Compromiso secundario de glándulas mamarias por linfoma folicular / Secondary involvement of breasts by follicular lymphoma

Resumen Las lesiones metastásicas que comprometen la glándula mamaria son excepcionales, ocupando las neoplasias hematolinfoides el segundo lugar en orden de frecuencia en series de casos reporta dos en la literatura con una prevalencia de 0.04% a 1.6% en relación a todos los tumores malignos de la mama, alcanzando una incidencia anual de 0.07%, los cuales corresponden principalmente a linfomas secundarios. El 80% de estos son linfomas B difusos de células grandes, seguido de linfoma folicular y linfoma de la zona marginal. Presentamos una mujer de 60 años con diagnóstico de linfoma folicular que comenzó con una masa perirrenal derecha y ganglios linfáticos ipsilaterales retroperitoneales e inguinales, quien, durante su tratamiento, presentó avance en el estadio clínico con compromiso secundario inusual de ambas glándulas mamarias por esta neoplasia hematolinfoide. Se evaluó el comportamiento biológico de esta enfermedad para comprender los mecanismos fisiopatológicos, mediante el análisis de factores clínicos, histológicos y pronósticos, permitiendo la estadificación definitiva, la cual fue determinante para la elección de la terapia individualizada acorde a las guías de práctica clínica basada en la evidencia científica, impactando positivamente en la evolución médica de la paciente.

Abstract Metastatic lesions involving the breast are exceptional; hematolymphoid neoplasias rank second as per their frequency in case series reported in the literature with a prevalence of 0.04% to 1.6% when consider ing all malignant breast tumors and reaching an annual incidence of 0.07%, mainly accounted for by secondary lymphomas. Eighty percent of them are diffuse, large B cells lymphomas (DLBCL), followed by follicular lymphoma and marginal zone lymphoma. This case is about a 60 year-old woman with a diagnosis of follicular lymphoma, who presented with a right perirenal mass and ipsilateral retroperitoneal and inguinal lymph nodes, whose clinical status progressed during the treatment with unusual secondary involvement of both breasts by hematolymphoid neoplasia. The biological behavior of the condition was evaluated to understand the pathophysiological mecha nisms; this was done analyzing clinical, histologic and prognostic factors that led to a definitive staging, which was key to select the individualized therapy following the clinical practice guidelines based on scientific evidence, with a positive impact on the patient's medical progress.

Secondary involvement of breasts by follicular lymphoma / Compromiso secundario de glándulas mamarias por linfoma folicular

Abstract Metastatic lesions involving the breast are exceptional; hematolymphoid neoplasias rank second as per their frequency in case series reported in the literature with a prevalence of 0.04% to 1.6% when considering all malignant breast tumors and reaching an annual incidence of 0.07%, mainly accounted for by secondary lymphomas. Eighty percent of them are diffuse, large B cells lymphomas (DLBCL), followed by follicular lymphoma and marginal zone lymphoma. This case is about a 60 year-old woman with a diagnosis of follicular lymphoma, who presented with a right perirenal mass and ipsilateral retroperitoneal and inguinal lymph nodes, whose clinical status progressed during the treatment with unusual secondary involvement of both breasts by hematolymphoid neoplasia. The biological behavior of the condition was evaluated to understand the pathophysiological mechanisms; this was done analyzing clinical, histologic and prognostic factors that led to a definitive staging, which was key to select the individualized therapy following the clinical practice guidelines based on scientific evidence, with a positive impact on the patient's medical progress.

Resumen Las lesiones metastásicas que comprometen la glándula mamaria son excepcionales, ocupando las neoplasias hematolinfoides el segundo lugar en orden de frecuencia en series de casos reportados en la literatura con una prevalencia de 0.04% a 1.6% en relación a todos los tumores malignos de la mama, alcanzando una incidencia anual de 0.07%, los cuales corresponden principalmente a linfomas secundarios. El 80% de estos son linfomas B difusos de células grandes, seguido de linfoma folicular y linfoma de la zona marginal. Presentamos una mujer de 60 años con diagnóstico de linfoma folicular que comenzó con una masa perirrenal derecha y ganglios linfáticos ipsilaterales retroperitoneales e inguinales, quien, durante su tratamiento, presentó avance en el estadio clínico con compromiso secundario inusual de ambas glándulas mamarias por esta neoplasia hematolinfoide. Se evaluó el comportamiento biológico de esta enfermedad para comprender los mecanismos fisiopatológicos, mediante el análisis de factores clínicos, histológicos y pronósticos, permitiendo la estadificación definitiva, la cual fue determinante para la elección de la terapia individualizada acorde a las guías de práctica clínica basada en la evidencia científica, impactando positivamente en la evolución médica de la paciente.

