Bednar Tumor Research Articles

A rare case of Bednar tumor

A rare case of Bednar tumor

Bednar’s tumor at right shoulder in an adult male: a case report of a rare entity

Introduction and importance: Bednar tumor, a rare variant of dermatofibrosarcoma protuberans is a tumor with low malignant potential. Owing to limited studies, the pathogenesis and risk factor of this tumor are not clearly understood. The tumor extends from the epidermis to the dermal layers and even affects the surrounding bone. Here, the authors present a case of a 48-year-old male with a Bednar tumor, which was successfully managed by wide local excision and flap repair. Case presentation: Our patient presented to the hospital after the reappearance of mass, 6 years after the excision of the previously misdiagnosed lipoma. Vitals and systematic examination were normal. Local examination revealed an oval-shaped mass with a glistening surface on the right shoulder. The patient underwent wide excision and skin flap surgery for the treatment, and the diagnosis was confirmed through histopathological examination and immunohistochemistry for the CD34 marker. Clinical discussion: Bednar tumor is an infrequent skin tumor linked to genetic anomalies and is one of the rare variants [(<0.1%) of skin tumors]. Diagnosis can be done by histopathological examination and CD34 marker positivity via immunohistochemistry. This tumor is mistaken for lipoma, leading to recurrent growth postexcision. The tumor demands a broader resection due to the high chances of reoccurrence. Although Mohs micrographic surgery is the ideal approach, its limited availability in resource-constrained settings prompts alternative strategies. Conclusion: This case highlights the challenges of diagnosis, the rarity of the condition, and the need for vigilant follow-up due to the tumor’s propensity for recurrence.

Dermatofibrosarcoma Protuberans at Unusual Sites: A Case Series

Dermatofibrosarcoma Protuberans (DFSP) is a rare, locally aggressive malignant subcutaneous soft tissue tumour that often goes undiagnosed due to its indolent nature and non protuberant growth. Recurrences after surgical excision are a common issue. While the trunk and extremities are commonly affected sites, rarer locations such as the genitalia and breast have been reported. The rarity of the lesion, its occasional unusual presentation sites, the indolent yet locally aggressive nature, and the diagnostic and therapeutic challenges are the distinctive features of this tumour. This study presents three unusual sites of DFSP: scrotum, vulva, and pubic region, one of which is an uncommon variant known as pigmented DFSP (Bednar tumour).

Pigmented dermatofibrosarcoma protuberans: A case report and review of literature

Dermatofibrosarcoma protuberans (DFSP) is a rare cutaneous sarcoma. Bednar tumor (BT) or pigmented DFSP is a rarely reported variant of it. The only differentiating feature between them is the presence of melanocytes on histopathological examination. We report a case of a 23-year-old female who presented with a recurrent lump in the right thigh that on histopathological evaluation was diagnosed as BT. Less than hundred cases of BT have been recorded to date, with only five cases originating from the thigh. Clinicopathological features of recurrent cases have been compared as local recurrence is less commonly reported.

A Case of Bednar Tumour in the Sacral Region- A Rare Pigmented Variant of Dermatofibrosarcoma Protuberans

Dermatofibrosarcoma Protuberans (DFSP) is a relatively rare soft tissue neoplasm of intermediate malignancy. Metastasis of this tumour seldom occurs. The DFSP is a locally aggressive tumour with a high recurrence rate. The DFSP accounts for less than 0.1% of all malignant neoplasms and approximately 1% of all soft tissue sarcomas. Bednar tumour is an uncommon variant of dermatofibrosarcoma protuberans. Also known as pigmented dermatofibrosarcoma protuberans, this tumour is a rare, aggressive, cutaneous tumour that constitutes 1-5% of all DFSPs of intermediate grade. These tumours can be easily mistaken for fibrosarcoma and melanoma. It is seen in adults with a predisposition to affect the shoulder region, and trunk. Authors hereby report a case of 50- years-old female presented with complaints of recurrent swelling in the lower back for a duration of 4 months. Pain aggravated on lying in the supine position, swelling was painless otherwise. Patient also had complaints of itching over the swelling with occasional blood stained discharge on self-inflicted trauma. Magnetic Resonance Imaging (MRI) scan and histopathological examination of excised tumour confirmed the diagnosis of bednar tumour. She underwent wide local excision with split skin grafting and was on follow-up for the last two years with no evidence of recurrence. Hence, adequate clearance and thorough and regular follow-up is essential to prevent recurrence.

