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 829 records · Page 46Linked to original sources
PubMed2000

Expression of CD34 in sclerotic ("plywood") fibromas.

CD34 antigen is expressed in normal human skin on endothelium, in spindle cells located around adnexal structures, and in a subset of interstitial cells in the reticular dermis. CD34 expression has also been identified in a number of fibrohistiocytic neoplasms, such as dermatofibrosarcoma protuberans and solitary fibrous tumors of soft tissue. CD34 expression has not previously been described in sclerotic, or "plywood" fibromas. Here presented are three lesions from three patients, in which histologic examination revealed a well-circumscribed dermal nodule composed of spindled cells with focal nuclear pseudo-inclusions. There was extensive fibrosis with hypocellular, storiform areas, characteristic of sclerotic fibroma. The spindled cells strongly expressed CD34, but not factor XIIIa or markers of melanocytic, neural, or muscular differentiation. A diagnosis of Cowden syndrome was considered in one of the cases. These cases provide evidence that CD34 expression can occur in sclerotic fibromas, either solitary or associated with Cowden syndrome. When diagnosing a sclerotic fibroma, one should comment in the report regarding the possibility of Cowden syndrome.

Adult↗
PubMed2001

Benign fibrous histiocytoma (dermatofibroma) of the face: clinicopathologic and immunohistochemical study of 34 cases associated with an aggressive clinical course.

Thirty-four cases of fibrous histiocytoma (dermatofibroma) arising on the face are reported. These neoplasms occurred frequently in females (24 female, 10 male) and showed a broad age range (12 to 85 years; mean: 43.6 years, median: 41 years). The neoplasms originated on the forehead (nine cases), the cheek (eight cases), the eyebrow (four cases), the temporal region (three cases), the nose (two cases), and the ear (one case); in seven cases the location face was given only. Five of 27 cases with follow-up information (median: 5 years) recurred locally; in one case four recurrences were excised within 8 years. The majority of cases extended into the subcutis and deep soft tissue including striated muscle (50% of cases). Histologically, only the minority of cases was composed entirely of histiocytoid and spindle-shaped tumor cells arranged in a storiform growth pattern. In many cases cellular fascicles and bundles of spindle-shaped tumor cells were noted in addition to classical morphological features of fibrous histiocytoma. A moderate mitotic rate (mean: 2.97 mitoses in 10 HPFs) was observed, and in few cases increased atypia was evident. Frank tumor necrosis and/or vascular invasion were not identified. Immunohistochemical studies revealed Factor XIIIa positivity in 13 out of 17, focal CD68 positivity in 6 out of 10, and alpha-smooth muscle actin positivity in 16 out of 19 cases tested. These lesions should be distinguished from dermatofibrosarcoma protuberans, including its fibrosarcomatous variant, leiomyosarcoma, and low-grade myofibroblastic sarcoma. Cases of fibrous histiocytoma of the face have to be excised with wider margins in comparison with examples of classical fibrous histiocytoma occurring on the extremities because of diffuse infiltration, involvement of deeper structures, and an increased rate of local recurrences.

Actins↗
PubMed2004

Cutaneous and subcutaneous fibrohistiocytic tumors of intermediate malignancy: an update.

The fibrohistiocytic tumors of intermediate malignancy are uncommon mesenchymal tumors, which typically occur in the skin and subcutis and which may pose significant problems for the dermatopathologist. This article reviews the clinical, histopathologic, and genetic features of dermatofibrosarcoma protuberans, giant cell fibroblastoma, angiomatoid (malignant) fibrous histiocytoma, plexiform fibrous histiocytoma, and soft tissue giant cell tumor (of low malignant potential). The differential diagnosis of these tumors with a variety of benign and fully malignant cutaneous soft tissue neoplasms is discussed.

DNA, Neoplasm↗
PubMed1994

Spindle cell liposarcoma, a hitherto unrecognized variant of liposarcoma. Analysis of six cases.

