Search PubMed⌕ Search

SEARCH · Search PubMed

Results for “Dermatofibrosarcoma”

Search indexed PubMed citations on genomics, clinical trials, systematic reviews and public health. Explore titles, authors and supplied subject terms, then open the PubMed record.

Quote a phrase for an exact phrase match. Source license links do not imply unrestricted reuse.

At least 919 records · Page 51Linked to original sources
PubMed1994

Cellular benign fibrous histiocytoma. Clinicopathologic analysis of 74 cases of a distinctive variant of cutaneous fibrous histiocytoma with frequent recurrence.

We report seventy-four cases of a distinctive variant of cutaneous fibrous histiocytoma, which is often mistaken histologically for sarcoma and which carries a high local recurrence rate. These tumors appeared most commonly in young or middle-aged adults, with a predominance in men (male/female ratio 1.9:1). Anatomic distribution was wide, with cases occurring mainly in the upper limb/limb girdle (34%), lower limb/limb girdle (27%), and head and neck region (20%). Most lesions had been present for only a few months, and their sizes ranged from 0.5 cm to 2.5 cm in maximum diameter. Twelve (26%) of 46 cases with follow-up (mean duration 3 years) recurred locally, in one case twice. Distinctive histologic features were a commonly fascicular growth pattern, predominance of eosinophilic spindle cells with tapering nuclei, a moderate mitotic rate (mean three per 10 high-power fields), and frequent extension into the subcutaneous fat (33% of cases). In addition, all cases showed at least focal cytologic polymorphism (inflammatory cells, foam cells, giant cells), and 58% showed associated epidermal alterations in common with usual cutaneous fibrous histiocytomas. Nine cases (12%) showed foci of central necrosis. Immunohistochemical studies (ABC method) found only vimentin and very focal smooth muscle actin positivity. Tests for CD34, desmin, S-100, keratin, and Factor XIIIa were negative in all cases. These lesions should be distinguished from dermatofibrosarcoma protuberans and leiomyosarcoma, with which many of these cases were initially confused.

Adolescent↗
PubMed1993

[Giant cell fibroblastoma. Apropos of 2 cases].

Two cases of giant cell fibroblastoma (GCF) are reported. One presented as cervical tumor in a 11 year old child and the second localized in axillar region of 14 year old boy. Histologically both showed a typical distinctive appearance of this entity when focal fusocellular cells arranged in a storiform pattern was also constated in one. The immunohistochemical and ultrastructural study ruled out a vascular origin. The clinical and pathological findings suggest that CGF represent a juvenile form of dermatofibrosarcoma protuberans.

Adolescent↗
PubMed1993

The impact of resection margins in the treatment of primary sarcomas of the breast. A clinicopathological study of 8 cases with review of literature.

We reviewed the clinical and pathological features of eight patients (seven females, one male), who underwent surgery for sarcomas of the breast, to evaluate the effect of the margins of resection and histological tumor type on survival. Patients with carcinosarcoma, cystosarcoma phyllodes or dermatofibrosarcoma protuberans were excluded. Tumor size ranged between 4.5 and 26 cm (median 12.8 cm); there were three grade 3, four grade 2 and one grade 1 lesions. Of the five patients treated by radical resection margins, two with large grade 3 sarcomas died of distant metastasis 3 and 10 months later. One patient out of the five radical resected patients developed a local recurrence 96 months later. The recurrence was resected radically, radiotherapy was administered, and the patient is still alive at 168 months. The remaining four radical resected patients are free of disease until now. One of three patients, treated by wide or marginal resection, received adjuvant radiotherapy. This patient developed local recurrence and solitary distant metastasis at 26 and 49 months, respectively, and died of multiple distant metastasis at 54 months. Regional axillary lymph node metastasis was not observed in five patients in whom lymph node dissection was performed, with a median of 14.2 dissected lymph nodes. Two of eight patients received preoperative chemo- or radiotherapy because of large tumor size; five of eight received postoperative radiotherapy. Local tumor control was achieved in six of eight patients. The median follow-up time was 53.9 months (3-168 months), of all survivors median 72.2 months. Recurrence and disease-free survival rates were analysed according to histologic grade, tumor size, stage, surgical and adjuvant treatment.

