scholarly journals Histomorphological Spectrum of Mediastinal Masses with Special Emphasis on Rare Lesions

2020 ◽  
Author(s):  
Sakthisankari Shanmuga Sundaram ◽  
S Vidhyalakshmi
Keyword(s):  
Disease Process ◽  
B Cell Lymphoma ◽  
Posterior Mediastinum ◽  
Nodal Metastasis ◽  
Late Relapse ◽  
Diagnostic Challenge ◽  
Mediastinal Node ◽  
Mediastinal Masses ◽  
Diagnostic Difficulties ◽  
Mediastinal Tumours

Introduction: Mediastinal lesions are not frequently observed in routine clinical practice. A wide variety of disease process can occur within the mediastinum. Tissue from needle biopsies forms the major diagnostic material. There is need for detailed studies to make pathologists aware of the wide histological spectrum and to know the unusual lesions that can be encountered during diagnosis. Aim: To describe the histomorphological spectrum of various mediastinal lesions and to highlight the rare lesions that posed a diagnostic challenge. Materials and Methods: This was a retrospective study done on all mediastinal masses over a period of two years from January 2016 to December 2018. The clinical data and imaging findings were collected from the test request forms and patients’ files for all the cases. Haematoxylin and eosin stained slides of all cases and immunohistochemistry markers and cytochemical stains wherever done were reviewed and analysed. Mean, median and percentage were used for statistical analysis. Results: Thirty-four cases of mediastinal lesions were included in the study. Of these, 28 cases (82.4%) presented as anterior mediastinal masses, three cases (8.8%) each in the middle and posterior mediastinum. Thymoma (n=15) followed by lymphoma (n=4) were the most frequent neoplasms reported. There were five lesions which were rare in the mediastinum with unusual presentation that posed diagnostic difficulties. These lesions included Primitive neuroectodermal tumour in an adult, Primary Mediastinal B Cell Lymphoma (PMBCL), Malignant Peripheral Nerve Sheath Tumour (MPNST) with nodal metastasis, Seminoma deposits in mediastinal node occurring as late relapse and dedifferentiation of thyroid carcinoma in mediastinal node metastasis without an obvious primary. Conclusion: Primary mediastinal tumours were more frequent than metastasis. Although thymomas and lymphomas are dealt extensively in literature, this article highlights the fact that pathologists must be aware of those lesions that don’t display the classical histological and/or clinical features as observed in this study.

scholarly journals Immunohistochemical Markers of Apoptotic and Hypoxic Damage Facilitate Evidence-Based Assessment in Pups with Neurological Disorders

2021 ◽  
Vol 8 (10) ◽  
pp. 203
Author(s):  
Ksenia Orekhova ◽  
Sandro Mazzariol ◽  
Beatrice Sussan ◽  
Massimo Bucci ◽  
Federico Bonsembiante ◽  
...  
Keyword(s):  
Supportive Therapy ◽  
B Cell Lymphoma ◽  
Neurological Deficits ◽  
Intestinal Helminth ◽  
Symptom Duration ◽  
Protein Markers ◽  
Diagnostic Challenge ◽  
Immunohistochemical Markers ◽  
Initial Symptoms ◽  
Metabolic Imbalance

Seizures in puppies often present a diagnostic challenge in terms of identifying and treating the underlying cause. Dog breeds with mutations of the MDR1-gene are known to show adverse reactions to certain drugs, yet metabolic imbalance exacerbated by physiologically immature organs and other contributing pathologies require consideration before arriving at a diagnosis. This study analysed the brains of two male, 5-week-old Australian Shepherd siblings that died after displaying severe neurological symptoms upon administration of MilproVet® to treat severe intestinal helminth infection. Despite the initial symptoms being similar, their case histories varied in terms of the symptom duration, access to supportive therapy and post-mortem interval. Histopathology and immunohistochemistry were used to obtain more information about the phase of the pathological processes in the brain, employing protein markers associated with acute hypoxic damage (β-amyloid precursor protein/APP) and apoptosis (diacylglycerolkinase-ζ/DGK-ζ, apoptotic protease activating factor 1/Apaf1, and B-cell lymphoma related protein 2/Bcl-2). The results seem to reflect the course of the animals’ clinical deterioration, implicating that the hypoxic damage to the brains was incompatible with life, and suggesting the usefulness of the mentioned immunohistochemical markers in clarifying the cause of death in animals with acute neurological deficits.

