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Extranodal natural killer/T-cell lymphoma involving the gastrointestinal tract: analysis of clinical features and outcomes from the Asia Lymphoma Study Group.

Kim SJ, Jung HA, Chuang SS, Hong H, Guo CC, Cao J, Hong XN, Suzuki R, Kang HJ, Won JH, Chng WJ, Kwong YL, Suh C, Song YQ, Zhu J, Tay K, Lim ST, Suzumiya J, Lin TY, Kim WS, Asia Lymphoma Study Gro - J Hematol Oncol (2013)

Bottom Line: The most common GI site was the small intestine, including the jejunum and ileum (n = 57, 70.3%).Thus, the median overall survival was 7.8 months (95% Confidence interval: 3.9 - 11.7 months).Thus, more effective treatment strategies are required for this disease entity.

View Article: PubMed Central - HTML - PubMed

Affiliation: Department of Medicine, Division of Hematology and Oncology, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea. TongyuLim@hotmail.com.

ABSTRACT

Background: The gastrointestinal (GI) tract is one of the most common extranasal sites in extranodal NK/T-cell lymphoma (ENKTL). However, data regarding ENKTL involving the GI tract are relatively scarce. Thus, we performed a multicenter, multinational retrospective study to analyze clinical features and treatment outcomes of ENKTL involving the GI tract.

Patients and methods: Patients with ENKTL involving the GI tract diagnosed in twelve participating centers between 1991 and 2012 were retrospectively analyzed from five Asian countries.

Results: The analysis of 81 patients with ENKTL involving the GI tract revealed that more than 60% of patients presented as advanced disease with B symptoms. 55 patients (68%) had GI manifestations including abdominal pain (n = 26, 32%), GI tract bleeding (n = 17, 21%) and bowel perforation (n = 12, 15%). The most common GI site was the small intestine, including the jejunum and ileum (n = 57, 70.3%). There were 34 patients (42%) who received systemic chemotherapy while 33 patients (41%) underwent surgery plus chemotherapy. However, 35 patients (43%) died due to disease progression, and treatment-related mortality including sepsis occurred in 17 patients (21%). Thus, the median overall survival was 7.8 months (95% Confidence interval: 3.9 - 11.7 months). Patients who could undergo surgery plus chemotherapy showed a trend of better survival than those treated with chemotherapy alone.

Conclusion: Overall, the data indicated that ENKTL involving the GI tract has a dismal prognosis despite active treatment including chemotherapy and surgery. Thus, more effective treatment strategies are required for this disease entity.

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Related in: MedlinePlus

Clinical manifestation and gastrointestinal tract involvement. (A) Comparison of clinical manifestation and anatomic involvement (B) Pattern of gastrointestinal tract involvement. (C) Frequency of involvement of each part of the GI tract by extranodal NK/T-cell lymphoma from the esophagus to the recto-sigmoid colon (The percentage of each part of GI tract = the number of patients involving each part/total number of involved parts in 81 patients × 100).
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Figure 1: Clinical manifestation and gastrointestinal tract involvement. (A) Comparison of clinical manifestation and anatomic involvement (B) Pattern of gastrointestinal tract involvement. (C) Frequency of involvement of each part of the GI tract by extranodal NK/T-cell lymphoma from the esophagus to the recto-sigmoid colon (The percentage of each part of GI tract = the number of patients involving each part/total number of involved parts in 81 patients × 100).

Mentions: Eighty-one patients were included in this retrospective analysis. The median age of patients at diagnosis (45 years, range: 17–79 years), and male predominance (69%, 56/81) were similar to the overall characteristics reported for ENKTL [10]. However, approximately 65% of patients had two or more sites of extranodal involvement (Table 1). Thus, the proportion of advanced disease was over 60%, and B symptoms were also frequently observed (63%, 51/81). As a result, risk stratification based on the NKPI showed that > 50% of patients belonged to the high-risk category of group 4. In contrast, the IPI showed only 11 patients were grouped as high-risk, because the majority of patients were younger than 60 years of age (Table 1). GI manifestations were the main symptom at diagnosis in 55 patients (68%) and included abdominal pain (n = 26, 32%), GI tract bleeding (n = 17, 21%) and bowel perforation (n = 12, 15%). Thus, a substantial number of patients underwent surgery to remove their primary mass for diagnostic as well as therapeutic purposes. The other 26 patients (32%) presented as non-GI manifestations including nasal obstruction (n = 6) or systemic symptoms (n = 20) such as fever and night sweat. During the evaluation of these 26 patients including endoscopic examination of nasal cavity and CT scans of neck, chest and abdomen-pelvis, the simultaneous involvement of nasal cavity or nasopharynx as well as GI tract was found in 18 patients (22%). Thus, these 18 patients were pathologically diagnosed with ENKTL by biopsy of nasal areas, and the involvement of GI tract was documented in abdominal CT scan and/or positron emission tomography (PET)/CT scan. The remaining eight patients out of 26 patients presented with non-GI manifestation also underwent surgery to remove mass of GI tract for pathologic diagnosis (Figure 1A).


Extranodal natural killer/T-cell lymphoma involving the gastrointestinal tract: analysis of clinical features and outcomes from the Asia Lymphoma Study Group.

