Primary Cutaneous Anaplastic Large Cell Lymphoma with Angioinvasive Features and Cytotoxic Phenotype: A Rare Lymphoma Variant within the Spectrum of CD30+ Lymphoproliferative Disorders

Abstract

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.

Disseminated CD8‐positive, CD30‐positive cutaneous lymphoproliferative eruption with overlapping features of mycosis fungoides and primary cutaneous anaplastic large cell lymphoma following remote solitary lesional presentation

“…The majority of primary cutaneous ALCLs are CD4 and CD30 positive and present as a rapidly growing solitary nodule or few nodules; however, multifocal and generalized variants have also been reported. In addition, there have been several reports of solitary CD8+ primary cutaneous ALCLs described in the literature (Table ) . These lesions appear to exhibit a similar behavior to that of CD4+ primary cutaneous ALCLs.…”

CD30+ clonal T‐cell lymphoid proliferation of the skin in a patient with hypereosinophilic syndrome

“…In summary, we have reported a hitherto undescribed unusual CD30+ clonal T‐cell lymphoproliferation in a patient with lymphocytic hypereosinophilic syndrome. With respect to its biologic nature as to neoplastic vs. reactive, we favor the latter possibility, as the condition is clearly distinct from all known cutaneous CD30 positive lymphoproliferative disorders, including their clinicopathologic subtypes lymphomatoid papulosis and primary cutaneous anaplastic large cell lymphoma and their mimics . Further observations of similar cases are warranted to elucidate the exact nature of this apparently distinctive lymphoproliferation in lymphocytic hypereosinophilic syndrome.…”

Chronic Reparative Changes in Medium-Sized Vessels in a Case of Primary Cutaneous Anaplastic Large-Cell Lymphoma With Angioinvasive Features and Cytotoxic Phenotype

“…Such changes are commonly observed at reparative stages of vasculitis involving medium-sized arteries. 9 We report a case of PCALCL with angioinvasive features and cytotoxic phenotype, as described by Kempf et al, 8 depicting reparative changes in medium-sized (muscular) vessels involved by the neoplasm. To our knowledge, this type of association has not been previously reported.…”

“…8 In the original series of 6 patients, all cases were EBV negative but expressed CD30 and CD8, a similar immunoprofile to lymphomatoid papulosis (LyP) type E, which also harbors angiocentrism/angiodestruction. 11 In contrast to LyP type E, however, lesions of PCALCL with angioinvasion and cytotoxic features did not regress spontaneously.…”