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PubMed1985

[Pseudomalignant, hyperacute circumscribed myositis. Contribution and limitations of echography. Review of the literature. Apropos of a case].

The very sudden onset of a painful muscular tumefaction was suggestive of a para-ostal osteosarcoma or myosarcoma. Ultrasound imaging showed a heterogeneous echogenic mass with several extending shadow cones, raising the possibility of the benign nature of the lesion, but urgent operation showed the pseudomalignant circumscribed myositis nature of the affection.

Adult↗

PubMed1988

Chemically induced rhabdomyosarcomas in rats. Ultrastructural, immunohistochemical, biochemical features and expression of alpha-actin isoforms.

A series of 14 primary and two metastatic rat rhabdomyosarcomas (RMS) induced with nickel sulfide was studied by light microscopy, transmission electron microscopy, indirect immunofluorescence, avidin-biotin-peroxidase immunohistochemistry and two-dimensional gel electrophoresis. Monoclonal or affinity-purified polyclonal antibodies were used for the immunohistochemical demonstration of vimentin, desmin, alpha-smooth muscle (alpha-sm) actin and alpha-sarcomeric (alpha-sr) actin. By histological and ultrastructural studies, four categories of RMS were diagnosed on the basis of the neoplastic cell types. These were: (1) well-differentiated RMS (n = 2), (2) pleomorphic RMS (n = 8), (3) embryonal RMS (n = 4), and (4) embryonal myosarcomas (n = 2). Immunohistochemically, all these neoplasms expressed desmin and alpha-sr actin, reflecting their rhabdomyoblastic origin. Two dimensional gel electrophoresis performed on five neoplasms demonstrated alpha, beta and gamma actins spots in all cases. This study demonstrates that the alpha-sr actin antibody represents a good marker for rhabdomyoblastic differentiation is useful in the diagnosis of RMS since it was present in all morphologically confirmed RMS and in two ultrastructurally undifferentiated sarcomas positive for desmin. Neoplastic cells positive for alpha-sm actin were noted in 11 confirmed RMS. Double indirect immunofluorescence showed that all alpha-sm and alpha-sr positive cells also contained desmin. Co-expression of alpha-sr and alpha-sm actins was studied in serial sections of formalin-fixed, paraffin-embedded tumor tissue. Both alpha-sm and alpha-sr actins were localized in some rhabdomyoblasts. This study confirms our previous observations in human tumors and shows, for the first time, that alpha-sr and alpha-sm actins can be present in the same neoplastic cell in vivo.

Actins↗

PubMed1988

[A case of primary malignant fibrous histiocytoma of the right lung].

A malignant fibrous histiocytoma (MFH) is the most common soft tissue sarcoma found in adults, but a primary MFH of the lung is very rare. A chest X-ray taken of a 73-year-old man revealed a massive lesion in the right upper lobe, and a subsequent right upper lobe lobectomy uncovered a soft, light yellow tumor in the right S3 area, measuring 2.8 x 2.6 x 1.7 cm. Histologically, the tumor manifested a typical storiform pattern of spindle-sharp cells with no sign of a myosarcoma. As no abnormal lesion was detected in the abdomen and extremities, the tumor was diagnosed as a primary MFH of the lung.

Aged↗

PubMed2004

Myofibrosarcoma.

Myofibrosarcomas are malignant tumours of myofibroblasts, which have been recognised for many years, but have become clearly defined only recently. They are low- or high-grade sarcomas that arise in soft tissue or bone in adults or children. Low-grade myofibrosarcomas are infiltrative tumours, usually in deep soft tissue, with a predilection for the head and neck region, which display a range of microscopic appearances from fasciitis-like to fibrosarcoma-like; all cases at least focally display nuclear pleomorphism. They express smooth-muscle actin and calponin, and some express desmin, but most lack h-caldesmon. Low-grade myofibrosarcomas can recur but rarely metastasise. Their differential diagnosis is from benign myofibroblastic proliferations, such as fasciitis and fibromatosis, as well as from fibrosarcoma and leiomyosarcoma. Pleomorphic myofibrosarcomas are high-grade pleomorphic sarcomas (malignant fibrous histiocytoma), which show ultrastructural evidence of myofibroblastic differentiation. They closely resemble malignant fibrous histiocytoma clinically and morphologically, but are more frequently actin positive. This article describes the low- and high-grade variants of myofibrosarcoma and other malignant tumours with myofibroblastic differentiation.