Kim SJ, Jung HA, Chuang SS, Hong H, Guo CC, Cao J, Hong XN, Suzuki R, Kang HJ, Won JH, Chng WJ, Kwong YL, Suh C, Song YQ, Zhu J, Tay K, Lim ST, Suzumiya J, Lin TY, Kim WS, Asia Lymphoma Study Gro - J Hematol Oncol (2013)

Clinical manifestation and gastrointestinal tract involvement. (A) Comparison of clinical manifestation and anatomic involvement (B) Pattern of gastrointestinal tract involvement. (C) Frequency of involvement of each part of the GI tract by extranodal NK/T-cell lymphoma from the esophagus to the recto-sigmoid colon (The percentage of each part of GI tract = the number of patients involving each part/total number of involved parts in 81 patients × 100).
© Copyright Policy - open-access
Related In: Results  -  Collection

License 1 - License 2
Show All Figures
getmorefigures.php?uid=PMC4225665&req=5

Figure 1: Clinical manifestation and gastrointestinal tract involvement. (A) Comparison of clinical manifestation and anatomic involvement (B) Pattern of gastrointestinal tract involvement. (C) Frequency of involvement of each part of the GI tract by extranodal NK/T-cell lymphoma from the esophagus to the recto-sigmoid colon (The percentage of each part of GI tract = the number of patients involving each part/total number of involved parts in 81 patients × 100).
Mentions: Eighty-one patients were included in this retrospective analysis. The median age of patients at diagnosis (45 years, range: 17–79 years), and male predominance (69%, 56/81) were similar to the overall characteristics reported for ENKTL [10]. However, approximately 65% of patients had two or more sites of extranodal involvement (Table 1). Thus, the proportion of advanced disease was over 60%, and B symptoms were also frequently observed (63%, 51/81). As a result, risk stratification based on the NKPI showed that > 50% of patients belonged to the high-risk category of group 4. In contrast, the IPI showed only 11 patients were grouped as high-risk, because the majority of patients were younger than 60 years of age (Table 1). GI manifestations were the main symptom at diagnosis in 55 patients (68%) and included abdominal pain (n = 26, 32%), GI tract bleeding (n = 17, 21%) and bowel perforation (n = 12, 15%). Thus, a substantial number of patients underwent surgery to remove their primary mass for diagnostic as well as therapeutic purposes. The other 26 patients (32%) presented as non-GI manifestations including nasal obstruction (n = 6) or systemic symptoms (n = 20) such as fever and night sweat. During the evaluation of these 26 patients including endoscopic examination of nasal cavity and CT scans of neck, chest and abdomen-pelvis, the simultaneous involvement of nasal cavity or nasopharynx as well as GI tract was found in 18 patients (22%). Thus, these 18 patients were pathologically diagnosed with ENKTL by biopsy of nasal areas, and the involvement of GI tract was documented in abdominal CT scan and/or positron emission tomography (PET)/CT scan. The remaining eight patients out of 26 patients presented with non-GI manifestation also underwent surgery to remove mass of GI tract for pathologic diagnosis (Figure 1A).

Bottom Line: The most common GI site was the small intestine, including the jejunum and ileum (n = 57, 70.3%).Thus, the median overall survival was 7.8 months (95% Confidence interval: 3.9 - 11.7 months).Thus, more effective treatment strategies are required for this disease entity.

View Article: PubMed Central - HTML - PubMed

Affiliation: Department of Medicine, Division of Hematology and Oncology, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea. TongyuLim@hotmail.com.

ABSTRACT

Background: The gastrointestinal (GI) tract is one of the most common extranasal sites in extranodal NK/T-cell lymphoma (ENKTL). However, data regarding ENKTL involving the GI tract are relatively scarce. Thus, we performed a multicenter, multinational retrospective study to analyze clinical features and treatment outcomes of ENKTL involving the GI tract.

Patients and methods: Patients with ENKTL involving the GI tract diagnosed in twelve participating centers between 1991 and 2012 were retrospectively analyzed from five Asian countries.

Results: The analysis of 81 patients with ENKTL involving the GI tract revealed that more than 60% of patients presented as advanced disease with B symptoms. 55 patients (68%) had GI manifestations including abdominal pain (n = 26, 32%), GI tract bleeding (n = 17, 21%) and bowel perforation (n = 12, 15%). The most common GI site was the small intestine, including the jejunum and ileum (n = 57, 70.3%). There were 34 patients (42%) who received systemic chemotherapy while 33 patients (41%) underwent surgery plus chemotherapy. However, 35 patients (43%) died due to disease progression, and treatment-related mortality including sepsis occurred in 17 patients (21%). Thus, the median overall survival was 7.8 months (95% Confidence interval: 3.9 - 11.7 months). Patients who could undergo surgery plus chemotherapy showed a trend of better survival than those treated with chemotherapy alone.

Conclusion: Overall, the data indicated that ENKTL involving the GI tract has a dismal prognosis despite active treatment including chemotherapy and surgery. Thus, more effective treatment strategies are required for this disease entity.

Show MeSH
Related in: MedlinePlus