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

Abstract 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.

Congenital giant Bednar tumor in a child: surgical challenges in a young infant: a case report

BackgroundBednar tumor is a pigmented variant of the uncommon dermatofibrosarcoma protuberans (DFSP). It is a disease of the middle aged and elderly and has been rarely described in the pediatric population.Case presentationWe report a rare case of a giant Bednar tumor present since birth, in a 2-year-old male child. It was present over the lower back and managed by surgical excision.ConclusionsBednar tumor is a rarity in children and is managed with aggressive wide local excision with margin of at least 1 cm. A close clinical follow-up is mandatory.

A Bednar tumor in a healthy woman.

A Bednar tumor (BT) is a soft tissue sarcoma that mimics neurofibroma and rarely occurs in the deeper layers of the skin. Local recurrence of BT is possible with incomplete resection, but the occurrence of distant metastasis is rare.

Bednar Tumor – a Case Report

Abstract Bednar tumor is a rare pigmented type of the dermatofibrosarcoma protuberans characterized histologically by the coexistence of two distinct cell populations, including spindle-shaped cells and melanin-containing dendritic cells. We report dermoscopic features of Bednar tumor observed in a 54-year-old female patient. The dermoscopy of Bednar tumor revealed a multicomponent pattern composed of a homogeneous blue-gray pigmentation with shiny white lines, structureless light-brown pigmented areas and a peripheral pigment network. The dermoscopic features observed in the present case are consistent with reported dermoscopic descriptions of Bednar tumor. Although dermoscopy may be suggestive of the diagnosis of Bednar tumor, pathohistological examination remains a gold standard for diagnosis.

Fine needle aspiration cytology of paediatric soft tissue tumours highlighting challenges in diagnosis of benign lesions and unusual malignant tumours.

The type of soft tissue lesions seen in children differs from that seen in adults. The role of fine needle aspiration (FNA) cytology in their diagnosis is not well documented. To study the cytopathological spectrum of paediatric soft tissue tumours to highlight uncommon benign and malignant lesions and the challenges in their diagnosis. A 3-year retrospective audit of all paediatric soft tissue FNA cytology cases from 2015 to 2017 was performed. Smears were reviewed along with cell block immunocytochemistry and follow-up histopathology of resected specimens wherever available. A total of 127 cases were reviewed, which included 72 benign and 55 malignant soft tissue tumours. Uncommon lesions described herein are myxoid fibrohistiocytic tumour, myxoma, lipoblastoma, Bednar tumour, malignant extra-renal rhabdoid tumour and desmoplastic small round cell tumour. Histopathology confirmation was available in 25 cases, out of which 16 cases were completely concordant. In eight cases, all benign diagnoses, histopathology provided more accurate subtyping than FNA. These included cases of lipoblastoma, myxoma and spindle cell haemangioma. FNA cytology of paediatric soft tissue tumours is accurate in classifying lesions as benign or malignant which helps in treatment planning. Immunocytochemistry performed on cell blocks is useful for subtyping malignant lesions.

Case of a Gourd－shaped Bednar Tumor

37歳，女性。約4年前に腰部の腫瘍に気づいた。徐々に大きくなり，201x年11月，腫瘍の部分をぶつけて出血したため近くの病院を受診し，当科を紹介された。初診時，腰部右側に手拳大で，だるま型の形状を呈する紅色腫瘤を認めた。腫瘤は皮膚と連続する母結節と，皮膚と連続せず母結節と連続する娘結節とから構成されていた。母結節の紅色調の部分と黒色斑を各々，4 mmパンチ生検した。どちらも真皮内にCD34陽性の紡錘形の腫瘍細胞が錯綜して増生していた。黒色斑部ではメラニン含有細胞が散在していた。Bednar腫瘍と診断し，側方は皮下に腫瘍を触れる部分から20 mm離して，深部は一部胸腰筋膜をつけて切除し，modified rhomboid flapで再建した。自検例の病理組織学的所見や過去の文献をもとに，Bednar腫瘍の発生起源について考察し報告する。

