Paraneoplastic disseminated lentigines heralding aggressive Langerhans cell sarcoma
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LETTER TO THE EDITOR
Paraneoplastic disseminated lentigines heralding aggressive Langerhans cell sarcoma Wing Y. Au & Chris Lai & Nigel J. Trendell-Smith & Wai-Man Ng & Donna L. S. N. Chow
Received: 25 July 2012 / Accepted: 8 September 2012 / Published online: 20 September 2012 # The Author(s) 2012. This article is published with open access at Springerlink.com
Dear Editor, A 21-year-old man presented with a rapidly growing (9 cm), non-tender right flank mass with superficial excoriation and inflammation (Fig. 1a). The lesion was accompanied by rapid onset of disseminated brown papules, involving the torso, back, and limbs (Fig. 1b). Facial and axillary skins were spared, and there was no particular direction of spread. The skin patches did not coalesce or show pruritus or inflammation. There was also weight loss of 15 lb in 3 months. A positron emission tomogram scan (PET) showed FDG uptake in the right waist (SUV 5.8) and right groin (SUV 2.1) (Fig. 1c). Wide margin resection of the mass and lymph node excision was performed with clear margins. Histology of both specimens showed sheets and loose clusters of pleomorphic malignant cells infiltrating the W. Y. Au Blood-Med Hematology Center, Hong Kong, People’s Republic of China C. Lai Department of Pathology, Baptist Hospital, Hong Kong, People’s Republic of China N. J. Trendell-Smith Department of Pathology, Queen Mary Hospital, Hong Kong, People’s Republic of China W.-M. Ng Pedder Surgical Group, Hong Kong, People’s Republic of China D. L. S. N. Chow Hong Kong Pacific Cancer Centre, Hong Kong, People’s Republic of China W. Y. Au (*) 1108 Crawford House, 70 Queen’s Road Central, Hong Kong, People’s Republic of China e-mail: [email protected]
dermis and subcutis with frequent mitosis (Fig. 1d). In view of the anaplastic morphology and unusual presentation, extensive immunophenotyping was performed. The cells were positive for CD1a, S100, and Langerin stains. They were negative for melanocytic markers (HMB45, melan-A, MITF) EMA, cytokeratin (CK, Cam 5.2, 34betaE12), actin, desmin, CD31, CD34, c-kit, B and T markers (CD20, CD79a, CD3), CD68, CD30, CD56, CD21, MPO, and ALK1. Electron microscopy for Birbeck granules was not performed. The picture was compatible with Langerhans cell sarcoma (LCS). A separate skin biopsy of a pigmented lesion on the torso showed elongated rete ridges with increased basal melanin pigmentation within keratinocytes and the tips, with no increased Langerhans cells and no evidence of malignancy or dysplasia. The lesion was compatible with common lentigo. The patient was treated with consolidation radiotherapy to the primary sites, but there was no regression of the skin lentiges. In the World Health Organization classification, LCS is the malignant end of the spectrum of benign Langerhans cell histiocytosis (LCH) [1]. The typical LCH cell is replaced by pleomorphic tumor cells in the LCS, only identifiable by phenotype or ultrastructure. Initial unifocal subcutaneous involvement is not unusual. However progressive disease, especially with
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