A series of six cases of a previously unrecognized variant of liposarcoma characterized by a prominent spindle cell component is reported herein. Clinically, all of the tumors arose in adults and developed around the shoulder girdle or upper limbs; all but one arose in subcutaneous tissue. Three patients developed multiple local recurrences over a period of 4-20 years. Recurrences in one case were purely lipoma-like. Following dedifferentiation in a recurrence, one patient developed distant metastases and eventually died, 46 months after the primary excision. Grossly, these lesions are characterized by multinodularity, and microscopically they show a relatively bland spindle cell proliferation arranged in fascicles and whorls and set in a variably myxoid stroma. The spindle cell areas are accompanied by an adipocytic component, which exhibits the morphologic features required for inclusion in the well-differentiated liposarcoma-atypical lipoma group, including definite lipoblasts. Main differential diagnoses include benign lesions such as spindle cell lipoma and diffuse neurofibroma, as well as dermatofibrosarcoma protuberans and other malignancies such as sclerosing liposarcoma, low-grade myxofibrosarcoma, low-grade malignant peripheral nerve sheath tumor, and low-grade fibromyxoid sarcoma. In view of their distinctive histologic appearance, and because aggressive clinical behavior was observed despite their superficial location, we propose that these lesions be regarded as spindle cell variants of well-differentiated liposarcoma.

Adult↗
PubMed1994

Solitary fibrous tumor. Consistent CD34 immunoreactivity and occurrence in the orbit.

The distinction of solitary fibrous tumors (SFTs) from histologically similar neoplasms relies heavily on a characteristic microscopic appearance. No discriminating ultrastructural or immunohistochemical features are known. We evaluated 22 SFTs and 118 other tumors often considered in the differential diagnosis for immunoreactivity using a monoclonal antibody directed against the human hematopoietic progenitor cell antigen, CD34. All the SFTs (22 of 22, 100%) demonstrated strong CD34 immunoreactivity, irrespective of tumor site and histologic grade. Strong and generalized CD34 positivity was also found in most dermatofibrosarcoma protuberans (11 of 12, 92%) and occasional smooth-muscle tumors (leiomyomas 2 of 11, 18%; leiomyosarcomas 2 of 11, 18%). Variable numbers of CD34 positive cells were present in all neurofibromas (9 of 9, 100%) and focally present in most schwannomas (8 of 9, 89%). Some of the hemangiopericytomas (7 of 16, 44%) exhibited CD34 immunoreactivity, however, generally with weak intensity and patchy distribution. CD34 immunoreactivity was not observed in mesotheliomas (0 of 20, 0%), synovial sarcomas (0 of 13, 0%), fibrosarcomas (0 of 12, 0%), or spindle-cell thymomas (0 of 5, 0%). We conclude that CD34 immunoreactivity is a sensitive marker for SFT and, in conjunction with an appropriate immunohistochemical panel, may be useful in discriminating SFTs from other histologically similar neoplasms. The observation that some mesenchymal stromal cells and SFTs share a CD34-positive immunophenotype suggests a histogenetic relationship. The inclusion in this study of two cases of SFTs arising in the orbit establishes another site of origin for this tumor and provides further support for a mesenchymal histogenesis.

Antibodies, Monoclonal↗
PubMed1997

Immunoreactivity for the human hematopoietic progenitor cell antigen (CD34) in lipomatous tumors.

