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Results for “Dermatofibrosarcoma”

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PubMed1996

Scarpa's adipofascial flap for repair of wide scalp defects.

Scarpa's fascia is a prominent superficial fascial system of the body. It consists of a single membrane between the superficial fatty layer and deep fatty layer, and lies widely in the lower abdominal wall. We describe a case with a wide scalp defect resulting from a resection of a dermatofibrosarcoma, and reconstruction of the defect with Scarpa's adipofascial flap (i.e., a combined paraumbilical perforator-based adipofascial flap-groin adipofascial flap). The primary advantage of Scarpa's adipofascial flap for scalp defects is that (1) the donor site is most acceptable for a free flap with a minimal donor scar and minimal dysfunction; (2) even in cases in which large flaps are used, donor defects can be closed directly without skin grafting; (3) in the obese patient, this flap is preferable because of cosmetic improvement of the abdominal wall; (4) the donor area has so many perforators that an extended adipofascial flap can be obtained with a combination of these perforators; and (5) the flap may be nourished with one of several arteries, such as the superficial or deep inferior epigastric artery, or the superficial or deep circumflex iliac artery. The disadvantages of this flap are that the territory with a single artery may be smaller than a skin flap with the same artery and oversurfacing of the graft results in a poor cosmetic appearance. Scarpa's adipofascial flap is indicated when the defects are in an exposed area, especially in children, young patients, and females, and when this procedure is combined with a skin-expanding method in the secondary repair.

Adipose Tissue↗

PubMed1997

Reconstruction of a large thoracoabdominal wall defect with a flow-through forearm flap and a latissimus dorsi-groin flap.

We report a case of a 45-year-old man with a recurrent, large, invasive dermatofibrosarcoma protuberans over the left lower chest and abdomen. Wide surgical excision of the tumor created a major thoracoabdominal wall defect. Wound coverage was achieved by using a flow-through forearm flap and an inferiorly based latissimus dorsi-groin flap. Follow-up at 1 year revealed no local recurrence or herniation.

Abdominal Muscles↗

PubMed2005

Innovative laboratory techniques to facilitate processing of large Mohs cases.

BACKGROUND: Processing multiple tissue sections in large Mohs cases is time consuming and labor intensive. OBJECTIVE: To present innovative laboratory techniques to facilitate processing of large Mohs cases. METHODS: A method for processing a large dermatofibrosarcoma protuberans Mohs case is outlined. RESULTS: Modifications in tissue processing and equipment employed in a large Mohs case are presented. CONCLUSION: Innovative modifications to the standard Mohs laboratory technique can facilitate processing of large Mohs cases, resulting in high-quality, rapid frozen sections while optimizing efficiency.

Dermatofibrosarcoma↗

PubMed2003

Skin cancer in children and young adults: 28 years' experience from the Northern Region Young Person's Malignant Disease Registry, UK.

Little population-based data has been published about skin cancers in children and young adults. In this study, 200 cases of melanoma and non-melanoma skin cancers diagnosed under 25 years of age in the North of England from 1968-1995 were obtained from the Northern Region Young Persons' Malignant Disease Registry. The incidence was 1.2 cases per million per year for children (aged 0-14 years) and was 13 cases per million per year for young adults (aged 15-24 years). Melanoma accounted for 138 cases, of which 16 were in subjects aged < 15 years at diagnosis. The incidence of melanoma increased in females at a rate of 5.6 per million per decade (95% confidence interval [CI] 2.2-8.9, P = 0.002), largely due to an increased incidence of primary lower limb tumours. The incidence for males was unchanged. Survival improved significantly over time for both males and females (P < or = 0.02). Of the 62 patients with non-melanoma skin cancers, 66% were diagnosed with primary non-basal cell carcinoma, 13% with dermatofibrosarcoma protuberans, 10% with squamous cell carcinoma and 11% with other tumours. Two cases were iatrogenic second malignancies following treatment for an earlier primary brain tumour. The incidence of non-melanoma skin cancers was significantly higher during 1982-1995 than during 1968-1981 (rate ratio 1.7, 95% CI 1.0-2.8). There were three deaths from non-melanoma skin cancer, and the overall 5 year survival rate was 98% (95% CI 89-100%). The reason for the increasing incidence of both melanoma and non-melanoma skin cancer in young people is unknown, but it is likely that ultraviolet exposure plays an aetiological role. It is important that families continue to be advised of the need for vigilance with regard to childhood sun exposure.

Adolescent↗

PubMed2003

Preoperative pneumoperitoneum used for tissue expansion before abdominal wall reconstruction.

