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WHO Classification of Tumours
Primary cutaneous anaplastic large cell lymphoma
Skin


Definition

Primary cutaneous CD30-positive lymphoproliferative disorders (LPD) are the second most common group of CTCL, accounting for approximately 30% of CTCL. This group includes primary cutaneous anaplastic large lymphoma (C-ALCL), lymphomatoid papulosis (LyP), and borderline cases. It is now generally accepted that C-ALCL and LyP form a spectrum of disease, and that histologic criteria alone are often insufficient to differentiate between these two ends of this spectrum
Click to access Pubmed
Bekkenk MW, Geelen FA, van Voorst Vader PC, Heule F, Geerts ML, van Vloten WA, Meijer CJ, Willemze R (2000)
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.
Blood 95: 3653-61



. The clinical appearance and course are used as decisive criteria for the definite diagnosis and choice of treatment. The term “borderline case” refers to cases in which, despite careful clinicopathologic correlation, a definite distinction between C-ALCL and LyP cannot be made. Clinical examination during further follow-up will generally disclose whether the patient has C-ALCL or LyP
Click to access Pubmed
Bekkenk MW, Geelen FA, van Voorst Vader PC, Heule F, Geerts ML, van Vloten WA, Meijer CJ, Willemze R (2000)
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.
Blood 95: 3653-61



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C-ALCL is presented below, while LyP is discussed elsewhere.


> Definition of C-ALCL
Primary cutaneous anaplastic large cell lymphoma (C-ALCL) is a T-cell neoplasm composed of large cells with an anaplastic, pleomorphic or immunoblastic cytomorphology. The CD30 antigen is expressed by the majority (>75%) of tumour cells

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Swerdlow SH, Campo E, Harris NL, Jaffe ES, Pileri SA, Stein H, Thiele J, Vardiman JW (Eds.)
WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues
4th Edition
International Agency for Research on Cancer: Lyon 2008



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Patients with a history of mycosis fungoides (MF) should be excluded and the disease has to be distinguished from systemic ALCL with cutaneous involvement.