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PubMed1999

A comparative study of P53/MDM2 genes alterations and P53/MDM2 proteins immunoreactivity in soft-tissue sarcomas.

In the present study, the expression of P53 and MDM2 proteins were examined in 94 soft-tissue sarcomas (35 malignant fibrohistiocytomas, 15 neurosarcomas, 14 liposarcomas, 13 leiomyosarcomas, 11 fibrosarcomas and 6 dermatofibrosarcomas) by immunohistochemistry. The immunohistochemical findings were correlated with P53 mutation analysis using PCR-SSCP, PCR-HDF and direct sequencing, and MDM2 amplification studies by differential PCR. P53 immunopositivity was found in 25 out of 94 (26.6%) cases. Alterations of the P53 gene were detected in 12 (12.8%) tumors; eight of these tumors revealed P53 immunoreactivity. A high number of P53 positive and P53 mutated tumors were histologically defined as poorly differentiated G3 (64.0% and 75.0%, respectively). MDM2 immunopositivity was revealed in 36 out of 94 (38.3%) cases. MDM2 amplification occurred in 17 tumors (18.1%); only nine of these tumors exhibited MDM2 immunoreactivity. Overall, MDM2 positivity was not associated with MDM2 amplification in 27 out of 94 tumors (28.7%). There was no significant correlation between MDM2 overexpression and histological grade. However, when the samples were stratified by immunophenotype, the majority of tumors (52.5%) with isolated MDM2 overexpression (dissociated from P53 positivity) were defined histologically as low grade (G1 + G2). These results support the notion that besides P53 alterations, MDM2 gene deregulation seems to be an important event in sarcomas evolution. Additionally, the mechanism of MDM2-mediated degradation of P53 protein, without involving stabilization and inactivation of P53 gene, should be considered for better understanding of all features of tumor progression processes.

Adult↗
PubMed1999

[Covering soft tissue defects after tumor resection around the knee with vascularized musculo-cutaneous and fascio-cutaneous flaps].

The application of musculo-cutaneous of the medial head of gastrocnemius for covering skin and soft tissues defects following total knee replacement in treatment for proximal tibial osteosarcoma in 4 patients is presented. In 2 patients the fascio-cutaneous flaps originated from lower leg were used to cover defects of anterior aspect of the knee after resection of dermatofibrosarcoma of the patellae and fibrohistiocytoma localized in proximal tibia.

Adolescent↗
PubMed2000

A case of a hard inguinal nodule.

Dermatofibrosarcoma protuberans (DFSP) is an uncommon tumor with its onset typically in the second to fifth decades of life. It most commonly presents on the trunk, and recent cytogenetic studies suggest a neural origin. A case presentation and review of the recent literature on the diagnosis, differential diagnosis, and treatment of DFSP is presented.

Adult↗
PubMedDate not supplied

Syndecan-1 expression in different soft tissue tumours.

BACKGROUND: In vitro studies have suggested that expression of syndecan-1 (CD138) is correlated with morphologic phenotype (epithelioid or spindle) of cultured tumour cells. MATERIALS AND METHODS: Fifty-seven different soft tissue tumours were selected to analyse their syndecan-1 (CD138) reactivity. Immunohistochemical staining of paraffin sections, following a high temperature unmasking technique, was performed. The intensity and the pattern of staining was studied. RESULTS: Cell membrane positivity was observed in epithelioid sarcomas and epithelial elements of synovial sarcomas. GISTs, some malignant epithelioid schwannoma and some fibromatosis showed intracytoplasmatic reaction, while pyogenic granuloma, Kaposi's sarcoma, fibrosarcoma and dermatofibrosarcoma protuberans were negative. CONCLUSION: At first it seemed that the cell membrane positivity of syndecan-1 accompanied true epithelial differentiation in soft tissue sarcomas, but the results further highlighted the non specific nature of this expression. Therefore the heterogeneity in the appearance of syndecan-1 in various soft tissue tumours is not simply associated with the phenotype, suggesting more complex functions.

Dermatofibrosarcoma↗
PubMed2001

[Importance of dermatologic screening within the frame work of a general cancer prevention program].