Clinical Research Advances of Duodenal-Type Follicular Lymphoma--Review / 中国实验血液学杂志

Duodenal-type follicular lymphoma (DFL) is a unique subtype of follicular lymphoma (FL), which often involves the second portion of duodenum (descending part of duodenum). Due to its specific pathological features, such as lack of follicular dendritic cells meshwork and disappearance of activation-induced cytidine deaminase expression, DFL presents an inert clinical course and is often confined to the intestinal tract. Inflammation-related biomarkers suggest that the microenvironment may play a likely role in the pathogenesis and favorable prognosis of DFL. Since patients generally have no obvious clinical symptoms and low progression rate, the treatment regimen for DFL is mainly observation and waiting (W&W) strategy. This study will review the latest research progress of epidemiology, diagnosis, treatment and prognosis of DFL in recent years.

Comparison of efficacy and safety of BR regimen and R-CHOP regimen in the treatment of newly diagnosed follicular lymphoma / 中国综合临床

Objective:Compare the clinical efficacy and safety of bendamustine combined with rituximab (BR regimen) and rituximab combined with standard CHOP regimen (R-CHOP regimen) in the treatment of newly diagnosed follicular lymphoma (FL).Methods:Adopting a prospective case-control study method. 104 newly diagnosed FL patients admitted to Beijing Aerospace General Hospital from January 2018 to January 2022 were selected and randomly divided into an observation group and a control group using a random number table method, with 52 patients in each group. The observation group was treated with bendamustine combined with rituximab, while the control group was treated with rituximab combined with standard CHOP regimen. Both groups were treated for 6 consecutive courses of treatment, with a 21 day treatment period. Compare the serum lactate dehydrogenase (LDH) levels before and after treatment between two groups β 2-Microglobulin (β 2-microglobulin, β 2-MG level, improvement in quality of life after treatment, long-term survival, clinical efficacy, and incidence of adverse reactions. Measurement data is represented by paired t-tests for intra group comparisons, and independent sample t-tests for inter group comparisons; Counting data is represented as an example (%), and inter group comparisons are made using χ 2-test, Wilcoxon rank sum test was used for comparing rank data. Survival analysis was conducted using the Log Rank test. Results:After treatment, serum LDH and The levels of β 2-MG were lower than before treatment [LDH: (262.34±37.24) U/L ratio (323.45±44.46) U/L, (287.23±43.19) U/L ratio (318.28±52.35) U/L; β 2-MG: (2.72±0.30) mg/L compared to (3.45±0.37) mg/L, (2.93±0.28) mg/L compared to (3.37±0.42) mg/L, t-values of 7.60, 3.30, 11.05, 6.29, P values of <0.001, 0.001, <0.001, <0.001, <0.001], and the observation group was lower than the control group ( t-values of 3.15, 3.69, P values of 0.002, <0.001, respectively). After 6 courses of treatment, the quality of life in the observation group improved in 27 cases, stabilized in 22 cases, and decreased in 3 cases; The quality of life in the control group improved in 18 cases, stabilized in 26 cases, and decreased in 8 cases. The improvement of quality of life in the observation group was better than that in the control group ( Z=-2.03, P=0.042). The progression free survival period in the observation group was longer than that in the control group [52.53 months (95% confidence interval: 49.16-55.89 months) compared to 38.84 months (95% confidence interval: 32.44-45.24 months)], and the difference was statistically significant (Log Rank χ 2=4.06, P=0.044), there was no statistically significant difference in overall survival between the two groups ( P=0.217). The complete remission rate in the observation group was higher than that in the control group [88.46%(46/52) vs 71.15%(37/52)], χ 2=4.83, P=0.028], there was no statistically significant difference in objective response rates between the two groups ( P=0.485). The incidence of nausea, vomiting, leukopenia, neutropenia, alopecia, and fatigue in the observation group was lower than that in the control group, and the differences were statistically significant (all P<0.05). Conclusions:Both the BR regimen and R-CHOP regimen can significantly reduce serum tumor marker levels in the treatment of newly diagnosed FL. However, the BR regimen has a higher complete response rate, better patient quality of life, longer PFS, fewer toxic side effects, and more significant overall efficacy.

