Primary Intestinal Lymphoma: Clinicopathological Characteristics of 55 Patients.

INTRODUCTION: Gastrointestinal (GI) tract is the most common site of extranodal lymphoma accounting for 30-40% of the cases. In Western countries, stomach is the most common site of GI lymphoma, whereas in the Middle East and Mediterranean countries, small intestine is commonly involved. Studies about primary intestinal lymphoma (PIL) are heterogeneous in anatomical distribution, presentation, and histological subtypes. The present study was aimed at studying the anatomical distribution, histological subtypes, and clinical characteristics at tertiary care centers. MATERIALS AND METHODS: The present study was retrospective, conducted between 2006 and 2020. Patient's data were collected from institutional medical records. PIL was diagnosed by Lewin's criteria. After histological diagnosis, PIL was classified as per the World Health Organization (WHO) criteria and staging was done according to the Ann Arbor classification as modified by Musshoff. RESULTS: A total of 941 lymphoma cases were diagnosed during the study period between 2006 and 2020 consisting of 238 Hodgkin's lymphoma and 703 non-Hodgkin's lymphoma (NHL) cases. PIL constituted 5.8% of all lymphoma cases (55 out of 941) and 50.9% (55 of 108) of all primary GI lymphoma. Median age at diagnosis was 44 years and comprised predominantly males (85.45%). Diffuse large B-cell lymphoma (DLBCL) and mucosa-associated lymphoid tissue (MALT) lymphoma were the most common histological subtype (78%) seen. Two patients with primary Hodgkin's lymphoma involving the intestine were seen. T-cell lymphoma was seen in three (5.4%) patients. Ileocecal region was the most common site involved (27%). The common presenting complaints were intestinal obstruction (40%) requiring surgical resection and abdominal pain (32%). Majority of the patients presented in the early stages (I and II). CONCLUSION: Our study demonstrates the pattern of distribution and various histological subtypes of PIL including the rare variants like primary intestinal Hodgkin's lymphoma. Relatively more number of patients presented with intestinal obstruction requiring surgery in comparison with other studies. HOW TO CITE THIS ARTICLE: Malipatel R, Patil M, Rout P, et al. Primary Intestinal Lymphoma: Clinicopathological Characteristics of 55 Patients. Euroasian J Hepato-Gastroenterol 2021;11(2):71-75.

Identification of colorectal cancers with defective DNA damage repair by immunohistochemical profiling of mismatch repair proteins, CDX2 and BRCA1.

Colorectal cancer (CRC) is a complex disease as shown by consensus classification. The present study attempted to identify subtypes with known prognostic markers for better clinical management. A total of 72 CRC tumors were examined for the expression of mismatch repair (MMR) proteins, along with caudal-type homeobox protein 2 (CDX2) and BRCA1, by immunohistochemistry. Tumors were assigned based on the presence or loss of MMR proteins as proficient or deficient. Correlations were examined with CDX2 and BRCA1 along with clinico-pathological features. Expressional pattern of microRNAs (miRs/miRNAs), such as miR-183-96-182, known to be associated with defective DNA damage repair were evaluated by reverse transcription-quantitative PCR. A total of 22% of the CRC tumors were assigned as deficient in mismatch repair. 71% of the tumors expressed CDX2 while only 21% had nuclear expression of BRCA1. Loss of CDX2 protein was higher in the deficient subtype compared with the proficient subtype. A total of 14% of the tumors had dual loss of MMR and BRCA1 proteins and showed aggressive clinical features in addition to elevated expression of DNA damage repair microRNAs. The present study shows the presence of a small proportion of colorectal tumors with dual loss of key proteins involved in DNA damage repair which may be amenable to specific therapy. The implication of the present observations warrants investigation in a larger patient cohort with prognostic information.

Non Syndromic Paucity of Interlobular Bile Ducts in Children - A Clinicopathological Study.