Adult↗
PubMed1993

[The assessment of tumor blood flow factors using dynamic CT--a comparison with positron emission tomography].

The dynamic computed tomography (CT) was performed by using rapid-sequence scanning following an intravenous bolus injection of contrast material. Time-density curve was applied for Gamma variate curve fitting and CT attenuation values were applied for two compartment model. The value of 1/CM, k1 and k2 were defined as the blood flow factors in this study. The inhalation of C15O2 using positron emission tomography (PET) can be useful for determining regional tumor blood flow (rBF). CT and PET were performed in twelve patients. The diagnosis was malignant lymphoma in four, and others; two liver metastasis, one focal nodular hyperplasia of the liver, one dermatofibrosarcoma, one hepatocellular carcinoma, one malignant melanoma, one malignant meningioma, one bone metastasis. The correlations among rBF, 1/CM, and k1 were good, and better correlations were obtained among tumor blood flow factors and rBF in the case of the tumors which existed far from air way and/or had low blood flow. The distance from air way effected both the value of rBF and tumor blood flow factors. Both the distance from air way and pathology caused conflicting results between rBF and tumor blood flow factors. Dynamic CT was particularly useful for evaluated the blood flow of tumors that had contact with air way.

Adult↗
PubMed1995

New entities, concepts, and questions in childhood tumor pathology.

There are major differences between tumors in children and adults, viz. the incidence of tumor types, the predisposition of certain organs and tissues (e.g. sympathetic nervous tissue, kidney, and soft tissues) to develop tumors, problems related to tumor classification, and the biologic behavior of childhood malignancies, which are usually characterized by high rates of proliferation activity. A large number of new entities, especially in soft tissue tumors, have been published over the past years, including nodular mesothelial hyperplasia, which is a tumor-like lesion derived from peritoneal macrophages; infantile myofibromatosis, which can mimic leiomyosarcoma; intermediate grade fibrohistiocytic tumors, like dermatofibrosarcoma protuberans-related giant-cell fibroblastoma, plexiform fibrohistiocytic tumor and angiomatoid malignant fibrous histiocytoma displaying evidence of myogeneous differentiation; finally, the high-grade intraabdominal desmoplastic small cell tumor. With modern methods we can gain better insights into the biology of tumors. For example, tumors of the Ewing's sarcoma family have in common a characteristic t(11; 22) chromosomal translocation, the Ewing's sarcoma (EWS) (22q12) gene rearrangement, and the MIC2 gene. The EWS gene rearrangement is not restricted to tumors of the Ewing's sarcoma family (classic Ewing's sarcoma and malignant peripheral neuroectodermal tumor), however, but occurs in malignant melanoma of the soft tissue and in intraabdominal desmoplastic small cell tumor. Rhabdomyosarcomas (RMS) can be divided into two basic types with different prognoses: embryonal RMS, including botryoid and spindle-cell variants, and alveolar RMS, including the solid variant. The prognosis of alveolar RMS is poorer than that of classic embryonal RMS, mainly due to early tumor dissemination in alveolar RMS. The prognosis of neuroblastoma is mainly based on chromosomal and molecular biologic findings. Structural chromosome 1 abnormalities, double minute chromosomes, homogeneously staining regions, N-myc amplifications, and DNA diploidy are indications for an unfavorable outcome. Despite progress in childhood solid tumor pathology, many questions remain open, including those relating to basic chromosomal defects in germ cell tumors and the obscure nature of tumor heterogeneity.

Child, Preschool↗
PubMed1996

Argyrophilic nucleolar organizer regions in soft tissue tumors.

