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BACKGROUND: Aspartate aminotransferase (AST), an indicator of liver cell damage, was related to the prognosis of certain malignant tumors. OBJECTIVE: This study examined the predictive value of AST in patients with extranodal natural killer/T cell lymphoma (ENKTL). METHODS: We reviewed 183 cases diagnosed with ENKTL and selected 26 U/L as the optimum cut-off value of AST. We used the univariate and multivariate Cox regression to compare the different AST groups' overall survival (OS) and progression-free survival (PFS). RESULTS: Prior to propensity score matching (PSM), Kaplan-Meier analysis showed that patients in the low AST subgroup had better OS and PFS than the high AST subgroup. Multivariate analysis revealed that AST was an independent indicator for prognosis. After PSM, the low AST subgroup maintained a significantly better OS and PFS than the high AST subgroup. CONCLUSION: AST might represent a significant prognostic marker for ENKTL patients. [ABSTRACT FROM AUTHOR]

BACKGROUND: At present, peripheral blood markers are easily accessible information and clinically valuable prognostic indicators in extranodal nasal-type natural killer/T-cell lymphoma (ENKTCL). Nevertheless, the role of its comprehensive score in ENKTCL remains to be determined. OBJECTIVE: Therefore, this study aimed to investigate the prognostic effect of the peripheral inflammation score on ENKTCL. METHODS: The retrospective study included 183 patients with ENKTCL. Univariate Cox regression analyses and least absolute shrinkage and selection operator (LASSO) Cox regression were used to construct the inflammation-related prognostic index named Risk. Univariate and multivariate Cox regression analyses and regression adjustment with propensity score matching (PSM) were used to evaluate the prognostic ability of risk. The performance of the modified nomogram-revised risk index (NRI) by integrating risk was evaluated with the area under the time-dependent receiver operating characteristic (ROC) curve (AUC), decision curve analysis (DCA), and integrated Brier score (IBS). RESULTS: The risk cut-off value, constructed by the lymphocyte count, platelet count, albumin level, LMR, and PNI, was - 1.3486. Before PSM, multivariate analysis showed that risk was significantly associated with OS (HR = 2.577, 95% CI = 1.614–4.114, P < 0.001) and PFS (HR = 2.679, 95% CI = 1.744–4.114, P < 0.001). After PSM adjustment, risk was still an independent factor for OS (HR = 2.829, 95% CI = 1.601–5.001, P < 0.001) and PFS (HR = 2.877, 95% CI = 1.735–4.770, P < 0.001). With the NRI, the modified NRI by integrating risk increased the AUC and clinical net benefit and decreased the IBS. CONCLUSIONS: Risk is an easily accessible and inexpensive indicator that may be used as a prognostic marker and could improve NRI predictive power in patients with ENKTCL. [ABSTRACT FROM AUTHOR]

Extranodal Natural Killer/T Cell Lymphoma Nasal Type (EN-NK/T-CL-NT) is a rare type of non-Hodgkin lymphoma in which the lesion is usually located in the upper respiratory tract, such as nasal cavity, palate, and nasopharynx. In addition, the primary lesion of EN-NK/T-CL-NT can rarely originate in extranasal sites such as the skin, gastrointestinal tract, testicles, central nervous system, and lungs. We describe an 82-year-old male smoker was brought to the hospital with 8 months of fever, cough, sputum production, chest pain, and chest tightness. Computed tomography (CT) of the chest showed subpleural high-density shadow in the lower lobe of the right lung with unclear borders and surrounding patchy ground-glass shadow. Initially, the patient's right lower lobe lesion progressed after receiving anti-inflammatory treatment. He subsequently underwent two computerized tomography (CT)-guided percutaneous transthoracic needle aspiration biopsies and a bronchoscopy, but no tumor cells were found. Through multidisciplinary team discussions, the patient was then transferred to the department of cardiothoracic surgery for right lower lobectomy. Finally, extranodal natural killer/T-cell lymphoma (ENKTCL), nasal type, was confirmed by pathology of the surgical specimen. The diagnosis of primary pulmonary ENKTCL was made because no evidence other than extrapulmonary site was found at the time of diagnosis and treatment. Here we report a case of primary pulmonary extranodal natural killer/T-cell lymphoma of nasal type presenting as pneumonia in the right lower lobe and enhance the understanding of the disease.

Anti-SARS-CoV-2 vaccines were developed in response to the coronavirus disease 2019 (COVID-19) pandemic caused by severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2). Although the BNT162b2 mRNA vaccine is effective, adverse effects have been reported. Here, we report a case of extranodal NK/T-cell lymphoma, nasal type (ENKL), of the left arm following BNT162b2 mRNA vaccination. A 73-year-old male presented with a lump in the left arm, which was the site where he received the BNT162b2 mRNA vaccine 3 months prior. He was treated with topical corticosteroids and debridement, but the tumor progressed. Additionally, fever, night sweats, and general fatigue were observed. Laboratory findings included thrombocytopenia, elevated lactate dehydrogenase, and soluble interleukin-2 receptor levels. Skin biopsy led to a diagnosis of ENKL. The patient was treated with a 50% dose of SMILE therapy and radiotherapy, resulting in regression of the tumor. It seems that latent Epstein–Barr virus (EBV)-infected NK/T cells were reactivated by vaccination and contributed to the onset of ENKL. This is the first report of ENKL after BNT162b2 mRNA vaccination. The present case highlights the possible risk of development of malignant lymphoma, including ENKL at the injection site, after BNT162b2 COVID-19 vaccination. [ABSTRACT FROM AUTHOR]

Abstract Background Extranodal natural killer/T-cell lymphoma, nasal type (ENKL) of the small intestine, is a disease with extremely poor prognosis. We describe treatment in a case which is novel in that it demonstrated long-term survival. Case presentation A 68-year-old man was admitted to the emergency department of our hospital with the complaint of severe umbilical pain with tenderness and muscular defense. An abdominal computed tomography scan revealed a thick-wall mass on the small intestine and intra-abdominal free air. He was suspected of perforation of a small intestinal tumor and underwent emergency surgery. The surgery revealed a perforated tumor ulcer, and ENKL was diagnosed from the postoperative pathological findings. The patient’s postoperative course was uneventful. He was further treated with adjuvant chemotherapy by hematologist comprising six courses of dexamethasone, etoposide, ifosfamide, and carboplatin. The patient demonstrated long-term survival and was in remission at the time of writing, four years and five months after surgery. Conclusions We report a rare case of long-term survival of perforated ENKL of the small intestine achieved by surgery and adjuvant chemotherapy with dexamethasone, etoposide, ifosfamide, and carboplatin. It is essential to consult with a hematologist to determine the most appropriate chemotherapy such as DeVIC if one encounters rare postoperative pathological findings of ENKL. To elucidate the pathophysiology of this disease and to prolong survival of affected patients, accumulation of cases of long-term survival and examination of associated characteristics is necessary.

