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PubMed1982

Telangiectatic osteogenic sarcoma: a clinicopathologic study of 124 patients.

One hundred-twenty-four patients with this rare and special variant of osteogenic sarcoma were treated at Memorial Sloan-Kettering Cancer Center from 1921 through 1979, representing 11% of all of osteogenic sarcomas. The lesions were predominantly lytic, destructive tumors with only minimal sclerosis on roentgenograms and soft as well as cystic on gross examination. Histologically, aneurysmally dilated spaces lined or traversed by sarcoma cells producing osteoid were noted. The differential diagnosis both radiographically and histologically included several benign lesions like aneurysmal bone cyst and giant cell tumor, among many others. It was found that telangiectatic osteogenic sarcoma is relatively frequent in the femoral diaphysis and in the distal end of the femur. Twenty-nine percent of the patients present with pathologic fracture, or this develops later. Age and sex distribution, or clinical signs or symptoms were those of ordinary osteogenic sarcomas. No differences in survival rates were found in lesions that were purely lytic or those with minimal sclerosis. Similarly, no differences in survival were noted when comparing patients with telangiectatic or ordinary osteogenic sarcoma. As a matter of fact, definite increase in survival was found in patients treated since 1975 with preoperative multidrug chemotherapy employing high-dose methotrexate. Adriamycin, and the combination of bleomycin, cyclophosphamide, and dactinomycin.

Adolescent↗
PubMed1985

Soft tissue sarcomas arising in the retroperitoneal space in children. A report from the Intergroup Rhabdomyosarcoma Study (IRS) Committee.

One hundred one eligible children with soft-tissue sarcomas arising within the retroperitoneal space have been registered on Intergroup Rhabdomyosarcoma Study Committee (IRS) studies I and II and followed for at least 2 years or until death. The most common presenting symptoms and signs were pain in the abdomen or lower extremities, and/or an abdominal mass, usually noted by a parent or a physician seeing the child for other complaints. The median age at diagnosis was 6.5 years and the sex ratio (M/F) 1.7:1. Histologic types were embryonal or botryoid rhabdomyosarcoma (RMS) in 58 patients, alveolar RMS in 8, pleomorphic RMS in 2, undifferentiated sarcoma in 20, extraosseous Ewing's sarcoma in 4 and unspecified sarcoma in 9. Median tumor size was 10 cm, significantly larger than the 7.5 cm noted in the IRS studies as a whole (P less than 0.05). One patient had complete tumor removal (Group I); 12 had grossly complete removal with microscopic residual tumor (Group II). Fifty-one patients had residual local tumor after biopsy or partial resection (Group III) and 37 patients had distant metastases at diagnosis (Group IV). Treatment included surgery, radiation therapy (RT) and combination chemotherapy with vincristine and actinomycin D with or without cyclophosphamide and Adriamycin (doxorubicin) according to protocol. Thirty-nine of 99 patients (39%) had major difficulties in the delivery of specified RT. Seventy patients received sufficient therapy to be evaluable for treatment response. Forty-one (58%) achieved a complete remission and 16 (23%) achieved partial remission. Twenty-four of 41 children (59%) achieving a CR have relapsed. The proportion of children who remained relapse-free at 2 and 3 years of follow-up was 44% or 42%, respectively. Overall, 40 children have developed recurrent sarcoma and the median disease-free interval and overall survival times were 54 and 88 weeks, respectively. Most children experienced severe myelosuppression; there were three early deaths from infection which occurred during granulocytopenic periods. One third of the patients experienced cystitis. Children with soft-tissue sarcomas arising in the retroperitoneal space have a poor prognosis associated with large tumors which, because of location and/or extent (Groups III or IV), are unresectable and difficult to treat.

Antineoplastic Combined Chemotherapy Protocols↗
PubMed1985

Osteogenic sarcoma in pregnant women. Prognosis, therapeutic implications, and literature review.

