Epithelioid leiomyosarcoma of the external deep soft tissue.
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Diagnostic Errors↗
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Results for “Neoplasms, Connective and Soft Tissue”
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Malignant cartilaginous tumors of the mediastinum: clinicopathological study of six cases presenting as extraskeletal soft tissue masses.
Cartilage and bone-forming tumors of the mediastinum are extremely rare neoplasms with very few cases having been reported in the literature. We studied six cases of primary malignant cartilaginous tumors presenting as extraskeletal soft tissue masses in the posterior mediastinum. The patients were five women and one man aged 11 to 63 years (median, 31 years). Histologically, the lesions showed a spectrum of features that ranged from mesenchymal chondrosarcoma, to extraskeletal myxoid chondrosarcoma, to moderately well to poorly differentiated chondrosarcoma. In all cases, the lesions presented as well-circumscribed tumor masses centered in the soft tissues in the posterior mediastinum without radiographic evidence of origin from bone. Because of their relatively small size, good circumscription, focal areas of calcification, and posterior mediastinal location, the preoperative clinical diagnoses included benign neurogenic tumor and neuroblastoma. All of the lesions were treated by complete surgical excision, followed in two cases by postoperative radiation therapy. Clinical follow-up was available in five cases: two patients with mesenchymal chondrosarcoma presented with local recurrence after 3 and 7 years, one developed metastases to the sacrum 8 years after initial diagnosis and died, and one was alive and well without evidence of disease after 6 years. The patient with myxoid chondrosarcoma of the posterior mediastinum developed bilateral pulmonary metastases 10 months after surgery and has been lost to follow-up since. Our findings reinforce previous observations on the occurrence of extraskeletal cartilaginous tumors in the mediastinum and indicate that these tumors can show a propensity for local aggressive behavior with high recurrence rate and a definite potential for distant metastases. Such tumors should be considered in the differential diagnosis of malignant neoplasms presenting as a soft tissue mass in the posterior mediastinum.
Adolescent↗
Immunohistochemical studies in the differential diagnosis of small round cell soft tissue tumors.
In this review the authors focus on the differential diagnosis of small round cell tumors of soft tissue origin, giving emphasis on their specific immunohistochemical profiles. The sources of diagnostic errors and misinterpretations of the results of immunohistochemical staining are discussed in detail. It is thorough knowledge of specific characteristics of each tumor and appropriate interpretation of different staining techniques that will lead to precise classification of any given tumor, thus allowing accurate determination of prognosis and implementation of the optimal treatment.
Biomarkers, Tumor↗
Quantitative assessment of basement membranes in soft tissue tumours. Computerized image analysis of laminin and type IV collagen.
Basement membranes (BMs) in 201 soft tissue tumours were quantified using computerized image analysis of tissues immunostained for laminin and type IV collagen. The purpose of the study was to compare and quantify the extent of BM deposition in a large and varied group of benign and malignant tumours. Laminin and type IV collagen gave similar results. The difference between benign and malignant was statistically highly significant (P = 0.0001), with greater deposition in benign tumours. BM deposition was homogeneous in benign tumours and heterogeneous in sarcomas and appeared to correlate with the degree of differentiation. Some poorly differentiated sarcomas showed cytoplasmic laminin staining but little or no extracellular BM. Immunohistochemical evaluation of BM has some advantages over electron microscopy; specialized equipment is not needed and since large samples can be studied with little sampling error, heterogeneity can be studied more readily. Subjective visual assessment gives a good overall indication of the extent of BM deposition and in many situations is likely to be a suitable alternative to image analysis. Because of staining heterogeneity, BM immunohistochemistry is unlikely to be of significant value in the diagnosis of specific types of sarcoma.
Basement Membrane↗
Desmoplastic small round cell tumors of the paratesticular region. A report of six cases.
Desmoplastic small round cell tumor (DSRCT) typically occurs in the abdomen but may also present at other sites. We report six cases of paratesticular DSRCT. The patients, who ranged in age from 17 to 37 (mean, 28) years, presented with a scrotal mass (five cases) or testicular pain (one case). Grossly, the tumors were white to tan and firm. Typically, they involved the paratesticular soft tissue, serosal surfaces and the epididymis near the junction with the rete testis. Microscopically, the tumors consisted of nests of mitotically active "small blue cells" with scant cytoplasm embedded in a densely fibrotic stroma. Two tumors showed focal tubule formation; one of these also formed rosettes. The tumors exhibited the typical immunophenotype of DSRCT (positivity for keratin, vimentin, desmin, and neuron-specific enolase but nonreactivity with HBA-71 and anti-S-100). Four tumors metastasized to lymph nodes (retroperitoneal, cervical, and two unspecified); pulmonary metastases occurred in one of these cases and in one patient without lymph node metastases. One of the above patients treated with chemotherapy, died of disease at 16 months. The patients with pulmonary metastases (one of whom also had lymph node metastases) were treated with aggressive chemotherapy and are alive and apparently disease-free at 2.5 and 3 years, respectively. Three of the six patients, two of whom had known metastases, were lost to follow-up. The DSRCT of the paratestis has histologic and immunohistochemical features identical to its abdominal counterpart and must be differentiated from other "small blue cell" tumors of the paratesticular region.
