Acute myeloid leukemia following remission of AIDS-associated extra-nodal NK/T-cell lymphoma.

Background: AIDS-related NK/T-cell lymphoma is a rare subtype of AIDS-related lymphomas, characterized by a poor prognosis and lack of standardized treatment protocols. To date, there have been no reported cases of AIDS-associated NK/T-cell lymphoma in remission followed by treatment-related acute myeloid leukemia (t-AML), where both the lymphoma and AML achieved remission and long-term survival through chemotherapy alone. Case presentation: We report a case of a patient diagnosed with AIDS-related extra-nodal NK/T-cell lymphoma (ENKTCL). The patient achieved complete remission after receiving six cycles of chemotherapy, local radiotherapy, and combination antiretroviral therapy (cART). Throughout the follow-up period, the patient continued cART treatment, maintaining an HIV-RNA level below the lower limit of detection. However, 70 months later, the patient developed new symptoms and was subsequently diagnosed with acute myeloid leukemia (AML) M4 subtype. Following the completion of 10 cycles of chemotherapy and ongoing cART, the patient achieved complete remission of AML, with an overall survival time exceeding 103 months from the initial ENKTCL diagnosis. Conclusions: This case highlights the effectiveness of chemotherapy combined with cART in the treatment of AIDS-associated NK/T-cell lymphoma and secondary treatment-related leukemia. This approach may serve as a viable option for patients who are not candidates for bone marrow transplantation. Furthermore, this case underscores the importance of long-term follow-up in the management of AIDS-associated malignancies.

Unusual presentation in a case of diffuse large B-cell lymphoma.

Non-Hodgkin's lymphoma are neoplasms derived from T cells and B cells and their precursors in the lymphoid system with higher susceptibility in involvement of extra-nodal sites. Predominant ureteric involvement is an unusual presentation. We present a case of diffuse large B-cell lymphoma with secondary involvement of ureter who had symptoms of urinary tract infection in absence of positive urine culture, non-responsive to broad spectrum antibiotics and masquerading pyogenic infection leading to pyelonephritis with ureteritis. Radiological examination revealed mass like soft tissue thickening of ureter extending from renal pelvis throughout the length of ureter. FNAC as well as biopsy from the periureteric thickening revealed lymphomatous involvement of ureter. The following case report provides insight on differentials and varied symptoms of lymphomatous involvement of ureter.

Rosai-Dorfman Disease in a Middle-Aged Man With Recurrent Pleural Effusions and Lymphadenopathy.

Rosai-Dorfman disease (RDD) is a rare benign condition that presents most commonly with lymphadenopathy and skin lesions and is characterized by infiltration of histiocytes into the skin and soft tissues. We present a case of RDD in an Afro-Caribbean male in his 50s who presented to our chest clinic with shortness of breath, cough, and weight loss of 15 kg over one year. CT scan showed evidence of right pleural effusion, mediastinal and hilar lymphadenopathy, and bony lesions in the spine. Cytology from multiple pleural effusions and endobronchial ultrasound-guided fine needle aspiration from lymph nodes did not show any malignancy. Left axillary excisional biopsy showed a pattern consistent with RDD. The patient was started on interferon therapy by the hematologist and pleurodesis after repeated pleural taps failed to relieve recurrent right pleural effusions. This case emphasizes the importance of tissue diagnosis to avoid misdiagnosis and unnecessary treatment.

Clinicopathological Profile of Primary Extra Nodal Lymphoma from a Tertiary Care Center in South India.

Background & Objective: Primary extranodal lymphoma (pENL) is a malignant lymphoid neoplasm that presents with the main bulk of disease at an extranodal site. The incidence of primary pENL has risen sharply in recent years due to the advent of better diagnostic modalities. Diagnosing pENL can be challenging due to its morphological overlap with other tumors native to the site of origin. This study aims to establish the anatomic distribution, clinical presentations, possible etiologic correlations, and histological subtypes of pENL in a tertiary care center located in South India. Methods: This is a retrospective study of 109 patients with pENL (69 males, 40 females, M: F = 1.7:1) over 5 years (October 2012 to September 2017). The tumors were reclassified according to WHO classification of Haematolymphoid tumors, 5th edition, 2022. Results: pENL constituted 109/481 cases (22.6%) of all NHL cases, with the highest incidence in 7th decade. The gastrointestinal tract (39%) was the predominant site involved, followed by head and neck (26%). Diffuse large B cell lymphoma (DLBCL) was the most common histomorphological variant followed by Follicular lymphoma (FL). The majority of the patients were immunocompetent (89%) and presented with stage IV disease (31.1%) at diagnosis. Conclusion: This study presents an overview of the diverse distribution patterns of both common and rare pENL within a tertiary care center. The accurate diagnosis of pENL necessitates the elimination of secondary extranodal involvement. It is important to note that the accurate diagnosis of pENL requires careful evaluation and exclusion of other possible causes.