Purpose: To assess treatment outcomes in patients with stage I/II extranodal NK-/T-cell lymphoma, nasal type (ENKTCL-NT) and the feasibility of low-dose radiotherapy (RT) for achieving complete response (CR, defined as showing no residual hypermetabolic uptake on positron emission tomography [PET] or no residual lesions on computed tomography [CT]) after l-asparaginase-containing chemotherapy (l-ASP). Materials and methods: Between 1992 and 2018, 76 patients with early-stage ENKTCL-NT who achieved CR or partial response (PR) after induction chemotherapy received adjuvant RT. RT doses (using biologically equivalent doses in 2 Gy fractions [EQD2]) and rates of local recurrence-free survival (LRFS), locoregional recurrence-free survival (LRRFS), distant metastasis-free survival (DMFS), progression-free survival (PFS), and cancer-specific survival (CSS) were determined. Results: Median follow-up was 5.1 years (range, 0.5–20.8). The median RT dose was 45 Gy (range, 20–54). The 5-year LRFS, LRRFS, DMFS, PFS, and CSS rates were 82.7 %, 78.2 %, 81.1 %, 68.7 %, and 84.4 %, respectively. CR after induction chemotherapy was notably linked to better survival outcomes across each endpoint. Survival outcomes were not affected either by the administration of l-ASP or EQD2

Background: Extranodal natural killer/T-cell lymphoma, nasal type (ENKL) of the small intestine, is a disease with extremely poor prognosis. We describe treatment in a case which is novel in that it demonstrated long-term survival. Case presentation: A 68-year-old man was admitted to the emergency department of our hospital with the complaint of severe umbilical pain with tenderness and muscular defense. An abdominal computed tomography scan revealed a thick-wall mass on the small intestine and intra-abdominal free air. He was suspected of perforation of a small intestinal tumor and underwent emergency surgery. The surgery revealed a perforated tumor ulcer, and ENKL was diagnosed from the postoperative pathological findings. The patient's postoperative course was uneventful. He was further treated with adjuvant chemotherapy by hematologist comprising six courses of dexamethasone, etoposide, ifosfamide, and carboplatin. The patient demonstrated long-term survival and was in remission at the time of writing, four years and five months after surgery. Conclusions: We report a rare case of long-term survival of perforated ENKL of the small intestine achieved by surgery and adjuvant chemotherapy with dexamethasone, etoposide, ifosfamide, and carboplatin. It is essential to consult with a hematologist to determine the most appropriate chemotherapy such as DeVIC if one encounters rare postoperative pathological findings of ENKL. To elucidate the pathophysiology of this disease and to prolong survival of affected patients, accumulation of cases of long-term survival and examination of associated characteristics is necessary. [ABSTRACT FROM AUTHOR]

The following fictional case is intended as a learning tool within the Pathology Competencies for Medical Education (PCME), a set of national standards for teaching pathology. These are divided into three basic competencies: Disease Mechanisms and Processes, Organ System Pathology, and Diagnostic Medicine and Therapeutic Pathology. For additional information, and a full list of learning objectives for all three competencies, see http://journals.sagepub.com/doi/10.1177/2374289517715040.1.

Relapsing polychondritis (RP) is a rare autoimmune disease in which recurrent and progressive chondritis occurs throughout the body. We report a case of a 56-year-old female subject presented as intermittent fever and cough, who was found obvious luminal stenosis and intense 18F-fluorodeoxyglucose (FDG) uptake in her larynx and trachea via bronchoscopy and FDG positron emission tomography/computed tomography (PET/CT). The auricular cartilage biopsy demonstrated chondritis. At first she was diagnosed as RP and treated by glucocorticoid and methotrexate, leading to completely response. Fever and cough recurred after 18 months, and FDG PET/CT were performed again and targeted a newfound nasopharyngeal lesion, where the biopsy proved to be an extranodal natural killer (NK)/T-cell lymphoma, nasal type. [ABSTRACT FROM AUTHOR]

Extranodal natural-killer/T-cell lymphoma (ENKTL) is a rare type of non-Hodgkin lymphoma. However, it is common in Asia and South America. ENKTL, nasal type (ENKTL-NT), predominantly presents initial unspecific clinical manifestations involving the nasal cavity and its adjacent structures. We present two cases to increase the awareness of the ENKTL-NT cases masquerading inflammatory processes. Although the main clinical feature is a rapidly progressive facial destruction, none of these patients experienced the mentioned complaint. Its various manifestations frequently lead to misdiagnosis and delayed treatment, particularly in those with marked ocular, not nasal symptoms. Our patients were previously diagnosed with inflammatory conditions, namely sinusitis, idiopathic orbital inflammation, dacryocystitis, and orbital cellulitis. The combined approach of chemotherapy and radiotherapy has been proposed as the treatment of choice. Both cases showed young adults treated with combined therapy, yet showing poor outcomes. Clinicians should be aware of its existence and have to consider ENKTL-NT as one of the differential diagnoses in sinonasal or orbital inflammatory cases with unusually rapid progression or unresponsive to treatment.

The nasal type of extranodal natural killer (NK)/T-cell lymphoma (ENKL) is an aggressive form of non-Hodgkin's lymphoma. Although ENKL is most commonly seen in the midline of the nose, face and Waldeyer's ring, it can also occur in the skin, gastrointestinal tract, soft tissues and other parts of the body. Severe ENKL cases are accompanied by hemophagocytosis, with clinical manifestations such as high fever, hepatosplenomegaly, and decreased blood cell count. ENKL at different locations exhibits similar histological features and immunophenotypes, such as a strong affinity for T cell markers CD2 and CD56, cytotoxic molecules, as well as a strong positive for EBER after in situ hybridization. Although indolent ENKL is extremely rare, we hereby present a case study of primary NK/T cell lymphoma in the spinal canal with the initial manifestation of a diffuse growth of small cells, and the survival and recurrence details after 11 years, accompanied by CD30-positive large cell transformation. The patient's condition after treatment has improved and is currently in good health. [ABSTRACT FROM AUTHOR]