Adolescent↗
Fine needle aspiration in myxoid tumors of the soft tissues.
Myxoid tumors of soft tissues constitute a histogenetically heterogeneous group of lesions, both benign and malignant, that show an overproduction of mucopolysaccharide substances. A correlative fine needle aspiration (FNA) cytologic and histologic study was performed on 16 such cases (3 intramuscular myxomas, 2 lipoblastomas, 6 myxoid liposarcomas, 4 myxoid malignant fibrous histocytomas [MFHs] and 1 extraskeletal myxoid chondrosarcoma), with electron microscopic examination of the aspirate in 4 cases. FNA of all tumors produced a gelatinous material. Smears from all but one of the tumors had a granular myxoid background substance that stained blue to blue-red with the Diff-Quik stain; the exception was the extraskeletal myxoid chondrosarcoma, whose smears stained intensely blue-red to red. On light microscopy, only lipomatous tumors had distinctive cells with diagnostic value: the lipoblasts. Although the arrangement of lipoblasts in lipoblastomas differed somewhat from that found in myxoid liposarcomas, clinical data were important for making a definitive diagnosis. Intramuscular myxomas generally showed a low cellularity composed of fibroblastlike cells with no cytologic atypia. Myxoid MFHs were composed of fibroblastlike cells and macrophagelike cells; the elongated cells with slight-to-moderate nuclear atypia predominated in tumors of low-grade malignancy, while polygonal or round cells with one or more nuclei and marked nuclear atypia predominated in high-grade tumors. Although extraskeletal myxoid chondrosarcoma was mainly differentiated from low-grade myxoid MFH by its metachromatic matrix, ultrastructural studies showed the distinctive distended cisternae with microtubular aggregates in the extraskeletal myxoid chondrosarcoma, thus contributing to the definitive diagnosis.
Adult↗
Mesenchymal tumours of the thorax: CT findings and pathological features.
Mesenchymal tumours of the thorax are uncommon and may originate from the muscle, bone, cartilage, vessel, nerves, fat and fibrous tissue. These tumours occur at different sites including the lung, pleura, mediastinum or chest wall. Mesenchymal tumours tend to show protean and non-specific imaging characteristics. However, in some instances, the imaging characteristics of the tumour may allow a specific diagnosis to be made, such as with lipoid or vascular tumours.
Adolescent↗
The histologic features of musculoskeletal tumors.
An atlas of 32 captioned color photomicrographs of typical musculoskeletal tumors is presented to demonstrate the need for close radiologic and pathologic correlation of these tumors. Diagnostic and key microscopic features of these soft tissue and bone lesions are highlighted.
Bone Diseases↗
Tumoral, quasitumoral and pseudotumoral lesions of the superficial and somatic soft tissue: new entities and new variants of old entities recorded during the last 25 years. Part IV: excerpta II.
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Adolescent↗
Regaud Lecture, Granada 1994. Tumors of the connective and supporting tissues.
There has been a continuous acceleration of medical/scientific inquiry and of actual improvements in management of patients with neoplasms of the mesenchymal tissues over the last four decades. The number of publications in this field has increased from 1140 in 1970 and then to 1700 in 1990. Important advances discussed over this period include: establishment of sarcoma teams in major oncology centers; staging systems for both soft tissue and osseous sarcomas; demonstration of genetic determinants in the development of, at least, some of the sarcomas; the revolutionary change in quality of diagnostic imaging by the introduction of CT and MRI; use of immunohistochemistry in diagnostic pathology; the drastic gains in survival of patients with osteogenic sarcoma, Ewing's sarcoma and rhabdomyosarcoma due to the efficacy of multi-drug and multi-cycle chemotherapy protocols; major advances in surgical techniques which have made limb salvage practical; cell lines derived from human sarcomas have been shown to have in vitro radiation sensitivity comparable to that of cell lines from epithelial tumors; the combination of conservative surgery and moderate doses of radiation yields local control and survival results equivalent to that of radical surgery with a much improved functional and cosmetic outcome; intra-operative electron beam radiation therapy improves the outcome of patients with retroperitoneal sarcomas when given after grossly complete resection combined with external beam radiation therapy (pre- or postoperatively); radiation is a highly effective alternative to extensive surgery for desmoid tumors; local control of giant cell tumors by modern radiation techniques is approximately 80% and the incidence of radiation induced tumors at 10 years is approximately 3%; to decrease the incidence of radiation induced sarcoma, resection has replaced radiation in the management of selected patients with primary Ewing's sarcoma when the response to chemotherapy has been excellent and the morbidity/functional decrement consequent upon the surgery judged reasonable; proton beam radiation therapy has been accepted as being superior to conventional external beam radiation therapy for chondrosarcoma and chordoma of the skull base; and attempts to utilize brachytherapy for sarcomas of the spine/sacrum appear to offer promise. Projected advances in the coming two decades includes:Designation of sarcoma type on genetic characterization; molecular genetics will provide prognostic information as to probability of distant metastasis, response to chemotherapeutic agents and radiation; important further reductions in the radiation treatment volume due to the many technical developments entering, or soon to enter the clinic; non-invasive assessment of the response to chemotherapy; much increased appreciation of the late sequella of treatment, both radiation and chemotherapy.
