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.     

A case of dermatofibrosarcoma protuberans in the skin over the breast of a young woman

A 19-year-old woman with dermatofibrosarcoma protuberans (DFSP) of the breast is described. She presented with a tumor of the left breast with redness and swelling. She had noticed a small tumor five years previously, but it had begun to grow rapidly three months previously. Mammography, ultrasonography and aspiration biopsy cytology demonstrated an apparently benign tumor of the breast preoperatively. Wide excision was performed to confirm the diagnosis. Histologically, the tumor was composed of a uniform population of fibroblasts in a distinct storiform pattern. DFSP was diagnosed in the skin over the breast. This lesion ordinarily originates from the dermis in the trunk and proximal extremities. It is important to diagnose a soft tissue lesion accurately when it occurs in the breast.     

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.     

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.     

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.     

The Role of Radiation Therapy in the Management of Dermatofibrosarcoma Protuberans

Purpose: To evaluate the outcome for dermatofibrosarcoma protuberans treated with conservation surgery and radiation therapy.Methods and Materials: A retrospective review was performed of 19 consecutive patients with pathologically confirmed dermatofibrosarcoma protuberans who received radiation as an adjuvant to surgical resection.Results: The patients ages ranged from 19–76 years (median, 40 years); 12 were men. Lesions were located on the trunk in 8, in the head and neck area in 7, and in an extremity in 4. Tumor size ranged from 1.2 to 15 cm (median, 4 cm). Ten patients had at least 1 prior recurrence following earlier resection. Two patients received preoperative radiation to 50 Gy in 5 weeks. Sixteen patients underwent resection followed by radiation (6 of these had positive resection margins). In another patient, the tumor regrew rapidly after resection and definitive radiation was delivered for gross disease. The 6 patients with positive microscopic margins received a median dose of 60 Gy, as did the 10 with negative margins. The 1 patient with gross disease received 65 Gy. At a median follow-up of 6 years, the only patient to develop local recurrence was treated with definitive radiotherapy for gross disease. Actuarial local control was 95% at 10 years.Conclusion: Dermatofibrosarcoma protuberans is a radioresponsive tumor and radiation to doses of 50–60 Gy should be considered as an adjuvant to resection if margins are positive. Combined conservation resection and postoperative radiation should also be considered for situations where adequate wide excision alone would result in major cosmetic or functional deficits.     

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 non-protuberans: description and report of five cases of a morpheaform variant of dermatofibrosarcoma

Five cases of dermatofibrosarcoma are reported. All showed features typical of dermatofibrosarcoma protuberans except that in four cases, and a portion of the fifth case, no protusion of the tumor was noted clinically despite the rather advanced stage of growth of the tumor. These lesions resembled morphea or a morpheaform basal cell carcinoma clinically but could be recognized as "dermatofibrosarcoma non-protuberans" by physicians who had observed a previous case.     

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.     

74例隆突型皮肤纤维肉瘤的治疗与预后分析

Objective： To study treatment and prognostic factor in 74 patients with dermatofibrosarcoma protuberans. Methods： From August 1990 to November 1999, 74 patients with dermatofibrosarcoma protuberans （DFSP） confirmed by pathology were treated in Cancer Hospital of Sun Yat-sen University. 72 cases were given wide excision and 2 cases were given local excision. All of 74 cases, 52 cases had surgical resection alone, and 22 cases had surgical resection combined with radiotherapy. Total dose of radiotherapy was 50-70 Gy. Results： The rate of recurrence was 36.1% for all patients. The 5-year recurrence-free survival （RFS） rate was 66%. The 5-year recurrence-free survival rates for resection alone and combined with radiotherapy were 58% and 90%, respectively （P=-0.0187）. The 5-year recurrence-free survival rates for positive microscopic margins and negative microscopic margins were 57% and 75%, respectively （P=0.0468）. Conclusion： Post-operation radiotherapy is an effective treatment to decrease the recurrence rate for the patients with positive microscope, or the patients without suitable surgical treatment.     

Dermatofibrosarcoma protuberans

In the current review, the authors set out to discuss the natural history and treatment of dermatofibrosarcoma protuberans (DFSP), a rare indolent cutaneous tumor. Approximately 10-15% of all DFSPs contain areas of fibrosarcoma (DFSP-FS), and such cases tend to exhibit more aggressive behavior. The optimal treatment for DFSP is resection with wide margins; the likelihood of local control associated with this procedure exceeds 90%. The probability of regional or distant metastases is /= 85% in such patients. Postoperative radiotherapy also is indicated in the rare event that a patient has unresectable macroscopic disease. Experience with the use of radiotherapy alone to treat macroscopic disease is limited.     

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。     

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.     