BACKGROUND: Skin tumours represent about 11% of all the malignant neoplasms and their frequency is increasing annually. Skin tumours (melanoma, basal and squamous cell carcinoma, etc.) can be used for a good screening activity, but in relation to breast or cervix uteri cancer needs to be better defined. A test on a population of selected patients against skin malignant neoplasms has been carried out in our Centre. All of them had skin lesions and further checks were necessary. METHODS: The diagnostic protocol used in our Centre for Oncological Prevention uses the collection of anamnestic data and an objective examination. Between 1996 and 2000, 222 patients between the ages of 18 and 80 have been selected. All of them had suspected skin lesions. The patients were selected by the oncologist, particularly for pigmentation, asymmetry, irregular borders and heterogeneous colour of their skin lesions. Subsequently, the patients were sent for a further examination to the dermatologist oncologist, who on the basis of the objective dermatological examination with possible dermatoscopy, made a clinical diagnosis of the skin injuries or suggested surgical removal for the histological control of the same. RESULTS: Requested consultations: 222. Exami-nations made: 195. Patients considered: 190. Skin injuries examined: 190. The following skin lesions were identified: melanoma: 4 (2.1%) [2: I Clark level; 2: II Clark level]; basal cell carcinoma: 14 (7.37%); dermatofibrosarcoma: 1 (0.53%); keratoacanthoma: 1 (0.53%); dysplastic nevus: 4 (2.1%); actinic keratosis: 7 (3.68%); benign lesions: 159 (83.68%). CONCLUSIONS: These data were obtained by a screening program and it is therefore not a random study. This study shows interesting results because tumoral skin lesions and in particular melanoma were recognised at early stages. This is more than enough for us to create a specific screening program for skin lesions to cut down the rate of morbidity and mortality.

Adult↗
PubMed2002

[Giant cell fibroblastoma: a clinicopathologic analysis of seven cases].

OBJECTIVE: To study the clinical, pathological and immunohistochemical features of giant cell fibroblastoma (GCF), with emphasis on its differential diagnosis and histogenesis. METHODS: Seven cases of GCF were investigated by light microscopy and immunohistochemistry. RESULTS: Six cases occurred in children, and one occurred in a 35 year-old adult (mean 9.4 years). Five were male and two were female. Clinically, all cases appeared as slowly growing painless nodules located in the dermis or subcutis of the trunk and extremities. Microscopically, the poorly circumscribed tumor was composed of a proliferation of slightly to moderately atypical spindle cells which were arranged in parallel or wavy fascicles, and embedded in a fibromyxoid to collagenous background. The pathognomonic feature consisted of irregular distributed cleft-like or sinusoid-like pseudovascular spaces lined with a discontinuous layer of pleomorphic spindle cells and multinucleate giant cells. There was transition in shape between these two cells. Immunohistochemially, both cells expressed vimentin and CD34. Follow-up information in five cases showed local recurrences in two cases. CONCLUSIONS: (1) GCF is a distinctive fibroblastic tumor of intermediate malignancy that occurs predominantly in children. Recognizing its clinical and pathological characteristics is important to avoid misdiagnosis with other lesions with similar features. (2) GCF shared clinical, immunohistochemical and cytogenetic features with its adult counterpart-dermatofibrosarcoma protuberans (DFSP). The additional coexistence of GCF and DFSP areas in some primary cases and the reciprocal transformation in recurrent tumors all suggest that they are two closely related entities, possibly representing two members of the CD34 positive dendritic neoplasms.

Adult↗
PubMed2003

[New oncological treatment principle with imatinib].

One of the undoubted major breakthroughs in the recent treatment of cancer is imatinib, a tyrosine-kinase inhibitor of the bcr-abl fusion protein, the stem-cell factor receptor c-kit (KIT) and the platelet-derived growth-factor receptor. The successes obtained with imatinib in the treatment of chronic myeloid leukaemia (CML), gastrointestinal stroma-cell tumours (GIST), and dermatofibrosarcoma protuberans, demonstrate that targeted therapy with a rationally designed, small, synthetic molecule can be highly successful. However, experience so far with imatinib in KIT-positive tumours indicates that imatinib seems only to be effective in those tumours with a gain-of-function mutation in c-kit. There are arguments in favour of investigating a combined therapy of imatinib and classical chemotherapy.

Antineoplastic Agents↗
PubMed2004

Fibrosarcomatous Bednar tumor with distant metastases--a case report.

In July 1994, a 27 year-old man presented with a twice recurrent, pigmented tumor on his left wrist. Wide excision and histological examination showed a pigmented dermatofibrosarcoma protuberans (PDFSP) or Bednar tumor with foci of fibrosarcomatous (FS) change. Immunohistochemistry helped to distinguish it from cellular blue nevus and malignant melanoma. In January 1997, he presented with 3 swellings on his thighs which showed features of fibrosarcoma. In March 1998, he developed multiple tumors on his thighs, chest wall, supraclavicular region, parapharyngeal region and two lung nodules. To the best of our knowledge, there are only 3 previously published reports of metastases in patients with Bednar tumor. We present the fourth such case.

