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PubMed2002

Mohs micrographic surgery for dermatofibrosarcoma protuberans: University of Miami and NYU experience.

BACKGROUND: Dermatofibrosarcoma protuberans (DFSP) is a relatively rare cutaneous malignancy with the potential for significant local destruction and morbidity. This neoplasm has a tendency for recurrence following excision due to its infiltrative nature. Surgical excision with wide margins has been considered the standard therapy for DFSP but has had reported recurrence rates of up to 60%. OBJECTIVE: The results of 20 patients with DFSP treated with Mohs micrographic surgery (MMS) are reported. We also reviewed the world literature on the role of MMS for treatment of this tumor. METHODS: Twenty patients between the ages of 20 and 82 years with DFSP were treated with MMS. The patients were followed for recurrences between 4 and 216 months (mean 56.4 months). RESULTS: None of the 20 patients had a recurrence of DFSP following MMS. Of 221 DFSP patients in our review of the world literature treated with MMS, only 5 (2.3%) had a recurrence. CONCLUSION: MMS is an effective treatment of DFSP.

Adult↗

PubMed2003

Giant fibrosarcoma arising in dermatofibrosarcoma protuberans on the scalp during pregnancy.

BACKGROUND: Dermatofibrosarcoma protuberans (DFSP) is a low-grade malignant soft tissue tumor of subcutaneous tissues that has a propensity for local recurrence; however, it seldom metastasizes. Most lesions occur over the trunk or proximal extremities, and there is slight male preponderance. DFSP is rare on the head and neck. Less than 5% tumors are located on the scalp. OBJECTIVE: To describe a case of giant fibrosarcoma arising in DFSP on the scalp during pregnancy. METHOD: In our patient, the tumor was on scalp and measured 15 x 11 x 7 cm. The recurrent tumor that developed in the same region was measured 33 x 30 x 15 cm. Histopathologic examination of this mass revealed fibrosarcoma. The mass was excised from the subperiosteal plane with a 3-cm free margin. Because of the high possibility of recurrence, the defect site was reconstructed with a split-thickness skin graft. RESULTS: After 1 year of follow-up, no recurrence or metastasis was detected. CONCLUSION: We present a unique case in which the person has a giant fibrosarcoma on her scalp arising from DFSP with fibrosarcomatous change during pregnancy.

Adult↗

PubMedDate not supplied

A pediatric case of dermatofibrosarcoma protuberans: an immunohistochemical study.

Dermatofibrosarcoma protuberans (DFSP) is a fibrohistiocytic tumor of intermediate malignancy that usually occurs in early or mid-adult life as a nodular cutaneous mass. DFSP has rarely been reported during childhood or at birth. We report a case of childhood DFSP that illustrates the usefulness of immunohistochemical analysis in the differential diagnosis between DFSP and other fibrohistiocytic proliferations occurring in the skin. A prompt and correct diagnosis is very important in order to ensure appropriate treatment and to prevent local recurrences.

Child↗

PubMed2001

Tenascin differentiates dermatofibroma from dermatofibrosarcoma protuberans: comparison with CD34 and factor XIIIa.

Differentiation of dermatofibroma (DF) from dermatofibrosarcoma protuberans (DFSP) can be difficult. CD34 and Factor XIIIa have been used to differentiate DF from DFSP. However, there is overlap and lack of specificity of their expression. Tenascin is an extracellular matrix glycoprotein that is involved in embryogenesis, carcinogenesis, and wound healing. The aim of the study was to assess the role of tenascin in DF and DFSP and compare the results with those obtained with CD34 and Factor XIIIa. Immunohistochemical staining was performed on 20 cases each of DFSP and DF, using antibodies to tenascin, CD34 and Factor XIIIa, and the streptavidin biotin technique. Positivity for all 3 antibodies was assessed within the tumors. Tenascin expression was also assessed at the dermal-epidermal junction. Strong tenascin positivity was noted at the dermal-epidermal junction overlying the lesion in 20 of 20 cases of DF (100%) and was negative over the lesion in 20 of 20 cases DFSP (100%). Tenascin was noted within the lesion of 80% of both DF and DFSP (16/20 cases). CD34 was strongly expressed in 16 of 20 (80%) DFSP and 5 of 20 (25%) DF, whereas Factor XIIIa was strongly expressed in 19 of 20 (95%) DF and 3 of 15 (15%) DFSP. Although CD34 was expressed in 80% DFSP and Factor XIIIa in 95% of DF, there was overlap in their expression in the 2 types of tumors. The increased expression of tenascin at the dermal-epidermal junction overlying the lesion in DF but not in DFSP, differentiated these 2 tumors. In contrast, tenascin expression within the lesion did not differentiate DF from DFSP.