Background: Non syndromic paucity of interlobular bile ducts (NS-PILBD) constitutes a miniscule of infantile cholestasis. Method: Clinical details, investigations, surgical findings, management and outcome of cases of NS-PILBD at liver biopsy were analyzed. Specific histopathological features including bile duct to portal tract ratio were studied. Results: Eighteen cases (1993-2013) are detailed. Clinical presentation and investigations were similar to biliary atresia. Hepatic scintigraphy showed no gut excretion in 13/18 and operative cholangiogram was normal in all. Liver biopsy showed a median Scheuer fibrosis stage of 2, the mean bile duct/portal tract ratio was 0.29. The average age at last follow up of twelve cases was 54.9 months . Ten were asymptomatic and anicteric, the liver function tests had normalized over 3-15 months. Conclusion: Histopathology differentiated NS-PILBD from other causes of infantile cholestasis .The idiopathic form generally had a favorable long term outcome with medical management.

Primary Gastric Lymphoma: Clinicopathological Profile.

INTRODUCTION: Gastrointestinal tract (GIT) is the most common site of involvement of extranodal non-Hodgkin's lymphoma (NHL). There is regional variation in anatomical distribution of extranodal NHL, stomach being the most common site followed by small intestine. Primary gastric lymphoma (PGL) predominantly involves the antrum and corpus of the stomach. It arises from mucosa-associated lymphoid tissue (MALT) and is of B-cell lineage and often associated with Helicobacter pylori infection. Primary gastric lymphoma often presents with nonspecific symptoms. The present study was undertaken to ascertain the clinicopathological characteristics of PGL at a tertiary care center in South India. MATERIALS AND METHODS: It is a retrospective study from 2006 to 2016. Patient's data were obtained from institutional medical records. The histopathology slides were reviewed. The relevant immunohistochemistry (IHC) markers done were leukocyte common antigen (LCA), CD3, CD20, CD79a, CD10, Bcl-2, Bcl-6, CD5, Cyclin D1, CD138, and Ki-67. Correlating with the immunoprofile, further subtyping was done. RESULTS: A total of 405 patients of NHL were seen during the study period, out of which 43 patients were PGL. There were 32 males and 11 females, with M:F of 2.9:1. The mean age at diagnosis was 58 years. Abdominal pain and new-onset dyspepsia were the commonly observed presenting symptoms. The common site of involvement was antrum (20). Diffuse large B-cell lymphoma (DLBCL) was the most common histological subtype. Helicobacter pylori infection was seen in 18 (41%) patients. Majority of the patients were in stages II and III. CONCLUSION: In our study, the initial presentation of PGL was with nonspecific symptoms like abdominal pain and new-onset dyspepsia. High degree of suspicion of such symptoms and biopsy of all suspicious lesions is essential for early detection. Diffuse large B-cell lymphoma was the most common histological subtype seen in our study.How to cite this article: Malipatel R, Patil M, Rout P, Correa M, Devarbhavi H. Primary Gastric Lymphoma: Clinicopathological Profile. Euroasian J Hepato-Gastroenterol 2018;8(1):6-10.

Type 1 Gastric Carcinoid in the Indian Population and Its Association with Multifocal Gastric Atrophy.