scholarly journals Intravascular Lymphoma in Patient With Celiac Disease With Multi Glandular Involvement

2021 ◽  
Vol 5 (Supplement_1) ◽  
pp. A938-A939
Author(s):  
Mustafa Alam ◽  
Mohamad Hosam Horani
Keyword(s):  
Celiac Disease ◽  
B Cell ◽  
Cell Lymphoma ◽  
Case Reports ◽  
B Cell Lymphoma ◽  
Diagnostic Challenge ◽  
Pituitary Microadenoma ◽  
Non Hodgkin Lymphoma ◽  
Large B Cell Lymphoma ◽  
History Of

Abstract Case Presentation: The patient is a 60 year old male with a past medical history of celiac disease, paroxysmal Afib, iron deficiency, and CAD who presented with lightheadedness, dizziness, and fatigue. Notable workup revealed that the patient had Afib with RVR, a TSH of 0.189, Free t4 0.51an LDH of 2726, hemoglobin of 8.7, AST of 155, ALT of 19, WBC of 4.5, and serum iron of 20. The patient’s cardizem dose was adjusted and repeat transthoracic echocardiogram was unremarkable compared to history. The patient presented again with complaints of abdominal distension, postural dizziness, occasional night sweats, and fevers. Repeat workups revealed pancytopenia, proteinuria, hypotension, and anasarca most pronounced in the lower extremity and scrotum. Ultimately, a kidney biopsy revealed an intravascular B cell non-Hodgkin lymphoma (IVBCL). Notable repeat labs include a CRP of 44 and a failed ACTH stimulation test. A brain MRI revealed a 6mm pituitary microadenoma. The patient placed on an R-CHOP regiment and is scheduled for repeat MRI to rule out pituitary involvement. Discussion: IVBCL’s are a rare form of diffuse B cell lymphoma and remain a diagnostic challenge due to the variety of involved systems including skin, CNS, and endocrine. IVBCL is also known to not produce a mass or lymphadenopathy. Celiac disease is a known risk factor for non-Hodgkin’s lymphoma. A literature search reveals a few case reports with common themes of increased LDH and inflammatory markers, anemia, and hepatic and renal dysfunction. Postural hypotension can also be a presenting symptom due to IVBCL’s ability to infiltrate neurovascular tissue to cause autonomic neuropathy. However, in this case, the patient’s history of primary adrenal insufficiency makes this unlikely. Hypothyroidism secondary to pituitary and thyroid involvement was suspected due to TSH level suppressed enough for central hypothyroidism. Repeated MRI showed resolution of Pituitary Microadenoma post Chemo therapy. Sylvain Raoul Simeni Njonnou, Bruno Couturier, Yannick Gombeir, Sylvain Verbanck, France Devuyst, Georges El Hachem, Ivan Theate, Anne-Laure Trepant, Virginie De Wilde, Frédéric-Alain Vandergheynst, “Pituitary Gland and Neurological Involvement in a Case of Hemophagocytic Syndrome Revealing an Intravascular Large B-Cell Lymphoma”, Case Reports in Hematology, vol. 2019, 6 pages, 2019. https://doi.org/10.1155/2019/9625075 Catassi C, Fabiani E, Corrao G, et al. Risk of Non-Hodgkin Lymphoma in Celiac Disease. JAMA. 2002;287(11):1413 Khan MS, McCubbin M, Nand S. Intravascular Large B-Cell Lymphoma: A Difficult Diagnostic Challenge. J Investig Med High Impact Case Rep. 2014 Mar 6;2(1):2324709614526702. Pearce C, Hope S, Butchart J. Intravascular lymphoma presenting with postural hypotension. BMJ Case Rep. Published 2018 Jan 29.