Background: The main etiology of acute pancreatitis includes biliary origin and alcohol, although various other causes include drugs (i.e., L-asparaginase) or malignant tumors. Since accurate identification of etiologies is crucial for determining therapeutic planning, the assessment of cause should be performed as early as possible. Case presentation: A 57-year-old Korean man was admitted for chemotherapy. The patient did not drink alcohol for religious reason. 26 days prior to admission, a 4 cm-sized testicular mass was observed in ultrasound and he received right radial orchiectomy. Extranodal natural killer/T-cell lymphoma, nasal type, was diagnosed. After confirming no additional abnormal findings, chemotherapy (using the regimens Dexamethasone, methotrexate, ifosfamide, L-asparaginase, and etoposide) was begun. On Day 8 of chemotherapy, L-asparaginase was started and he complained of sudden onset epigastric pain after 2 days. Acute pancreatitis was diagnosed and, in order to determine if the acute pancreatitis occurred due to L-asparaginase or pancreas involvement of extranodal natural killer/T-cell lymphoma, endoscopic ultrasonography guided fine needle biopsy was performed and observed diffusely infiltrated tumor cells. Therefore, he was given a final diagnosis of acute pancreatitis due to pancreas involvement of extranodal natural killer/T-cell lymphoma, nasal type. Discussion: Acute pancreatitis caused by pancreas involvement of extranodal natural killer/T-cell lymphoma, nasal type, is a very rare disease but can occur during chemotherapy. To identify the cause of acute pancreatitis, endoscopic ultrasonography guided fine needle biopsy can be considered. [ABSTRACT FROM AUTHOR]

Extranodal natural killer/T-cell lymphoma (ENKTL) is an aggressive disorder with heterogeneous clinical characteristics and poor prognosis. The combined value of baseline serum albumin level and absolute peripheral lymphocyte count showed prognostic information in a variety of malignancies, but its evidence is limited in ENKTL. The purpose of this study is to evaluate the impact of prognostic nutritional index (PNI) in ENKTL, and to provide some nutritionally and immunologically relevant information for better risk stratification. We conducted a retrospective study in 533 patients newly diagnosed with ENKTL. The PNI was calculated as albumin (g/L) + 5 × lymphocyte count (109/L). The optimal cutoff values for serum albumin and lymphocyte count were 40.6 g/L and 1.18 × 109/L, respectively, and 47.3 for PNI. After a median follow-up of 70 months, the 5-year overall survival (OS) and progression-free survival (PFS) were 56.2% and 49.5%, respectively. Patients in low PNI group had more unfavorable clinical features, and tended to have worse 5-year OS and PFS compared with those in high PNI group. According PNI-associated prognostic score, patients were classified into different risk groups. Significant difference has been found in 5-year OS and PFS in different risk groups. When PNI and PNI-associated prognostic score were superimposed on the International Prognostic Index (IPI), prognostic index of natural killer lymphoma (PINK), or nomogram-revised risk index (NRI) categories, the PNI and PNI-associated prognostic score provided additional prognostic information. Therefore, PNI and PNI-associated prognostic score could be independent prognostic factors for ENKTL and may be useful for risk stratification and clinical decision-making. [ABSTRACT FROM AUTHOR]

We report a rare case of metachronous lymphoma with two distinct cell lineages in a 75-year-old man. The patient complained about having nasal obstruction for 2 years and extranodal natural killer (NK)/T-cell lymphoma of the nasal type was diagnosed from a biopsy. The immunohistochemical staining for CD56 and in situ hybridization for Epstein-Barr virus (EBV)–encoded small RNA (EBER-ISH) were positive and the tumor cells were negative for CD20. After 13 months of concurrent chemoradiotherapy, the patient presented with swelling of the left testis. Positron emission tomography scan detected an abnormal uptake in the testis. A diffuse large B-cell lymphoma, not otherwise specified, was diagnosed from subsequent radical orchiectomy. The immunohistochemical staining revealed to be positive for CD20, BCL2, BCL6, and MYC and negative for CD10 and EBER-ISH.

Cardiac tumors range from benign to high grade malignancies. The incidence of cardiac involvement either by primary, or secondary tumors during autopsy is reported to be extremely low. Extranodal NK/T-cell lymphoma (ENKTL), nasal type is an unusual type of lymphoma. The skin is the second most common site of involvement after the respiratory tract. We present a case of a 63-year-old male, who was recently diagnosed with ENKTL, nasal type, who received chemotherapy, and died without any evident cause. The corpse was referred for routine medicolegal examination. Macroscopical determination of the cause of death was not feasible and subsequent histopathological examination revealed heart infiltration by ENKTL that was found in vivo in cutaneous lesions. Similar infiltrations existed in the pancreatic tissue. To the best of our knowledge, myocardial infiltration of ENKTL, inducing severe myocardial lesions that eventually caused death, is rare, with limited cases reported in the literature.

Extranodal natural-killer/T-cell lymphoma (ENKTL) is a rare type of non-Hodgkin lymphoma. However, it is common in Asia and South America. ENKTL, nasal type (ENKTL-NT), predominantly presents initial unspecific clinical manifestations involving the nasal cavity and its adjacent structures. We present two cases to increase the awareness of the ENKTL-NT cases masquerading inflammatory processes. Although the main clinical feature is a rapidly progressive facial destruction, none of these patients experienced the mentioned complaint. Its various manifestations frequently lead to misdiagnosis and delayed treatment, particularly in those with marked ocular, not nasal symptoms. Our patients were previously diagnosed with inflammatory conditions, namely sinusitis, idiopathic orbital inflammation, dacryocystitis, and orbital cellulitis. The combined approach of chemotherapy and radiotherapy has been proposed as the treatment of choice. Both cases showed young adults treated with combined therapy, yet showing poor outcomes. Clinicians should be aware of its existence and have to consider ENKTL-NT as one of the differential diagnoses in sinonasal or orbital inflammatory cases with unusually rapid progression or unresponsive to treatment. [ABSTRACT FROM AUTHOR]