AIM: Recent studies have shown an increase in the incidence of gastric neuroendocrine tumors (NETs) (carcinoids). This may be attributable to the frequent employment of endoscopy in clinical practice and the increasing use of proton pump inhibitors. From the literature that is available, it is interesting to note that the profile of patients with gastric carcinoids is different in the Asian population when compared to the western societies. As limited data is available from India, we evaluated retrospectively the clinical profile and pathology of gastric carcinoids presenting to our hospital. MATERIALS AND METHODS: A total of 31 patients with gastric carcinoids who presented to our institution from 2006 till 2013 were included in this study. The clinical data were obtained from the case files and the histopathology slides were reviewed. RESULTS: Gastric carcinoids constituted about 32% of all gastrointestinal (GI) NETs and were second only to duodenal carcinoids in frequency. Men were more commonly affected (74%) and the majority were of type 1 (90%). Multifocal gastric atrophy with intestinal metaplasia was additional features seen in the majority of cases with type 1 carcinoids. CONCLUSION: This study, one of the largest series reported from India, shows that the frequency and profile of gastric carcinoids is different in this population when compared to the west. It also raises the possibility that Helicobacter pylori induced multifocal gastric atrophy might be a triggering factor for the most common type 1 gastric carcinoid rather than autoimmune gastritis. CLINICAL SIGNIFICANCE: Eradication of H.pylori may be a potential preventive strategy for the occurrence of gastric carcinoids. HOW TO CITE THIS ARTICLE: Ananthamurthy A, Correa M, Patil M. Type 1 Gastric Carcinoid in the Indian Population and Its Association with Multifocal Gastric Atrophy. Euroasian J Hepato-Gastroenterol 2016;6(2):106-110.

Chylothorax in gastric adenocarcinoma: A case report and systematic review of the English literature.

BACKGROUND: Chylothorax is a rare complication of gastric adenocarcinoma and data on its identification, prevalence and outcomes are scant. OBJECTIVES: To enable identification of gastric carcinoma as a cause of chylothorax. METHODS: A case report and a systematic review were conducted of all reported cases of gastric adenocarcinoma with chylothorax as the presenting complaint in the English literature. RESULTS: Chylothorax is a rare presenting complaint of gastric adenocarcinoma. There are only 18 case reports in the world literature, of which six are in English. Chylothorax occurred variably in gastric adenocarcinoma, either as a presenting feature or as a complication of therapy. Here, we analyze the index case and six patients in whom gastric carcinoma presented with chylothorax as the initial symptom. Respiratory features of cough and dyspnea preempted any abdominal complaint. Bilateral chylothorax (66%) with associated chylous ascites (50%) was common. Four of the six patients had skin lymphedema also as a prominent feature. The chylothoraces have been treated by therapeutic pleurocentesis, intercoastal tube drainage and restriction of oral intake. Gastric adenocarcinoma was associated with high mortality (50%) and morbidity. CONCLUSIONS: Chylothorax can be the presenting feature of gastric adenocarcinoma. A thorough search for this life-threatening disease should be done before labeling the chylothorax as idiopathic.

Implementing an online reporting system in the anatomical pathology department of a tertiary care teaching hospital in India: a case study.

This article presents a case study in designing, developing, and implementing a web-enabled reporting application for the anatomical pathology (histopathology) department of a tertiary care teaching hospital in India. The article describes workflows, requirements assessment, and implementation methods that the investigators adopted for deploying the solution. The primary focus of the study was to demonstrate the requirements assessment performed, the strategies adopted, and the challenges encountered during the development and implementation. The study demonstrates a successful deployment as well as successful adoption of healthcare information technology by the end users. Factors that played a crucial role in adoption included the combination of people, processes, and technology. The lessons learned from this study would help application developers design efficient systems that suit the requirements of the end users while keeping in mind the ever-changing need for workflows and scalability in a developing country.

Lingual thyroglossal duct cysts--a review.