Mediastinal tumours and cysts

2020 ◽  
pp. 4368-4376
Author(s):  
Y.C. Gary Lee ◽  
Helen E. Davies
Keyword(s):  
Weight Loss ◽  
Posterior Mediastinum ◽  
Anatomical Site ◽  
Detailed Knowledge ◽  
Malignant Tumours ◽  
Important Group ◽  
Abnormal Chest ◽  
Mediastinal Tumours ◽  
Middle Mediastinum ◽  
Systemic Symptoms

Mediastinal masses are most conveniently categorized by their anatomical site in the anterior, middle, or posterior mediastinum. Most present as a radiographic abnormality alone, or in association with symptoms arising from compression of other mediastinal structures. Systemic symptoms such as fever or weight loss are more likely with malignant tumours such as lymphomas or thymomas. Detailed knowledge of normal mediastinal anatomy is a prerequisite to the interpretation of both normal and abnormal chest radiographs. Lymph nodes are present in all three compartments thereby knowledge of their anatomical relationships, together with sites of drainage, is important when interpreting radiographic mediastinal enlargement. The most important group of visceral nodes lie in the middle mediastinum and are predominantly subcarinal and paratracheal. Bronchopulmonary and hilar nodes are numerous but not visible radiographically unless pathologically enlarged.

scholarly journals Late Relapses in Patients With Diffuse Large B-Cell Lymphoma Treated With Immunochemotherapy

2019 ◽  
Vol 37 (21) ◽  
pp. 1819-1827 ◽  
Author(s):  
Yucai Wang ◽  
Umar Farooq ◽  
Brian K. Link ◽  
Melissa C. Larson ◽  
Rebecca L. King ◽  
...  
Keyword(s):  
B Cell ◽  
Cell Lymphoma ◽  
B Cell Lymphoma ◽  
Late Relapse ◽  
Indolent Lymphoma ◽  
Large B Cell Lymphoma ◽  
Germinal Center B Cell ◽  
Competing Events ◽  
First Relapse ◽  
Large B Cell

PURPOSE In patients with diffuse large B-cell lymphoma (DLBCL), most relapses occur within the first 2 years of diagnosis. We sought to define the rate and outcome of late relapses that occurred after achieving event-free survival at 24 months (EFS24). METHODS We prospectively followed 1,324 patients with newly diagnosed DLBCL from 2002 to 2015 and treated with immunochemotherapy. Cumulative incidences of late DLBCL and indolent lymphoma relapses were analyzed as competing events. Postrelapse survival was defined as time from first relapse to death from any cause. RESULTS In 847 patients who achieved EFS24, the cumulative incidence of late relapse was 6.9% at 3 years, 9.3% at 5 years, and 10.3% at 8 years after EFS24. The incidence of DLBCL relapse was similar in patients with DLBCL alone at diagnosis (6.3% at 5 years), compared with patients with concurrent indolent lymphoma at diagnosis (5.2%; P = .46). However, the rate of indolent lymphoma relapse was higher in patients with concurrent indolent lymphoma (7.4% v 2.1% at 5 years; P < .01). In patients with DLBCL alone, the rate of DLBCL relapse was similar in the germinal center B-cell–like (GCB) (4.1% at 5 years) and non-GCB (4.0%; P = .71) subtypes, whereas the rate of indolent lymphoma relapse was higher in patients with the GCB subtype (3.9% v 0.0% at 5 years; P = .02). Postrelapse survival was inferior for patients who relapsed with DLBCL than for those who relapsed with indolent lymphoma (median 29.9 months v unreached; P < .01). CONCLUSION Patients with DLBCL with a concurrent indolent lymphoma and those with the GCB subtype had a higher rate of late relapse, owing to increased relapses with indolent lymphoma. Patients who relapsed with DLBCL had a worse prognosis than those who relapsed with indolent lymphoma.

scholarly journals P1302 B-cell lymphoma presenting as an ejection systolic murmur

