scholarly journals Primary breast lymphoma of childhood: a case report and review of literature

2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Giulia A. Restivo ◽  
Marta Pillon ◽  
Lara Mussolin ◽  
Clara Mosa ◽  
Angela Guarina ◽  
...  

Abstract Background Primary breast lymphoma (PBL) is an extremely rare neoplasm in children; by definition, it manifests in the breast without evidence of lymphoma elsewhere, except ipsilateral axillary nodes. Case presentation We report a case of a 15-year-old girl diagnosed with diffuse large B-cell lymphoma (DLBCL) of the right breast: the patient received chemotherapy and rituximab, achieving complete remission. A literature review revealed other 11 cases of pediatric PBL; it mainly affects female adolescents and can involve right and left breast equally. Different histologic subtypes have been described, arising from both B-cell and T-cell. Therapeutic approaches were very different, from chemotherapy to local treatment with surgery and/or radiotherapy. Conclusions Our case is the first in which rituximab was administered, suggesting to be a promising therapy in B-cell PBL, as already demonstrated in pediatric B-cell lymphoma from other sites. Further investigations are needed to identify prognostic factors and establish the most effective treatment.

2021 ◽  
Vol 108 (Supplement_2) ◽  
Author(s):  
R Alam ◽  
B Basak ◽  
A Ahsan ◽  
A S Gupta ◽  
S Islam ◽  
...  

Abstract Primary breast lymphoma (PBL) is an unusual clinical entity accounting for 0.4–0.5% of all breast neoplasms. The usual presentation includes a painless palpable mass similar to that of breast carcinoma. Diffuse large B-cell lymphoma (DLBCL) is the most common identifiable type of PBL based on the histopathological examination. We report an unusual case of 22 years old Bangladeshi woman presented with a 6-month history of a lump on left breast. Although the lump was initially small, it began a rapid growth after 4 months. The swelling was localized and did not show any skin involvement or discharge and as she didn’t have any positive familial history of breast carcinoma her primary attending physician diagnosed it as a case of breast abscess. When local incision and drainage proved ineffective, she was referred to us. After doing an immunohistochemistry from incisional biopsy the diagnosis was confirmed as Diffuse Large B-cell Lymphoma. The patient was treated initially by chemotherapy with CHOP therapy followed by wide local excision. Early and accurate diagnosis of PBL is crucial for selecting the appropriate MDT treatment strategies to avert potentially harmful surgical interventions.


Blood ◽  
2004 ◽  
Vol 104 (11) ◽  
pp. 1371-1371 ◽  
Author(s):  
Davide Rossi ◽  
Eva Berra ◽  
Mirella Marino ◽  
Daniela Capello ◽  
Michaela Cerri ◽  
...  

Abstract Primary lymphoma of the breast accounts for <2% of extranodal non-Hodgkin’s lymphoma and generally displays morphology consistent with diffuse large B-cell lymphoma (DLBCL). The pathogenesis and histogenesis of primary breast lymphoma are currently unknown and represented the aim of this study. Thirteen cases of primary breast lymphoma (all DLBCL) were analyzed for physiological somatic hypermutation (SHM) of IgV and BCL-6 genes, as well as for aberrant SHM of PAX-5, PIM-1, RhoH/TTF and c-MYC genes. A functional IgVH rearrangement was identified in 8/13 (61.5%) primary breast lymphomas. All IgVH genes displayed SHM, with a mutation frequency ranging from 4.0% to 25.9%, suggesing that primary breast lymphomas derive from GC-related B-cells. The IgVH gene families utilized included VH4 (4/8 cases), VH3 (3/8 cases) and VH2 (1/8 case). Three cases utilized the same VH 4.30.1/4-31 gene. Mutations of BCL-6 were detected in 7/13 (53.8%) cases, further confirming the finding that primary lymphomas of the breast derive from GC-experienced B cells. Analysis of aberrant SHM of proto-oncogenes was performed on selected regions known to contain >90% of mutations found in lymphoma. Overall, mutations in at least one of the four proto-oncogenes targeted by aberrant SHM were found in 9/13 (69%) primary breast lymphomas, whereas mutations in more than one gene were found in 4/9 (44%) cases. Each of the four proto-oncogenes was altered in a significant fraction of primary breast lymphomas (PAX-5 in 4/9 cases; RhoH/TTF in 5/9 cases; PIM-1 in 5/9 cases and c-MYC in 2/9 cases). The overwhelming majority of mutations was represented by single base-pair substitutions (n=36), whereas in only one instance a deletion of a short DNA stretch was observed. Among the 36 single base-pair substitutions detected in primary breast lymphoma, the transition/transversion ratio was 1.76 (expected 0.5; p<0.01; Chi square test), reflecting the mutational profile seen in nodal DLBCL of immunocompetent hosts. In PIM-1, a fraction of mutations led to amino acid substitution, with potential functional consequences. In particular, three primary breast lymphomas displayed four missense mutations localized within the serine-threonine kinase domain of PIM-1. The association of primary breast lymphoma with aberrant SHM of proto-oncogenes expands the types of aggressive lymphomas marked by this molecular abnormality and provides clues for understanding breast lymphoma pathogenesis. In particular, missense mutations in the PIM-1 coding region can deregulate its function, whereas mutations of the 5′ regulatory regions of PAX-5, RhoH/TTF and c-MYC are expected to influence the expression and regulation of these genes in a fashion similar to that reported for the BCL-6 gene in B-cell lymphoma. Consistent with the role of PAX-5 in B-cell differentiation, of RhoH/TTF in signal transduction, and of c-MYC in B-cell growth and fate, deregulation of these genes by aberrant hypermutation may contribute to breast lymphoma pathogenesis by multiple pathways.


