Primary cutaneous anaplastic large cell lymphoma
- Eric Jacobsen, MD
Eric Jacobsen, MD
- Assistant Professor of Medicine
- Harvard Medical School
Primary cutaneous T cell lymphoma (PCTL) refers to those cases of T cell lymphoma that present in the skin where there is no evidence of systemic (extracutaneous) disease at the time of diagnosis and after the completion of an initial staging evaluation.
The 2016 revision of the World Health Organization (WHO) classification of lymphoid neoplasms defines several main subtypes of PCTL, of which primary cutaneous CD30-positive T cell lymphoproliferative disorders are the second most common [1,2]. This is a spectrum of disorders that ranges from the more benign lymphomatoid papulosis (which almost always regresses spontaneously) to the neoplastic primary cutaneous anaplastic large cell lymphoma (PC-ALCL), and includes "borderline" cases with features of each.
This topic will focus on the diagnosis and management of PC-ALCL. The diagnosis and management of systemic ALCL and lymphomatoid papulosis are presented separately. (See "Clinical manifestations, pathologic features, and diagnosis of systemic anaplastic large cell lymphoma" and "Treatment of systemic anaplastic large cell lymphoma" and "Lymphomatoid papulosis".)
PC-ALCL is rare and the exact incidence is not known partially because it is difficult to distinguish cases of PC-ALCL within the larger group of CD30-positive cutaneous lymphoproliferative disorders (ie, lymphomatoid papulosis and borderline cases). In an analysis of the Surveillance, Epidemiology, and End Results (SEER) database, 157 cases of primary, localized CD30-positive cutaneous lymphoproliferative disorder were documented over a 30-year timespan . The median age at diagnosis was 61 years (range 5 to 98 years). There was a slight male predominance (58 percent) and most cases were diagnosed in Whites (87 percent). Other epidemiologic reports have had similar findings [4,5].
CLINICAL AND PATHOLOGIC FEATURES
Most patients with PC-ALCL present with solitary or grouped nodules growing over weeks to months and measuring up to several centimeters. These nodules typically ulcerate with time (picture 1). The lesions regress in up to 50 percent of cases, but rarely completely resolve . Multifocal disease is rare at the time of diagnosis (picture 2 and picture 3) and extracutaneous spread occurs in up to 13 percent of cases, at the time of relapse .
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- CLINICAL AND PATHOLOGIC FEATURES
- DIFFERENTIAL DIAGNOSIS
- Initial management
- Recurrent disease
- - Localized lesions
- - Multiple lesions
- Choice of agent
- Oral methotrexate
- Brentuximab vedotin
- Other agents
- - Widespread nodal or visceral involvement
- SUMMARY AND RECOMMENDATIONS