Unusual Presentation of Non-Hodgkin Lymphoma of Two Cases: Case Report.

Lymphomas are a diverse group of neoplastic disorders arising primarily in lymph nodes. They have been majorly classified into Hodgkin and Non-Hodgkin lymphomas(NHL). NHL can be of B, T and Null cell categories having further subtypes based on their histological characteristics. Lymphomas can be nodal and extra nodal. The head and neck area are the second most common site of extra nodal lymphoma, with tonsils being the most common site of involvement; other sites include the nasopharynx and tongue base. B- Cell type being the most common type. Predominantly occurs in elderly. Presentations depends on the site involved. Various modalities like surgical treatment, chemotherapy (or) radiotherapy is available. Each stage has varied survival rates and prognosis and responses to the treat depending on the patient factors. In this paper,  we report two cases of patients with non-Hodgkin lymphoma of tonsil, where the preoperative clinical diagnosis and radiological diagnosis was inconclusive and final diagnosis was established based on histopathological examination.

Characteristics and distinct prognostic determinants of individuals with hepatosplenic T-cell lymphoma over the past two decades.

BACKGROUND: Hepatosplenic T-cell lymphoma (HSTCL) is a rare and aggressive peripheral T-cell lymphoma with historically dismal outcomes, representing less than one percent of non-Hodgkin lymphomas. Given its rarity, the true incidence of HSTCL is unknown and most data have been extrapolated through case reports. To the best of our knowledge, the largest and most up to date study addressing the epidemiology and outcomes of patients with HSTCL in the United States covered a period from 1996 to 2014, with a sample size of 122 patients. AIM: To paint the most updated epidemiological picture of HSTCL. METHODS: A total of 186 patients diagnosed with HSTCL, between 2000 and 2017, were ultimately enrolled in our study by retrieving data from the Surveillance, Epidemiology, and End Results database. We analyzed demographics, clinical characteristics, and overall mortality (OM) as well as cancer-specific mortality (CSM) of HSTCL. Variables with a P value < 0.01 in the univariate Cox regression were incorporated into the multivariate Cox model to determine the independent prognostic factors, with a hazard ratio of greater than 1 representing adverse prognostic factors. RESULTS: Male gender was the most represented. HSTCL was most common in middle-aged patients (40-59) and less common in the elderly (80+). Non-Hispanic whites (60.75%) and non-Hispanic blacks (20.97%) were the most represented racial groups. Univariate Cox proportional hazard regression analysis of factors influencing all-cause mortality showed a higher OM among non-Hispanic black patients. CSM was also higher among non-Hispanic blacks and patients with distant metastasis. Multivariate Cox proportional hazard regression analysis of factors affecting CSM revealed higher mortality in patients aged 80 or older and non-Hispanic blacks. CONCLUSION: Overall, the outlook for this rare malignancy is very grim. In this retrospective cohort study of the United States population, non-Hispanic blacks and the elderly had a higher CSM. This data highlights the need for larger prospective studies to investigate factors associated with worse prognosis in one ethnic group, such as treatment delays, which have been shown to increase mortality in this racial/ethnic group for other cancers.

Neurolymphomatosis as primary presentation of extra-nodal NK/T-cell lymphoma, nasal type.

Neurolymphomatosis (NL) describes an infiltration of cranial and peripheral nerves by lymphoma cells, most frequently in non-Hodgkin B-cell lymphoma. This clinical entity is rare and poses a challenging diagnosis. We describe a case of a 64-year-old female patient with NL associated with extra-nodal NK/T-cell lymphoma (ENKTL), nasal type, presenting as a painful progressive mononeuropathy multiplex with an oral cavity lesion. ENKTL is usually associated with Epstein-Barr virus (EBV) infection and rarely affects the central and peripheral nervous system. Lumbar puncture, magnetic resonance imaging (MRI), nerve biopsy, and 18F-fluorodeoxyglucose positron emission tomography (FDG-PET) help to establish the diagnosis. Thereby, NL should be considered in the differential diagnosis of painful progressive multiple neuropathies, even in patients without previous history of cancer.

