Bednar Tumor (Pigmented Dermatofibrosarcoma Protuberans)

Añón-Requena, M.J.; Pico-Valimaña, M.; Muñoz-Arias, G.

doi:10.1016/j.adengl.2016.06.017

Cited by 1 publication

(3 citation statements)

References 7 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…Its genetic features, as well as the clinical presentation and histological characteristics are similar to those of ordinary dermatofibrosarcoma protuberans, but its histological hallmark is the presence of scattered dendritic cells that contain melanin [1,3,12]. In most of the cases, these lesions clinically present as slowly-growing plaques or subcutaneous nodules, as it was also documented in our patient's case [7,8]. The number of melanin-containing cells can vary and, as a result, black discoloration can be observed in lesions with many pigment laden cells, whereas other tumors only have microscopically detectable melanin [12].…”

Section: Discussionsupporting

confidence: 77%

“…Its clinical features are usually uncharacteristic. It usually presents as a dark-colored nodule or plaque than can easily be mistaken for other skin tumors such as melanoma, cellular blue nevus or neurofibroma [7,8]. It is defined by the presence of melanin-filled dendritic cells intermingled with blunt spindle cells in a storiform growth pattern [6,7].…”

Section: Introductionmentioning

confidence: 99%

“…It usually presents as a dark-colored nodule or plaque than can easily be mistaken for other skin tumors such as melanoma, cellular blue nevus or neurofibroma [7,8]. It is defined by the presence of melanin-filled dendritic cells intermingled with blunt spindle cells in a storiform growth pattern [6,7]. This unusual tumor was first described in 1957 by Bednar, who, at that time, considered it to be a type of "storiform neurofibroma" [9].…”

Section: Introductionmentioning

confidence: 99%

See 2 more Smart Citations

When an old pigmented lesion becomes serious: the problematic diagnosis of low-grade skin malignancies – Bednar tumor with fibrosarcomatous transformation

Dumitru

Ciongariu

Aliuș

et al. 2021

Romanian Journal of Orthopaedic Surgery and Traumatology

View full text Add to dashboard Cite

Bednar tumor is a rare cutaneous neoplasm, regarded as a variant of dermatofibrosarcoma protuberans with melanotic colonization and it usually affects young and middle-aged adults or children. This lesion is considered a low-grade malignant tumor, which can be associated with multiple local recurrences after surgical excision. Although a rare phenomenon, these lesions may undergo fibrosarcomatous transformation, which implies a poorer prognosis of the disease, as the tumor has a more locally aggressive behavior and patients might also develop distant metastases. We present the case of a 53-year-old female patient, with no significant medical history, who presented with a subcutaneous nodule on her upper back, for which a wide surgical excision was performed. The gross examination of the specimen showed a solitary protuberant grey-white nodule with a bluish shade and flecked with pigment. The microscopic examination revealed a malignant proliferation with a predominantly fascicular growth pattern, composed of spindle cells with highly pleomorphic nuclei and high mitotic rate, as well as the presence of dendritic cells with abundant melanin. Upon immunohistochemical analysis, the proliferation showed negative staining for CD 34 and AE 1/3, whereas the scattered dendritic cells stained positive with S100 protein. Ki 67 was positive in 15% of the tumor cells and the absence of p53 expression was noted. Thus, the diagnosis of Bednar tumor with fibrosarcomatous transformation was established. The aim of this paper was to gain further knowledge about the histopathological and immunohistochemical features, as well as about the treatment of Bednar tumor, especially considering its rarity.

show abstract