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PubMed1993

Dermatofibrosarcoma protuberans appearing during pregnancy.

Dermatofibrosarcoma protuberans is an uncommon skin tumor with a high potential for local recurrence if not adequately excised. There are only two reports in the literature describing dermatofibrosarcoma protuberans that enlarged considerably during pregnancy. On the other hand, the new development or enlargement of dermatofibromas in pregnancy is well documented. We present 2 additional patients in whom a dermatofibrosarcoma protuberans appeared and grew rapidly during pregnancy. Immunohistochemical studies were negative for estrogen and progesterone receptors. The possible pathophysiology and surgical management are presented and discussed.

Adult↗
PubMed2005

Dermatofibrosarcoma protuberans.

Dermatofibrosarcoma protuberans is a rare, locally aggressive, rarely metastatic skin tumor which tends to recur. A 45-year-old female presented with dermatofibrosarcoma protuberans on her breast as a recurrent nodule after excision. Lump was re-excised with wide margins and irradiated. Follow-up was done monthly for two years, with no recurrence.

Breast↗
PubMed2003

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.

Adult↗
PubMedDate not supplied

Dermatofibrosarcoma protuberans of the breast.

Dermatofibrosarcoma protuberans is a rare soft tissue cancer of the skin with the potential for intermediate malignity, characterized by local invasion and recurrence. Presenting with local atrophic and sometimes erythematous plaques, these lesions are best treated with surgery in which large excisions are necessary to reduce the risk of recurrence and to prevent rarely seen metastases. We report on a 26-year-old female patient with dermatofibrosarcoma protuberans on her breast, an very unusual site. As a novel surgical approach to treat the patient's tumor, a keyhole pattern was used, allowing wide safety margins during resection, while preserving the cosmetic appearance of the breast.

Adult↗
PubMed2004

Metastatic Bednar tumor (pigmented dermatofibrosarcoma protuberans) with fibrosarcomatous change: a case report.

Bednar tumor (pigmented dermatofibrosarcoma protuberans) is a variant of dermatofibrosarcoma protuberans (DFSP) that constitutes 5% of all DFSP and has a very low rate of distant metastases. We encountered a rare case of Bednar tumor with multiple different distant metastases. A 51-year-old man, who had had a history of mass resection in his left shoulder 4 years previously, was referred to our institution, complaining of a recurrence of the shoulder mass. The histological diagnosis of primary tumor was Bednar tumor, and he underwent resectional surgery for the recurrent lesion. A second local relapse, lung metastasis, retroperitoneal metastasis, and metastasis to the sigmoid colon have occurred. The recurrence lesion and all metastatic lesions were resected surgically. The histological features of all specimens showed fibrosarcomatous change. Seven months after the last surgical resection, the tumor recurred and the patient died of multiple abdominal metastases 10 years after the first surgical treatment.

Dermatofibrosarcoma↗
PubMed2002

[Dermatofibrosarcoma protuberans of the breast: a case report].

We describe a dermatofibrosarcoma protuberans (DFSP) of the breast and briefly report about such cases previously mentioned in the literature. A 27-year-old woman was referred because of the progressive increase, during pregnancy, of a mammary nodule located between the internal quadrants of the right breast. Its clinical and radiologic features suggested a fibroadenoma. Lumpectomy revealed a 3-cm, gray-whitish, fasciculated nodule. Histological examination showed a neoplasm characterized by a highly and monomorphic cellular proliferation of spindle-shaped cells, arranged in bundles displaying repetitive storiform growth pattern and infiltrating the adjacent mammary tissue. Lack of necrosis and low mitotic rate was observed. At immunohistochemistry the tumor cells were diffusely positive for vimentin and CD34, but negatively stained with CD99, bc-2, desmin, smooth-muscle actin, S100 protein and cytokeratins. A diagnosis of mammary dermatofibrosarcoma protuberans (DF-SP) was posed. Neoplastic involvement of surgical margins led to a subsequent quadrantectomy without regional lymphadenectomy. The patient was alive and disease-free at the 8-month follow-up. Mammary DFSP is rare. Its preoperative diagnosis is extremely difficult, particularly when radiologic images show an intraparenchymal lesion with round borders. We report such a case and briefly review the pertinent literature. Morphologic parameters to distinguish DFSP from other spindle cell lesions of the breast are discussed.

Adult↗
PubMed1995

Dermatofibrosarcoma protuberans with 46,XY,t(X;7) abnormality in a child.

A 9-year-old child with dermatofibrosarcoma protuberans demonstrated a balanced translocation, 46,XX,t(X;7)(q21l2;q11.2), in the untreated tumor, an abnormality not previously reported. Unlike seven of eight other reports of dermatofibrosarcoma protuberans, no ring chromosomes were present.

Child↗
PubMed2006

Intracranial recurrence of the scalp dermatofibrosarcoma.

