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PubMed1998

Osteosarcoma of the larynx.

About 11,600 cases of laryngeal cancer were reported in the United States in 1995, accounting for 1% of all reported cancers. Fewer than 1% of laryngeal cancers are sarcomas, osteosarcoma being the rarest type. Twelve cases of laryngeal osteosarcoma have been cited in the literature. A 47-year-old man presented with hoarseness following a benign vocal polypectomy. The entire larynx appeared swollen, although both vocal folds moved well. Biopsies did not reveal pathologic tissue. Computed tomographic imaging revealed a destructive, expansile lesion of the thyroid cartilage. A computed tomography-guided biopsy of this cartilage revealed a high-grade sarcoma. Laryngeal osteosarcoma was diagnosed following total laryngectomy. Laryngeal osteosarcoma is a highly malignant neoplasm with early hematogenous spread. Survival statistics for peripheral osteosarcoma are poor. Limited experience reveals that a combination of surgery and radiotherapy with adjuvant chemotherapy can offer some palliation. The literature and published cases of laryngeal osteosarcoma are reviewed.

Combined Modality Therapy↗
PubMed1999

ImmTher, a lipophilic disaccharide derivative of muramyl dipeptide, up-regulates specific monocyte cytokine genes and activates monocyte-mediated tumoricidal activity.

ImmTher, a liposome-encapsulated lipophilic disaccharide tripeptide derivative of muramyl dipeptide, previously showed activity against liver and lung colorectal metastases in a phase I trial. The purpose of the current studies was to investigate whether ImmTher could up-regulate specific cytokine gene expression and protein production, as well as activate the tumoricidal or cytostatic activity of human monocytes. ImmTher induced the expression and production of interleukin(IL)-1alpha IL-1beta, IL-6, IL-8, IL-12, macrophage chemotactic and activating factor, and tumor necrosis factor alpha but not IL-2 or IL-10. Cytostatic or cytotoxic monocyte activity was stimulated against human Ewing's sarcoma, osteosarcoma, and melanoma cells but not breast cancer cells. Production and secretion of these cytokine proteins may play a role in the antitumor activity of ImmTher.

Acetylmuramyl-Alanyl-Isoglutamine↗
PubMed2004

Management of sarcomas of the head and neck.

Soft tissue and bone sarcomas in the head and neck are rare tumors. The 1000 to 1500 yearly cases in the United States are distributed among at least 10 main histologies and multiple head and neck subsites. Although this makes structured studies difficult to perform and high-level evidence-based treatment algorithms difficult to find, basic treatment recommendations can be made from the existing literature for most histologies and subsites. This paper discusses the epidemiology, natural history, and treatment approaches for several of the most common head and neck sarcomas, including the "adult soft tissue sarcomas," osteosarcoma, chondrosarcoma of the larynx, angiosarcoma, rhabdomyosarcoma, desmoid tumors, and dermatofibrosarcoma protuberans.

Chondrosarcoma↗
PubMed1999

Malignant bone tumors of the chest wall.

Malignant bone tumors constitute only 0.2% of all tumors. Bone sarcomas occur at a rate approximately one tenth that of sarcomas of the soft tissue. Malignant bone tumors of the chest wall and sternum are even more rare because most bone tumors occur in the long bones or joints. Because of the relative paucity of experience treating these malignancies, progress in successful therapies has been limited. Chondrosarcomas remain the most common bony malignant chest wall lesions and are discussed elsewhere in this issue. Other lesions in descending order of incidence include Ewing's sarcoma, osteosarcoma, malignant fibrous histiocytoma, solitary plasmacytoma, and Askin tumors. This article reviews these remaining five malignant bony chest wall tumors, along with their symptoms, presentations, and current approaches to therapy.

Bone Neoplasms↗
PubMed1988

[In vivo 31 phosphorus spectroscopy of tumors: pre-, intra- and post-therapy].

Using 31P-MR spectroscopy spectra with good signal-to-noise ratio were obtained in five different types of tumours (Ewing's sarcoma, osteosarcoma, malignant melanoma, metastases from a squamous cell carcinoma, parotid adenoma). Surface coils were used. Short- and long-term follow-up after chemotherapy was possible in some cases. In the short-term follow-up, changes in the phosphocreatine and inorganic phosphate resonances could be observed within minutes after the start of the infusion. In the longer follow-ups, changes in phosphodiester and phosphomonoester resonances were observed within two days. There were no significant changes in tissue pH during treatment, but increased pH values were observed in all tumours.

Adenoma↗
PubMed1996

Resting energy expenditure in dogs with nonhematopoietic malignancies before and after excision of tumors.