2020 ◽  
Vol 21 (Supplement_1) ◽  
Author(s):  
T Constantinides ◽  
M Ioannides ◽  
K Yiagou ◽  
P Avraamides
Keyword(s):  
B Cell ◽  
Transthoracic Echocardiography ◽  
Cell Lymphoma ◽  
Imaging Modality ◽  
Pulmonary Stenosis ◽  
B Cell Lymphoma ◽  
Large Mass ◽  
Clinical Findings ◽  
Ct Guided ◽  
Mediastinal Masses

Abstract OnBehalf Nicosia General Hospital We present the case of a young patient with large B-cell lymphoma causing severe extrinsic pulmonary stenosis. This patient presented to the emergency department with chest discomfort ,fatigue and dyspnea on minimal exertion. A loud ejection mid-systolic crescendo-decrescendo murmur with widely split S2 located in 2nd left parasternal border, raised the suspicion of pulmonary stenosis and patient underwent immediate transthoracic echocardiography as the first imaging modality of choice.On transthoracic echocardiography a large mass was seen, causing severe extrinsic pulmonary stenosis, and was further diagnosed as B-cell lymphoma after CT-guided biopsy. We discuss the high index of lymphoma suspicion in cases of pulmonary stenosis findings such as loud ejection murmur in pulmonic valve auscultation area, in otherwise healthy patients, with no history of congenital heart disease. Lymphoma and other mediastinal masses represent the most common aetiology of acquired pulmonary stenosis and should be suspected in otherwise healthy patients who present with clinical findings of pulmonary stenosis and vice-versa, pulmonary stenosis should be suspected in the presence of symptomatic mediastinal masses. Abstract P1302 Figure. Lymphoma PSAX

scholarly journals Methotrexate-Associated Lymphoproliferative Disorders Mimicking Granulomatosis With Polyangiitis: A Radiological Diagnostic Challenge

2020 ◽  
pp. 014556132097068 ◽  
Author(s):  
Tomoyasu Tachibana ◽  
Tomoaki Sasaki ◽  
Yoji Wani ◽  
Yasutoshi Komatsubara ◽  
Kazunori Kuroda ◽  
...  
Keyword(s):  
Nasal Cavity ◽  
Granulomatosis With Polyangiitis ◽  
B Cell Lymphoma ◽  
Pulmonary Involvement ◽  
Lymphoproliferative Disorders ◽  
The Body ◽  
Complete Regression ◽  
Diagnostic Challenge ◽  
History Of ◽  
Enhanced Computed Tomography

Methotrexate-associated lymphoproliferative disorders (MTX-LPD) frequently involve the extranodal organs throughout the body. Among the extranodal occurrences of MTX-LPD, pulmonary involvement is most frequent. In contrast, there are only a few reports of MTX-LPD in the nasal cavity or paranasal sinuses. Moreover, there are no previous reports of MTX-LPD mimicking granulomatosis with polyangiitis (GPA) in imaging examinations. We describe a case of a 53-year-old woman with MTX-LPD mimicking GPA in the nasal cavity and lungs. She complained of left nasal obstruction and discharge, general fatigue, and continual fever for 2 months. The patient had been diagnosed with rheumatoid arthritis and received methotrexate (MTX) for over 10 years. Contrast-enhanced computed tomography revealed unenhanced masses in the nasal cavity and multiple masses with cavitary changes in the bilateral lungs, suggesting GPA. However, histological examination of the nasal lesion and a history of MTX treatment indicated a diffuse large B-cell lymphoma type MTX-LPD. Two weeks after MTX withdrawal, prominent improvements in both lesions were observed. Complete regression of the nasal lesion was observed 3 months after discontinuation of MTX. Thus, MTX-LPD may mimic GPA in imaging examinations.

scholarly journals Intravascular Large B-Cell Lymphoma Presenting as Acute Axonal Polyneuropathy: A Case Report and Literature Review

