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PubMed1994

[Increased incidence of sarcoma in patients treated for seminoma].

In a nationwide cancer registry analysis of second primary malignancies in 6187 men with testicular cancer in the period 1943-1987, 13 sarcomas were found, yielding a 4-fold increase of the relative risk (RR). The majority of sarcomas occurred in men with seminoma, and the increased incidence was seen irrespective of time since the diagnosis of testicular cancer. The interval between the testicular cancer and the sarcoma varied from five to 34 years. After investigation of the hospital records and re-examination of the histological specimens, three patients were excluded. In spite of this, the RR was still considerably increased (at least three-fold). Seven of the 10 sarcomas were found to be located within the field of the radiation treatment administered and three at the periphery. The histological type of sarcomas varied, with leiomyosarcoma as the most frequent (three cases), followed by malignant fibrous histiocytoma (two cases) and fibrosarcoma (two cases). Since none of these patients had received chemotherapy and did not harbour sarcomatous components within the primary testis cancer, the sarcomas were probably caused by the radiation treatment. The absolute number of these secondary sarcomas is low, but the risk of developing such neoplasms and other malignancies should, even so, be kept in mind in the follow-up of testicular cancer patients.

Adolescent↗
PubMed1994

Metastatic alveolar soft part sarcoma presenting as a dural-based cerebral mass.

Sarcoma metastatic to the brain is uncommon and rarely occurs as the initial manifestation of tumor. Alveolar soft-part sarcoma, a rare but well studied subtype of a soft tissue sarcoma with a propensity for central nervous system invasion, presenting with brain metastases, has been reported only once previously. We report the case of a 28-year-old man who presented with partial seizures and who was found to have a homogeneously enhancing frontal lesion on a broad dural base disclosed by computed tomography. preoperatively, the lesion was thought to be a meningioma. The tumor was excised easily and had features typical of an alveolar soft-part sarcoma, which were revealed by light and electron microscopy as well as immunohistochemical analysis. Multiple lung nodules compatible with metastases were found on a chest film. Meningeal dissemination has been reported in a variety of sarcoma types, including rhabdomyosarcoma, fibrosarcoma, and leiomyosarcoma. We add alveolar soft-part sarcoma to this list and suggest that increased recognition of the propensity for these tumors to exhibit metastatic spread to the dura should eliminate diagnostic confusion and provide an earlier diagnosis of these rare lesions. The patterns of spread in metastatic sarcoma deserve further study.

Adult↗
PubMedDate not supplied

Kaposi's sarcoma in organ transplant recipients. The Collaborative Transplantation Research Group of Ile de France.

OBJECTIVES: Kaposi's Sarcoma (KS) is a tumour of multicentric origin with increased frequency after organ transplantation. To date, only North American data from the Cincinnati Transplant Tumor Registry have given some information about this disease in organ transplant recipients, but its true prevalence still has to be determined. In order to analyze Kaposi's sarcoma after kidney, liver and heart transplantation, we performed a retrospective study using the oldest registry of organ transplant recipients in Europe. METHODS: Among all 7923 organ transplant recipients recorded in the Groupe Collaboratif de Recherche en Transplantation de l'Ile de France (GCIF) registry from 1968 to 1990, we analyzed the prevalence and the clinical characteristics of Kaposi's sarcoma in 6229 kidney, 727 liver and 967 heart transplant recipients. In the subgroup of kidney transplant recipients, we assessed the role of cyclosporine on disease evolution. RESULTS: Overall prevalence of Kaposi's sarcoma after organ transplantation was 0.52%, but it was significantly higher among liver (1.24%) than among kidney (0.45%) and heart (0.41%) transplant recipients. Chronic hepatitis B surface antigen carriers were more frequent in liver than in kidney transplant recipients who developed Kaposi's sarcoma (66% vs 21%, p < 0.03). Following kidney transplantation, Kaposi's sarcoma was more severe in patients receiving cyclosporine (n = 16) when compared with those under conventional immunosuppression (n = 12). CONCLUSION: True prevalence of Kaposi's sarcoma among European transplant recipients is high (0.52%) and appeared significantly higher in liver compared with other organ transplant recipients. Cyclosporine seems to increase severity of the disease among kidney transplant recipient.