2009 ◽  
Vol 17 (3-4) ◽  
pp. 80-82
Author(s):  
Lazar Popovic ◽  
Darjana Jovanovic ◽  
Gorana Matovina-Brko ◽  
Dragana Petrovic ◽  
Zoran Nikin ◽  
...  

Primary breast lymphoma is rare location of this disease. Diffuse large B-cell lymphoma is the most common histological subtype. We presented two cases of primary diffuse large B-cell lymphoma of the breast and reviewed a literature about this topic.


2020 ◽  
Vol 154 (Supplement_1) ◽  
pp. S97-S97
Author(s):  
A Herrmann ◽  
B Mai ◽  
S Elzamly ◽  
A Wahed ◽  
A Nguyen ◽  
...  

Abstract Introduction/Objective A 46-year-old female presented with severe back pain associated with progressive bilateral lower extremity weakness and paresthesia, urinary retention, and constipation. Computed tomography revealed a retroperitoneal mass encasing the right psoas muscle, obstructing the right kidney, and extending to the thoracolumbar region resulting in severe spinal compression. An epidural tumor resection was subsequently performed at an outside hospital. Methods Histological sections showed sheets of blastoid neoplastic cells with intermediate to large nuclei, irregular membranes, fine chromatin, and prominent nucleoli. Immunohistochemical stains showed that these cells were positive for CD43, CD79a (weak, focal), BCL2, C-MYC, and PAX5 (weak, focal) and negative for CD10, CD20, CD30, ALK1, BCL6, MUM1, and Tdt. The Ki-67 proliferation index was 75-80%. With this immunophenotype, this patient was diagnosed with a high grade B-cell lymphoma and transferred to our institution for further work-up. On review of the slides, further immunohistochemical testing was requested which revealed positivity for CD117 and myeloperoxidase (MPO). Results The overall morphological and immunophenotypical features are most compatible with myeloid sarcoma (MS) with aberrant expression of B-cell markers and this patient’s diagnosis was amended. Interestingly, the patient’s bone marrow examination only showed 2% myeloblasts with left shifted granulocytosis and concurrent fluorescence in situ hybridization (FISH) studies were negative. Conclusion A literature review showed that 40-50% of MS are misdiagnosed as lymphoma. MS can frequently stain with B-cell or T-cell markers, as seen in this case, which makes it challenging for an accurate diagnosis and sub- classification. In addition, our case is interesting in that there was only extramedullary presentation without bone marrow involvement. Typically, MS develops after the diagnosis of acute myeloid leukemia (AML) with an incidence of 3–5% after AML. It can also manifest de novo in healthy patients, who then go on to develop AML months to years later. Therefore, this patient will require close follow-up.


2021 ◽  
pp. 1-5
Author(s):  
Karishma Habbu ◽  
Roshan George ◽  
Miguel Materin

<b><i>Purpose:</i></b> This report describes a case of relapsed primary breast lymphoma (PBL) presenting as vitreoretinal lymphoma (VRL). <b><i>Methods:</i></b> We describe the clinical and hematopathologic findings in a patient with relapsed PBL involving the vitreous of both eyes. <b><i>Results:</i></b> A 59-year-old woman was treated for PBL with systemic and intrathecal chemotherapy 5 years prior to presentation. Three years later, she presented to an outside clinic with blurred vision in both eyes and bilateral vitritis. She was referred to our clinic with concern for ocular lymphoma. On presentation, the patient’s best-corrected visual acuity was 20/40 in the right eye and 20/25 in the left eye with 3+ vitreous cells in the right eye and 2+ vitreous cells in the left eye. Vitreous biopsy of the right eye revealed CD5-negative/CD10-negative B-cell lymphoma cells on flow cytometry. She had no evidence of disease on brain MRI, lumbar puncture, bone marrow biopsy, or full-body CT scans. She was treated with a regimen of rituximab, methotrexate, procarbazine, and vincristine for central nervous system penetration as well as multiple intraocular injections of methotrexate and rituximab with improvement in vision and ocular inflammation bilaterally. <b><i>Conclusion:</i></b> Relapsed PBL can present as bilateral VRL.


2021 ◽  
Vol 14 (1) ◽  
Author(s):  
Lilla Prenek ◽  
Klára Csupor ◽  
Péter Beszterczán ◽  
Krisztina Boros ◽  
Erika Kardos ◽  
...  

