A case of dermatofibrosarcoma protuberans in the skin over the breast

Dermatofibrosarcoma protuberans (DFSP) is a slow-growing but locally aggressive, fibrous tumor that has a high rate of local recurrence after surgical resection. This tumor occurs most commonly in the trunk and proximal extremities. In this report we present a case of dermatofibrosarcoma protuberans in the skin over the breast which was re-excised after pathological diagnosis, considering cosmetic aspects. Only three other cases of dermatofibrosarcoma protuberans of the anterior chest wall have been encountered in our hospital, and all of these cases were male. The presence of this tumor in the skin over the breast appears to be rare in females. Careful complete resection is recommended for this type of tumor.     

Intracranial recurrence and distant metastasis of scalp dermatofibrosarcoma protuberans

Only few cases of scalp dermatofibrosarcoma protuberans with intracranial and distant metastasis have been reported. Here we report a case of scalp dermatofibrosarcoma protuberans with frequent local recurrence, intracranial invasion and with distant lung metastasis during 6 years of treatment. We would like to emphasize difficulties in surgical treatment of such invasive and locally recurrent tumors of scalp, and necessity to understand new molecular pathogenesis of dermatofibrosarcoma protuberans and potential treatment strategy with imatinib for patients with surgically untreatable disease. Close surveillance of patients with scalp dermatofibrosarcoma is necessary due recurrence nature of tumor.     

Arsenic exposure followed by the development of dermatofibrosarcoma protuberans

A case of documented significant arsenic exposure followed by the development of dermatofibrosarcoma protuberans is reported. Exposure to arsenic is associated with an increased chance of the subsequent development of a variety of neoplasms. It is possible that dermatofibrosarcoma protuberans may be one such tumor.     

肢体软组织肉瘤34例外科治疗体会

目的探讨肢体软组织肉瘤的诊断与手术疗效。方法回顾性分析外科手术治疗并经病理证实的34例肢体软组织肉瘤患者的诊治资料。结果术后病理:韧带样瘤型纤维瘤病4例,纤维肉瘤5例,浅表型纤维瘤病1例,隆突性皮肤纤维肉瘤4例,恶性纤维组织细胞瘤4例,脂肪肉瘤5例,平滑肌肉瘤5例,恶性周围神经鞘膜瘤2例,滑膜肉瘤2例,原始神经外胚层瘤2例。28例获术后随访1～4 a,4例(14.3%)复发,复发时间12～26个月,平均20个月。结论外科手术是肢体软组织肉瘤最重要的治疗手段,合理应用综合治疗和个体化治疗可提高切除率、降低复发率。     

Dermatofibrosarcome de Darrier et Ferrand: une localisation mammaire

Introduction

The dermatofibrosarcoma protuberans (DFP) is a rare skin tumor. It represents 0.1% of the malignant skin tumor. Surgery is its only treatment. The breast involvement is exceptional.

Observation

A 32-year-old woman was referred for a right breast nodule lasting for 5 years. The diagnosis of dermatofibrosarcoma protuberans (DFP) was confirmed by biopsy. The treatment was based on a large tumor excision.

Discussion

Breast localization of DFP remains rare and is difficult to treat. The breast is a part of women’s aesthetic identity. The excision margins remain a debatable subject.     

Dermatofibrosarcoma protuberans metastatic to a regional lymph node. Report of a case and review.

A case of dermatofibrosarcoma protuberans of the lower extremity with metastasis to an inguinal lymph node appearing 5 years after wide excision and skin grafting of the primary lesion is presented. The world literature is reviewed. This case is the 24th instance of metastasis and the 7th case of lymphatic metastasis. The other 17 cases were hematogenous metastases. The clinical and pathologic features of dermatofibrosarcoma protuberans are reviewed, and treatment is discussed, with the aim of emphasizing the need for long-term followup examination of the regional lymph nodes following wide and deep local excision.     

Dermatofibrosarcoma Protuberans and Small Bowel Adenocarcinoma: Coincidental Occurrence or Genetic Association?

A case of multiple malignancies in a 41 year old African American man is described. After surgical excision and radiotherapy of dermatofibrosarcoma protuberans, the patient developed small bowel adenocarcinoma. We propose a common etiology to these canceres via tumor suppressor gene p53.     

A ten-year-old boy with dermatofibrosarcoma protuberans of the face

A rare case of dermatofibrosarcoma protuberans on the face of a 10-year-old boy is presented. The patient's young age and the localization of the tumor led to limited excisions that resulted in two local recurrences. Primary wide excision with 2.5 cm of free margins is recommended to avoid local recurrences that are otherwise frequent.     