A Case of Lymphoma with Multiple Identities

@#Discordant lymphoma (DL) is the coexistence of two or more distinct subtypes in separate anatomic sites. There are limited reports on DL cases especially involving more than two subtypes in more than two sites. We report a 76-year-old man who presented with constitutional symptoms, flank mass and painless lymphadenopathies for six months. Laboratory tests revealed moderate anaemia, markedly elevated serum IgM (13400 mg/dL), IgM Lambda paraproteinemia and Lambda light chain paraproteinuria with unmeasurable serum lactate dehydrogenase due to hyperviscous sample. CT scan showed multiple subcutaneous masses over chest wall and retroperitoneum, with lytic bone lesions, and hepatosplenomegaly. Further biopsy findings with morphological, immunohistochemical and molecular analysis of the tissue sections revealed diffuse large B-Cell lymphoma in the chest wall mass, follicular lymphoma in the inguinal lymph node and lymphoplasmacytic lymphoma in the bone marrow. This case highlights the rare DL. The importance of histopathological evaluation of lymphoma despite the availability of PET-CT scans for disease staging is undeniable.

Comparison of primary follicular lymphoma of gastrointestinal tract and secondary involvement: A study from South India

Primary follicular lymphoma of the gut (PFL-GI) is a rare entity. This study aims to compare the clinicopathologic features of PFL-GI with cases of gastrointestinal involvement by disseminated nodal follicular lymphoma. This is a retrospective study with 6 cases of primary follicular lymphoma and 8 cases of secondary involvement of the gut, over a period of 9 years. The slides and blocks were retrieved and reviewed. Clinical data was obtained from hospital records. Clinicopathologic features were compared. PFL-GI cases had a slightly higher median age group (p value 0.23) and no gender predilection when compared to cases with secondary involvement which showed a female preponderance. Para-aortic lymphadenopathy was seen in all secondary cases whereas none of the primary cases showed significant lymphadenopathy. The only microscopic feature that was different was the presence of hollowed out pattern of immunostaining for follicular dendritic cells seen in all cases of PFL-GI but in none of the secondary cases

Research Advances of Immunotherapy for Follicular Lymphoma --Review / 中国实验血液学杂志

Follicular lymphoma is an indolent malignant tumor originating from lymph nodes and lymphoid tissues, which may affect the patients' quality of survival due to the recurrence and progression. In recent years, with the deepening understand of the molecular biology and signaling pathways, many new targeted drugs for follicular lymphoma have been discovered, such as monoclonal antibodies, checkpoint inhibitors, epigenetic regulation related targeted therapies and signaling pathway inhibitors. In this review, the new progress of immunotherapy for follicular lymphoma is summarized briefly.

Linfoma no Hodgkin: primera y segunda neoplasia o, transformación histológica: dificultad diagnóstica: reporte de un caso / Non-Hodgkin's lymphoma: first and second neoplasia or, histological transformation: diagnostic difficulty: a case report

El LF es una enfermedad heterogénea con muchos diferentes subgrupos, en términos de edad de inicio, órganos involucrados (especialmente sitios extranodales) y anormalidades genéticas. Con grandes variaciones en el curso clínico, algunas veces muy indolente o no; puede mostrar transformación a linfomas de alto grado, se han reportado transformaciones a Linfoma de Células Grandes B Difusas (siendo este el más común), de Burkitt, Leucemia/Linfoma, Linfoma Linfoblástico (reportado en raras ocasiones), con un curso clínico agresivo. En el 2008 la OMS estableció 3 nuevas variantes: 1) LF Pediátrico, 2) LF Primario Intestinal y 3) LF in situ. Patólogos y clínicos debemos considerar estas patologías cuando se decide el diagnóstico y el tratamiento. La transformación histológica se refiere al evento biológico que lleva al desarrollo de un Linfoma No Hodgkin agresivo de alto grado en pacientes con un LF subyacente. El Linfoma Folicular (LF) pediátrico es una neoplasia excesivamente rara, que difiere del LF del adulto.

