[PRIMARY SARCOMA OF THE GALLBLADDER].
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Fibrosarcoma↗
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Results for “SARCOMA, FIBROSARCOMA”
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Moloney murine sarcoma virus 349 induces Kaposi's sarcomalike lesions in Balb/c mice.
Moloney murine sarcoma virus (MoMuSV349) is produced by MuSV349 cells in at least eight-fold excess over the replication-competent helper virus. Less than 48-hours-old Balb/c mice inoculated intraperitoneally with supernatant from MuSV349 cells containing approximately 10(4) MuSV349 infectious units developed clinical symptoms, including severe generalized wasting, 15 to 20 days after inoculation. These infected mice became moribund 35 to 45 days after inoculation. Gross examination of the bodies revealed the presence of cutaneous and subcutaneous 0.2-cm to 1.5-cm macules, plaques, or nodules located predominantly on the ventral abdomen and legs. Nodules also were found in the spleen, liver, ovaries, testes, meninges, nerves, and skin. The nodules were semisoft, cystic, or solid and some expressed variable amounts of blood. Histologic examination of the macules, plaques, and nodules showed spindlelike cells intermingled with tortous, jagged vascular channels lined by plump and normal endothelial cells and unlined slitlike spaces filled with erythrocytes. These angiomatous lesions were infiltrated extensively with neutrophils, lymphocytes, macrophages, and some plasma cells. In some cases the lesions also included foci of densely packed eosinophils. These angiomatous lesions are clearly distinguishable from the fibrosarcomas induced by the myeloproliferative sarcoma virus (MPSV), and resemble the sarcomas induced in mice by Gz-MSV and Balb MSV, the sarcomas induced in rats by MPSV and Ha-MSV, and the acute generalized form of Kaposi's sarcoma (KS) associated with acquired immune deficiency syndrome (AIDS) in humans. Electron microscopy also revealed the presence of numerous extracellular type C virions and virions budding from the plasma membrane of endothelial and spindlelike cells. Erythrophagocytosis by the endothelial and spindlelike cells was demonstrated by light and electron microscopy. The widely disseminated lesions appear to have developed simultaneously as a consequence of viremia rather than metastasis.
Animals↗
The ubiquitous fibroblast. Multiple oncogenic potentials with illustrative cases.
The wide range of oncogenic proliferative potentials of the fibroblast is demonstrated with a series of eight patients. Diagnoses included infantile digital fibromatosis, "aggressive fibromatosis," aggressive fibromatosis progressing to poorly differentiated sarcoma, infantile myofibromatosis, recurrent desmoid tumor, fibrosarcoma arising in a keloid, dermatofibrosarcoma protuberans, and malignant fibrous histiocytoma of left atrium. Still other types of fibroblastic tumefactions might have been included. Oncogenic factors that may have been operative in the causation of the lesions presented include: genetic factors, sex-linked factors, hormonal factors, numerous growth factors, and certain viruses, especially retroviruses. Certain fibromatoses in children are commonly self-limited and need only be monitored carefully as the process regresses. Aggressive fibromatosis, on the other hand, can prove fatal if the lesion is not completely resected with a wide margin and, occasionally, the process may become frankly malignant, with metastases. The standard triad of excisional surgery, radiotherapy, and chemotherapy has been used to treat frankly malignant fibrous tumors with variable results.
Adult↗
Cyclophosphamide versus ifosfamide: final report of a randomized phase II trial in adult soft tissue sarcomas.