Adult↗

PubMed2001

Low-affinity nerve growth factor receptor (p75) in dermatofibrosarcoma protuberans and other nonneural tumors: a study of 1,150 tumors and fetal and adult normal tissues.

Low-affinity nerve growth factor receptor (p75) is a member of the tumor necrosis factor receptor family. It may modulate the binding of nerve growth factor (NGF) to the functional high-affinity receptor tyrosine kinase (trk) A. NGF is thought to be responsible for growth, apoptosis, and function of the nervous system. The presence of this receptor (p75) was determined in a large group of neural and nonneural tumors and fetal and adult tissues. One thousand one hundred fifty tumors were analyzed with monoclonal antibody for p75, along with selected normal fetal and adult tissues. Immunoreactivity for p75 was present in adult pericytes, perivascular fibroblasts, basal cells of several types of epithelia, perineurial cells, and dendritic reticulum cells. Additionally, a wide zone of subepithelial mesenchyme and skeletal muscle were positive in the first-trimester fetus, but were diminished or negative in the adult. Consistently positive nonneural mesenchymal tumors included dermatofibrosarcoma protuberans (DFSP), embryonal and alveolar rhabdomyosarcoma, synovial sarcoma, and spindle cell hemangio(endotheli)oma. Schwann cell tumors, ganglioneuroma, granular cell tumor, and malignant peripheral nerve sheath tumor (MPNST) were also p75 positive. Mesenchymal nonneural tumors that were variably positive (32% to 69%) for p75 included fibrosarcoma variants, solitary fibrous tumor, hemangiopericytoma, spindle cell lipoma, Ewing's sarcoma, mesenchymal chondrosarcoma, and malignant melanoma. Nervous system tumors such as paragangliomas, neuroblastoma, meningioma, and perineurioma and nonneural mesenchymal tumors, including extraskeletal osteosarcoma, benign fibrous histiocytomas, fibromas, alveolar soft part sarcoma, epithelioid sarcoma, smooth muscle and gastrointestinal stromal tumors, and angiosarcomas, were almost always negative for p75. Epithelial tumors that were consistently positive included mixed tumor and adenoid cystic carcinoma, whereas mesothelioma, adenocarcinomas, and most squamous cell carcinomas were negative. p75 is not a specific marker for nerve sheath tumors. It is present in a variety of other mesenchymal tumors including synovial sarcoma and in CD34-positive tumors such as DFSP, spindle cell lipoma, and hemangiopericytoma. The presence of p75 in nonneural tumors such as DFSP and rhabdomyosarcoma mimic its presence in early fetal mesenchyme and skeletal muscle, suggesting oncofetal expression in these tumors. p75 may be useful to distinguish DFSP from benign fibrous histiocytoma.

Connective Tissue↗

PubMed2004

Molecularly targeted treatment for dermatofibrosarcoma protuberans.

Traditionally, treatment for dermatofibrosarcoma protuberans (DFSP), a rare cutaneous tumor that is locally aggressive, has been limited to wide surgical excision with negative margins. Although not usually metastatic, DFSP has significant potential for recurrence and interference in local structures. The pathogenesis of DFSP stems from a chromosomal rearrangement involving chromosomes 17 and 22, in which the collagen 1alpha1 gene is fused to the gene for platelet-derived growth factor (PDGF) B-chain. The resultant deregulated expression of PDGFB leads to continuous activation of the PDGF receptor beta (PDGFRbeta) protein-tyrosine kinase that promotes DFSP tumor cell growth. Imatinib is a potent and specific inhibitor of several protein-tyrosine kinases, including the PDGFRs. Preclinical investigations and clinical reports have shown the efficacy of imatinib in DFSP. Imatinib may provide an alternative for the treatment of unresectable or partially resectable tumors, thereby possibly improving the effectiveness of surgery.

Antineoplastic Agents↗

PubMed2002

Stromelysin 3 expression: a useful marker for the differential diagnosis dermatofibroma versus dermatofibrosarcoma protuberans.