Adult↗
PubMed1994

Mutation and overexpression of the p53 tumor suppressor gene frequently occurs in uterine and ovarian sarcomas.

OBJECTIVE: To determine the frequency of mutation and overexpression of the p53 tumor suppressor gene in female genital tract sarcomas. METHODS: Immunostaining for p53 was performed in frozen sections of 46 ovarian and uterine sarcomas. Single-stranded conformation polymorphism analysis of exons 4-9 of the p53 gene was performed in 33 sarcomas. We performed DNA sequencing of the p53 gene in 22 cases in which we found p53 protein overexpression and/or shifted bands on single-stranded conformation polymorphism analysis. RESULTS: Overexpression of p53 was seen in 27 of 46 sarcomas (59%), including 26 of 41 (63%) mixed mesodermal tumors, one of four (25%) leiomyosarcomas, and zero of one endometrial stromal sarcoma. Among the 33 sarcomas subjected to molecular analysis, 21 demonstrated mutations in the p53 gene (64%). Eighteen cancers had a single mutation, whereas three cases showed two mutations in the p53 gene. There was one mutation in exon 4, seven mutations in exon 5, three mutations in exon 6, six mutations in exon 7, six mutations in exon 8, and one mutation in exon 9. With the exception of one microdeletion, which predicted a truncated protein product, all of the mutations were missense point mutations. All but one of the point mutations resulted in changes in the predicted amino acid sequence. There were 18 transition mutations (75%), five transversions (21%), and one deletion (4%). CONCLUSIONS: Mutation of the p53 tumor suppressor gene, with resultant overexpression of p53 protein, frequently occurs in ovarian and uterine sarcomas. Because most of the mutations are transitions, p53 mutations in these cancers likely arise from spontaneous errors in DNA synthesis and repair rather than from exposure to carcinogens.

Adult↗
PubMed1996

Soft tissue sarcoma metastasis from clonal expansion of p53 mutated tumor cells.

Although soft tissue sarcoma has a high incidence of p53 mutations, it is not clear if such alterations facilitate tumor growth and metastasis. In this study, fresh autologous normal lymphocytes, normal muscle, primary and metastatic sarcoma tissues from a single synovial sarcoma patient were examined for p53-related alterations that potentially associated with sarcoma tumor development and metastasis. Normal tissues contain two wild-type p53 alleles. Primary sarcoma had one chromosome 17p p53 allelic deletion without apparent p53 mutation in the other allele. However, metastatic tumor had deletion of one p53 allele with an exon 5 codon 135 missense mutation in the other allele. This p53 gene point mutation in the metastasis was associated with the production of mutated p53 protein. A small clone of cells harboring the identical p53 gene point mutation was identified in the primary tumor using mutant allele specific PCR amplification, albeit at levels much less than in the metastatic sarcoma. This single patient example indicate that soft tissue sarcoma metastasis can develop from clonal expansion of primary tumor cells bearing p53 mutations.

Base Sequence↗
PubMed1996

[Prognostic factors of bone and soft tissue sarcoma].

The prognosis of bone and soft tissue sarcoma is influenced by factors such as pulmonary metastasis, local curability and sensitivity to chemotherapy. In cases with pulmonary lesion, the prognosis has not been favorable, but recently long-term survivors are increasing in number by effective chemotherapy, and the removal of primary and metastatic lesions. As in cases without metastatic lesions, the prognosis of high-grade sarcoma were influenced by local curability and sensitivity to chemotherapy. On the other hand, the prognosis in low grade sarcoma is mainly decided by local curability. To assure local curability the following should be considered. 1) safety surgical margin, 2) preoperative radiation therapy if the safety margin is not predicted, and 3) risk factors in local curability, which are lymph node metastasis, skip metastasis and tumor thrombus. If these risk factors are detected before operation, a more radical procedure should be planned. Moreover, cutaneous angiosarcoma and epithelioid sarcoma, which are occasionally associated with these risk factors, should be operated by ablative procedure even if the risk factors are not detected. 4) In cases with pathological fracture or inadequately operated, surgical intervention to prevent transplantation should be undertaken as soon as possible. Effective chemotherapy could improve the prognosis of high grade sarcoma. Thus, chemotherapy should be used for osteosarcoma, Ewings sarcoma, round cell sarcoma etc. However, non-effective chemotherapy might make the prognosis worse. Therefore, alternative administration of effective chemotherapeutic agents proved by clinical evaluation would be more reasonable than remaining with a certain protocol.