Ifosfamide (IFOS) 5 g/m2 and its parent analog Cyclophosphamide (CYCLO) 1.5 g/m2 were studied in a randomized phase II study, accruing 171 patients with advanced soft tissue sarcoma. Both drugs were administered as 24 hr infusions, every 3 weeks, with comcomitant Mesna 400 mg/m2 i.v. bolus 4 hourly X 9 doses. Twenty-four patients were ineligible and 12 were not evaluable. The groups were well matched for age, previous chemotherapy (42% of the total) or radiotherapy, the presence of distant metastases and performance status, but there were more females (59% vs. 45%) in the IFOS arm. Among the 68 evaluable patients receiving IFOS, there were 2 CR, 10 PR (overall response 18%), 27 SD and 29 PD. For CYCLO, the corresponding results in 67 patients were 1 CR, 4 PR (overall response 8%), 23 SD and 39 PD. Using the chi-square test the P values for response rate and linear trend were 0.13 and 0.04 respectively. Response rates were higher for females (20% vs. 5%, P = 0.01) and patients who had not received previous chemotherapy (19% vs. 4%, P = 0.01). Fourteen of the 17 responses came from a group of 43 females, who had not received previous chemotherapy, for whom the overall response rate was 37.5%. Remissions were noted in only 4 histological subtypes (centrally reviewed material), i.e., 5 of 17 synovial sarcomas, 7 of 13 mixed mesodermal sarcomas and 2 of 7 fibrosarcomas. One of the 31 leiomyosarcomas responded to Cyclophosphamide. Durations of response did not differ significantly between the 2 arms--median 26, range 10-81+ weeks. Leucopenia was significantly more severe on CYCLO, particularly in patients who had received previous chemotherapy (P = 0.007). Serious infections occurred in approx. 7% of patients with no difference between the two drugs, although there was one toxic death on CYCLO. Nausea and vomiting were significantly worse on IFOS and alopecia, related in extent to dose, was seen in both arms. Other side-effects, such as hematuria or rises in serum creatinine and encephalopathy, were infrequent and mild. A higher response rate with less myelosuppression suggests that IFOS may have advantages over CYCLO in combination therapy.
Adolescent↗
Differentiation of sarcomas.
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Bone Neoplasms↗
EXTRACORPOREAL ARTERIAL PERFUSION.
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Animals↗
[Breast sarcoma (examination of 35 cases)].
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Adult↗
Neoplasms induced by megavoltage radiation in the head and neck region.
Radiation-induced cancer, although fortunately a rare complication of radiotherapy, is nonetheless observed occasionally even after megavoltage radiation has been used. Over a 22-year period at the Curie Institute, four patients were found to have malignant neoplasms within the fields of megavoltage treatment given for various cancers of the head and neck region. Three of the neoplasms were sarcomas, two osteogenic and one fibrosarcoma, and the other tumor was a sarcomatoid epithelioma. The latent period ranged from 3 1/2-15 years. Although the evidence is strong that the neoplasms were causally related to the precedent irradiation, it is acknowledged that rare examples of the "double primary" phenomenon exist, even separated by five or more years, and that only one such instance would induce a large error in the estimated frequency of postirradiation neoplasms. Because clinical estimates after megavoltage irradiation are usually compounded by an association with a relatively high total absorbed dose, the issue of the incidence of postirradiation neoplasms as a function of the type of external beam (orthovoltage vs. megavoltage) may require resolution by experimental means. Another rare sequela of radiotherapy is injury to a peripheral nerve. One of the four patients with a second neoplasm after radiation also developed left hypoglossal nerve palsy 2 1/2 years post-therapy, and left optic nerve atrophy seven years postradiation treatment of a squamous cell carcinoma of the ethmoid and maxillary sinuses. The 51-year-old patient had received a tumor dose of 6800 rads (2043 rets).
Carcinoma, Squamous Cell↗
Expression of Ki-1 antigen (CD30) in mesenchymal tumors.
Expression of CD30(Ki-1) antigen has long been considered to be restricted to activated lymphocytes and related tumors. However, expression of this antigen has also been detected in embryonal carcinomas, in nonembryonal carcinomas, in malignant melanomas, and even in some myeloid cell lines and macrophages at late stages of differentiation. In this study, using monoclonal antibody Ki-1, expression of CD30 antigen was immunohistochemically examined in frozen sections of 28 benign and 63 malignant mesenchymal tumors. The authors found CD30 expressed in two of four leiomyomas, seven of 11 leiomysarcomas, one of six rhabdomyosarcomas, two of two aggressive fibromatoses, one of three fibrosarcomas, two of four synovial sarcomas, one giant cell tumors of tendon sheaths, all five malignant fibrous histiocytomas, all three osteosarcomas, one of three Ewing's sarcomas, in a tumor cell subpopulation of two of ten malignant schwannomas, and in the Schwann cell compartment of one of two ganglioneuromas tested. Furthermore, CD30 was consistently expressed in the myoepithelial compartment of 13 fibroadenomas. However, all five lipomas, all seven liposarcomas, all three neuroblastomas, both ganglioneuroblastomas, both chondrosarcomas, and tumors of disputed origin tested were consistently CD30 negative. These findings indicate that, outside the lymphatic system, CD30 antigen is not restricted to epithelial neoplasms but may also be present in tumors of mesenchymal origin. The authors conclude that CD30 antigen, although having limited utility in the differential diagnosis of tumors of questionable histogenesis, may eventually define relevant subgroups within the main tumor categories.