Microphthalmia-associated transcription factor (MiTF): Promiscuous staining patterns in fibrohistiocytic lesions is a potential pitfall

Microphthalmia-associated transcription factor (MiTF) is used as a marker of melanocytic differentiation. However, MiTF immunoexpression has also been observed in histiocytes, macrophages, smooth muscle cells and fibroblasts, which raise the concern of fibrohistiocytic (FH) lesions being misdiagnosed as melanoma based on MiTF immunoreactivity. MiTF has been known to be positive in FH tumors, but this is the first study evaluating ninety-three fibrohistiocytic neoplasms to understand and delineate the staining pattern of MiTF in these tumors. Ninety-three cases of FH, 30 cases of melanocytic lesions, and 20 miscellaneous cases were studied. The FH cases included benign fibrous histiocytoma (BFH, n = 29), angiofibroma (AF, n = 11), fibromatosis (FM, n = 14), keloid (KE, n = 10), atypical fibroxanthoma (AFX, n = 7), dermal scar (DS, n = 9), dermatofibrosarcoma protuberans (DFSP, n = 12), and pigmented DFSP (Bednar tumor, n = 1). Benign fibrous histiocytoma were sub-categorized into dermatofibroma (n = 15) and epithelioid fibrous histiocytoma (n = 14). The melanocytic lesions included desmoplastic melanoma (DM, n = 8), melanoma in-situ (MIS, n = 5), re-excision-free of melanoma (RFM, n = 10), blue nevus (BN, n = 5), and spitz nevus (SN, n = 3). The miscellaneous category included osteosarcoma (OS, n = 3), pigmented basal cell carcinoma (PBCC, n = 5), spindle cell squamous cell carcinoma (SCA, n = 2), and giant cell tumor of tendon sheath (GCTTS, n = 10). All BFH, AF, AFX, KE, and DS cases showed a positive MiTF staining of variable extent and intensity. MiTF positivity was observed in 86% (n = 12) cases of FM and 17% (n = 2) cases of DFSP. Amongst the miscellaneous category, all cases of PBCC and GCTTS and 50% (n = 1) cases of SCA were immunoreactive for MiTF. All melanocytic lesions were positive for MiTF. None of the OS and pigmented DFSP showed positive labeling. Because of the promiscuity of MiTF labeling, awareness of its pattern in FH proliferations may avoid potential pitfalls in the diagnosis of spindle cell lesions.

Subcutaneous dermatofibrosarcoma protuberans, a rare subtype with predilection for the head: A retrospective series of 18 cases

Dermatofibrosarcoma protuberans (DFSP) typically affects the dermis and subcutaneous tissue. The subcutaneous variant is rare. We sought to characterize the subcutaneous DFSP (SC-DFSP) variant and compare it with cutaneous DFSP (C-DFSP). This work was a retrospective study of DFSP treated in our institution. Of 124 cases of DFSP, 18 were SC-DFSP (14.5%). Except for the deep location, the pathologic and genetic features were indistinguishable from the C-DFSP variant. Histologically, of 18 SC-DFSP cases, 13 were classic DFSP, 3 fibrosarcomatous DFSP (FS-DFSP), 1 Bednar tumor, and 1 giant-cell fibroblastoma. All tumors expressed CD34 and the COL1A1-PDGFB fusion transcripts. In our series, higher proportions of SC-DFSP tumors (61%) than C-DFSP tumors (8.5%) were located on the head (P<.001). Of the 20 DFSP tumors on the head (16.1%), 11 were SC-DFSP and 9 were C-DFSP. In addition, half the SC-DFSP tumors affected muscle or periosteum, compared with a quarter of the C-DFSP tumors (P=.009). SC-DFSP needed a higher number of Mohs stages than did C-DFSP (P=.009). Median follow-up time was 63months, and 2 FS-DFSP tumors recurred (1 SC-DFSP, 1 C-DFSP). Limitations include the retrospective aspect of the study. Most DFSP tumors involving the head were subcutaneous and required more complex surgery. Dermatologists should be aware of this atypical presentation, especially in lesions involving the head.