Adult↗
PubMed2004

Bednar tumor.

Pigmented dermatofibrosarcoma protuberance DSFP, also known as Bednar tumor or formerly known as pigmented storiform neurofibroma is a rare variant of DSFP. Bednar first described it in 1957. The histology is striking because of the presence of multipolar pigment laden dendritic cells scattered among spindle shaped cells with a storiform pattern, the latter being characteristic of DSFP. The spindle cells are positive for CD34, and Vimentin and negative for S100. The pigment represents melanin. We describe such a tumor because of its rarity and very striking microscopic appearance.

Adult↗
PubMed2005

[Clinical studies with imatinib in 2004].

The successful period of targeted molecular therapy of malignancies started with the beneficial clinical application of trastuzumab and imatinib. Imatinib is a targeted molecule capable to inhibit tyrosine kinase active in the production of abl-bcr protein responsible for the translocation of 9 and 22 chromosome in chronic myeloid leukemia. Simultaneously the drug may be active against C-kit PDGFRalpha and beta, COL 1a1 and FIPI-LI gene mutations as well. Consequently, imatinib exerts a therapeutic effect upon chronic myeloid leukemia, gastrointestinal stroma tumor, dermatofibrosarcoma protuberans and hypereosinophilic syndroma. Besides, several studies are ongoing in other solid tumours characterized by those mutations, which might be blocked through imatinib treatment. Studies are in progress determining the place of imatinib in the "complex therapy". Resistance developed against the drug may be overcome by new molecules, such as SU 11248 or everolimus. It can be stated that imatinib--a drug which is applied orally and has a highly tolerable toxicity profile--signifies a new perspective for a successful targeted molecular therapy.

Antineoplastic Agents↗
PubMed2005

Mohs micrographic surgery: established uses and emerging trends.

Mohs micrographic surgery is a surgical technique that seeks to ensure the clearance of cutaneous tumors while maximizing normal tissue conservation. This is accomplished through the sequential removal of thin layers of tissue in which the entire peripheral and deep margins are examined for residual tumor. This approach appears to be superior to conventional surgical excision in the treatment of basal cell carcinoma (BCC) and squamous cell carcinoma (SCC), the two most common cancers of the skin. Its efficacy in treating BCC and SCC has led clinicians to explore the role of Mohs micrographic surgery in the management of less common cutaneous neoplasms, such as melanoma, Merkel cell carcinoma, dermatofibrosarcoma protuberans, extramammary Paget's disease, and microcystic adnexal carcinoma.

Adenoma↗
PubMed2006

A comprehensive review of imatinib mesylate (Gleevec) for dermatological diseases.

Imatinib mesylate (Gleevec, also known as STI-571), is an approved oral treatment for patients with chronic myeloid leukemia (CML). It blocks the activity of Abelson cytoplasmic tyrosine kinase (ABL), c-Kit and the platelet-derived growth factor receptor (PDGFR). As an inhibitor of PDGFR, imatinib mesylate appears to have utility in the treatment of a variety of dermatological diseases. Imatinib has been reported to be an effective treatment for FIP1L1-PDGFRalpha+ mast cell disease, hypereosinophilic syndrome, and dermatofibrosarcoma protuberans. One report notes its effectiveness for treating HIV related Kaposi's sarcoma; imatinib has not been effective for the treatment of melanoma.

Animals↗
PubMed1995

Angiomatoid features in fibrohistiocytic sarcomas. Immunohistochemical, ultrastructural, and clinical distinction from vascular neoplasms.