The use of preoperative pneumoperitoneum was described initially for the repair of large ventral hernias. Large volumes of air are injected into the peritoneal cavity, which distends the abdominal wall progressively. This method is usually used to facilitate the reinstatement of the viscera into the abdominal cavity and improves postoperative diaphragmatic function. The authors use this method for preoperative tissue expansion of the abdominal wall before full-thickness resection of the abdominal wall for dermatofibrosarcoma protuberans in a 12-year-old girl. In total, 6.8 L air was injected over 3 weeks without problem. The subsequent laxity of the abdominal wall permitted the primary closure of a 14- x 16-cm resection. The postoperative course was uncomplicated. Preoperative pneumoperitoneum can be used when reconstruction can be delayed. This is so in cases of slow tumor growth and in cases of infectious or posttraumatic full-thickness abdominal defects. The parietal laxity obtained with the pneumoperitoneum not only increases the possibilities of direct closure but also increases the possibilities of pedicled flap reconstructions.

Abdominal Wall↗

PubMed2006

Refractory eczematous dermatitis associated with retained suture material.

We present a patient who developed a cutaneous-id reaction and eczematous dermatitis related to a residual Prolene suture following excision of dermatofibrosarcoma protuberans. Following a wider excision of the site, with removal of the retained suture material, the patient's symptoms dramatically improved. Although this reaction is rare, retained sutures should be considered a possible cause of a postoperative eczematous dermatitis that does not respond to standard therapies.

Adult↗

PubMed2006

p63 is a useful marker for cutaneous spindle cell squamous cell carcinoma.

BACKGROUND: Cutaneous spindle cell squamous cell carcinoma (SCSCC) is a rare variant of SCC. This lesion is sometimes difficult to diagnose based purely on morphologic features. p63 is a member of the p53 gene family that can be identified in epithelial malignancies. METHODS: Thirteen cases of spindle SCC were stained with p63, CK34betaE12, MNF116, vimentin, and S100. Control cases included desmoplastic melanoma (eight cases), atypical fibroxanthoma (AFX) (10 cases), dermatofibrosarcoma protuberans (eight cases), and cutaneous leiomyosarcoma (LMS) (four cases). RESULTS: p63 was expressed diffusely in the nuclei of 100% (13/13) of SCSCCs. Of controls, p63 showed focal labeling of two LMS and two AFX. MNF116 and CK34betaE12 were positive in 13/13 SCSCCs. Of controls, one LMS was focally positive for MNF116. All SCSCCs and all control cases were positive for vimentin. CONCLUSIONS: In the given differential diagnosis, p63 appears relatively specific to SCSCC and adds a useful nuclear marker to the available repertoire. The findings also suggest that cytokeratins MNF116 and CK34betaE12 may be more useful than standard cytokeratins in labeling SCSCC.

Adult↗

PubMed1993

Current topics of immunohistochemistry as applied to skin tumors.

Suppressor oncogene p53 is expressed more frequently in the nodular portion than the superficial spreading portion of the same melanoma. PCNA expression follows the same pattern but it is strongly expressed already in the superficial spreading portion. CD34 is found to label dermatofibrosarcoma protuberans. There is the mixture of negative strands but the immunostains are predominantly positive in the three cases studied. Neurogenic tumors are reactive but the intensity of staining is only moderate. Fibrous histiocytoma, dermatofibroma and other fibrohistiocytic tumors are non-reactive. CD34 is expressed in the outer root sheath of hair follicle below the sebaceous gland level. A case of piloepidermal cyst is CD34 positive. CD34 is expressed in vascular endothelial cells; not only hemangiomas but also lymphangiosarcoma is CD34 positive. A case of indeterminate cell histiocytosis following scabies and superficial variant of clear cell sarcoma are discussed as examples of new entities.

Antigens, CD↗

PubMed1996

A case of Bednar tumor recurring after 23 years.

We reported a patient with a Bednar tumor who developed local recurrence 23 years later. The recurrent Bednar tumor showed the histopathological features dermatofibrosarcoma protuberans (DFSP), and expressed CD34. We favor the term "Bednar tumor" rather than pigmented DFSP and consider that Bednar tumor is an intermediate type between cellular blue nevus and DFSP.

Adult↗

PubMed2006

A very large, rapidly developing, congenital giant cell fibroblastoma in a 5-month old infant.

Giant cell fibroblastoma is an uncommon soft tissue neoplasm that occurs in childhood. It seems to be the juvenile form of dermatofibrosarcoma protuberans, which is seen less frequently in children. Only one congenital case has been reported previously. In this report, we present a 5-month-old girl with a rapidly and atypically growing mass located on the upper part of the right scapula. We think that our case is very interesting, because no similarly large lesion (12 cm x 10 cm x 8 cm) related to giant cell fibroblastoma has been previously reported in the published work.

