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Giant cell fibroblastoma (GCF) is a rare neoplasm occurring mostly in children. It has a tendency to recur locally after treatment. We report a new case noticed in an five year-old child who developed recurrence a first time three years later with the picture of GCF and at a second time with the picture of dermatofibrosarcoma protuberans 17 months later.
We report an unusual case of composite giant cell fibroblastoma-dermatofibrosarcoma protuberans (DFSP) that, in its second recurrence, contained a pattern of Bednár tumor (BT) and giant cell fibroblastoma (GCF). The recurrent tumor showed extreme myxoid change with creation of mucoid lakes, which mimicked a pattern of myxoid liposarcoma. One area in the recurrent lesion contained amputation neuroma overgrown with neoplastic spindle cells, which simulated a nerve sheath neoplasm. This case demonstrates common histogenesis of GCF, DFSP and BT, and it shows how broad morphological spectrum can be produced by a composite tumor, especially when the tumor includes unconventional growth pattern or additional non-neoplastic lesion.
Dermatofibrosarcoma protuberans has a high rate of recurrence, presumably as a consequence of persistent residual tumour. This is probably the main reason why repeated surgical treatment is frequently required. In the article we present our experience of surgical treatment of such malignancy on the neck and shoulder in the elder male after ten years follow-up period. The case is interesting because we started with aggressive treatment relatively late in the unfavourable, fully-developed stage of the disease, with multiple satellite nodules spreading and signs of partial myxoid degeneration of the largest tumour mass itself. Despite initial wide excision, five local recurrences--fortunately without regional or distant metastases--were observed during the first eight years. The recurrences appeared mostly in the region of greatest tumour degeneration and infiltration and where the excision margins were less than 2 cm apart from the visible tumour mass. A sufficiently wide excision, long-term follow-up and good co-operation between surgeon and experienced pathologist are crucial for the strategy of treatment and the prognosis of this unusual malignancy.
A 46-year-old Saudi male underwent excision of right anterior upper chest wall lesions. On histopathological examination, the lesion was thought to be dermatofibroma. Nine years later, he presented with a recurrence at the site of excision. Review of previous studies and histopathological examination of the recurrent lesion was suggestive of dermatofibrosarcoma protuberans. He underwent wide excision with satisfactory results.
Taking the starting point from their own experience and looking at the most recent literature, the Authors report the prospects about the surgical treatment of dermatofibrosarcoma protuberans.
OBJECTIVE: To emphasize the importance of correct and standardized surgical treatment on dermatofibrosarcoma protuberans (DFSP), and discuss the suitable synthesized therapy on it. METHODS: 163 cases of DFSP, which were treated between January 1985 and September 2002,were submitted to a retrospective study. RESULTS: Among the 163 cases, 150 (92.0%) were treated with local excision as benign tumors before accepted to Cancer Hospital, Fudan University. 69 cases (46.0%) were approved by pathological examination to have tumor remnants after they were treated with wide excision, and 49 (71.0%) of them couldn't be found to have any tumor remnants by physical examination or B-ultrasonic examination before that operation. It was easy for the tumor to recur after excision, especially the local excision. The recurrent rate after it was 45.1%, which was much higher than the one after wide excision (5.6%). Among the 142 cases which wide excision were performed, 99 ones had excision margins >/= 3 cm and 5 of them (5.1%) developed local recurrence while 36 ones had excision margins 1 approximately 2 cm and 3 of them (8.3%) developed local failure. 46 cases (32.4%) were given skin graft, 11 cases were given flap, and 1 case had dacron mending in skin defection area. The main complications after these operations were necrosis of the skin flap (20 cases) and infection of the wound (6 cases). They could all be cured in 2 months. 17 cases were given complimentary radiotherapy with the dose range from 3275 cGy to 7000 cGy because of their recurrences for times or positive resection margins after wide excision. Only one case had wet molting after radiotherapy and 2 developed local recurrence. Among all the 163 cases, only 2 (1.2%) were dead, and 1 of them was died of metastasis of lung and liver. 2 cases got lymph node metastasis, then were given surgical treatment and still alive now. 13 cases (8.0%) were DFSP-FS with their malignancies increased. 11 of them were the recurrent ones after local excision or wide excision. CONCLUSIONS: In order to avoid misdiagnosis, it is necessary for the clinician to know much about DFSP. Once the tumor was diagnosed of DFSP after local excision, it is necessary to take wide excision. Because DFSP is a malignancy of a high recurrent rate after local excision, standardized wide excision is the key in reducing local failure. Adjuvant radiotherapy is an effective treatment for the patients with positive resection margin or the patients don't suit for surgical treatment. The DFSP-FS need to use more energetic treatment in curing it.
Dermatofibrosarcoma protuberans (DFSP) is an uncommon cutaneous malignancy. Unusual presentations described as atrophic have been documented. A case of DFSP with both clinical and histologic atrophy is presented, and all cases purporting atrophy with this tumor are reviewed. Meaningful trends are extracted from this data. In addition, the imprecise use of the term atrophic in regard to DFSP is clarified. We maintain that the variant of atrophic DFSP that mimics atrophoderma or anetoderma, as in this case, is the rarest variant of atrophic DFSP. Atrophic DFSP should be in the differential for depressed lesions on the trunks of women or on the lower extremities of children.
The Authors reported a case of dermatofibrosarcoma protuberans of the urinary bladder in a 17-year old boy. The biology of this unusual urinary tumor is discussed.
