Primary Gastrointestinal T/NK Cell Lymphoma

Kim, Eun Kyung; Jang, Mi; Yang, Woo Ick; Yoon, Sun Och

doi:10.3390/cancers13112679

Cited by 10 publications

(10 citation statements)

References 35 publications

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“…The most common site of involvement in MEITL is the small intestine, especially the jejunum [ 4 , 58 , 67 ]. The stomach, duodenum, and large intestine may also be affected, with reported rates of 2.4–12.0%, 31%, and 8.3–23%, respectively [ 57 , 67 , 68 ]. Symptoms include abdominal pain, diarrhea, weight loss, and GI hemorrhage [ 57 , 59 ].…”

Section: Monomorphic Epitheliotropic Intestinal T-cell Lymphoma (Meitl)mentioning

confidence: 99%

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Primary Gastrointestinal T-Cell Lymphoma and Indolent Lymphoproliferative Disorders: Practical Diagnostic and Treatment Approaches

Nishimura

Nishikori

et al. 2021

Cancers

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Primary gastrointestinal (GI) T-cell neoplasms are extremely rare heterogeneous disease entities with distinct clinicopathologic features. Given the different prognoses of various disease subtypes, clinicians and pathologists must be aware of the key characteristics of these neoplasms, despite their rarity. The two most common aggressive primary GI T-cell lymphomas are enteropathy-associated T-cell lymphoma and monomorphic epitheliotropic intestinal T-cell lymphoma. In addition, extranodal natural killer (NK)/T-cell lymphoma of the nasal type and anaplastic large cell lymphoma may also occur in the GI tract or involve it secondarily. In the revised 4th World Health Organization classification, indolent T-cell lymphoproliferative disorder of the GI tract has been incorporated as a provisional entity. In this review, we summarize up-to-date clinicopathological features of these disease entities, including the molecular characteristics of primary GI T-cell lymphomas and indolent lymphoproliferative disorders. We focus on the latest treatment approaches, which have not been summarized in existing reviews. Further, we provide a comprehensive review of available literature to address the following questions: How can pathologists discriminate subtypes with different clinical prognoses? How can primary GI neoplasms be distinguished from secondary involvement? How can these neoplasms be distinguished from non-specific inflammatory changes at an early stage?

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Section: Monomorphic Epitheliotropic Intestinal T-cell Lymphoma (Meitl)mentioning

confidence: 99%

“…Endoscopically, MEITL appears as a single or multiple masses, deep ulcers, superficial ulcers, or relatively normal findings. Mass formation and superficial ulceration are most frequently seen, in approximately 40% of cases [ 67 ].…”

Section: Monomorphic Epitheliotropic Intestinal T-cell Lymphoma (Meitl)mentioning

confidence: 99%

Primary Gastrointestinal T-Cell Lymphoma and Indolent Lymphoproliferative Disorders: Practical Diagnostic and Treatment Approaches

Nishimura

Nishikori

et al. 2021

Cancers

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“… 11 In 2021, a Korean group reported a median OS of 5.1 months for 13 patients with PGINKTL. 5 Furthermore, an OS of 6 months was observed in a pooled database of 126 cases of PGINKTL. 12 However, there are limited data available to summarize the clinical features, survival outcomes, and prognostic factors of this rare lymphoma.…”

Section: Introductionmentioning

confidence: 99%

“… 1 , 2 , 3 In contrast, primary gastrointestinal NK/T‐cell lymphoma (PGINKTL) has been insufficiently studied because of its low incidence and diagnostic difficulty. The incidence of PGINKTL was observed in 8% (84/1010) of patients with primary gastrointestinal (GI) tract lymphoma, 4 34.2% (13/38) of patients with primary gastrointestinal T/NK‐cell lymphoma (GI‐TNKL), 5 and 19.6% (18/92) of patients with nonupper aerodigestive tract ENKTL. 6 …”

Section: Introductionmentioning

confidence: 99%

Real‐World clinical features and survival outcomes associated with primary gastrointestinal natural killer/T‐cell lymphoma from 1999 to 2020

