Duodenal-Type Follicular Lymphoma: A Newly Recognized Entity in the 2016 World Health Organization Classification Update

2020 ◽  
Vol 154 (Supplement_1) ◽  
pp. S61-S61
Author(s):  
F Rajack ◽  
L Taddasse-Heath ◽  
T J Naab
Keyword(s):  
Small Bowel ◽  
Follicular Lymphoma ◽  
Duodenal Bulb ◽  
World Health ◽  
Deficiency Anemia ◽  
Chain Gene ◽  
Low Grade ◽  
Sessile Polyp ◽  
Term Survival ◽  
Health Organization

Abstract Introduction/Objective Most cases of primary follicular lymphoma (FL) in the gastrointestinal tract occur in the second part of the duodenum, presenting with multiple small polyps. Duodenal type follicular lymphoma (D-FL) is a newly recognized entity in the 2016 WHO classification update. Methods Upper endoscopy in a 72 year old female with iron deficiency anemia revealed a single 8 mm sessile polyp with no bleeding in the duodenal bulb. Histology revealed dense infiltrate of small mature lymphocytes with a nodular growth pattern. Follicles, composed predominantly of centrocytes with scattered centroblasts, diffusely expressed CD20, CD10 and BCL-2. Results This immunoprofile characterizes follicular lymphoma. Real-time polymerase chain restriction assay detected BCL-2 MBR/JH DNA fusion sequence, usually resulting from t(14;18)(q32;q21) translocation and confirming D-FL. D- FL is often asymptomatic, discovered incidentally at endoscopy. It is usually low grade (grade 1–2), presenting at low stage, with immunophenotype and t(14;18)(q32;q21) similar to other FLs. However, in comparison to nodal FL, D-FL has higher selective use of V4 and V5 immunoglobulin heavy chain gene rearrangements, which has been linked to antigen dependent mechanisms in lymphoma development; this feature is shared with Mucosa-Associated Lymphoid Tissue (MALT) lymphoma. Conclusion D-FL often remains localized to the small bowel and has a good outcome even with local small bowel recurrences. It has a long term survival of >12 years. There is a low (<10%) risk of progression to nodal disease and given the indolent clinical course, a watch-and-wait approach is reasonable for most patients.

scholarly journals Genetic Abnormalities, Clonal Evolution, and Cancer Stem Cells of Brain Tumors

2018 ◽  
Vol 6 (4) ◽  
pp. 85 ◽  
Author(s):  
Ugo Testa ◽  
Germana Castelli ◽  
Elvira Pelosi
Keyword(s):  
Brain Tumors ◽  
Clonal Evolution ◽  
Pediatric Brain Tumors ◽  
Genetic Alterations ◽  
World Health ◽  
Driver Mutations ◽  
Low Grade ◽  
High Grade ◽  
Genetic Profiling ◽  
Health Organization

Brain tumors are highly heterogeneous and have been classified by the World Health Organization in various histological and molecular subtypes. Gliomas have been classified as ranging from low-grade astrocytomas and oligodendrogliomas to high-grade astrocytomas or glioblastomas. These tumors are characterized by a peculiar pattern of genetic alterations. Pediatric high-grade gliomas are histologically indistinguishable from adult glioblastomas, but they are considered distinct from adult glioblastomas because they possess a different spectrum of driver mutations (genes encoding histones H3.3 and H3.1). Medulloblastomas, the most frequent pediatric brain tumors, are considered to be of embryonic derivation and are currently subdivided into distinct subgroups depending on histological features and genetic profiling. There is emerging evidence that brain tumors are maintained by a special neural or glial stem cell-like population that self-renews and gives rise to differentiated progeny. In many instances, the prognosis of the majority of brain tumors remains negative and there is hope that the new acquisition of information on the molecular and cellular bases of these tumors will be translated in the development of new, more active treatments.

scholarly journals Well-Differentiated Liposarcoma of The Larynx: A Case Report and Review of Literature

2016 ◽  
Vol 9 (1) ◽  
pp. 85-89
Author(s):  
Svetlana A. Mateva ◽  
Margarita R. Nikolova ◽  
Alexandar V. Valkov ◽  
Margarita R. Nikolova
Keyword(s):  
Case Report ◽  
Soft Tissue ◽  
Soft Tissue Sarcomas ◽  
World Health ◽  
Low Grade ◽  
Review Of Literature ◽  
Grade Malignancy ◽  
Well Differentiated ◽  
Health Organization ◽  
Histologic Types