We report a case of recurrent scalp dermatofibrosarcoma in a 30-year-old woman who underwent surgical intervention on three separate occasions during a 60-month period, and who received post-operative radiotherapy. A small, hard, elastic mass on the right parieto-occipital scalp was initially treated by simple resection in another clinic. Despite surgical intervention and radiotherapy, a recurrent tumor associated with infiltration to the calvarium was detected. The patient was then referred to our institution and a wide resection performed. Two years later, however, the patient was readmitted to our institution as a result of tumor recurrence with intracranial involvement. Scalp dermatofibrosarcoma is an uncommon but aggressive scalp tumor; therefore, wide local excision with good margins is essential to decrease the risk of regional recurrence. Close surveillance in these cases is necessary due to late tumor recurrences.

Adult↗
PubMed2000

Congenital and childhood dermatofibrosarcoma protuberans: a case report and review of the literature.

Dermatofibrosarcoma protuberans (DFSP) occurs most commonly on the trunk, affects all races, and often develops between the second and fifth decades of life. It is uncommon in childhood and is sometimes mistaken for a vascular lesion, as it often presents as a blue macule or small nodule. Review of the English literature revealed approximately 152 cases of DFSP developing before 16 years of age and only 19 claimed congenital cases. A case of congenital dermatofibrosarcoma protuberans is presented with a literature review and discussion of congenital and childhood presentations of the tumor.

Age Factors↗
PubMed1997

Dermatofibrosarcoma protuberans of the upper lip: an overview and a case report.

Dermatofibrosarcoma protuberans is a slow-growing but locally aggressive malignant neoplasm of the skin. The preferential locations are on the trunk and proximal extremities but it can occur in the head and neck region in about 15% of the cases. This is a report of such a case and a review of the literature of head and neck cases attempting to analyse the profile of patients and surgical prognosis factors. The recurrence rate is important (20% to 55%) and dependent on the free tissue margins. Those margins are at least 3 cm in all directions; this is difficult to assess in the head and neck region because of both functional and cosmetic aspects. Distant metastases are rare but related to a long recurrence history. This behaviour gives the impression that prognosis is poorer for dermatofibrosarcoma in the head and neck than within other locations.

Aged↗
PubMed2002

Dermatofibrosarcoma protuberans with onset in early childhood: a case report.

Dermatofibrosarcoma protuberans is a tumour that grows locally, infiltrating the subcutaneous tissue; it is uncommon in children. We report the case of a 27-year-old man who had had such a lesion from the age of 3 years, and it had enlarged during his lifetime. A wide local excision with margins of 3.0 cm was performed, but the lesion was not entirely removed. This is a new report of infantile dermatofibrosarcoma protuberans, and this case reflects the need for Mohs micrographic surgery.

Adult↗
PubMed1999

Revision surgery in dermatofibrosarcoma protuberans of the trunk and extremities.

AIMS: Dermatofibrosarcoma protuberans is a rare condition which is frequently misdiagnosed at presentation, resulting in a high incidence of local recurrence due to inadequate resection. The archives of the Department of Orthopaedics at the University of Florida were analysed to investigate the natural history and results of treatment for this tumour. METHODS: Between 1975 and 1996, 35 cases of DFSP were treated at the University of Florida. Of these, one was treated primarily, five were treated for local recurrence, 17 had tumour bed excisions following inadequate primary excisions elsewhere and 12 had tumour bed excisions following inadequate resection of local recurrences elsewhere. The data were analysed to assess the impact of age, gender, duration of symptoms, tumour site and size, surgical margin, number of operations and adjuvant treatments on survival and local recurrence outcomes. RESULTS: Complete follow-up was available for 34 patients. Mean follow-up was 58 months (range 12-144 months). Thirty-three patients remain alive and disease-free. One patient died of unrelated causes. The margins obtained were wide in 28 patients, marginal in six and intralesional in one. Of the seven patients with inadequate surgical margins, four received adjuvant radiation therapy and remain disease-free. No patient with an adequate margin developed a local recurrence, but there were three local recurrences in the patients with an inadequate margin who did not receive adjuvant radiation therapy (local recurrence rate: 8%). No patient developed lymphatic or distant metastasis. Local recurrences were more likely to be classified Stage IB (17/17) than primary tumours (1/18) (P<0.001). Local recurrence was more likely where the surgical margin was less than 2.5 cm from the lesion. CONCLUSIONS: Dermatofibrosarcoma protuberans is a low-grade tumour that has a high potential for local recurrence unless it can be completely excised. The overall rate of local recurrence in referred patients in this series was 20/35 cases (57%). All occurred after inadequate margins at previous surgery in other institutions. Revision surgery in these patients showed a local recurrence rate of 8%. To avoid extensive surgery for recurrences, initial treatment should be by wide excision incorporating the underlying deep fascia and a cuff of 2.5-3 cm of normal skin tissue. Radiation therapy provides a useful adjunct where adequate margins cannot be obtained.

Adolescent↗
PubMed2002

Dermatofibrosarcoma protuberans: how wide is wide in surgical excision?