OBJECTIVES: Lingual thyroglossal duct cysts (TGDC) are rare and liable to be missed in a cursory clinical examination. This study aimed to report the details of lingual TGDC from the authors' series and review existing literature on the entity. METHODS: A 12 year retrospective survey of all cases of thyroglossal duct anomalies managed at a tertiary teaching hospital was conducted to identify those with lingual TGDC. Their clinical presentation, investigations, diagnosis and management were analysed. Case series from anecdotal published English literature were critically reviewed with particular regard to diagnosis and management. RESULTS: Of 78 cases of thyroglossal duct anomalies, 3 were lingual TGDC. All were females. One neonate presented with feeding difficulty and was clinically misdiagnosed as a ranula. The two older children presented with a cyst at the foramen caecum. The varied imaging and diagnostic dilemma are presented. The older children had cysts abutting the hyoid and were managed with transoral excision and a Sistrunk procedure; the neonate was managed with transoral excision only. This report also reviews the sparse literature and discusses specific issues in their treatment. The differential diagnoses encompass a wide array of developmental and neoplastic entities. Specific anatomic imaging with USG/CT/MRI and functional evaluation with radionuclide thyroid scan are essential investigative modalities. Besides a classical Sistrunk procedure and simple transoral excision, newer less invasive treatment options including marsupialisation and alcohol ablation have been reported. CONCLUSIONS: In conclusion, the diagnosis and management of lingual TGDC needs to be individualised depending on their presentation and anatomic location. The Sistrunk's procedure is ideal for those in close proximity to the hyoid; however complete cyst excision would suffice in the rest.

Papillary thyroid carcinoma with nodular fasciitis-like stroma: a case report.

Papillary thyroid carcinoma (PTC) is the most frequently occurring malignant neoplasm of the thyroid gland and is known to have several morphologic variants. PTC with nodular fasciitis-like stroma (PTC-NFS) is one of the unusual variants of PTC, with only a few cases being reported in the literature. This neoplasm is characterized by extensive reactive stromal proliferation, which may occupy 60 to 80% of the tumor along with areas of a typical papillary carcinoma. We report a case of PTC-NFS and address the diagnostic difficulties posed by the condition's extensive reactive stromal proliferation. We also emphasize that when one encounters a fibroproliferative lesion of the thyroid, a diligent search should also be made for PTC to avoid diagnostic errors.

Estrogen Receptor Negative Breast Cancer in India: Do We Really Have Higher Burden of this Subtype?

ER negative and Triple negative breast cancers carry a poorer prognosis and are not amenable to hormone therapy. It has been previously observed that Indian patients with breast cancer have a higher tendency to have these tumours. Whether this is due to inherent biological differences in the tumours of our patients is a matter of much debate. We have analysed 250 patients of breast cancer for hormone receptor status, compared them with western series, and attempted to support the hypothesis that the higher ER negativity and triple negativity is indeed due to different tumour biology.

Primary synovial sarcoma of the mediastinum : a case report.

Synovial sarcomas commonly occur in the extremities of young adults. A primary occurrence in the mediastinum is very rare with only a few reported cases in the world literature. This paper is about a 42-year-old male who presented with chest pain and dyspnoea on exertion. Imaging showed an anterior mediastinal mass with adhesions to the lung. Pathological examination of the resected mass showed a biphasic neoplasm with a spindle cell component admixed with gland-like elements. The tumour showed positive staining with cytokeratin, epithelial membrane antigen, and Bcl-2 confirming the diagnosis of a biphasic synovial sarcoma. A wide range of neoplasms, both primary and metastatic, occur in the mediastinum, which pose considerable diagnostic difficulties. A synovial sarcoma should always be considered in the differential diagnosis, and immunohistochemistry is an important adjuvant tool in this situation. This paper highlights the importance of recognizing an unusual presentation of this aggressive neoplasm to aid appropriate clinical management.

Pilomatricoma: a tumor with hidden depths.

BACKGROUND: Pilomatricoma is a benign tumor of hair matrix differentiation and has been classically described as comprising of basaloid and shadow cells admixed with multinucleated giant cells and areas of calcification. However, there are a diverse range of histologic features this tumor displays that are often unrecognized. AIMS: This study was undertaken to record the histopathologic features of pilomatricoma with an emphasis on the occurrence of other forms of differentiation. METHODS: The study included all skin biopsy specimens over a 13-year period from 1995 to 2007 that had a histologic diagnosis of pilomatricoma. Hematoxylin and eosin-stained slides were reviewed. RESULTS: This study included 21 cases of pilomatricoma. Supramatrical differentiation was seen in all cases and three-quarters of the cases showed matrical differentiation. Also observed in some of the cases were clear cell differentiation toward the outer root sheath, infundibular differentiation, calcification, ossification and secondary inflammation with a foreign body giant cell reaction. Epidermal induction in the form of a downward plate-like growth of the epidermis was seen in a few cases. CONCLUSION: Pilomatricoma, although considered a tumor of hair matrix differentiation, can show cellular evolution toward the other parts of the hair follicle, such as the outer and inner root sheaths, sebaceous and infundibular components and, therefore, can be considered a panfollicular neoplasm.

