scholarly journals Sarcoidosis-Lymphoma Syndrome Associated With Primary Thyroid Lymphoma: A Case Report

2021 ◽  
Vol 5 (Supplement_1) ◽  
pp. A900-A900
Author(s):  
Saori Yamashita ◽  
Yayoi Matsuda ◽  
Hiroki Muta ◽  
Toshihiko Nagao ◽  
Hiroshi Nakao ◽  
...  
Keyword(s):  
Thyroid Gland ◽  
B Cell ◽  
Clinical Symptoms ◽  
Cell Lymphoma ◽  
B Cell Lymphoma ◽  
High Grade ◽  
Thyroid Lymphoma ◽  
Chronic Thyroiditis ◽  
Primary Thyroid Lymphoma ◽  
High Grade Transformation

Abstract Background: Sarcoidosis is occasionally accompanied by hematologic malignancies, including lymphoma, called sarcoidosis-lymphoma syndrome. Although the mechanism underlying the induction of lymphomas is still unknown, understanding the immunological background of sarcoidosis could help explain the possible mechanisms of the induction of lymphomas. Case Presentation: A 52-year-old woman was diagnosed chronic thyroiditis with normal thyroid function. One year later, she underwent a screening chest radiograph and identified bilateral hilar adenopathy and mediastinum lymphadenopathy. Subsequent mediastinoscopy demonstrated sarcoidosis. Because of the lack of clinical symptoms, steroid treatment was not initiated and regular follow-up was performed. One and a half years after the diagnosis of chronic thyroiditis, she presented with rapid swelling of the thyroid gland. FDG-PET/CT showed intense uptake of FDG in the thyroid gland and multiple lymphadenopathy. Fine-needle aspiration (FNA) cytology of the thyroid gland was only suggestive of a lymphoproliferative disorder and did not provide a definitive diagnosis. Partial thyroidectomy was performed, and the pathology indicated diffuse large B-cell lymphoma (DLBCL) such as high-grade transformation of extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue (MALT) lymphoma. The results of an examination of a paraffin block histopathology specimen by fluorescence in-situ hybridization (FISH) detected BCL6 rearrangement (3q27), which is the most common chromosomal abnormality in DLBCL. After the treatment with R-EPOCH (rituximab, etoposide, vincristine, doxorubicin, cyclophosphamide, and prednisone) chemotherapy, the thyroid gland enlargement has improved markedly, while the lymph nodes remained swelling, that suggested lymph node involvements were due to sarcoidosis. Conclusions: Rapid swelling of the thyroid gland in the setting of chronic thyroiditis should raise suspicion for thyroid lymphoma. Furthermore, our present case might suggest that sarcoidosis accelerate the development and high-grade transformation of thyroid lymphoma. To our knowledge, this is the first reported case of sarcoidosis and primary thyroid lymphoma in the same patient.

High-grade Transformation of Low-grade B-cell Lymphoma

2016 ◽  
Vol 40 (1) ◽  
pp. e1-e16 ◽  
Author(s):  
Rose Lou Marie C. Agbay ◽  
Sanam Loghavi ◽  
L. Jeffrey Medeiros ◽  
Joseph D. Khoury
Keyword(s):  
B Cell ◽  
Cell Lymphoma ◽  
B Cell Lymphoma ◽  
Low Grade ◽  
High Grade ◽  
High Grade Transformation

scholarly journals FBXO11, a Regulator of BCL6 Stability, Is Recurrently Mutated in Burkitt Lymphoma

Blood ◽  
2015 ◽  
Vol 126 (23) ◽  
pp. 3673-3673
Author(s):  
Chiara Pighi ◽  
Mara Compagno ◽  
Qi Wang ◽  
Taek-Chin Cheong ◽  
Teresa Poggio ◽  
...  
Keyword(s):  
B Cell ◽  
Splice Site ◽  
Burkitt Lymphoma ◽  
Cell Lymphoma ◽  
Point Mutations ◽  
B Cell Lymphoma ◽  
Acceptor Site ◽  
Low Grade ◽  
High Grade ◽  
High Grade Transformation

