Primary and secondary cutaneous CD30+lymphoproliferative disorders: a report from the Dutch Cutaneous Lymphoma Group on the long-term follow-up data of 219 patients and guidelines for diagnosis and treatment

Abstract

To evaluate our diagnostic and therapeutic guidelines, clinical and long-term follow-up data of 219 patients with primary or secondary cutaneous CD30+ lymphoproliferative disorders were evaluated. The study group included 118 patients with lymphomatoid papulosis (LyP; group 1), 79 patients with primary cutaneous CD30+ large T-cell lymphoma (LTCL; group 2), 11 patients with CD30+ LTCL and skin and regional lymph node involvement (group 3), and 11 patients with secondary cutaneous CD30+ LTCL (group 4). Patients with LyP often did not receive any specific treatment, whereas most patients with primary cutaneous CD30+ LTCL were treated with radiotherapy or excision. All patients with skin-limited disease from groups 1 and 2 who were treated with multiagent chemotherapy had 1 or more skin relapses. The calculated risk for systemic disease within 10 years of diagnosis was 4% for group 1, 16% for group 2, and 20% for group 3 (after initial therapy). Disease-related 5-year-survival rates were 100% (group 1), 96% (group 2), 91% (group 3), and 24% (group 4), respectively. The results confirm the favorable prognoses of these primary cutaneous CD30+ lymphoproliferative disorders and underscore that LyP and primary cutaneous CD30+ lymphomas are closely related conditions. They also indicate that CD30+ LTCL on the skin and in 1 draining lymph node station has a good prognosis similar to that for primary cutaneous CD30+ LTCL without concurrent lymph node involvement. Multiagent chemotherapy is only indicated for patients with full-blown or developing extracutaneous disease; it is never or rarely indicated for patients with skin-limited CD30+ lymphomas.

Evaluation of treatment results in multifocal primary cutaneous anaplastic large cell lymphoma: report of the Dutch Cutaneous Lymphoma Group

“…Previous studies reported that approximately 15-20% of the patients with C-ALCL have multifocal lesions. [2][3][4] However, from the 283 patients with C-ALCL that had been included in the Dutch Cutaneous Lymphoma Registry between 1985 and 2017, only 28 had multifocal skin lesions at first presentation (10%), and only nine of them had first presented with more than five affected skin sites (3%). Consistent with previous studies, patients with multifocal C-ALCL had a much higher relapse rate (67%), more often developed extracutaneous disease (33%) and had a lower 5-year DSS (78%) and 5-year OS (66%) compared with patients with solitary or localized C-ALCL (40% relapse rate: 10% extracutaneous relapse rate; 5year DSS 95% and 5-year OS 85%).…”

“…2 Approximately 15-20% of the patients with C-ALCL present with multifocal lesions involving multiple anatomical areas. [2][3][4] Although there is broad consensus that radiotherapy is the first choice of treatment in solitary or localized C-ALCL, there is uncertainty about the optimal treatment of patients with multifocal C-ALCL. 1,2,[5][6][7][8] Initial studies suggested that these patients should be treated with doxorubicin-based chemotherapy.…”

“…9 However, in more recent studies of our group, it was found that all patients treated with multiagent chemotherapy developed relapses during follow-up. 2 It was therefore concluded that multiagent chemotherapy should no longer be considered as first-choice therapy in patients with multifocal C-ALCL, but should be reserved for patients developing extracutaneous disease. 2 In that study, in which 17 of 79 patients presented with multifocal C-ALCL, we suggested that patients with multifocal C-ALCL can best be treated with radiotherapy in the case of few lesions or with low-dose methotrexate (MTX) in the case of more widespread disease.…”

“…2 In that study, in which 17 of 79 patients presented with multifocal C-ALCL, we suggested that patients with multifocal C-ALCL can best be treated with radiotherapy in the case of few lesions or with low-dose methotrexate (MTX) in the case of more widespread disease. 2 These guidelines were adopted by the International Society for Cutaneous Lymphomas and by the European Society for Medical Oncology, but have not been substantiated by further publications. 5,10 In particular, studies on MTX are scarce and restricted to a few case reports.…”

Abnormal activator protein 1 transcription factor expression in CD30-positive cutaneous large-cell lymphomas

“…3,4 Clinically, most C-ALCL patients present with limited disease characterized by solitary or localized skin nodules or tumours, while multicentric cutaneous disease is also seen in approximately 20% of cases, and lesions may show partial or complete spontaneous regression in over 25% of patients with frequent relapses. 3,5 There are clinicopathological similarities between C-ALCL and LyP; 6 however, LyP runs a benign clini-cal course with spontaneous disappearance of individual skin lesions in all cases and hence belongs to a self-resolving form of CTCL. 7 LyP typically occurs in early adulthood and presents with recurrent nodules and papules at distant sites, usually less than 1 cm in diameter, which become necrotic before resolving to form an atrophic scar.…”