Primary cutaneous Langerhans cell histiocytosis showing malignant phenotype in an elderly woman: report of a fatal case.
BACKGROUND
Langerhans cell histiocytosis (LCH) is a proliferating disorder of Langerhans cells (LC) that are characterized by the presence of Birbeck granules. LCH has been considered to be a disease of childhood and there have been limited cases of adult LCH. We report here a fatal case of histiocytic tumor showing Langerhans cell phenotype, arising in the skin of a 74-year-old woman.


METHODS
In addition to routine histological and immunohistological sections, electron microscopic examination and human androgen receptor gene (HUMARA) assays were performed.


RESULTS
Histological examination revealed a dense dermal infiltrative proliferation of fairly large tumor cells with abundant ill-defined cytoplasms and oval or indented nuclei, in which numerous eosinophils were associated with the tumor nests. Tumor cells were positive with anti-S-100 and CD1a antibodies but negative with HMB-45 antibody or other epithelial or lymphocytic markers. Ultrastructural analysis showed typical Birbeck granules in the cytoplasm of the tumor cells. HUMARA assay of the tumor tissue revealed the nonrandom X inactivation pattern, indicating the clonal proliferation.


CONCLUSIONS
We diagnosed this tumor as Langerhans cell histiocytosis with a clonal neoplastic phenotype originated in the skin. Although she demonstrated no recurrence nor metastases for 6 months after surgical resection of primary skin lesion and subsequent radiation therapy, the tumor recurred and extended multisystemically, and she died of multiple organ failure 14 months after initial diagnosis. Therefore, we would like to emphasize this case as LC "sarcoma" or "malignant" LCH.
Itoh
H
H
Second Department of Pathology, Miyazaki Medical College, Kiyotake, Miyazaki, Japan. hitoh@post.miyazaki-med.ac.jp
Miyaguni
H
H
Kataoka
H
H
Akiyama
Y
Y
Tateyama
S
S
Marutsuka
K
K
Asada
Y
Y
Ogata
K
K
Koono
M
M
D000368:Aged; D001415:Back; D017809:Fatal Outcome; D005260:Female; D006225:Hand; D006646:Histiocytosis, Langerhans-Cell / Q000235:genetics* / Q000473:pathology; D006801:Humans; D010641:Phenotype; D012871:Skin Diseases / Q000235:genetics* / Q000473:pathology