Clinicopathological Profile of Primary Extra Nodal Lymphoma from a Tertiary Care Center in South India.

Background & Objective: Primary extranodal lymphoma (pENL) is a malignant lymphoid neoplasm that presents with the main bulk of disease at an extranodal site. The incidence of primary pENL has risen sharply in recent years due to the advent of better diagnostic modalities. Diagnosing pENL can be challenging due to its morphological overlap with other tumors native to the site of origin. This study aims to establish the anatomic distribution, clinical presentations, possible etiologic correlations, and histological subtypes of pENL in a tertiary care center located in South India. Methods: This is a retrospective study of 109 patients with pENL (69 males, 40 females, M: F = 1.7:1) over 5 years (October 2012 to September 2017). The tumors were reclassified according to WHO classification of Haematolymphoid tumors, 5th edition, 2022. Results: pENL constituted 109/481 cases (22.6%) of all NHL cases, with the highest incidence in 7th decade. The gastrointestinal tract (39%) was the predominant site involved, followed by head and neck (26%). Diffuse large B cell lymphoma (DLBCL) was the most common histomorphological variant followed by Follicular lymphoma (FL). The majority of the patients were immunocompetent (89%) and presented with stage IV disease (31.1%) at diagnosis. Conclusion: This study presents an overview of the diverse distribution patterns of both common and rare pENL within a tertiary care center. The accurate diagnosis of pENL necessitates the elimination of secondary extranodal involvement. It is important to note that the accurate diagnosis of pENL requires careful evaluation and exclusion of other possible causes.

Clinical and pathological characteristics of blastoid mantle cell lymphoma: a single institution experience.

Background: Blastoid mantle cell lymphoma (B-MCL) is a rare aggressive lymphoma. It is characterized by blastoid morphology with high proliferation and inconsistent immunohistochemistry (IHC), making it a diagnostic challenge for the pathologist. Methods: This is a retrospective analytical cohort study. We reviewed biopsy confirmed cases of B-MCL diagnosed over a period of 10 years (January 2012 to December 2022). The clinical presentation, histopathological and IHC findings, treatment received, and survival outcomes were studied. Randomly selected cases of classic MCL (n=12), diagnosed during the same period served as controls. Results: A total of 12 cases were studied. Four cases were transformed from previously diagnosed MCL; 8 cases arose de novo. Mean age was 61.17 years and the male: female ratio was 5:1. Half of the cases showed extra nodal extension and 81.8% had bone marrow involvement. Gastrointestinal tract was the most common site of extra nodal involvement. Histopathological examination showed diffuse involvement of the lymph node with medium sized cells. On immunohistochemistry, one of the cases showed loss of CD5 expression while the other had aberrant CD10 expression. Mean Ki-67 index was 58.09% in the cases and 16.33% in controls and was statistically significant ( p=0.005). The median overall survival (OS) for cases was 2 years vs 8 years in controls. The p53 over expression (>30% nuclear positivity) was seen in 66.6% cases (4/6). Conclusion: There are several factors that contribute to the aggressiveness of B-MCL, and new treatment approaches might be required to improve patient outcomes.

Intraperitoneal Dedifferentiated Liposarcoma: Unveiling a Rare Diagnosis in Peritoneal Fluid Cytology A Case Report with Literature Review.

Introduction Sarcomas presenting as malignant effusion are rare and the diagnosing them on fluid cytology requires expertise and clinicoradiological correlation as cells undergo morphological changes, mimicking carcinoma, or mesothelioma. Case presentation We present a case of 70-year old man with abdominal distention and pain, initially suggestive of carcinoma on peritonal fluid cytology. However, subsequent analysis with immunohistochemistry on the cell block revealed diffuse nuclear positivity for MDM2, leading to the diagnosis of dedifferentiated liposarcoma. Conclusion The cytological diagnosis of dedifferentiated liposarcoma is challenging and requires high index of suspicion, with clinicoradiological correlation. Utilising immunohistochemistry on cell block samples, enhances diagnostic accuracy and guiding appropriate patient management.

Bing-Neel syndrome: A rare complication of waldenstrom macroglobulinemia.

ABSTRACT: Central nervous system involvement is a rare complication of Waldenstrom macroglobulinemia (WM) accounting for 1-2% cases. This syndrome is called Bing-Neel syndrome (BNS) after the people who first described it in 1936. Although WM has a good prognosis, with the onset of this syndrome, most patients fare poorly with a high mortality rate. A 77/male, a known case of WM, presented with left upper limb weakness of 2 weeks duration. Magnetic resonance imaging brain showed diffuse pachymeningeal thickening along bilateral frontoparietal convexity. Biopsy showed lymphoplasmacytic lymphoma favoring a diagnosis of BNS. The patient was started on chemotherapy (rituximab + ibrutinib). At 3 months following diagnosis, the patient was admitted with Gram-negative septicemic shock and failed to recover from it. BNS is a rare complication of WM, associated with poor prognosis and an aggressive clinical course. It can occur during the course of treatment of WM, as was seen in this case. Accurate diagnosis with appropriate treatment plays a crucial role in patient management.

