Testicular Lymphoma manifesting as neurological symptoms due to secondary neurolymphomatosis: A case report

Neurolymphomatosis is a rare manifestation of lymphoma presenting as diffuse invasion and involvement of peripheral and spinal nerves. Due to the common presenting symptomatology of neurologic complaints localizing to the affected peripheral nerve, lymphoma as the underlying etiology can be diffificult to diagnose. Here we present the case of a gentleman presenting with right extremity neuropathic symptoms, subsequently discovered to have diffuse large B-cell lymphoma of testiticular orgin after nerve biopsy revealed neurolymphomatous involvement of a spinal nerve. This case highlights the importance of the consideration of neurolymphomatosis in the work up of neuropathic symptoms, as well as the full assessment for the site of primary involvement.

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Incorporation of digital gene expression profiling for cell-of-origin determination (Lymph2Cx testing) into the routine work-up of diffuse large B cell lymphoma

Journal of Hematopathology ◽

10.1007/s12308-019-00344-0 ◽

2019 ◽

Vol 12 (1) ◽

pp. 3-10 ◽

Cited By ~ 3

Author(s):

Ryan S. Robetorye ◽

Colleen A. Ramsower ◽

Allison C. Rosenthal ◽

Tameson K. Yip ◽

Amy J. Wendel Spiczka ◽

...

Keyword(s):

Expression Profiling ◽

Cell Lymphoma ◽

Digital Gene Expression ◽

B Cell Lymphoma ◽

Cell Of Origin ◽

Digital Gene Expression Profiling ◽

Large B Cell Lymphoma ◽

Routine Work ◽

Origin Determination ◽

Work Up

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Primary Testicular Diffuse Large B-Cell Lymphoma, M.D. Anderson Cancer Center Experience.

Blood ◽

10.1182/blood.v114.22.2697.2697 ◽

2009 ◽

Vol 114 (22) ◽

pp. 2697-2697

Author(s):

Ali Mazloom ◽

Nathan Fowler ◽

Puneeth Iyengar ◽

Bouthaina S. Dabaja

Keyword(s):