The human hematopoietic progenitor cell antigen (CD34) recently was shown to react with a variety of nonhematopoietic tissues and their tumors, including vascular endothelium, dendritic interstitial fibroblastic cells, and endoneurial cells as well as with the neoplastic cells in a variety of mesenchymal neoplasms of unknown etiology, such as Kaposi's sarcoma, dermatofibrosarcoma protuberans, epithelioid sarcoma, gastrointestinal stromal tumors, and solitary fibrous tumors. Additionally, it has been claimed that normal adipocytes may also react with this antibody. We studied a series of 90 lipomatous lesions to examine the pattern of immunoreactivity of the CD34 antigen in adipose tissue neoplasms. The study included 14 lipomas, 19 angiolipomas, 4 atypical lipomas, 18 spindle cell lipomas, 3 renal angiomyolipomas, 1 intramuscular lipoma, and 31 liposarcomas. Immunostains identified a network of CD34+ spindle cells admixed with the adipose tissue elements in all cases of lipoma, angiolipoma, angiomyolipoma, intramuscular lipoma, and well-differentiated lipoma-like liposarcoma. Additionally, the spindle cell component in all cases of spindle cell lipoma were strongly positive for this antigen. Atypical, stellate spindle cells and multinucleated "floret" cells in all cases of atypical lipoma as well as in six of 12 cases of well-differentiated lipoma-like liposarcoma of deep soft tissue were also positive for CD34. Scattered spindle cells in all cases of myxoid liposarcoma and in one case of round cell liposarcoma, as well as the sarcomatous component in one case of "dedifferentiated" liposarcoma, were strongly positive for this antigen. The round cells in myxoid liposarcoma and round cell liposarcoma, the signet-ring and multivacuolated lipoblasts in well-differentiated liposarcoma, and the pleomorphic atypical cells in pleomorphic liposarcoma were uniformly negative. The results of this study appear to indicate that lipomatous tumors may harbor a population of CD34+ interstitial dendritic spindle cells. Overgrowth or clonal expansion of this dendritic cell subpopulation may account for the development of spindle cell lipomas and for the spindle cell component in some cases of "dedifferentiated" liposarcoma.

Antigens, CD34↗
PubMed1998

Expression of bcl-2 oncoprotein in benign and malignant spindle cell tumors of soft tissue, skin, serosal surfaces, and gastrointestinal tract.

An immunohistochemical study to determine the pattern of immunoreactivity for bcl-2 oncoprotein was performed in 380 spindle cell tumors of soft tissue, skin, serosal surfaces, and gastrointestinal tract. The cases studied included examples of benign, reactive spindle cell proliferations to benign and malignant spindle cell neoplasms, including nodular fasciitis (10), fibromatosis (5), dermatofibroma (10), dermatofibrosarcoma protuberans (18), Kaposi's sarcoma (15), spindle cell lipomatous tumors (24), benign and malignant smooth muscle tumors (35), neural/peripheral nerve sheath neoplasms (53), synovial sarcomas (70) solitary fibrous tumors of serosal surfaces and other sites (56), gastrointestinal stromal tumors (GIST) (47), and malignant undifferentiated fibroblastic spindle cell proliferations of soft tissue (37 cases). The results of bcl-2 staining was additionally correlated with CD34 immunoreactivity. Bcl-2 was uniformly negative in all cases of nodular fasciitis, fibromatosis, and dermatofibroma, as well as in benign and malignant smooth muscle proliferations. Strong positivity for bcl-2 was observed in all cases of spindle cell lipoma, dendritic fibromyxolipoma, Kaposi's sarcoma, solitary fibrous tumors, gastrointestinal stromal tumors, and in the spindle cell component of synovial sarcoma. With the exception of the last, there appeared to be a close correlation between the expression of bcl-2 and CD34 in these tumors. Strong bcl-2 positivity also was found, at least focally, in approximately one third of benign and malignant peripheral nerve sheath tumors, particularly in the better-differentiated (Antoni type A) areas. Sarcomas of fibroblastic type, including low-grade myxofibrosarcoma, malignant fibrous histiocytoma, and fibrosarcoma, showed variable expression of bcl-2 in the tumor cells. Our results appear to indicate that bcl-2 may have a wide distribution among benign and malignant spindle cell neoplasms. Strong expression of this marker in some of these conditions, particularly solitary fibrous tumor, gastrointestinal stromal tumors, and synovial sarcoma, may be of aid for differential diagnosis.

Antigens, CD34↗
PubMed2000

Pigmented (melanotic) neurofibroma: a clinicopathologic and immunohistochemical analysis of 19 lesions from 17 patients.

