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Introduction: Rosai-Dorfman disease (RDD), also known as sinus histiocytosis, is a rare disorder characterized by histiocyte proliferation. Case presentation: A 33-year-old man consulted the emergency department of a tertiary care institution in Cartagena de Indias, Colombia, due to a 6-month history of progressive deformity in the frontal right side of the face, associated with pain of slow progression, without any other symptoms or dermatological involvement. There were no other major findings on physical examination and laboratory tests performed were negative. Imaging scans obtained showed extensive inflammatory involvement of the frontal bone, which led to suspect osteomyelitis as the first diagnostic possibility. A biopsy of the lesion was performed with negative cultures for bacteria, which allowed establishing a diagnosis of extranodal Rosai-Dorfman disease with isolated bone involvement. Treatment with systemic corticosteroids was indicated with poor response, so methotrexate was added, achieving an evident improvement after 2 months. Conclusions: Little is known about the manifestations of Rosai-Dorfman disease and its treatment in the adult population. The present case report contributes to expanding the literature on this topic, which can present with rare symptoms that may pose challenges for its diagnosis. RESUMEN Introducción. La enfermedad de Rosai-Dorfman, también conocida como histiocitosis sinusal, es un trastorno poco frecuente caracterizado por la proliferación de histiocitos. Presentación del caso. Hombre de 33 años quien consultó al servicio de urgencias de una institución de tercer nivel de Cartagena de Indias, Colombia, por una deformidad de progresión lenta que apareció 6 meses atrás en el costado frontal derecho de la cara y generaba dolor. Al examen físico no hubo hallazgos mayores y los estudios paraclínicos fueron normales o negativos. Se obtuvieron imágenes que evidenciaron extenso compromiso inflamatorio/infeccioso del hueso frontal, por lo que se sospechó de osteomielitis y se realizó biopsia de la lesión que permitió establecer diagnóstico de enfermedad de Rosai-Dorfman variante extranodal con compromiso óseo aislado. El paciente recibió tratamiento con corticoide sistémico sin obtener respuesta, por lo que se adicionó metotrexato, con lo cual se logró mejoría a los 2 meses. Conclusiones. Las manifestaciones de la enfermedad de Rosai-Dorfman en población adulta son poco conocidas, además no hay mucha información sobre su tratamiento, por lo que el presente caso contribuye a ampliar la literatura sobre esta enfermedad que puede presentarse con sintomatología completamente inusual.

BACKGROUND Rosai–Dorfman disease (RDD) is a rare histiocytic proliferation of unknown etiology commonly found in children and adolescents. The common manifestation of RDD is massive and painless bilateral cervical lymphadenopathy with extranodal disease. While extranodal involvement in RDD is common, the spleen is an infrequent site of disease. CASE SUMMARY We report a 10-mo-old female infant with RDD presenting multiple splenic masses without cervical lymphadenopathy. She had fever, and blood tests showed leukocytosis, anemia, and elevated erythrocyte sedimentation rate and C-reactive protein. Ultrasound, computed tomography, and magnetic resonance images demonstrated multiple splenic masses. Despite antibiotic therapy, her symptoms were not relived. She underwent diagnostic splenectomy and was discharged with recovery. CONCLUSION In pediatric patients with refractory infectious symptoms or hematological abnormalities, clinicians should suspect RDD, even in patients without significant lymphadenopathy.

In the staging of cancer patients, transient and spontaneously reversible bilateral adrenal hypertrophy may mimic a secondary localization of the disease. We discuss the case of an 82-year-old male patient with suspected testicular neoplasia in which abdominal CT examination reveals the onset of a bilateral macronodular adrenal enlargement, suggesting the diagnostic hypothesis of primary testicular neoplasia with secondary adrenal localization. The subsequent 18FDG-PET/CT study showed hyper-metabolism of the testicular mass, while the adrenal glands, surprisingly, did not show increased uptake of the radiotracer. After right orchifunicolectomy, primary testicular diffuse large B-cell lymphoma was diagnosed. The subsequent staging PET/CT study with iodine contrast medium, three months after the first CT examination, showed spontaneous complete regression of the adrenal hypertrophy without any use of drug therapy. The differential diagnosis of this finding considered the lack of hypermetabolism and the densitometric characteristics of the adrenal glands, the absence of possible pharmacological interactions throughout the time of the diagnostic procedures, and the available clinical-laboratory data. By excluding the main causes of adrenal hypertrophy, the most likely diagnostic hypothesis was transient adrenal hypertrophy due to stress induced by testicular lymphoma, meaning by stress a disturbance not only emotional but also an alteration of organic homeostasis. Our case suggests that the analysis of adrenal lesions appeared in cancer patients should take into account non-metastatic conditions that must be studied with a multimodal approach and with serial investigations.