Bone Neoplasms↗
PubMed1996

Molecular and immunohistochemical identification of p53 alterations in bone and soft tissue sarcomas.

p53 has been shown to suppress tumor growth by regulating the cell cycle and by triggering apoptosis. Acquired somatic mutations of the p53 gene have been observed in a variety of human malignancies, and these result in a loss of its tumor suppressor function. To examine the occurrence of p53 abnormalities in bone and soft tissue sarcomas, 113 tumors were subjected to molecular analysis and mutations were confirmed in 16 tumors. The frequency of p53 alterations varied among the different subtypes of bone and soft tissue sarcomas, being observed predominantly in osteosarcomas (8/34 cases), rhabdomyosarcomas (2/3 cases), Ewing's sarcomas (1/5 cases), and liposarcomas (3/21 cases). In contrast, p53 gene mutations were detected at a lower frequency in malignant fibrous histiocytomas (2/34 cases) and not at all in nine chondrosarcomas and five leiomyosarcomas. Immunohistochemical staining of p53 protein was performed on 69 cases and compared to the DNA results. For 64 cases the results were concordant: 56 sarcomas were considered to have wild-type p53 by both techniques. As well, increased p53 protein expression was observed in eight of the nine tumors with p53 gene mutations. However, positive p53 staining was also seen in four sarcomas which had no detectable p53 mutations in exons 5 through 9. Because some sarcomas exhibit amplification and overexpression of MDM-2, which may interact with p53 and cause stabilization of wild-type p53 protein, we examined these tumors for MDM-2 amplification. None of the tumors with MDM-2 amplification exhibited p53 immunopositivity. Very weak p53 reactivity was detected in four malignant fibrous histiocytomas that had received either chemotherapy or radiotherapy. Of 16 metastatic lesions examined, only one contained a p53 mutation. In addition, for five cases in which both the original lesion and its metastases were analyzed, p53 alterations were not observed in the metastases if the tumor was wild-type at presentation. These data suggest that p53 alterations occur at different frequencies in various subtypes of sarcoma and, although detected in metastatic lesions, are not associated more frequently with progression.

Bone Neoplasms↗
PubMed1996

[Detection of herpes virus-like DNA in various forms of Kaposi's sarcoma but not in other mesenchymal tumors or inflammatory changes in skin].

Recently, herpes-virus like DNA sequences defining a new herpes virus termed human herpes virus 8 (HHV8), were detected in Kaposi's sarcoma of AIDS and non-AIDS patients. We describe the successful detection of HHV8 DNA in archival skin biopsies of the various forms of Kaposi's sarcoma. DNA was extracted from archival skin biopsies of Kaposi's sarcoma, other mesenchymal skin tumors and various inflammatory skin lesion of HIV seropositive and negative patients. The extracted DNA was analyzed for the presence of HHV8 DNA using a nested PCR assay. All samples were tested for the presence of appropriate DNA using a internal cellular control PCR-reaction. A total of 23 Kaposi's sarcoma were analyzed, including 12 of the endemic type, 9 HIV-associated and 2 transplant related. HHV8 DNA was detected by nested PCR in all forms of Kaposi's sarcoma. In contrast, no HHV8 DNA could be found in 17 mesenchymal, especially vascular skin tumors or in 7 biopsies with unspecific inflammatory skin lesions of HIV seropositive and negative patients. HHV8 DNA was present in all forms of Kaposi's sarcoma tested but not in other mesenchymal tumors or unspecific inflammatory lesions of the skin. This data support the idea of a strong association of HHV8 and Kaposi's sarcoma.