FL is a heterogeneous disease with many different subgroups, in terms of age of onset, organs involved (especially extranodal sites) and genetic abnormalities. With great variations in the clinical course, sometimes very indolent or not; can show transformation to high-grade lymphomas, transformations to Diffuse Large B Cell Lymphoma (this being the most common), Burkitt's, Leukemia / Lymphoma, Lymphoblastic. Lymphoma (reported rarely), with an aggressive clinical course have been reported. In 2008 the WHO established 3 new variants: 1) Pediatric FL, 2) Intestinal Primary FL and 3) in situ FL. Pathologists and clinicians must consider these pathologies when deciding on diagnosis and treatment. Histological transformation refers to the biological event that leads to the development of high-grade aggressive Non-Hodgkin's Lymphoma in patients with an underlying FL. Pediatric Follicular Lymphoma (FL) is an excessively rare neoplasm, which differs from adult FL.

Linfomas en la era COVID-19 / Lymphoma and COVID-19

Resumen El coronavirus 2 del síndrome respiratorio agudo grave (SARS-CoV-2) y su traducción clínica, la enfermedad por coronavirus 2019 (COVID-19), representan una enfermedad con manifestaciones respiratorias potencialmente fatales. Actualmente existen aproximadamente 12,700,000 personas afectadas por esta virus, el cual ha ocasionado 561,517 muertes en el mundo. Los pacientes con diagnóstico de linfoma, al igual que otros pacientes con cáncer activo, presentan compromiso inmunitario, ya sea por su propia patología o debido al tratamiento que reciben, por lo que son especialmente susceptibles a desarrollar cuadros graves de COVID-19. La transmisión comunitaria del SARS-CoV-2 dificulta el acceso al sistema de salud y, por ende, el seguimiento estricto que requieren lo pacientes bajo tratamiento oncológico. En la etapa en la que nos encontramos actualmente, la transmisión global de la infección por SARS-CoV-2 continúa en ascenso, por lo que un control epidemiológico cercano es poco probable. Ante este contexto, surge la necesidad de establecer guías de tratamiento de pacientes con neoplasias hematológicas.

Abstract The SARS-CoV-2 virus and its clinical translation COVID-19 represent a disease with potentially fatal respiratory manifestations. Currently, there are approximately 12,700,000 people affected by this virus, which has caused 561,517 deaths worldwide. Patients with a diagnosis of lymphoma, like other patients with active cancer, have immune compromise either due to their own pathology or due to the treatment they receive, making them especially susceptible to developing severe cases of COVID-19. The community transmission of SARS-CoV-2 hinders access to the health system and, therefore, the strict monitoring required by patients undergoing cancer treatment. At the stage we are currently in, global transmission of SARS-CoV-2 infection continues to rise, making close epidemiological control unlikely. In this context, the need arises to establish guidelines for the treatment of patients with hematological malignancies.