Adult↗

PubMed2005

Myofibrosarcoma of the adrenal gland.

Adrenal masses have varying presentations. Most commonly, adrenal masses are discovered incidentally on CT or MRI during an evaluation for an unrelated complaint. Although the majority of these are nonfunctional cortical adenomas, hormonally active tumors and adrenocortical carcinoma must also be considered in the differential diagnosis. Rarely, retroperitoneal tumors may mimic an adrenal mass. We report a case of a 49-year-old man with anemia and weight loss who was found to have a large retroperitoneal mass arising from the adrenal gland. Surgical treatment involved en bloc resection of the right kidney, adrenal gland, segments 7 and 8 of the liver, and a portion of the right hemidiaphragm. Final pathology revealed a low-grade myofibrosarcoma. We believe that this is the first case report of a myofibrosarcoma of the adrenal gland. Myofibrosarcomas are rare malignant tumors composed of myofibroblasts that arise from the deep soft tissues. These tumors have a predilection for the head and neck, trunk, or extremities. Myofibrosarcomas can be differentiated from other sarcomas by immunohistochemical staining and pathologic features. We will briefly discuss the workup of an adrenal mass and focus on the diagnosis of myofibrosarcoma.

Adrenal Gland Neoplasms↗

PubMed1993

[Non-urothelial tumors of the urinary tract].

Only about 2% of the urinary tract are not of urothelial origin. Our knowledge of their morphology and biology is mainly based on single case reports, and therefore apart from a few exceptions very poor. Generally, the most often affected site is the urinary bladder (79.2%), followed by the urethra (12.7%), pelvis (4.9%) and ureter (3.2%). The urinary bladder also is the only organ in which all different histological types of these tumors were described. According to their histogenesis non-urothelial tumors (NUT) can be classified by the following main groups: soft tissue tumors, mixed epithelial and non epithelial tumors (carcinosarcomas), neuroendocrine carcinomas, carcinoids, malignant lymphomas, malignant melanomas and extragonadal germ cell tumors. Moreover some very interesting tumor-like lesions, like malakoplakia and inflammatory pseudosarcoma, mainly occur in this region. About 75% of all NUT of the urinary tract belong to the soft tissue tumors. Rhabdomyosarcomas in children and leiomyomas and -myosarcomas in adults are the more frequent histological types. Leiomyosarcomas can easily be confused with other tumor types or even with inflammatory pseudotumors. The use of immunohistochemistry to achieve a correct diagnosis is mandatory but not always successful. A relatively frequent tumor occurring in the bladder of young adults is the paraganglioma (pheochromocytoma), which usually produces typical symptoms of catecholamine excess. Carcinosarcomas of the urinary bladder contain both epithelial and mesenchymal components. They have to be distinguished from collision tumors (coexistent but separate carcinoma and sarcoma), spindle cell transitional carcinomas as well as from carcinomas with osseous or cartilaginous metaplasia. Carcinoids and neuroendocrine carcinomas developed from the neuroendocrine cells scattered all over the transitional epithelium of the bladder. Neuroendocrine carcinomas of the bladder are also called "oat cell carcinomas" since they show the same histological features and immunoreactivity as the oat cell carcinomas of the lung. They share also the same poor prognosis. The affection of the urinary tract in generalized malignant lymphomas and leukemias occur in more than 30% of cases. Lymphomas, primarily localised in the urinary bladder are, however, extremely rare. The most frequent ones are low grade non Hodgkin lymphomas, although 3 cases of Hodgkin disease and a few cases of primary extramedullary plasmacytoma of the bladder have been reported, too.(ABSTRACT TRUNCATED AT 400 WORDS)

Carcinoma↗

PubMed2007

Myofibroblastic sarcoma of the nasal cavity and paranasal sinus: a clinicopathologic study of 6 cases and review of the literature.