OBJECTIVE: To determine whether apparently resting dogs with nonhematopoietic malignancies have increased resting energy expenditure (REE), compared with clinically normal dogs. ANIMALS: 46 client-owned dogs with nonhematopoietic malignancies and 30 client-owned dogs that were clinically normal. PROCEDURE: Apparently resting, client-owned dogs with nonhematopoietic malignancies before (n = 46) and 4 to 6 weeks after (n = 30) surgical removal of tumors were compared with apparently resting, clinically normal, client owned dogs (n = 30). An open flow indirect calorimetry system was used to determine the following: rate of oxygen consumption (ml/min/kg of body weight); rate of carbon dioxide production (mls/min/kg), REE (kcal/kg/d) and respiratory quotient. Because of the wide range of body weight, REE and oxygen consumption were also expressed per kg of body weight 0.75. RESULTS: Surgical removal of the tumor did not significantly alter any of the variables measured when all dogs with tumors were assessed as a single group, or when the dogs were divided on the basis of having the following types of tumors: carcinomas and sarcomas, osteosarcomas, and mammary adenocarcinomas. None of the data obtained prior to surgical treatment from any of the dogs grouped by tumor type were significantly different from clinically normal dogs. CONCLUSIONS: REE (54.4 +/- 16 kcal/kg/d or 125 +/- 19 kcal/kg0.75/d) and, presumably, caloric requirements of dogs with nonhematopoietic malignancies are not significantly different from those obtained from clinically normal dogs (53.9 +/- 16 kcal/kg/d or 116 +/- 32 kcal/kg0.75/d). Furthermore, these variables do not change significantly when the tumor is excised and the dog is reassessed after 4 to 6 weeks. CLINICAL RELEVANCE: Knowledge that REE in dogs with solid tumors is not significantly different from REE of clinically normal dogs may be of value when planning nutritional treatment for dogs with nonhematopoietic malignancies.

Adenocarcinoma↗
PubMedDate not supplied

The management of limb-length discrepancies in children after treatment of osteosarcoma and Ewing's sarcoma.

The authors present 15 cases of discrepancy of the lower limbs > 5 cm, in children in whom the histological diagnosis was Ewing's sarcoma or osteosarcoma, who were treated using polychemotherapy or radiotherapy or both in some cases and limb-salvage surgery. Ten of these patients required compensation for discrepancy by limb lengthening. In three patients, a second lengthening had to be carried out. A shortening of the contralateral femur was performed in two cases, and in another two, a femur-tibia epiphysiodesis was done. The average lengthening achieved was 8.1 cm with a healing index of 41 days/cm. Leg-length discrepancy in the treatment of sarcomas of the musculoskeletal system is an important sequela, especially when treating children. Compensation for this can be obtained by lengthening healthy bone, with a complication rate similar to that of other etiologies. The difference compensated depends on the biological capacity of bone regeneration of the individual and, therefore, is a definitive solution.

Adolescent↗
PubMed2004

Distal lower extremity sarcomas: frequency of occurrence and patient survival rate.

BACKGROUND: Primary sarcomas in the distal leg, tibia, fibula, ankle, and foot are uncommon and are believed to be less malignant than those that arise in other sites, but only limited information is available to support this contention. METHODS: Using a computerized system containing extensive information regarding over 14,000 patients, mostly with tumors treated by our center over a 25-year period, 175 sarcomatous lesions with MSTS stage I, II, and III were located in the distal lower extremity. These were compared with 2367 lesions of similar diagnoses in other body parts. The principal studies included diagnostic distribution and outcome (recorded as death as a result of disease). Data were compared for diagnosis, gender, age, Musculoskeletal Tumor Society (MSTS) stage, anatomic site, and treatment methods and evaluated statistically by chi-square methods. RESULTS: The most frequent distal lower limb tumors were synovial cell sarcoma, osteosarcoma, and Ewing's tumor and the percentage distribution of the various tumors for that site as compared with the rest of the body was quite different. Of even more importance was the remarkable difference in outcome with the death rate for the lower limb tumors set at 10%, while the same tumors at other sites had a death rate of 27% (p <.000002). Furthermore, gender, stage, age, and the type of operative procedure showed highly significant differences between the tumors of the lower leg and those of the remainder of the body. CONCLUSIONS: On the basis of these data, it is evident that far fewer sarcomas occur in the lower leg, tibia, fibula, ankle, and foot than in other body sites and that their diagnostic distribution is different. Of perhaps greater interest is the fact that the rate of metastasis and death is markedly reduced for this site as compared with others. The authors speculate on the causes of this remarkable alteration in numbers and outcome.

Adult↗
PubMed2000

Analysis of the p16INK4, p14ARF, p15, TP53, and MDM2 genes and their prognostic implications in osteosarcoma and Ewing sarcoma.