Dermatofibrosarcoma↗

PubMed1998

Deep penetrating dermatofibroma.

BACKGROUND: Deep penetrating dermatofibroma is an unusual and histologically distinctive form of dermatofibroma that usually occurs on the lower extremities of adults. OBJECTIVE: The dermatologic surgeon and pathologist should be aware of this entity to prevent excessive treatment of a benign condition. METHODS: We are reporting a case of deep penetrating dermatofibroma with the results of histologic examination and immunohistochemical studies. RESULTS: The neoplasm consisted of a fibrohistiocytic tumor penetrating into the subcutis accompanied by scalloped extensions along the septae. Immunohistochemical stains were positive for Factor XIIIa and negative for CD34. CONCLUSION: Recognition of the histologic pattern is important in distinguishing deep penetrating dermatofibroma from dermatofibrosarcoma protuberans.

Dermatofibrosarcoma↗

PubMed1997

Immunohistochemical analysis of platelet-derived growth factor and its receptors in fibrohistiocytic tumors.

Platelet-derived growth factor (PDGF) is known to stimulate the proliferation of fibroblasts, although the role of PDGF and its receptors in the development of fibrohistiocytic tumors has not been clarified. In this study, we investigated this role by immunohistochemically staining PDGF and PDGF beta-receptors in paraffin-embedded dermatofibroma (DF), dermatofibrosarcoma protuberans (DFSP) and malignant fibrous histiocytoma (MFH) tissue. We also immunohistochemically investigated the relationship between PDGF beta-receptors and CD34, which is a known immunohistochemical marker for DFSP. Immunohistochemical studies using anti-PDGF-AA or BB antibodies showed that PDGF-AA and BB was found in 20 to 40% of the tumor cells in DF, DFSP, and MFH. No definite relationship for each tumor type was found. The expression of PDGF beta-receptors in DFSP and that of MFH tissue was significantly greater in comparison to DF and scar tissue. The expression of CD34 and PDGF beta-receptors in DFSP was observed in identical areas. These findings suggest that autocrine or paracrine growth stimulation, through PDGF beta-receptors, is related to the tumorous proliferation of fibrohistiocytic tumors, and the expression of PDGF beta-receptors might play a role in the proliferation of CD34 positive tumor cells.

Antigens, CD34↗

PubMed1998

Differential expression of connective tissue growth factor gene in cutaneous fibrohistiocytic and vascular tumors.

Connective tissue growth factor (CTGF) is a member of a family of immediate early gene products that may play an important role during tissue regeneration, wound repair and skin fibrosis. In this study, CTGF gene expression in mesenchymal tumors was investigated by in situ hybridization and CD34 antigen expression was studied by means of immunohistochemical staining. CTGF mRNA was expressed in fibroblasts of all nine dermatofibromas examined, but five of seven dermatofibrosarcoma protuberans (DFSP) or two cases of malignant fibrous histiocytoma were negative for its expression. In contrast, CD34 antigen was expressed only in DFSP. In vascular tumors, CTGF mRNA was expressed in pyogenic granuloma but not in angiosarcoma. In addition, the endothelial cells in angiolipoma and angioleiomyoma, but not in venous lake, expressed CTGF mRNA. These vascular lesions were all positive for CD34 expression. Tumors of other origins were negative for CTGF mRNA. Our findings indicated that benign fibroblasts and/or vascular endothelial cells have the capability to express CTGF mRNA when activated, but these cells lose this ability when they achieve malignant potency. Thus, examination of CTGF gene expression may be useful for differentiating between benign and malignant mesenchymal tumors, or to determine the origin of the tumors in connective tissue.

Antigens, CD34↗

PubMed2005

CD34 expressing ameloblastic fibrosarcoma arising in the maxilla: a new finding.

Ameloblastic fibrosarcoma (AFS) is a rare malignant tumor of the jaw. The malignant mesenchymal component of AFS has been described as 'fibroblast-like', although little is known about the immunophenotype, except for vimentin expression. Here, we present a case of AFS in a 62-year-old woman. The mesenchymal component displayed the features of either dermatofibrosarcoma protuberans or fibrosarcoma, and was positive for CD34. This is the first reported case of CD34 expressing AFS in the maxilla.

Aged↗

PubMed1999

Micrographic surgery ('slow Mohs') in cutaneous sarcomas.