A case of dermatofibrosarcoma protuberans of the trunk involving the underlying soft tissue and muscle fascia, in early stage of development in 27 year old patient, operated by taking safety margin of 3 cm and the underlying muscle fascia, is being reported. Modality of treatment has been discussed.
Dermatofibrosarcoma protuberans (DFSP) usually occurs in adults, however it can also occur in infancy and childhood. Diagnosis of DFSP in children is quite difficult given the rarity of the tumor and its variegated appearance. The behavior of this neoplasm is of borderline or intermediate malignancy with a high rate of recurrence but limited metastatic potential. We present the case of an eleven-year old boy who presented with a slightly raised, not tender lesion on his right shoulder. It had appeared two years before as a red-brown plaque and since then it had gradually grown to a nodule. An excisional biopsy was performed, and the diagnosis of DFSP was made based on the histological and immunohistochemical findings.
Dermatofibrosarcoma protuberans (DFSP) is a rare, slowly growing, malignant dermal tumor first described by Darier and Ferrand in 1924. It typically occurs in individuals aged 20 to 40 years and frequently arises on the trunk. Although the cellular origin of DFSP is not entirely clear, dermal stem cells or undifferentiated mesenchymal cells with fibroblastic, muscular, and neuroectodermal features have been implicated. DFSP is a locally aggressive tumor with a high recurrence rate, largely attributed to the spread of tumor by direct local invasion through peripheral and deeply penetrating neoplastic cells into subcutaneous tissue and muscle. As such, there is growing evidence that Mohs micrographic surgery is the treatment of choice. Although reports of chromosomal aberrations and gene mutations have been described in the literature, excessive sun exposure is not regarded as a risk factor for the development or progression of DFSP. We describe a case of DFSP in a patient with a history of prolonged tanning bed use.
Dermatofibrosarcoma protuberans (DFSP) is a rare mesenchyma skin tumor. It is characterized by a slow growth with a high rate of recurrence but limited potential for metastasis. We report the result of a retrospective study of 18 cases of DFSP collected over 16 years in the dermatology department of La Rabta Hospital. The mean age was 32 years with a sex ratio of 1.57. The site of predilection was the trunk (77.5%). Histological features were typical in all cases with positivity for CD34 in 14 cases. Treatment was surgical in 13 cases. DFSP is a tumor of intermediate malignancy, which can have aggressive course. This tumor is best treated with surgery in which large excision are necessary to reduce the risk of recurrence.
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.
Ring chromosomes are found in most dermatofibrosarcoma protuberans (DFSPs), and recent reports demonstrate that portions of the DFSP ring chromosomes derive from chromosome 17. In this study we characterized ring chromosomes in three DFSPs using a combined approach of karyotyping, chromosome painting, and comparative genomic hybridization. Chromosome painting demonstrated that the ring chromosomes in each DFSP were composed of discontinuous, interwoven sequences from chromosomes 17 and 22. Amplification of chromosomes 17 and 22 sequences was confirmed in each of these cases by comparative genomic hybridization, and over-representation of chromosomes 17 and 22 sequences was also demonstrated by comparative genomic hybridization in 1 of 2 cytogenetically unremarkable DFSPs. We conclude that amplification of chromosomes 17 and 22 sequences, in ring form, is a characteristic aberration in DFSP.
OBJECTIVE: On rare occasion, fibrosarcomatous change may arise in a dermatofibrosarcoma protuberans (DFSP), and this alteration is of prognostic significance. Histologically, this change may be subtle and difficult to recognize. The goal of this study was to evaluate the pattern of CD34 immunoreactivity in DFSP with fibrosarcomatous areas (DFSP-FS). DESIGN: Of 100 cases of DFSP identified at a large metropolitan hospital, 7 were found to have fibrosarcomatous change. Thirty cases of typical DFSP and seven cases of DFSP-FS were stained with an antibody for CD34. RESULTS: All 30 cases of typical DFSP stained intensely for CD34. In the seven cases of DFSP-FS, all cases showed a similar staining pattern in the DFSP portion. However, focal CD34 immunoreactivity was noted in only one (14%) of seven cases in the fibrosarcomatous portion of these tumors. CONCLUSIONS: CD34 immunoreactivity is rare in the fibrosarcomatous portions of DFSP-FS, and this immunophenotype may be diagnostically useful in those cases in which it is difficult to recognize this change histologically.
Dermatofibrosarcoma protuberans (DFSP) is a slowly growing tumor that occurs in the dermis, and it has a tendency to recur repeatedly. We reported a case, 50-year-old woman, who underwent chest wall reconstruction due to recurrence of DFSP. Since the first operation for the tumor 35 years ago, she had undergone surgical procedures 30 times. We had to resect parts of the rib cartilages and the sternum because we suspected the tumor had reached to the anterior mediastinum. There has been no recurrence for 8 months since this operation. Importance of extensive resection of the tumor to prevent a recurrence and long term follow-up should be stress.
Local reoccurrence of protuberant dermatofibrosarcoma was seen in a female patient 3 years after exeresis. We reviewed the data in the literature on the physical examination, outcome, histology and therapeutic approach. The Darier and Ferrand dermatofibroma is a very unusual skin tumour which develops in the dermal layer. Diagnosis can only be confirmed by histologicaal examination of the specimen. Wide surgical exeresis is required to avoid local relapse. Despite the high risk of reoccurrence, the prognosis is excellent.
A review of dermatofibrosarcoma protuberans diagnosed at the University Hospital of the West Indies over a 10-year period revealed 23 cases. The mean age of the patients was 36.3 years. There were 13 females and 10 males. The trunk was the most common site. The clinical diagnosis was commonly missed. The results of this study are compared with those from other countries.