Antibodies, Monoclonal↗
The cytogenesis of macrophages and osteoclast-like giant cells in bone tumors with special emphasis on the so-called fibrohistiocytic tumors.
Recent investigations have suggested that osteoclasts and osteoblasts belong to different cell systems: osteoclasts originate from hemopoietic stem cells, most probably via precursors of the mononuclear phagocyte system. Osteoblasts, however, arise from local mesenchyme. The present classification of bone tumors issued by the WHO, however, is still based on the assumption of osteoclasts and osteoblasts being merely different manifestations or differentiations of the same basic cell type. Consequently, histiocytes or macrophages as well as osteoclast-like giant cells are interpreted in most bone tumors as an autochthonous component of the tumor. In the present study, this theory is contradicted by histological immunohistological, electron microscopic, and autoradiographic-electron microscopic results on a larger number of osteosarcomas, chondromas, chondrosarcomas, chondroblastomas, aneurysmal bone cysts, giant cell tumors of bone, malignant fibrous histiocytomas, fibrosarcomas, desmoplastic fibromas, Ewing's sarcomas, fibrous dysplasias, nonossifying fibromas, and malignant hemangioenkdotheliomas of bone. In order to elucidate the role of macrophages and osteoclast-like giant cells, different monoclonal antibodies were applied to bone tumor specimens as markers of mononuclear macrophages and giant cells. The concept of what is called fibrohistiocytic tumors should be reconsidered. Immunohistological studies have shown that in malignant fibrous histiocytoma as well as in giant cell tumors of bone only a certain portion of macrophages will react with the highly specific antibodies, whereas the majority of tumor cells are negative. This finding alone suggests that the infiltration of macrophages is a reactive phenomenon. It is in agreement with earlier autoradiographic and electron microscopic investigations on giant cell tumors of bone, confirming that only the fibroblast-like tumors are actually proliferating. These data were verified in the present study with the aid of double labeling immunohistological techniques, using antibodies against mature tissue macrophages and others against a proliferation-associated nuclear antigen. Only the fibroblast-like cells, which do not react with the macrophage-specific antibody, will express the proliferation-associated nuclear antigen. Analogous results were obtained in malignant fibrous histiocytoma. We may infer from these results that the majority of these tumors must be neoplasms of local mesenchyme, mostly in fibroblastic differentiation, while the considerable number of macrophages is seen as a reactive phenomenon.(ABSTRACT TRUNCATED AT 400 WORDS)
Bone Neoplasms↗
Bone tumours in Cameroon: incidence, demography and histopathology.
A multicentre analysis was carried out on bone tumours in Cameroon during a 10-year period. Registers and patient records of five pathology laboratories were consulted, and all patients with a histological report of a bone tumour were included in the study. A total of 268 bone tumours were studied and the average incidence was 27 tumours a year, or two per one million inhabitants. Of these tumours 48% were benign, 45% were primary bone cancers and only 6% were metastatic disease. Among the primary malignant bone tumours, osteosarcoma was the most frequent (39%), followed by non-Hodgkin's primary bone lymphoma, fibrosarcoma, chondrosarcoma, and Ewing's sarcoma. Primary site of the metastatic bone tumours was prostatic adenocarcinoma, breast cancer, hepatocarcinoma and thyroid cancer. In Cameroon many bone tumours are not diagnosed due to lack of medical facilities and little awareness among our medical staff. It is likely that the real incidence is at least ten times higher than that shown in our report.
Adolescent↗
[Desmoid-type fibromatosis (aggressive fibromatosis)].