We examined alterations of the p16INK4, p14ARF, p15, TP53, and MDM2 genes in 30 osteosarcomas and 24 Ewing sarcomas. Among 21 osteosarcomas and 24 Ewing sarcomas, p16INK4, p14ARF, and p15 abnormalities were found in 4 (19%), 2 (9%), and 3 (14%) osteosarcomas, respectively, and in 4 (17%), 3 (13%), and 4 (17%) Ewing sarcomas, respectively. The alterations of p16INK4, p14ARF, and p15 included homozygous deletions spanning all 3 genes, methylation of p16INK4 or p15, and a nonsense mutation of p16INK4, which simultaneously caused a missense mutation of p14ARF. Alterations of TP53 were found in 15 (50%) of 30 osteosarcomas and 1 (3%) of 24 Ewing sarcomas. None of the sarcomas showed MDM2 amplification. While TP53 abnormalities were far more frequent in osteosarcoma than in Ewing sarcoma, alterations of p16INK4, p14ARF, and p15 were present at similar frequencies in the two types of sarcoma. The event-free survival (EFS) was worse in Ewing sarcoma patients with p16INK4 and p14ARF mutation/deletion than in those without the mutation/deletion (P = 0.019), and EFS was worse in osteosarcoma patients with TP53 alterations than in those without TP53 alterations (P = 0.048). The different incidence of TP53 abnormalities in the 2 types of sarcoma may reflect differences of the molecular processes through which the 2 types of tumor develop.

Adolescent↗
PubMed1996

Cytogenetic studies on fine-needle aspiration samples from osteosarcoma and Ewing's sarcoma.

The preoperative multidrug chemotherapy of osteosarcoma and Ewing's sarcoma patients requires a reliable diagnosis. There are several advantages with obtaining the diagnosis by fine-needle aspiration (FNA) and cytologic examination. Although cytologic criteria have been defined, adjunctive methods such as cytogenetic analysis are valuable to support the diagnosis. We have cytogenetically analyzed FNAs from 18 primary bone sarcomas (six osteosarcomas and 12 Ewing's sarcomas). Two of the osteosarcomas showed abnormal, complex karyotypes seen in most highly-malignant osteosarcomas. Seven Ewing's sarcoma aspirates displayed abnormal karyotypes; five of these had the characteristic 11;22 translocation, and in one of these cases molecular genetic analysis revealed the hybrid EWS/FLI1 transcript. Since only two of six osteosarcomas showed clonal changes, chromosomal analysis of FNAs from suspected osteosarcoma seems to be of limited value, but may in some cases support the diagnosis of high-grade malignancy. In Ewing's sarcomas, however, the finding of an 11;22 translocation was valuable and strongly supported the cytologic diagnosis. As shown in one case, the material obtained by FNA is sufficient for cytologic, cytogenetic, and molecular genetic analysis.

Adolescent↗
PubMed2002

Surgical treatment of malignant tumors of the right heart.

Seven patients with malignant cardiac tumors were treated surgically in the Department of Cardiothoracic Surgery of the University of Tokyo between 1981 and 2000. Their treatments and outcomes are summarized and discussed. The ages of the patients ranged from 21 to 70 years old (mean: 49.5+/-15) and there were three males and four females. The histopathological diagnoses were hepatocellular carcinoma (HCC), spindle cell sarcoma, round cell sarcoma, osteosarcoma, renal cell carcinoma, and leiomyosarcoma. In four of the cases, the tumor extended or metastasized from other organs, while in the other three cases it originated in the heart. Before the cardiac operation, an above-knee amputation, left nephrectomy, transarterial embolization, or extended right hepatic lobectomy had been performed to treat the primary site of the tumor. Tumor resection using cardiopulmonary bypass was performed in every case. The NYHA classification of heart failure was significantly improved (preop: 3.3+/-0.8, postop: 1.9+/-0.7 [P<0.001]). The mean survival period of the patients who died was 8.8+/-7.0 months. A patient with renal cell carcinoma is still alive after 87 months of follow-up. In summary, surgical treatment of malignant tumors of the right heart can improve the QOL in patients with cardiac failure. However, its effectiveness was temporary in all cases except one case of renal cell carcinoma.

Adult↗
PubMed1994

Point mutations in the mdr1 promoter of human osteosarcomas are associated with in vitro responsiveness to multidrug resistance relevant drugs.

Among human sarcomas, osteosarcomas usually display high intrinsic mdr1 expression while malignant fibrous histiocytomas (MFH) do not. A comparative polymerase chain reaction (PCR)-based sequence analysis of the mdr1 promoter revealed point mutations in seven out of nine osteosarcomas at nucleotides +103 (2 cases T-->C) and +137 (5 cases G-->T). No changes were seen in eight MFHs. When COS cells transfected with CAT constructs containing the T-->C chloramphenicol acetyltransferase mutant mdr1 promoters were treated with vincristine or doxorubicin, expression of the CAT gene was enhanced to a higher extent than with constructs containing wild-type or G-->T-mutant mdr1 promoters. We suggest that there is a correlation between the type of mdr1 promoter mutation and responsiveness to MDR relevant drugs.