BACKGROUND: Micrographic surgery (MS) results in very low local recurrence rates in all sorts of skin tumours that grow by extensive subclinical infiltration. Therefore, MS should prove useful in the treatment of cutaneous sarcomas. OBJECTIVE: To treat cutaneous sarcoma with MS in order to minimize local recurrence rates. METHODS: We treated 5 cases of dermatofibrosarcoma protuberans and 5 cases of cutaneous sarcomas of different origin (atypical fibroxanthoma, malignant fibrous histiocytoma, malignant peripheral nerve tumour) with micrographic surgery using paraffin-embedded sections. In primary cutaneous sarcomas, tumour extensions were readily detected in HE sections. In recurrent tumours, special stains were needed to distinguish tumour extensions from scar tissue. RESULTS: All patients were treated successfully and have remained free of local recurrences as of yet. Solitary pulmonary metastasis occurred in 1 patient with high-grade malignant peripheral nerve tumour. CONCLUSION: MS is an excellent procedure to minimize local recurrence in cutaneous sarcomas. Cutaneous sarcomas with low metastatic potential can be cured with MS.

Adult↗

PubMed2003

Flow-cytometric DNA analysis in cutaneous fibrohistiocytic tumors.

BACKGROUND: Fibrohistiocytic tumors are rare skin tumors. There are many kinds of fibrohistiocytic tumors. It is often difficult to differentiate dermatofibrosarcoma protuberans (DFSP) and malignant fibrous histiocytoma (MFH) from dermatofibroma (DF) and other benign tumors. OBJECTIVE: In this study, the possible usefulness of DNA ploidy for the differentiation was studied. METHODS: Five MFH, 9 DFSP, 3 DF, 2 lipofibromas and 2 scars were studied for DNA ploidy using a flow cytometer. RESULTS: All the 7 benign tumors showed a diploid pattern. Of 14 malignant fibrohistiocytic tumors, 8 cases (57.1%) showed an aneuploid and 6 a diploid pattern. Two of 5 MFH (40.0%) and 6 of 9 DFSP (66.7%) showed an aneuploid pattern. These results showed that fibrohistiocytic tumors with an aneuploid pattern are malignant, though those with a diploid pattern may be benign or malignant. CONCLUSION: Analysis of DNA ploidy using flow cytometry was concluded to be useful for the differentiation of malignant fibrohistiocytic tumors from benign ones.

DNA↗

PubMed1997

A case of Bednar tumor. Immunohistochemical positivity for CD34.

A 42-year-old Japanese man with a Bednar tumor is described. The skin showed a slightly elevated, red, partially dark blue, dermal nodule growing into the deep subcutaneous tissue. The histological specimen showed two types of tumor cells, spindle-formed cells with no melanin granules and melanin-laden cells. Immunohistochemical stainings for CD34 and S-100 protein revealed that the former cells showed positivity only for CD34, while the latter were only positive for S-100 protein. These results suggest that the Bednar tumor is a dermatofibrosarcoma protuberans in which melanin-laden cells coexist.

Adult↗

PubMed2005

The development and application of imatinib.

The hallmark characteristics of cancer include an unrestrained proliferation involving activation of growth signals, loss of negative regulation and dysfunctional apoptotic pathways. Targeting abnormal cell signalling pathways should provide a more selective approach to cancer treatment than conventional cytotoxic chemotherapy. Tyrosine kinases play an essential role in the signalling pathways involved in the control of cellular proliferation and growth. Imatinib is a small-molecule tyrosine kinase inhibitor of the ABL fusion gene, platelet derived growth factor receptors (PDGFR) and KIT. This agent has demonstrated considerable activity in chronic myeloid leukaemia (CML) by inhibiting the BCR-ABL fusion protein and gastrointestinal stromal tumours (GISTs), which are predominantly driven by activating mutations in KIT. A number of other rare conditions are also responsive, for example, dermatofibrosarcoma protuberans, which is driven by a chromosomal translocation involving PDGF-B and Col1A1, resulting in overexpression of PDGF-B, and hypereosinophillic syndrome, which can be caused by activating PDGFR mutations. The pivotal registration study for newly diagnosed CML was a large randomised trial comparing 400 mg/day of imatinib to a combination of IFN-alpha and cytarabine, which demonstrated a significantly higher complete haematological and cytogenetic response rate in the imatinib arm. In the case of GIST a randomised study in patients with inoperable or metastatic disease explored doses of 400 - 600mg and reported a response rate of > 50% in each arm plus disease stabilisation and an improvement in performance status. Large randomised trials have subsequently been performed, comparing 400 with 800mg/day. The first to report indicates that the larger dose is associated with improved progression-free survival, although it is not yet known whether or not this will translate into a difference in overall survival. The most common KIT mutation involves exon 11 and is associated with a statistically significant better response and prognosis compared with other mutations or no detectable mutations. Mutational analysis is likely to become increasingly important in the selection of patients for neoadjuvant and adjuvant treatment and in helping to understand the nature of acquired resistance.

Antineoplastic Agents↗