OBJECTIVE: We describe the clinicopathologic features of 6 cases of myofibroblastic sarcoma (MS) occurring in the nasal cavity and paranasal sinus. STUDY DESIGN: The paraffin-embedded tissues of 6 cases of MS were stained immunohistochemically and examined by electron microscopy. RESULTS: Clinically, a painless enlarging mass was the most common symptom, followed by the nasal obstruction, epistaxis, copious rhinorrhea, and proptosis. Histologically, the tumors showed a diffusely infiltrative growth pattern and consisted mainly of spindle cells with abundant eosinophilic cytoplasm. The hypocellular myxoid areas and the hypercellular fibrous areas were identified. Immunohistochemically, all 6 tumors were positive for vimentin, alpha-smooth muscle actin, calponin, and fibronectin. Ultrastructural examination in 3 cases showed characteristic features of myofibroblast. Follow-up in 6 patients revealed high local recurrence rate (6 out of 6). CONCLUSION: Myofibroblastic sarcoma of the nasal cavity and paranasal sinus exhibit diverse histologic appearances and a strong aggressive behavior.

Actins↗

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↗

PubMed1978

Carcinogenicity of nickel subsulfide for respiratory tract mucosa.

The carcinogenicity of nickel subsulfide, Ni3S2, for respiratory tract epithelium was studied in heterotopic tracheal transplants with doses of 1 and 3 mg Ni3S2 per trachea. Chemical determinations indicated that Ni3S2 persisted in the tracheas for seven to nine months. Ni3S2 showed marked toxicity for mucociliary epithelium, resulting in widespread atrophy and focal epithelial necrosis during the first two months of exposure. The submucosa showed mononuclear infiltration and signs of fibroblastic and capillary proliferation. Tumor studies indicated that Ni3S2 can induce carcinomas in tracheal epithelium. The carcinoma incidence was 10% at 1 mg and approximately 1.5% at 3 mg. The higher dose produced a 67% incidence of fibro- and myosarcomas. The data suggest that, compared to some carcinogenic polycyclic hydrocarbons, Ni3S2 may not be a strong carcinogen for the epithelium of conducting airways. The data are discussed in light of other experimental studies and of epidemiological findings on respiratory tract cancers in nickel workers.

Animals↗

PubMed2006

Stent fabrication for graft immobilization following wide surgical excision of myofibroblastic sarcoma of the buccal mucosa: a clinical report.

Sarcomas of the head and neck are rare tumors derived from cells of mesenchymal origin. This article briefly discusses the epidemiology, etiology, and classification of head and neck sarcomas. Emphasis is placed on myofibrosarcoma, a malignant tumor of the myofibroblasts. Histologic criteria, prognostic factors, and the multidisciplinary management of these tumors are reviewed. A situation of a surgically excised myofibrosarcoma of the buccal mucosa is reported. In this patient, a stent was fabricated to stabilize a split-thickness skin graft used for the buccal mucosa reconstruction.

Adult↗

PubMed1975

[Concentration of 99mTc-tin-phosphate complexes in soft tissues].

The concentration of 99mTc-pyrophosphate was determined in the lower extremities of rabbits (normal, abacterial and bacterial affected soft tissues), in osteoarthritis of the hip joint (capsule and muscle) as well as in knee joint effusions. Compared with the 85Sr-concentration, reflecting the calcification capacity, concentrations of 99mTc-pyrophosphate in soft tissues were found to be lower 2 hours p.i., but were up to elevenfold higher 24 hours p.i. These findings should be due to a fixation of 99mTc-pyrophosphate in collagen containing tissues as in the soft tissue tumors (myosarcoma, synvialioma, breast cancer) presented. A mechanism of delayed equilibration could explain augmented uptake in lymph-edema, ascites and effusions in florid osteoarthritis of the knee joint. The possible dependence of 99mTc-pyrophosphate concentration in bone and soft tissue on collagenous contents is discussed.

Animals↗

PubMed1983

[Proliferative myositis, a muscular pseudo-sarcoma. Apropos of a case with an ultrastructural study].

A 40-year old woman with a history of rapidly growing tumor of the brachio-radialis muscle is described. By light microscopy, the tumor had typical features of proliferative myositis, with alternating areas of well preserved muscular fibres and strands of proliferating spindle shaped cells and ganglion like cells. Electron microscopic study showed non specific degenerative muscle changes, fibroblasts and giant cells. The histogenesis and the nosologic place of proliferative myositis are discussed.

Adult↗

PubMed2004

Low-grade myofibroblastic sarcoma of the vulva.

