Atrophic dermatofibrosarcoma protuberans with diffuse eosinophilic infiltrate

Atrophic dermatofibrosarcoma protuberans (atrophic DFSP) is a variant of dermatofibrosarcoma protuberans (DFSP), and is clinically characterized by depressed lesions. We report a patient with a typical atrophic DFSP lesion with marked eosinophilic infiltration. The patient was a 55-year-old woman with a dark-red, depressed lesion in the epigastric region. Histopathological examination of the lesion showed proliferation of fibroblast-like cells in a storiform pattern in the dermis and subcutaneous tissue. Immunohistochemical staining of tumor cells was positive for CD34. The lesion was histopathologically typical of DFSP, but no elevated lesion was clinically observed. Thus, a diagnosis of atrophic DFSP was made. Moreover, this tumor tissue exhibited marked eosinophilic infiltration. To our knowledge, they are no reports of eosinophilic infiltration in DFSP tissue. Therefore, this seems to be an extremely rare case of DFSP.     

Dermatofibrosarcoma protuberans with atrophic appearance at early stage of the tumor

We report a case of dermatofibrosarcoma protuberans (DFSP) which had looked like an atrophic plaque on the face for 20 years and been diagnosed as morphea. At the late stage after subsequent development of a nodule, histopathological examinations including immunohistochemical stainings revealed the final diagnosis of DFSP. While DFSP is given typical "protuberant" morphology, our case indicates that DFSP sometimes appears as a non-protuberant lesion. Some reported variants of non-protuberant DFSP are suspected to be preceding features at the early stage of DFSP before the protuberant feature occurs. We should take this preprotuberant stage of DFSP into consideration of different diagnoses with non-protuberant lesions. Histopathological examination and immunohistochemical stainings are necessary for an accurate and early diagnosis of DFSP.     

Medallion-like dermal dendrocyte hamartoma: the main diagnostic pitfall is congenital atrophic dermatofibrosarcoma

Medallion-like dermal dendrocyte hamartoma is a newly described and rare clinical and pathological entity. This congenital, round, erythematous and atrophic lesion in the thoracic area is histologically characterized by a CD34+ dermal and hypodermal spindle-cell infiltration. We describe the clinical, histopathological, cytological and molecular features of three cases of dermal dendrocyte hamartoma. In all the cases, atrophic congenital dermatofibrosarcoma protuberans (DFSP) was the first histological diagnosis. In one case, wide surgery had been performed on the basis of the clinical and histological presentation. The histological pattern was similar in all the cases: epidermal atrophy and a spindle to ovoid cell proliferation in the dermis and in the subcutaneous fat. Immunochemical staining for CD34 and factor XIIIa was positive. Cytogenetic and molecular studies were performed; no chromosomal abnormality nor translocation t(17;22)(q22;q13) was observed. Fluorescence in situ hybridization analysis did not reveal the DFSP fusion gene COL1A1-PDGFB . We observed that the main diagnostic pitfall of medallion-like dermal dendrocyte hamartoma is atrophic congenital DFSP due to clinical and histological similarities. We emphasize that molecular studies to eliminate the t(17;22)(q22;q13) translocation of DFSP may provide determinant elements for diagnosis in order to avoid unnecessary mutilating surgery.     

Annular atrophic lichen planus

We describe a new case of annular atrophic lichen planus. Annular atrophic lichen planus is a rare variant of lichen planus that is clinically characterized by violaceous plaques of annular morphology with central atrophy and histopathologic findings of annular and atrophic lichen planus, respectively. There have been 4 cases of this entity reported previously.     

The atrophic variant of dermatofibrosarcoma protuberans in childhood: a report of six cases

Dermatofibrosarcoma protuberans (DFSP) is typically diagnosed during early adult life at a tumoral stage. It occurs only rarely in children. We report six childhood cases of DFSP which presented initially with the misleading clinical appearance of atrophic plaques, and we review over 140 cases of DFSP in childhood. As compared with adult forms, DFSP in children does not show distinctive features except for a tendency for acral localization. The diagnosis is difficult because of the slow course of the lesions, which present initially as apparently benign atrophic morphoeaor keloid-like plaques. We believe that DFSP in childhood is probably under-estimated, as a significant proportion of patients diagnosed as young adults had an onset several years earlier. Better knowledge of the initial appearance is important for making an early diagnosis and for an easier surgical treatment.     