This study of 18 pregnant women with concomitant osteogenic sarcoma of bone analyzes the important assertion whether this sarcoma and pregnancy have an adverse interaction. For comparison we matched the pregnant osteogenic sarcoma patients with nonpregnant women with the same skeletal tumor location and histologic appearance as well as similar age distribution. There was no worsening of prognosis of pregnant osteogenic sarcoma patients, and neither the pregnancy nor the disease appeared to act adversely toward the other. The 18 pregnant women with osteogenic sarcoma fared no better (nor worse) than the nonpregnant women with osteogenic sarcoma.

Adolescent↗
PubMed1993

Aggressive pulmonary metastasectomy for soft tissue sarcomas.

BACKGROUND: To evaluate the efficacy of aggressive pulmonary metastasectomy for treating soft tissue sarcomas, the clinical data on the surgical management of 23 patients with extensive pulmonary metastases from soft tissue sarcomas were reviewed. METHODS: Between January 1973 and April 1991, 9 male patients and 14 female patients were treated. Their ages ranged from 13-68 years (median, 42 years). Twenty-one patients (91%) had bilateral and multiple metastases, and two patients had solitary metastasis. The number of resected metastatic nodules ranged from 1-110 (mean, 30.5). As an initial surgical approach, median sternotomy was used on 18 patients and lateral thoracotomy on 5 patients. Eleven patients underwent two or more explorations for recurrent metastases using lateral thoracotomy. The neodymium:yttrium-aluminum-garnet (Nd:YAG) laser was adopted in 10 patients since 1986. RESULTS: The actuarial 2-year and 5-year survival rates after the first pulmonary resection were 49.7% and 24.8%, respectively. Histologic type (alveolar soft part sarcoma versus synovial sarcoma, P < 0.025), histologic grade (G1 and G2 versus G3, P < 0.01), and metastatic localization (subpleural versus extrapleural, P < 0.005) were the most significant prognostic factors for aggressive pulmonary metastasectomy of soft tissue sarcomas. Application of laser surgery, absence of local recurrences, and absence of extrapulmonary metastases before pulmonary resection also correlated with better prognosis with borderline significance. CONCLUSIONS: Aggressive pulmonary metastasectomy for soft tissue sarcomas is a recommended procedure, even in the case of extensive metastases. The combination of median sternotomy and Nd:YAG laser-assisted surgery is a useful technique, especially in bilateral multiple pulmonary metastases.

Adolescent↗
PubMed2002

Vinorelbine in previously treated advanced childhood sarcomas: evidence of activity in rhabdomyosarcoma.

BACKGROUND: Vinca alkaloids have proved active against a number of pediatric malignancies. The aim of this study was to assess the feasibility and effectiveness of using vinorelbine in previously treated pediatric patients with advanced sarcomas. METHODS: From September 1998 to August 2001, 33 previously treated patients with progressive sarcoma were treated: 13 had rhabdomyosarcomas, 5 had other soft tissue sarcomas, 9 had Ewing sarcomas, and 6 had osteosarcomas. Vinorelbine was given intravenously on Days 1 and 8 of a 21-day treatment cycle. Four patients with uncontrolled pain or central nervous system invasion received concurrent radiotherapy and were only evaluated for toxicity. RESULTS: One hundred seventy-eight treatment cycles were administered (median of four per patient, range 1-20). Grade 3 to 4 neutropenia occurred in 63% of patients, Grade 3 anemia in 9%, and Grade 3 thrombocytopenia in 3%. Nonhematological toxicity was mild or moderate, i.e., always lower than Grade 3, with the exception of one child who experienced paralytic ileus. Twenty-eight patients were assessable for response. Eight patients had a partial response, one patient had a minor response, and nine patients had stable disease. Objective responses were observed in 6 of 12 patients with rhadbomyosarcomas (five of six of the alveolar subtype), in one of five patients with osteosarcomas, and in one of seven patients with Ewing sarcomas. The median duration of response was 10 months (range, 3+ to 20). CONCLUSIONS: Vinorelbine has a favorable toxicity profile with evidence of biological activity in already heavily treated pediatric patients with sarcomas. In particular, the objective response rate obtained for patients with alveolar rhabdomyosarcoma seems very promising. Due to the few cases considered here, further Phase II studies are needed to establish a potential role of vinorelbine in the treatment of these tumors.