Extraskeletal myxoid chondrosarcoma: Diagnosis of a rare soft tissue tumor based on fine needle aspiration cytology.

Extraskeletal myxoid chondrosarcoma (EMC) is a rare soft tissue sarcoma with limited literature available on its cytological features. We report here one such case where a diagnosis of EMC was made based on fine needle aspiration cytology (FNAC). A 51 year-old male presented to our FNAC clinic with a slowly growing mass in the left thigh, which was subjected to fine needle aspiration biopsy. Radiological images showed no involvement of the underlying bone. Magnetic resonance imaging was suggestive of a malignant neoplasm. The FNA smears showed cell fragments and cords of monotonous cells embedded in abundant myxoid stroma. A diagnosis of a myxoid sarcoma favoring an EMC was made in this patient. Subsequent excision of the mass for histopathological examination confirmed this diagnosis. EMC has distinctive cytological features that are helpful in confidently making a diagnosis in the appropriate clinical setting.

An audit of cervicovaginal cytology in a teaching hospital: Are atypical glandular cells under-recognised on cytological screening?

BACKGROUND: Cervical cytology screening for carcinoma of the cervix in India is mainly opportunistic in nature and is practiced mainly in urban centres. The effectiveness of cervical cytology screening depends on various factors. The quality of cervicovaginal cytology service is assessed by various quality indices and by cyto-histology correlation, which is the most important quality assurance measure. AIMS: To describe the cervical cytology diagnoses, estimate the quality indices, and evaluate the discrepant cases on cytohistological correlation. SETTINGS AND DESIGN: Retrospective observational study from a tertiary care centre in South India. MATERIALS AND METHODS: Using a database search, all the cervicovaginal cytology reported during the period of 2002-2006 was retrieved and various diagnoses were described. The data was analysed to assess the quality indices. The cytohistologically discrepant cases were reviewed. RESULTS: A total of 10,787 cases were retrieved, of which 98.14% were labeled negative and 1.36% were unsatisfactory for evaluation. A few (0.81%) of the cases were labeled as squamous intraepithelial lesions and 0.38% as atypical squamous cells. The ASCUS: SIL ratio was 0.5. Cytohistological correlation revealed a total of ten cases with significant discrepancy. The majority of these were carcinomas that were misdiagnosed as atypical glandular cells. These cytology smears and the subsequent biopsies were reviewed to elucidate the reasons for the discrepancies. CONCLUSIONS: The cervical cytology service at our centre is well within the accepted standards. An increased awareness of cytological features, especially of glandular lesions, a good clinician-laboratory communication and a regular cytohistological review would further improve the diagnostic standards.

Malignant lymphoma without lymphadenopathy.

Hepatosplenic T-Cell lymphoma (HSTCL) is a rare form of extra-nodal post-thymic T-cell non-Hodgkin's lymphoma that primarily involves liver and spleen with B symptoms, with a characteristic absence of lymphadenopathy. We report such an entity in a 65-year-old man who was diagnosed to have multiple myeloma and treated for the same for two years. A clinical diagnosis of secondary myelofibrosis was suspected and was investigated, when he developed pancytopenia and massive hepatosplenomegaly at one of his follow-up visits. The patient underwent therapeutic splenectomy with a simultaneous wedge biopsy of the liver and with their corresponding histopathological and immunohistochemical features, the diagnosis of HSTCL was clinched.

Giant gastrinoma in a child: case report and review.