Jin Li,1 Yajun Li,2 Ruolan Zeng,2 Jingguan Lin,1 Meizuo Zhong,3 Xianling Liu,4 Yizi He,2 Junqiao He,2 Zhou Ouyang,2 Lijun Huang,2 Ling Xiao,5 Hui Zhou2 1Department of Comprehensive Chemotherapy/Daytime Chemotherapy, Hunan Cancer Hospital, Changsha, Hunan, People’s Republic of China; 2Department of Lymphoma and Hematology, Hunan Cancer Hospital, Changsha, Hunan, People’s Republic of China; 3Department of Oncology, Xiangya Hospital, Central South University, Changsha, Hunan, People’s Republic of China; 4Department of Oncology, The Second Xiangya Hospital, Central South University, Changsha, Hunan, People’s Republic of China; 5Department of Histology and Embryology, School of Basic Medical Science, Central South University, Changsha, Hunan, People’s Republic of ChinaCorrespondence: Hui ZhouDepartment of Lymphoma and Hematology, Hunan Cancer Hospital, Changsha, Hunan, People’s Republic of ChinaTel +86 731 89762281Email zhouhui9403@126.comPurpose: This retrospective study compared effectiveness between ≤ 4 cycles and ≥ 5 cycles of L-asparaginase/pegaspargase-based chemoradiation in newly diagnosed low-risk extranodal natural killer/T-cell lymphoma (ENKTL), nasal type classified according to the Prognostic Index of Natural Killer (PINK) lymphoma model.Patients and Methods: Patients were categorized into ≤ 4-cycle (2– 4 chemotherapy cycles, n = 166) and ≥ 5-cycle groups (5– 6 cycles, n = 86). Propensity score matching analysis was used to reduce potential confounding bias between the two groups. Treatment responses, adverse events, and survival outcomes between the two groups were analyzed.Results: No matter before or after matching (65 in the ≤ 4-cycle group, 65 in the ≥ 5-cycle group), response rates and survival outcomes were similar between the ≤ 4-cycle and ≥ 5-cycle groups. Incidences of grade 1– 2 anemia and transaminase elevation were higher in the ≥ 5-cycle group. After matching, for stage IE disease, there were no differences in response rates and survival outcomes between the two groups. For stage IIE disease, the complete response rate was higher in the ≥ 5-cycle group (72.4% vs 92.6%, p = 0.049), and the 3-year overall survival (65.5% vs 85.2%, p = 0.024) and 3-year progression-free survival (58.6% vs 81.5%, p = 0.027) rates were significantly extended in the ≥ 5-cycle group.Conclusion: When chemoradiotherapy strategies with L-asparaginase/pegaspargase-based regimens are applied to modern low-risk ENKTL patients classified according to the PINK model, it may be better to moderately extend chemotherapy courses in patients with stage IIE disease.Keywords: extranodal natural killer/T-cell lymphoma, nasal type, chemotherapy courses, low-risk

Yuka Kawaji-Kanayama,1 Ayako Nishimura,2 Makoto Yasuda,3 Emiko Sakiyama,1,4 Yuji Shimura,1 Taku Tsukamoto,1 Tsutomu Kobayashi,1 Shinsuke Mizutani,1 Shota Okamoto,3 Gaku Ohmura,3 Shigeru Hirano,3 Eiichi Konishi,2 Kazutoshi Shibuya,5 Junya Kuroda1 1Division of Hematology and Oncology, Department of Medicine, Kyoto Prefectural University of Medicine, Kyoto, Japan; 2Division of Surgical Pathology, Department of Pathology, Kyoto Prefectural University of Medicine, Kyoto, Japan; 3Department of Otolaryngology-Head and Neck Surgery, Kyoto Prefectural University of Medicine, Kyoto, Japan; 4Department of Hematology, Matsushita Memorial Hospital, Moriguchi, Japan; 5Department of Surgical Pathology, Toho University Graduate School of Medicine, Tokyo, JapanCorrespondence: Junya KurodaDivision of Hematology and Oncology, Department of Medicine, Kyoto Prefectural University of Medicine, 465 Kajii-Cho, Kamigyo-Ku, Kyoto 602-8566, JapanTel +81 75 251 5740Fax +81 75 251 5743Email junkuro@koto.kpu-m.ac.jpAbstract: Invasive fungal rhinosinusitis (FRS) is a rare but intractable infectious disease of the sinonasal region with destructive direct infiltration into surrounding tissues, such as the bone, orbit and brain, and potential dissemination to systemic organs. Symptomatic assessments and imaging are frequently not sufficiently diagnostic, and histopathological examination is essential for definite diagnosis of FRS. We herein report a case of chronic invasive FRS (CIFRS) in a 58-year-old Japanese male with end-stage diabetic nephropathy that required maintenance dialysis after graft rejection of living kidney transplantation. His initial main clinical presentation was sinus gangrene, which gradually progressed from the paranasal sinus to the nasal septum and oral palate, but not towards the intracranial or orbital region, for two months. The patient was first strongly suspected to have extranodal natural killer/T cell lymphoma (ENKTL), nasal type, a subtype of malignant lymphoma, based on the macroscopic appearance of the gangrene, expansion pattern and high serum soluble interleukin-2 level; however, repeated biopsies and eventual resection led to diagnosis of CIFRS due to Aspergillus niger and Mucor. The disease was improved by surgical resection in combination with antifungal pharmacologic treatment with liposomal amphotericin B and voriconazole. CIFRS typically occurs in immunocompetent patients and shows intracranial progression, but this case shows that atypical CIFRS with an uncommon expansion pattern can occur in an immunodeficient patient.Keywords: chronic invasive fungal rhinosinusitis, diabetes mellitus, maintenance dialysis, extranodal natural killer/T cell lymphoma, nasal type

Objective: To compare the efficacy and safety of the novel SVILE regimen with the P-GemOx regimen in patients with newly diagnosed extranodal natural killer/T-cell lymphoma, nasal type (ND-ENKTL). Methods: From April 2015 to July 2018, 103 patients with ND-ENKTL were randomly assigned to SVILE (experimental group) or P-GemOx (control group) chemotherapy followed by radiotherapy and consolidation chemotherapy. The primary endpoint was the overall response rate after 3 cycles of chemotherapy, and secondary study endpoints were complete response (CR), progression-free survival (PFS), and overall survival (OS). Safety was also evaluated. Results: There were no significant differences in baseline characteristics in the experimental vs. control groups. In experimental and control groups, respectively, the overall response rates were 91.7% vs. 97.0% for stage I/II and 75.0% vs. 72.2% for stage III/IV. The CR rates were 83.4% vs. 97.0% for stage I/II and 68.8% vs. 61.1% for stage III/IV. None of those differences were significant. There was no significant difference in PFS and OS between groups and between patients in stage I/II and stage III/IV. The 3-year PFS and OS in stage I/II were 88.3% vs. 93.3% and 88.8% vs. 97.0%, respectively. The 3-year PFS and OS in stage III/IV were 46.2% vs. 65.7% and 68.8% vs. 72.2%, respectively. The common adverse events were hematological toxicity, hepatotoxicity, and coagulation abnormalities, which were found to be reversible with supportive therapy. Conclusions: The novel SVILE regimen has comparable effects to those of P-GemOx in patients with ND-ENKTL and is well tolerated. SVILE is a therapeutic option for ND-ENKTL.

