Extranodal nasal-type natural killer/T-cell lymphoma of the skin: a clinicopathologic study of 16 cases in China

Summary Extranodal nasal-type natural killer/T-cell lymphoma presenting in skin, either primary or secondary, is relatively rare in China, accounting for about 4.1% of tumors. The clinicopathologic features of the neoplasm are still poorly understood. Sixteen Chinese cases of cutaneous natural kille...

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Bibliographic Details
Published in:Human pathology 2009-06, Vol.40 (6), p.807-816
Main Authors: Yu, Jian-Bo, PhD, Zuo, Zhuo, MD, Tang, Yuan, MD, Zhao, Sha, PhD, Zhang, Ying-Chun, MD, Bi, Cheng-Feng, MD, Wang, Wei-Ya, MD, Zhang, Wen-Yan, PhD, Wang, Lin, MD, Liu, Wei-Ping, MD
Format: Article
Language:English
Subjects:
Adolescent
Adult
Aged
Biological and medical sciences
Blood
Cancer
China - epidemiology
Classification
Cytotoxicity
Deoxyribonucleic acid
DNA
Female
Hematologic and hematopoietic diseases
Humans
Investigative techniques, diagnostic techniques (general aspects)
Killer Cells, Natural - pathology
Leukemias. Malignant lymphomas. Malignant reticulosis. Myelofibrosis
Lymphoma
Lymphoma, T-Cell - mortality
Lymphoma, T-Cell - pathology
Male
Medical sciences
Middle Aged
Natural killer cell
Nose - pathology
Nose Neoplasms - mortality
Nose Neoplasms - pathology
Pathology
Pathology. Cytology. Biochemistry. Spectrometry. Miscellaneous investigative techniques
Polymerase chain reaction
Proteins
Radiation therapy
Retrospective Studies
Skin
Skin Neoplasms - mortality
Skin Neoplasms - pathology
T cell
Tumors
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Summary:Summary Extranodal nasal-type natural killer/T-cell lymphoma presenting in skin, either primary or secondary, is relatively rare in China, accounting for about 4.1% of tumors. The clinicopathologic features of the neoplasm are still poorly understood. Sixteen Chinese cases of cutaneous natural killer/T-cell lymphomas were investigated retrospectively by pathology, immunophenotype, genotype, Epstein-Barr virus status, and survival analysis. The mean age of the patients was 36.3 years; 81% of the patients presented with multiple cutaneous lesions. Angiocentricity and epidermotropism were observed in 43.7% and 25%, respectively; the average number of mitoses was 12 per 10 high-power fields. The neoplastic cells of all 16 cases were positive for CD3 ɛ /CD45RO and granzyme B and negative for CD4, CD5, CD8, and CD20; 87.5% of the tumors expressed CD56. Signals for EBER were detected in 81.3% of cases. No clonal TCRγ/TCR β gene rearrangement was demonstrated. Thirteen of 15 patients received chemotherapy; a partial response was achieved in 10 patients; 10 (66.7%) of 15 patients died of the disease, and the average survival time was 6.6 months. In conclusion, both primary and secondary cutaneous natural killer/T-cell lymphomas are highly aggressive. The outcome is closely related to the number and distribution of the lesions as well as the stage. In patients with generalized skin lesions and extracutaneous involvement, the prognosis usually is poor.
ISSN:0046-8177
1532-8392
DOI:10.1016/j.humpath.2008.08.020