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PubMedDate not supplied

Myofibrosarcoma (low-grade myofibroblastic sarcoma) with intracytoplasmic hyaline (fibroma-like) inclusion bodies.

Myofibrosarcoma is a controversial neoplasm composed of cells with differentiation toward myofibroblasts. The authors report an unusual case of myofibrosarcoma in which, in addition to the characteristic features reported, tumor cells contained intracytoplasmic hyaline (fibroma-like) inclusion bodies. A 66-year-old man complained of a painless enlarged mass in his right shoulder over the previous 6 months. The tumor recurred 2 years after operation. On histologic examination, the tumor displayed diffusely infiltrative growth with isolation of individual skeletal muscle fibers. The tumor cells were most often arranged in an intersecting (herringbone) fascicular pattern but a vaguely storiform pattern was also observed. Cellularity varied from one area to another. The cells were spindle-shaped, with ill-define pale eosinophilic cytoplasm, and a wavy or tapering nucleus with fine chromatin and small nucleoli. Intracytoplasmic hyaline inclusion bodies of variable size, often located adjacent to the nucleus, were visible in numerous spindle and stellate cells. The mitotic index was 3/10 HPF. The tumor was scored as grade 1. Immunohistochemistry revealed positive staining to muscle-specific actin, desmin and vimentin in most tumor cells. Intracytoplasmic hyaline inclusion bodies did not react specifically to any one antibody, but showed a marked ring-like immune reaction, particularly to muscle-specific actin. Electron microscopy showed tumor cells with indented nuclei and small nucleoli, abundant rough endoplasmic reticulum, micropinocytotic vesicles, and longitudinally arranged fine filaments with focal electron-dense patches and subplasmalemmal plaques. The most striking feature was the presence of large, globular or ball-like, non-membrane-bound, randomly scattered clusters of fine filament, usually adjacent to the nucleus.

Aged↗
PubMed1993

Divergent myosarcomatous differentiation in retroperitoneal liposarcoma.

We describe four patients with retroperitoneal liposarcomas undergoing myosarcomatous differentiation. The patients (two men and two women) were 47, 48, 68, and 72 years of age when first seen. The primary tumors were large retroperitoneal, well-differentiated liposarcomas, one featuring areas of dedifferentiation (without muscle elements). A myosarcomatous component became evident at the first recurrence in three cases and at the second recurrence in one. This component was always within dedifferentiated areas and in three of the cases coincided with the emergence of the latter. The muscle component had exclusively leiomyosarcomatous phenotype (alpha-smooth-muscle actin reactivity) in one case, exclusively rhabdomyosarcomatous phenotype (myoglobin reactivity) in two cases, and combined leiomyosarcomatous and rhabdomyosarcomatous phenotype (alpha-smooth-muscle actin and myoglobin) in one case. Ultrastructural studies of one of the tumors with a rhabdomyosarcomatous component revealed the presence of sarcomeres. Two patients died of extensive retroperitoneal disease, one patient died following the attempted removal of a recurrence, and one patient is alive and free of disease. These cases demonstrate that the dedifferentiated component of liposarcoma may exhibit a myosarcomatous component, a feature analogous to that previously described in dedifferentiated chondrosarcoma.

Aged↗
PubMed1998

Low-grade myofibroblastic sarcoma: analysis of 18 cases in the spectrum of myofibroblastic tumors.