隆突性皮肤纤维肉瘤21例临床病理分析

目的:探讨隆突性皮肤纤维肉瘤（dermatofibrosarcoma protuberans,DFSP）的临床病理特点及鉴别诊断。方法:对21例DFSP病例标本进行回顾性分析,常规石蜡切片进行HE染色和免疫组化染色,分析其组织学特征及免疫表达。结果:镜下观察,肿瘤由梭形细胞构成,漩涡状排列,免疫组化示21例中所有病例Vimentin、CD34均为阳性,S-100、HMB45部分病例呈阳性表达。结论:DFSP是一种少见的低度恶性肿瘤,形态多种多样,免疫组化检查有助于与其他皮肤纤维组织肿瘤鉴别诊断。     

Dermatofibrosarcome de Darier et Ferrand : à propos d’un cas désespéré

We report this hopeless case in order to describe the bad prognosis factors may be life-threatening on short and medium term. The patient is aged 29 years, with a dermatofibrosarcoma protuberans of Darier and Ferrand of considerable size, with multiple recidivisms, and necrotic and ulcerative colitis. After transfusion and antibiotic therapy, the patient was released from the hospital without medical advice for financial reasons, before being taken over surgically. Early diagnosis and management without prepayement will improve the prognosis in hopeless cases of dermatofibrosarcoma of Darrier and Ferrand.     

Dermatofibrosarcoma protuberans

We report the case a 69-year-old woman with dermatofibrosarcoma protuberans, present, apparently, since birth. To our knowledge, this case represents the longest reported duration of a dermatofibrosarcoma protuberans.     

Dermatofibrosarcome de Darier-Ferrand: deux cas à localisation cervicofaciale

The authors illustrate the diagnostic and therapeutic difficulties encountered with Darier and Ferrand dermatofibrosarcoma from 2 cases report. Patients were 40 and 31 years old. In the first case, the tumour had been evolving for 4 years and occupied the submandibular region. In the second case, it had been evolving for 14 years and was localized in the parotido-submandibular region. The two patients benefitted from at least a biopsy or a partial surgical gesture before admission in our service. Surgical excision was wide. Both patients are alive after 4 and 2 years without recurrence and metastasis. Darier and Ferrand dermatofibrosarcoma is a cutaneous tumour of local malignancy, often diagnosed after several recurrences. The best treatment is surgery and consists in a wide and deep excision sacrificing healthy tissue.     

隆突性皮肤纤维肉瘤18例临床分析

目的　探讨隆突性皮肤纤维肉瘤 (DFSP)术后复发的因素。方法　回顾性分析 18例隆突性皮肤纤维肉瘤诊治过程及复发情况。结果　 7例隆突性皮肤纤维肉瘤行扩大根治切除术 ,复发率为 43 % ;11例行扩大切除加术后局部放疗 ,复发率为 18%。同时DFSP复发与组织学分级显著相关。结论　对隆突性皮肤纤维肉瘤认识不足 ,首次手术切除不彻底 ,扩大根治不规范 ,术后未行放疗及瘤细胞分化程度低是其复发的主要因素。     

11例正常大小卵巢癌综合征的临床及病理分析

目的探讨正常大小卵巢癌综合征的临床特点、诊断标准、有效的治疗方法及其预后。方法回顾性分析１９８４～１９９３年在我院治疗及病理复核证实的１１例正常大小卵巢癌综合征,其中７例为性腺外苗勒氏管肿瘤,也称卵巢外腹膜浆液性乳头状腺癌（ＥＰＳＰＣ）,２例卵巢浆液性腺癌,１例恶性间皮瘤,１例原发灶不明的转移性腺癌。上述无论何种类型,均宜最大限度切除肿瘤,术后残存肿瘤＜２ｃｍ,并辅以有效多程综合化疗。结果１例恶性间皮瘤存活９０个月,随访至１９９７年６月仍健在。１例ＥＰＳＰＣ已存活８７个月,随访至１９９７年１１月仍健在。９例已死亡患者平均生存１７．８个月,比文献报道存活时间长。结论对此综合征患者必须高度重视鉴别诊断,首选手术治疗,尽量切除癌灶（残存肿瘤＜２ｃｍ）,术后辅以有效化疗或放疗,以获得最佳生存和预后。     

Metastatic potential of dermatofibrosarcoma protuberans with fibrosarcomatous change

Dermatofibrosarcoma protuberans (DFSP) is a relatively uncommon fibrohistiocytic tumor that is locally aggressive. DFSP is associated with frequent local recurrences but rarely metastasizes. It has been suggested that fibrosarcomatous change within a dermatofibrosarcoma protuberans (DFSP-FS) is associated with increased risk of local recurrence and distant metastases. Metastases remain rare, however, and are typically preceded by multiple local recurrences. We report a rare case of a DFSP-FS metastasis to the head of the pancreas without any preceding local recurrence.     