Vascular congenital dermatofibrosarcoma protuberans: a new histological variant of dermatofibrosarcoma protuberans

We describe a case of congenital dermatofibrosarcoma protuberans (DFSP) that masqueraded as a vascular tumor both clinically and histologically. Based on the infiltrative growth pattern, presence of capillary-sized vessels, and spindle cell areas with slit-like vascular spaces and numerous thin-walled vessels at the periphery of the tumor, a kaposiform hemangioendothelioma was initially diagnosed. Strong diffuse CD34 positivity and the extension into the subcutaneous fat with a sieve-like effect prompted the fluorescence in situ hybridization analysis, which demonstrated a reciprocal t(17;22) translocation. According to our knowledge, this is the first report of a vascular histological variant of DFSP. This unique variant represents a potential pitfall for dermatopathologists and underlines the importance of cytogenetic diagnostics in unusual cases of DFSP.     

Congenital Myxoid and Pigmented Dermatofibrosarcoma Protuberans: A Case Report

Dermatofibrosarcoma protuberans (DFSP) is a low‐grade, mesenchymal, spindle cell tumor. In addition to the classical form characterized by a storiform pattern of tumor cells, pigmented (Bednar's tumor) and myxoid variants can be observed. Classical DFSP and Bednar's tumor are easily diagnosed. The myxoid variant represents a diagnostic challenge. Pigmented and myxoid variants are rare and thus far have never been reported in association in congenital DFSP. We came across a unique DFSP that was, at the same time, congenital, pigmented, and myxoid. The tumor was surgically excised with broad free margins and no recurrence. The differential diagnosis with other entities such as giant cell fibroblastoma, CD34‐positive plaque‐like dermal fibroma, superficial plaque‐like CD34 DFSP, and neurocristic hamartoma is discussed. The recognition of this hybrid variant of congenital DFSP is important to avoid under‐ or overtreatment.     

Pedunculated Nodules as a Variant of Dermatofibrosarcoma Protuberans

Dermatofibrosarcoma protuberans (DFSP) is a rare disease of dermal fibroblastic origin that accounts for less than 5% of all soft tissue sarcomas in adults. DFSP grows slowly and is an asymptomatic lesion at the initial diagnosis. Herein, we report a case of multiple pedunculated nodules as a variant of DFSP. A 47-year-old man presented with a 7-month history of multiple well-circumscribed, firm, pedunculated nodules on the inguinal area. Histopathologic examination results showed densely packed uniform spindle cells with a storiform and cartwheel pattern, and positivity for CD34. Wide excision and skin graft were performed and at the 6-month follow-up, there was no evidence of recurrence or metastasis.     

Dermatofibrosarcoma Protuberans in Childhood

Abstract: Dermatofibrosarcoma protuberans (DFSP) is a rare cutaneous tumor with a possibly misleading presentation. It is rare in childhood. We report an 18-month-old child with an acquired atrophic plaque of the left ankle. Based on clinical findings, initial proposed diagnoses included localized scleroderma and localized lipoatrophy. At the age of 5 years the plaque had increased in size and become a purplish color. Several firm nodules were seen at its periphery. A diagnosis of DFSP was proposed and confirmed histologically. A large surgical resection was performed. This case illustrates that DFSP may occur In childhood, during which initial diagnosis is generally more difficult than in adulthood.     

Giant cell fibroblastoma with dermatofibrosarcoma protuberans-like transformation

A giant cell fibroblasloma (GCF) presented in the subcutis of the breast in a 13-month-old girl which recurred 6 years later as a dermal and subcutaneous neoplasm whose patterns were in part GCF and spindle cells with a storiform configuration resembling dermatofibrosarcoma protuberans (DFSP). The issue remains unsettled as to whether GCF is the juvenile variant of DFSP, but an argument about this relationship is supported circumstantially by a case such as ours.     