The clinicopathologic, immunohistochemical, and ultrastructural features of a seemingly distinctive low-grade spindle cell sarcoma showing myofibroblastic differentiation have been analyzed in a series of 18 patients. The age range of the patients (7 women and 11 men) was 19-72 years (median: 42 years). A painless, enlarging mass was the most common clinical presentation. Five tumors arose in the oral cavity (including four lesions in the tongue), four in the lower extremities and three in the upper extremities, four cases in the abdominal/pelvic cavity, and two on the trunk. Eight soft-tissue cases involved skeletal muscle, three cases were located in perifascial tissues, and two arose in subcutaneous tissue. Tumor size ranged from 1.4 to 17 cm (median: 4 cm); in six cases (of which four were abdominal/pelvic) the lesion was larger than 5 cm. All patients were treated surgically, and four received additional adjunctive therapy. Histologically, most cases were cellular lesions showing a diffusely infiltrative pattern, and were composed of spindle-shaped tumor cells arranged mainly in fascicles. Tumor cells had poorly defined, palely eosinophilic cytoplasm and fusiform nuclei, which were either tapering and wavy or plumper and vesicular with indentations and small inconspicuous nucleoli. Tumor cells were set in a collagenous matrix often with prominent hyalinization. Mild nuclear atypia was noted in 16 cases; in the other 2 cases, and in the metastases of one other lesion, a greater degree of nuclear atypia was seen. In all but one case, the mitotic rate ranged from 1 to 6 mitoses in 10 HPFs (mean: 2/10 HPFs); in a single case, there were more than 20 mitoses in 10 HPFs. Immunohistochemically, all cases stained positively for at least one myogenic marker; 12 cases were positive for desmin, 11 for alpha-smooth muscle actin, and 6 for muscle actin (HHF35). Seven neoplasms were desmin positive/ alpha-smooth-muscle actin negative, and five cases were desmin negative/alpha-smooth-muscle actin positive emphasizing the variable immunophenotype of myofibroblastic lesions. In addition, 7 of 10 tumors stained at least focally positive for fibronectin. Ultrastructural examination in five cases showed characteristic features of myofibroblasts. Follow-up in 11 patients (median: 29 months) revealed local recurrence in 2 cases, and multiple distant soft-tissue, intraosseous, and pulmonary metastases in one other patient. Low-grade myofibroblastic sarcoma seems to represent a distinct entity in the spectrum of low-grade myofibroblastic neoplasms and is distinguishable from fibromatosis, myofibromatosis, solitary fibrous tumor, fibrosarcoma, and leiomyosarcoma.

Actins↗
PubMed2001

Myofibrosarcoma of the bone: a clinicopathologic study.

Myofibroblastic tumors are fairly recently established soft tissue neoplasms. Although most of them appear to be benign, myofibrosarcoma of the soft tissue, seemingly their malignant counterpart, have been reported. We describe the clinicopathologic and radiologic features of four cases of myofibrosarcoma arising from the bone. All but one of the patients were women ranging in age from 60 to 71 years. Two tumors occurred in the metaphyses of distal femurs and the others arose in the iliac bones. On radiologic examination all tumors exhibited well-demarcated lytic destructive lesions without periosteal reaction. Two tumors were localized in the bone, whereas the other two extended into surrounding soft tissues. Histologically, all tumors were composed principally of a mixture of a cell-rich fascicular area and a hypocellular fibrous area. In the former area tumor cells had rather eosinophilic spindle-shaped wavy cytoplasm and were arranged in interlacing fascicles and small storiform patterns with variable numbers of inflammatory cells. Tumors occasionally showed prominent pleomorphism, and large cells with hyperchromatic nuclei were seen. In contrast, hypocellular areas had various features, including collagenous, hyalinous scar-like and rarely keloid-like areas. Focal coagulation necroses were present in all but one tumor. Immunohistochemically, the tumors were positive for vimentin, muscle actin (HHF35), alpha-smooth muscle actin, calponin, and desmin, whereas all of them were negative for high molecular weight caldesmon. On follow-up there was one fatal case with distant metastases, whereas the clinical courses of other cases after wide resection were excellent. Myofibrosarcoma of the bone has distinctive histopathologic features, which should be distinguished from those of other bone tumors with myoid differentiation.

Actins↗
PubMed1998

Cancer of the endometrium and corpus uteri.

