Primary Cutaneous Langerhans Cell Sarcoma: A Report of Five Cases and Review of the Literature
Matthew J Sagransky, April C Deng, Cynthia M Magro. Wake Forest University School of Medicine, Winston-Salem, NC; University of Massachusetts School of Medicine, Worcester, MA; Weill Medical College of Cornell University, New York, NY
Background: Langerhans cell sarcoma (LCS) is a rare but potentially life-threatening neoplastic condition. The diagnosis of LCS requires morphological and immunophenotypic characterization to distinguish it from other malignancies.
Design: Five cases of LCS were encountered in the consultative practices of two of the authors.
Results: Two cases had a prior history of acute myelogenous leukemia or chronic myelomonocytic leukemia with blast transformation. The remaining patients presented initially with localized disease. In those with underlying leukemia, the eruptions occurred after initiation of chemotherapy. In all cases, the biopsies showed a sheet-like growth of atypical epithelioid cells with features of Langherhans cells by virtue of an eccentrically disposed reniform nucleus although with marked pleomorphism. In two cases, the close apposition of the tumor to the epidermis along with concomitant ulceration was initially thought to be consistent with an invasive melanoma. Phenotypic studies revealed a profile supportive of LCS including positivity for CD4, CD1a and S100 in all and variable staining for langerin lysozyme, CD83, CD31, and CD68. A PubMed literature search was also conducted using the keywords Langerhans cell sarcoma, malignant histiocytosis X, and indeterminate cell sarcoma. To the best of our knowledge, only 31 cases (including the present cases) of Langerhans cell sarcoma have been reported in the English-Language literature. Five (16%) of these were associated with an underlying myeloproliferative disease. Of the 29 patients for whom follow-up data was available, 14 (48%) died from disease, 12 (41%) achieved and remained in complete remission, 1 (4%) achieved a partial remission, and 2 (7%) lived with their disease. Only 2 of the 8 (25%) reported cases with only cutaneous involvement died.
Conclusions: Cutaneous Langerhans cell sarcoma represents a terminally differentiated myeloid tumor with a variable clinical course. The tumors can be confused with acute leukemia cutis and nonhematopoietic malignancies such as melanoma. Immunohistochemistry is of cardinal importance in establishing the correct diagnosis. A malignancy of terminally differentiated myeloid cells could imply a common stem cell defect hence explaining the potential association with underlying myeloproliferative disease.
Tuesday, March 20, 2012 9:30 AM
Poster Session III # 99, Tuesday Morning