Radiotherapy for early and advanced stages Follicular Lymphoma

OBJECTIVES To evaluate the results of radiotherapy (RT) for follicular lymphoma (FL) under different management scenarios. METHODS We retrospectively assessed consecutive patients with FL who had undergone irradiation between 2010 and 2018. All patients had biopsy-proven FL and were positron emission tomography-staged, although some (35.3%) were reassessed with computed tomography after treatment alone. Rituximab was only available to FL patients after 2016. RESULTS Thirty-four patients were selected, with a mean age at diagnosis of 61.6 years (34-89 years). The median follow-up duration was 49.4 months. Most patients were female (58.8%) and showed good performance on the Eastern Cooperative Oncology Group (ECOG) scale (ECOG 0-55.9%). The mean overall survival (OS) and progression-free survival were 48.7 and 33.6 months, respectively, with four deaths reported. OS rates at 2 and 3 years were 94.1% and 91.2%, respectively. Four patients showed transformation into aggressive lymphomas and underwent rituximab-based systemic treatment. Transformation-free survival was 47.8 months, and all patients with transformed disease were alive at assessment. Five patients had in-field relapse, all of them also relapsed elsewhere, and the mean relapse-free survival time was 40.3 months. No median end points were reached on assessment. CONCLUSION FL is an indolent disease. Our findings show good outcomes for patients treated with radiation, with a low transformation rate and excellent management of relapsed disease. RT is an important part of these results.

Leptomeningeal involvement by relapsed follicular lymphoma detected by flow cytometry despite exceedingly low white blood cell counts in cerebrospinal fluid: A case report

Follicular lymphoma is usually an indolent non-Hodgkin lymphoma that rarely involves the central nervous system (CNS) and flow cytometric detection of such lymphoma in cerebrospinal fluid (CSF) is often challenging because of low cellularity and viability of CSF samples. Here, we report our experience in a 52-year-old man whose relapsed follicular lymphoma with leptomeningeal involvement was confirmed by flow cytometric analysis of CSF, despite an exceedingly low white blood cell count (3/cumm).

Clinico-pathological analysis of lymphoproliferative disorders: a 3 year study

Background: The incidence of lymphoproliferative disorders has increased in many parts of the world. Newer subtypes have been identified by the new WHO classification. Accurate subtyping of lymphomas is crucial for prompt treatment. Objective of the study was to assess the clinicopathological pattern of lymphoproliferative diseases diagnosed in Rajagiri hospital over a period of 3 years.Methods: A retrospective study on all patients who were diagnosed with lymphoma in Rajagiri hospital during January 2016 to December 2018 was conducted and the data were reviewed and analyzed.Results: A total of 151 patients were included in the study. Majority of the subjects (63%) were males. The predominant age group affected was 61-80 years. Mean age group was 58.46 years (SD=19.05 years). Most common presenting symptom was painless lymphadenopathy. B symptoms were seen in 18% of subjects and was found to be more commonly associated with B cell Non-Hodgkin lymphoma. Most common lymph node involved was cervical lymph node, while the most common extra nodal site was bone marrow. Most common lymphoproliferative disorder was Diffuse large B cell Lymphoma. Lymph node involvement was found in 74% of patients, while hepatomegaly and splenomegaly were seen in 11% and 18.5% of patients. 20% of patients had secondary bone marrow involvement. 8 patients developed recurrent lesions involving other organs.Conclusions: Clinicopathologic patterns of lymphoproliferative disorders vary across various regions. A proper understanding of demographical distribution of lymphomas is very essential, as it can provide valuable clues for accurate diagnosis and treatment.

Brief review of the prognostic factors of follicular lymphoma / 中国肿瘤临床

Follicular lymphoma (FL) is a disease that features strong biological, clinical, and prognostic heterogeneity and is affected by various factors. The historically used prognostic factors such as those involved in the Follicular Lymphoma International Prognostic Index (FLIPI) and FLIPI-2, which are broadly based on clinical and laboratory features at the time of diagnosis, are of limited value. With the development of molecular imaging and gene sequencing technology, the prognostic value of the metabolic tumor burden parameters and molecular biological features (e.g., characteristics of the tumor microenvironment and epigenetic deregulation) in FL have been gradually recognized as promising, particularly in combination with the well-established clinically based prognostic indexes such as m7-FLIPI, a clinicogenetic risk model that includes the mutation status of seven genes. In addition to the baseline prognostic factors, the prognostic value of response to therapy has also been a hot topic in recent years. This review discusses the prognostic factors that affect patients with FL, referring to histological grade, metabolic tumor burden, clinically based prognostic indexes, tumor microenvironment, response to therapy, and molecular genetic characteristics, according to domestic and abroad research progress.