Introduction: Extranodal natural killer/T-cell lymphoma-nasal type (ENKTL-NT) is an aggressive rare non-Hodgkin's lymphoma (NHL) subtype. It presents with involvement of the nasal and upper aerodigestive region causing extensive destruction of these midline structures. It has a predilection for the Asian population. Most cases present in the early-stage disease. Treatment outcomes are usually poor, and no consensus for optimal treatment strategy exists. Patients and Methods: This is a retrospective analysis of ENKTL-NT, diagnosed in a regional cancer center in India during the period of 2013–2018. The demographic and clinical features, laboratory parameters, radiological, histopathological features, and treatment outcomes were studied. Patients were treated with SMILE or AspaMetDex regimes sandwiched with radiotherapy. Statistical analysis was performed using software “Epi Info” Version 7.2, (CDC, Atlanta, Georgia, USA). Results: Fourteen patients were diagnosed with ENKTL-NT during this period. Eight patients received SMILE, and six patients have received AspaMetDex as induction chemotherapy. Ten (71.42%) of 14 patients have achieved CR. At the median follow-up of 30 months (4–47 months), nine patients relapsed with median progression-free survival of 22 months. The median overall survival was not reached. There were two induction deaths, one in AspaMetDex and one in the SMILE group. All patients receiving SMILE experienced at least one episode of Grade 3/4 hematological toxicity. Patients receiving AspaMetDex did not have any Grade 3/4 hematological toxicity. Discussion: ENKTL-NT in India is not as common as reported in other Asian countries. Patients usually present at an earlier stage because of the peculiar site of affection. It has high response rates, but relapses are common. Most of the relapses occur within the first 2 years of follow-up. Conclusion: ENKTL-NT is rare aggressive NHL subtype with good response to L-asparaginase-based chemotherapy sandwiched with radiation therapy. SMILE is a more toxic regime than AspaMetDex but can be managed with proper supportive care.

Extranodal Natural Killer/T Cell Lymphoma Nasal Type (EN-NK/T-CL-NT) is a rare type of non-Hodgkin lymphoma in which the lesion is usually located in the upper respiratory tract, such as nasal cavity, palate, and nasopharynx. In addition, the primary lesion of EN-NK/T-CL-NT can rarely originate in extranasal sites such as the skin, gastrointestinal tract, testicles, central nervous system, and lungs. We describe an 82-year-old male smoker was brought to the hospital with 8 months of fever, cough, sputum production, chest pain, and chest tightness. Computed tomography (CT) of the chest showed subpleural high-density shadow in the lower lobe of the right lung with unclear borders and surrounding patchy ground-glass shadow. Initially, the patient's right lower lobe lesion progressed after receiving anti-inflammatory treatment. He subsequently underwent two computerized tomography (CT)-guided percutaneous transthoracic needle aspiration biopsies and a bronchoscopy, but no tumor cells were found. Through multidisciplinary team discussions, the patient was then transferred to the department of cardiothoracic surgery for right lower lobectomy. Finally, extranodal natural killer/T-cell lymphoma (ENKTCL), nasal type, was confirmed by pathology of the surgical specimen. The diagnosis of primary pulmonary ENKTCL was made because no evidence other than extrapulmonary site was found at the time of diagnosis and treatment. Here we report a case of primary pulmonary extranodal natural killer/T-cell lymphoma of nasal type presenting as pneumonia in the right lower lobe and enhance the understanding of the disease., Competing Interests: No one in the manuscript has a conflict of interest., (© 2024 The Authors. Published by Elsevier Ltd.)

Primary cutaneous T-cell lymphomas (CTCLs) other than mycosis fungoides (MF) and Sézary syndrome (SS) encompass a heterogenous group of non-Hodgkin lymphomas with variable clinical courses, prognoses, and management approaches. Given the morphologic and histologic overlap among the CTCL subtypes and other T-cell lymphomas with cutaneous manifestations, thorough evaluation with clinicopathologic correlation and exclusion of systemic involvement are essential prior to initiating therapy. Staging and treatment recommendations vary, depending on the subtype, clinical behavior, and treatment response. Generally, for subtypes in which staging is recommended, Ann Arbor or tumor, node, metastasis staging specific to CTCL other than MF or SS are used. For many subtypes, there is no standard treatment to date. Available recommended treatments range widely, from no active or minimal intervention with skin-directed therapy to aggressive systemic therapies that include multi-agent chemotherapy with consideration for hematopoietic stem cell transplant. Emerging targeted therapies, such as brentuximab, a chimeric antibody targeting CD30, show promise in altering the disease course of non-MF/SS CTCLs. [ABSTRACT FROM AUTHOR]

Primary cutaneous T-cell lymphomas (CTCLs) are defined as lymphomas with a T-cell phenotype that present in the skin without evidence of systemic or extracutaneous disease at initial presentation. CTCLs other than mycosis fungoides and Sézary syndrome (SS) account for approximately one third of CTCLs and encompass a heterogenous group of non-Hodgkin lymphomas, ranging from indolent lymphoproliferative disorders to aggressive malignancies with a poor prognosis. The spectrum of CTCLs continues to broaden as new provisional entities are classified. Given the morphologic and histologic overlap among CTCLs and other diagnoses, a thorough clinical history, physical evaluation, and clinicopathologic correlation are essential in the work up and diagnosis of these rare entities. This article will summarize the epidemiologic, clinical, pathologic, and diagnostic features of CTCLs other than mycosis fungoides and SS. [ABSTRACT FROM AUTHOR]

Nasal-type, extranodal nature killer (NK)/T-cell lymphoma-associated hemophagocytic syndrome (NK/T-LAHS) is a rare and life-threatening disease, requiring investigation of risk stratification. We conducted a retrospective study and proposed nomograms to predict NK/T-LAHS. The discriminative ability and calibration of the nomograms for prediction were tested using C statistics and calibration plots. We analyzed 533 patients with extranodal NK/T-cell lymphoma (ENKTL), out of which 71 were diagnosed with hemophagocytic syndrome (HPS), with a cumulative incidence of 13.3%. Significant difference for 2-year survival was found between patients with and without HPS (14.7% vs. 77.5%). Analyses showed that Eastern Cooperative Oncology Group (ECOG) performance status (PS) ≥2, B symptoms, and bone marrow (BM) invasion were significantly associated with NK/T-LAHS. We used these data as the basis to establish a nomogram of risk index for ENKTL (RINK). In 335 patients with available data for Epstein-Barr virus DNA (EBV-DNA), we found high viral copies (≥4,450 copies/ml) were correlated with NK/T-LAHS. When these data were added to RINK, we developed another nomogram that included EBV-DNA data (RINK-E). The nomograms displayed good accuracy in predicting NK/T-LAHS with a C-statistics of 0.919 for RINK and a C-statistics of 0.946 for RINK-E, respectively. The calibration chart also showed an excellent consistency between the predicted and observed probabilities. The proposed nomograms provided individualized risk estimate of HPS in patients with ENKTL.