Abstract INTRODUCTION: We recently described inactivating mutations of the FBXO11 gene in Diffuse Large B Cell Lymphoma (DLBCL). One major function of FBXO11 is to regulate BCL6 stability as well as other targets such as SNAIL. BCL6 acts as an oncogene in several human B-cell lymphomas and its expression levels can be increased by several mechanisms including chromosomal translocations, point mutations in the promoter region and reduced degradation by inactivation of FBXO11. Thus, FBXO11 acts as an oncosuppressor in DLBCL by promoting the accumulation of BCL6. However, a clear and complete picture of the distribution of FBXO11 mutations in different lymphoma subtypes is missing, and the in vivo functions of FBXO11 in normal tissue and lymphoma development are completely lacking. In the present work, we investigated the frequency and distribution of FBXO11 mutations in an extended panel of human BCL6 positive lymphoma and we functionally validated novel FBXO11 mutations for their ability to induce BCL6 degradation. METHODS: We sequenced the entire FBXO11 coding sequence by classical Sanger sequencing in 100 cases of Follicular Lymphoma (FL), 36 cases of Burkitt Lymphoma (BL), 8 BL cell lines and 8 Anaplastic Large cell lymphoma (ALCL) cell lines, which are typically BCL6-positive lymphomas. Moreover, we sequenced 50 cases of Marginal Zone B cell Lymphoma (MZL), which show variable expression of BCL6. To investigate whether the FBXO11 mutations interfere with FBXO11 activity we generated complementary DNA constructs containing these mutations. Wild-type FBXO11 or FBXO11 mutants were expressed in HEK-293T cells with constructs expressing BCL6 or SNAIL. Twenty-four hours after transfection, HEK-293T cells were treated with cycloheximide and harvested at different time points to check substrates expression and degradation by immunoblotting. RESULTS: BL carried the highest frequency of FBXO11 mutations (12/44 cases analyzed, 27%), whereas FL and MZL were very rarely affected by FBXO11 mutations (1/100 FL and 1/50 MZL). The analysis identified several mutations, either located in the coding sequence mostly in the CASH (functional) domains or located in intronic sequences controlling exon splicing. Sequence changes included missense mutations (n=8), deletions (n=2), a frameshift deletion introducing a premature stop codon (n=1) and nucleotide substitutions at consensus splice donor sites (n=2) and acceptor site (n=1). Remarkably, these last mutations represent the first report of splice site mutations affecting the FBXO11 gene. By cDNA amplification and sequencing, we demonstrated skipping of entire exons: the splice site mutations located at the donor site of exon 15 and 19 lead to the loss of the exon 15 and 19 respectively, while the splice site mutation affecting the acceptor site of exon 16 results in the deletion of the adjacent exon 16. Two of the point mutations (K631N and Y122C), affecting one case of FL and MZL respectively, were also found in the previous panel of DLBCLs (Duan et al, Nature 2012). Both cases consisted of a low grade component adjacent to an area of high grade transformation. By microdissection, we separately studied the two components of each of these cases to demonstrate that the mutation was present only in the high grade component. Finally, to validate all the mutations found in BL, we generated mutant constructs corresponding to each of them. By this approach, we showed that all the mutations identified are loss of function variants because they impaired the FBXO11 ability to promote BCL6 and SNAIL degradation. CONCLUSIONS: We identified several FBXO11 novel mutations in a large panel of BCL6-positive human lymphomas. All the mutations identified were inactivating the FBXO11 ability to induce BCL6 and SNAIL degradation. In contrast, mutations of FBXO11 are rare in low grade FL and MZL and, when present, are associated with high grade transformation. Together with our previous findings, this study showed that FBXO11 is mostly mutated in aggressive lymphomas such as DLBCL and BL, thus suggesting that FBXO11 mutations could contribute to the de-novo onset or transformation into high grade lymphoma. Disclosures No relevant conflicts of interest to declare.

scholarly journals Primary Thyroid Lymphoma

2012 ◽  
Vol 27 (1) ◽  
pp. 38-40
Author(s):  
Claudine Ann Musngi-Paras ◽  
Ansarie P. Salpin ◽  
Januario D. Veloso
Keyword(s):  
B Cell ◽  
Marginal Zone ◽  
Cell Lymphoma ◽  
B Cell Lymphoma ◽  
Lymphoid Cells ◽  
Follicular Epithelium ◽  
Thyroid Lymphoma ◽  
Large B Cell Lymphoma ◽  
Primary Thyroid Lymphoma ◽  
Large B Cell