B Cell ◽

Cell Lymphoma ◽

B Cell Lymphoma ◽

Intrathecal Chemotherapy ◽

Cancer Center ◽

Large B Cell Lymphoma ◽

Testicular Lymphoma ◽

Stage Disease ◽

Large B Cell ◽

Primary Testicular Lymphoma

Abstract Abstract 2697 Poster Board II-673 Background: Primary testicular lymphoma (PTL) is the most common testicular neoplasm in males over the age of 60, representing 1% to 7% of all testicular malignancies. Histologically, the most common subtype of PTL is diffuse large B-cell lymphoma (DLBCL) representing 80–90% of diagnosed tumors. Primary testicular lymphoma has a tendency to disseminate to the contralateral testis and the central nervous system. Treatment approaches can include loco-regional treatment regimens (orchiectomy, radiotherapy) and systemic chemotherapy with or without intra-thecal chemoprophylaxis. The purpose of this study is to determine the clinical characteristics, patterns of failure, and survival of patients with PTL at our institution and to determine any correlation between outcome and treatment strategies. Methods: We retrospectively reviewed the medical records of patients with diffuse large B cell lymphoma of the testis. All patients were diagnosed and managed at the University of Texas MD Anderson Cancer Center between 1964 and 2008. Pathological diagnosis of DLBCL of the testis was made from orchiectomy specimens or fine needle aspiration of the testis. Factors analyzed included: age, stage at diagnosis, presence of b-symptoms, serum lactate dehydrogenase (LDH), beta-2 microglobulin, and modality of treatment. Cox proportional hazards model was used to generate hazard ratios for prognostic factors that affect the overall survival (OS) and disease free survival (DFS). Estimates of survival were calculated using the Kaplan-Meier method and the log-rank test was used to compare OS and DFS by the type of treatment. Results: Seventy-five patients with DLBCL of the testis were identified. The median age at diagnosis was 64 years (range 22 – 82). Ann Arbor stage I was present in 34 (45%), stage II in 13 (17%), stage III in 4 (5%), stage IV in 23 (31%), and unknown in 1 (1%). On univariate analysis, patients with advanced stage disease (stage III and IV; p = 0.042), elevated serum LDH levels (p = 0.029), B-symptoms at presentation (p = 0.003), and high-intermediate and high IPI score (p = 0.013), had a significantly decreased OS and DFS. The 5-years OS and DFS for all patients was 53.3%, and 45.9%, respectively. Treatment details are described in Table 1. A greater proportion of patients who received trimodality regimen (doxorubicin based chemotherapy, scrotal RT, and intrathecal methotrexate) had limited stage disease compared to those receiving less than a trimodality approach, however this variation was not statistically significant (71% vs 60%; p = 0.374, chi-square test). The 5-year OS and DFS for those treated with trimodality regimen was 90.4% and 79.5%, respectively, while for those treated with doxorubicin-based chemotherapy and testicular irradiation but without intrathecal chemotherapy it was 63.5%, and 55.6%, respectively, and for those treated with doxorubicin-based chemotherapy alone it was 39.3%, and 28.6% (p = 0.009 for OS and p = 0.012 for DFS). Figures 1 & 2 Conclusion: Prognostic factors for DLBCL of the testis include stage, LDH, B-symptoms, and IPI score. Patients with DLBCL of the testis who received trimodality regimen (doxorubicin based chemotherapy combined with scrotal RT and intrathecal chemotherapy) had significantly improved OS and DFS, which support the use of this approach as standard of care. Disclosures: No relevant conflicts of interest to declare.

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Genetic Map of Relapsed and Refractory Diffuse Large B-cell Lymphoma: A Systemic Review and Association Analysis

10.21203/rs.3.rs-93266/v1 ◽

2020 ◽

Author(s):

Fan Gao ◽

Lei Tian ◽

Jing Wang ◽

Fei Dong ◽

Kai Hu ◽

...

Keyword(s):

B Cell ◽

Cell Lymphoma ◽

Strong Association ◽

Gene Mutations ◽

B Cell Lymphoma ◽

Data Set ◽

Common Gene ◽

Large B Cell Lymphoma ◽

The Common ◽

Large B Cell

Abstract Diffuse large B-cell lymphoma (DLBCL) is the most common histological subtype of non-Hodgkin lymphoma (NHL). In recent years, a deeper understanding of the genetic subtypes of diffuse large B lymphoma has been reached, and these advances have also been applied to research on relapsed and refractory diffuse large B-cell lymphoma (RRDLBCL). We screened 1495 documents, compiled the whole-exome sequencing data of several studies, formed a data set including 92 observations, and performed association analysis on the high-frequency mutations among them. The most common mutations in the data set include TTN (34/92, 37.0%), KMT2D (29/92, 31.5%), TP53 (25/92, 27.2%), IGLL5 (25/92, 27.2%), CREBBP (21 /92, 22.8%), BCL2 (21/92, 22.8%), MYD88 (20/92, 21.7%), and SOCS1 (19/92, 20.7%). Among these, CREBBP, KMT2D, and BCL2 have a strong association with each other, and SOCS1 has a strong association with genes such as ACTB, CIITA, and GNA13. There is also a strong association between SOCS1 and STAT6. Though TP53 and MYD88 lack significant associations with most genes, the association between MYD88 and PIM1 is significant. Through SOM clustering and expression-level analysis of common gene mutations, we believe that RRDLBCL can be divided into four main types: (1) JAK-STAT-related type, including STAT6, SOCS1, ITPKB, CIITA, and B2M. The expression lineage is similar to PMBL and cHL. (2) EZB type: BCL2 and EZHZ are the main types of mutations. Epigenetic mutations such as KMT2D and CREBBP are more common in this type, and are often accompanied by BCL2 mutations. (3) MCD type, including MYD88, CD79B and PIM1. These genes are involved in the BCR signaling pathway and related pathways, and are connected by the common NF-κB pathway. (4) Undefined type (Sparse Mutation type). These patients are mainly individuals with sparse mutations, including some patients with TP53 mutations (30.3%, 10/33), but who generally lack characteristic mutations. Among the common gene mutations, the expression changes in BCL2, PIM1, STAT6, ITPKB, and GNA13 have more significant prognostic significance. We also reviewed the literature from recent years concerning the previously mentioned common gene mutations.