Non-Hodgkin’s Lymphoma is the commonest among all forms of Lymphomas. It may manifest with nodal and extranodal involvement. Salivary glands, waldeyer’s ring, paranasal sinuses are the common sites of extranodal involvement in the head and neck region. Gingiva, buccal mucosa and palate are the intraoral sites of extranodal involvement. Oral manifestations include painful and mobile teeth, gingival or mucosal swellings which may mimic odontogenic pathologies. Intraoral and orbital presentation of Non-Hodgkin’s Lymphoma is a sign of aggressive behavior and poor prognosis. Lymphomas have a possibility of relatively early diagnosis with systematic examination of oral tissues and the head and neck region, and timely histopathological and radiological investigations. Keywords: Extranodal, NonHodgkin’s Lymphoma, Optic nerve.

An IgG4 ( +) plasma cell increase can be observed in classic Hodgkin lymphoma (CHL) in addition to IgG4-related disease. IgG4 ( +) plasma cells can affect therapy or prognosis in CHL because the microenvironment of CHL can help cancer escape immune surveillance. Therefore, we aimed to determine the relationship between IgG4 ( +) plasma cell increase and the clinicopathological parameters of CHL subtypes. A total of 76 cases diagnosed with CHL by lymph node excision between 2006 and 2020 in our hospital were selected. The probable histological features of IgG4-related disease were utilized for diagnosis. The presence of IgG4 was determined by immunohistochemistry. IgG4 ( +) plasma cell increase was mostly seen in nodular sclerosis CHL, but it was also observed in other CHL subtypes, except for lymphocyte-rich CHL. While IgG4 ( +) plasma cell increase was related to a limited stage of progression, it was not related to mean age, gender, pediatric or adult status, involvement of the mediastinum in the cancer, the presence or absence of the Epstein–Barr virus, overall survival, and neutrophil or eosinophil infiltration. It was also inversely correlated with extranodal involvement, granuloma, and necrosis. IgG4 ( +) plasma cell increase can affect stage, subtype, and microenvironment in CHL and has more influence on disease via changes in rising cases; thus, these findings can serve as a guide for large studies, which can help improve treatment and prognosis in CHL.

Diffuse large B-cell lymphoma (DLBCL) is the most common type of non-Hodgkin lymphoma in Indian population and is divided into the prognostically important subtypes, germinal center B-cell (GCB) and activated B-cell-like (ABC), using immunohistochemistry-based algorithm.The present study aims to evaluate the influence of immunohistochemical derived DLBCL subtype, GCB or ABC on prognostically significant variables - extranodal involvement and serum lactate dehydrogenase (LDH) level at presentation, and response to chemotherapy assessed on pre- and posttreatment fluorodeoxyglucose-positron emission tomography study.This was a retro-prospective, 2-year observational study at a tertiary health-care center, New Delhi.The study population includes a total 236 cases of DLBCL. According to the Hans algorithm, DLBCL cases were allocated to the GCB and ABC subgroups.For comparison of mean values, independent t-test and analysis of variance were used. For this purpose, we used SPSS 20.0 software. P0.05 was considered as statistically significant.Ninety-eight patients (41.5%) had GCB immunophenotype and 138 patients (58.5%) were ABC. A significant difference was observed between mean baseline level of LDH between GCB and ABC subtypes (P0.05). The proportion of cases with extranodal involvement was comparatively higher in ABC subtype (P0.05). Association between response to chemotherapy with DLBCL immunophenotypes was found to be highly significant (P0.00). The response rates were much better in GCB subtype.The mean baseline level of LDH is significantly higher in ABC subtype. The proportion of cases with extranodal involvement was comparatively higher in ABC and shows poor response to chemotherapy as compared to GCB. Baseline LDH level was found to be important prognostic marker in the DLBCL.