Adolescent↗
PubMed2006

Occupational factors and risk of adult bone sarcomas: a multicentric case-control study in Europe.

We investigated the association between occupational factors and risk of bone sarcoma, a rare tumor with a largely unknown aetiology. A multicentric case-control study was conducted in 7 European countries in 1995-97. Ninety-six cases aged 35-69 years with a centrally reviewed diagnosis of bone sarcoma (68 chondrosarcomas and 28 osteosarcomas) were compared to 2,632 population (68%) or colon cancer (32%) controls. Subjects were interviewed to obtain information on occupational, medical and reproductive history, smoking and alcohol consumption and selected exposures including use of pesticides. Response proportions were 90% among cases and 66% among controls. Odds ratios (OR) and 95% confidence intervals (CI) were estimated for selected categories of job titles and branches of industry and for use of pesticides. We found an increased OR for bone sarcoma among blacksmiths, toolmakers, machine-tool operators (OR = 2.14, 95% CI 1.08-4.26), woodworkers (OR = 2.68, 95% CI 1.36-5.29) and construction workers (OR = 1.62, 95% CI 0.92-2.87). Ever users of pesticide had an OR of 2.33 (95% CI 1.31-4.13), with similar risks for exposure to insecticides and exposure to herbicides. Neither duration of employment in any of the analyzed occupational categories nor duration of use of pesticides showed an increasing trend in the risk of bone sarcoma. ORs of bone sarcoma were 1.03 (95% CI 0.23-4.57), 3.13 (95% CI 1.26-7.76) and 1.44 (95% CI 0.43-4.85) for the first, second and third tertile of days of use of pesticides. Our study suggests that novel and previously reported (woodworking) occupational factors play a role in the aetiology of bone sarcomas.

Adult↗
PubMed1986

New monoclonal antibodies specific for human sarcomas.

Monoclonal antibodies (MAbs) against sarcoma-associated cell membrane antigens were prepared by immunizing BALB/c mice with tumor cells from a human osteosarcoma, TPX, grown as a xenograft in athymic BALB/c nude mice. Spleen cells from immunized mice were hybridized with X-63 Ag. 8.653 mouse myeloma cells which yielded 260 growing hybridomas. Seven of these produced antibodies that bound to TPX cells and to cells from another osteosarcoma, but not to autologous skin fibroblasts. MAbs from 2 (TP-1 and TP-3) of these 7 clones did not cross-react with non-sarcomatous tumor cells or peripheral blood lymphocytes. Immunohistochemical studies on frozen tissue sections showed that the TP-1 (IgG-2a) and TP-3 (IgG-2b) antibodies had characteristic and identical specificity profiles. Binding of TP-1 (TP-3) was demonstrated to 15/15 (15/15) osteosarcomas, 3/3 (2/2) synovial sarcomas, 7/9 (6/8) malignant fibrous histiocytomas, 2/2 (1/1) malignant hemangiopericytomas, 1/2 (1/2) chondrosarcomas and 3/6 (1/3) unclassified sarcomas. The antibodies did not bind to any of 16 sarcomas belonging to other histological subtypes, including liposarcomas and leio- and rhabdomyosarcomas. Moreover, they failed to bind to sections of 66 different non-sarcomatous malignancies, or to any of a range of normal adult and fetal tissues, although some weak staining of proximal kidney tubules was seen. The restricted specificity of these antibodies to some major subtypes of human sarcomas makes them promising tools for identification and subclassification of sarcomas.