Isolated Rosai-Dorfman disease of the spine: A systematic literature review.

INTRODUCTION: Rosai-Dorfman disease (RDD) is a rare non-Langerhans cell histiocytosis involving the central nervous system in 5% of cases. Spinal location occurs in less than 1% of extranodal RDD and can be responsible for neurological manifestations. We present a systematic review of cases of isolated spinal RDD. We also report a new case of isolated spinal RDD revealed by spinal cord compression. MATERIALS AND METHODS: The systematic review was performed according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses guideline using the MEDLINE and SCOPUS databases and included case reports and case series describing isolated RDD of the spine. RESULTS: There were 53 patients with isolated spinal RDD (including our case). The mean age was 35.85±16.48 years. Neurological deficit was the most frequent clinical presentation (89%). RDD lesions were mainly located in the thoracic spine (51%), then the cervical spine (32%). The lesion was reported to be extradural (57%), intradural extramedullary (26%), intramedullary (7%), and in the vertebral body (10%). Histological examination showed emperipolesis in 73%. Histocytes were positive for S-100 protein in 83%. Treatment was based on surgery 96%), radiotherapy, chemotherapy, and adjunctive steroid therapy were indicated in four, one, and eight cases. After a mean follow-up period of 14.84±13.00 months, recurrence of RDD was noted in 15%. CONCLUSION: Spinal RDD is a rare condition, requiring meticulous histological examination for accurate diagnosis. Complete surgical resection is the treatment of choice. Adjuvant chemotherapy and radiotherapy can also be indicated in patients demonstrating partial improvement following surgery.

Multicenter development of a PET-based risk assessment tool for product-specific outcome prediction in large B-cell lymphoma patients undergoing CAR T-cell therapy.

PURPOSE: The emergence of chimeric antigen receptor (CAR) T-cell therapy fundamentally changed the management of individuals with relapsed and refractory large B-cell lymphoma (LBCL). However, real-world data have shown divergent outcomes for the approved products. The present study therefore set out to evaluate potential risk factors in a larger cohort. METHODS: Our analysis set included 88 patients, treated in four German university hospitals and one Italian center, who had undergone 2-[18F]fluoro-2-deoxy-D-glucose positron emission tomography (PET) before CAR T-cell therapy with tisagenlecleucel or axicabtagene ciloleucel. We first determined the predictive value of conventional risk factors, treatment lines, and response to bridging therapy for progression-free survival (PFS) through forward selection based on Cox regression. In a second step, the additive potential of two common PET parameters was assessed. Their optimal dichotomizing thresholds were calculated individually for each CAR T-cell product. RESULTS: Extra-nodal involvement emerged as the most relevant of the conventional tumor and patient characteristics. Moreover, we found that inclusion of metabolic tumor volume (MTV) further improves outcome prediction. The hazard ratio for a PFS event was 1.68 per unit increase of our proposed risk score (95% confidence interval [1.20, 2.35], P = 0.003), which comprised both extra-nodal disease and lymphoma burden. While the most suitable MTV cut-off among patients receiving tisagenlecleucel was 11 mL, a markedly higher threshold of 259 mL showed optimal predictive performance in those undergoing axicabtagene ciloleucel treatment. CONCLUSION: Our analysis demonstrates that the presence of more than one extra-nodal lesion and higher MTV in LBCL are associated with inferior outcome after CAR T-cell treatment. Based on an assessment tool including these two factors, patients can be assigned to one of three risk groups. Importantly, as shown by our study, metabolic tumor burden might facilitate CAR T-cell product selection and reflect the individual need for bridging therapy.

Laryngeal tuberculosis: about 04 cases.

The purpose of this study is to present epidemiological, clinical, radiological, histological characteristics and treatment of laryngeal tuberculosis. It is also aimed at making the point about diagnosis difficulties. This retrospective study was conducted over three years; it concerned 4 cases, 3 males and one female. The average age was 35 years. Three of the 4 cases have had a cervical CT scan. All patients have had a laryngoscopy with biopsy and anatomopathological study. The onset modes of the disease have been progressive for all the patients. Topographical study has shown two epiglottic locations, one at the vocal cords and the other one at the posterior commissure. The diagnosis was orientated in the 3 cases by the CT scan and confirmed by anatomopathological exam in all cases. All patients have received anti-TB drugs with good evolution. The laryngeal location of tuberculosis is unusual. The clinical picture is nonspecific, raising the issue of differential diagnosis with tumor pathology. Sectional imaging and CT scan can guide the diagnosis and a positive diagnosis is often discovered on the occasion of a tumor biopsy of a pseudo-tumor lesion. Treatment is based on anti-TB drugs.