Summary Liposarcoma is one of the most common soft tissue sarcomas in adults with a relative incidence amongst other sarcomas ranging from 9.8% to 16%. It usually locates in the limbs and retroperitoneum. Primary liposarcomas of the larynx and hypopharynx are rare, comprising less than 20% of all head and neck liposarcomas. According to World Health Organization, these tumors are divided into four histologic types, and well-differentiated liposarcoma is the most common one. It is a tumor of low-grade malignancy that may recur locally, but does not metastasize. We present a case of laryngopharyngeal well- differentiated liposarcoma in an old patient with two previous removals. We also discuss recently published cases with this unusual location of liposarcoma.

miR-9-1 Is a New Circulating Biomarker for Higher-grade Meningioma Regulated via the EGFR-FOS Network

2020 ◽  
Author(s):  
Daniele Baiz ◽  
Caterina Negroni ◽  
Sara Ferluga ◽  
Emanuela Ercolano ◽  
Claire L Adams ◽  
...  
Keyword(s):  
Tumor Grade ◽  
World Health ◽  
Tumor Growth Rate ◽  
Low Grade ◽  
Serum Samples ◽  
Circulating Biomarker ◽  
Mirna Array ◽  
Malignant Grade ◽  
Health Organization

Abstract Background: Meningiomas are the most common primary CNS tumors. According to the World Health Organization Classification (WHO), they are classified as benign (grade I), atypical (grade II), and anaplastic/malignant (grade III). Chemotherapy has proven ineffective in treating these tumors, which are primarily managed by surgery, radiotherapy, or a combination of them. Morbidity and mortality correlate with meningioma grade. Currently, risk assessment for treatment is based on the radiological assessment of tumor size, tumor growth rate, and/or clinical progression of symptoms. Methods: We performed a cancer miRNA array in an in vitro model of meningioma in order to identify circulating biomarkers in meningioma patients. We validated the miRNA biomarker candidate in cells and tissues and analyzed its regulation. We then investigated expression in tissues and blood. Results: We identified miR-9-1 as significantly overexpressed in atypical and anaplastic cells compared to benign. We further demonstrated that miR-9-1 overexpression is due to increased levels of FOS via upregulation of the EGFR receptor, and showed that miR-9-1 and FOS are upregulated in a cohort of higher-grade meningioma biopsies. Next, we isolated circulating exosomes from meningioma patients’ serum samples, and found higher levels of miR-9-1 in higher-grade compared to low-grade meningiomas patients. Conclusions: Overall, our study shows overexpression and the mechanism of miR-9-1 regulation and suggests miR-9-1 as a novel circulating biomarker candidate to identify tumor grade in meningioma.

Management for Different Glioma Subtypes: Are All Low-Grade Gliomas Created Equal?

2019 ◽  
pp. 133-145 ◽  
Author(s):  
Martin C. Tom ◽  
Daniel P. Cahill ◽  
Jan C. Buckner ◽  
Jörg Dietrich ◽  
Michael W. Parsons ◽  
...  
Keyword(s):  
Molecular Genetic ◽  
World Health ◽  
Low Grade ◽  
Lower Grade ◽  
Future Directions ◽  
Molecular Alterations ◽  
Lower Grade Glioma ◽  
Grade 3 ◽  
Health Organization

Following the identification of key molecular alterations that provided superior prognostication and led to the updated 2016 World Health Organization (WHO) Central Nervous System (CNS) Tumor Classification, the understanding of glioma behavior has rapidly evolved. Mutations in isocitrate dehydrogenase (IDH) 1 and 2 are present in the majority of adult grade 2 and 3 gliomas, and when used in conjunction with 1p/19q codeletion for classification, the prognostic distinction between grade 2 versus grade 3 is diminished. As such, the previously often used term of “low-grade glioma,” which referred to grade 2 gliomas, has now been replaced by the phrase “lower-grade glioma” to encompass both grade 2 and 3 tumors. Additional molecular characterization is ongoing to even further classify this heterogeneous group of tumors. With such a colossal shift in the understanding of lower-grade gliomas, management of disease is being redefined in the setting of emerging molecular-genetic biomarkers. In this article, we review recent progress and future directions regarding the surgical, radiotherapeutic, chemotherapeutic, and long-term management of adult lower-grade gliomas.