局部扩大切除术治疗隆突性皮肤纤维肉瘤43例临床分析

对我院43例隆突性皮肤纤维肉瘤(dermatofibrosarcoma protuberance, DFSP)临床病理资料进行回顾性分析。43例患者中,男18例,女25例;发病前有肿瘤家族史者2例,有局部外伤史者5例,无明显诱因者36例;皮损表现为肿块40例,斑片1例,表皮萎缩1例,瘢痕样增生1例。组织病理示40例为经典型DFSP,2例为萎缩型,1例为黏液型。39例沿皮损边界扩切2~4 cm,其中随访28例（1例复发）;3例扩切＜2 cm,2例失访,1例转移;1例扩切＞4 cm,失访。     

Pigmented dermatofibrosarcoma protuberans with prominent meningothelial-like whorls

Dermatofibrosarcoma protuberans (DFSP) is a locally aggressive skin tumor. In addition to the conventional type, several morphologic variants have been described. Recognition of these uncommon variants will facilitate the diagnosis. We report herein a peculiar case of pigmented DFSP (Bednar tumor) with prominent meningothelial-like whorls, a distinctive pattern that has not been described previously in DFSP. The tumor occurred in a 40-year-old man who presented with a slowly growing mass on his left shoulder. The overall histological features were consistent with Bednar tumor. However, unexpected numerous meningothelial-like whorls were found in some areas of the tumor. Like the tumor cells in typical areas of Bednar tumor these meningothelial-like whorls were also positive for CD34 but negative for epithelial membrane antigen and S100 protein. The meningothelial-like whorls in Bednar tumor represent an eccentric arrangement of the tumor cells. We propose the term 'pigmented DFSP with prominent meningothelial-like whorls' to highlight the distinctive pattern of this novel DFSP variant.     

Myoid differentiation and EMA expression in fibrosarcomatous dermatofibrosarcoma protuberans

Dermatofibrosarcoma protuberans (DFSP) is a relatively unusual, locally aggressive cutaneous tumor of intermediate malignancy. Fibrosarcomatous DFSP (FS-DFSP), a rare variant of DFSP, has a higher tendency for recurrence and metastasis. Recently, a small number of cases of another variant of FSDFSB characterized by areas of myoid differentiation have been reported. We present here a 35 yearold female patient with myoid differentiation in FS-DFSP. The tumor on the left scapular region had slowly grown over six years. Examination revealed a domeshaped, firm, nontender, violaceous dermal nodule. Histologically, it was composed of a monotonous spindle cell population arranged predominantly in a storiform pattern and to a lesser extent in a fascicular fibrosarcomatous pattern with a parallel arrangement of the cells. Immunohistochemically, the tumor cells showed diffuse expression for vimentin and CD34. In the center of the tumor areas with frequent mitosis, hypercellular and negative reactive for CD34 were seen. In addition, approximately 10% of the cells were positive for epithelial membrane antigen. Myoid differentiation was found around the blood vessels. The myoid areas were positive for smooth muscle actin and negative for desmin. It is possible that the presence of hyperplastic myofibroblasts is a reactive phenomenon to the proliferation of tumor cells. We believe that this finding around blood vessels may be present in DFSP or FS-DFSP. However, when myoid areas, myoid fascicles and myoid nodules are seen in the stroma, it may be a new morphological variant of DFSP and/or FS-DFSP.     

萎缩型隆突性皮肤纤维肉瘤二例

报道2例萎缩型隆突性皮肤纤维肉瘤.患者分别为16岁和24岁的男性,临床最初均表现为界限清楚的萎缩型红斑,逐渐在红斑基础上发生结节.病程分别为6年和5年.皮损组织病理检查显示表皮萎缩或正常,肿瘤细胞在真皮浅层呈波浪状排列,平行于表皮;肿瘤细胞在真皮下部呈席纹状、车幅状、编织状排列,并可见肿瘤细胞侵犯皮下脂肪,分割脂肪细胞.肿瘤细胞表达波形蛋白、CD34,不表达CD68和S100蛋白.

Abstract：

Two cases of atrophic dermatofibrosarcoma protuberans (DFSP) are reported.The patients were a 16-year-old and a 24-year-old boy with a clinical course of 6 and 5 years respectively.The lesions began as well-marginated atrophic erythema,and subcutaneous nodules appeared gradually on the erythema.Histopathology showed atrophic or normal epidermis,wavy arrangement of tumor (spindle) cells in the superficial dermis which was aligned parallel to the epidermis,storiform arrangement of tumor cells in the lower dennis,and typical lacelike pattern of infiltration of subcutaneous fat tissue with tumor cells.Immunohistochemistry showed that the tumor cells stained positive for vimentin and CD34,but negative for S100 or CD 68.     