Acquired Immunodeficiency Syndrome↗
PubMed1997

Purification and characterization of human lung fibroblast motility-stimulating factor for human soft tissue sarcoma cells: identification as an NH2-terminal fragment of human fibronectin.

Paracrine motogenic factors, including motility cytokines and extracellular matrix molecules secreted by normal cells, can stimulate metastatic cell invasion. Both intact extracellular matrix molecules and their degradative products may exhibit these activities. We have found that human lung fibroblasts produce paracrine motility-stimulating factors for recently established human sarcoma cell strains. We purified the major fibroblast motility-stimulating factor (FMSF) from human lung fibroblast-conditioned medium by sequential heparin affinity chromatography and DEAE anion exchange chromatography. Lysylendopeptidase C digestion of FMSF and sequencing of peptides purified by reverse-phase high-pressure liquid chromatography identified FMSF as an NH2-terminal fragment of human fibronectin. Using SYN-1 sarcoma cells, FMSF predominantly stimulated chemotaxis and some chemokinesis, and it was chemotactic for a variety of human sarcoma cells, including fibrosarcoma, leiomyosarcoma, liposarcoma, synovial sarcoma, and neurofibrosarcoma cells. The FMSF activity present in human lung fibroblast-conditioned medium was completely eliminated by either neutralization or immunodepletion with a rabbit antihuman-fibronectin antibody, thus further confirming that the NH2-terminal fibronectin fragment was the FMSF responsible for the motility stimulation of human soft tissue sarcoma cells. Because human soft tissue sarcomas have a distinctive hematogenous metastatic pattern (predominantly lung), and lung-derived fibroblasts secrete large amounts of FMSF, FMSF and fibronectin may play a role in stimulating sarcoma invasion into lung tissue.

Amino Acid Sequence↗
PubMed1998

Primary ovarian sarcoma: analysis of prognostic variables and the role of surgical cytoreduction.

BACKGROUND: Data regarding the value of cytoreduction and cell histology in ovarian sarcomas are limited. The goal of this study was to assess the value of surgical cytoreduction, preoperative CA 125 levels, stage, histology, and platinum-based chemotherapy in the primary treatment of ovarian sarcomas. METHODS: A retrospective analysis of 47 women with primary ovarian sarcomas was performed. RESULTS: Forty-one patients (87%) presented with advanced stage disease (International Federation of Gynecology and Obstetrics Stage III or IV). Optimal surgical cytoreduction (< 1 cm residual tumor burden) was achieved in 25 patients (53%). Forty patients (85%) had a malignant mixed müllerian tumor whereas 7 patients had a pure sarcoma. Eighteen women with mixed müllerian tumors had homologous tumors and 22 had heterologous elements. Patients treated with platinum-based chemotherapy were significantly more likely to have a response (P = 0.008) compared with those treated with other regimens. Treatment with platinum-based chemotherapy also showed a survival advantage (P = 0.03). Preoperative CA 125 levels were elevated (> 35 U/mL) in 93% of patients with ovarian sarcomas. A preoperative CA 125 level < 75 U/mL was significantly associated with better survival (P = 0.01). In univariate analysis, other significant predictors of improved survival were early stage (P = 0.04), homologous tumors (P < 0.05), and optimal surgical cytoreduction (P < 0.001). In multivariate analysis of various prognostic variables, optimal surgical cytoreduction (P < 0.001) was the most significant factor, followed by histologic subtype (P < 0.02). CONCLUSIONS: Ovarian sarcomas are rare malignancies with a poor prognosis. All women with suspected ovarian carcinoma or sarcoma should have a preoperative CA 125 level taken. Surgical cytoreduction to a residual tumor burden of < or = 1 cm improves outcome and should be the goal of surgery. Although the optimal consolidation chemotherapy regimen remains unknown, platinum should be included as part of the regimen.

Adolescent↗
PubMed1998

[Surgical aspects in the multidisciplinary treatment of soft tissue sarcomas].