Abstract Background Cardiac tumors are very uncommon compared to other cardiac diseases. Their clinical symptoms can vary from absent to non-specific. The most common symptoms are arrhythmias, blood flow obstruction due to valvular dysfunction, shortness of breath, systemic embolization, and accumulation of pericardial fluid. Hereby, we describe a very rare case of a diffuse large B cell lymphoma patient who presented with the symptoms and signs of acute coronary syndrome (ACS) but the patient’s complaints were caused by his intramyocardial lymphoma metastasis. Case presentation Forty-eight-year-old diffuse large B cell lymphoma patient was admitted to our emergency department with chest pain, effort dyspnea, and fever. The patient had normal blood pressure, blood oxygen saturation, sinus tachycardia, fever, crackles over the left lower lobe, novum incomplete right bundle branch block with Q waves and minor ST alterations, elevated C-reactive protein, high-sensitivity troponin-T, and d-dimer levels. Chest X-ray revealed consolidation on the left side and enlarged heart. Bed side transthoracic echocardiography showed inferior akinesis with pericardial fluid. Coronary angiography showed no occlusion or significant stenosis. Chest computed tomography demonstrated the progression of his lymphoma in the myocardium. He was admitted to the Department of Hematology for immediate chemotherapy and he reached complete metabolic remission, followed by allogeneic hematopoietic stem cell transplantation. Unfortunately, about 9 months later, he developed bone marrow deficiency consequently severe sepsis, septic shock, and multiple organ failure what he did not survive. Conclusions Our case demonstrates a very rare manifestation of a heart metastasis. ACS is an unusual symptom of cardiac tumors. But our patient’s intramyocardial lymphoma in the right atrium and ventricle externally compressed the right coronary artery and damaged the heart tissue, causing the patient’s symptoms which imitated ACS. Fortunately, the quick diagnostics and immediate aggressive chemotherapy provided the patient’s remission and suitability to further treatment.


2020 ◽  
pp. 194187442096756
Author(s):  
Prashant Anegondi Natteru ◽  
Shashank Shekhar ◽  
Lakshmi Ramachandran Nair ◽  
Hartmut Uschmann

Primary central nervous system lymphoma (PCNSL) is an uncommon variant of extra-nodal non-Hodgkin’s lymphoma. Three regions can be involved in PCNSL: the brain, the spine, or the vitreus and retina. Spinal PCNSL is rare. It can mimic neoplasm, infection, and inflammation. Diagnostic confirmation is by tissue biopsy, and even then, tissue corroboration may be altered by an inflammatory overlay. We report a 59-year-old woman who we saw after she had 4 weeks of ascending tetraparesis plus bowel and bladder incontinence. Upon presentation, the patient was ventilator-dependent and locked-in. She reported normal sensation through eye-blinking. Magnetic resonance imaging (MRI) brain revealed signal intensity in the bilateral corona radiata and restricted diffusion in the right thalamus, whereas, MRI cervical, and thoracic spine showed T2 prolongation in the anterior medulla and upper cervical cord, with enhancement to C2-C3, and long segment hyperintensity from T1-T9 levels, respectively, suggestive of neuromyelitis optica spectrum disorder. Cerebrospinal fluid cytomorphology and flow cytometry were inconclusive for lymphoma/leukemia, but oligoclonal bands were present. Serum aquaporin-4 (AQP-4) antibodies were negative. MR spectroscopy demonstrated NAA reduction, mild lipid lactate peak, and relative reduction of choline on the side of the lesion, favoring demyelination. She received 5-days of intravenous methylprednisolone, followed by 7 sessions of plasma exchange without clinical improvement. Stereotactic biopsy of the right thalamic lesion revealed diffuse large B-cell lymphoma. PCNSL can mimic a demyelinating process early on, as steroid treatment could disrupt B-cell lymphoma cells, thus masking the correct diagnosis.


2021 ◽  
Vol 14 (6) ◽  
Author(s):  
Mastane Saneii ◽  
Pedram Fadavi ◽  
Kambiz Novin ◽  
Maryam Garousi

Introduction: PBL is a rare form of extranodal lymphoma. The most common pathology is diffuse large B cell lymphoma and most patients are diagnosed at stages 1 and 2. The therapeutic options undertaken so far include surgery, radiotherapy, and chemotherapy Case Presentation: The patient was a 54-year old woman with localized primary breast DLBCL. The patient underwent 6 courses of chemotherapy with an RCHOP regimen followed by radiotherapy of the breast and regional lymph nodes with a 40 Gy dose. The patient was in complete remission on PET scan 3 months later. Around one year after, the patient experienced relapse in the contralateral breast. Conclusions: In the pattern of relapse of patients, there is a tendency for extranodal relapse. In some studies maximum level of relapse occurring in CNS and some advocate CNS prophylaxis in these patients. The best outcome is for patients treated with chemotherapy including rituximab followed by radiation. We reviewed some studies in the aspect of treatment modalities and site of relapsed.


2020 ◽  
Vol 10 ◽  
Author(s):  
Claudia C. V. Lang ◽  
Egle Ramelyte ◽  
Reinhard Dummer

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