Nasal-type, extranodal nature killer (NK)/T-cell lymphoma-associated hemophagocytic syndrome (NK/T-LAHS) is a rare and life-threatening disease, requiring investigation of risk stratification. We conducted a retrospective study and proposed nomograms to predict NK/T-LAHS. The discriminative ability and calibration of the nomograms for prediction were tested using C statistics and calibration plots. We analyzed 533 patients with extranodal NK/T-cell lymphoma (ENKTL), out of which 71 were diagnosed with hemophagocytic syndrome (HPS), with a cumulative incidence of 13.3%. Significant difference for 2-year survival was found between patients with and without HPS (14.7% vs. 77.5%). Analyses showed that Eastern Cooperative Oncology Group (ECOG) performance status (PS) ≥2, B symptoms, and bone marrow (BM) invasion were significantly associated with NK/T-LAHS. We used these data as the basis to establish a nomogram of risk index for ENKTL (RINK). In 335 patients with available data for Epstein-Barr virus DNA (EBV-DNA), we found high viral copies (≥4,450 copies/ml) were correlated with NK/T-LAHS. When these data were added to RINK, we developed another nomogram that included EBV-DNA data (RINK-E). The nomograms displayed good accuracy in predicting NK/T-LAHS with a C-statistics of 0.919 for RINK and a C-statistics of 0.946 for RINK-E, respectively. The calibration chart also showed an excellent consistency between the predicted and observed probabilities. The proposed nomograms provided individualized risk estimate of HPS in patients with ENKTL. [ABSTRACT FROM AUTHOR]

Cardiac tumors range from benign to high grade malignancies. The incidence of cardiac involvement either by primary, or secondary tumors during autopsy is reported to be extremely low. Extranodal NK/T-cell lymphoma (ENKTL), nasal type is an unusual type of lymphoma. The skin is the second most common site of involvement after the respiratory tract. We present a case of a 63-year-old male, who was recently diagnosed with ENKTL, nasal type, who received chemotherapy, and died without any evident cause. The corpse was referred for routine medicolegal examination. Macroscopical determination of the cause of death was not feasible and subsequent histopathological examination revealed heart infiltration by ENKTL that was found in vivo in cutaneous lesions. Similar infiltrations existed in the pancreatic tissue. To the best of our knowledge, myocardial infiltration of ENKTL, inducing severe myocardial lesions that eventually caused death, is rare, with limited cases reported in the literature. [ABSTRACT FROM AUTHOR]

Background: The main etiology of acute pancreatitis includes biliary origin and alcohol, although various other causes include drugs (i.e., L-asparaginase) or malignant tumors. Since accurate identification of etiologies is crucial for determining therapeutic planning, the assessment of cause should be performed as early as possible. Case presentation: A 57-year-old Korean man was admitted for chemotherapy. The patient did not drink alcohol for religious reason. 26 days prior to admission, a 4 cm-sized testicular mass was observed in ultrasound and he received right radial orchiectomy. Extranodal natural killer/T-cell lymphoma, nasal type, was diagnosed. After confirming no additional abnormal findings, chemotherapy (using the regimens Dexamethasone, methotrexate, ifosfamide, L-asparaginase, and etoposide) was begun. On Day 8 of chemotherapy, L-asparaginase was started and he complained of sudden onset epigastric pain after 2 days. Acute pancreatitis was diagnosed and, in order to determine if the acute pancreatitis occurred due to L-asparaginase or pancreas involvement of extranodal natural killer/T-cell lymphoma, endoscopic ultrasonography guided fine needle biopsy was performed and observed diffusely infiltrated tumor cells. Therefore, he was given a final diagnosis of acute pancreatitis due to pancreas involvement of extranodal natural killer/T-cell lymphoma, nasal type. Discussion: Acute pancreatitis caused by pancreas involvement of extranodal natural killer/T-cell lymphoma, nasal type, is a very rare disease but can occur during chemotherapy. To identify the cause of acute pancreatitis, endoscopic ultrasonography guided fine needle biopsy can be considered.

Objective: The prognostic nutritional index (PNI) is a significant prognostic factor in diffuse large B cell lymphoma, follicular lymphoma, and other malignancies. The current study aimed to explore its prognostic role in extranodal natural killer/T cell lymphoma (ENKTL).Methods: Patients diagnosed with ENKTL and treated during 2002 and 2018 (n = 184) were retrospectively recruited. PNI was calculated from albumin concentration (g/L) and total lymphocyte count (*109/L). The association of PNI and overall survival (OS) or progression-free survival (PFS) was assessed in univariate analysis and multivariate Cox regression validated by the 10-fold cross-validation method.Results: Survival analyses showed that both OS and PFS differed significantly between PNI groups stratified by a cutoff value of 49.0. The 3- and 5-year OS were 42.5 and 36.3% in the low-PNI (PNI < 49) subgroup and 70.6% and 63.9% (P < 0.001) in the high-PNI (PNI ≥ 49) subgroup, respectively. The corresponding PFS showed a similar pattern (38.4, 32.4 vs. 64.8, 54.0%, P < 0.001). Multivariate analysis indicated that PNI was significantly independent for both OS (HR = 0.517, 95% CI = 0.322–0.831, P = 0.006) and PFS (HR = 0.579, 95% CI = 0.373–0.899, P = 0.015). Furthermore, integrating PNI into the models of IPI (International Prognostic Index), KPI (Korean Prognostic Index), and PINK (prognostic index of natural killer lymphoma) could improve the area under the curve (AUC) and reduce the integrated Brier score (IBS) and Akaike Information Criterion (AIC) value of each model.Conclusion: PNI was a significant prognostic indicator for ENKTL.

Qin Tong,1,* Shuming Ouyang,2,* Lingling Feng,3,* Hanyu Wang,3 Yunfei Xia,3 Yujing Zhang3 1Department of Radiation Oncology, The First Affiliated Hospital of University of South China, Hengyang 421001, People’s Republic of China; 2Department of Obstetrics and Gynecology, The First Affiliated Hospital of University of South China, Hengyang 421001, People’s Republic of China; 3Department of Radiation Oncology, Sun Yat-sen University Cancer Center, State Key Laboratory of Oncology in South China, Guangzhou 510060, People’s Republic of China *These authors contributed equally to this work Background: This study aims to retrospectively analyze the salvage treatment outcomes and prognostic factors of patients with early stage locoregionally recurrent (LRR) extranodal natural killer (NK)/T-cell lymphoma, nasal type (ENKTCL).Methods: Between January 1995 and December 2014, 540 patients with stage IE–IIE ENKTCL received chemotherapy (ChT) and/or radiotherapy (RT) in our hospital, and among these, 56 patients who experienced LRR were included in this study. Salvage treatments included RT alone in 4 patients (7.1%), ChT alone in 30 patients (53.6%), and ChT combined with RT in 22 patients (39.3%). Median RT dose was 50 Gy (range 36–60 Gy). The effect of salvage treatment on overall survival (OS) rate from start of initial treatment (IT) as well as that after recurrence was analyzed.Results: The overall median follow-up time from IT was 35.9 months, with a 3-year OS of 72.7%. The median follow-up time after relapse was 14.8 months, and the 3-year OS after relapse was 57.8%. Compared to ChT alone (n=30), treatment with salvage RT (n=26) improved the OS from IT (p=0.040) and after relapse (p=0.009); further, re-irradiation improved the OS from IT (p=0.018) and after relapse (p=0.019). Acute and late toxicities after re-irradiation were mostly grades 1–2 (84.3%). At both univariate and multivariate analyses, better Karnofsky Performance Score (KPS), RT in IT, and RT in salvage treatment were found to be significant factors influencing OS after recurrence.Conclusion: Salvage RT improved survival in patients with LRR stage IE–IIE ENKTCL, and the treatment toxicity was acceptable. Keywords: extranodal NK/T-cell lymphoma, nasal type, radiotherapy, recurrence, prognostic analysis