BACKGROUND: Stromelysin 3 (ST3) is a member of the metalloproteinase family, which is expressed in tissue remodeling processes such as scarring, embryogenesis, or tumoral invasion. Although the prognosis of breast cancers and extracutaneous squamous cell carcinomas is correlated with the level of expression of ST3, this staining has not yet found a routine application in dermatopathology. OBJECTIVE: Our purpose was to study by immunohistochemistry the expression of ST3 in dermatofibromas and dermatofibrosarcoma protuberans (DFP). METHODS: We selected 40 cases of dermatofibromas, 40 histologically typical DFPs, and 10 giant dermatofibromas. Immunohistochemistry was carried out by means of the LSAB method, with monoclonal anti-ST3 antibody (provided by MC Rio, IGBMC Strasbourg). A semiquantitative scale (0-3) was used to evaluate the level of ST3 expression. RESULTS: Positively stained cells were observed in all cases of dermatofibromas (100%), including the 10 giant cases, but never in DFP (0%). The staining was intense (class 2 or 3) in 39 of the 50 dermatofibromas. The CD34 staining used as a control proved to be less efficient; 6 DFP were CD34 negative, whereas some of the dermatofibromas showed a marginal CD34 positivity. CONCLUSION: Our results are consistent with those obtained by in situ hybridization in previous studies of smaller series of fibrous tumors. The study of ST3 expression in fibrous tumors of the skin shows that this immunostaining could be a useful tool in the purpose of differentiating DFP from giant or invasive dermatofibromas. Although ST3 is a "negative" marker for DFP and therefore does not demonstrate the margins of the neoplasm, it is more reliable than CD34 staining in differentiating this tumor from a dermatofibroma.

Adult↗

PubMed2003

Multiple spindle cell lipomas and dermatofibrosarcoma protuberans within a single patient: evidence for a common neoplastic process of interstitial dendritic cells?

This case report describes a 48-year-old man with multiple spindle cell lipomas of the neck and a dermatofibrosarcoma protuberans (DFSP) with fibrosarcomatous transformation of the chest. The presence of familial and nonfamilial multiple spindle cell lipomas within a single patient is a rare event, with only two reports in the current literature. This case represents the first report of multiple spindle cell lipomas occurring in association with a DFSP. It is of particular interest in that both spindle cell lipoma and DFSP represent, at least in part, neoplastic proliferations of CD34(+) spindled cells. The exact nature and differentiation of these spindled cells remains controversial, but prior studies have suggested that they could represent neoplastic interstitial dendritic cells. The association of DFSP and spindle cell lipoma within this single patient suggests that these two tumors (and their histologic variants) may well be linked, conceptually, as neoplastic proliferations of CD34(+) interstitial dendritic cells.

Dendritic Cells↗

PubMed2003

Dermatofibrosarcoma protuberans occurring in a smallpox vaccination scar.

Dermatofibrosarcoma protuberans (DFSP) is a locally aggressive, rarely metastatic, spindle cell tumor. Trauma has been associated with its development. Since the 1940s, malignant tumors have been described to occur in sites of smallpox vaccination scars. Five cases in the literature document DFSP arising in sites of prior immunizations. We report a case of DFSP occurring in a smallpox vaccination scar and review the available literature.

Aged↗

PubMed2001

Composite tumor consisting of dermatofibrosarcoma protuberans and giant cell fibroblastoma associated with intratumoral endometriosis. Report of a case.

We present a unique case of composite skin tumor of the vulva consisting of dermatofibrosarcoma protuberans (DFSP) and giant cell fibroblastoma (GCF) with an intratumoral focus of endometriosis. A 31-year-old female with a 10-year-history of a recurring subcutaneous tumor in the vulvar area underwent excision of the seventh recurrence of the tumor. Microscopic examination revealed a composite fibrohistiocytic tumor consisting of DFSP and GCF. Additionally, a focus of endometriosis within the tumor tissue was found. Malignant transformation of extragonadal endometriosis has already been described; we present, however, the occurrence of a focus of endometriosis within the tissue of a hormonally independent soft tissue tumor. There is a possible link to the occurrence of cutaneous endometriosis at previous surgery sites and in the scars. The presence of endometriosis within the soft tissue tumor represents, to the best of our knowledge, a previously undescribed collision phenomenon.

Adult↗

PubMed2002

Pediatric pigmented dermatofibrosarcoma protuberans (Bednár tumor): case report and review of the literature with emphasis on the differential diagnosis.