BACKGROUND: Vulvar sarcomas are rare tumors. A distinctive low-grade myofibroblastic sarcoma is described. CASE: A 46-year-old female presented with a progressively enlarging vulvar mass. Pathological evaluation revealed a low-grade myofibroblastic sarcoma. The immunophenotype is outlined and ultrastructural features are highlighted. Tumor cells were positive for p53 protein, smooth muscle actin, steroid receptors, and showed myofibroblastic differentiation on electron microscopy. The patient has been followed for >14 months without evidence of recurrence. CONCLUSION: The tumor was positive for p53, mitotically active, but was categorized as a low-grade malignancy. Immunohistochemical and ultrastructural criteria were utilized to distinguish this tumor from other neoplasms.

Female↗

PubMedDate not supplied

Leiomyosarcoma versus myofibrosarcoma: observations and terminology.

Two spindle cell sarcomas, which showed similarities in light microscopic histology and immunostaining and were diagnosed as leiomyosarcomas by these criteria, were compared ultrastructurally to show the value of electron microscopy in subtyping these neoplasms. Both were subcutaneous, case 1 occurring in the nasolabial fold and case 2 in the upper calf. Both consisted of fascicles of spindle cells, and both stained positively for vimentin and alpha-smooth muscle actin; only case 2 stained additionally for desmin. Case 1 showed strong and case 2 weak or negative staining for fibronectin. By electron microscopy, case 1 contained prominent rough endoplasmic reticulum, peripheral fine filaments with focal densities, and fibronexus junctions. By contrast, case 2 was characterized by an external lamina and well-developed bundles of fine filaments with focal densities. Case 2 was considered a typical leiomyosarcoma, and case 1 was interpreted as showing myofibroblastic differentiation. The nomenclature for these myofibroblastic tumors is discussed, and myofibrosarcoma and leiomyosarcoma, myofibroblastic variant are suggested as suitable terms.

Adult↗

PubMed1989

Primary cardiac tumours--operative treatment of 20 patients.

This report describes a series of 20 patients operated on for a primary cardiac tumour. The majority of the tumours (16) were benign myxomas; 12 of them were located in the left atrium, two in the right atrium and two were biatrial. Two lipomas were found; one was epicardial and the other was located in the left atrium. The only intraventricular tumour was a malignant left ventricular myosarcoma. The propensity of intracardiac tumours to embolize was distinctive. Nine of the 16 myxomas presented with peripheral embolization, and in two patients surgery was complicated by fatal perioperative cerebral embolization of myxomatous tissue. Furthermore, in one patient embolization of a left atrial lipoma necessitated amputation of her left arm before cardiac surgery. Late postoperative recurrences were found in two patients with atrial myxomas. In one of them, reoperation showed that the tumour had grown at that site in the interatrial septum where the original pedicle had been excised. One patient developed severe mitral valve regurgitation and underwent replacement with a prosthetic valve at reoperation. Otherwise our late follow-up study showed that the results of surgery were usually excellent even though mild echocardiographic abnormalities were not uncommon. Our experience emphasizes the embolic potential of intracardiac myxomas and suggests, furthermore, that to avoid recurrences excisions with wide margins should be preferred. Echocardiography is an optimal method for the follow-up of these patients.

Echocardiography↗

PubMed1992

Myofibrosarcoma of subcutaneous soft tissue of the cheek.

A low grade soft tissue sarcoma from the naso-labial fold and designated as myofibrosarcoma (sarcoma of myofibroblasts) is described using standard light microscopy techniques, immunohistochemistry and electron microscopy. Pink fibrillated cytoplasm, regarded as one of the typical histological features of leiomyosarcoma, was not obvious but immunostaining for alpha-smooth muscle actin was positive suggesting smooth muscle differentiation. By electron microscopy, tumour cells contained abundant rough endoplasmic reticulum cisternae and a large Golgi body; there were modest but numerous bundles of fine filaments with focal densities. A conventional lamina was lacking but the cell surface was characterised by fibronexus junctions in which there was a conspicuous extracellular, fibronectin-containing fibril, apparently mediating contact between cell and matrix. The tumour cells therefore showed myofibroblastic differentiation. The cell surfaces showed strong immunoreactivity with an anti-fibronectin antibody. Myofibrosarcoma is not a widely recognised entity and this case is only the 7th example to be documented in detail by means of both immunohistochemistry and ultrastructure. The combination of electron microscopy for detecting the fibronexus junction and immunostaining for fibronectin at the cell periphery is suggested as potentially useful for distinguishing myofibrosarcoma from leiomyosarcoma.

Actins↗

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↗