In situ mantle cell neoplasia (ISMCN) is a rare entity of disputed clinical significance. We report an additional case, unusual by its presentation in the large intestine and its multifocal involvement of several nodal and extranodal sites. The diagnosis was made in a 46-year-old male patient from a surgical specimen resected for cecal adenocarcinoma. Gross examination showed multiple small polypoid lesions surrounding the ileocecal valve, corresponding to lymphoid aggregates with hyperplastic follicles. Numerous cyclin D1/SOX11+ lymphoid cells, harboring the t(11;14)(q13;q32) translocation, were present in the inner layers of mantle zones. The same lesions were found in the ileum, the appendix, and the regional lymph nodes. The final diagnosis was multifocal ISMCN of the ileocecal region, with both nodal and extra-nodal involvement. A simple surveillance was decided. Our observation expands the clinical spectrum of the disease and underlines the necessity to closely examine even normal-appearing reactive lymphoid tissues.

BACKGROUND Rosai-Dorfman disease (RDD), or sinus histiocytosis with massive lymphadenopathy, is a benign histiocytic disorder. Extranodal involvement is common, occurring in > 40% of patients, but bone involvement occurs in < 10% of cases. In addition, primary bone RDD is extremely rare. The majority of patients are adolescents and young adults, and the mean age at onset is 20-years-old. CASE SUMMARY We report an 8-year-old Chinese girl who presented to our hospital with an insidious onset of swelling and pain in the middle shaft of her right tibia for 4 mo. We performed total surgical resection of the right tibia lesion and allograft transplantation. A good prognosis was confirmed at the 6 mo follow-up. Pain and swelling symptoms were totally relieved, range of motion of her right knee and ankle returned to normal, and there was no clinical evidence of lesion recurrence at last follow up. Our case is the second reported case of osseous RDD without lymphadenopathy in the shaft of the tibia of a child. CONCLUSION Extranodal RDD is a rare disease and can be misdiagnosed easily. Lesion resection and allograft transplantation are an option to treat extranodal RDD in children with good short term result. Pediatric orthopedist should be aware of this rare disease, especially extranodal involvement.

Background/Aims: Compared with Western countries, chronic lymphocytic leuke mia (CLL) rarely occurs in Asia and has different clinical characteristics. Thus, we aimed to evaluate the clinical characteristics, treatment outcomes, and prognostic significance of Korean patients with CLL. Methods: We retrospectively analyzed 90 patients with CLL who had received che motherapy at 6 centers in Korea between 2000 and 2012. Results: Compared with Western patients with CLL, Korean patients with CLL express lambda (42.0%) and atypical markers such as CD22 and FMC7 (76.7% and 40.0%, respectively) more frequently. First-line chemotherapy regimens included chlorambucil (n = 43), fludarabine and cyclophosphamide (FC) (n = 20), fludarabine (n = 13), rituximab-FC (n = 4). The remaining patients were treated with other various regimens (n = 10). The 5-year overall survival (OS) and progression-free survival (PFS) rates were 79.3% and 28.1%, respectively. Multivariate analyses showed that hyper leukocytosis (≥ 100 × 103/μL), extranodal involvement, and the Binet C stage were sig nificant negative prognostic factors for OS (hazard ratio [HR] 4.75, p = 0.039; HR 21.6, p = 0.002; and HR 4.35, p = 0.034, respectively). Cytogenetic abnormalities including complex karyotypes (≥ 3), del(11q), and del(17) had a significantly adverse impact on both OS and PFS (p < 0.001 and p = 0.010, respectively). Conclusions: Initial hyperleukocytosis, extranodal involvement, complex karyotype, del(17) and del(11q) need to be considered in the risk stratification system for CLL.