2020 ◽  
Vol 8 ◽  
pp. 232470962095999
Author(s):  
Jordan M. Minish ◽  
Amar H. Kelkar ◽  
Amol R. Mehta ◽  
Maira Gaffar ◽  
Nam H. Dang
Keyword(s):  
B Cell ◽  
Cell Lymphoma ◽  
Disease Process ◽  
B Cell Lymphoma ◽  
Intrathecal Methotrexate ◽  
Common Disease ◽  
Success Rates ◽  
Large B Cell Lymphoma ◽  
Axonal Polyneuropathy ◽  
Large B Cell

Intravascular large B-cell lymphoma (ILBL) is a rare and difficult to diagnose subtype of large B-cell lymphoma. The most common locations of presentation are in the central nervous system and the skin, but there are reports of other organ involvement. Due to the indolence, nonspecific symptoms, and rarity of the disease, this form of lymphoma is most often diagnosed postmortem. In this article, we describe a case of ILBL that presented as a rapidly progressive acute axonal polyneuropathy. Acute axonal polyneuropathy is a common disease process with a wide differential diagnosis, but there is limited literature on its prevalence as the presenting symptom of ILBL. This patient was treated with R-EPOCH and intrathecal methotrexate with significant improvement in his polyneuropathy after 1 cycle, and complete remission after 6 cycles. Data on chemotherapy regimens and their success rates for this disease are lacking.

scholarly journals Primary central nervous system lymphoma in scleroderma: A case series

2020 ◽  
pp. 239719832097039
Author(s):  
Danielle M Robinett ◽  
Laura K Hummers ◽  
Meaghan Morris ◽  
Amy S Duffield ◽  
Ami A Shah
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Systemic Sclerosis ◽  
Immunosuppressive Therapy ◽  
Disease Process ◽  
Central Nervous System Lymphoma ◽  
B Cell Lymphoma ◽  
Neurologic Symptoms ◽  
Increased Risk ◽  
Risk Of Cancer

Many studies have demonstrated an increased risk of cancer in patients with rheumatologic diseases, including systemic sclerosis. Less explored is the role of immunosuppressive therapy as a contributing factor in cancer emergence or detection. This series introduces two cases of patients with systemic sclerosis who demonstrated clinical improvement in their rheumatic disease process with immunosuppression, but both of whom developed neurologic symptoms in the setting of decreasing or discontinuing immunosuppressive therapy, leading to the ultimate diagnosis of Epstein–Barr virus positive diffuse large B-cell lymphoma of the central nervous system. To our knowledge, primary central nervous system lymphoma has not been previously described in systemic sclerosis patients. Immunosuppressive therapies could promote the development of virus-associated malignancies due to decreased viral clearance. We hypothesize that removing immunosuppression could allow the immune system to generate an inflammatory response to an underlying tumor or viral antigen, contributing to development of neurologic symptoms and detection of underlying disease.

Late-Relapse Diffuse Large B-Cell Lymphoma Frequently Represents Recurrence of the Original Disease, and Demonstrates Evidence of Superimposed Clonal Heterogeneity and Clonal Evolution

Blood ◽  
2014 ◽  
Vol 124 (21) ◽  
pp. 2955-2955 ◽  
Author(s):  
Rena R. Xian ◽  
Genevieve M. Crane ◽  
Lisa M. Haley ◽  
Christopher D. Gocke ◽  
Ming-Tseh Lin ◽  
...  
Keyword(s):  
Gene Rearrangement ◽  
Cell Lymphoma ◽  
Clonal Evolution ◽  
B Cell Lymphoma ◽  
Late Relapse ◽  
Clonal Heterogeneity ◽  
Snp Microarray ◽  
Large B Cell Lymphoma ◽  
Igh Gene Rearrangement ◽  
Over Time