Animals↗
PubMed1978

Epithelioid and fibroblastic rat kidney cell clones: epidermal growth factor (EGF) receptors and the effect of mouse sarcoma virus transformation.

Fibroblastic and epithelioid clones have been isolated from the normal rat kidney line, NRK. These clones were studied for their ability to bind epidermal growth factor (EGF), susceptibility to transformation by mouse sarcoma virus (MSV), and alteration in EGF binding upon sarcoma virus transformation. The epithelioid clones bound much more EGF than the fibroblastic clones; Scatchard plots on two of these clones, one epithelioid and one fibroblastic, showed that the higher EGF binding (1.3 x 10(5) molecules per cell for the epithelioid clone and 1.3 x 10(4) molecules per cell for the fibroblastic clone) was due to a greater number or receptors on the epithelioid cells rather than to a difference in the apparent affinity constant. When the clones were transformed by Moloney murine sarcoma virus the EGF binding decreased, the effect being greater with the fibroblastic clones. In 20 out of 20 independently isolated sarcoma virus transformed fibroblastic clones, the level of EGF binding was either greatly reduced or completely eliminated. In contrast to EGF, another growth factor, multiplication stimulating activity (MSA), bound to a greater extent to the fibroblastic clones than the epithelioid clones, and its binding was not decreased by sarcoma virus transformation. The results show that loss of EGF binding ability correlates with expression of the murine sarcoma virus transformation.

Animals↗
PubMed1999

Absence of Kaposi's sarcoma-associated virus (human herpesvirus-8) sequences in angiosarcoma.

Kaposi's sarcoma-associated virus (human herpesvirus-8 [HHV8]) sequences have been identified in both AIDS-associated and AIDS-non-associated Kaposi's sarcoma, but data relating to the detection of HHV8 sequences in angiosarcoma are variable. One study showed HHV8 sequences in 29% (7/24) of angiosarcomas, but others have not confirmed these results. We evaluated 33 angiosarcomas for HHV8 sequences to determine the frequency of this virus in angiosarcomas and its possible pathogenetic significance. Five cases of Kaposi's sarcoma from HIV-positive patients were used as positive controls. Five additional cases of Kaposi's sarcoma collected approximately 40-50 years ago were also analysed, and three were HHV8 positive. None of the angiosarcomas revealed HHV8 sequences after the standard 35 cycles of PCR. In 6 cases, nested PCR revealed the presence of HHV8 sequences. These results were not reproducible when outer primers (based on sequences outside of the earlier PCR products) were used for amplification. This suggests that the HHV8 sequences detected in 6 cases represent a low level of contamination. In contrast, HHV8 sequences were found in all Kaposi's sarcomas with well-preserved DNA after standard 35-cycle PCR amplification. These findings confirm a close association between Kaposi's sarcoma and HHV8 infection and suggest that HHV8 is not involved in the pathogenesis of angiosarcoma.

Adult↗
PubMed1981

Transforming proteins of some feline and avian sarcoma viruses are related structurally and functionally.

Transformation of chicken cells by Fujinami sarcoma virus (FSV), PRC II or Y73 (three independently isolated avian sarcoma viruses that are replication-defective and lack the Rous sarcoma virus src gene) resulted in significant elevation (4-13 fold) of phosphotyrosine levels in cellular protein. The gag-related proteins encoded by these avian sarcoma viruses (ASVs) were all associated with tyrosine-specific protein kinase activity when assayed in immune complexes and were phosphorylated at both tyrosine and serine residues in vivo. Both the phosphotyrosine level in protein of FSV-infected cells and the protein kinase activity assayed in immune complexes containing the FSV protein P140 were temperature-sensitive. The presumed transforming proteins of these ASVs were compared with those of Rous sarcoma virus (RSV), Abelson murine leukemia virus and the Snyder-Theilen and Gardner-Arnstein strains of feline sarcoma virus (FeSV), which have previously been associated with tyrosine-specific protein kinase activity. FSV and PRC II proteins were shown to be structurally related to one another and to the FeSV proteins by tryptic peptide mapping and by immunological studies. No homology was observed, however, between the transforming proteins of RSV, Y73, Abelson murine leukemia virus and the FSV/PRC II/FeSV class, suggesting there may be at least four classes of retroviruses whose transformation mechanisms involve aberrant phosphorylation of cellular protein at tyrosine residues.

