Cutaneous Mantle Cell Lymphoma: A Clinicopathologic Review
Alejandro A Gru, Louis P Dehner, Maria Y Hurley, Lyndsey Brodell, Milan Anadkat, John L Frater. Washington University in St Louis, St. Louis, MO; Saint Louis University, St. Louis, MO
Background: Mantle cell lymphoma (MCL) is a moderately aggressive lymphoma believed to arise from the mantle zone or primary lymphoid follicles. Cutaneous MCL is exceedingly rare, and occurs usually as a dissemination of systemic MCL, and rarely as a primary cutaneous B-cell lymphoma. To date, only ∼16 cases have been described in the literature. We report a series of 6 cases of MCL involving the skin, and provide a clinicopathologic review of the histologic and clinical characteristics of these patients.
Design: Cases of cutaneous MCL were retrieved from our database after performing a search from 1990 till present, after an internal board review approval. Clinicopathologic characteristics were reviewed by 2 independent hematopathologists, with emphasis on the morphology, immunophenotype, and clinical parameters of this population.
Results: 6 cases were identified. Typically, they occurred in older individuals (mean age=70) and were more frequent in males (n=5, 83%). Half of them presented in the head and neck region as a mass, and the remainder in the trunk and extremities as cutaneous nodules. All patients had stage IV disease with bone marrow involvement. In 1 of the 6 cases (17%) the cutaneous lesions preceded the diagnosis of disseminated disease. In 2/6 cases (33%) involvement of the skin coincided with initial systemic involvement by MCL, and in 3/6 the skin was a site of recurrence following a diagnosis of systemic disease(mean interval=57 months). The mean time to recurrence was 45.4 months and the overall survival was 66.3 months. Histologically 4/6 cases (67%) had either pleomorphic (n=3) or blastoid (n=1) morphology. The mean number of mitosis per 10 high-power fields was 23. All cases showed a dermal-based infiltrate with extension into the subcutis, sparing of the epidermis, and associated perivascular and periadnexal accentuation. The immunophenotype included positive cyclin-D1 expression in all cases. Limited cytogenetic data showed additional trisomy 14 in one of the cases, in addition to the t(11;14) translocation.
Conclusions: Cutaneous MCL is an exceedingly rare secondary manifestation of systemic MCL. The cutaneous lesions can precede or subsequently develop after the diagnosis of systemic MCL. The more aggressive morphologic variants are more common in the skin, and the proliferation rates are typically high. Ours represents the largest single series of cases of MCL with emphasis on the clinicopathologic features of this entity.
Wednesday, March 6, 2013 9:30 AM
Poster Session V # 72, Wednesday Morning