Primary Cutaneous Peripheral T-Cell Lymphoma, Not Otherwise Specified
Andy C. Hsi
Amy C. Musiek
András Schaffer
DEFINITION
Peripheral T-cell lymphoma, not otherwise specified (PTCL-NOS) includes a heterogeneous group of non-Hodgkin lymphomas that, by definition, do not correspond to any T-cell lymphoma entities defined by the World Health Organization (WHO) criteria.1 Cutaneous presentation of PTCL-NOS is usually secondary to lymphomatous spread of an extracutaneous disease. Primary cutaneous PTCL-NOS is designated when there is no evidence of extracutaneous involvement within 6 months of diagnosis.2
EPIDEMIOLOGY
PTCL-NOS is relatively common and represents ∼30% of PTCLs in Western countries, but primary cutaneous PTCL-NOS is exceedingly rare, accounting for only 2% of all primary cutaneous T- and B-cell lymphomas.3,4 Similar to other primary cutaneous T-cell lymphomas (CTCLs), primary cutaneous PTCL-NOS generally occurs in older adults and has a higher prevalence in males.4,5 Treatment often consists of multiagent chemotherapy, often with disappointing results.4
ETIOLOGY
Unknown.
CLINICAL PRESENTATION/PROGNOSIS/TREATMENT
Primary cutaneous PTCL-NOS presents as solitary or generalized nodules with no particular site predilections (Fig. 18-1).4,6 Primary cutaneous PTCL-NOS is associated with a poor prognosis, with a median overall survival of 2.4 years and a disease-specific 5-year survival of 16%.4,5,6 However, clinically indolent cases, including those with resolution after excision, have been reported.7,8,9,10,11
 FIGURE 18-1. Clinical presentations of primary cutaneous PTCL-NOS. A. Solitary nodule on the face with central ulceration. B. Large, erythematous nodule on the abdomen. |
HISTOLOGY
Skin biopsies show predominately medium-to-large pleomorphic mononuclear or immunoblastic T cells. Large neoplastic cells, by definition, represent at least 30% of the infiltrate. Central ulceration is a common feature. The neoplastic lymphocytes show nodular or diffuse dermal infiltrates, sometimes into the subcutaneous tissue. Mild epidermotropism may be evident.4,6 Angiocentricity and adnexal involvement are occasionally seen (Fig. 18-2A–B).12
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