Desmoid-type fibromatoses (aggressive fibromatoses) represent infiltrative, locally destructively growing soft tissue tumors with a high potential for recurrence. Desmoid tumors of 33 adult patients were analysed regarding clinical and morphological aspects (sex, age distribution, site, size, mitotic rate, tumor microvessel density, surgical margins, additional radiotherapy). Possible statistical correlations were examined using log-rank-tests. No prognostic significance of tumor microvessel density was evident. A correlation between mitotic index (1 or more mitoses per 50 high power fields) and local relapse rate was notably striking, but not statistically significant (log-rank: 0.17). Additional postoperatively applied radiotherapy proved to be statistically significant to avoid local recurrences (log-rank: 0.01). The presented results may indicate an increased risk for local relapse in those desmoid-type fibromatoses which are mitotically active. Postoperative radiotherapy seems to be effective in the treatment of aggressive fibromatosis to avoid tumor recurrence. Differential diagnosis of desmoid-type fibromatosis/aggressive fibromatosis in adulthood include various fibroblastic/myofibroblastic soft tissue tumors such as nodular fasciitis, fibrosarcoma, low-grade fibromyxoid sarcoma, myofibroblastic sarcoma as well as leiomyosarcoma and soft tissue leiomyoma.
Adult↗
Anaplastic carcinoma of the thyroid with rhabdomyosarcomatous differentiation: a report of two cases.
Anaplastic carcinoma of the thyroid gland (ACT) is a highly malignant tumor that is almost invariably associated with a fatal outcome. It demonstrates a variety of peculiar histological features, with squamoid, giant cell and spindle cell growth patterns. The spindle cell variant of ACT is usually indistinguishable from a true sarcoma and it can simulate fibrosarcoma, malignant fibrous histiocytoma (MFH), hemangiopericytoma and angiosarcoma or rhabdomyosarcoma. Although a rhabdomyosarcomatous appearance has sometimes been mentioned in the literature, true skeletal muscle differentiation has never been consistently proved. We report two cases of ACT with rhabdomyosarcomatous differentiation, as demonstrated by means of immunohistochemistry and electron microscopy. Both cases disclosed a very similar histological appearance, with a main population of small, pleomorphic, round-to-oval cells arranged in a storiform pattern, admixed with scattered pleomorphic giant cells, an image similar to that of the usual type of MFH. Stains for epithelial markers showed only few, scattered, weakly positive cells. Thyroglobulin and calcitonin were negative in tumor cells in both cases. On the contrary, positivity to vimentin was strong and generalized. Immunomarkers of muscular differentiation showed a consistent positivity. At the ultrastructural level, the cells disclosed the same spindle and pleomorphic morphology, with large, bizarre nuclei and cytoplasm with abundant mitochondria, rough endoplasmic reticulum, secretory granules and lipid droplets. There were also cells with wide cytoplasm filled with filamentous material, either of actin or myosin, as well as Z-band material. In conclusion, the cases reported here show a clear-cut rhabdomyosarcomatous differentiation of ACT, confirmed both immunohistochemically and ultrastructurally, a feature not previously reported in the literature. These findings may contribute to the broadening of the differentiation spectrum of this unusual neoplasm.
Carcinoma↗
gamma-Propoxy-sulfo-lichenin, an antitumor polysaccharide derived from lichenin.
A water-soluble semisynthetic polysaccharide, gamma-propoxy-sulfo-lichenin (PSL), was prepared by reaction of propansultone with lichenin, a natural occurring beta-1.3/1.4-linked glucan originating from Cetraria sp. PSL represents a class of mixed-linked beta-glucans with long and hydrophilic side chains in position C-6 of the glucan backbone. PSL with a degree of substitution of 0.8 and an average molecular weight of 250 kDa exhibited a strong antitumor activity in doses of 25 and 5 mg/kg against solid sarcoma 180 (100% resp. 82% tumor inhibition). The antitumor activity of PSL was shown to be dependent on the dimension of the molecule: the higher the average molecular weight, the higher was the inhibition rate obtained in the antitumor assay. No antitumor effect was observed by using a pretreatment of animals prior to transplantation of sarcoma 180. On syngenic DBA/2-MC.SC1 fibrosarcoma PSL inhibited tumor growth by about 88% at a concentration of 25 mg/kg. PSL failed to exhibit any direct cytotoxic effects on hormone-independent MDA-MB 231 mammacarcinoma. For PSL an indirect antitumor effect via modulation of the host mediate immune defence is postulated.
Animals↗
An unusual case of malignant cystosarcoma phyllodes of the breast.
A unusually large tumor of the left breast diagnosed as a cystosarcoma phyllodes with multiple malignant sarcomatous changes of the stroma, consisting of liposarcoma, myxoid fibrosarcoma, anaplastic, and giant cell sarcoma is described. The weight of 6200 g (13.5 lb) seems to be the largest so far presented in the literature reviewed by the authors.