Humans↗
Neoplasia in zebrafish (Danio rerio) treated with 7,12-dimethylbenz[a]anthracene by two exposure routes at different developmental stages.
Using zebrafish, Danio rerio, initial pioneering work in the 1960s revealed carcinogen responsiveness of fish, yet very few subsequent tumorigenesis investigations have utilized this species. We exposed embryos (60 hours postfertilization) and fry (3 week posthatch) to 7,12-dimethylbenz[a]anthracene (DMBA) by immersion in aqueous solutions for 24 hours, at concentrations of 0-1 or 0-5 ppm (mg/L), respectively. Juvenile zebrafish 2 months posthatch were fed a diet containing 0-1,000 ppm DMBA for 4 months. Fish were sampled for histologic evaluation at 7-12 months after the onset of carcinogen treatment. Fry were most responsive to DMBA and showed the widest diversity of target tissues and histologic types of neoplasia, having several types of epithelial, mesenchymal, and neural neoplasia. The principal target tissues for carcinogenic response were liver following embryo or fry exposure, with gill and blood vessel the second and third most responsive tissues in fry. Intestine was the primary target and gill a secondary target in fish that received dietary DMBA as juveniles. These studies indicate that young zebrafish are most responsive to DMBA, showing a greater diversity of neoplasm types than rainbow trout. Thus, zebrafish are a valuable model system in which to study mechanistic aspects of the carcinogenesis process.
9,10-Dimethyl-1,2-benzanthracene↗
MRI after magnetic drug targeting in patients with advanced solid malignant tumors.
The purpose of this study was to evaluate the ability of MRI to detect magnetic particle uptake into advanced solid malignant tumors and to document the extension of these tumors, carried out in the context of magnetic drug targeting. In a prospective phase I trial, 11 patients were examined with MRI before and after magnetic drug targeting. The sequence protocol included T1-WI and T2-WI in several planes, followed by quantitative and qualitative evaluation of the signal intensities and tumor extensions. In nine patients, a signal decrease was observed in the early follow-up (2-7 days after therapy) on the T2-weighted images; two patients did not show a signal change. The signal changes in T1-WI were less distinct. In late follow-up (4-6 weeks after therapy), signal within nine tumors reached their initially normal level on both T1-WI and T2-WI; two tumors showed a slight signal decrease on T2-WI and a slight signal increase on T1-WI. Within the surveillance period, tumor remission in 3 out of 11 patients was observed, and in 5 patients tumor growth had stopped. The remaining three patients showed significant tumor growth. There was no statistically significant correlation between signal change and response. MRI is a suitable method to detect magnetite particles, deposited at the tumor site via magnetic drug targeting. MRI is therefore eligible to control the success of MDT and to assess the tumor size after the end of therapy.
Adolescent↗
Surgical strategies in the removal of malignant tumors and benign lesions of the anterior skull base.
The choice of surgical approaches to the tumors of the anterior skull base is determined by the location, dimensions of such lesions and their relations to the surrounding structures. Furthermore, the need for the reconstruction of the dura and skull base structures has an important influence on the decision about the surgical procedure. Transfacial approaches provide limited exposure, especially when tumors damage the floor of the anterior cranial fossa and involve the frontobasal dura and brain. Transcranial, craniofacial and subcranial approaches in particular may aid a surgeon in the removal of such lesions, and often these surgical procedures are the only beneficial methods. Our study comprised 15 patients. Transcranial approaches were used in ten cases. In five further cases, we adopted craniofacial or subcranial approaches. Total removal of these lesions was possible in 13 cases. Neither important complications nor death after surgery was observed except for two cases (craniofacial/subcranial approach) where the CSF leak and CNS infection were reported. We deem that the transcranial approach creates a good possibility for total removal of anterior skull base tumors, particularly of the benign lesions, and permits reconstruction of the skull base damaged by the tumor. However, in patients with large malignant tumors, the en bloc resection via the combined craniofacial/subcranial approach achieved better outcome.