Gastrinomas are rare neuroendocrine tumours. A 9-year-old boy who initially presented with recurrent upper abdominal pain and was managed as hyperacidity syndrome was later diagnosed to have a primary, retroperitoneal, extra pancreatic gastrinoma after an asymptomatic period of 6 years is presented. At the second presentation, the contrast-enhanced CT revealed an epigastric mass and serum gastrin was grossly elevated. A complete excision of the mass was done; histopathological evaluation showed a well-differentiated neuroendocrine tumour. At a year follow up, the child is asymptomatic and the review imaging and serum gastrin levels are normal.

Atypical thyroglossal duct anomalies.

OBJECTIVE: Thyroglossal duct (TD) anomalies are generally considered to be a distinct clinical entity. However occasional cases do not conform to the standard picture and are complex. This study aimed to scrutinise consecutive cases of TD anomaly managed surgically over 7 years at a tertiary teaching hospital and analyse those with atypical features. METHODS: This is a retrospective study of 62 children managed for a clinical diagnosis of TD anomaly from 1998 to 2005. Their medical records were reviewed for clinical presentation, investigations, operative findings, histopathology, management and outcome. After a clinical diagnosis, they had individualized investigations (USG, MRI, radioiodine scan, FNA) prior to the Sistrunk operation. RESULTS: 5/62 cases had atypical features. 2/5 were adolescents with a short history while 3 were below 5 years of age with onset since infancy. 4/5 were females. 1/5 had a Sistrunk operation earlier, 2/5 had a redo surgery prior to cure. 3/5 had a grossly identifiable tract at surgery, one with a midline course and two deviating laterally to open at the left pharyngeal wall. The final histological diagnosis was a mixed thyroglossal-dermoid cyst (case 1), TD anomalies with aberrant pharyngeal communication (cases 2 and 3), isthmic thyroglossal 'cold' cyst (case 4), and a tuberculous cyst (case 5). Postoperatively, Cases 2 and 3 had wound infections that were managed conservatively while case 5 developed an incisional sinus that healed after a month of anti-tuberculous chemotherapy. Cases 1 and 4 had an uneventful recovery. At an average f/u of 2 years, all are asymptomatic and well. CONCLUSIONS: 5-10% of apparent TD anomalies may present with atypical clinicopathologic characteristics. Atypical cases and late presentations may warrant additional investigations to establish the diagnosis and tailor management.

Clinicopathologic analysis of 21 cases of nevus sebaceus: a retrospective study.

BACKGROUND: Nevus sebaceus (NS), otherwise designated as 'organoid nevus', involves proliferative changes of the sebaceous glands, sweat glands, and the hair follicles. It displays a range of appearances, depending on the lesion's age. AIMS: To study the histopathological features of NS and correlate these with clinical findings. METHODS: All skin biopsy specimens over a 12-year period from 1995 to 2007 which had a diagnosis of NS were included. Clinical data with follow-up notes and histopathology were reviewed. RESULTS: Half of the cases had a verrucous clinical appearance, while the rest presented as papules, plaques, or patches. All the cases showed immature hair follicles, and 24% of cases showed immature sebaceous glands. Normal terminal hair follicles were characteristically absent in the lesion. Nineteen percent of the cases showed dilated apocrine glands, and 14% showed hyperplasia of eccrine glands. Epidermal changes in the form of acanthosis, papillomatosis, and hyperkeratosis were seen in 86% of cases. Dilated keratin-filled infundibula were observed in 24% of cases. One case was associated with a squamous cell carcinoma. CONCLUSIONS: Nevus sebaceus is a cutaneous hamartoma, consisting of various elements indigenous to the organ. Normal terminal hair follicles are characteristically absent in the lesion although the same may be seen in rest of the epidermis, a feature of diagnostic importance, not usually highlighted in literature. The divergent differentiation observed in NS is consistent with the common embryologic origin of the folliculosebaceous-apocrine unit and should not mislead the pathologist.