Ann Dermatol.  2011 Sep;23(Suppl 1):S100-S104. 10.5021/ad.2011.23.S1.S100.

Fatal Cutaneous gamma/delta T-Cell Lymphoma with Central Nerve System Metastasis

• Youn SH
• Lee YW
• Min SK
• Park HR
• Kim KH
• Kim KJ

• KMID: 2156763
• DOI: http://doi.org/10.5021/ad.2011.23.S1.S100

Abstract

Cutaneous gamma/delta T-cell lymphoma is an extremely rare and highly aggressive tumor that is often resistant to treatment, such as polychemotherapy and radiotherapy. Due to the complexity of clinical, pathologic, and immunohistochemical features of this disease entity, the physician should perform a careful evaluation; however, treatment should be rapid and aggressive. We present a case of fatal cutaneous gamma/delta T-cell lymphoma of a 55-year old woman who died after recurrence with central nerve system metastasis.

Keyword

CNS; Cutaneous T cell lymphoma; Gamma delta TCR; Neoplasm metastasis

MeSH Terms

Drug Therapy, Combination
Female
Humans
Lymphoma, T-Cell
Lymphoma, T-Cell, Cutaneous
Neoplasm Metastasis
Recurrence
T-Lymphocytes

Figure

• 

  Fig. 1 Localized, dusky red colored, centrally umbilicated plaque, with a peripheral erythematous patch, on the left foot.

• 

  Fig. 2 (A, B) Epidermal atrophy, dermoepidermal vacuolization, massive cellular infiltration of the entire dermis, as well as a mostly lobular, inflammatory infiltrate of the subcutaneous fat of lymphoid cells and histiocytes (H&E, ×100). (C, D) At high power magnification, the "rimming" of fat cells by pleomorphic, atypical T lymphocytes in the subcutaneous fat was visible (H&E, ×400).

• 

  Fig. 3 Tumor cells in the dermis were positive for CD3 (A, ×400) and negative for CD4 (B, ×400), CD8 (C, ×400), and βF1 (D, ×400).

• 

  Fig. 4 MRI showed metastasis (white arrows) in the spinal cord.

Reference

1. Koch R, Jaffe ES, Mensing C, Zeis M, Schmitz N, Sander CA. Cutaneous gamma/delta T-cell lymphoma. J Dtsch Dermatol Ges. 2009. 7:1065–1067.

2. Willemze R, Jansen PM, Cerroni L, Berti E, Santucci M, Assaf C, et al. EORTC Cutaneous Lymphoma Group. Subcutaneous panniculitis-like T-cell lymphoma: definition, classification, and prognostic factors: an EORTC Cutaneous Lymphoma Group Study of 83 cases. Blood. 2008. 111:838–845.

3. Kempf W, Sander CA. Classification of cutaneous lymphomas-an update. Histopathology. 2010. 56:57–70.

4. Amado A, McDonnell JK, Somani N, Bunting ST, Winfield HL. Cutaneous gamma-delta T-cell lymphoma. Leuk Lymphoma. 2008. 49:2003–2005.

5. Kao GF, Resh B, McMahon C, Gojo I, Sun CC, Phillips D, et al. Fatal subcutaneous panniculitis-like T-cell lymphoma gamma/delta subtype (cutaneous gamma/delta T-cell lymphoma):report of a case and review of the literature. Am J Dermatopathol. 2008. 30:593–599.

6. Ralfkiaer E, Willemze R, Meijer CJLM, Dummer R, Jaffe ES, Swerdlow SH, et al. Leboit PE, Burg G, Weedon D, Sarasin A, editors. Primary cutaneous peripheral T-cell lymphoma, unspecified. Pathology and genetics of skin tumours. 2006. Lyon: IARC Press;184–188.

7. Kumakura S. Hemophagocytic syndrome. Intern Med. 2005. 44:278–280.

8. Kong YY, Dai B, Kong JC, Zhou XY, Lu HF, Shen L, et al. Subcutaneous panniculitis-like T-cell lymphoma: a clinicopathologic, immunophenotypic, and molecular study of 22 Asian cases according to WHO-EORTC classification. Am J Surg Pathol. 2008. 32:1495–1502.

9. Toro JR, Liewehr DJ, Pabby N, Sorbara L, Raffeld M, Steinberg SM, et al. Gamma-delta T-cell phenotype is associated with significantly decreased survival in cutaneous T-cell lymphoma. Blood. 2003. 101:3407–3412.

10. Cerroni L, Gatter K, Kerl H. Skin lymphoma: the illustrated guide. 2009. 3rd ed. Chichester: Wiley-Blackwell;97–113.

11. Takeshita M, Imayama S, Oshiro Y, Kurihara K, Okamoto S, Matsuki Y, et al. Clinicopathologic analysis of 22 cases of subcutaneous panniculitis-like CD56- or CD56+ lymphoma and review of 44 other reported cases. Am J Clin Pathol. 2004. 121:408–416.

12. Nakashima H, Sugaya M, Minatani Y, Ohmatsu H, Asano N, Fujimoto M, et al. Cutaneous gamma/delta T-cell lymphoma treated with retinoid and narrowband ultraviolet B. Clin Exp Dermatol. 2009. 34:e345–e346.