We retrospectively analyzed the treatment outcomes of elderly patients (aged ≥ 60 years) with extranodal natural killer/T-cell lymphoma, nasal type (ENKTL) and investigated the prognostic factors. Fifty-two elderly patients received chemotherapy alone, radiotherapy alone, or chemotherapy followed by radiotherapy ± consolidation chemotherapy as induction therapy. Overall, 97.26% patients in stage I/II had overall response (OR) and 86.1% had complete response (CR), whereas 71.4% of patients in stage III/IV had OR and 35.7% had CR. The 3-year freedom from progression (FFP) rate and overall survival (OS) rate of patients with stage I/II were 78.2% and 85.0%, respectively, and those with stage III/IV were 23.3% and 33.3%. Following multivariate analysis of Cox regression, ECOG performance status scores of 3–4 and stage III/IV were independent prognostic factors for elderly ENKTL patients. Elderly patients with stage I/II or stage III/IV and good or poor performance status can benefit from the commonly used or personalized treatment. [ABSTRACT FROM AUTHOR]

Extranodal NK/T-cell lymphoma (ENKTCL), nasal type is a very rare and aggressive non-Hodgkin lymphoma. Most commonly it occurs in the upper aerodigestive tract. But, it can also manifest at locations such as the skin, soft tissue, gastrointestinal tract (GI), lungs, testis, etc. These locations are designated as extranasal ENKTCL. The patients with the latter have often more adverse clinical features and poorer survival rate compared with nasal sites. We present a case of an 83-year-old patient with a primary ENKTCL, nasal type, with extranasal presentation in the right upper eyelid. Materials for the literature review was obtained by a comprehensive search on PubMed, which yielded 82 eligible cases with extranasal ENKTCL. Sixty-eight cases (83 %) were localized as primary ENKTCL in the lungs (17), central nervous system (CNS) (14), testis (11), GI-tract (7), skin (6), orbit and intraocular tissue (4), pancreas (2), adrenal gland (2), breast (1), etc. 14 cases (17 %) presented as extended or disseminated diseases involving exclusively organs outside the upper aerodigestive tract. There was no systematic pattern of organ involvement in the extended/disseminated ENKTCL. 63 % of the patient with localized extranasal ENKTCL and about 50% of patients with extended/disseminated disease were reported to have died of the disease. Treatment strategies varied with no preferred option. Among the used treatment options were chemotherapy, radiotherapy, surgery, stem cell transplantation alone or in different combinations. ENKTCL is a highly aggressive disease which may present in extranasal areas. Although the tumors respond to both chemotherapy and radiotherapy, durable complete remissions are very rare. [ABSTRACT FROM AUTHOR]

Objective: The prognostic nutritional index (PNI) is a significant prognostic factor in diffuse large B cell lymphoma, follicular lymphoma, and other malignancies. The current study aimed to explore its prognostic role in extranodal natural killer/T cell lymphoma (ENKTL). Methods: Patients diagnosed with ENKTL and treated during 2002 and 2018 (n = 184) were retrospectively recruited. PNI was calculated from albumin concentration (g/L) and total lymphocyte count (*109/L). The association of PNI and overall survival (OS) or progression-free survival (PFS) was assessed in univariate analysis and multivariate Cox regression validated by the 10-fold cross-validation method. Results: Survival analyses showed that both OS and PFS differed significantly between PNI groups stratified by a cutoff value of 49.0. The 3- and 5-year OS were 42.5 and 36.3% in the low-PNI (PNI < 49) subgroup and 70.6% and 63.9% (P < 0.001) in the high-PNI (PNI ≥ 49) subgroup, respectively. The corresponding PFS showed a similar pattern (38.4, 32.4 vs. 64.8, 54.0%, P < 0.001). Multivariate analysis indicated that PNI was significantly independent for both OS (HR = 0.517, 95% CI = 0.322–0.831, P = 0.006) and PFS (HR = 0.579, 95% CI = 0.373–0.899, P = 0.015). Furthermore, integrating PNI into the models of IPI (International Prognostic Index), KPI (Korean Prognostic Index), and PINK (prognostic index of natural killer lymphoma) could improve the area under the curve (AUC) and reduce the integrated Brier score (IBS) and Akaike Information Criterion (AIC) value of each model. Conclusion: PNI was a significant prognostic indicator for ENKTL. [ABSTRACT FROM AUTHOR]

The quantification of circulating Epstein Barr virus (EBV) DNA loads has played an important role in the diagnosis and management of EBV-associated lymphoid malignancies. Viral load measurement is particularly useful for monitoring EBV-DNA in hematopoietic stem cell transplant patients, and for assessing the prognosis or response to therapy of EBV-associated intractable lymphomas like extranodal NK/T-cell lymphoma, nasal type. Cell-free EBV-DNA in plasma can be used as a biomarker for estimating the severity or prognosis of these lymphomas. In addition to plasma, whole blood has been used for the management of transplant patients. Although measuring EBV-DNA has been useful, there is a lack of standardization and the optimal specimens for measuring viral loads are unknown. This can be attributed to the different forms of EBV-DNA that exist in peripheral blood and the different pathologies that result from diverse EBV disease states. As a result, guidelines for EBV diagnosis or the initiation of treatment are unclear. However, the newly established World Health Organization standard for EBV quantification will encourage collaborative studies across institutions and countries to establish proper guidelines for EBV diagnosis and the initiation of treatment.

