Primary cutaneous anaplastic large cell lymphoma with angioinvasive features and cytotoxic phenotype: a rare lymphoma variant within the spectrum of CD30+ lymphoproliferative disorders

Dermatology. 2013;227(4):346-52. doi: 10.1159/000355479. Epub 2013 Nov 12.


Background: Primary cutaneous anaplastic large cell lymphoma (PCALCL) presents with solitary or grouped exophytic tumors and cohesive infiltrates of large CD30+ T cells.

Objective: To report an angioinvasive variant of PCALCL.

Methods: Retrospective analysis of clinicopathological features of this variant.

Results: The group consisted of six patients (median age 46 years) with a solitary flat necrotic lesion preferentially located on the upper extremity. Histologically, there were angiocentric and angiodestructive infiltrates of medium-sized to large pleomorphic and anaplastic cells co-expressing CD30 and CD8. Five patients were treated with surgical excision and one patient with radiotherapy. A relapse was observed in one patient with spontaneous regression of the lesions suggesting a link to the recently described angioinvasive lymphomatoid papulosis (type E). All patients were alive without evidence of disease after a median follow-up of 31 months (range 15-96), indicating an excellent prognosis.

Conclusions: The angioinvasive variant of PCALCL is rare but distinctive and prone to misinterpretation as aggressive lymphoma due to its histological features.

MeSH terms

  • Adult
  • Aged
  • Blood Vessels / pathology*
  • CD8 Antigens / analysis
  • Female
  • Humans
  • Ki-1 Antigen / analysis
  • Lymphoma, Primary Cutaneous Anaplastic Large Cell / chemistry
  • Lymphoma, Primary Cutaneous Anaplastic Large Cell / pathology*
  • Lymphoma, Primary Cutaneous Anaplastic Large Cell / therapy
  • Male
  • Middle Aged
  • Neoplasm Invasiveness
  • Phenotype
  • Retrospective Studies
  • Skin Neoplasms / metabolism
  • Skin Neoplasms / pathology*
  • Skin Neoplasms / therapy


  • CD8 Antigens
  • Ki-1 Antigen