scholarly journals Succinate Dehydrogenase–Deficient Renal Cell Carcinoma

2018 ◽  
Vol 143 (5) ◽  
pp. 643-647 ◽  
Author(s):  
Tsung-Heng Tsai ◽  
Wen-Ying Lee
Keyword(s):  
Renal Cell Carcinoma ◽  
Cell Carcinoma ◽  
Succinate Dehydrogenase ◽  
Renal Cell ◽  
World Health ◽  
Low Grade ◽  
Long Term Follow Up ◽  
Health Organization

Succinate dehydrogenase (SDH)–deficient renal cell carcinoma is a recently recognized distinct subtype of renal cell carcinoma in the 2016 World Health Organization classification. It is associated with SDH gene germline mutations, which also cause paraganglioma/pheochromocytoma, gastrointestinal stromal tumor, and pituitary adenoma. The tumor most commonly presents in young adulthood. The tumors are arranged in solid nests or in tubules and frequently show cystic change. The tumors are composed of cuboidal to oval cells with round nuclei, dispersed chromatin, and inconspicuous nucleoli. The cytoplasm is eosinophilic or flocculent but not truly oncocytic. The most distinctive histologic feature is the presence of cytoplasmic vacuoles or inclusions. Loss of SDH subunit B immunostaining is needed for a definite diagnosis. The prognosis is good for low-grade tumors but worse for tumors with high-grade nuclei, sarcomatoid change, or coagulative necrosis. Long-term follow-up is indicated.

Accuracy of Intraoperative Examination in Central Nervous System Lesions: A Study of 133 Cases

2019 ◽  
Vol 63 (3) ◽  
pp. 224-232
Author(s):  
Ludmila Barbosa de Souza Balsimelli ◽  
Jamille Costa de Oliveira ◽  
Flora Ávila Adorno ◽  
Clarissa Almeida Brites ◽  
Giuliano Stefanello Bublitz ◽  
...  
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Frozen Section ◽  
Final Diagnosis ◽  
World Health ◽  
Histopathological Diagnosis ◽  
Low Grade ◽  
Predictive Values ◽  
Health Organization ◽  
Cns Lesions

Objective: Intraoperative examination is a highly valuable tool for the evaluation of central nervous system (CNS) lesions, helping the neurosurgeon to determine the best surgical management. This study aimed to evaluate the accuracy and to analyze the diagnostic disagreements and pitfalls of the intraoperative examinations through correlation with the final histopathological diagnosis in CNS lesions. Study Design: Retrospective analysis of intraoperative examination of CNS lesions and their final diagnosis obtained during 16 consecutive years. All diagnoses were reviewed and classified according to World Health Organization (WHO) grading for CNS tumors. Squash was performed in 119 cases, while frozen section and both methods were done in 7 cases each. Results: Among the 133 intraoperative examinations considered, 114 (85.7%) presented concordance and 19 (14.3%) diagnostic disagreement when compared with subsequent histopathological examinations. The sensitivity and specificity for the detection of neoplasia in intraoperative examination was 98 and 94%, respectively. The positive and negative predictive values were 99 and 88%, respectively. The accuracy for neoplastic and nonneoplastic disease was 85.7%. Disagreements were more frequent among low-grade (WHO grades I and II) neoplasms and nonmalignant cases. Conclusions: Our results showed good accuracy of the intraoperative assessments for diagnosis of CNS lesions, particularly in high-grade (grades III and IV) lesions and metastatic neoplasms.

scholarly journals Gastric mixed adenoneuroendocrine carcinoma case report

2019 ◽  
Vol 7 ◽  
pp. 2050313X1982891
Author(s):  
Miguel Angel Moyón Constante ◽  
Fernando Xavier Moyón Constante ◽  
Jorge Fernando Tufiño ◽  
Andres Cárdenas Patiño ◽  
Gabriel Alejandro Molina ◽  
...  
Keyword(s):  
Case Report ◽  
World Health ◽  
Low Grade ◽  
Radical Gastrectomy ◽  
Upper Endoscopy ◽  
Mixed Adenoneuroendocrine Carcinoma ◽  
First Case ◽  
The World ◽  
Medical Teams ◽  
Health Organization