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Primary Diffuse Large B Cell Lymphoma of the Common Bile Duct

Journal of Gastrointestinal Surgery ◽

10.1007/s11605-020-04569-8 ◽

2020 ◽

Vol 24 (10) ◽

pp. 2376-2378 ◽

Cited By ~ 1

Author(s):

Denise L. Wong ◽

Benjamin W. Deschner ◽

Lauren C. King ◽

Evan S. Glazer

Keyword(s):

Bile Duct ◽

Common Bile Duct ◽

B Cell ◽

Cell Lymphoma ◽

B Cell Lymphoma ◽

Large B Cell Lymphoma ◽

The Common ◽

Large B Cell

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Intravascular large B cell lymphoma: when lymphoma is suspected but routine diagnostic work-up is negative

Leukemia & Lymphoma ◽

10.1080/10428190903234005 ◽

2009 ◽

pp. 1-4

Author(s):

Valentina Bozzoli ◽

Maria Chiara Tisi ◽

Francesco D'Alo ◽

Giuseppina Massini ◽

Giovanna Mansueto ◽

...

Keyword(s):

B Cell ◽

Cell Lymphoma ◽

B Cell Lymphoma ◽

Large B Cell Lymphoma ◽

Diagnostic Work ◽

Work Up ◽

Diagnostic Work Up ◽

Large B Cell

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Primary diffuse large B cell lymphoma of the common bile duct causing obstructive jaundice

International Cancer Conference Journal ◽

10.1007/s13691-015-0238-2 ◽

2015 ◽

Vol 5 (2) ◽

pp. 107-112 ◽

Cited By ~ 1

Author(s):

Hiroaki Kato ◽

Masanori Tsujie ◽

Tomoko Wakasa ◽

Shuhei Kogata ◽

Hirofumi Kanaizumi ◽

...

Keyword(s):

Bile Duct ◽

Common Bile Duct ◽

Obstructive Jaundice ◽

B Cell ◽

Cell Lymphoma ◽

B Cell Lymphoma ◽

Large B Cell Lymphoma ◽

The Common ◽

Large B Cell

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Diffuse Large B-cell Lymphoma of Testis: a Case Report and Current Literature Review

Folia Medica ◽

10.3897/folmed.62.e47874 ◽

2020 ◽

Vol 62 (1) ◽

pp. 200-203

Author(s):

Yavuz Guler ◽

Burak Ucpinar ◽

Akif Erbin

Keyword(s):