Analysis of prognostic and risk factors during the early relapse/progression of follicular lymphoma / 中国肿瘤临床

Objective: To investigate the incidence, prognostic value, and risk factors associated with progression of disease within 24 months (POD24) in patients with follicular lymphoma (FL). Methods: In Tianjin Medical University Cancer Institute and Hospital from January 2010 to December 2015,the clinical and follow-up data of 140 patients who were newly diagnosed with FL and received chemotherapy was collected.The expression levels of Myc, Bcl-2, Bcl-6, P53, CD10,MUM-1, and Ki-67 in tumor tissues from 75 patients before treatment were assessed with immunohistochemical (IHC) staining. Kaplan-Meier method and Cox regression model were applied to evaluate the effect of POD24 on the prognosis of FL. Logistic analysis was performed to identify the risk factors associated with POD24. Results: Among the 140 patients newly diagnosed with FL, 42 (30%) patients showed POD24. The results of Kaplan-Meier analysis revealed that patients with POD24 showed significantly reduced overall survival (OS) compared with patients without POD24 (Pupper limit of normal, high Ki-67 expression, and positive MUM-1 expression were risk factors associated with POD24 (all Pupper limit of normal, high Ki-67 expression, and positive MUM-1 expression were risk factors associated with POD24. Moreover, FLIPI ≥3 and positive MUM-1 expression were independent risk factors associated with POD24. The sensitivity and accuracy of FLIPI combined with MUM-1 (FLIPI-M) in predicting POD24 were higher than that of FLIPI alone, and thus have high value in clinical practice.

Frequent loss of CD10 expression in follicular lymphoma with leukaemic presentation

@# Follicular lymphoma (FL) is usually a nodal lymphoma expressing CD10, rarely with leukaemic presentation (FL-LP). Materials and Methods: We searched for FL-LP in our institution from 2000 to 2018 and characterised the neoplastic cells by flow cytometry, immunohistochemistry and fluorescence in situ hybridization. Thirteen (6.1%) of 212 FL cases were FL-LP, all de novo neoplasms. The leukaemic cells were small in 12 cases and large in one. All had concurrent FL, mostly (92%; 12/13) low-grade. The single case with large leukaemic cells had a concurrent primary splenic low-grade FL and a double-hit large B-cell lymphoma in the marrow. Results: CD10 was expressed in the leukaemic cells in 38% (5/13) cases by flow cytometry and in 77% (10/13) cases in tumours (p= 0.0471). IGH/BCL2 reciprocal translocation was identified in 85% (11/13) cases. Most patients were treated with chemotherapy. In a median follow-up time of 36 months, nine patients were in complete remission. The 2- and 5-year survival rates were at 100% and 83%, respectively. In this study, we characterised a series of de novo FL-LP in Taiwan. All patients had concurrent nodal and/or tissue tumours, which might suggest that these patients seek medical help too late. Conclusion: The lower CD10 expression rate by flow cytometry than by immunohistochemistry might be due to different epitopes for these assays. Alternatively, loss of CD10 expression might play a role in the pathogenesis of leukaemic change. The clinical course of FL-LP could be aggressive, but a significant proportion of the patients obtained complete remission with chemotherapy.

Clinical observation of five pediatric-type follicular lymphoma in adult / 中华血液学杂志

Objective@#To investigate the characteristics in pathological diagnosis, clinical features, treatment and prognosis of adult patients with pediatric-type follicular lymphoma (PTFL) .@*Methods@#The clinical and pathological features, laboratory examination, diagnosis and treatment, follow-up results of 5 adult PTFL patients admitted in Jiangsu Province Hospital were retrospectively analyzed, and literature review was conducted in combination with related reports.@*Results@#All 5 patients developed PTFL in their adulthood with a median age of 22 years old (15-33 years) . The initial inanifestation of the disease was local painless lymphadenopathy with no fever, night sweats, emaciation or other systemic B symptoms. Pathological characteristics including typical large follicular structures and high proliferation index were found. Meanwhile, additional clonal rearrangement of immunoglobulin heavy chain gene was observed. However, there was no BCL-2 expression in histochemistry as well as BCL-2 gene abnormality in fluorescence in situ hybridization among these PTFL patients. These adult PTFL patients were all in stage Ⅰ-Ⅱ of the disease. For treatment, they were only treated with local surgical excision after diagnosis while didn’t receive subsequent local radiotherapy or systemic immunochemotherapy. During a median follow-up of 27 months, the 5 cases of PTFL kept in a state of sustained complete remission.@*Conclusion@#Adult-onset PTFL is characterized by high pathological proliferation index, while no BCL-2 expression or BCL-2 gene abnormality. Besides, PTFL is clinically manifested as a localized disease that can achieve a quite good prognosis through local surgical intervention. The aforementioned attributes of PTFL are distinctly different from classic adult follicular lymphoma.