Eight cases of primary thyroid lymphoma were reported in a tertiary government hospital from January 2005 to August 2011. All patients presented with a diffuse enlargement of both thyroid lobes with associated obstructive symptoms. Five of these cases were extranodal marginal zone lymphoma and three were diffuse large B-cell lymphoma. Clinical features that would favor a thyroid lymphoma include tumor size of greater that 7cm, obstructive symptoms, clinical hypothyroidism or history of Hashimoto thyroiditis. Thus, these features must be considered in evaluating thyroid nodules during fine-needle aspiration biopsy. Histologically, extranodal marginal zone B-cell lymphoma shows vaguely nodular to diffuse infiltrates of small to intermediate size atypical lymphoid cells infiltrating the thyroid follicles, while diffuse large B-cell lymphoma shows sheets of large atypical lymphoid cells infiltrating the thyroid follicular epithelium. Keywords: Primary Thyroid Lymphoma, Extranodal Marginal Zone B-cell Lymphoma, Diffuse Large B-cell Lymphoma.

scholarly journals Primary thyroid lymphoma with double expression of MYC and B-cell lymphoma-2 Gene

2021 ◽  
Vol 7 (5) ◽  
pp. 217-219
Author(s):  
Ramesh Mundle ◽  
Kunal Ajay Patankar ◽  
Jignesh Rajguru ◽  
Mohan Paliwal
Keyword(s):  
B Cell ◽  
Cell Lymphoma ◽  
B Cell Lymphoma ◽  
Thyroid Lymphoma ◽  
Primary Thyroid Lymphoma

scholarly journals Primary Thyroid Lymphoma: Could Surgery Be Avoided

2021 ◽  
Vol 5 (Supplement_1) ◽  
pp. A897-A897
Author(s):  
Romena Laukiene ◽  
Karolina Miseviciute
Keyword(s):  
B Cell ◽  
Cell Lymphoma ◽  
B Cell Lymphoma ◽  
Submandibular Salivary Gland ◽  
Thyroid Malignancy ◽  
Thyroid Lymphoma ◽  
Large B Cell Lymphoma ◽  
Airway Pathology ◽  
Primary Thyroid Lymphoma ◽  
Large B Cell

Abstract Background: Primary thyroid lymphoma is a rare thyroid disease that makes up only 1 to 5% of all thyroid oncological disorders. The average patient with primary thyroid lymphoma is a woman in her sixth or seventh decade with a history of Hashimoto’s thyroiditis. Clinical Case: 28-year-old woman complained of hoarseness, rapidly enlarging mass of the neck. She was referred to an otorhinolaryngologist by her family physician who suspected upper airway pathology. Otorhinolaryngologist observed swelling of patients’ larynx and prescribed treatment for suspected bacterial larynx infection. Symptoms kept worsening despite of treatment and patient was referred to an endocrinologist for a consultation. Blood lab tests were unremarkable. Ultrasound of the thyroid was performed which revealed a large (4,5 cm), hypoechoic, solid, homogenous node with Doppler signs of increased intranodular vascularity. Additionally, enlarged submandibular salivary gland lymph nodes on both sides of the neck were observed. FNAC (fine-needle aspiration cytology) was performed to diagnose possible thyroid malignancy, however findings showed atypia of undetermined significance (3rd category of Bethesda classification), to differentiate from lymphocytic thyroiditis. Because of high risk of malignancy, it was decided to perform thyroidectomy. During surgery, urgent intraoperative biopsy revealed undifferentiated thyroid carcinoma. As radical tumor extirpation due to prominent surrounding fibrosis was impossible, then only one side lobectomy was performed. Final histological examination revealed large B cell lymphoma, phenotype: CD20+, BCL6+, MuM1+, CD21+, cMyc-, BCL2-, CD10-, CD30-, Ki-67 up to 97%. More accurate disease staging was performed post-operatively, PET scan and computed tomography revealed disseminated primary thyroid lymphoma. Final diagnosis was Stage IV primary large B cell lymphoma of the thyroid. Patient was treated with chemotherapy according to Rx7-CHOP14x6 (Rituximab, cyclophosphamide, doxorubicin, vincristine, prednisone) protocol. The treatment was tolerated well, 3 months after, complete remission of the disease was observed. During outpatient visits patient remained in remission for all 5 years of planned regular check-ups. Conclusion: This case demonstrates the diagnostic challenge of primary thyroid lymphoma. In the presence of rapidly enlarging thyroid mass, thyroid lymphoma is not usually suspected. FNAC as golden standard of thyroid malignancy often does not allow differentiation of this pathology. In this case, a core biopsy could have helped to make correct preoperative diagnosis and to avoid unnecessary surgery.

