Primary cutaneous T‐cell lymphoma of unspecified type with cytotoxic phenotype: Clinicopathological analysis of 27 patients

Abstract

The objective of our study was to investigate the clinicopathological features of the currently ill-defined subtype of primary cutaneous T-cell lymphoma of unspecified type (CTCLU) with a cytotoxic phenotype and no Epstein-Barr virus (EBV) association. A series of 27 patients with CTCLU (median age 49 years; range 25-87 years; 18 men) was reviewed. Performance status scores above 1 (7%), clinical stages above 2 (15%), B symptoms (26%), extracutaneous involvement (30%), and a fatal course within 1 year of diagnosis (19%) were observed infrequently. The International Prognostic Index was high or high to intermediate in 11%, and the Prognostic Index for Peripheral T-cell Lymphoma unspecified was above group 2 in 22%. Notably, the rates of spontaneous regression and T-cell receptor gene rearrangements by polymerase chain reaction analysis were seen in 26 and 17% of our cases, respectively. Histologically, 22 patients had subcutaneous involvement of whom eight showed a lethal clinical course, and five patients without subcutaneous involvement were all survivors. Immunophenotypical and morphological features allowed us to subclassify our cases according to the following four categories: (1) epidermotropic CD8 + T-cell lymphoma (n = 5); (2) cutaneous g/d T-cell lymphoma (n = 8); (3) cutaneous a/b pleomorphic T-cell lymphoma (n = 8); and (4) cutaneous medium/large pleomorphic T-cell lymphoma, not otherwise specified (n = 6). All four of these groups of lymphomas exhibited a relatively favorable clinical course compared to previous reports. However, epidermotropic CD8 + T-cell lymphoma appeared to be unique with a higher ratio (80%) of spontaneous regression, a lower ratio (40%) of subcutaneous involvement, and a more favorable clinical course than the other three subcategories. (Cancer Sci 2009; 100: 33-41)

Pathologic Diagnosis of Cutaneous Lymphomas

“…Histologically, in most cases diffuse or nodular dense infiltrates of mostly medium to large-sized lymphocytic tumor cells with significant nuclear pleomorphism are found. 65,66 Epidermotropism is found in a subset of cases. An angiocentric component can be present.…”

“…Variable T-cell phenotypes (CD4 1 or CD8 1 or CD4/CD8-double-negative and -double-positive) with or without expression of cytotoxic proteins are found. [66][67][68] By definition, most tumor cells do not express CD30. 1 An aberrant expression of CD20, but not additional B-cell markers, can be observed in a subset of cases.…”

A Challenging Cutaneous T-Cell Lymphoma

“…1 Only a few/Very few case series have been documented in the literature. 2,3 Despite the rarity of these tumors, clinicians as well as dermatopathologists and pathologists should be familiar with these rare CPTLs, especially because most of these lymphomas exhibit an unfavourable prognosis. 4…”

Peripheral T cell lymphoma in Asia

“…The prognosis of this disease is usually poor, which can be attributed to highly aggressive clinical lesions that usually result in a rapid metastatic spread, a lower response rate to chemotherapy, and a higher incidence of relapse [77]. Conversely, some cases with a favorable outcome have been reported [78,79], suggesting that CD8TCL has a heterogeneous clinical behavior. In this regard, it was suggested that patients presenting with a single or few nonaggressive lesions and who respond well to conventional CD4?…”

“…mycosis fungoides, lymphomatoid papulosis type D, and indolent CD8? lymphoproliferation of the ear or acral sites, should be differentiated based on pathological and clinical findings [77][78][79]. The prognosis of this disease is usually poor, which can be attributed to highly aggressive clinical lesions that usually result in a rapid metastatic spread, a lower response rate to chemotherapy, and a higher incidence of relapse [77].…”