Dermatofibrosarcoma protuberans (DFSP) is a fibrous tumor of intermediate malignant potential that usually affects the trunk of young to middle-aged adults. On histological examination, it is characterized by a monomorphous population of spindle cells arranged in a storiform or cartwheel pattern. Bednár tumor (BT), formerly known as storiform pigmented neurofibroma, is currently considered the pigmented variant of DFSP due to the histological and cytogenetic similarities between these two lesions. There are very few reports on BT affecting pediatric patients. We describe a case of BT affecting the dorsal aspect of the left forearm of a 6-year-old-male patient and emphasize the diagnostic clues to distinguish this unusual cutaneous neoplasm from other pigmented lesions, including pigmented (melanotic) neurofibroma (PMN), psammomatous melanotic schwannoma (PMS), neurocristic cutaneous hamartoma (NCH), and desmoplastic malignant melanoma (DMM). We would like to stress that surgical pathologists and dermatopathologists need to be aware of the prototypical histological appearance of BT as there is the risk of misdiagnosing it either as pigmented tumors associated with neurocutaneous syndromes, such as PMN and PMS, or as a highly malignant melanocytic neoplasm (DMM).

Child↗

PubMed2002

Dermatofibrosarcoma protuberans with EMa+ cells. Report of a case suggesting perineurial cell differentiation.

A case of dermatofibrosarcoma protuberans (DFSP) with epithelial membrane antigen (EMA)-positive cells is described. The tumor was excised from the left groin of a 28-year-old woman. It showed characteristic histologic features of DFSP with typical diffuse immunohistochemical positivity for CD34. Moreover, scattered neoplastic cells expressed EMA, suggesting perineural cell differentiation. Ultrastructural study found perineurial cell features, such as thin long bipolar cytoplasmic processes, pinocytotic vesicles, fragments of external lamina and/or external lamina-like material, attachment plaques, and desmosome-like junctions. This observation, together with previous immunohistochemical findings of EMA-positive cells in a subset of DFSPs, strongly suggests perineurial cell differentiation in these tumors. DFSP should be included in the differential diagnosis of EMA-positive spindle cell lesions of superficial soft tissue and skin.

Adult↗

PubMedDate not supplied

Dermatofibroma and dermatofibrosarcoma protuberans: a comparative ultrastructural study.

Dermatofibroma (DF) and dermatofibrosarcoma protuberans (DFSP) are dermal tumors whose histogenesis has not been well defined to date. The differential diagnosis in most cases is established in routine H/E sections and may be confirmed by immunohistochemistry, but there are atypical variants of DF with less clear histological differences and non-conclusive immunohistochemical results. In those cases, electron microscopy studies may be useful in establishing the diagnosis. The authors describe in detail the ultrastructural characteristics of 38 cases of DFSP and 10 cases of DF. The objective was to establish the ultrastructural features for differential diagnosis, and to identify the possible histogenesis of both neoplasms. DFSP is formed by stellate or spindled cells with long, slender, ramified cell processes joined by primitive junctions. Subplasmalemmal densities were frequently seen in the processes. Another common finding was the presence of multivesicular buds (MVB), peculiar structures that contain microvesicles abutting from the cell membrane. In contrast, DF is characterized by a proliferation of multiple capillary vessels with prominent endothelium and a perivascular population of ovoid or spindled cells devoid of cell processes. These latter cells featured intracytoplasmic lipid material (p < .001), infrequent subplasmalemmal densities (p < .001), and absence of MVB (p < .001). With the ultrastructural characteristics and the constant expression of CD34 in DFSP, a probable origin in dermal dendrocytes is postulated for this tumor. The histogenesis of DF is less clear, but an origin from FXIIIa modified perivascular dermal dendrocytes is proposed.

Capillaries↗

PubMed2001

Dermatofibrosarcoma protuberans metastasizing to cavernous sinuses and lungs: a case report.

Dermatofibrosarcoma protuberans (DFSP) is a locally aggressive tumor with a low potential for distant metastases. We report a 22-year-old female patient with a typical cutaneous DFSP who developed five local recurrences followed by left cavernous sinus metastasis at the eighth year and right cavernous sinus and lung metastases at the ninth year. In each local recurrence the tumor showed histological signs of progression as being more cellular, having higher mitotic index and being aggressively invasive through the underlying soft tissues. The histopathological evaluation of the metastatic tumor resected from the left cavernous sinus revealed dedifferentiation from low-grade DFSP to higher grade fibrosarcomatous morphology. Immunohistochemical studies of the primary tumor and also the recurrent and metastatic tumors showed diffuse CD34 positivity in all specimens and p53 positivity was detected in the metastatic tumor resected from left cavernous sinus.

Adult↗

PubMed1993

CD34 and factor XIIIa in the differential diagnosis of dermatofibroma and dermatofibrosarcoma protuberans.