Argyrophilic nucleolar organizer region (AgNOR) staining was employed on 51 apparently normal representative soft tissues, 53 benign soft tissues tumors and 52 malignant soft tissue tumors with an aim to study the sensitivity and specificity of method in differentiating between the benign and malignant soft tissue tumors. The mean AgNORs count in apparently normal fibrous tissue was 1.02, whereas it was 0.94 in adipose tissue, 1.14 in smooth muscle tissue, 1.115 in skeletal muscle tissue, 1.025 in blood vessels endothelial lining cells and 1.04 in nerve tissue. The mean AgNOR count was found to be higher in benign soft tissue tumors as compared to respective apparently normal soft tissue and was found to be statistically significant. The mean AgNOR count in soft tissue sarcomas was found to be higher as compared to both apparently normal soft tissue and benign soft tissue tumors. An increase AgNOR score in both benign and malignant soft tissue tumors as compared to apparently normal soft tissue indicates high proliferative activity. The neurofibrosarcoma showed low AgNOR count as compared to other soft tissues sarcomas. The fibrohistiocytic sarcoma, leiomyosarcoma and angiosarcoma showed a mean AgNOR score of 4 or more than four. The mean AgNOR score was found to increase with high grade of the tumor. The AgNOR staining is simple and useful method in estimating tumor cell proliferation thereby differentiating normal soft tissue from non-neoplastic proliferative growth, benign and malignant soft tissue tumors. It may help in differentiating fibromatosis from fibrosarcoma, dermatofibrosarcoma protuberans of low grade malignancy from high grade malignant fibrous histiocytoma and benign hemangiopericytoma from malignant hemangiopericytoma.

Adult↗
PubMed1997

[Experience in the treatment of breast sarcomas].

Authors operated on 6339 malignant breast tumors at the Department of Surgery, National Institute on Oncology between 1980 and 1994. The records of 14 patients with sarcomas of the breast were analyzed. These included 2 malignant phyllodes tumors, 3 malignant fibrous histiocytomas, 2-2 fibrosarcomas and carcinosarcomas and liposarcoma, angiosarcoma, leiomyosarcoma, osteosarcoma and dermatofibrosarcoma protuberans one of each. During this period 5 patients died, 9 are living without evidence of tumor. Analysing these 14 cases authors present their policy in surgical therapy of breast sarcomas compared with literature data. They emphasise the importance of wide resection margins for prevention of tumor progression. The above mentioned principle is valid for local recurrences so radicalization of the previous conservative breast surgery may be necessary in selected cases. Intraoperative histological examination is recommended to verify that the resection margins are tumor free. Axillary block dissection is not necessary except in cases when palpable, firm lymph nodes are present in the region. Depending on the histological grade adjuvant radiotherapy could be considered but its effectiveness is not proven.

Adult↗
PubMed1997

[Perineurioma].

Perineurioma (storiform perineurial fibroma) is a rarely diagnosed benign tumour of perineurial cells. Presented tumour occurred subcutaneously in the right thigh of a 53-year-old man. It was well limited and consisted of whirl like and concentric laminar bundles of spindle and oval cells in variable local density. A storiform pattern was not present. Cells with richer cytoplasm, bland nuclei and solitary intranuclear pseudoinclusions formed focal meningioma like structures. Differential diagnosis had to distinguish benign neuronal tumours, myxoid variant of dermatofibrosarcoma protuberans and especially a "low grade" fibromyxoid sarcoma. A relevant immunohistochemical marker of perineurioma (for differential diagnosis) was the expression of epithelial membrane antigen (EMA) with negativity of S-100 protein.

Diagnosis, Differential↗
PubMed1998

Cytologic features of synovial sarcoma with emphasis on the monophasic fibrous variant: a morphologic and immunocytochemical analysis of bcl-2 protein expression.