Neurofibromas with melanin-laden pigmented cells are rare, accounting for less than 1% of all neurofibromas accessioned to the Soft Tissue Registry of the Armed Forces Institute of Pathology between the years 1970 and 1996. This study analyzes the clinicopathologic features associated with 19 specimens removed from 17 patients. Eleven males and six females, ranging in age from 2 to 61 years (median, 28 years), participated in the study. Nine of 15 patients whose race was provided were black. Eight patients (47%) are known to have neurofibromatosis, and two others (12%) are strongly suspected of having this disorder; two patients have similarly affected family members. Eight patients were noted to have multiple skin tumors, and in each of two cases, two pigmented neurofibromas were available for review. Two patients had hypertrichosis and cutaneous hyperpigmentation resembling a hairy nevus, and one had a café au lait spot directly overlying a pigmented neurofibroma. Tumors ranged in size from 1.7 to 50 cm in greatest dimension and involved the buttock or leg (n = 6), head or neck (n = 8), trunk (n = 2), wrist or hand (n = 2), and an unspecified site (n = 1). The neurofibromas exhibited diffuse (n = 15), combined diffuse and plexiform (n = 2), combined diffuse and intraneural epithelioid (n = 1), and nonspecific (n = 1) growth patterns. The process involved the skin (n = 14), subcutis (n = 18), and/or skeletal muscle (n = 3). Wagner-Meissner-like bodies were identified in 11 tumors, and mitoses (average, less than one mitosis per 10 high-power fields) were present in three lesions. All examples contained scattered pigmented cells with dendritic, tadpole-shaped, spindled or epithelioid morphology. These cells were positive with Fontana-Masson (nine of nine) and Warthin-Starry (pH, 3.2; four of four) stains, and were depigmented with a melanin bleach method (two of two). An iron stain was negative. The tumors had immunoreactivity for S-100 protein (11 of 11), HMB-45 ( 10 of 11), Melan-A (four of four), tyrosinase (four of four), and CD34 (four of four). Although recurrences are documented, none of the tumors are known to have undergone malignant transformation. A pigmented neurofibroma can be confused with a pigmented dermatofibrosarcoma protuberans (Bednár tumor) because the melanin-laden cells of both processes are similar. However, the latter entity exhibits a more extensive storiform growth, has greater immunoreactivity for CD34, and lacks a diffuse proliferation of S-100 protein-positive Schwann cells.

Adolescent↗
PubMed1987

The ubiquitous fibroblast. Multiple oncogenic potentials with illustrative cases.

The wide range of oncogenic proliferative potentials of the fibroblast is demonstrated with a series of eight patients. Diagnoses included infantile digital fibromatosis, "aggressive fibromatosis," aggressive fibromatosis progressing to poorly differentiated sarcoma, infantile myofibromatosis, recurrent desmoid tumor, fibrosarcoma arising in a keloid, dermatofibrosarcoma protuberans, and malignant fibrous histiocytoma of left atrium. Still other types of fibroblastic tumefactions might have been included. Oncogenic factors that may have been operative in the causation of the lesions presented include: genetic factors, sex-linked factors, hormonal factors, numerous growth factors, and certain viruses, especially retroviruses. Certain fibromatoses in children are commonly self-limited and need only be monitored carefully as the process regresses. Aggressive fibromatosis, on the other hand, can prove fatal if the lesion is not completely resected with a wide margin and, occasionally, the process may become frankly malignant, with metastases. The standard triad of excisional surgery, radiotherapy, and chemotherapy has been used to treat frankly malignant fibrous tumors with variable results.

Adult↗
PubMed1995

Cytogenetics and experimental models of sarcomas.

Bone and soft tissue sarcomas are diagnostically challenging. Recognition of specific cytogenetic abnormalities in these neoplasms has significantly reduced some of the associated difficulties and has provided valuable information on histopathogenesis. Commonly, translocations involving an exchange of chromosomal material and creation of novel chimeric genes are detected. These fusion genes frequently function as aberrant transcription factors that contribute to sarcomagenesis. New studies indicate that less commonly occurring variant fusion genes are also present in some tumors, eg, Ewing's sarcoma and alveolar rhabdomyosarcoma. The clinical consequences, if any, of these variant hybrids are not yet known. Reverse transcriptase polymerase chain reaction and are useful approaches in detecting these transcripts. In addition to translocations, supernumerary ring chromosomes are often encountered in sarcomas, particularly those of intermediate or borderline malignancy. Traditional fluorescence in situ hybridization, and recently, comparative genomic hybridization have uncovered the chromosomal composition of these rings as well as some associated gene amplifications in well-differentiated liposarcoma and dermatofibrosarcoma protuberans.

