Paraganglioma-like dermal melanocytic tumor: A case report with particular features

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Paraganglioma-like dermal melanocytic tumor: A case report with particular features Article in International journal of clinical and experimental pathology · November 2009 Source: PubMed

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3 authors: Cimpean Anca Maria

Raluca Amalia Ceauşu

Victor Babes University of Medicine and Pharm…

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Int J Clin Exp Pathol 2010;3(2): 222-225 www.ijcep.com /IJCEP906003

Case Report Paraganglioma-like dermal melanocytic tumor: a case report with particular features Anca Maria Cimpean, Raluca Ceauşu, Marius Raica Department of Histology, “Victor Babeş” University of Medicine and Pharmacy Timişoara, Romania Received June 20, 2009, accepted October 31, 2009, available online November 20, 2009 Abstract: Childhood dermal tumors with melanocytic features is an unusual tumor that can create diagnostic confusion. Among them, paraganglioma-like melanocytic tumors- previous included in melanocytic tumors of uncertain malignant potential- has some particular histopathologic and immunohistochemical features. We describe a case of 13 years old girl with a paraganglioma-like dermal melanocytic tumor of the left leg. Key Words: Paraganglioma like dermal melanocytic lesion

Introduction Intradermal tumors with melanocytic features combined which lack of jonctional or intraepithelial components represent a special group of lesions with particular microscopic features and clinical behaviour. Primary dermal melanoma, solitary metastatic melanoma [1], solitary dermal melanoma [2], clear cell sarcoma [3] are several lesions included in differential diagnosis of paraganglioma like dermal melanoytic tumor (PDMT) which is very rare lesion with a benign behaviour compared with previous ones. Clinical and therapeutic management of PDMT are totally different from other melanocytic lesion. No PDMT was previous described in children. Many authors observed particular pattern of tumor blood vessels and low proliferative index of tumor cells in PDMT but no data are available concerning proliferative index of endothelial cells lining tumor vasculature neither characterization of other factors involved in PDMT angiogenesis. Case presentation A 13 years old girl was admitted one year ago with unique nodular non pigmented and painless lesion 2-2, 5 cm in size on the left leg. Medical history revealed no other family mem-

bers with similar lesions, slow tumor growth rate and no other associated clinical symptoms. Excisional biopsy was performed. Microscopically, we found a pseudo-nodular lesion restricted in the deep dermis without any contact with the overlying normal epidermis (Figure 1). Lymphocyte rich inflamatory infiltrate was found deep in the dermis, as a border between the proliferation and normal tissue. Tumor cells were large acidophilic oval or round in shape with distinct large nuclei and proeminent nucleoli mixed with spindle cells arranged in small nests, cords and sheets separated by fibrous septae. No mitosis or necrosis was seen. There was no cytoplasmic melanin pigment in tumor cells by conventional microscopy. Immunostaining for vimentin, S-100 protein and Melan A revealed a strong positive reaction indicating the melanocytic lineage for tumor cells (Figure 2b, c). Low proliferative index (