Chunli

Jiang

et al. 2022

Cancer Medicine

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Background Primary gastrointestinal natural killer (NK)/T‐cell lymphoma (PGINKTL) is a rare T‐/NK‐cell lymphoma subtype, and the clinical features and survival outcomes remain largely unknown. Methods To summarize the clinical features and survival outcomes of PGINKTL, PGINKTL cases diagnosed at our hospital from May 1999 to December 2020 were reviewed; and the clinical data, information on treatment strategies, and survival were collected. Survival analysis was performed using the Kaplan–Meier method and multivariable Cox proportional hazards regression. We constructed a nomogram to visualize the survival prediction of PGINKTL. The discriminative ability and calibration of the nomogram for prediction were tested using the concordance index (C‐index) and calibration plots. Results The cohort included 81 cases, the median age was 36 years (range, 7–80 years), and the male‐to‐female ratio was 1.7:1. The most common clinical symptom at the time of diagnosis was abdominal pain (71.6%). The most common lesion site was the colon (59.3%). During a median follow‐up period of 37.7 months, the median overall survival (OS) time of 81 patients was 4.0 months (95% confidence interval [CI], 3.1–4.9 months), and the 2‐year OS rate was 30.7% (95% CI, 20.3%–40.1%). The multivariate analyses indicated that patients with an Eastern Cooperative Oncology Group (ECOG) performance status (PS) score ≥2, serum lactic dehydrogenase (LDH) level ≥ the upper limit normal (ULN), and perforation had worse OS. We used these data to establish a nomogram to predict survival for PGINKTL. The nomogram displayed good accuracy, with a C‐index of 0.726. Conclusion The clinical features and poor outcomes of PGINKTL, which is a rare and fatal lymphoma type, are presented. The proposed nomogram provides an individualized estimate of survival for these patients. In the future, the study focused on exploring a better treatment strategy to improve survival is required in PGINKTL.

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“…[4][5][6] Although this tumor most commonly involves the colon in the gastrointestinal tract and portions of the small intestine, gastric involvement is seldom observed. [7][8][9] The cluster of differentiation (CD) 56 antigen is typically detected following most immunohistochemistry of tumor cells. 1,4 CD56 is the most sensitive marker for ENKTCL-NT.…”

Section: Introductionmentioning

confidence: 99%

Gastric CD56-negative Extranodal Natural Killer/T-cell Lymphoma: A Case Report

Chung

Jee

Choi

et al. 2022

Korean J Gastroenterol

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Extranodal natural killer (NK)/T-cell lymphoma, nasal type (ENKTCL-NT) is the most common subtype of Epstein-Barr virus-associated NK/T-cell lymphomas. ENKTCL-NT occurs infrequently in the gastrointestinal tract. In particular, reports of ENKTCL-on NT arising from the stomach are extremely rare. Several clusters of differentiation (CDs) have been useful in recognizing NK-cells, T-cells, and tumor cells of NK/T-cell lymphomas. Among them, the CD56 antigen is considered the most sensitive marker for ENKTCL-NT and is expressed in almost all cases of ENKTCL-NT. Thus, the development of CD56-negative ENKTCL-NT is highly atypical. This paper reports a case of a young Asian female who presented with gastric ulcer bleeding. The patient was histologically diagnosed with ENKTCL-NT. No tumor cells for CD56 were observed, whereas no monoclonality of the T-cell receptor gamma gene rearrangement was detected in the tumor cells. The patient was scheduled for systemic chemotherapy six times and achieved complete remission. Peripheral blood-hematopoietic stem cell transplantation was performed later.

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Primary Gastrointestinal T/NK Cell Lymphoma

Cited by 10 publications

References 35 publications

Primary Gastrointestinal T-Cell Lymphoma and Indolent Lymphoproliferative Disorders: Practical Diagnostic and Treatment Approaches

Primary Gastrointestinal T-Cell Lymphoma and Indolent Lymphoproliferative Disorders: Practical Diagnostic and Treatment Approaches

Real‐World clinical features and survival outcomes associated with primary gastrointestinal natural killer/T‐cell lymphoma from 1999 to 2020

Gastric CD56-negative Extranodal Natural Killer/T-cell Lymphoma: A Case Report

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