Tags

Type your tag names separated by a space and hit enter

Cutaneous gamma-delta T-cell lymphoma with central nervous system involvement: report of a rarity with review of literature.
J Cutan Pathol 2014; 41(12):936-43JC

Abstract

Primary cutaneous gamma-delta (γδ) T-cell lymphoma is an extremely rare and aggressive variant of cutaneous lymphoma. Central nervous system (CNS) involvement, a rare finding, and hemophagocytic syndrome are two complications that are commonly fatal. We describe a 58-year-old patient presenting with skin plaque who subsequently developed subcutaneous nodules diagnosed as cutaneous T-cell lymphoma (CTCL), clinically resembling 'mycosis fungoides'. The patient was treated with repeat topical radiation therapies but had frequent relapsed disease. Approximately 4.5 years after, the patient presented with third and sixth cranial nerve palsies and was found to have CNS involvement by lymphoma per positron emission tomography-computed tomography (PET/CT) and a biopsy of foramen magnum. Phenotypically, the tumor cells were CD3(+)/CD4(-)/CD8(-)/CD7(+)/CD5(-)/CD30(-)/TCRαβ(-)/TCRγδ(+). Despite aggressive strategies taken, the patient expired 3 months after the diagnosis of the CNS lesion. A retrospective investigation proved the original CTCL to be γδ T-cell in origin, confirming an indolent cutaneous γδ T-cell lymphoma with eventual CNS manifestation. We present this case to draw attention to the entity, which can occasionally present with misleading histopathologic and clinical features. In addition, we provide a review of the literature to summarize clinical and pathologic features of the reported similar cases.

Authors+Show Affiliations

Department of Pathology, University of South Florida Morsani College of Medicine, Tampa, FL, USA.No affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Case Reports
Journal Article
Review

Language

eng

PubMed ID

25292289

Citation

Harrington, Lacey, et al. "Cutaneous Gamma-delta T-cell Lymphoma With Central Nervous System Involvement: Report of a Rarity With Review of Literature." Journal of Cutaneous Pathology, vol. 41, no. 12, 2014, pp. 936-43.
Harrington L, Sokol L, Holdener S, et al. Cutaneous gamma-delta T-cell lymphoma with central nervous system involvement: report of a rarity with review of literature. J Cutan Pathol. 2014;41(12):936-43.
Harrington, L., Sokol, L., Holdener, S., Shao, H., & Zhang, L. (2014). Cutaneous gamma-delta T-cell lymphoma with central nervous system involvement: report of a rarity with review of literature. Journal of Cutaneous Pathology, 41(12), pp. 936-43. doi:10.1111/cup.12395.
Harrington L, et al. Cutaneous Gamma-delta T-cell Lymphoma With Central Nervous System Involvement: Report of a Rarity With Review of Literature. J Cutan Pathol. 2014;41(12):936-43. PubMed PMID: 25292289.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Cutaneous gamma-delta T-cell lymphoma with central nervous system involvement: report of a rarity with review of literature. AU - Harrington,Lacey, AU - Sokol,Lubomir, AU - Holdener,Stephanie, AU - Shao,Haipeng, AU - Zhang,Ling, Y1 - 2014/11/11/ PY - 2014/01/15/received PY - 2014/06/19/revised PY - 2014/07/20/accepted PY - 2014/10/9/entrez PY - 2014/10/9/pubmed PY - 2015/9/18/medline KW - T-cell lymphoma KW - central nervous system (CNS) KW - cutaneous KW - dual CD4/CD8 negative KW - gamma-delta (γδ) SP - 936 EP - 43 JF - Journal of cutaneous pathology JO - J. Cutan. Pathol. VL - 41 IS - 12 N2 - Primary cutaneous gamma-delta (γδ) T-cell lymphoma is an extremely rare and aggressive variant of cutaneous lymphoma. Central nervous system (CNS) involvement, a rare finding, and hemophagocytic syndrome are two complications that are commonly fatal. We describe a 58-year-old patient presenting with skin plaque who subsequently developed subcutaneous nodules diagnosed as cutaneous T-cell lymphoma (CTCL), clinically resembling 'mycosis fungoides'. The patient was treated with repeat topical radiation therapies but had frequent relapsed disease. Approximately 4.5 years after, the patient presented with third and sixth cranial nerve palsies and was found to have CNS involvement by lymphoma per positron emission tomography-computed tomography (PET/CT) and a biopsy of foramen magnum. Phenotypically, the tumor cells were CD3(+)/CD4(-)/CD8(-)/CD7(+)/CD5(-)/CD30(-)/TCRαβ(-)/TCRγδ(+). Despite aggressive strategies taken, the patient expired 3 months after the diagnosis of the CNS lesion. A retrospective investigation proved the original CTCL to be γδ T-cell in origin, confirming an indolent cutaneous γδ T-cell lymphoma with eventual CNS manifestation. We present this case to draw attention to the entity, which can occasionally present with misleading histopathologic and clinical features. In addition, we provide a review of the literature to summarize clinical and pathologic features of the reported similar cases. SN - 1600-0560 UR - https://www.unboundmedicine.com/medline/citation/25292289/Cutaneous_gamma_delta_T_cell_lymphoma_with_central_nervous_system_involvement:_report_of_a_rarity_with_review_of_literature_ L2 - https://doi.org/10.1111/cup.12395 DB - PRIME DP - Unbound Medicine ER -