this study. Hematoxylin and eosin (H and E) stained slides and immunohistochemistry results were analyzed and TCR gene rearrangement was performed. Results: There were five cases of Lennert's lymphoma diagnosed in our institution during this period, which included two males and three females. All cases showed effacement of lymph node architecture by diffuse infiltration of small lymphoid T cells [CD3+, CD4+, CD8+, T-cell intracellular antigen 1 (TIA-1+), Granzyme B−] and clusters of epithelioid histiocytes throughout the lymph node and scattered large transformed cells (CD20−, CD30+, CD15−/+). TCR rearrangement was done in three cases by polymerase chain reaction (PCR) and showed the presence of a clonal T-cell population. Conclusions: Lennert's lymphoma constituted 0.11% of all non-Hodgkin lymphomas (NHLs) in our institution. Differentiation from classical Hodgkin's lymphoma is sometimes difficult by morphology and immunohistochemistry alone and TCR gene rearrangement was extremely useful in diagnosis.

Alveolar Soft Part Sarcoma (ASPS) is a very rare type of soft tissue sarcoma. Its cell of origin is unclear. It usually presents in the second to fourth decade of life. The most common reported sites of ASPS are the lower extremities, the head and the neck. Because of the rarity of this disease, there is no standard treatment plan. Surgical excision with negative margins is considered as the treatment of choice. We are reporting a rare presentation of ASPS as a male breast lump.

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.

Editor, Although the gastrointestinal tract is the most common site of extranodal lymphoma, colorectal lymphomas account for only 15 % to 20 % of these [1]. A 29-year-old man, with no previous comorbid illness, presented with severe lower abdominal pain, fever and vomiting for 2 days. On evaluation, he had signs of peritonitis, hemodynamic shock and lactic acidosis. The differential diagnoses were appendicular perforation, with a lesser possibility of obstructed ileocecal tuberculosis with perforation or enteric fever perforation. X-ray abdomen showed dilated bowel loops and the ultrasound reported thickened bowel loops in the right iliac fossa with moderate free fluid in the peritoneal cavity. In view of his deteriorating general condition, a CT scan abdomen was not done and he underwent an emergency laparotomy. Intraoperatively, we found two large areas of cecal gangrene with imminent perforation. There were few enlarged pericecal lymph nodes but there was no obvious mass lesion. The cecal wall was thickened and edematous. There was approximately 200 mL of serosanguinous fluid in the peritoneal cavity. We performed a right hemicolectomy and ileotransverse anastomosis as there was no fecal contamination. He made an uneventful recovery. The gross specimen (Fig. 1a) showed congested, dusky, outer surface of the cecum and proximal colon with the mucosal surface appearing dark brown, ulcerated, edematous and focally lined by exudate and small nodules, 0.1 cm to 0.5 cm in diameter. Microscopy (Fig. 1b) showed a patchy, focally perivascular infiltrate of medium-sized lymphoid cells with vesicular nuclei accompanied by transmural acute inflammation, edema and patchy foci of necrosis of the cecal wall. Few enlarged pericecal lymph nodes, largest 1.5 cm in diameter, were found. These were replaced by an atypical infiltrate which was positive for CD3, TIA1, CD8 and granzyme B, and focally positive for CD56 and CD30. There were no features of enteropathy. Immunohistochemistry for Epstein-Barr virus latent membrane protein was negative. The final report was high grade peripheral Tcell lymphoma of cytotoxic T/NK cell phenotype of the cecum, with regional lymph nodes. Further investigations included complete blood count, LDH levels in serum, HIV serology, abdominal and chest imaging to look for hepatosplenomegaly, intraabdominal, retroperitoneal or mediastinal lymphadenopathy and bone marrow biopsy. The bone marrow was free of disease. Abdominal and chest imaging did not reveal hepatosplenomegaly or lymphadenopathy elsewhere. Postoperative colonoscopy was not done. Our patient fulfilled the Dawsons criteria needed to identify a primary GI lymphoma [2]

Background and Aim: Gastrointestinal tract (GIT) is one of the major sites of extra-nodal lymphomas constituting 10-15% of all non-Hodgkin′s 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 Burkitt′s 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.

Eosinophilic disorders of the gastrointestinal tract are increasingly rare but remain an important cause of long-standing gastrointestinal symptoms. Diagnosis is usually delayed because the disease mimics other inflammatory disorders and is often not suspected initially. We report a series of four cases to highlight the various imaging appearances of this condition. Two patients presented with upper gastrointestinal involvement, one patient presented with small and large bowel involvement, and one patient presented with diffuse involvement of the entire gastrointestinal tract.

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.

Eight cases of a histological entity that we name 'acute eosinophilic appendicitis' (AEA) are described and compared with classical acute appendicitis and appendices, which turned out to be normal after emergency appendicectomy. These 8 cases formed part of a dataset of 128 emergency appendicectomies, which also included 78 cases of classical acute appendicitis and 42 cases of normal appendix. The clinical and pathological variables were compared between these three. Acute eosinophilic appendicitis is characterized by acute presentation and a grossly inflamed appendix, but with absence of neutrophils in the muscle layer. The histological hallmark of the entity is eosinophil infiltration of the muscularis propria with accompanying edema separating muscle fibers (the Eosinophil - Edema lesion). The duration and prevalence of symptoms, the male female ratio and the gross maximum circumference of the appendix in these 8 cases resembled classical acute appendicitis rather than those cases in which the morphology of the appendix was normal. The Eosinophil-Edema lesion was also seen in many cases of classical appendicitis in non-suppurative areas where neutrophils were sparse or absent. We hypothesize that this lesion is an allergic response and is probably an early event in the evolution of acute phlegmonous appendicitis. Acute eosinophilic appendicitis may merely be those cases that do not proceed on to suppuration.

Three patients referred for MRI of the foot were found to have imaging features characteristic of mycetoma. Two patients presented with recurrent soft tissue masses, which were operated on several times and not suspected to be of infective aetiology. The third patient had typical clinical features with a history of blackish granule discharge. In all three patients, MRI showed conglomerate areas of small round discrete T(2) weighted hyperintense lesions, representing granulation tissue surrounded by a low-signal-intensity rim representing intervening fibrous septa. Within many of these hyperintense lesions, there was a central low-signal-intensity dot, which gives rise to the "dot-in-circle" sign that has been very rarely described in the literature. This sign is an easily recognisable and unique appearance that is highly suggestive of mycetoma.

We report a case of primary jejunal dedifferentiated liposarcoma presenting as a submucosal polyp mimicking a benign neoplasm. This is an extremely rare presentation. The histological feature of interest was the spindle cell lipoma-like appearance of the well-differentiated component. The use of MDM2 immunostaining in differentiating benign lipomatous tumours from well-differentiated liposarcomas is mentioned, which is of value especially in lipomatous tumours of the gut where ulcerated benign tumours can show varying degrees of atypia.

Splenic marginal zone lymphomas (SMZL) constitute about 20% of primary splenic NHLs. We report a case of primary SMZL with a florid granulomatous reaction which obscured the underlying lymphoma. Although granulomas have been described in splenic non-Hodgkin lymphoma, it can be extensive and mask the underlying lymphoma. A careful search for the cytoarchitectural features of SMZL is warranted in such a case.

To date, sarcocystis has been considered an asymptomatic infection in humans. Even though cases with glomerulonephritis have been reported in animals with sarcocystis, there have been no reports of a similar occurrence in humans. We report a case of acute proliferative glomerulonephritis and leukocytoclastic vasculitis in a patient with sarcocystis infestation.