Primary cutaneous CD30-positive T-cell lymphoproliferative disorders, C-ALCL

Definition

A purely cutaneous T-cell lymphoma composed of CD30-positive anaplastic lymphoid cells. By definition, more that 75% of the tumour cells are CD30 positive.

Synonyms

Epidemiology

Accounts for 25% of primary T-cell lymphomas of skin. It is a disease of adults, predominantly of the elderly.

Clinical features

Disease is limited to the skin at the time of diagnosis. Most cases show a solitary skin lesion or localised disease; in 20% it is multicentric. Lesions show regression, as for lymphomatoid papulosis, but relapses are frequent. Extension to regional lymph nodes occurs in 10% of cases, usually in association with multicentric disease.

Macroscopic appearances

Skin lesions may be tumours, which may ulcerate, nodules or papules.

Histopathology

The cytomorphology resembles that of ALCL, involving both superficial and deep dermis, often with extension into the subcutis1. Multinucleate and Reed-Sternberg-like cells are common. Epidermotropism is uncommon. An abundant inflammatory component is more typical of lymphomatoid papulosis.

Variants1

Immunohistochemistry

 

CD1a

   

CD2

may be aberrantly negative1

CD3

may be aberrantly negative1

CD4

usually positive, occasional CD4-/CD8- cases are seen1

CD5

may be aberrantly negative1

CD7

 

CD8

usually negative1

CD11b

 

CD15

usually negative1

CD16

 

CD25

usually positive1

CD30

positive, in >75% of cells

CD56

 

CD57

 

CD71

usually positive1

HLA-DR

usually positive1

TdT

 

granzyme B

positive in 70% of cases

perforin

positive in 70% of cases

TIA-1

positive in 70% of cases

HECA-452

positive in 50% of cases

ALK

negative

EMA

almost always negative1

 

Cytogenetics

Differential diagnosis

Prognosis

The prognosis is good, with a 5 year survival of 90%. Spontaneous regression of skin lesions is associated with a better prognosis. Extracutaneous disease is associated with an inferior survival.

10-20% of cases of lymphomatoid papulosis show progression to lymphoma - variously mycosis fungoides, C-ALCL and Hodgkin lymphoma.

References

World Health Organization Classification of Tumours, Tumours of the haematopoietic and lymphoid tissues, IARC Press 2001.

1Liu, V. and McKee, P.H. Cutaneous T-cell lymphoproliferative disorders: approach for the surgical pathologist: recent advances and clarification of confused issues. Adv Anat Pathol 2002;9:79-100.

This page last revised 23.12.2002.

©SMUHT/PW Bishop