scholarly journals Diffuse large B cell lymphoma of thyroid as a masquerader of anaplastic carcinoma of thyroid, diagnosed by FNA : A case report

CytoJournal ◽  
2006 ◽  
Vol 3 ◽  
pp. 23 ◽  
Author(s):  
Yahya Daneshbod ◽  
Shapour Omidvari ◽  
Khosrow Daneshbod ◽  
Shahrzad Negahban ◽  
Mehdi Dehghani
Keyword(s):  
Case Report ◽  
B Cell ◽  
Cell Lymphoma ◽  
B Cell Lymphoma ◽  
Anaplastic Thyroid Carcinoma ◽  
Anaplastic Carcinoma ◽  
High Grade ◽  
Thyroid Lymphoma ◽  
Large B Cell Lymphoma ◽  
Large B Cell

Background: Both thyroid lymphoma and anaplastic carcinoma of thyroid present with rapidly growing mass in eldery patients. Anaplastic carcinoma has high mortality rate and combination of surgery, radiation therapy and multidrug chemotherapy are the best chance for cure. Prognosis of thyroid lymphoma is excellent and chemotherapy for widespred lymphoms and radiotherapy with or without adjuvant chemotherapy for tumors localized to the gland, are the treatment of choice. Case report: This article reports a 70 year old man presenting with diffuse neck swelling and hoarseness of few weeks duration. Fine needle aspiration was done and reported as anaplastic carcinoma of thyroid which thyroidectomy was planned. The slides were sent for second opinion. After review, with initial diagnosis of anaplastic carcinoma versus lymphoma, immunocytochemical study was performed. Smears were positive for B cell markers and negative for cytokeratin, so with the impression of diffuse large B cell lymphoma, the patient received two courses of chemotherapy by which the tumor disappeared during two weaks. Conclusion: Despite previous reports, stating easy diagnosis of high-grade thyroid lymphoma on the grounds of cytomorphological features we like to emphasize, overlapping cytologic features of the curable high grade thyroid lymphoma form noncurable anaplastic thyroid carcinoma and usefulness of immunocytochemistry to differentiate these two disease.

High Grade Transformation in Splenic Marginal Zone Lymphoma: A Description of a Series of 8 Cases.

Blood ◽  
2006 ◽  
Vol 108 (11) ◽  
pp. 4599-4599
Author(s):  
Moez R. Dungarwalla ◽  
Andrew Wotherspoon ◽  
Gareth Morgan ◽  
Daniel Catovsky ◽  
Claire E. Dearden ◽  
...  
Keyword(s):  
Bone Marrow ◽  
B Cell ◽  
Cell Lymphoma ◽  
B Cell Lymphoma ◽  
Low Grade ◽  
Splenic Marginal Zone Lymphoma ◽  
High Grade ◽  
Large B Cell Lymphoma ◽  
High Grade Transformation ◽  
Large B Cell

Abstract Splenic marginal zone lymphoma (SMZL) without or with villous lymphocytes (SLVL) is a low grade B cell non-Hodgkin’s lymphoma that comprises <1% of lymphoid malignancies. SLVL is indistinguishable histologically from SMZL and it is believed that the 2 entities represent different phases of the same process in the spleen and the bone marrow. Characteristic features of SMZL are splenomegaly, moderate lymphocytosis, immunophenotype with a CLL score < 2 and nodular/intrasinusoidal pattern of bone marrow infiltration. Despite its slow clinical course, and response to splenectomy, approximately 50% of deaths in patients with SMZL are caused by progressive disease. As in other low grade lymphoproliferative disorders, SMZL may undergo high grade transformation. We describe 8 cases of SMZL that showed clinical, morphological and histological features of progression to large B cell lymphoma. These 8 cases represent 19% of our series of 41 SMZL patients followed up between 1996 and 2006. Patients were aged between 44 and 76 years, and there were 4 men and 4 women. Progression to high grade B cell lymphoma occurred between 1 and 48 months from diagnosis. The cases can be divided into 2 distinct groups on the basis of the site of transformation. Those cases with high grade transformation in the bone marrow had a median overall survival of 10 months, although 1 patient did attain a complete remission with combination chemotherapy (R-CHOP) and is alive at 12 months. In contrast, there were 3 cases of transformation in peripheral lymph nodes with a median overall survival of 60 months. Large B cell lymphoma was also diagnosed in the spleen in the initial stages of the disease in 1 case. This patient achieved a complete remission with R-CHOP post splenectomy, and is still alive at 36 months follow up. In a previous report of transformation to large B cell lymphoma in SMZL (Camacho et al, Am J Surg Pathol 2001) a 13% incidence was reported. In our series of 8 cases the incidence of transformation is 19%. Hence it appears that while the incidence of large B cell transformation seems to be higher than in CLL (1–10%) it is lower than that reported in follicular lymphoma (25–60%). Two of the 4 cases of high grade transformation in the bone marrow had previously been treated with fludarabine in the year prior to their progression to large B cell lymphoma. In 1 of these cases Epstein Barr Virus encoded RNA was detected in the malignant cells at the time of transformation. This may suggest that EBV is involved in the pathology of SMZL transformation, and treatment with immunosuppressive agents such as the purine analogues, may increase this risk. In our series of 41 patients, 6 have received fludarabine therapy and the risk of high grade transformation in this small group is significantly higher at 33% (2/6).

