Abstract
OBJECTIVE To report results of radiation therapy treatment of 30 B-cell lymphoma patients with an initial cutaneous presentation according to the new classification by the WHO/EORTC.
METHODS Thirty patients with cutaneous B-cell lymphoma (CBCL) were treated by cutaneous irradiation based on the number and location of the lesions and the stage of their tumor. Treatment was conducted using a Saturne Clinac.
RESULTS A complete response (CR) from the treatment for our series was 86%. The length of complete remission ranged from 4 to 301 months. Three patients (11%) developed a partial response (PR). One patient was progressive. Disease-free survival(DFS) at 10 years was 87%. Three patiens died [One PCMZL two PCLBCL leg type (29%)]. Radiotherapy was generally well tolerated.
CONCLUSION According to the WHO/EORTC classification, the survivor results were good for PCMZL and PCFCL. The PCLBCL leg type had a poor prognosis. Localized field irradiation is an effective treatment for some localized forms of primary cutaneous B-cell lymphoma, and this mode of therapy can produce prolonged remissions. The patients with wide-spread skin involvement are usually candidates for extended field irradiation and/or chemotherapy. For advanced stages of cutaneous B-cell lymphoma, where chemotherapy is the treatment of choice, a degree of palliation can be achieved using local field irradation.
keywords
Introduction
Primary cutaneous B-cell lymphoma (PCBCL) is one of a special type of cutaneous lymphomas, making up 25% of the total[1,2]. It is classified into big and small cell subtypes. The patients are diagnosed by many nodes/lumps/or plaques on the skin of the trunk, skull, and mainly lower limbs for senior patients. The tumor shows diffused infiltration into the dermis and subcutaneous fat. For the B-cell group is not included in the lymph tissue of the skin, PCBCL is developed by aquired B-cell lymph tissue. Radiotherapy is the first treatment of choice producing a 85%~100% local control rate. However, the recurrence rate is high with the development of new focals.
According to the 2005 WHO-EORTC[3,4], cutaneous B-cell lymphomas are divided into 4 types:1) Primary cutaneous marginal zone B-cell lymphoma (PCMZL); 2) Primary cutaneous follicle center lymphoma (PCFCL); 3) Primary cutaneous diffuse large B-cell lymphoma, leg type (PCLBCL); 4) Primary cutaneous diffuse large B-cell lymphoma, other type (PCLBCL other type). The goal of the study was to report the efficacy of radiotherapy of 30 cases with PCBCL.
Materials and Methods
Thirty cases of PCBCL, from the Department of Oncology, Henri Mondor University Hospital comprised of 21males, 9 females of ages 23~89 (mean 48) were treated from October, 1978 to June, 2002. The patients underwent skin biopsy, routine blood tests, chest X-ray and a CT scan of the abdomen and pelvic cavity. Follow-ups started from the end of the radiotherapy for 4~301 months (mean 47 months).
All the patients were reclassified according to the WHO/EORTC as follows: 2 cases with PC-MZL(6.7%); 18 with PCFCL (60%); 8 with PCLBCL, leg type (26.7%); 1 with vascular PCLBCL; 1 unknown type. In terms of the number of foci, there were 13 cases (43%) with a single focus, 13 (43%) with multi-foci and 4 (13%) with disseminated foci (Table 1).
Relationship between the 2005 WHO/EORTC and foci numbers (%).
Location of the foci were as follows: 7 on the skull and neck; 6 on the trunk; 7 on the lower limbs; 3 on the upper limbs and 7 with diffuse foci (Table 2).
Relationship between 2005 WHO/EORTC and focal location (%).
Eleven cases (36.7%) received CHOP before irradiation. EFI (extended field irradiation) or LFI (localized field irradiation) was applied according to the focus area and the number of foci[5-7]. EFI was administered to 9 cases with multi-diffuse foci (Table 3).
Relationship between the 2005 WHO/EORTC and mode of treatment (%).
The Saturne Clinac was used with energy 4~8 MV, dosage of 30 Gy (4/week; 2.5 Gy/time). The whole body or half of the body was irradiated as the target areas with lead cover protection of eyes and mouth. A total of 22 cases were treated by both EFI and LFI using a DARPAC X-ray machine with a 0.55 cm aluminium filter, 45 kV to the small focus, 100 kV to the deeper focus and 30~40 Gy (2 Gy/time, 5/week). The lumps plus 2.5 cm around the lump were the irradiated areas. The total survival rate was accounted from the end of radiotherapy to the time of death, while the disease-free survival rate was considered to be from the end of radiotherapy to a recurrence of the disease. Disappearance of the foci was termed complete remission, CR; a 50% shrinkage, partial remission, PR; less than 50%, non-responsive, NR. The graded skin reactions were found after radiotherapy (grade I, skin erythema; II, mid-reaction; III, severe reaction with skin inflammation and IV, ulcer and necrosis).
Statistical analysis
The statistic software SSPS10.0 was utilized to analyze the data.
Results
A total of 26 cases (87%) were CR during 4~301 months, 3 cases (10%) developed a PR, 1 deteriorated, 2 had metastasis and 3 died. The 10-year total survival rate was 87%. All the patients tolerated the radiotherapy.
The CR for PCMZL was 100%; for PCFCL 89%; and for PCLBCL leg type 88%. The PR for PCFCL was 11% and 1 vascular PCLBCL. Fourteen cases (41.7%) developed a recurrence including 1 PCMZL (50%), 9 PCFCL (50%), 3 PCLBCL (43%) (Table 4).