Mixed adenoneuroendocrine carcinomas are rare tumors that contain both an exocrine and an endocrine component. Since the latest classification by the World Health Organization and with the aid of immunostaining, more mixed adenoneuroendocrine carcinomas are now identified and diagnosed. Nonetheless, our knowledge of these tumors is still limited, notably concerning gastric variants, as the cases reported in the literature are very limited. The clinical and surgical treatment, including the chemotherapy schemes, the prognosis, and recurrence still represent challenges for the medical teams. We present the case of a 62-year-old woman. After an upper endoscopy revealed multiple polyps and a low-grade neuroendocrine tumor, a D2 radical gastrectomy was performed. A low output esophageal anastomotic leak was discovered in the postoperative period and successfully managed. Pathology revealed a gastric mixed adenoneuroendocrine carcinoma, the first case of this kind reported in Ecuador. Patient is doing well and under constant surveillance up until her 13th postoperative month.

scholarly journals MYC rearrangements in histologically progressed follicular lymphomas

Blood ◽  
1992 ◽  
Vol 80 (3) ◽  
pp. 758-767 ◽  
Author(s):  
T Yano ◽  
ES Jaffe ◽  
DL Longo ◽  
M Raffeld
Keyword(s):  
Follicular Lymphoma ◽  
Molecular Organization ◽  
Aggressive Lymphoma ◽  
Chain Gene ◽  
Low Grade ◽  
Immunoglobulin Gene ◽  
High Grade ◽  
Light Chain Gene ◽  
Myc Gene ◽  
Follicular Lymphomas

Abstract Histologic transformation of low-grade follicular lymphoma to an aggressive-grade lymphoma occurs in 60% to 80% of patients during their clinical course. The events that drive the transformation process are poorly understood. Deregulation of the MYC gene has been implicated in a small number of cases. This observation led us to examine the molecular organization of the MYC oncogene in 38 cases of histologically transformed lymphomas that arose from follicular lymphomas, and in 18 of the initial pretransformation follicular lymphomas. In addition, we examined 58 “control” low-grade follicular lymphomas that had not yet shown evidence of histologic progression. Immunoglobulin heavy chain and light chain gene rearrangements were detected in all biopsies and rearrangements of the BCL-2 locus were seen in 36 of 38 of the transformed lymphomas (consistent with their origin from follicular lymphomas), in 18 of 18 of the pretransformation follicular lymphomas, and in 51 of 58 of the control follicular lymphomas. All 18 pretransformation follicular lymphoma specimens displayed at least one immunoglobulin gene and BCL-2 rearrangement in common with the corresponding histologically progressed lymphoma, indicating a clonal relationship between the original follicular lymphoma and the histologically transformed lymphoma. MYC rearrangements were detected in 3 of 38 (8%) transformed lymphomas and in 1 of 58 (2%) control follicular lymphomas. The latter MYC rearranged follicular lymphoma was clinically aggressive and transformed to a high- grade lymphoma that led to the death of the patient within 20 months. None of the 18 pretransformation follicular lymphomas showed MYC rearrangement, including two from patients who later demonstrated MYC rearrangement in the progressed aggressive lymphoma. PvuII mutational analysis failed to identify additional MYC gene abnormalities in the progressed lymphomas. Because the Epstein-Barr virus (EBV) is associated with a fraction of high-grade lymphomas and is known to upregulate BCL-2, we looked for a potential role for this agent in our progressed lymphomas. We did not detect viral sequences in any case indicating that EBV does not play a major role in progression. The presence of MYC rearrangements in a small fraction of progressed aggressive lymphomas, and not in the corresponding antecedent follicular lymphomas, suggests that acquisition of a MYC rearrangement is in some cases associated with the transformation event.

Simultaneous Phenotypically Distinct but Clonally Identical Mucosa-Associated Lymphoid Tissue and Follicular Lymphoma in a Patient With Sjögren’s Syndrome

Blood ◽  
1999 ◽  
Vol 94 (7) ◽  
pp. 2247-2251 ◽  
Author(s):  
Antonella Aiello ◽  
Ming-Qing Du ◽  
Tim C. Diss ◽  
Huai-Zheng Peng ◽  
Francesco Pezzella ◽  
...  
Keyword(s):  
Lymph Node ◽  
Parotid Gland ◽  
Follicular Lymphoma ◽  
Malt Lymphoma ◽  
Lymphoid Tissue ◽  
Molecular Genetic ◽  
Pcr Amplification ◽  
Pcr Analysis ◽  
Chain Gene ◽  
Low Grade