Alpharetrovirus↗
PubMed1984

Bone resorption in osteogenic sarcoma--II. Resorption of the bone collagenous matrix by tumor cells, normal fibroblasts and macrophages.

Three osteogenic sarcoma cell lines of human and canine origin were compared to normal fibroblastic cells and peptone-induced murine peritoneal macrophages in terms of bone collagenous matrix (BCM) resorption capacity. The dissolution of the BCM was measured in an in vitro system consisting of the tested cells and live or killed [3H]-proline-labeled fetal mouse long bones. Experiments with osteogenic sarcoma cells revealed a paradoxical phenomenon indicating an inverse relationship between the number of tumor cells and the rate of collagen resorption from live bones. On the other hand, collagen matrix of devitalized bones, particularly those denatured by exposure to heat, is strongly resorbed by osteogenic sarcoma cells even in the presence of serum. Contrary to osteogenic sarcoma cells, normal fibroblasts do not resorb collagen from either live or killed bones, regardless of the devitalization method and culture conditions utilized. Macrophages resorb collagenous matrix from live bones, but their collagen resorption activity from devitalized bones depends greatly on choice of the incubation condition. Results have shown that osteogenic sarcoma tumor cells, when acting alone, may not have the capacity to destroy healthy bone. We suggest, therefore, that bone destruction seen in osteogenic sarcoma patients depends on the metabolic condition of the affected bone, and the interaction between the tumor and normal host cells and tissues.

Animals↗
PubMed1976

Distribution of envelope-specific and sarcoma-specific nucleotide sequences from different parents in the RNAs of avian tumor virus recombinants.

The distribution of leukosis-virus- and sarcoma-virus-specific oligonucleotide sequences was investigated in the RNAs of viral recombinants selected for an envelope gene (env) from a leukosis parent and a sarcoma gene (src) from a sarcoma parent. For this purpose 20 to 30 RNase-T1-resistant oligonucleotides were chemically analyzed and mapped within the 10,000 nucleotides of each viral RNA relative to the 3'-poly(A) end. The resulting oligonucleotide maps were compared. Proceeding from the 3' to the 5' end, the maps of four recombinants contained: (i) in a segment of 2000 nucleotides, three to four src-specific oligonucleotides, so identified because they were shared only with the sarcoma parent; and (ii) in a segment of 8000 nucleotides, 20 oligonucleotides shared with the leukosis parent, of which six to seven were also shared with the sarcoma parent. Two other recombinants contained: (1) in a segment of 2000 (one) or 3000 (the other) nucleotides, three src-specific oligonucleotides; (ii) in a segment of 3000 (one) or 2000 (the other) nucleotides, five (one) or four (the other) oligonucleotides, all or some of which are env-specific, because they were shared with the leukosis parent; (iii) in a segment of 5000 nucleotides (both), 11 functionally unidentified sarcoma-virus-derived oligonucleotides, of which seven were also shared with the leukosis parent. The map locations of parental oligonucleotides were not changed in recombinants and all viral strains tested shared six to eight highly conserved oligonucleotides at equivalent map locations. The partial map -env-src-poly(A) emerged from the analyses of these recombinants.

Alpharetrovirus↗
PubMedDate not supplied

Abdominal recurrence of osteogenic sarcoma: a case report.

