Peripheral T-cell lymphoma

Published Date: 19th July 2016

Publication Authors: Sharma N, Tappin J, Ngan K

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Abstract

A 70-year-old Caucasian gentleman presented with a four-month history of a rapidly enlarging skin tumor on his right lower abdomen, episodes of night sweats and generalized pruritus. No other relevant medical or drug history was found.

Clinical examination revealed a large 16 by 13 centimeters fungating tumor on his right lower abdomen and palpable inguinal lymphadenopathy. Blood tests showed raised lactate dehydrogenase level of 1015 units/L (normal range 90-500 units/L). A subsequent computed tomography (CT) scan demonstrated a large mass in the right anterior abdominal wall infiltrating into the underlying subcutaneous fat, along with widespread lymphadenopathy.

Histological examination of an incisional biopsy sample taken from the abdominal mass revealed pandermal infiltration of atypical medium to large sized lymphoid cells, which were separated from the epidermis by a Grenz zone. These cells were positive for pan-T-cell antigens (CD3 and CD5), and predominantly CD4 positive. Some of the large cells stained positive for CD30, but were negative for ALK-1 and EMA. CD56 and EBER were negative.

The patient was diagnosed with peripheral T-cell lymphoma, not otherwise specified (PTCL, NOS) based on the WHO classification for lymphomas. He received treatment with six-cycle course of CHOP (cyclophosphamide, doxorubicin, vincristine, prednisolone) chemotherapy under the local Hemato-Oncology team. Post-treatment examination and CT scan revealed almost complete regression of the abdominal mass. The widespread lymphadenopathy was no longer apparent.

PTCL is a rare and heterogeneous group of T-cell non-Hodgkin lymphomas (NHL). Classification of PTCL is complex and the term PTCL, NOS is used when a tumor does not fit into any specific subgroup. PTCL, NOS can present with nodal and/or extra-nodal (commonly bone marrow and/or liver) disease, with cutaneous involvement reported in only 10 to 18% of cases. Multiple, generalized or solitary skin tumors may develop as a primary or secondary manifestation. Diagnosis is confirmed via histological examination with immunostaining. Variable numbers of pleomorphic or immunoblast-like T cells with little, if any, epidermotropism may be seen. These cells show variable loss of pan-T-cell antigens, and are often CD4 positive. Management traditionally involves a combination chemotherapy regimen e.g. CHOP commonly employed to treat aggressive NHLs. Prognosis is generally poor, with reported 5-year survival rates of less than 20%.

Wong, JLC; Sharma, N; Tappin, J; Ngan, K. (2015).  A rare case of peripheral T-cell lymphoma, not otherwise specified presenting as a solitary abdominal mass . Journal of the American Academy of Dermatology. 72 (5 Suppl 1), AB155

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