A 44-year-old woman with a 12-year history of Sjögren’s syndrome (SS) developed a low-grade mucosa-associated lymphoid tissue (MALT) lymphoma in the parotid gland. Two years later, she presented with generalized lymphadenopathy and hepatosplenomegaly and a follicular lymphoma was diagnosed. To investigate the relationship of the two histologically distinct lymphomas, we re-examined their histology and immunophenotype and studied the lymphomatous tissue from the parotid, cervical lymph node, and spleen using molecular genetic methods. Histologic and immunophenotypic studies confirmed the previous diagnoses and also identified a previously unnoticed focus of follicular lymphoma in the second parotid gland biopsy. Polymerase chain reaction (PCR) amplification of the rearranged Ig heavy-chain gene showed the same sized dominant product in the MALT lymphoma and the follicular lymphoma. Similarly, PCR analysis of the t(14:18) translocation yielded an identical sized band from both MALT and follicular lymphoma. Cloning and sequencing of the Ig PCR products showed an identical CDR3 sequence from each lesion, indicating a common clonal lineage. The follicular lymphoma of the parotid gland lymph node and the follicular lymphoma of the spleen showed an identical mutation signature to that of the salivary gland MALT lymphoma. We propose that follicular lymphoma in the parotid gland lymph node may have resulted from colonization of lymphoid follicles by MALT lymphoma cells, following which the tumor cells were induced to express a follicular lymphoma phenotype, due to Bcl-2 overexpression caused by t(14;18), leading to a change in clinical behavior resulting in rapid widespread dissemination of disease. These observations suggest that the distinct phenotypes of low-grade B-cell lymphomas may be the consequence of interplay between genetic and local microenvironmental factors.

scholarly journals MYC rearrangements in histologically progressed follicular lymphomas

Blood ◽  
1992 ◽  
Vol 80 (3) ◽  
pp. 758-767 ◽  
Author(s):  
T Yano ◽  
ES Jaffe ◽  
DL Longo ◽  
M Raffeld
Keyword(s):  
Follicular Lymphoma ◽  
Molecular Organization ◽  
Aggressive Lymphoma ◽  
Chain Gene ◽  
Low Grade ◽  
Immunoglobulin Gene ◽  
High Grade ◽  
Light Chain Gene ◽  
Myc Gene ◽  
Follicular Lymphomas

Histologic transformation of low-grade follicular lymphoma to an aggressive-grade lymphoma occurs in 60% to 80% of patients during their clinical course. The events that drive the transformation process are poorly understood. Deregulation of the MYC gene has been implicated in a small number of cases. This observation led us to examine the molecular organization of the MYC oncogene in 38 cases of histologically transformed lymphomas that arose from follicular lymphomas, and in 18 of the initial pretransformation follicular lymphomas. In addition, we examined 58 “control” low-grade follicular lymphomas that had not yet shown evidence of histologic progression. Immunoglobulin heavy chain and light chain gene rearrangements were detected in all biopsies and rearrangements of the BCL-2 locus were seen in 36 of 38 of the transformed lymphomas (consistent with their origin from follicular lymphomas), in 18 of 18 of the pretransformation follicular lymphomas, and in 51 of 58 of the control follicular lymphomas. All 18 pretransformation follicular lymphoma specimens displayed at least one immunoglobulin gene and BCL-2 rearrangement in common with the corresponding histologically progressed lymphoma, indicating a clonal relationship between the original follicular lymphoma and the histologically transformed lymphoma. MYC rearrangements were detected in 3 of 38 (8%) transformed lymphomas and in 1 of 58 (2%) control follicular lymphomas. The latter MYC rearranged follicular lymphoma was clinically aggressive and transformed to a high- grade lymphoma that led to the death of the patient within 20 months. None of the 18 pretransformation follicular lymphomas showed MYC rearrangement, including two from patients who later demonstrated MYC rearrangement in the progressed aggressive lymphoma. PvuII mutational analysis failed to identify additional MYC gene abnormalities in the progressed lymphomas. Because the Epstein-Barr virus (EBV) is associated with a fraction of high-grade lymphomas and is known to upregulate BCL-2, we looked for a potential role for this agent in our progressed lymphomas. We did not detect viral sequences in any case indicating that EBV does not play a major role in progression. The presence of MYC rearrangements in a small fraction of progressed aggressive lymphomas, and not in the corresponding antecedent follicular lymphomas, suggests that acquisition of a MYC rearrangement is in some cases associated with the transformation event.

Sign in / Sign up

Export Citation Format

Share Document