Clinical observation of five pediatric-type follicular lymphoma in adult / 中华血液学杂志

Objective: To investigate the characteristics in pathological diagnosis, clinical features, treatment and prognosis of adult patients with pediatric-type follicular lymphoma (PTFL) . Methods: The clinical and pathological features, laboratory examination, diagnosis and treatment, follow-up results of 5 adult PTFL patients admitted in Jiangsu Province Hospital were retrospectively analyzed, and literature review was conducted in combination with related reports. Results: All 5 patients developed PTFL in their adulthood with a median age of 22 years old (15-33 years) . The initial inanifestation of the disease was local painless lymphadenopathy with no fever, night sweats, emaciation or other systemic B symptoms. Pathological characteristics including typical large follicular structures and high proliferation index were found. Meanwhile, additional clonal rearrangement of immunoglobulin heavy chain gene was observed. However, there was no BCL-2 expression in histochemistry as well as BCL-2 gene abnormality in fluorescence in situ hybridization among these PTFL patients. These adult PTFL patients were all in stage Ⅰ-Ⅱ of the disease. For treatment, they were only treated with local surgical excision after diagnosis while didn't receive subsequent local radiotherapy or systemic immunochemotherapy. During a median follow-up of 27 months, the 5 cases of PTFL kept in a state of sustained complete remission. Conclusion: Adult-onset PTFL is characterized by high pathological proliferation index, while no BCL-2 expression or BCL-2 gene abnormality. Besides, PTFL is clinically manifested as a localized disease that can achieve a quite good prognosis through local surgical intervention. The aforementioned attributes of PTFL are distinctly different from classic adult follicular lymphoma.

Predictive Value of Interim and End-of-Therapy 18F-FDG PET/CT in Patients with Follicular Lymphoma / 대한핵의학회잡지

PURPOSE: ¹⁸F-fluorodeoxyglucose (FDG) positron emission tomography/computed tomography (PET/CT) is the standard imaging modality for response evaluation in FDG-avid lymphoma, but the prognostic value is not established in follicular lymphoma (FL). This study investigated the prognostic value of Deauville 5-point scale (D5PS) from paired interim PET/CT (PET(Interim)) and end-of-induction therapy PET/CT (PET(EOI)) in patients with FL.METHODS: FL staging and response assessment PET/CT images from 2013 to 2015 were retrospectively reviewed. PET(Interim) was performed 3 or 4 cycles after chemotherapy and PET(EOI) after 6 or 8 cycles. D5PS scores of 1, 2, and 3 were considered as negative (−), and scores 4 and 5 were considered as positive (+). Statistical analysis was done using Cox regression analysis, Kaplan-Meier survival analysis, and the log-rank test.RESULTS: Thirty-three patients with set of baseline, interim, and end-of-induction therapy PET/CTstudies were included. Ten patients (30.3%) had progression. The median progression-free survival (PFS) was 38.8 months (range 3.5–72.7 months). On PET(Interim), 23 patients were negative and 10 were positive. On PET(EOI) scans, 29 patients were negative, and 4 were positive. On multivariate analysis, PET(EOI)(−) was associated with longer PFS. PET(Interim)(+) and PET(EOI)(+) patients had a significantly shorter PFS than PET(Interim)(−) patients (39.9 months, 95%confidence interval [CI] 23.0–56.9, versus 55.5months, 95%CI 49.7–61.2, p=0.005) and PET(EOI)(−) patients (14.2 months, 95% CI 8.5–19.8, versus 60.5 months, 95% CI 52.1–69.0, p<0.001).CONCLUSION: For patients with FL, PET(Interim) and PET(EOI) response is predictive of PFS, and PET(EOI)(+) is an independent prognostic factor for progression of FL.