B Cell ◽

Current Literature ◽

Cell Lymphoma ◽

Prognostic Index ◽

B Cell Lymphoma ◽

Population Based ◽

Large B Cell Lymphoma ◽

Testicular Lymphoma ◽

Large B Cell ◽

Primary Testicular Lymphoma

Introduction: Testicular cancers detected in older males are mostly testicular lymphomas. Primary testicular lymphoma (PTL) is a rare, clinically aggressive form of extranodal lymphoma. In population-based studies, the  incidence of PTL is 0.09-0.26/100.000. The vast majority of PTL are diffuse large B-cell lymphoma (DLBCL).   Case presentation: We present a case of PTL diagnosed in a 68-year-old male patient and also, we discuss the incidence, clinical presentation, prognostic factors and management of this rare pathology in the light of current literature. Histopathological and immunohistochemical exams of the patient confirmed the diagnosis of DLBCL after radical orchiectomy. According to the internal prognostic index (IPI), patients’ IPI score was evaluated as 5 and according to Ann Arbor staging, patients’ stage was interpreted as grade 3E. Cyclophosphamide, vincristine, etoposide, and prednisolone chemotherapy was planned for the patient and until now, the patient received his first chemotherapy regimen.   Discussion: Primary testicular lymphoma should be kept in mind for every patient who admits with a testicular mass, especially in advanced age.  Misinterpreatation of the clinical findings can delay the definitive diagnosis. Primary testicular lymphoma should be managed with a multi-disciplinary team including urologists, medical and radiation oncologists. 

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SAT-245 A Rare Case of Clival Primary Diffuse Large B-Cell Lymphoma Presenting with Panhypopituitarism and Unmasked Diabetes Insipidus

Journal of the Endocrine Society ◽

10.1210/jendso/bvaa046.828 ◽

2020 ◽

Vol 4 (Supplement_1) ◽

Author(s):

Hajerah Sonnabend ◽

Muriel Tania L Go ◽

Ravi M Iyengar

Keyword(s):

Diabetes Insipidus ◽

B Cell ◽

Cell Lymphoma ◽

Brain Mri ◽

Free Water ◽

B Cell Lymphoma ◽

Critically Ill Patient ◽

Large B Cell Lymphoma ◽

Work Up ◽

Large B Cell

Abstract Background: Central adrenal insufficiency (AI) is known to conceal diabetes insipidus (DI) by impairing free water clearance from the renal system and inhibiting polyuria, which is later unmasked by the initiation of glucocorticoids. The instances of masked DI in the literature are confined to case reports - the actual incidence is unclear. There have been no documented cases of masked DI secondary to a clival tumor, which remains a rare cause of hypopituitarism. Here we discuss a case of primary diffuse large B-cell lymphoma (DLBCL) of the clivus presenting with panhypopituitarism and the development of DI upon glucocorticoid initiation. Clinical Case: A 60-year-old man with DLBCL initially presented to an outside institution with headache, diplopia, and right eye ptosis. Brain MRI showed enhancement of the clivus without obvious pituitary gland involvement. He underwent biopsy with partial resection of the tumor and pathology confirmed DLBCL. His course was complicated by persistent fevers. Extensive work-up was unrevealing and he was transferred to our institution for further management. On admission, infectious work-up was positive for C. Difficile. He later developed septic shock requiring vasopressors and broad-spectrum antibiotics. Despite prolonged antibiotics, he remained hypotensive. Due to proximity of the clivus and sella, there was concern for pituitary involvement. A random cortisol was 9.1 [3.7-19.4 ug/dL], subsequent 250ug cosyntropin stimulation test yielded 13.1 and 14.9 at 30- and 60-minutes, respectively. Given inadequate stimulation and persistent hypotension in this critically ill patient, he was then started on stress dose hydrocortisone (HC) with prompt resolution of hypotension. Further work-up of the pituitary axes was consistent with anterior hypopituitarism: ACTH 5 [6-50 pg/mL], TSH 0.336 [0.350-4.940 uIU/mL], free T4 0.5 [0.7-1.5 ng/dL], FSH 0.3/LH 0.1 [1-10 mIU/mL], IGF-1 <16 [41-279 ng/mL], prolactin <1 [3-16 ng/mL]. On day two of HC stress dosing, he developed hypernatremia and polyuria. Urine studies were consistent with DI and desmopressin was started with subsequent improvement. Conclusion:This is the first documented case of DLBCL of the clivus leading to panhypopituitarism. For tumors in close proximity to the pituitary, there should be a low threshold for pituitary axes evaluation. In addition, masked DI from central AI remains rare and requires close attention by the Endocrinologist following initiation of glucocorticoids.

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