PURPOSE: Nonpulmonary metastases from osteogenic sarcoma are rare. A patient had a localized osteogenic sarcoma of the left femur which recurred in the abdomen, a previously unreported metastatic site. PATIENT AND METHODS: An 18-year-old boy was treated for osteosarcoma. He had abdominal pain, vomiting, weight loss, and symptoms of intestinal obstruction at the time of relapse. RESULTS: The patient had diffuse widespread intraabdominal osteogenic sarcoma as the only site of initial recurrence. Abdominal computerized tomography revealed ascites and calcified masses on the hepatic and peritoneal surfaces. Laparoscopic visualization of the abdomen showed hemorrhagic ascites and multiple calcified tumor on the peritoneum, diaphragm, and liver. A biopsy of a representative lesion confirmed the diagnosis of osteogenic sarcoma. The patient died from progressive disease. CONCLUSION: As the initial treatment for patients with osteogenic sarcoma is intensified, the pattern of metastases may change. Unusual sites of recurrence such as in this patient may become more prevalent. A clinical presentation of an acute abdomen in a patient previously treated for osteogenic sarcoma should prompt suspicion of intraabdominal recurrence.

Abdominal Neoplasms↗
PubMed2003

Osteogenic sarcoma of the rib: a case presentation and literature review.

STUDY DESIGN: A case report is presented. OBJECTIVES: Primary tumors of the rib are relatively uncommon in the adult population, and even more rare in children. A case of osteogenic sarcoma of the rib and a literature review are presented. SUMMARY OF BACKGROUND DATA: Osteogenic sarcoma represents approximately 30% of all malignant sarcomas diagnosed in the United States. A single case of osteogenic sarcoma of the rib has been reported in the literature involving a 9-year-old child. METHODS: Clinical data analysis. RESULTS: A 9-year-old white boy presented with a mass of the left posterior thorax. The initial chest radiograph showed a nonhomogeneous mass with calcifications adjacent to the 11th rib. The final diagnosis was osteogenic sarcoma. Chemotherapy was initiated. The patient underwent radical excision of the mass. Given the extent of the patient's resection, it was thought that he would be at high risk for the development of spinal deformity. He was placed in a TLSO brace (Bolt Systems, Orlando, FL) and followed closely. At 15 months after excision of his tumor, he was noted to have progression of a thoracolumbar scoliosis and significant kyphosis. At this writing, it has been 52 months since resection. The patient has no evidence of local recurrence or metastatic disease, and his spinal curvature remains stable. CONCLUSIONS: The patient's short-term (4-year) disease-free survival illustrates the efficacy of neoadjuvant chemotherapy and radical surgical resection. Patients with osteogenic sarcoma of the rib should be monitored closely for the development of spinal deformity if the required resection includes the vertebral column. Casting and bracing may help to limit progression of the deformity.

Bone Neoplasms↗
PubMed1981

Genetic structure, transforming sequence, and gene product of avian sarcoma virus UR1.

We analyzed the genetic structure and gene products of the newly isolated avian sarcoma virus UR1, which recently has been shown to be replication defective and to contain no sequences homologous to the src gene of Rous sarcoma virus. The sizes of the genomic RNAs of UR1 and its associated helper virus, UR1AV, were determined to be 29S and 35S (5.9 and 8.5 kilobases), respectively, by gel electrophoresis and sucrose gradient sedimentation. RNase T1 oligonucleotide mapping of purified viral RNAs indicated that UR1 RNA contains eight unique oligonucleotides in the middle of the genome and shares four 5'-terminal and three 3'-terminal oligonucleotides with UR1AV RNA. The unique sequences of UR1 and Fujinami sarcoma virus were found to be closely related to each other by molecular hybridization of UR1 RNA with DNA complementary to the unique sequence of Fujinami sarcoma virus RNA, but minor differences were found by oligonucleotides fingerprinting. In the regions flanking the unique sequences, UR1 and Fujinami sarcoma viral RNAs contain distinct oligonucleotides, which are shared with oligonucleotides of the respective helper viral RNAs. Cell transformed with UR1 produce a single 29S RNA species which contains a UR1 unique sequence; this species is most likely the mRNA coding for the transforming protein. In UR1-transformed cells, a phosphoprotein fo 150,000 daltons (p150) was detected by immunoprecipitation with antiserum against gag proteins. p150 was associated with a protein kinase activity that was capable of phosphorylating p150 itself, immunoglobulin G of antiserum, and a soluble substrate, alpha-casein. This enzyme transferred phosphate exclusively to tyrosine residues of substrates in vitro, but p 150 labeled in vivo with 32P contained both phosphoserine and phosphotyrosine. The in vitro kinase reaction was not affected by the presence of cyclic AMP or cyclic GMP and strongly preferred Mn2+ over Mg2+. Thus, the properties of UR1 protein are almost identical to those of Fujinami sarcoma virus protein.