Sarcomas are rare malignant tumors with a large variety of histologic subtypes. The surgical approach depends more on the histologic grade, the size and the site of the tumor. Radiologic diagnosis relies predominantly on MR-imaging. Discernible improvements have taken place in soft tissue sarcoma patient survivorship and quality of life over the past 20 years, with overall 5-year survival currently at approximately 50-80%. The place of surgery in the treatment of soft-tissue sarcoma is defined in the light of a review of the recent literature. Radical surgical resection is the mainstay of therapy. Local recurrence is the most common type of failure. Local recurrence is resectable and limb preservations possible in the majority of patients. Survival after treatment of local recurrence is determined mainly by the grade and secondarily by the size of the tumor. The essential risk factor for local recurrence is the quality of surgical resection, defined by the definitive resection margins. A lateral safety margin of 5 cm and of 2 cm to the depth should be respected. In sarcoma of the extremity the compartment is defined based on clinical, radiographic, histopathologic and operative findings. The use of muscle flaps to fill the surgical defects can improve the functional result and reduce the complication rate. Only about 5% of the patients need amputation. Evaluation of functional results must be based on objective criteria. In retroperitoneal sarcoma the significant factors for determining prognosis are grade and completeness of exzision. Multidisciplinary treatment according to common protocols is essential. Shifts in treatment have taken place over the past decade, from single-modality treatment involving radical surgery with compartment resection to sophisticated limb-salvage strategies combined with radiation therapy. In case of inadequate surgery e.g. in a large tumor with positive margins in high-grade soft tissue sarcomas the addition of radiotherapy can improve local control, but cannot ensure that obtained by adequate surgery. Patients with large (greater than 5 cm), high grade soft tissue sarcoma are at high risk for distant recurrence and disease-related mortality. Investigations of combined modality therapy with newer chemotherapy agents and dose intensification treatment strategies are warranted.

Combined Modality Therapy↗
PubMed1983

Extremity preservation by combined modality therapy in sarcomas of the hand and foot: an analysis of local control, disease free survival and functional result.

A primary tumor arising in the hand or foot represents an uncommon presentation for patients with Ewing's sarcoma (ES) or soft tissue sarcoma (STS). While there exists considerable literature on the treatment of extremity sarcomas, very little deals specifically with lesions of the hand or foot. It remains controversial whether these lesions can be successfully treated with combined modality therapy which preserves the extremity and maintains function. From 1972 to 1979, 10 patients with sarcomas arising in the hand or foot were treated with combined modality therapy at the National Cancer Institute. Seven patients with ES of bone received local irradiation to 5000 rad and combination chemotherapy following an incisional biopsy. Three patients with STS received a gross tumor excision and local irradiation to 6000 rad. One STS patient also received combination chemotherapy. Local control was achieved in nine patients (90%) with a follow-up of 30-119 months (median 56 months). These patients have complete or almost complete function of the treated extremity. Nine patients are alive with five patients remaining disease-free following the initial combined modality treatment. Two patients with Ewing's sarcoma relapsed (1 patient with both local and distant failure) at 26 and 58 months and were again rendered disease-free with surgery, total body irradiation and further chemotherapy. One patient relapsed for a second time, being disease-free from the first relapse for 30 months. We conclude that for selected patients with sarcomas arising in the hand or foot, combined modality therapy which leaves the extremity intact results in excellent local tumor control and preserves function. Careful treatment planning is an essential aspect of successful radiation therapy of a hand or foot primary. Our treatment recommendations are outlined. This approach is a viable alternative to amputation in these patients.

Adolescent↗
PubMed2004

Soft tissue sarcoma of the upper extremity.

Soft tissue sarcomas of the upper extremities are rare and hand surgeons typically encounter only one or two undiagnosed soft tissue sarcomas during their careers. It is incumbent on the physician to review repeatedly the characteristics of these tumors and remain suspicious, because these lesions typically are misdiagnosed and treatment is delayed. The most common soft tissue sarcomas of the upper extremity are the epithelioid sarcoma, synovial cell sarcoma, and malignant fibrous histiocytoma. Limb salvage surgery is the treatment of choice for soft tissue sarcomas to preserve upper extremity function. Following wide tumor resection, adjuvant therapies such as chemotherapy, external beam radiation therapy, and brachytherapy may lessen local recurrence rates, but their effect on overall survival remains unclear.