Dermatofibrosarcoma protuberans: a surgical disease with a molecular savior

PURPOSE OF REVIEW: Dermatofibrosarcoma protuberans is a low-grade malignancy of the skin and subcutaneous tissues with low potential for the development of distant metastases. This tumor is characterized by infiltrative growth, and a chromosomal translocation involving the COL1A1 gene on chromosome 17 and the gene platelet-derived growth factor B gene on chromosome 22. This review will examine recent data confirming the central role of surgery in managing this disease and new findings for the application of molecularly targeted therapy in patients with unresectable disease. RECENT FINDINGS: The largest published series of dermatofibrosarcoma protuberans cases has reported that surgery can lead to excellent local control in dermatofibrosarcoma protuberans in over 95% of cases. In patients with metastatic disease, or with locally advanced disease not suitable for surgical excision, inhibition of platelet-derived growth factor receptors with imatinib (Gleevec) can lead to striking clinical results, including complete responses in some patients. SUMMARY: The identification of platelet-derived growth factor B as a near universal translocation partner in chromosomal rearrangements in dermatofibrosarcoma protuberans has led to the successful application of therapy targeted at platelet-derived growth factor receptors. Although wide surgical excision remains the standard of care, patients with locally advanced disease not suitable for wide surgical excision or with metastatic disease can be managed with the platelet-derived growth factor receptors inhibitor imatinib with high probability of response.     

Molecular targeting of platelet-derived growth factor B by imatinib mesylate in a patient with metastatic dermatofibrosarcoma protuberans.

PURPOSE: Dermatofibrosarcoma protuberans is caused by activation of the platelet-derived growth factor B (PDGFB) receptor, a transmembrane tyrosine kinase. We investigated the response of dermatofibrosarcoma protuberans to the tyrosine kinase inhibitor imatinib mesylate. PATIENTS AND METHODS: A patient with unresectable, metastatic dermatofibrosarcoma protuberans received imatinib mesylate (400 mg bid). Response to therapy was assessed by [18F]fluorodeoxyglucose (FDG) positron emission tomography, magnetic resonance imaging, and histopathologic and immunohistochemical evaluation. RESULTS: The patient was treated for 4 months with imatinib mesylate. The hypermetabolic uptake of FDG fell to background levels within 2 weeks of treatment, and the tumor volume shrank by over 75% during the 4 months of therapy, allowing for resection of the mass. There was no residual viable tumor in the resected specimen, indicating a complete histologic response to treatment with imatinib mesylate. CONCLUSION: Imatinib mesylate is highly active in dermatofibrosarcoma protuberans. The dramatic response seen in this patient demonstrates that inhibition of PDGFB receptor tyrosine kinase activity can significantly impact viability of at least one type of solid tumor.     

Giant cell fibroblastoma. A juvenile form of dermatofibrosarcoma protuberans

A clinicopathologic analysis of 28 cases of giant cell fibroblastoma (GCF), a rare mesenchymal tumor occurring predominantly in the first decade of life, is presented. This disease presented as a painless, slowly enlarging, subcutaneous mass. The tumor recurred locally in 47% of the patients; however, metastasis was not detected. On microscopic examination, GCF showed an unique combination of spindle cell patterns, pleomorphic and multinucleated giant cells, myxoid areas, and distinctive sinusoid-like spaces. This unrecognized histomorphologic picture led to a misdiagnosis of sarcoma in 40% of the cases. The histogenesis of this lesion remains uncertain; however, based on both clinical and morphologic similarities, it is proposed that GCF is a juvenile form of dermatofibrosarcoma protuberans (DFSP).     

Giant cell fibroblastoma in a 3-year-old boy

Giant cell fibroblastoma (GCF) is a rare soft tissue tumor most often discovered during the first two decades of life. We present a case of a 3- year- old boy with a history of a recurrent lesion in the knee, the tumor growth progressively and enlarged to 2.1 cm in the previous two years before diagnosis. It involved the subcutaneous tissue, had infiltrative borders and extended into the superficial dermis. The tumor was surgically excised with free margins. There was no evidence of local recurrence, and a metastatic workup was negative after 10 years of follow up. We review herein the clinicopathologic features, histogenesis, differential diagnosis and relationship to dermatofibrosarcoma protuberans (DFSP).     

Pleomorphic adenoma ("mixed" tumor) of human female breast mimicking carcinoma clinico-radiologically.

A case of pleomorphic adenoma ("mixed" tumor) of the breast is described Although rare, this is a distinctive primary neoplasm which can be mistaken for a malignant tumor both clinically and radiologically. Brief reviews of the pertinent literature and of the possible origin of the tumor are presented.