BACKGROUND: Monophasic fibrous synovial sarcoma (SS) is the most common variant of SS but few cytologic studies concerning this entity are available. bcl-2 protein immunoexpression has been described as a characteristic marker of SS and is useful for its differentiation from other sarcomas. METHODS: Twelve cytologic specimens from 11 patients with histologically proven SS were reexamined. Ten samples were obtained by fine-needle aspiration and in two cases fluid from cystic tumoral areas was obtained at surgery. Nine cases corresponded to monophasic fibrous tumors and two to biphasic tumors. Immunoexpression of bcl-2 was evaluated in all histologic specimens and in six cytologic samples. bcl-2 protein also was evaluated in 13 soft tissue tumors. RESULTS: All cytologic specimens had a similar appearance. Most smears were highly cellular and were comprised of densely packed, tridimensional groups and numerous isolated, round to oval cells. Remarkable findings included cellular monomorphism and vascular structures within the cell groups. Only one case showed cytologic evidence of epithelial differentiation. Immunoexpression of bcl-2 protein was present in 10 of the 11 histologic specimens (90.9%) and in 5 of the 6 cytologic samples (83.3%). A positive result also was observed in malignant fibrous histiocytoma, dermatofibrosarcoma protuberans, infantile hemangiopericytoma, pleomorphic liposarcoma, and malignant solitary fibrous tumor. CONCLUSIONS: Although cytomorphologic features of SS are characteristic enough to permit its recognition, clinical correlation is necessary for its correct diagnosis. The immunoexpression of bcl-2 protein is not specific of SS and other soft tissue tumors can show a similar positive reaction.

Adolescent↗
PubMed1998

CD117: a sensitive marker for gastrointestinal stromal tumors that is more specific than CD34.

Gastrointestinal stromal tumors (GISTs) represent a distinct and the most important subset of mesenchymal tumors of the GI tract. These tumors are both phenotypically and genotypically different from true leiomyomas and usually express CD34, a hematopoietic progenitor cell antigen. CD34, however, is also present in a wide variety of fibroblastic and endothelial cell tumors. In this immunohistochemical study of CD117, we evaluated 85 cases of GIST and more than 150 other mesenchymal tumors, including leiomyomas and schwannomas. CD117, the c-kit proto-oncogene product, is expressed in subsets of hematopoietic stem cells, mast cells, melanocytes, and interstitial cells of Cajal of the GI tract. CD117 was almost always (85%) expressed in both benign and malignant GISTs. CD117 was observed both in the spindle cell and epithelioid subtypes of GISTs in all locations. In addition to reacting with the CD34-positive GISTs, CD117 was positive in some CD34-negative cases. Approximately one-third of GISTs coexpressed CD117 and smooth muscle actins. In contrast, true leiomyomas (desmin and actin-positive) and schwannomas in both GI and peripheral locations were consistently negative for CD117. Solitary fibrous tumors and Kaposi's sarcomas, which are typically CD34 positive, were consistently CD117 negative. Among the CD34-positive tumors that showed occasional CD117 reactivity were dermatofibrosarcoma protuberans (1 of 7) and hemangiopericytoma (2 of 10). Other mesenchymal tumors that were variably CD 117 positive included clear cell sarcoma (7 of 15), metastatic melanoma (9 of 25), and malignant fibrous histiocytoma (1 of 20). These results indicate that CD117 is a specific marker for GIST among tumors that occur in the GI tract and adjacent regions. CD117 expression also separates GISTs from true leiomyomas and gastric schwannomas.

Adult↗
PubMed1998

[Therapy of non-melanocytic skin tumors].