Arm↗
PubMed1982

[The effect of sodium nitrite on rats infected with avian sarcoma virus].

A potential effect of sodium nitrite was studied as exerted on carcinogenesis elicited by the avian sarcoma virus in the body of rats. In the case of the infection of the rats with indefective avian sarcoma virus B77 (Bratislava strain; ID50 10-25/0.1 ml) and i. p. inoculation of sodium nitrite, great mortality occurred already on the 20th day p. i. On the 125th day p. i. the cumulative mortality in this group was 76.92%; on the other hand, in the control groups (rats inoculated with sodium nitrite or avian sarcoma virus) no deaths occurred before this period. The rats infected with a double dose of the virus were not observed to show pronounced changes in mortality. It was only on the 125th day that cumulative mortality in the group of rats infected with the avian sarcoma virus and rats inoculated s. c. with sodium nitrite was 87.5% and in the group of animals which had only been infected with the virus the mortality was 64.26%. The patho-morphological changes were in keeping with the hitherto described changes in rats infected with the avian sarcoma virus. In the trial no effect of sodium nitrite on the patho-morphological changes induced by the avian sarcoma virus was observed.

Animals↗
PubMed1976

Chemotherapy, en bloc resection, and prosthetic bone replacement in the treatment of osteogenic sarcoma.

In an attempt to shrink primary osteogenic sarcoma and allow complete surgical removal of the primary tumor, without amputating the involved limb, intensive preoperative chemotherapy with high dose methotrexate (HDMTX) with citrovorum factor rescue (CFR) and adriamycin (ADR) was initiated in 20 patients with biopsy-proven primary osteogenic sarcoma of the distal femur (15 patients) and proximal tibia (five patients). Following intensive chemotherapy, en bloc resection of the primary tumor with prosthetic replacement of the involved bone was planned. After surgery, adjuvant chemotherapy, consisting of HDMTX with CFR, ADR, and high dose cyclophosphamide was given sequentially for 1 year. Of 20 patients with primary osteogenic sarcoma (two with evidence of pulmonary metastases), 18 had primary tumors that could be clinically measured. Of these 18, 17 demonstrated a decrease in the size of primary tumor prior to surgery, while on chemotherapy. To date, 12 of these patients with osteogenic sarcoma of the distal femur have had total femur and knee joint replacement, and three patients with osteogenic sarcoma of the proximal tibia have had total knee replacement. In all 15 patients, surgical margins were grossly and microscopically free of tumor. There has been no evidence of soft tissue recurrence in any of the 15 patients who have undergone surgery for from 2 to 15 months postoperatively. These preliminary results indicate that with the use of aggressive chemotherapy, it is possible to demonstrate objective tumor regression in primary osteogenic sarcoma, allowing the surgeon to perform en bloc resection of tumor and prosthetic replacement of the involved bone. Although the limb is preserved, it is important to stress that extensive surgery yielding tumor-free margins is performed. The ultimate evaluation of this approach to the treatment of primary osteogenic sarcoma awaits longer observation, to determine limb function and the continued disease-free status, once adjuvant chemotherapy is discontinued.

Adolescent↗
PubMedDate not supplied

Paget's sarcoma of the mandible.