Desmoid fibromatosis-a diagnostic dilemma.

PURPOSE: Desmoid fibromatosis in head and neck is infrequent and poses a significant challenge to the clinicians due to its non-specific characteristics. METHODS: This case report focuses on a 69-year-old male who presented to a tertiary healthcare center in Karnataka, India with a swelling in the oral cavity. RESULTS: Despite initial suspicions of malignancy based on clinical examination and findings on computed tomography imaging, subsequent histopathology and immunohistochemistry revealed an unexpected finding. CONCLUSION: The case highlights the importance of clinical suspicion and histopathological evaluation as well as the need for greater awareness to facilitate early diagnosis and appropriate management of desmoid fibromatosis. We also present a literature review of varied presentations of desmoid tumors afflicting various subsites of the head and neck.

Multifocal intraosseous pseudomyogenic (epithelioid sarcoma-like) hemangioendothelioma: A rare presentation of an uncommon tumor.

ABSTRACT: Pseudomyogenic hemangioendothelioma (PHE) is an uncommon mesenchymal neoplasm of intermediate malignant potential showing endothelial differentiation. Around 20 cases of primary osseous PHE have been reported to date. A 16-year-old boy presented with complaints of pain in his right leg. Imaging revealed multifocal intramedullary and cortical-based lytic lesions involving long and small bones. Microscopic examination revealed plump, spindled cells arranged in fascicles and admixed "epithelioid" and "rhabdoid" cells sans vasoformative areas. By immunohistochemistry, the lesional cells were reactive for AE1/AE3, CD31, Erg, Fli1, and SMA, while immunonegative for CD34, myogenin, and S100. Nuclear expression of the INI1/SMARCB1 protein was retained. PHE is a rare entity, more so as a primary osseous lesion; therefore, awareness of the presence of this entity in the bone is the key to making a diagnosis. We discuss its clinicopathological features, differential diagnosis, and an attempt a short review of the literature.

Colorectal Carcinoma With Sarcomatoid Components: Report of 15 Cases and Literature Review of an Exceedingly Rare Carcinoma Subtype.

Colorectal carcinoma with sarcomatoid components (which includes so-called carcinosarcomas and sarcomatoid carcinomas) is a rare subtype with 50 reported cases in the literature and overlapping criteria with undifferentiated carcinoma. We collected and described 15 cases from 10 men and 5 women, with a mean age of 66 years. Symptoms included abdominal pain and gastrointestinal bleeding. Most tumors presented in the rectosigmoid region, with a mean size of 8.2 cm. The sarcomatoid component, on average, represented 58% of the tumors and took many forms, including spindled (10 cases), anaplastic (9 cases), and rhabdoid (3 cases); one case showed osteoid matrix. Tumor budding was usually high, and tumor-infiltrating lymphocytes were usually low. The sarcomatoid component was keratin-positive in 10 cases. One case showed loss of mismatch repair protein expression, and 2 cases showed SMARCA4 loss (1 also with SMARCA2 loss). Molecular testing identified mutations in KRAS (n=1), NRAS (n=2), BRAF (n=2), APC (n=1), and TP53 (n=1) in a few cases. Tumors often presented at advanced stage, with 11 cases pT4, 9 cases with nodal metastases, and 7 cases with distant metastases. Follow-up was available for 10 cases (median: 2 months), with 2 alive without disease, 3 alive with disease, and 5 dead. Our findings roughly corresponded with those in previously reported cases. Colorectal carcinoma with sarcomatoid components is rare and aggressive, with a poor prognosis for many patients. We suggest that spindled cells, anaplasia, heterologous elements, and/or a component with definable sarcomatous lineage be used to distinguish colorectal carcinoma with sarcomatoid components from undifferentiated carcinoma.

Anxiolytic activity of ultra-diluted Aconitum napellus on Wistar rats

BackgroundAnxiety disorders are highly prevalent that affect millions of people worldwide. Homeopathic system is gaining popularity due to its efficacy in chronic diseases. Ultra-diluted Aconitum napellusis a homeopathic medicine used by clinicians for treatment ofacute anxiety.AimObjective of this study is to test the efficacy of Aconiteon experimental anxiety models of Wistar rats. Methodology18 Female Wistar rats were dividedinto Control, Anxiety, andAnxiety + Aconite(ANX+ACO) groups. Acute stress was induced by restraining the rats for sixhours daily for fivedays in Anxiety and ANX + ACO groups. After fivedays of stress,a single dose of Aconite200cH was given to ANX + ACO group through oral gavage, whereas control and anxiety group rats were maintainedwith a normaldiet. Following this, behavioral assessment was done. Histological sections of the hippocampal area weretaken and stained with Hematoxylin and Eosin and also Cresyl violet stain. ResultsIt was observedthat the Aconitetreated group had morenumber of entries and also the percentage of time spent in the open arm was higher than the stressed anxiety group. Histological study of the brain tissue procured from all three groups was analyzed. ConclusionIt was inferred that ultra-diluted homeopathicAconitehas decreased anxiety in Wistar rats which were subjectedto acute restrainstress. However,the decrease was not statistically significant to deduce Aconiteas an anxiolytic.(AU)