Objective and importance Rosai-Dorfman disease (RDD) is a benign and rare non-Langerhans cell histiocytic proliferative disorder. Laryngeal involvement is an unusual site of extranodal involvement of RDD. Laryngeal RDD can cause life-threatening airway obstruction that requires effective control of the disease. In this study, we report three cases of laryngeal RDD with excellent and durable responses to thalidomide. Clinical presentation Patient 1 was a 39-year-old male who presented with a two-year history of nasal obstruction. Patient 2 was a 26-year-old woman who presented complaining of a hoarse voice for one year. Patient 3 was a 24-year-old man who presented with complaints of a hoarse voice and progressing dyspnea for five months. Electronic laryngoscopy revealed submucous nodular lesions in the nasal cavity, nasopharynx, and larynx of the three patients. Biopsy of the lesions showed large histiocytes with abundant pale cytoplasm which were S-100 and CD68 positive consistent with RDD. Intervention Before thalidomide treatment, patient 1 received chemotherapy and six times surgical excision due to the recurrence of laryngeal lesions. Patient 2 failed steroid treatment. Patient 3 underwent an emergency tracheostomy due to airway obstruction. All three patients then received thalidomide 100 mg/d treatment and achieved satisfactory and durable responses with the longest follow-up of 45 months. Conclusion Thalidomide may induce long-term remission in laryngeal RDD.

Because non–anthracycline-based chemotherapy with l-asparaginase has improved survival outcomes in patients with extranodal natural killer/T-cell lymphoma (ENKTL), the incidence of central nerve system (CNS) relapse can be different when compared with that in previous reports. In this research, we sought to identify the incidence of and predictors for CNS relapse and to evaluate the necessity of CNS prophylaxis with intermediate-dose methotrexate (ID-MTX). The records of 399 patients in the training cohort and 253 patients in the validation cohort with ENKTL who received non-anthracycline–based chemotherapy were reviewed. Patients were divided into 2 groups according to whether the chemotherapy regimen included ID-MTX above 2 g/m2. A new central nervous system-prognostic index of natural killer (CNS-PINK) model was developed using 1-point powerful predictors of CNS relapse (PINK; hazard ratio [HR], 2.908; P = .030 and extranodal involvement [≥2]; HR, 4.161; P = .001) and was calculated as a sum of scores. The high-risk group of CNS-PINK was defined as 2 points. The cumulative incidence of CNS relapse was different between the CNS-PINK risk groups in the training (P < .001) and validation (P = .038) cohorts. Patients in the high-risk CNS-PINK group who were treated with SMILE or SMILE-like regimens with ID-MTX (S-ID-MTX) displayed a lower incidence rate of CNS relapse than did those who received other regimens without ID-MTX in the training cohort (P = .029). The CNS-PINK was demonstrated its strong predictability of CNS relapse in ENKTL patients. The effectiveness of S-ID-MTX in preventing CNS events in high-risk CNS-PINK patients should be verified in future studies.

Background/aim TP53 mutation is recognized as a negative prognostic factor for patients with diffuse large B-cell lymphoma (DLBCL). Here, we present the characteristics of TP53 mut DLBCL patients following investigation of the effect of a treatment approach on survival of TP53 mut DLBCL patients. Methods A total of 44 DLBCL patients with TP53 mut and treated with an R-CHOP regimen were included for analysis. Patients who failed to achieve a complete response (CR) to initial treatment or relapsed in the first 6 months after initial CR were deemed to have primary refractory disease. Results Among 44 patients harboring TP53 mutations who underwent upfront R-CHOP or R-CHOP-like treatment, 21 (47.7%) had limited-stage and 23 (52.3%) presented advanced-stage disease. Apart from the seven patients receiving upfront surgical resection, 37 had measurable disease under the R-CHOP regimen, with 59.1% (n=26) developing primary refractory disease. Seven limited-stage patients after early complete resection and one with residue resection remained event-free at median follow-up of 37 months. Multivariate analysis revealed that elevated baseline lactate dehydrogenase (LDH), extranodal involvement (two or more), Ann Arbor stage, and locoregional treatment (surgery or radiation therapy) were independent indicators for progression-free survival (PFS). After adjustment for baseline LDH and extranodal involvement, adding locoregional treatment including surgery and radiation to the R-CHOP regimen significantly improved PFS (p=0.008) and overall survival (p=0.017) in limited-stage TP53 mut DLBCL patients compared to R-CHOP-only treatment. Conclusion This study presents the characteristics of TP53-mutated DLBCL and implies a potential benefit of locoregional treatment in limited-stage DLBCL patients with TP53 mutations.