A cerebral lymphoma mimicking a meningioma: case report.

Introduction: Cerebral lymphoma is a rare and aggressive brain tumor. It accounts for 1% of all non-Hodgkin's lymphomas (NHL) and 2% of all brain tumors. Untreated brain lymphoma has a very poor prognosis, with an overall life expectancy of around 1.5 months. Case presentation: The authors report the case of a 35-year-old patient, with no previous pathological history, who presented for 3 weeks with deafness and recently aggravated otalgia. In MRI, brain imaging revealed a formation initially suggestive of an aggressive meningioma, and the histological study of the operative specimen was in favor of a diffuse large-cell non-germ-center B NHL. Clinical discussion: Primary central nervous system lymphoma is an extra-nodal NHL localized to the brain, meninges, spinal cord, and eyes. In 90% of cases, these are diffuse large B-cell lymphomas, the other types being poorly characterized low-grade lymphomas, T-cell lymphomas, and Burkitt's lymphomas. MRI with gadolinium contrast is the gold standard for diagnosis which enhancement is homogeneous and well-limited, frequently associated with perilesional vascular edema. In T2-weighted sequences, there is a weak signal with restricted diffusion on diffusion-weighted imaging. The management of brain lymphoma is currently based on chemotherapy with high-dose methotrexate combined with the other agents, mainly rituximab. Conclusion: Cerebral lymphoma remains a non-negligible entity of central nervous system tumors, which can be confused with several other tumors, mainly glial and meningioma.

Rare Presentation of Primary Classical Hodgkin Lymphoma of a Rectosigmoid Colon in a 79-Year-Old With a History of Irritable Bowel Syndrome.

We discuss the case of a 79-year-old immunocompetent male who presented with weight loss and diarrhea and ultimately was found to have a rectosigmoid mass on a colonoscopy. Even though initial biopsies obtained during colonoscopy were non-diagnostic, considering the likelihood of malignancy, lower anterior resection was performed, and pathology confirmed the diagnosis of primary Hodgkin's lymphoma of the sigmoid colon. Hodgkin's lymphoma typically presents as painless supra-diaphragmatic lymphadenopathy with B symptoms such as fever, unexplained weight loss, and drenching night sweats. Due to the rarity of primary Hodgkin lymphoma in the colon and its non-specific initial presentation, we believe sharing this case will bring awareness to the atypical presentation of Hodgkin lymphomas.

Lymph Node Ratio as a Prognostic Factor for Oral Tongue Squamous Cell Carcinoma: A Retrospective Study.

Background Oral squamous cell carcinoma (OSCC) incidence and its mortality have increased recently. The oral part of the tongue is one of the commonest sites for OSCC. Apart from Tumour-Node-Metastasis (TNM) staging, lymph node ratio (LNR) has been implicated as one of the useful predictors for the better clinical outcome of the disease. The aim of the present study was to assess the LNR as a prognostic factor for patients having oral tongue squamous cell carcinoma (OTSCC). Materials and methods It is a retrospective study of 122 patients with OTSCC who were managed primarily by surgery with curative intent from January 2014 to December 2016. The mean lymph node ratio was measured and compared with various parameters of clinical outcome such as five-year overall survival (OS), five-year disease-free survival (DFS), locoregional failure (LRF) within three years, and distant metastasis (DM) within five years using Kruskal-Wallis Test followed by Mann-Whitney Post Hoc Test. The association of LNR with other tumor characteristic features like perineural invasion, extra-nodal extension (ENE), and histopathological grading was also elicited. Results The study population's mean age was 50.5 ± 11.77 years. Among them, 85 were males and 37 were females. On comparing the mean LNR value with patient status after primary treatment, the patients with minimal LNR value had statistically significant five-year OS and five-year DFS (p< 0.001). High mean LNR values were associated with other adverse features like perineural invasion and ENE, which were statistically significant (p<0.001). Receiver operator characteristics (ROC) curve analysis for the LNR parameter for determining the cut-off (0.02) between OS and DFS had 86% sensitivity and 40% specificity. Conclusion The LNR could be an important prognosis factor for OTSCC that helps in determining better clinical outcomes.

