Primary Mucosal CD30-Positive T-Cell Lymphoproliferative Disorders Have Clinicopathologic Features Similar to Primary Cutaneous Cases.
Andrew P Sciallis, Mark E Law, David J Inwards, William R Macon, Ahmet Dogan, Andrew L Feldman. Mayo Clinic, Rochester, MN
Background: Cutaneous CD30-positive T-cell lymphoproliferative disorders (CD30+ TLPDs) are classified as primary or secondary, but no such stratification exists for mucosal CD30+ TLPDs. In the skin, this distinction is critical since cutaneous and systemic anaplastic large cell lymphomas (ALCLs) have markedly different prognoses and treatments. Genetically, ALK translocations are associated with systemic ALCL, while IRF4 translocations are more common in primary cutaneous ALCL. We hypothesized that primary mucosal lesions have clinicopathologic features similar to cutaneous CD30+ TLPDs, and thus should not be classified as systemic ALCL.
Design: We studied CD30+ TLPDs involving mucosal sites in 13 patients (9M, 4F; mean age, 59 y). Sites were tongue (3), oral cavity (5), nasal cavity (3) and conjunctiva/orbit (3). Immunohistochemistry was performed for B- and T-antigens, CD30, and ALK. Fluorescence in situ hybridization was used to detect IRF4 translocations. Clinical data included lymphoma history, stage, treatment, and follow-up status.
Results: Histologically, all cases had large tumor cells, often resembling hallmark cells, with a varying inflammatory infiltrate. All cases were CD30 positive (>75%) and expressed at least one T-lineage antigen. One was ALK positive. Six patients had mucosal disease only, 2 had mucosal and cutaneous disease (1 ALCL, 1 mycosis fungoides), and 4 had systemic ALCL (3 ALK-negative, 1 ALK-positive). Stage and follow-up was unavailable in 1 case. IRF4 translocations were present in 2/10 cases tested (1 localized, 1 stage unknown). Systemic chemotherapy was given in 4/6 localized cases, 1/2 mucocutaneous cases, and 4/4 systemic cases. Follow-up ranged from 5 to 93 mos (median, 19 mos). Six of eight patients with localized or mucocutaneous disease were alive at last follow-up and 2 without disease died of unrelated causes. Two of four patients with systemic ALCL (both ALK-negative) died of disease.
Conclusions: Only the minority of CD30+ TLPDs involving mucosal sites represented systemic ALCL. Most were localized lesions, while 2 occurred with cutaneous TLPDs. IRF4 translocations were seen in 20%, similar to cutaneous ALCL. No systemic spread or death from disease was seen. Thus, primary mucosal CD30+ TLPDs have clinicopathologic features similar to cutaneous CD30+ TLPDs. Study of additional patients with longer follow-up is necessary to determine the natural history of these lesions. Meanwhile, we provisionally recommend use of the term primary mucosal CD30+ TLPD, reserving the designation ALK-negative ALCL for patients with documented systemic disease.
Monday, February 28, 2011 9:30 AM
Poster Session I Stowell-Orbison/Surgical Pathology/Autopsy Awards Poster Session # 171, Monday Morning