Rosai-Dorfman disease (RDD), also known as sinus histiocytosis with massive lymphadenopathy, is a rare disease typically characterized by a histiocytic proliferation within lymph nodes, which is due to an unknown etiology. Extranodal involvement can occur, and it more rarely can involve the skin. RDD generally presents with an indolent nature and follows a benign disease course, although more aggressive cases have been reported. The condition predominately affects children and young adults. It is classically characterized by massive, bilateral painless lymphadenopathy and accumulation of CD68-positive, S100-positive, CD1a-negative histiocytes, with the presence of emperipolesis as a hallmark. Herein, we present an aggressive case in a 76-year-old male with past medical history significant for prostate cancer, who presented with a 7-month history of lymphadenopathy and new onset of multiple large abdominal wall, cutaneous, lymph node, liver, and lung masses, all of which were histopathologically atypical, but showed features consistent with RDD, including emperipolesis and strong S100 positivity. Molecular studies showed a KRAS 117N mutation, which has been recently reported in RDD. While most cases present as a benign tumor, this case demonstrated aggressive features clinically, showed partial response to MEK inhibitor immunotherapy in the setting of a KRAS mutation, and demonstrated atypical cytologic features on histopathology.

Blastoid and pleomorphic mantle cell lymphoma (MCL) are among the worst prognostic, aggressive histology, high-risk variants of MCL, and, in this article, they are presented as blastoid MCL. Blastoid MCL have not been systematically studied, probably due to their rarity. De novo blastoid MCLs have superior outcomes compared with transformed MCL. Compared with classic MCL, extranodal involvement (mainly skin, central nervous system), frequent relapses, and inferior responses to conventional chemoimmunotherapy, BTK inhibitors and venetoclax are frequent in blastoid MCL. KTE-X19 induces excellent response in blastoid MCL. Combinations with novel agents are actively investigated. This article presents a comprehensive review on blastoid MCL in 2020.

Background: Diffuse large B-cell lymphoma (DLBCL) is the most common subtype (68.2%) of B cells Non Hodgkin Lymphoma in Indonesia. This tumor heterogeneity is characterized by a variety of clinical conditions, morphology, genetic profiles, therapeutic response, prognosis and survival. Recent studies have shown that CD30 immunohistochemical staining also plays an important role in determining the therapy and prognosis of DLBCL disease. CD30 can also be expressed in DLBCL in approximately 9.5-40%. However, CD30 expression and clinicopathological characteristics of Indonesian DLBCL remain unknown. This study aimed to determine the prevalence of CD30 expression and its correlation with clinicopathological characteristics of Indonesian DLBCL patients. Methods: During a study period of four years, a total of 104 FFPE of DLBCL cases were collected from Anatomical Pathology Department, Sardjito Hospital, Special Region of Yogyakarta, Indonesia. CD30 expression was studied using immunohistochemical techniques (Mouse monoclonal antibody MoAb CD30 cell marque Ber-H2). Correlations between positive CD30 immunoreactivity and clinicopathological characteristics in DLBCL patients were statistically analyzed using chi-square tests. Result: Positivity rate of CD30 expression in 104 DLBCL samples was 13.5% (14/104) using cutoff value of 0% while using a 20% cutoff, it was 1.9% (2/104). Statistical associations of positive CD30 expression and clinicopathological characteristics (age, sex, Ann Arbor stage, extranodal involvement and morphological variants) were not significant (p > 0.05).Conclusions: The prevalence of positive CD30 expression in Indonesian DLBCL patients is 13.5%. There was no statistically significant associations between positive CD30 expression and clinicopathological characteristics.