Uterine cancer is often diagnosed at an early stage and is therefore considered one of the most curable gynecologic malignancies. Despite this, a substantial number of women who present at more advanced stage or with unfavorable histologies suffer significant morbidity and death from this disease. Research continues along several fronts in an attempt to improve the prognosis for this group of women. Basic scientific research has continued to evaluate mechanisms of carcinogenesis in the hope that better targets for treatment and prevention of disease will be found. Epidemiologic studies have attempted to further define risk factors as well as elucidate risk in those patients receiving combination estrogen and progestin hormone replacement therapy. Clinical studies have further defined prognostic factors, and examined new surgical staging techniques and the need for adjuvant therapy after primary surgery. However, treatment options for advanced and recurrent disease remain limited.

Combined Modality Therapy↗
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↗
PubMed2003

Myxofibrosarcomas contain large numbers of infiltrating immature dendritic cells.

Myxofibrosarcoma is a malignant tumor with distinctive histologic features and is believed to be derived from fibroblasts. The function of infiltrating myeloid cells in myxofibrosarcoma is poorly understood. It previously has been shown that a combination of dendritic morphologic features and expression of the C-type lectin, dendritic cell-specific intercellular adhesion molecule-3 grabbing nonintegrin (DC-SIGN), is useful for identifying DC populations in tissue sections. In the present study, we found that 3% to 61% (median, 22%) of cells in myxofibrosarcomas express DC-SIGN and have dendritic morphologic features. These DC-SIGN--positive cells are not in cell cycle and are consistent with infiltrating DCs. The percentage of DCs in myxofibrosarcomas is independent of tumor grade. It previously has been shown that DC-SIGN--positive cells are either immature DCs or DCs that predominantly activate TH2 cells, both subsets likely to give rise to ineffective antitumor responses. The DC-SIGN--positive DCs that we have identified in myxofibrosarcoma may, therefore, be involved in the induction of ineffective immune responses or even tolerance to tumor antigens.

Biomarkers, Tumor↗
PubMed2006

The role of myostatin and bone morphogenetic proteins in muscular disorders.

Skeletal muscle is the largest organ in the human body, and plays an important role in body movement and metabolism. Skeletal muscle mass is lost in genetic disorders such as muscular dystrophy, muscle wasting and ageing. Chemicals and proteins that restore muscle mass and function are potential drugs that can improve human health and could be used in the clinic. Myostatin is a muscle-specific member of the transforming growth factor (TGF)-beta superfamily that plays an essential role in the negative regulation of muscle growth. Inhibition of myostatin activity is a promising therapeutic method for restoring muscle mass and strength. Potential inhibitors of myostatin include follistatin domain-containing proteins, myostatin propeptide, myostatin antibodies and chemical compounds. These inhibitors could be beneficial for the development of clinical drugs for the treatment of muscular disorders. Bone morphogenetic protein (BMP) plays a significant role in the development of neuromuscular architecture and its proper functions. Modulation of BMP activity could be beneficial for muscle function in muscular disorders. This review will describe the current progress in therapy for muscular disorders, emphasising the importance of myostatin as a drug target.

Animals↗
PubMed1983

Restriction of host range of xenotropic pseudotype murine sarcoma virus by helper leukemia virus.

We investigated the restriction of the host range to infectivity of MSV by helper leukemia virus in vivo. When newborn SD-rats were inoculated intracerebrally, subcutaneously, intraperitoneally or intramuscularly with xenotropic pseudotype Kirsten MSV, Ki-MSV(BV2), either brain tumors or myogenic sarcomas were induced, depending upon the route of inoculation. However, no tumors developed in SW-Icr mice inoculated with Ki-MSV(BV2) either intracerebrally or intramuscularly at birth. Ecotropic Ki-MSV(Ki-MuLV) induced myogenic sarcomas in mice when inoculated intramuscularly and also induced brain tumors and myogenic sarcomas in rats when inoculated intracerebrally and intramuscularly, respectively. Thus, the host range of pseudotype MSV appeared to depend on a helper leukemia virus.

Animals↗