Cranial Fossa, Anterior↗
The clinical and surgical aspects of spinal tumors in children.
A series of 20 pediatric patients underwent surgery for spinal tumor at the Department of Neurosurgery, Gulhane Military Medical Academy between 1995 and 2003. Motor weakness and reflex changes were the main initial signs in these patients. Epidural tumors and intradural-extramedullary tumors were in equal number, and total tumor removal was achieved in most of the patients without adjuvant treatment. Laminotomy was the main surgical method in 60% of the patients with spinal tumor, especially in children younger than 3 years of age.
Adolescent↗
Some clinical experiences with soft tissue surgery in birds.
The general principles of soft tissue surgery in birds are briefly discussed. The surgical techniques for dealing with swellings about the head, the neck, the thoracic inlet and the rest of the body are described and the problems of abdominal surgery, including gastrotomy or ventriculotomy, the surgery of the remainder of the alimentary canal and surgery of the gonads, are described.
Abscess↗
Neoplasia in zebrafish (Danio rerio) treated with N-methyl-N'-nitro-N-nitrosoguanidine by three exposure routes at different developmental stages.
We exposed embryos (83 hours postfertilizaton) and fry (3 weeks posthatch) to N-methyl-N'-nitro-N-nitrosoguanidine (MNNG) by immersion in aqueous solutions of 0-10 ppm for 1 hour (embryo) or 0-2 ppm for 24 hours (fry). Zebrafish embryos were microinjected with MNNG at levels of 0 or 96 ng/egg. Diets containing 0-2,000 ppm MNNG were fed to juvenile zebrafish for 3 months beginning at 2 months posthatch. Fish were sampled for histopathologic study at 6-12 months after initiation of carcinogen exposure. Embryos and fry were both quite responsive to MNNG; however, juvenile zebrafish were remarkably refractory to MNNG-induced neoplasia. Principal target organs in zebrafish treated as embryos with MNNG were liver and testis, with hepatocellular adenoma the most prevalent hepatic neoplasm. A variety of mesenchymal neoplasms occurred in zebrafish following embryo exposure to MNNG, including chondroma, hemangioma, hemangiosarcoma, leiomyosarcoma, and rhabdomyosarcoma. Testis and blood vessels were primary target organs for MNNG following fry exposure, with seminoma, hemangioma, hemangiosarcoma, and various other epithelial and mesenchymal neoplasms occurring. The zebrafish is a responsive, cost-effective lower vertebrate model system in which to study mechanisms of carcinogenesis.
Animals↗
Pharmacokinetics of irinotecan and its metabolites SN-38 and APC in children with recurrent solid tumors after protracted low-dose irinotecan.
Irinotecan (IRN), a topoisomerase I interactive agent, has significant antitumor activity in early Phase I studies in children with recurrent solid tumors. However, the disposition of IRN and its metabolites, SN-38 and APC, in children has not been reported. Children with solid tumors refractory to conventional therapy received IRN by a 1-h i.v. infusion at either 20, 24, or 29 mg/m2 daily for 5 consecutive days for 2 weeks. Serial blood samples were collected after doses 1 and 10 of the first course. IRN, SN-38, and APC lactone concentrations were determined by an isocratic high-performance liquid chromatography assay. A linear four-compartment model was fit simultaneously to the IRN, SN-38, and APC plasma concentration versus time data. Systemic clearance rate for IRN was 58.7 +/- 18.8 liters/h/m2 (mean +/- SD). The mean +/- SD ng/ml x h single-day lactone SN-38 area under the concentration-time curve (AUC(0-->6) was 90.9 +/- 96.4, 103.7 +/- 62.4, and 95.3 +/- 63.9 at IRN doses of 20, 24, and 29 mg/m2, respectively. The relative extent of IRN conversion to SN-38 and metabolism to APC measured after dose 1 were 0.49 +/- 0.33 and 0.29 +/- 0.17 (mean +/- SD). No statistically significant intrapatient difference was noted for SN-38 area under the concentration-time curve. Large interpatient variability in IRN and metabolite disposition was observed. The relative extent of conversion and the SN-38 systemic exposure achieved with this protracted schedule of administration were much greater than reported in adults or children receiving larger intermittent doses.
Adolescent↗
[Three new cases of induction of osteogenic tumors with the aid of intra-osseous administration of radium bromide].
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Animals↗