Occasionally, non‐Hodgkin lymphomas (NHL) occur simultaneously or subsequently to Hodgkin disease. We report on a case of a woman with Hodgkin lymphoma treated with ABVD, who developed 4 years later T‐cell NHL with both nodal and extranodal involvement. Brentuximab vedotin could be an effective choice in treating metachronous T‐cell NHL.

Hodgkin lymphoma (HL) is an uncommon malignancy usually limited to the lymph nodes and lymphatic system while extranodal involvement is much less common than in non-Hodgkin lymphoma. The current report presents an unusual case of primary classical HL (cHL), nodular sclerosis type with mixed cellularity in buttock soft tissue of 78-year old man. Primary lymphoma of the gluteal muscle is a rare disease and primary cHL is even rarer. In addition, to this unusual extranodal presentation, the present case highlight a diagnostic challenge in fine-needle biopsy masquerading a low grade sarcoma, primarily myxoinflammatory fibrosarcoma or an inflammatory lesion. However, surgical biopsy and immunohistochemistry guided correct diagnosis that was of major interest for further successful treatment.

Purpose: The aim of this study is to assess the diagnostic value of magnetic resonance imaging (MRI), alone or combined with positron emission tomography (PET), for evaluation of mediastinal disease in lymphoma in comparison with PET/computed tomography (CT). Material and methods: Eighteen prospective studies with sample size ranging between 17 and 140 patients were included in this analysis. MRI, regardless of the sequence obtained, where used for the evaluation of lymphoma. Histopathology results and clinical or imaging follow-up were used as the reference standard. Studies were excluded if the sample size was less than 15 cases or if less than 15 lymph nodes assessment were presented. Papers not reporting sufficient data were also excluded. Results: As compared to PET/CT, MRI proved to be an alternative to use in evaluation of mediastinal disease of lymphoma. MRI has high sensitivity and specificity for nodal and extranodal involvement in lymphoma and the agreement between MRI with the standard technique was high. The ALARA principle reinforces these findings because it is a radiation-free technique and its use is preferred in young population at risk of being subjected to several studies. Conclusion: MRI seems to be a promising and free-radiation alternative due to its high sensitivity and specificity, as well as its high level of agreement with the standard reference. This suggests that MRI could play a significant role in lymphoma study. Resumen: Objetivo: El objetivo de este estudio es evaluar el valor diagnóstico de la resonancia magnética (RM), sola o combinada con la tomografía por emisión de positrones (PET), para la valoración de la afectación mediastínica en el linfoma en comparación con la tomografía computarizada (TC)/PET. Material y métodos: Se incluyeron en el análisis 18 estudios prospectivos con un tamaño de muestra que oscilaba entre los 17 y los 140 pacientes. Se utilizó la resonancia magnética, independientemente de la secuencia obtenida para la evaluación del linfoma. Los resultados de histopatología y el seguimiento clínico o de imagen se utilizaron como estándar de referencia. Se excluyeron los estudios si el tamaño de la muestra era inferior a 15 casos, o si se presentaron evaluaciones de menos de 15 ganglios linfáticos. Los artículos que no proporcionaban datos suficientes también fueron excluidos. Resultados: En comparación con PET/TC, la RM demostró ser una alternativa útil en la evaluación de la afectación mediastínica del linfoma. La RM tiene una alta sensibilidad y especificidad para detectar afectación nodal y extranodal en el linfoma y la concordancia entre la RMy la técnica estándar fue alta. El principio ALARA refuerza estos hallazgos porque es una técnica libre de radiación y su uso se prefiere en la población joven en peligro de ser sometida a varios estudios. Conclusión: La RM parece ser una alternativa prometedora y libre de radiación debido a su alta sensibilidad y especificidad, así como a su alto nivel de concordancia con la referencia estándar. Esto sugiere que la RM podría desempeñar un papel importante en el estudio del linfoma.