Actinic keratosis on sun-damaged skin are very common in individuals with fair complexion. Management encompasses cryosurgery, tretinoin or 5-fluorouracil-cream. Bowen's disease, however, requires surgical excision or radiotherapy. Basal cell carcinoma and squamous cell carcinoma are the two most common malignant skin tumours in Western Europe. Typically these tumours can be managed either by excision and primary wound closure, by cryosurgery or by radiotherapy. The method of choice is determined by the type and location of the tumour and the general condition of the patient. For more difficult-to-treat malignant skin tumours surgical resection with histological margin control is required. Mohs' micrographic surgery is a specialized procedure. This method entails to a full work-up of the excisional margins. The defect is closed only after histological verification of tumour-free surgical margins. Difficult-to-treat tumours are recurrent, sclerodermiform and large (diameter more than 20 mm) basal cell carcinomas. Indications for margin control in squamous cell carcinomas are tumours with more than 20 mm of diameter, with more than 5 mm thickness and with poor histologic differentiation (Broders grade III and IV, desmoplastic squamous cell carcinoma). Therefore, a skin biopsy is often required to plan the optimal treatment of a malignant skin tumour. The collaboration of primary care providers and specialists is beneficial in the management of difficult skin tumours. Renal transplant recipients under immunosuppression are prone to have squamous cell carcinoma of a more aggressive type. Dermatofibrosarcoma protuberans is another good indication for Mohs' micrographic surgery. A regular follow-up for recurrences or secondary tumours, as well as an effective secondary prevention of sun damage are important for skin cancer patients.

Bowen's Disease↗
PubMedDate not supplied

p53/MDM-2 immunohistochemical expression correlated with proliferative activity in different subtypes of human sarcomas: a ten-year follow-up study.

The aim of this study was the evaluation of p53/MDM-2 protein overexpression in different subtypes of human sarcomas, and their correlation with proliferative activity and patient outcome. We selected 40 cases of human sarcomas comprising 6 malignant fibrous histiocytomas (MFH), 1 fibrosarcoma, 1 dermatofibrosarcoma protuberans, 5 liposarcomas, 9 leiomyosarcomas, 1 rhabdomyosarcoma, 3 synovial sarcomas, 2 osteosarcomas, 1 chondrosarcoma, 4 Ewing's sarcomas, 2 Kaposi's sarcomas, 1 malignant haemangiopericytoma, 1 phylloides cystosarcoma, 1 neuroblastoma, 1 chordoma and 1 unclassified sarcoma. All the immunohistochemical markers, which had been used for the characterization of these sarcomas were re-examined. Additionally, the Streptavidin-Biotin peroxidase method was performed on paraffin sections using the monoclonal antibodies: anti-p53 antibody DO7, anti-MDM-2 antibody IF2 and anti-Ki-67 antibody MIB-1. According to our results, p53 protein nuclear expression was detected in 20% (8/40) of the tumours (1 fibrosarcoma, 2 liposarcomas, 1 leiomyosarcoma, 1 rhabdomyosarcoma, 2 Ewing's sarcomas and 1 unclassified sarcoma). MDM-2 nuclear staining was determined in 7.5% (3/40) of the cases (1 MFH and 2 liposarcomas). A high proliferative index was demonstrated in 27.5% (11/40) of the tumours (2 MFH, 4 leiomyosarcomas, 1 rhabdomyosarcoma, 1 osteosarcoma, 2 Ewing's sarcomas and 1 unclassified sarcoma). p53 overexpression was associated with high tumour grade (p < 0.05) and MIB-1 expression was correlated with reduced survival (p < 0.05), but p53 overexpression was not significantly associated with either MIB-1 score or with overall survival of the patients. In conclusion, from this limited and heterogeneous sample of cases, we suggest that the p53/MDM-2 pathway is involved in the tumourigenesis of several sarcoma subtypes, but it is unclear if the overexpression of these genes may become prognostic marker for patients affected with these highly aggressive tumours.

Adolescent↗
PubMed2001

Genetics of nonmelanoma skin cancer.

Cancer is in essence a genetic disease characterized by genomic instability. Unlike classic genetic syndromes in which a single inherited mutation is often sufficient to determine the perturbed phenotype, most cancers, especially solid tumors, develop after an accumulation of multiple genetic lesions. Inherited mutations that predispose individuals to cancer formation are termed germline, while acquired mutations that contribute to tumor development are designated somatic. Bona fide hereditary cancers account for only a small proportion of all documented cancers. Most tumors result from mutations caused by inherent infidelities in DNA replication, carcinogens, or defects in the DNA reparative apparatus. When mutations occur in critical growth regulatory genes, variations in cellular proliferation and survival contribute to the selection of dominant tumor population(s). Furthermore, these mutations may alter the antigenic properties of the cancerous cell and encourage escape from the host response. Thus, cancer is evolution at the microscopic level.

