Primary cutaneous anaplastic large cell lymphoma with angioinvasive features and cytotoxic phenotype: a rare lymphoma variant within the spectrum of CD30+ lymphoproliferative disorders.
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.
Kempf und Pfaltz, Histologische Diagnostik, University Hospital, Zürich, Switzerland.
Werner
W
Kempf
Dmitry V
DV
Kazakov
Bruno E
BE
Paredes
Hubert R
HR
Laeng
Gabriele
G
Palmedo
Heinz
H
Kutzner
D000328:Adult; D000368:Aged; D001808:Blood Vessels / Q000473:pathology*; D016827:CD8 Antigens / Q000032:analysis; D005260:Female; D006801:Humans; D017730:Ki-1 Antigen / Q000032:analysis; D054446:Lymphoma, Primary Cutaneous Anaplastic Large Cell / Q000737:chemistry / Q000473:pathology* / Q000628:therapy; D008297:Male; D008875:Middle Aged; D009361:Neoplasm Invasiveness; D010641:Phenotype; D012189:Retrospective Studies; D012878:Skin Neoplasms / Q000378:metabolism / Q000473:pathology* / Q000628:therapy
D016827:CD8 Antigens; D017730:Ki-1 Antigen