Breast Neoplasms↗
Crosstalk between the Wnt pathway and other signaling pathways.
The Wnt/β-catenin signaling pathway is a deeply conserved regulatory network that governs embryonic development, stem cell maintenance, and tissue homeostasis. Aberrant activation of the Wingless/Integrated protein (Wnt) signaling is a hallmark of numerous human diseases, most prominently in colorectal cancer, where it cooperates with additional oncogenic pathways to drive tumor initiation, progression, and therapeutic resistance (See Supplementary Table 1 for a list of the abbreviations used in this manuscript and their definitions.). Increasing evidence indicates that Wnt signaling does not function as an isolated linear cascade but rather as an integrative signaling hub that dynamically interfaces with major signaling pathways, including the RAS-RAF-MAPK and PI3K-AKT-mTOR pathways. Rat Sarcoma protein (RAS)- Rapidly Accelerated Fibrosarcoma protein (RAF)- Mitogen-Activated Protein Kinase (MAPK) and Phosphoinositide 3-Kinase (PI3K)- Ak strain transforming protein (AKT)- Mechanistic Target of Rapamycin (mTOR) pathways. These interactions occur at multiple molecular levels, encompassing shared kinases, transcriptional regulators, metabolic nodes, and cytoskeletal components, thereby coordinating proliferative, metabolic, and migratory programs. In this review, we synthesize current mechanistic and clinical insights into the crosstalk between Wnt signaling and the RAS-RAF-MAPK and PI3K-AKT-mTOR pathways, with particular emphasis on colorectal cancer. We discuss how these signaling networks converge to regulate β-catenin stability, transcriptional activity, cell adhesion, and metabolic reprogramming, thereby generating oncogenic phenotypes that cannot be explained by activation of individual pathways alone. To illustrate the evolutionary conservation and biological significance of these interactions, we integrate developmental paradigms from early Xenopus embryogenesis, where Wnt signaling governs zygotic genome activation, body axis formation, and the regulation of cell growth, protein stability, and biomass accumulation. Finally, we examine how an improved understanding of Wnt-centered signaling networks is informing emerging therapeutic strategies, including combinatorial pathway inhibition and nanoparticle-based drug delivery. Collectively, this review highlights Wnt signaling as a central integrator of developmental and oncogenic programs, providing a conceptual framework for understanding signaling network crosstalk and identifying new therapeutic opportunities in cancer.
Humans↗
An analysis of inadvertent perforations of mucosa and skin concurrent with mandibular reconstruction.
PURPOSE: This article reports on the incidence and outcome of inadvertent perforations of mucosa and skin during the reconstruction of segmental defects of the mandible using cancellous cellular bone. PATIENTS AND METHODS: This study reviews 11 patients experiencing an inadvertent perforation of skin and/or mucosa among 211 consecutive patients (5.2%) undergoing reconstruction of the mandible with this graft model. RESULTS: Eleven patients experienced 16 perforations of either mucosa (n = 14) or skin (n = 2). Six diagnoses necessitated these 11 reconstructions where a perforation was encountered, including ameloblastoma (n = 3), chronic osteomyelitis (n = 2), stage III osteoradionecrosis (n = 2), fibrosarcoma (n = 1), synovial cell sarcoma (n = 1), stage IV squamous cell carcinoma (n = 1), and odontogenic myxoma (n = 1). Fourteen of the 16 perforations occurred intraoperatively, and 2 occurred postoperatively (dehiscence). Eleven of the 16 perforations occurred at either the distal or proximal segment, while 5 of the perforations occurred in the mid portion of the segmental defect tissues. Postreconstruction follow-up ranged from 11 to 70 months (mean, 28.5 months). Infection occurred in 1 of the 11 patients (9.1%) with resultant partial graft loss. Protocols are proposed that serve to preserve a contamination-free tissue bed and minimize or eliminate infection of the graft when a perforation is encountered. CONCLUSION: An inadvertent perforation of mucosa or skin does not result in automatic graft infection and failure when proper intraoperative management is carried out. When the graft perforates through the mucosa postoperatively, proper wound management similarly can preserve the graft. This information reinforces the fact that it is not necessary to abort reconstructive surgery once a perforation is identified intraoperatively, nor to debride an entire graft that becomes exposed postoperatively.
Bone Transplantation↗