To assess the use of anti-CD34 and anti-factor-XIIIa antibodies for the differential diagnosis of dermatofibroma (DF) and dermatofibrosarcoma protuberans (DFSP), we stained 40 DFs and 13 DFSPs. A significant population of dendritic and spindle cells was reactive with anti-factor-XIIIa in 90% of DFs. In DFSP, most cases had very few or no reactive cells for this antiserum and reactivity was confined to the infiltrating edges of the neoplasm or to a very low percentage of cells within the bulk of the tumor. A significant overlap was detected, a reason why the stain is useful but lacks absolute specificity for this purpose. Human progenitor cell antigen (CD34) was found in all cases of DFSP that had 50-100% of reactive cells, but in only five DFs. In DFs, the reactivity for CD34 was focal, with only < or = 20% positive cells. When used together with other histopathological criteria, factor-XIIIa antibody and particularly CD34 may help to differentiate these two processes.

Actins↗

PubMed1997

CD34 and factor-XIIIa immunoreactivity in dermatofibrosarcoma protuberans and dermatofibroma.

The histologic distinction between dermatofibrosarcoma protuberans (DFSP) and the fibrous type of benign fibrous histiocytoma (dermatofibroma, DF) may be extremely difficult. In this study, we evaluated the extent and pattern of immunoreactivity of both CD34 and factor XIIIa in a large number of DFSPs and DFs in order to determine the utility of these markers in their distinction. Using histologic criteria alone, the authors independently evaluated and agreed upon 30 cases of DF and 24 cases of DFSP, and a representative section was stained with antibodies to both factor XIIIa and CD34. Immunopositivity was evaluated semiquantitatively and assigned a score from 0 to 5. CD34 immunoreactivity was seen in 22 (92%) of 24 DFSPs (mean CD34 score, 4.60 +/- 0.3). Only 12 (40%) of 30 DFs showed CD34 immunopositivity (mean CD34 score, 0.6 +/- 0.1). Factor XIIIa was seen in 29 (97%) of 30 DFs (mean factor-XIIIa score, 4.1 +/- 0.3). In contrast, 18 (75%) of 24 DFSPs stained for factor XIIIa (mean factor-XIIIa score, 1.3 +/- 0.2), but in most of these cases the factor-XIIIa-positive cells were felt to be entrapped nonneoplastic dermal dendrocytes. Thus, an immunoprofile using antibodies to CD34 and factor XIIIa is capable of distinguishing between DFSP and the fibrous type of DF in the vast majority of cases, as long as there is recognition that there may be some CD34-positive cells in DFs, as well as some factor-XIIIa-positive cells in DFSPs.

Antigens, CD34↗

PubMed1997

Dermatofibrosarcoma protuberans with fibrosarcomatous areas: a clinico-pathologic and immunohistochemic study in four cases.

Dermatofibrosarcoma protuberans (DFSP) with fibrosarcoma (FS)-like areas (DFSP-FS) is a peculiar neoplasm that combines microscopic findings of DFSP and FS. Because of the scarce number of cases published, tumor prognosis remains controversial. The clinical histories and the histologic material of 27 cases of DFSP were reviewed. Four of them showed fibrosarcomatous areas. Follow-up data, ranging from 12 to 125 months, were obtained in all four cases. Two patients had repeated local recurrences. One patient developed pulmonary metastases and died of disease 49 months after diagnosis. In the other two patients, no recurrences or metastases were detected at 12 and 70 months after local excision, respectively. Progressive increase of FS areas, cellular density, cellular atypia, and mitotic activity were observed during the recurrences. All cases showed diffuse positive immunostaining for CD34 in DFSP areas. Three cases were also CD34-positive in FS areas. Based on a careful review of the literature and our personal experience, we conclude that DFSP-FS is a rare variant of DFSP with a higher rate of local recurrences and more distant metastases than typical DFSP.

Adult↗

PubMed1998

Atrophic and plaquelike dermatofibrosarcoma protuberans.

It is clinically important to realize that dermatofibrosarcoma protuberans (DFSP) often begins as a nonprotuberant plaque and occasionally persists as such. Cases of what has been called atrophic DFSP have recently been reported. None of these cases varied either histologically or prognostically from classical nodular DFSP, and all should be considered part of the clinical spectrum of DFSP. However, histologic variants of DFSP can portend a worse prognosis. The fibrosarcomatous variant, which is fascicular and does not immunostain with anti-CD34, is an example. Recently, another fascicular variant that does positively immunostain with anti-CD34 has been described as plaquelike DFSP. We described a case of a DFSP that had histologically banal, slender fascicles of anti-CD34 immunostaining spindle cells in a clinically nonprotuberant plaque despite subsequently developing a nodule with typical storiform histology. The importance of anti-CD34 immunoperoxidase staining in the diagnosis and prognosis of DFSP variants is emphasized.

Antigens, CD34↗