Abstract BACKGROUND: Diffuse large B cell lymphoma (DLBCL) is the most common non-Hodgkin lymphoma. While most relapses occur within 2 years, a small proportion of patients present with late relapse (LR) after 5 years. As there are very few studies addressing the pathobiology of LR-DLBCL, the aim of this study is to further characterize the clinical, pathologic and molecular features of these neoplasms. METHODS: A retrospective analysis of all patients with DLBCL treated at Johns Hopkins Hospital between 1984 and 2013 was performed. Patients with low-grade lymphoma at any time-point were excluded. Disease-free intervals (DFI) of 5 years or greater were designated as LR. Five paired diagnostic (D) and relapse (R) samples were available for further studies. DNA was extracted from formalin fixed paraffin embedded tissue. IGH gene rearrangement status was determined by PCR. SNP microarray was performed, and copy number variations (CNV) were defined as loss or gain of signal over at least 2 megabases. Targeted next generation sequencing (NGS) using a cancer hotspot panel was also performed. Variant calls were generated using Torrent variant caller and a laboratory-developed analysis pipeline. RESULTS: One hundred thirty-three patients with relapsed DLBCL were identified. Forty-three (32.3%) patients were diagnosed in the pre-rituximab era. One hundred fourteen (85.7%) patients had early relapse (ER) with 99 (74.4%) patients recurring within 2 years. Nineteen (14.3%) patients had LR (mean 7.9 years; median 7.3 years; up to 15.6 years). There were no significant differences in age at diagnosis, race, staging marrow status, or overall survival (OS) in ER versus LR patients. Extra-nodal presentation at diagnosis (89.5% vs. 65.8%; p = 0.04) and extra-nodal-only disease over time (73.7% vs. 48.2%; p = 0.04) were more common in LR cases. Both groups had similar rates of recurring at a different site from the original disease (79.3% vs. 89.5%; p = 0.30). Table 1. Molecular profile of paired D and R DLBCL Patient IGH clonality comparison (D vs. R) Clonal Heterogeneity (D / R) Total CNVs (D / R) Shared CNVs Unique CNVs (% of D / % of R) 1 Same + / + 24 / 21 15 37.5 / 28.6 2 Same + / + 15 / 32 11 26.7 / 65.6 3 Same + / + 32 / 15 7 78.1 / 53.3 4 2 in D / 1 persists in R + / + 8 / 20 1 87.5 / 95.0 5 Different - / + 4 / 5 0 100 / 100 The average DFI was 7.1 years in the 5 LR patients selected for additional studies. IGH gene rearrangement analysis demonstrated identical D and R IGH clones in 3 cases (Table 1). Patient 4 showed 2 rearranged alleles at D with only 1 persisting at R. Patient 5 had lymphomas with unique IGH rearrangements. SNP microarray data demonstrated the presence of clonal heterogeneity in all but 1 sample (4 of 5 at D; 5 of 5 at R). Among the 4 patients with clonally related IGH gene rearrangements, there was only partial overlap in CNVs (approximately 40% on average) between the D and R lymphomas. The average CNVs was similar in the D and R samples (16.6 vs. 18.6 respectively; p = 0.75). Chromosomes 2, 3, 6, 9, and 17 were frequently altered, and CNVs involving the BCL-6, CDKN2A, TP53, and MYC loci were also commonly seen; but there was no systematic difference between the CNVs identified at D and R. NGS showed a variety of mutations, but no consistent pattern of mutations acquired at R. There was a nonsense mutation in exon 2 of CDKN2A in the R sample in patient 1, and both D and R samples showed the same copy-neutral loss-of-heterozygosity of 9p encompassing the CDKN2A gene. In addition, missense mutations of TP53 were detected in patients 4 (only at R) and 5 (only at D). CONCLUSIONS: This study demonstrates that LR-DLBCL is an uncommon phenomenon with most cases representing recurrence of the original disease. LR patients have similar OS as ER patients, and the only clinical factors segregating LR from ER are higher rates of extra-nodal presentation and extra-nodal-only sites of disease. Although most paired D and R cases share IGH clones, there is clear evidence of clonal heterogeneity with clonal evolution over time. This suggests that DLBCL may contain minor subclones not susceptible to chemotherapy, which persist subclinically acquiring additional mutations over time eventually generating clinically-evident relapse. In rare cases, the late “relapse” may occur as an unrelated lymphoma that arises spontaneously or secondary to the mutagenic effects of chemotherapy. The precise mechanism of this long latency is yet unclear, and requires further investigation. Disclosures Borowitz: Becton Dickinson Biosciences: Research Funding.

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