TY - JOUR
T1 - Primary cutaneous non-Hodgkin's lymphoma of Ann Arbor stage I
T2 - Preferential cutaneous relapses but high cure rate with doxorubicin-based therapy
AU - Sarris, A. H.
AU - Braunschweig, I.
AU - Medeiros, L. J.
AU - Duvic, M.
AU - Ha, C. S.
AU - Rodriguez, M. A.
AU - Hagemeister, F. B.
AU - McLaughlin, P.
AU - Romaguera, J.
AU - Cox, J.
AU - Cabanillas, F.
PY - 2001/1/15
Y1 - 2001/1/15
N2 - Purpose: Establish frequency, presenting features, response and relapse patterns, and outcome of primary cutaneous non-Hodgkin's lymphoma (PCNHL). Patients and Methods: Review of untreated patients, older than 16 years, presenting between 1971 and 1993 with cutaneous lymphoma, not mycosis fungoides, and Ann Arbor stage I. Results: We identified 46 patients, 27 males, with median age of 57 years. Treatment was radiotherapy in 10 patients, doxorubicin-based therapy in 33 patients that was followed by radiotherapy in 25 patients, and other combination with radiotherapy in one patient. The complete response rate was 95%. After a median follow-up of 140 months (range, 61 to 284 months), 18 patients have relapsed, and 14 have died from lymphoma. The first failure was exclusively cutaneous in 50% of relapses. For the 44 treated patients, progression-free survival (PFS; actuarial ± SE) was 61% ± 7% and survival was 58% ± 9% at 12 years. For the 18 patients with diffuse large B-cell lymphoma, after doxorubicin-based regimens, PFS was 71% ± 12% (P = .0003) versus 0% after radiotherapy; survival was 77% ± 12% versus 25% ± 22% (P = 004), respectively. For the nine patients with follicular center-cell lymphoma treated with combined modality, the 12-year PFS was 89% ± 11% and survival 70% ± 18%. Conclusion: PCNHL is rare, and its first relapse is exclusively cutaneous in 50% of patients. Patients with diffuse large B-cell lymphoma are curable with doxorubicin-based regimens but not with radiotherapy. Prospective studies in PCNHL should define the cytogenetics, the basis for cutaneous tropism, the prognosis of histologic subtypes, and the role of radiotherapy.
AB - Purpose: Establish frequency, presenting features, response and relapse patterns, and outcome of primary cutaneous non-Hodgkin's lymphoma (PCNHL). Patients and Methods: Review of untreated patients, older than 16 years, presenting between 1971 and 1993 with cutaneous lymphoma, not mycosis fungoides, and Ann Arbor stage I. Results: We identified 46 patients, 27 males, with median age of 57 years. Treatment was radiotherapy in 10 patients, doxorubicin-based therapy in 33 patients that was followed by radiotherapy in 25 patients, and other combination with radiotherapy in one patient. The complete response rate was 95%. After a median follow-up of 140 months (range, 61 to 284 months), 18 patients have relapsed, and 14 have died from lymphoma. The first failure was exclusively cutaneous in 50% of relapses. For the 44 treated patients, progression-free survival (PFS; actuarial ± SE) was 61% ± 7% and survival was 58% ± 9% at 12 years. For the 18 patients with diffuse large B-cell lymphoma, after doxorubicin-based regimens, PFS was 71% ± 12% (P = .0003) versus 0% after radiotherapy; survival was 77% ± 12% versus 25% ± 22% (P = 004), respectively. For the nine patients with follicular center-cell lymphoma treated with combined modality, the 12-year PFS was 89% ± 11% and survival 70% ± 18%. Conclusion: PCNHL is rare, and its first relapse is exclusively cutaneous in 50% of patients. Patients with diffuse large B-cell lymphoma are curable with doxorubicin-based regimens but not with radiotherapy. Prospective studies in PCNHL should define the cytogenetics, the basis for cutaneous tropism, the prognosis of histologic subtypes, and the role of radiotherapy.
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U2 - 10.1200/JCO.2001.19.2.398
DO - 10.1200/JCO.2001.19.2.398
M3 - Article
C2 - 11208831
AN - SCOPUS:0035863559
SN - 0732-183X
VL - 19
SP - 398
EP - 405
JO - Journal of Clinical Oncology
JF - Journal of Clinical Oncology
IS - 2
ER -