Dermatofibrosarcoma protuberans is a rare mesenchimal tumour mainly characterized by its local aggressiveness and high reported recurrence rates. Classically, wide excisional surgery with at least 3 cm of free tissue margins is recommended as treatment. We present a series of 18 patients with variable gross free tissue margins but confirmed histological free margins in all cases whatever the surgical excision really was. Only one patient presented a local recurrence (5.5%) of a very large (14 cm) sarcomatous variant of dermatofibrosarcoma protuberans. Mean follow-up in this series was 52.1 months. Those results emphasize that histological rather than theoretical "golden rules" are mandatory to locally control this aggressive neoplasm.

Adult↗
PubMedDate not supplied

An uncommon soft tissue tumour of the chest wall: dermatofibrosarcoma protuberans.

Dermatofibrosarcoma protuberans is a slow-growing tumour with propensity for local recurrence. We report herein a case of a 36-year-old man, who presented with a huge, recurrent tumour arising from the left upper anterior part of his chest wall. We performed a wide excision with 4 cm free skin margins. Reconstruction was performed with a pedicled latissimus dorsi musculocutaneous flap and free split skin grafts. Histologic and immunohistochemical studies revealed the lesion to be dermatofibrosarcoma protuberans.

Adult↗
PubMed2001

Fibrosarcomatous dermatofibrosarcoma protuberans with giant rosettes.

A rare case of dermatofibrosarcoma protuberans, which contained low-grade fibrosarcoma with giant rosettes in its recurrence areas is described. The giant rosettes lying in the fibrosarcomatous area produced a pattern similar to that of so-called hyalinizing spindle cell tumor with giant rosettes. The transitions between the tumor components were gradual, and both primary and recurrent lesions showed diffuse immunoreactivity for CD34. The presence of giant rosettes in fibrosarcomatous areas of dermatofibrosarcoma protuberans suggests a possible histogenetic relationship between fibrosarcomatous areas of DFSP and hyalinizing spindle cell tumor with giant rosettes.

Antigens, CD34↗
PubMed2004

Dermatofibrosarcoma protuberans arising from a burn scar of the axilla.

It is well-known that the development of a malignant tumor in a chronic burn scar is one of the long-term complications of a severe burn. Most of these tumors are squamous cell carcinomas, and sarcomas are much rarely seen in chronic burn scars. In the previous literature, 24 cases of burn scar sarcomas were reported. The authors report the second case of dermatofibrosarcoma protuberans arising in a chronic, severe burn scar. A 68-year-old woman with a reddish tumor in the right axilla was referred to the authors' hospital. The patient had sustained severe burns in the right axilla, upper arm, and trunk at the age of 6 years. A biopsy specimen revealed dermatofibrosarcoma protuberans. There was no evidence of distant metastasis. The tumor was excised widely to include the surrounding burn scar. Two years after the operation, there was no evidence of tumor recurrence. As surgeons, we should recognize the possibility of the occurrence of various malignant tumors including sarcomas on burn scars.

Aged↗
PubMed2006

Expression of CD163 in dermatofibroma, cellular fibrous histiocytoma, and dermatofibrosarcoma protuberans: comparison with CD68, CD34, and Factor XIIIa.

BACKGROUND: Distinction between cellular fibrous histiocytomas (FHs) with a deep component and dermatofibrosarcoma protuberans (DFSPs) can pose diagnostic problems. While CD68, CD34, and Factor XIIIa are helpful in distinguishing between these entities, none are diagnostically absolute. Recent work with CD163, a hemoglobin scavenger receptor, has demonstrated that this marker has high specificity for monocytes, macrophages, and histiocytes. Our goal is to evaluate the utility of CD163 in the diagnosis of dermatofibromas (DFs), cellular FHs, and DFSPs. METHODS: Sixty cases including 19 DFs, 23 cellular FHs with a deep component, and 18 DFSPs were tested with antibodies against CD163, CD68, CD34, and Factor XIIIa. RESULTS: CD163 was expressed in 17/19 (89%) DFs, 23/23 (100%) cellular FHs, and 3/18 (17%) DFSPs. CD68 was positive in 8/19 (42%) DFs, 19/23 (83%) cellular FHs, and 1/16 (6%) DFSPs. CD34 was expressed in 1/19 (5%) DFs, 5/23 (22%) cellular FHs, and 100% of DFSPs. Factor XIIIa labeled 4/19 (21%) DFs, 11/23 (48%) cellular FHs, and 0/17 cases of DFSPs. CONCLUSIONS: CD163 expression is helpful in distinguishing between cellular FHs and DFSPs and will be useful in a panel of antibodies when these entities are in the differential diagnosis. Sachdev R, Sundram U. Expression of CD163 in dermatofibroma, cellular fibrous histiocytoma, and dermatofibrosarcoma protuberans: comparison with CD68, CD34, and Factor XIIIa.

Adult↗