Carcinoma, Adenoid Cystic↗
PubMed1995

Skin cancer in African Americans.

Skin cancer is the most common type of malignancy in the United States. Incidence within the African American population remains relatively low, but data is limited for this racial group, making accurate determination of incidence and mortality difficult. Factors implicated as causative in the pathogenesis of cutaneous malignancy in African Americans include, but are by no means limited to, sunlight, albinism, burn scars, X-rays, preexisting pigmented lesions, chronic inflammation, and chronic discoid lupus erythematosus. Anatomic distribution of lesions may be similar to that seen in whites for basal cell carcinoma but not for other skin cancers. For squamous cell carcinoma, melanoma, and cutaneous T-cell lymphoma, African Americans do not as well in terms of mortality as do whites. This difference probably is due either to the fact that African Americans have more advanced stages of disease at diagnosis than do whites or, in some cases, because the course of the disease is more aggressive in African Americans for reasons yet unknown. There is a need for heightened awareness of skin cancer in African Americans by patients and physicians. Emphasis should be on education and early diagnosis with the primary goal in mind being the reduction of incidence of and mortality due to skin cancer in African Americans. In addition, because of environmental factors, African Americans will be exposed to more solar ultraviolet radiation in the future. Strategies should be developed for public education to keep this exposure to low levels in this racial group.

Black People↗
PubMed2000

Radial forearm free tissue transfer for head and neck reconstruction: versatility and reliability of a single donor site.

Since its description as a free flap, the radial forearm flap has undergone numerous modifications for reconstruction of various defects in the head and neck region. Fasciocutaneous, adipofascial, osteocutaneous, tendinofasciocutaneous, or osteotendinofasciocutaneous flaps may be designed and transferred from the radial forearm. This article illustrates the versatility and reliability of this donor site in 15 patients with a variety of head and neck oncologic defects who underwent immediate (12 patients) and delayed (3 patients) reconstruction using different free flaps from the radial forearm. Skin flaps were used in 11 patients (73.3%) with floor of mouth (4 cases), hemiglossectomy (2 cases) and partial maxillectomy (2 cases) defects, and for scalp (1 case), lower lip (1 case) and a central face (anterior maxilla/upper lip/nasal) (1 case) defect. Osteocutaneous flaps were used in four patients (26.6%) for reconstruction of bilateral subtotal maxillectomy defects (2 cases), a complex forehead and nasal defect (1 case), and for mandible reconstruction (1 case). In addition, the palmaris longus tendon was included with the flap in the two patients that required oral sphincter reconstruction. One patient required reexploration due to vein thrombosis, and no flap failures were detected in this series. The donor site healed uneventfully in all patients, except one, who had partial skin graft failure. Because of their multiple advantages, free flaps from the radial forearm have a definite role for reconstruction of head and neck defects. New applications of composite flaps from this donor site may continue to emerge, as illustrated in some of our patients.

Adult↗
PubMed1996

[Giant cell fibroblastoma. A rare soft tissue tumor in childhood].

We report on a 9-year-old girl with a giant cell fibroblastoma of the right anterior chest wall. This rare soft tissue tumour occurs predominantly in the first two decades of life. The typical clinical presentation is a solitary, usually 2-6 cm large, non-tender mass of blue-greyish colour which is mostly located on the back, anterior chest wall, thigh or groin. The histology shows a loose infiltrate of predominantly bland spindle cells in dermis and subcutis. Characteristic elements of the tumour are large angiectoid branching spaces lacking any endo- or epithelium, and relatively small multinucleated cells (floret cells). The recurrence rate is high if the tumour is not excised with adequate safety margins. Metastases are not reported. It is of pre-eminent importance to differentiate this rare benign tumor from sarcomas, in order to avoid an inappropriately aggressive therapy.

Biomarkers, Tumor↗