Extranodal NK/T-Cell Lymphoma, nasal type (ENKTL-NT) has some salient aspects. The lymphoma is commonly seen in Eastern Asia, has progressive necrotic lesions in the nasal cavity, makes midfacial destructive lesions, and shows poor prognosis. The lymphoma cell is originated from either NK- or γδ T-cells, which express CD56. Since the authors first demonstrated the existence of Epstein–Barr virus (EBV) DNA and EBV oncogenic proteins in lymphoma cells, ENKTL-NT has been recognized as an EBV-associated malignancy. Because the angiocentric and polymorphous lymphoma cells are mixed with inflammatory cells on a necrotic background, the diagnosis of ENKTL-NT requires CD56 immunostaining and EBER in situ hybridization. In addition, serum the EBV DNA level is useful for the diagnosis and monitoring of ENKTL-NT. Although ENKTL-NT is refractory lymphoma, the prognosis is improved by the development of therapies such as concomitant chemoradiotherapy. The basic research reveals that a wide variety of intracellular/cell surface molecules, cytokines, chemokines, and micro RNAs are involved in lymphomagenesis, and some of them are related to EBV. Understanding lymphoma behavior introduces new therapeutic strategies, such as the usage of immune checkpoint inhibitors, peptide vaccines, and molecular targeting therapy. This review addresses recent advances in basic and clinical aspects of ENKTL-NT, especially its relation to EBV features.

The objective of the present study was to describe the clinicopathological features of a patient with nasal NK/T cell lymphoma that was similar in morphology to mucosa-associated lymphoid tissue lymphoma (MALToma). The clinicopathological data of a patient diagnosed with nasal NK/T cell lymphoma mimicking MALToma was collected, and the clinicopathological characteristics were discussed. The female patient was 43 years old and had suffered from persistent congestion for ten days. The mucosa in the left nasal cavity was inflamed, resulting in congestion and it was also purulent on the surface, as observed by nasal endoscopy. The disease was considered to be inflammatory based on CT scan. A biopsy after operation showed that the tumor consisted of small lymphoid cells that resembled MALToma in morphology. On the basis of the immunohistochemistry and in situ hybridization laboratory tests, a diagnosis of left nasal NK/T cell lymphoma was made. The patient received chemotherapy and radiotherapy, and remission was achieved six months after diagnosis. The patient was in a good condition at 16 months follow-up. In conclusion, NK/T cell lymphoma composed of small cells may be a type of indolent lymphoma with special characteristics of clinical presentation, image, pathology and prognosis. This case highlights that more attention is required by radiologists, pathologists and hematologists to diagnose this type of lymphoma. [ABSTRACT FROM AUTHOR]

The quantification of circulating Epstein Barr virus (EBV) DNA loads has played an important role in the diagnosis and management of EBV-associated lymphoid malignancies. Viral load measurement is particularly useful for monitoring EBV-DNA in hematopoietic stem cell transplant patients, and for assessing the prognosis or response to therapy of EBV-associated intractable lymphomas like extranodal NK/T-cell lymphoma, nasal type. Cell-free EBV-DNA in plasma can be used as a biomarker for estimating the severity or prognosis of these lymphomas. In addition to plasma, whole blood has been used for the management of transplant patients. Although measuring EBV-DNA has been useful, there is a lack of standardization and the optimal specimens for measuring viral loads are unknown. This can be attributed to the different forms of EBV-DNA that exist in peripheral blood and the different pathologies that result from diverse EBV disease states. As a result, guidelines for EBV diagnosis or the initiation of treatment are unclear. However, the newly established World Health Organization standard for EBV quantification will encourage collaborative studies across institutions and countries to establish proper guidelines for EBV diagnosis and the initiation of treatment. [ABSTRACT FROM AUTHOR]

Extranodal natural killer/T-cell lymphoma (ENKL) of the nasal type is a rare, clinically aggressive disease. ENKL of the nasal type is often localized in the upper aerodigestive tract, including the nasal cavity, nasopharynx, paranasal sinuses, tonsils, hypopharynx and larynx, and usually presents as stage I/II. Extranasal involvement can occur, and a common site of extranasal involvement or metastatic disease includes the skin. Identifying skin metastases is important for the appropriate staging and treatment. We report a case of ENKL of the nasal type that presented with localized disease and subsequent skin lesions that were consistent with skin metastases.

Objective: To present pathological and molecular characterizations of a rare case that was diagnosed as nasal-type natural killer (NK)/T cell lymphoma primarily arising in the cervix. Case report: An Asian woman was admitted to hospital with a hysteromyoma, and laparotomy was performed. A large tumor of the uterus was found, which was limited to the cervix. Pathological examination showed NK/T cell lymphoma, which was supported by histological and immunohistochemical studies and was confirmed by evidence of Epstein–Barr virus infection. Less commonly, this case concerned a cytotoxic T cell phenotype, as molecular studies showed evidence of a clonal T cell receptor γ chain gene rearrangement. Microscopically, prominent and extensive necrosis was the distinctive feature of this case, which reminded us of considering it as a tumor. Conclusion: Primary NK/T lymphoma of the cervix is rare. Our experience in this case provided variable information on both pathological and molecular studies. This case may be of value in the differential diagnosis of lymphoid lesions and other small cell tumors of the cervix.

Extranodal NK/T cell lymphoma, nasal type（ENKTL） is a highly aggressive malignant tumor derived from NK cells. This article reports a case of ENKTL invading the larynx and digestive tract. The clinical clinical manifestations include hoarseness and intranasal masses., Competing Interests: The authors of this article and the planning committee members and staff have no relevant financial relationships with commercial interests to disclose., (Copyright© by the Editorial Department of Journal of Clinical Otorhinolaryngology Head and Neck Surgery.)

Background Collision tumors are defined as coexistence of 2 histologically different neoplasms occurring in the same anatomic location. Such co-occurrence of tumors in the brain is uncommon. To our knowledge, meningioma colliding with extranodal natural killer/T-cell lymphoma has not been described previously. Case Description A 50-year-old man presented with a 1-year history of progressive memory decline and 2 weeks of drowsiness, bradykinesia, and aphasia. Magnetic resonance imaging revealed a heterogeneously enhanced mass beside the left frontal cerebral falx resulting in midline shift. The left frontal lobe mass was resected. Pathologic examination showed the tumor consisted of whorled spindle cells and diffuse medium-sized lymphoid cells. The spindle cells were positive for epithelial membrane antigen and negative for S-100. The lymphoid cells expressed CD3ε, CD56, TIA-1, and granzyme B. Epstein-Barr virus encoded small RNAs were detected by in situ hybridization. No monoclonal T-cell receptor gamma gene rearrangement was detected. Four weeks after surgery, the patient was treated with polychemotherapy and intrathecal methotrexate, but he died 2 months later. Conclusions This is the first report of a unique brain collision tumor consisting of a meningioma and an extranodal natural killer/T-cell lymphoma. Diagnosis depends on histopathology. Awareness of this entity is important to distinguish it from other intracranial tumors. Highlights • Collision intracranial lymphomas are extremely rare. • This is the first report of a collision tumor consisting of ENKTCL, nasal type and meningioma. • Collision tumors are difficult to diagnose preoperatively owing to lack of special or unique clinical features. • Pathologic identification is often the best way to ensure a correct diagnosis. [ABSTRACT FROM AUTHOR]