Efficacy of types and 5-year total and disease-free survival rates (%).
The mean duration for a recurrence was 73 months. All subtypes had high recurrence rates but good efficacy after LFI.
The complications after radiotherapy included acute skin erathema in 4 cases and a late reaction in 2 cases. The patients showed good toleration from radiotherapy which caused few side effects.
Discussion
Our patients were classified according to the new WHO/EORTC. They were composed of a PCFCL type (60%), PCLBCL lower limb type (26.7%), PCMZL, PCLBCL and other rare types. These results were in accordence with a study by Zinzani et al.[8] who analyzed 467 cases in which PCFCL were 56.7%, PCMZL 31.4% and PCLBCL leg type 10.9%.
According to the WHO/EORTC, our study showed that both PCMBCL and PCFCL had effective results and high survival rates. The remission rate of our patient group was 86.7%, while 41.7% of the patients had recurrence within 73 months after radiotherapy. All the subtypes had high recurrence rates out of the localized irradiation field. Eich et al.[9] reported 35 cases of PCBCL for which they used 45 Gy of radiotherapy. They showed 97.1% of remission and 31% recurrent at 11 months after treatment (3 PCMZL local field, 8 outer field).
In our report, 2 PCMZL cases developed CR and 1 case recurred 15 months after treatment. In a study by Servitje et al.[10], 22 PCMBCL cases reached 100% CR and 11 (50%) were recurrent. Hoefnagel et al.[11] reported 40 cases of PCMZL receiving radiotherapy and nitrogen mustard with 100% CR and 19 (48%) recurrent cases. In our group, 7 of 18 cases of PCFCL (38.9%) received irradiation and CHOP reaching 88.9% CR, and 9 of them (50%) showed a recurrence after 30.4 months. Rijlaarsdam et al.[12] reported 40 cases of PCFCL with 100% CR and 8 (20%) recurrent patients. Piccinno et al.[13] reported 104 cases of PCFCL with 100% CR and 76(73%) recurrences by 20.0 months. We had 8 cases of PCLBCL lower limb types, of which 4 got CHOP with 87.5% CR, 1 deteriorated and 2 died, one at 4 months and the other at 21 months.
The effectiveness of therapy for the PCLBCL lower-limb types was poor, and the survival rate was lower as well. Hembury et al.[14] reported 15 patients with PCLBCL, 2 cases (50%) of PCLBCL lower limb type developed a recurrence, and 2 others (18%) had a recurrence. A study by Goodlad et al.[15] reported 30 cases of PCLBCL lower-limb type, a 5-year survival rate (67%,diseased) and 100% (focus). In a report [16] of 48 cases of PCLBCL lower limb type, and 97 cases of PCFCL, there was a 5-year diseased survival rate of 52% and 94% respectively. The 5-year total survival rate of PCLBCL lower limb type was 67% in a study by Smith et al.[17]
Localized field irradiation[12,13,18] was applied in most reports on PCLBCL. In Eich’s study[9], 34 of 35 cases who underwent single radiotherapy (29 cases) or of those who received an operation (6 cases) had CR. With only one case after 16 Gy of radiotherapy was treatment stopped because of acute pneumonia. The localized field irradiation to CBCL subtypes was effective and the remission period prolonged. The extended field irradiation or combined with chemotherapy to the wide-spread CBCL gained satisfied remission rates. For the advanced stages of CBCL, chemotherapy was the treatment of choice. Adequate palliation could be achieved by using local field irradiation, and the patients showed tolerence to the therapy.
In Smith’s[17] study, 34 cases of CBCL underwent radiotherapy, 12 with multiple-field irradiation; 3 with electronic irradiation on whole skin area under mean dosage of 40 Gy; 4 with CHOP. All patients reached CR while 13 cases recurred 10 months later. We had the similar response using more CHOP which significantly prolonged the recurrence on-set. It was shown that CHOP could be greatly helpful to prolonge the disease-free survival period.
Radio-chemotherapy was introduced in 55 cases of PCFCL in a report by Rijlaarsdam et al.[12] The 2-year disease free survival rate of 40 cases with radiotherapy was 85% and the 5-year survival rate 89%. Two of 10 cases with CHOP treatment had a recurrence; 4 with COP treament recurred again. One case received CHOP plus bleomysin and methotrexate treatment. The 5-year survival rate was about 93%. Eleven cases undergoing CHOP in our study developed recurrent CBCL.
Some other reports[19] provided evidence of a effective result using rituximab against CD20 monoclonal antibodies in the treatment of PCBCL, especially for large-cell lower-limb types. Imai et al.[20] reported one case of PCFCCL, who received the monoclonal antibody combined with chemotherapy. The patient reached CR and without hair loss after treatment.
According to the new classification of the WHO/EORTC, PCMZL and PCFCL had sound efficacy in both treatment and surviving rate, but not the PCLBCL and its subtypes. Localized field irradiation for some CBCL was shown to be effective and was to produce a long period of remission. A satisfied remission rate could be obtained in wide-spread CBCL receiving extended field irradiation or radio-chemotherapy. For advanced stages of CBCL, chemotherapy was the choice treatment. Some reasonable palliation could be achieved by using local-field irradiation, with the patients showing satisfactory tolerence to the therapy. However, following radiotherapy deterioration can occur years later. Therefore, long-term careful follow-up after radiotherapy is needed.
- Received July 26, 2007.
- Accepted January 3, 2008.
- Copyright © 2008 by Tianjin Medical University Cancer Institute & Hospital and Springer
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