纤维肉瘤超微结构的研究

Ultrastructure of 9 cases of fibrosarcoma was observed and compared with that of fibrous connective tissue of 4 human embryos and 9 cases of spindle cell sarcoma, including 3 each of leiomyosarcoma, neurofibrosarcoma and dermatofibrosarcoma protuberans. Ultrastructurally, fibrosarcoma consisted of well-differentiated fibroblast-like cells, poorly differentiated fibroblast-like cells (embryonic fibroblast-like cells), myofibroblast-like cells and primitive mesenchymal cells. It is suggested that fibrosarcoma may arise from primitive mesenchymal cells which are capable of differentiating into fibroblast and myofibroblast. There were two special cases of fibrosarcoma in this series. One was a congenital fibrosarcoma with ultrastructure resembling adult and the other was a sarcoma of myofibroblast. Diagnosis and differential diagnosis between fibrosarcoma and leiomyosarcoma, neurofibrosarcoma, dermatofibrosarcoma protuberans are discussed.     

Dermatofibrosarcoma protuberans treated by micrographic surgery

Dermatofibrosarcoma protuberans is an uncommon cutaneous tumour which rarely metastasises. However, local recurrence following apparently adequate surgical excision is well recognised, presumably as a result of sub-clinical contiguous growth, for which micrographically controlled excision would be a logical treatment. A retrospective study of all patients treated by micrographic surgery, from April 1995-March 2000, at a tertiary skin oncology centre. Twenty-one patients (11 males), age 14 to 71 years with dermatofibrosarcoma protuberans on the trunk (10 patients), groin (four), head and neck (four), and limbs (three) were treated. In 15 patients one micrographic layer cleared the tumour, and four were cleared with two layers. For one patient the second stage was completed by conventional excision guided by positive margins. Another patient with a multiply recurrent perineal dermatofibrosarcoma protuberans, not cleared in one area after two layers, died from a pulmonary embolus before total clearance could be achieved. There was no correlation between tumour size and lateral excision margin. No recurrence was observed during the follow-up, from 21 to 80 months, median 47 months. The study provides further support for micrographic surgery as the treatment of choice for dermatofibrosarcoma protuberans.     

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.     

Soft tissue sarcoma in the head and neck--analysis of 87 patients

From 1964 to 1984, 87 patients with soft tissue sarcoma (excluding lymphreticulum system sarcoma) in the head and neck were treated in our hospital. All were proved by pathology. The incidence rate of fibrosarcoma was the highest (36.8%), hemangiosarcoma the lowest (5.8%). There was no liposarcoma or synovial sarcoma in this group. The most frequently involved site was the area near the nasal cavity and maxillary sinus for fibrosarcoma (66%), the base of tongue for hemangiosarcoma (60%), scalp for dermatofibrosarcoma protuberans (54%), parapharyngeal space and soft tissue of the neck for neurogenic sarcoma (45%). Metastasis rate to the lymph nodes was 16-20% for rhabdomyosarcoma, hemangiosarcoma and malignant fibrohistiocyte tumor. No distant metastasis was found in dermatofibrosarcoma protuberans and malignant fibrohistiocyte tumor. Distant metastasis rate was 10-20% for the other types. Local recurrence rate was 9.1% for dermatofibrosarcoma protuberans, 88.9% for malignant fibrohistiocyte tumor, 30-66.7% for the other sarcomas. The 5- and 10-year survival rates were 20% and 0%, 37.5% and 0%, 91.6% and 91.6% for rhabdomyosarcoma, malignant fibrohistiocyte tumor and dermatofibrosarcoma protuberans. There were differences in clinical behavior for soft tissue sarcomas in the head, neck and in the trunk. Highly malignant sarcomas, such as liposarcoma, synovial sarcoma and rhabdomyosarcoma were rare in the head and neck but the low malignant sarcomas in the head and neck were 3-10 times as common as those in the trunk. Distant metastasis rate of the fibrosarcoma was 20% in head and neck and 6.7%in trunk, the 5- and 10-year survival rates were 36% and 77.9%, 25% and 73.6%, respectively.(ABSTRACT TRUNCATED AT 250 WORDS)     

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。     

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.     

Dermatofibrosarcome protuberans récidivant du sein: à propos d’un cas

The dermatofibrosarcoma protuberans (DFSP) is a sarcoma of low rank developing slowly from the fibroblasts of the dermis. The main characteristic of this tumour is its big potential for local recurrence. We report the case of a patient aged 44 presenting a DFSP of the breast that relapsed on two occasions. The remaining extension was negative. She benefited from a large timorous excision. The mammary localization of a DFSP is rare. The diagnosis is difficult. The therapeutic objective of DFSP is to reach a possible most complete surgical resection; the conventional surgical excision must let healthy margins (of 3 to 5 cm) with the inconvenience of the substance loss. The technique of Moh, verifying the limits of excision in preoperative, can palliate this inconvenience. The risk of recurrence is low when the resection margins are healthy. Radiotherapy as adjuvant treatment is very useful when the margins are not sufficient. The surveillance must be prolonged.