BACKGROUND: Sarcomatous change occurs in less than 1% of patients with Paget's disease. It has been reported in only 9 patients in the mandible, and has been uniformly fatal. Comparison of Paget's sarcoma of the mandible to osteosarcoma of the mandible and to osteosarcoma in other locations was made to attempt to explain and improve the outcome of patients with these tumors. METHODS: Two 78-year-old women with Paget's sarcoma of the mandible treated with radical resection are reported. The literature is reviewed to compare the clinical presentation and prognosis of patients with mandibular Paget's sarcoma to patients with osteosarcoma in non-Pagetoid mandibles and with osteosarcoma outside of the head and neck. RESULTS: Both patients died within 2 years of lung metastases. The patients with mandibular Paget's sarcoma were markedly older, with an average age of 65.6 years, compared to 32.5 years for mandibular osteosarcoma. Paget's sarcoma of the mandible was uniformly rapidly fatal; in non-Pagetoid mandibles the 5-year survival is 40%. There have been reports of only 7 patients who have survived with Paget's sarcoma in other locations. In osteosarcoma of the extremities 5-year disease-free survival exceeds 75% with multimodality therapy. CONCLUSIONS: Paget's sarcoma of the mandible is a rare tumor which affects elderly patients. It progresses rapidly and has a much poorer prognosis than osteosarcoma occurring in a non-Pagetoid mandible. The prognosis may be improved with early detection and treatment with multimodality therapy.

Adult↗
PubMed2003

Fibroblastic/myofibroblastic sarcoma of the skin: a report of five cases.

BACKGROUND: A number of malignant soft tissue tumors, particularly those of fibroblastic and fibrohistiocytic derivation, have been found to display myofibroblastic differentiation focally. The term myofibroblastic sarcoma, a controversial presumably distinctive entity, defines a malignant soft tissue tumor in which myofibroblasts are quantitatively the predominant cell type. METHODS: Five cases of cutaneous spindle-cell sarcomas showing fibroblastic-myofibroblastic differentiation with predominance of fibroblasts were retrieved from the files of three large centers of dermatopathology. Tumors were analyzed histopathologically, immunophenotypically, and, in two cases, ultrastructurally. Results were compared with those previously reported in fibrosarcoma, malignant fibrous histiocytoma, and myofibroblastic sarcoma. RESULTS: Immunophenotypic and ultrastructural profiles of the cases analyzed in this series were closer to fibrosarcoma and to malignant fibrous histiocytoma than to myofibroblastic sarcoma by virtue of quantitative predominance of fibroblasts over myofibroblasts. On the other hand, histopathologic findings were in keeping with those reported in myofibroblastic sarcoma. CONCLUSIONS: Our series highlights the intrinsic problems in attaching certain cutaneous sarcomas with fibroblastic-myofibroblastic differentiation to one of the recognized entities and gives support to the hypothesis that fibrosarcoma, malignant fibrous histiocytoma, and myofibroblastic sarcoma are related histogenetically.

Aged↗
PubMed1998

Neurogenic sarcomas: experience at the University of Toronto.

OBJECTIVE: Elucidation of the natural history and optimal management of neurogenic sarcomas is not straightforward, because of diagnostic difficulties and the low incidence of this disease. The majority of neurogenic sarcomas are categorized and treated as soft tissue sarcomas, and only a few centers have sufficient experience to add to our understanding of these malignant tumors. This article proposes an algorithm for the management of these tumors. METHODS: A cohort of 18 cases of neurogenic sarcoma (overall mean follow-up period after oncological diagnosis, 33.7 mo), representing approximately 3% of all soft tissue sarcomas treated at the University of Toronto Musculoskeletal Oncology Unit (Toronto, Canada) between 1989 and 1995, were reviewed. The clinical and pathological criteria used to establish the diagnosis of neurogenic sarcoma, the presenting symptoms, the tumor size and grade, the ability to obtain tumor-free margins during en bloc resection, the time to recurrence, and the overall survival rates were noted. A centralized pathological review of the biopsy was undertaken in all cases. RESULTS: A metastatic survey demonstrated localized disease for 16 of the 18 patients. The overall management strategy for these 16 neurogenic sarcomas with localized disease was to obtain local control by en bloc resection. Tumor size, tumor grade, and, most importantly, the ability to obtain tumor-free margins were all relevant prognostic factors for survival. Tumor-free surgical margins were obtained for 11 of 16 patients, with 9 of these 11 patients remaining disease-free. In contrast, all patients for whom tumor-free margins could not ultimately be achieved died as a result of their disease. CONCLUSION: Based on our experience, we propose an algorithm for the management of these rare tumors, for both optimal patient care and oncological research.

Adolescent↗