Primary cutaneous CD30+ anaplastic large-cell lymphoma with generalized skin involvement and involvement of one peripheral lymph node, successfully treated with low-dose oral etoposide

Abstract

Primary cutaneous CD30+ anaplastic large-cell lymphoma (PCALCL) in adults is rare, and the prognosis is generally excellent. Multifocal PCALCL tends to relapse after multiagent chemotherapy and is generally considered more prone to progress to extracutaneous involvement than is the localized disease. We report a 43-year-old woman with PCALCL who had generalized skin involvement accompanied by involvement of one peripheral draining lymph-node region. Although the disease relapsed after multiagent chemotherapy regimens, the disease was successfully treated with low-dose etoposide. We reviewed the previously reported cases of PCALCL treated with low-dose etoposide. We suggest that oral etoposide might be a useful effective treatment for treatment of relapsed multifocal PCALCL.

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

“…Moreover, relapses are common and continued or repeated maintenance treatment is often required. Apart from MTX, anecdotal case reports and small series suggest that etoposide, vinblastine and retinoids (bexarotene, acitretin and isotretinoin) alone, or in combination with interferon alpha may represent alternative effective and well‐tolerated options for refractory multifocal C‐ALCL …”

“…Apart from MTX, anecdotal case reports and small series suggest that etoposide, vinblastine and retinoids (bexarotene, acitretin and isotretinoin) alone, or in combination with interferon alpha may represent alternative effective and well-tolerated options for refractory multifocal C-ALCL. 7,8,[31][32][33][34][35] Recent studies report high response rates of BV in patients with primary cutaneous CD30-positive lymphoproliferations, including patients with relapsed and extracutaneous (multifocal) C-ALCL. 15,16,[18][19][20] A recent phase III trial compared the efficacy of BV in 16 patients with C-ALCL, including 12 patients with multifocal skin lesions and seven patients with extracutaneous disease, to MTX or bexarotene in 15 patients with C-ALCL including six with multifocal skin lesions and four with extracutaneous disease.…”

EORTC, ISCL, and USCLC consensus recommendations for the treatment of primary cutaneous CD30-positive lymphoproliferative disorders: lymphomatoid papulosis and primary cutaneous anaplastic large-cell lymphoma*

“…Low-dose MTX is widely used for LYP, but there are only anecdotal reports on its use in PCALCL. 38,46 Monoclonal antibodies SGN-30, a chimeric monoclonal antibody to CD30, has been used in 13 patients with PCALCL. 40,47,48 In an open-label multicenter phase 2 study, 6 of 11 (55%) patients achieved CR and 3 of 11 (27%) patients showed partial remission (PR).…”

Practical Management of CD30+ Lymphoproliferative Disorders

“…In a recent review, there were 3 PRs and 4 CRs in the 7 cALCL cases treated with oral etoposide, 50 mg/d. 59 Etoposide remains a well-tolerated therapeutic option for refractory, multifocal cALCL.…”