Radiation Therapy Outcome and Clinical Features of Duodenal-Type Follicular Lymphoma / Journal of the Korean Cancer Association, 대한암학회지

PURPOSE: Duodenal-type follicular lymphoma (FL) is a rare variant of FL. There is still no consensus on the initial treatment, and clinical features including endoscopic findings are not familiar to most physicians. The objective of this study was to evaluate the outcome of patients who were initially treated with radiation therapy for duodenal-type FL. MATERIALS AND METHODS: We retrospectively analyzed 20 patients who were consecutively diagnosed with duodenal-type FL between 2008 and 2017. All patients received radiation therapywith curative intent. RESULTS: The median age of the patients was 52 years (range, 26 to 66 years), and females were predominant. Most patients (n=18, 90%) had stage I disease, and were diagnosed by a regular health examination in an asymptomatic state. The histological grade was one in 19 patients (95%), and the endoscopic findings were diffuse nodular (n=8), whitish granular (n=8), and mixed pattern (n=4). Radiation therapy was delivered to 17 patients with 24 Gy in 12 fractions, and to three patients with 30.6-36 Gy in 18 fractions. All patients were evaluated with endoscopy for response to radiation therapy, and complete response was achieved in 19 patients (95%). At the time of analysis, all patients survived without any evidence of late toxicities related with radiation therapy. CONCLUSION: Taken together, radiation therapy alone could be effective in controlling duodenal lesion. A further study with longer follow-up duration is warranted to confirm our findings.

Prognostic Value of Baseline and Interim Total Metabolic Tumor Volume and Total Lesion Glycolysis Measured on ¹⁸F-FDG PET-CT in Patients with Follicular Lymphoma / Journal of the Korean Cancer Association, 대한암학회지

PURPOSE: The purpose of this study was to investigate the prognostic significance of total metabolic tumor volume (TMTV) and total lesion glycolysis (TLG) in patients with follicular lymphoma (FL) at baseline and mid-treatment with ¹⁸F-fluorodeoxyglucose positron emission tomography-computed tomography (PET-CT) scans. MATERIALS AND METHODS: The study analyzed data from 48 patients with FL who were treated in Jiangsu Province Hospital and reviewed their baseline PET-CT scans. TMTV and TLG were computed by using the absolute value of 2.0, 2.5, and 3.0 thresholding method, respectively. RESULTS: Median age was 53 years, 75.0% of patients had stage III to IV disease, 43.8% had a Follicular Lymphoma International Prognostic Index 1 (FLIPI1) score of 3 to 5 and 20.8% had a FLIPI2 score of 3 to 5. Receiver operating characteristic (ROC) curve analysis showed the optimal cut-off values for TMTV3.0 and TLG3.0 were 476.4 (sensitivity, 85.7%; specificity, 78.0%; area under the curve [AUC], 0.760; p=0.003) and 2,676.9 (sensitivity, 71.4%; specificity, 78.0%; AUC, 0.760; p=0.003). On multivariable analysis, TMTV3.0 and TLG3.0 were independent predictors of both progression-free survival (PFS) (hazard ratio [HR], 5.406; 95% confidence interval [CI], 1.326 to 22.040; p=0.019 and HR, 6.502; 95% CI, 1.079 to 39.182; p=0.042) and overall survival (OS) (HR, 4.111; 95% CI, 1.125 to 15.027; p=0.033 and HR, 5.885; 95% CI, 1.014 to 34.148; p=0.049). ROC curve analysis showed the optimal cut-off values for ΔTMTV3.0 and ΔTLG3.0 were 66.3% (sensitivity, 85.7%; specificity, 63.4%; AUC, 0.774; p 66.3%) and TLG (ΔTLG > 64.5%) reduction are valuable tools for early treatment response assessment in FL patients.