Sclerosing Dermatofibrosarcoma Protuberans Shows Significant Overlap With Sclerotic Fibroma in Both Routine and Immunohistochemical Analysis: A Potential Diagnostic Pitfall.

Dermatofibrosarcoma protuberans (DFSP) is an uncommon, low-to-intermediate grade sarcoma with several histologic variants, including pigmented (Bednar tumor), sclerosing, myxoid, atrophic, and DFSP with fibrosarcomatous changes. Two patterns of sclerosis in DFSP can be observed, a sclerotic fibroma-like pattern and a morphea/lichen sclerosus-like pattern. Partial biopsies of sclerosing DFSPs with the sclerotic fibroma pattern can be misdiagnosed as sclerotic fibroma or other benign sclerosing tumors (eg, perineurioma, dermatofibroma). DFSPs from our tissue archives were screened for tumors with a sclerosing pattern, and then studied with epithelial membrane antigen (EMA), CD34, and elastic tissue staining to investigate whether such stains can differentiate sclerosing DFSP from sclerotic fibroma. Ten cases of sclerotic fibroma were similarly studied. Two of the 27 DFSPs were predominantly sclerosing and 5 additional DFSPs had a mixed histopathologic pattern including a sclerosing component. Immunohistochemically, all DFSPs with sclerosing (predominant or mixed) pattern were positive for CD34, and 5/7 were at least focally positive for EMA. Elastic tissue staining was reduced or absent in the sclerotic areas. All cases of sclerotic fibroma were either positive or focally positive for CD34, whereas EMA was focally positive in 5/10. Elastic tissue staining ranged from reduced to totally absent in the sclerotic fibromas. In conclusion, the similar histopathologic and immunophenotypic characteristics in sclerotic fibroma and sclerosing DFSP found in this analysis highlight the importance of obtaining clinical information and potentially additional excision for partial biopsies showing a sclerotic fibroma-like pattern.

Pigmented Dermatofibrosarcoma Protuberans (Bednar Tumor) masquerading clinically as malignant melanoma - A Case Report

Bednar tumor is a rare neoplasm of intermediate malignant potential, which account for less than 5% of dermatofibrosarcoma protuberans (DFSP).This tumor is a pigmented variant of DFSP. The diagnosis is commonly made in early to mid adult life except in cases with melanin containing cells. The case report presents a 19 years old female who presented with a painless slow-growing 3.5×3×1.2 cm blackish mass on the dorsal aspect of her right foot. Histopathological examination of the biopsy specimen revealed typical features of a Bednar tumor, the diagnosis was confirmed through immunohistochemical study. DOI: 10.21276/AABS.2017.1279

Epidemiology of Bednar tumors in the United States

Epidemiology of Bednar tumors in the United States

Bednar Tumor with Areas of Giant Cell Fibroblastoma

Bednar Tumor with Areas of Giant Cell Fibroblastoma

Bednar tumor of the hand

Bednar tumor of the hand

Table of Contents

Dermatofibrosarcoma protuberans (DFSP) is a rare superficial tumor characterized by high rates of local recurrence and low risk of metastasis. DFSP occurs most commonly on the trunk and proximal extremities, affects all races, and often develops between the second and fifth decade of life. The tumor grows slowly, typically over years. Histologically, several variants of DFSP have been described and should be well characterized to avoid misdiagnosis with other tumors. These include pigmented (Bednar tumor), myxoid, myoid, granular cell, sclerotic, atrophic DFSP, giant cell fibroblastoma, and DFSP with fibrosarcomatous areas. Of all these variants, only the DFSP with fibrosarcomatous areas is high grade, with a higher rate of local recurrence and distant metastasis. DFSP is genetically characterized by the t(17;22)(q22;q13), resulting in the fusion of alpha chain type 1 of collagen gene and platelet-derived growth factor beta gene. This translocation is present in 90% of DFSP and represents a very useful tool in the differential diagnosis of DFSP with other tumors with similar histology. The standard treatment is wide local excision with at least a 2-cm margin. However, local recurrence after apparently adequate surgical excision is well recognized. Mohs micrographic surgery would be the treatment of choice with a better cure rate and maximal conservation of tissue. When surgery is insufficient, clinical evidence has suggested that imatinib mesylate is a safe and effective treatment in DFSP, especially in cases of local advanced or metastatic disease. This article presents an overview of the state of the art in the clinicopathological management of this disease.