Nasopharyngeal Lymphoma: Rare Case Report.

A case of Nasopharyngeal Non Hogkins lypmhomas, diagnosed on Histopathology (HPE) and immunohistochemistry. In this case report we present the clinical presentation, diagnosis and treatment protocol of nasopharyngeal NHL.

Evolution of TNM Classification for Clinical Staging of Oral Cancer: The Past, Present and the Future.

Purpose: The AJCC (American Joint Committee on Cancer) Cancer Staging Manual, 1st Edition, was published in 1977 which focused on the TNM classification and staging of cancer to allow easy communication, formulation of a treatment plan and predict the prognosis, among the medical fraternity. Methods: Ever since the beginning, various modifications of the classification were introduced and released by the joint collaboration of AJCC and UICC (International Union Against Cancer) in various editions of cancer staging manuals. Results: The present review article was kept focused onto the changes introduced in the clinical staging of cancers of oral cavity. These changes came a long way since 1st edition 1944, to the eighth edition which was published in 2017. Conclusions: This article is a critical review on the past and present perspectives of the TNM classification of the oral cavity that were addressed and changed, adding a light on the future trends or necessary inclusions that would formulate a much easily acceptable and useful classification system.

Primary Ovarian Non-Hodgkin's Lymphoma: An 18-year Retrospective Institutional Study and Review of Literature from India.

BACKGROUND: Primary lymphomas of the female reproductive tract are rare and the ovarian extranodal presentation of non-Hodgkin's lymphoma (NHL) accounts for only 0.5% of all NHLs and 1.5% of all ovarian malignancies. METHODS: We retrospectively reviewed the institutional medical oncology database for newly diagnosed NHL cases between 1999 and 2017. We aimed to study the clinical characteristics, pathology, and outcome of primary ovarian non-Hodgkin's lymphoma (NHL) cases presented to our institution. RESULTS: We identified three patients (3.7% of extranodal NHLs and 0.85% of all NHL patients) with primary ovarian NHL from 350 NHL patient records. They underwent total abdominal hysterectomy and bilateral salpingo-oophorectomy followed by six to eight cycles of (rituximab, adriamycin, cyclophosphamide, vincristine, prednisolone (R-CHOP/CHOP), and they attained complete remission. CONCLUSION: Given the heterogeneity of cancer incidence in India and the absence of state-wise cancer registries, our study argues a pressing need to develop a national representative registry for NHL for accurate incidence, mortality, and survival data. Additionally, fertility preservation is an important issue that must be discussed with women of fertile age and the parents of children.

Primary Diffuse Large B-Cell Lymphoma of the Penis: A Case and Literature Review.

Primary diffuse large B-cell lymphoma (DLBCL) of the penis is an exceptionally rare malignant disorder, and less than 20 cases have been previously reported. The diagnosis can be difficult, and the standard treatment has not been established yet. We reported an 86-year-old male patient with DLBCL of the penis with an annular penile ulcer, which was not sensitive to the classic R-C(H)OP regimen for three circles; then underwent surgical resection and achieved complete remission for 73 months until now. Including our patient, we collected the clinical characteristics of 20 patients with primary DLBCL of the penis. The median age was 69 years, and most patients manifested mass, diffuse swelling, non-healing ulcer in the penis, and difficulty with urination. Chemo-immunology and radiography were used as first-line therapy, and surgery still plays an essential role in refractory or recurrence. Due to its anatomical independence and physiological particularity, there is still no standard for diagnosing and treating primary DLBCL of the penis. Systemic chemotherapy and radiography were considered first-line therapy to induce remission and preserve the structure and function of the penile; however, surgery still plays a vital role in the refractory or recurrence of single extranodal lymphoma.

Primary diffuse large B-cell tumor of the uterus: a case report.

Primary diffuse large B-cell tumor of the uterus is a rare clinical condition with a similar clinical presentation to gynecologic tumors and is easily misdiagnosed. We report a case of a postmenopausal woman who presented with pelvic mass. Her ultrasound and MRI examinations suggested diffuse enlargement of the uterus, leading us to consider the diagnosis of lymphoma. The histological examination of the uterine mass confirmed the diagnosis of diffuse large B-cell lymphoma. The patient received 6 cycles of chemotherapy with R-CHOP (rituximab, cyclophosphamide, doxorubicin, vincristine and prednisone) and 2 cycles of consolidation therapy with R (rituximab). After treatment, the patient's uterus was significantly smaller than before and there was no sign of a recurrence.