Acral dermatofibrosarcoma protuberans with myoid differentiation: A report of 2 cases

Dermatofibrosarcoma protuberans (DFSP) is a low‐grade mesenchymal neoplasm with a tendency for local recurrence; it is rarely metastatic. Dermatofibrosarcoma protuberans typically arises in the trunk and proximal extremities. However, it can arise in unusual sites such as the hands and feet. Several variants have been described, including DFSP with myoid differentiation, which is very uncommon. This variant tends to be observed more with the fibrosarcomatous variant, and it can be either in the form of myoid bundles and nodules with no vascular relationship or represent hyperplastic blood vessels within the tumor. Here, we report 2 cases of DFSP with myoid differentiation (ordinary and fibrosarcomatous) on the foot. To the best of our knowledge, these cases represent the first 2 cases of acral DFSP with myoid differentiation.     

隆突性皮肤纤维肉瘤50例临床病理分析

目的 探讨隆突性皮肤纤维肉瘤（DFSP）的诊断及鉴别诊断要点。 方法 回顾性分析中国医学科学院皮肤病研究所1998—2014年诊治的50例DFSP患者的临床表现、组织病理学、免疫组化特点及治疗和预后。 结果 50例患者发病年龄（29.5 ± 15.9）岁,平均病程9.6年。皮损位于躯干部33例,占66.0%;其次为四肢和头颈部。典型皮损表现为,萎缩性斑片/斑块13例,占26.0%;在此基础上多发性大小不一结节30例,占60.0%;正常皮肤上单发或多发结节7例,占14.0%。组织病理学特征由单一编席样或车辐状排列的梭形细胞组成,呈浸润性生长,肿瘤细胞表达CD34和波形蛋白。20例患者皮损切除后原位复发,复发率43.5％,未见远处转移和死亡患者。 结论 DFSP皮损形态变化多样,容易误诊,组织病理及免疫组化检查可确诊。DFSP切除后局部复发率高,可多次复发,但罕见淋巴结和远处转移。     

A Case of Myxoid Dermatofibrosarcoma Protuberans

Dermatofibrosarcoma protuberans (DFSP) is a slowly growing dermal spindle cell tumor and its myxoid variant, a rare type of DFSP, is characterized by extensive myxoid degeneration. We present the case of a 69-year-old woman with a multinodular reddish plaque on her trunk. Histopathologically, the tumor was located in the dermis and consisted of uniform spindle-shaped cells, showing strongly positive reaction for CD34, and negative for both S-100 and desmin. In addition to the typical storiform pattern, prominent myxoid stromal changes were demonstrated. Herein, we report an interesting case of myxoid DFSP, rarely reported in the dermatology literature.     

Linear chronic discoid lupus erythematosus following the lines of Blaschko

Linear chronic discoid lupus erythematosus is a rare manifestation of cutaneous lupus in which erythematous, atrophic, dyschromic lesions are located along the lines of Blaschko. This report describes the case of a 15-year old boy with a 2-year history of discoid, erythematous, hyper and hypopigmented lesions with central atrophy, situated along the lines of Blaschko on his right arm. Histopathology showed epidermal atrophy, hyperkeratosis, follicular plugging, thickening of the basement membrane zone, and superficial and deep chronic perivascular and periadnexal inflammatory infiltrate, with dermal mucin deposit, thus confirming diagnosis. A total of 14 cases have been described of this variant, the onset of which is often in childhood. There is no difference in incidence between genders. Lesions most commonly develop on the face. There have been no reports of any association with systemic disease.     

MALIGNANT ATROPHIC PAPULOSIS (DEGOS'' SYNDROME)

A 29-year-old Japanese man with malignant atrophic papulosis had a 1-year history of scattered, asymptomatic, whitish or skin-colored papules, as well as erythematous papules with central atrophy showing a porcelain-like appearance on the trunk and extremities. Histologic examination revealed lymphocyte-mediated necrotizing vasculitis with considerable deposition of mucin in the dermis. The wedge-shaped necrosis of the dermis was not detected. Based on a review of the literature we think that this case represents the histologic features of the early skin lesions of malignant atrophic papulosis.     

Generalized atrophic benign form of junctional epidermolysis bullosa

There are at least six variants of junctional epidermolysis bullosa (JEB). About 20 cases of the generalized atrophic benign variant of JEB (GABEB) have been previously reported. We present an additional case of GABEB, occurring in a 14-year-old girl. Generalized cutaneous blisters occurred since birth and healed without severe scarring or milia, but with slight atrophy. In addition, mucous membrane involvement and hair, nail and tooth abnormalities were found. Electron microscopic examination showed a cleavage within the lamina lucida and the presence of numerically and structurally abnormal hemidesmosomes.