Animals↗
PubMed2001

Signal transduction pathways in sarcoma as targets for therapeutic intervention.

Investigations into the molecular alterations in sarcomas have made substantial progress during the past decade. Classical linkage analysis and the direct sequencing of chromosomal translocation fusions have identified candidate genes in many different sarcomas. A large group of these genes participate in signal transduction pathways and represent potential sites of disease intervention with targeted therapies. This review will discuss five types of sarcoma that display aberrant tyrosine kinase pathway signaling: gastrointestinal stromal tumor, inflammatory myofibroblastic tumor, congenital fibrosarcoma and mesoblastic nephroma, dermatofibrosarcoma protuberans, and desmoplastic small round cell tumor; one sarcoma predisposition syndrome with specific dysregulation of the ras pathway--neurofibromatosis--will also be discussed.

Antineoplastic Agents↗
PubMed2002

Chromosomal translocations and sarcomas.

This review examines how the identification of tumor-specific translocations and fusion proteins has advanced the basic scientific and clinical understanding of sarcomas. Recent genetic advances, including the ASPL-TFE3 fusion of alveolar soft part sarcoma, the JAZF1-JJAZ1 fusion of endometrial stromal sarcoma, and HMGIC fusions in liposarcoma, are discussed. Next, the review addresses the ways in which molecular genetic data have influenced diagnostic and prognostic paradigms. For example, recent studies describe the detection of occult tumor cells and the identification of primary renal neoplasms that are genetically related to alveolar soft part sarcoma. In addition, the review discusses potential therapies based on the targeting of sarcoma-specific fusion proteins. These reports describe the potential use of Gleevec (STI571) for dermatofibrosarcoma protuberans and the use of tumor-specific fusion proteins as potential targets for immunotherapy. Finally, basic scientific findings are reviewed that elucidate, for example, the aberrant functions of SYT-SSX in chromatin remodeling and of EWS-FLI1 in transcription and mRNA splicing. These and other emerging models of tumorigenesis will help identify new therapeutic targets.

Antineoplastic Agents↗
PubMed2006

Review of monitoring free muscle flap transfers in reconstructive surgery: role of 99mTc sestamibi scintigraphy.

BACKGROUND: Free tissue transfer is a method of moving any tissue from a donor area of the body to a recipient site and re-attaching the arteries and veins to the blood vessels at the recipient site by microvascular surgery. Improved microsurgical techniques have resulted in a high percentage of successful free tissue transfers. Post-operative monitoring of tissue viability can detect early problems in free tissue transfer which may allow early intervention and salvage. Although many flap monitoring methods have been described, there is still no consensus on which of these techniques will become the standard accepted method for monitoring free muscle flaps. OBJECTIVE: In present study, we investigated the use of 99mTc sestamibi scintigraphy in determining free muscle flap viability and complications, and also in directing treatment. METHODS: Thirteen patients were examined prospectively during the post-operative period after free tissue transfer for foot defects. The cause of the defect was diabetic foot ulcer in 10 patients, dermatofibrosarcoma in one patient, squamous cell carcinoma in one patient and gunshot wound in one patient. Foot defect covering was carried out with a free latissimus dorsi muscle flap and skin graft (n=12) and a free gracilis muscle flap (n=1). All patients were examined with a monitoring system that consisted of visual inspection, hand-held Doppler ultrasonography and scintigraphic examinations. Scintigraphic imaging of all cases was performed routinely within the first 48 h post-operatively, and also on days 10 and 91 in two patients. RESULTS: There were four flap failures during the study. One of these patients had viable findings upon visual inspection and no evidence of vascular compromise on Doppler at the first examination. In the other patient, visual inspection of the flap showed that it was ischaemic in one region, but there was no vascular compromise on Doppler examination. Scintigraphic images of each of these patients showed a partial hypoperfused area in the flap region. Later, these two flaps showed positive clinical indications of hypoperfusion (colour of muscle and appearance of skin graft) and Doppler abnormalities. The remaining two patients had non-viable scintigraphic images as well as positive clinical indicators of hypoperfusion and evidence of vascular compromise on Doppler. Nine patients each had a viable flap. In these patients, all three examination tools demonstrated that the flaps were totally viable and there were no vascular complications. CONCLUSION: According to the results of this study, 99mTc sestamibi scintigraphy appears to be a feasible and promising method in the evaluation of free muscle flap viability and complications. On the other hand, to demonstrate any impact on management or patient outcome, further evaluation of 99mTc sestamibi imaging, including comparative studies with different established methods in a larger patient population, is highly recommended.