The typical example of malignant fibrous histiocytoma (MFH) or dermatofibrosarcoma protruberans (DFSP) does not require ancillary studies for diagnosis. However, hemorrhage with cystic change consisting of blood-filled spaces may closely mimic a vascular neoplasm. Eight fibrohistiocytic sarcomas exhibiting these angiomatoid features, initially mistaken for vascular neoplasms, were identified from personal consultation files and review of 157 consecutive sarcomas (1985 through 1993) at the University of California-(Davis) Medical Center. They included five MFH giant-cell-type sarcomas, two MFH angiomatoid-type sarcomas, and one DFSP. Immunohistochemical analysis of paraffin-embedded material showed vimentin diffuse positive, CD68 (KP-1) diffuse positive, and factor VIII negative in all eight sarcomas; actin HHF-45 focal positive in six, diffuse positive in one, and negative in one sarcoma; desmin focal positive in two and negative in six sarcomas; and S100 protein, cytokeratin AE1:AE3, cytokeratin 10.11, and EMA negative in all eight sarcomas. Electron microscopy of three tumors exhibited neoplastic cells with fibroblastic, myofibroblastic, and histiocytic features. Weibel-Palade bodies or neolumens diagnostic of vascular differentiation were absent. The clinical characteristics and behavior of these sarcomas reflect entities in the spectrum of fibrohistiocytic lineage (MFH subtypes and DFSP) rather than vascular neoplasms. Patients with deep, large, giant-cell-type MFHs did poorly (two of four patients died from disease at 8 and 25 months). Both patients with angiomatoid MFHs showed local recurrences from large incompletely excised head and neck lesions. One died of disease at 21 months and the other is free of disease 12 months following excision of a local metastasis to the opposite side of the neck. The patient with DFSP had an 18-cm locally recurrent scalp tumor that extended into bone. Immunohistochemical and ultrastructural confirmation of fibroblastic, myofibroblastic, and histiocytic lineage and exclusion of vascular differentiation help to establish the correct diagnosis in these fibrohistiocytic sarcomas with angiomatoid features. The clinicopathologic features of these eight cases reaffirm the practical utility of MFH and DFSP as diagnostic entities in the spectrum of fibrohistiocytic sarcomas.

Adult↗
PubMedDate not supplied

[Bednar's syndrome: a case report].

The case of a tumor of Bednar, which is a rather rare form (less than 5% of cases) of dermatofibrosarcoma protuberans presenting similar biologic behavior, is reported. Therefore, the same curative procedures including a radical surgical approach and the application of the Limberg flap were adopted.

Dermatofibrosarcoma↗
PubMed1996

[A study of P53 protein expression in fibrous neoplasms].

An immunohistochemical method utilizing microwave oven treated avidin-biotin complex (ABC) technique was used in this study to detect P53 protein expression in 87 parafin-embedded fibrous neoplasm tissues. The results showed that the total positive staining rate was 20.7%. The positive staining rate in fibroma (FA), dermatofibrosarcoma protuberans (DFSP) fibrosarcoma (FS) and malignant fibrous histiocytoma (MFH) were 0%, 4.0%, 37.5% and 62.5% respectively. The positive staining rate of P53 protein was increased with the increase in malignancy of the neoplasma. The expression of P53 protein was not correlated with the subtypes of DFSP and MFH, but correlated with cell differentiation. Therefore, detection of P53 protein expression may have significant value in the evaluation of malignancy, metastatic potential and the prognosis of fibrous neoplasma.

Dermatofibrosarcoma↗
PubMed1998

Expression of cathepsins in dermal fibrous tumors: an immunohistochemical study.

Cathepsins are lysosomal proteases that are distributed in many normal tissues and are primarily responsible for intracellular catabolism and turnover. The increased level of cathepsins in tumors together with their ability to degrade extracellular matrix proteins has led to the hypothesis that they are involved in the process of invasion and metastasis. We studied immunohistochemically the expression of cathepsins B, pro-D and pro-L in 8 cases of dermatofibrosarcoma protuberans (DFS), five cases of atypical fibroxanthoma (AFX) and twenty cases of dermatofibroma (DF). Expression of cathepsins B and pro-D could be detected in 5 of the 8 cases (62.5%) of DFS, whereas cathepsin pro-L was found in 4 (50%) cases. All AFX expressed cathepsin pro-L, whereas cathepsins B and pro-D were observed in 4 out of 5 cases. None of the malignant tumors showed a recurrence or metastasis after a period of four years. We found no expression of cathepsins in DF. In the epidermis and appendages, an expression of cathepsins pro-D, pro-L and B was seen. We conclude that cathepsins may be markers of increased metabolism rather than specific markers of malignancy.

Adolescent↗
PubMed2007

A fibrous histiocytoma of intermediate malignancy arisen from the parotid gland.

The light microscopic and immunohistochemical characteristics of a case of fibrous histiocytoma of intermediate malignancy arising from the parotid gland are presented. This neoplasm is rare in this site and must be distinguished from other spindle cell tumors of the parotid gland, particularly those of epithelial and myoepithelial origins. Histologic characteristics similar to those displayed by dermatofibroma and dermatofibrosarcoma protuberans help to differentiate this tumor from other spindle cell tumors. The absence of cytochemical epithelial markers is useful for establishing the diagnosis. This tumor appears to have arisen from mesenchymal elements within the gland.

Adult↗