Alpharetrovirus↗
PubMed1999

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

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

Adult↗
PubMed2002

Postradiation high-grade myofibroblastic sarcoma of the prostate -- a rare entity of prostatic tumors -- responding to liposomal Doxorubicin.

BACKGROUND: Prostatic postradiation sarcoma (PRS) is a very rare malignant disease with a dismal prognosis. Aggressive surgical resection is the most widely used therapy for PRS. Chemotherapy so far has been unsatisfactory, with doxorubicin being the only drug with established activity. Liposomal doxorubicin has been shown to have comparable activity and a more favorable toxicity profile compared with unpegylated doxorubicin. CASE REPORT: A 78-year-old man presented with high-grade myofibroblastic sarcoma 13 years after curatively intended pion irradiation of the prostate with 30 Gy. He was treated with liposomal doxorubicin 40 mg/m(2) every 4-6 weeks up to now with a total dose of 800 mg. Partial remission has been achieved after 17 months of treatment with liposomal doxorubicin. Toxicity is minimal with palmoplantar erythrodysesthesia WHO grade 1, resolving completely after extending treatment intervals to 6 weeks. RESULT: Liposomal doxorubicin led to a sustained tumor stabilization over 17 months in a patient with prostatic postradiation high-grade sarcoma. Postradiation sarcoma (PRS) usually has a dismal prognosis and only very limited therapeutic possibilities. As in sarcomas of different origin, anthracyclines including liposomal doxorubicin seem to have potential antitumor activity in postradiation sarcoma of the prostate.

Adenocarcinoma↗
PubMed2004

The versatility of the free osteocutaneous fibula flap in the reconstruction of extremities after sarcoma resection.

BACKGROUND: An understanding of the biology of bone and soft-tissue sarcomas, knowledge of adjuvant therapies and refinement in techniques of reconstructive surgery have allowed limb-sparing and limb salvage surgery to become a reality in the management of malignant tumors of the extremities. Functional limb salvage following radical resection has become a possibility in many resectable tumors by the use of alloplastic prostheses, homograft or autogenous bone for skeletal reconstitution combined with vascularized soft tissue coverage. Although the free fibula flap has been well described for reconstructions of the mandible and oral cavity, it has not been widely presented as an ideal tool to preserve extremities and to circumvent amputation. PATIENTS AND METHODS: We describe the complex surgical reconstruction in four patients with primary sarcomas of the extremities. The sarcomas (Ewing's sarcoma, osteosarcoma and epitheloid sarcoma) were resected radically and the massive bone and soft tissue defect was replaced by vascularized free fibula transfer. RESULTS: We present our experience with versatility of this osteocutaneous flap to allow reconstruction and salvage of extremitity sarcomas. There were no operative or postoperative complication and all the four patients had good limb function. The flap was found to be versatile as it could be used for either upper limb or lower limb and for large defects. The results were better in upper limb than in lower limb. CONCLUSIONS: Free fibular graft was found to be effective for salvaging limb function where a massive bone defect resulted from wide tumor resection in the extremities.

Journal Article↗