ATP Binding Cassette Transporter, Subfamily B, Mem↗
PubMed1975

[Sarcomas associated with Paget's disease].

The authors report 12 cases of Paget's disease with sarcomatous degeneration that were observed among 311 patients with Paget's disease hospitalized in the Montpellier Rheumatology Clinic. They compare their experience with information they were able to extract from the literature in French and English. The frequency of such degeneration in cases of Paget's disease is difficult to determine but did not appear to exceed 1%. Degeneration rarely occurred before the age of 50 years, affected men twice as frequently as women, and occurred particularly in cases of diffuse Paget's disease, mainly in the femur or the humerus; the rachis was rarely affected. Pain was the main symptom, was practically constant, was remarkable because of its permanence and its intensity. Tumefaction was frequently seen. Pathological fractures were seen in almost a third of the patients with sarcomas of the long bones. Radiculo-medullary compression characterized the rare cases with involvement of the vertebral column or the sacrum. Radiography showed rupture of cortical layers with invasion of the soft parts without any periostal reaction. Histological investigation confirmed the diagnosis by demonstrating one of the three types of osteogenic sarcoma: osteosarcomas were the most frequent. Less frequently giant-cell sarcomas or reticulosarcomas were found. The affected patients nearly always died, survival at five years being rare. Treatment, amputation or radiotherapy, was disappointing.

Aged↗
PubMed2006

[Preliminary report of combination chemotherapy including Arsenic trioxide for stage III osteosarcoma and Ewing sarcoma].

OBJECTIVE: To determine the efficacy of Arsenic trioxide combined with other chemo agents in patients with metastatic osteosarcoma and Ewing sarcoma. METHODS: From December 2002 to June 2005, 32 patients with metastatic osteosarcoma and Ewing sarcoma were treated with chemotherapy comprised Arsenic trioxide, including 19 male and 13 female, aging from 7 to 32 years. Diagnoses included 27 cases with osteosarcomas and 5 with Ewing sarcomas. Twenty-five and 7 patients had multiple lung and skeletal metastasis respectively. All patients had received 4 to 6 cycles standard chemotherapy and surgical treatments before the metastatic lesions were found. The chemotherapy protocol consisted of Arsenic trioxide, VP-16 and paclitaxel. Twenty-eight patients accepted 2 cycles, and 4 patients accepted 3 cycles chemotherapy. RESULTS: Five patients (15.6%), whose lung focus disappeared and no new metastases proved, got complete response (CR); Six patients (18.8%) got partial response (PR); In thirteen stable disease (SD) patients (40.6%), the metastatic lesions did not reduce in evidence but completely calcified in 2 patients and partly calcified in 11 patients; Eight patients (25%) whose tumor size was even bigger after the chemotherapy were defined as progress disease (PD). The average follow-up period was 20 months (ranging from 6 months to 3 years). Three patients were eventually died; The disease was progressed in 5 patients, and the tumor growth was controlled in 24 patients. CONCLUSIONS: Arsenic trioxide is a low-toxic and effective remedy for treatment of stage III osteosarcoma and Ewing sarcoma.

Adolescent↗
PubMed2002

Primary skeletal neoplasms: osteosarcoma and ewing sarcoma.

The care for children and adolescents with malignant bone tumors such as osteosarcoma and Ewing sarcoma will be improved by the magnetic resonance imager that can recognize the tumor early in its course and can stage it correctly for proper chemotherapy and local control. This can be accomplished by attention to the proper magnetic resonance imaging technique, recognition of the magnetic resonance appearance, and knowledge of the prognostic factors at diagnosis. Determination of response on follow-up static and dynamic contrast-enhanced magnetic resonance imaging and knowledge of optimal methods to determine recurrent tumor will improve the outcome for these children.

Adolescent↗
PubMed2006

Pulmonary metastasectomy in pediatric patients.

This article describes the historical development of pediatric pulmonary metastasectomy but demonstrates that progress has been slow in understanding its proper applications. Because many pediatric metastatic tumors are rare, surgeons have grouped together patients of different histologies for the generation and analysis of case series. By examining tumor types individually, however, it is seen that certain histologies (adrenocortical carcinoma, alveolar soft part sarcoma, osteosarcoma) mandate surgical metastasectomy for patient survival. Other pediatric tumors (Wilms tumor, Ewing's sarcoma) are radiation sensitive, and the application of metastasectomy is controversial. In the case of still other types of tumor (neuroblastoma, differentiated thyroid cancer, rhabdomyosarcoma), metastasectomy is seldom performed except in highly unusual situations. Techniques for minimally invasive biopsy and for muscle-sparing thoracotomy are described for pediatric patients.

Biopsy↗