Adult↗
PubMed1995

Free tissue failure is not an all-or-none phenomenon.

A common misconception among microsurgeons is that free tissue failure is an "all-or-none" phenomenon. In other words, there is an instantaneous cessation of blood flow to a flap primarily due to thrombosis at the arterial or venous anastomotic site (primary thrombosis) with complete flap loss as a result. Contrary to this belief, we have found that free tissue transfers occasionally "die" a slow, progressive, and partial death. This most likely is due to gradual "shutting down" of the microcirculation by the showering of microemboli downstream from the arterial anastomosis (secondary thrombosis). We discuss our clinical experience during the past 6 years with 10 patients in whom free flap failure was not an all-or-none phenomenon and describe the expectant management of these failing free flaps. Microvascular reconstruction of the lower extremities was performed for recalcitrant sickle cell ulceration, chronic venous stasis ulceration in a patient with anti-thrombin III deficiency, dry gangrene of the plantar surface of the foot and toes secondary to posterior tibial artery injection in an intravenous drug abuser, Gustillo type IIIc injury requiring reconstitution of the femoropopliteal artery after an automobile bumper injury, Gustillo type IIIb injury resulting from gunshot wounds, an open ankle joint in a patient with severe peripheral vascular disease, and osteomyelitis. Other cases included cheek soft tissue reconstruction after wide resection of a recurrent dermatofibrosarcoma protuberans of the parotid gland, chest wall coverage after claviculectomy for osteoradionecrosis, and thumb replantation complicated by refractory vasospasm. Latissimus dorsi (four patients), rectus abdominis (three patients), scapular (one patient), and radial forearm fasciocutaneous (one patient) flaps were used. Flap compromise was detected between 4 hours and 6 weeks after surgery. Salvage attempts included Fogarty thrombectomy (four patients), anastomotic revision (two patients), streptokinase instillation (one patient), and leech application (one patient). Failing free flaps were managed expectantly with the use of daily dressing changes. This allowed for the survival of sufficient soft tissue to effect coverage of exposed bone, tendon, or joint. Hyperbaric oxygen was not administered. Tangenital excision of the eschar was performed between 6 and 51 days after free tissue transfer. Skin grafting was delayed for 15 to 80 days. In all cases, a successful outcome was achieved ultimately by either a single skin-grafting procedure (seven patients) or groin flap coverage (one patient). Follow-up ranged from 2 to 35 months.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗
PubMed2006

Microarray analysis of sarcomas.

Microarrays began to be used to study gene expression profiles in the mid-1990s, but it was only after 2000 that serious attempts have been made to apply this technology to investigate sarcomas. Microarray technologies provide a comprehensive survey of active molecular pathways and potential molecular targets for diagnosis and treatment, but are challenging to use because of issues of specimen collection, cost, and complexities in experimental design and data analysis. As a discovery-based technique, microarray analyses are most valuable when framed around specific gaps in our knowledge of tumor etiology and progression, challenges in differential diagnosis, and pressing therapeutic needs. To date, microarray analyses of sarcomas support their division into molecularly defined and molecularly heterogeneous categories, and have provided useful diagnostic markers for entities such as gastrointestinal stromal tumors, synovial sarcoma, and dermatofibrosarcoma protuberans. Signatures predicting outcome and response to therapy have been published for Ewing sarcoma and osteosarcoma, and receptor tyrosine kinase expression patterns have suggested novel therapeutic approaches which may be applied to several types of sarcoma. Nevertheless, results need to be interpreted in the context of histopathology and validated by complementary technologies and/or other research groups.

Biomarkers, Tumor↗
PubMedDate not supplied

Locoregional treatment for adult soft tissue sarcomas of the head and neck: an institutional review.