Background The programmed death receptor (PD-1) and ligand (PD-L1) pathway act by suppressing the antitumor response in chronic Hodgkin lymphoma (cHL). In this study, we aimed to investigate the effect of PD-1, PD-L1, and Epstein-Barr virus (EBV) positivity on prognosis at the initial diagnosis of cHL. Material and Methods Thirty-six patients with cHL were retrospectively analyzed. PD-L1 staining was performed for RS cells and tumor microenvironment in the biopsy materials of cases. The presence of EBV was investigated by EBER (EBV-encoded RNA) method in tumor cell. P Results The presence of advanced-stage disease, B symptoms, intermediate or high-risk international prognostic index (IPS), and extranodal involvement were found to be related to both PD-L1 positivity and EBV positivity in RS cells. PD-L1 positivity in RS cells was also associated with EBV positivity. There were 6 (16.7%) triple-positive (EBV+, RS-PD-L1+, mic-PD-1+) patients. All of these patients had advanced-stage disease, B symptoms at the time of diagnosis, and intermediate-high IPS score, and 4 of 6 patients had extranodal involvement. This group also had significantly shortened overall survival compared with others (38.4 months vs. 67.9 months P = .024). Conclusion Our data suggest that there is correlation between PD-L1 positivity and EBV positivity in tumor RS cells that are also associated with extranodal involvement, intermediate and high IPS score, presence of B symptoms, and advanced-stage disease. In addition, we identified a group of triple-positive (EBV+, RS-PD-L1+, mic-PD-1+) cHL patients who have a very high-risk disease.

Intralymphatic spread is a rare finding and is associated with poor prognosis in diffuse large B cell lymphoma (DLBCL). Here, we report a case of relapsed DLBCL with mucosal intralymphatic spread. A 69-year-old man had been diagnosed with gastric DLBCL stage IIE at 57 years. He had a relapse with generalized lymphadenopathy and extranodal involvement at 61 years; then, second complete remission was achieved after salvage chemotherapy. He then had a second relapse with involvement of the terminal ileum, spinal cord, and left tonsil. The terminal ileum showed intralymphatic spread in the lamina propria of the intestinal villi, which was confirmed by D2–40 immunostaining. Eleven months later, another biopsy showed prominent intralymphatic spread in the mucosa of the terminal ileum. After salvage therapies, the spinal cord and tonsillar tumors resolved, but the intestinal tumors were refractory. The patient eventually died of progressive disease. In contrast to previously reported cases, the involved lymphatic vessels were observed in the mucosa, and the lymphoma cells expressed CD5 in the first colonoscopic biopsy. This rare case broadens the spectrum of intralymphatic spread in DLBCL.

Mantle cell lymphoma (MCL) is a B-cell non-Hodgkin lymphoma often with extranodal involvement at diagnosis, and yet how this feature correlates with survival awaits elucidation. To address this issue, a correlative analysis between clinical features of 127 MCL patients and their overall survival (OS) was conducted. In this cohort, the median age at MCL diagnosis was 62 years and 81% were males. Eighty-four percent of patients were Ann Arbor stage 4, and 15% were blastoid variants. In patients with gastrointestinal MCL, approximately 40% had gastric involvement. In treatment, CHOP-based induction chemotherapy was given to 61.1% of patients. One-third of patients undertook autologous stem cell transplant (SCT), and 4.7% had allogeneic SCT. The median OS was 82 months and well-stratified in MIPI risk groups. In the multivariate analysis for OS, blastoid variants and gastric involvement were both independent risk factors whereas auto-SCT had a protective effect. Overall, this study corroborated with the current understandings and international therapeutic standards for MCL. Auto-SCT associated with a better OS while allo-SCT remained an option for blastoid variants and those who failed Auto-SCT. Interestingly, patients with gastric involvement tended to have worse survival, a finding that spawns more studies to investigate the mechanism.

Rosai-Dorfman disease (RDD) is a rare and self-limiting disease process that presents most commonly in young patients as massive, painless, cervical lymphadenopathy. Extranodal involvement may also occur. Histopathologic evaluation is the main diagnostic modality. We report an unusual presentation of RDD with cervical lymphadenopathy and an incidentally discovered sinonasal mass, clinically worrisome for malignancy. We emphasize that a high index of clinical suspicion is critical for accurate diagnosis of RDD. Clinicians and pathologists should consider RDD in a differential diagnosis of cervical lymphadenopathy, especially in young patients.