Diffuse Large B-cell Lymphoma of thyroid gland in an Adolescent girl with Hashimoto’s thyroiditis

2020 ◽  
Author(s):  
Mara Xatzipsalti ◽  
Evangelos Bourousis ◽  
Maria Nikita ◽  
Myrsini Gkeli ◽  
Evgenia Magkou ◽  
...  
Keyword(s):  
Thyroid Gland ◽  
B Cell ◽  
Hashimoto’S Thyroiditis ◽  
Adolescent Girl ◽  
Cell Lymphoma ◽  
B Cell Lymphoma ◽  
Hashimoto's Thyroiditis ◽  
Large B Cell Lymphoma ◽  
Large B Cell

Metody cytogenetyki molekularnej w różnicowaniu agresywnych B-NHL

2019 ◽  
Vol 50 (3) ◽  
pp. 109-115
Author(s):  
Beata Grygalewicz
Keyword(s):  
B Cell ◽  
Cell Lymphoma ◽  
B Cell Lymphoma ◽  
World Health ◽  
High Grade ◽  
Non Hodgkin Lymphoma ◽  
Large B Cell Lymphoma ◽  
Health Organization ◽  
Large B Cell

StreszczenieB-komórkowe agresywne chłoniaki nieziarnicze (B-cell non-Hodgkin lymphoma – B-NHL) to heterogenna grupa nowotworów układu chłonnego, wywodząca się z obwodowych limfocytów B. Aberracje cytogenetyczne towarzyszące B-NHL to najczęściej translokacje onkogenów takich jak MYC, BCL2, BCL6 w okolice genowych loci dla łańcuchów ciężkich lub lekkich immunoglobulin. W niektórych przypadkach dochodzi do wystąpienia kilku wymienionych aberracji jednocześnie, tak jak w przypadkach przebiegających z równoczesną translokacją genów MYC i BCL2 (double hit), niekiedy także z obecnością rearanżacji BCL6 (triple hit). Takie chłoniaki cechuje szczególnie agresywny przebieg kliniczny. Obecnie molekularna diagnostyka cytogenetyczna przy użyciu techniki fluorescencyjnej hybrydyzacji in situ (FISH) oraz, w niektórych przypadkach, aCGH jest niezbędnym narzędziem rozpoznawania, klasyfikowania i oceny stopnia zaawansowania agresywnych, nieziarniczych chłoniaków B-komórkowych. Technika mikromacierzy CGH (aCGH) była kluczowym elementem wyróżnienia prowizorycznej grupy chłoniaków Burkitt-like z aberracją chromosomu 11q (Burkitt-like lymphoma with 11q aberration – BLL, 11q) w najnowszej klasyfikacji nowotworów układu chłonnego Światowej Organizacji Zdrowia (World Health Organization – WHO) z 2016 r. Omówione zostaną sposoby różnicowania na poziomie cytogenetycznym takich chłoniaków jak: chłoniak Burkitta (Burkitt lymphoma – BL), chłoniak rozlany z dużych komórek B (diffuse large B-cell lymphoma – DLBCL) oraz 2 nowych jednostek klasyfikacji WHO 2016, czyli chłoniaka z komórek B wysokiego stopnia złośliwości z obecnością translokacji MYC i BCL2 i/lub BCL6 (high-grade B-cell lymphoma HGBL, with MYC and BCL2 and/or BCL6 translocations) oraz chłoniaka BLL, 11q.

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