PURPOSE: The purpose of this study was to review treatment results for primary soft tissue sarcomas of the head and neck in order to determine prognostic factors. PATIENTS AND METHODS: From 1970 to 2000, 44 adult patients were diagnosed with a biopsy-proven, nonmetastatic primary soft tissue sarcoma in a head and neck subsite; were treated with curative intent; and had adequate follow-up and records for our review. Patients with extraosseous Ewing's sarcoma, Kaposi's sarcoma, rhabdomyosarcoma, dermatofibrosarcoma protuberans, and desmoid tumor were excluded. The most common tumor histologies included malignant fibrous histiocytoma (15 patients), angiosarcoma (nine patients), fibrosarcoma (six patients), and leiomyosarcoma (six patients). RESULTS: The median overall survival for all patients was 79 months. The actuarial 5-year local control for all patients was 55% and was highly correlated with the extent of surgical excision: 25% for subtotal resection/debulking, 65% for wide local excision, and 100% for radical excision. Local control at 5 years was 60% for patients treated with both surgery and radiotherapy, 54% for those treated with surgery alone, and 43% for those treated with radiotherapy alone. Adjuvant radiotherapy significantly improved the local control rates (from 25% to 54%) for patients with close (<2 mm) or positive surgical margins. Of 14 patients with locoregional failure in whom salvage was attempted, nine (64%) were rendered disease free. CONCLUSIONS: Multimodality therapy with both surgery and radiotherapy improves local control, particularly in patients with close or positive surgical margins. Aggressive attempts at salvage therapy for locoregional failures are warranted and frequently produce long-term disease control.

Adolescent↗
PubMed2006

Rippled-pattern sebaceoma: a report of a lesion on the back with a review of the literature.

A 68-year-old Japanese man presented with a nodule that had been present for 5 to 6 years on the right side of the back. Physical examination revealed a dome-shaped, 12 x 13-mm, dark red nodule. It was excised with a 2 to 3-mm margin. The patient remained free of disease during 77 months of follow-up. Microscopic examination revealed a bulb-like tumor in the dermis, contiguous with the overlying epidermis. It was composed of small, monomorphous, cigar-shaped basaloid cells in linear, parallel rows, resembling the palisading of nuclei of Verocay bodies, and presenting a rippled-pattern. There were scattered cells showing sebaceous differentiation with vacuolated cytoplasm and scalloped nuclei. There were tiny, duct-like spaces. The tumor revealed characteristics of rippled-pattern sebaceoma. The present case is the first reported rippled-pattern sebaceous neoplasm on the back. Many spindle cell tumors, such as basal cell carcinoma, pleomorphic adenoma, dermatofibrosarcoma protuberans, myofibroblastoma, and leiomyoblastoma, in addition to trichoblastoma and sebaceoma, can have a rippled-pattern.

Aged↗
PubMed2006

Soft-tissue sarcomas of the head and neck: a retrospective analysis of the Alberta experience 1974 to 1999.

BACKGROUND: Soft-tissue sarcomas (STS) of the head and neck constitute a heterogeneous group of rare malignant tumors occurring in an uncommon site. The most common subtypes of STS in the head and neck are malignant fibrous histiocytoma, dermatofibrosarcoma protuberans, and fibrosarcoma. Evidence based subtype-specific treatment decisions are often not possible. METHODS: The medical records of 110 patients diagnosed with head and neck sarcomas were reviewed. All were treated at one of the two major Cancer Centers in Alberta, Canada, between 1974 and 1999. Potential prognostic factors including age, sex, tumor size, histology, grade, tumor location (superficial or deep), and use of adjuvant treatment were evaluated. Cox proportional hazards models were developed to study the impact of these covariates on survival. RESULTS: The median duration of follow-up was 61.5 months. Five year overall, disease specific, and relapse free survival were 65.8%, 83.4%, and 74.2%, respectively. With use of a Cox proportional-hazards model, tumor stage and grade were important prognostic factors affecting survival. CONCLUSIONS: Tumor size and grade were important prognostic factors affecting survival. Tumor location in relation to the superficial fascia (depth) was the best predictor of outcome. The overall and disease-free survival in this patient group was excellent. However, this likely caused by the high proportion of patients with low-grade tumors in our study.

Adolescent↗