Dermatofibrosarcoma protuberans on the chest with a variety of clinical features masquerading as a keloid: is the disease really protuberant?

Dear Editor: Dermatofibrosarcoma protuberans (DFSP) is regarded a locally invasive, low-grade sarcoma. DFSP is typically characterized by its protuberant appearance. The DFSP in our patient had a clinically heterogeneous appearance, which is very rare, so the DFSP was initially misdiagnosed as a keloid. A 46-year-old male presented with a progressing skin lesion on his anterior chest. The erythema with itching had developed in his 20s, and the nodules appeared gradually. The nodules were thought to be keloids, so he received intralesional steroid injections for a few years at a dermatological clinic. However, that therapy had little effect, and he was referred to our department. He presented with an 8.0×10.0-cm shiny, atrophic skin-colored plaque, in which indurated erythema and painful keloid-like nodules were observed (Fig. 1). These nodules were also found on his chest area. The atrophic plaque (Fig. 2A), erythema (Fig. 2B), and nodule (Fig. 2C) were biopsied. Fig. 1 Clinical features of the 3 different lesions: shiny atrophic plaque (8.0×10.0 cm), indurated erythema, and painful, keloid-like red nodules (largest nodule: 2.0×1.5 cm) on the anterior chest. Fig. 2 Histopathological features of the lesions (HE a few proliferating spindle cells are seen (B). Erythematous plaque (C); storiform pattern is evident, but cellularity is low (D). Keloid-like nodule (E); typical ... Histologically, the 3 samples showed common basic structures. The epidermis consisted of atrophic and spindle-shaped cells, showing little atypism; the cells proliferated in a storiform pattern throughout the dermis and even extended to the lobular structures of fatty tissue. The tumor cells were CD34 positive. To identify the histological differences according to clinical status, we examined the cellularity and positive staining rates for Mib-1 in the atrophic plaque, erythema, and nodule. The cellularity count for each sample was performed in 10 randomly selected areas at high magnification (×200). The degree of cellularity in the nodular lesion (Fig. 2F) was higher than that in the other lesions (Fig. 2D, E). The mitotic index was similar among the 3 lesions. Later, the tumor was resected along the planned 3-cm margin. The overlying skin defect was reconstructed using a flap from the greater pectoral muscle. At the follow-up after 2 years, the patient was found to be free of the disease. DFSP is typically characterized by its protuberant appearance during early or middle adulthood. However, several cases of clinical and pathological unusual variants, such as Bednar's tumor and myxoid, sclerosing, fibrosarcomatous, atrophic, and nonprotuberant DFSP have been reported1. Our patient showed unique and various clinical features in a single location, including atrophic plaque, indurated erythema, and keloid-like nodules. What factors cause these different clinical appearances? The atrophic variant of DFSP is most commonly found on the trunk of women2. Furthermore, atrophic DFSP is more common among children and young adults3 than among the elderly. Martin et al.4 reported a nonprotuberant form of DFSP, and nearly half of their patients identified their early DFSP-related skin changes as patches, and the nonprotuberant stage lasted for 7.6 years. In those cases, the clinical appearance of the lesions resembled that of morphea, morpheaform basal cell carcinoma, atrophoderma, or angioma lesions. The COL1A1-PDGFB fusion gene is present in all the cases of DFSP subtypes. The different types of fusions between COL1A1 and PDGFB, however, are not related to the differences in clinical or histological features5; the factors contributing to these clinical differences have not yet been clarified. DFSP is not always protuberant, and awareness of this rare clinical presentation may assist in the early diagnosis of unusual variants of DFSP.