Proliferative disorders of myofibroblasts.
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Fibrosarcoma is a rare tumour in children. The potential of malignancy has been questioned. We present three cases of fibrosarcoma in children . The follow-up periods range from 10 to 37 years. The first patient had pulmonary metastases at the time of diagnosis in 1958. The primary tumour in fossa ischio-rectalis was resected in 1960. Lung metastases were resected in 1960 and 1989. Radiotherapy was given in 1992. He is still alive with metastases 37 years after the first manifestation of disease. The second patient had a primary tumour and several local recurrences in the mandible. He is alive without evidence of disease 4 years after resection of pulmonary metastases and 21 years after resection of the primary tumour. The third patient has no signs of recurrence or metastatic spread 10 years after a wide excision of subcutaneous tumours of the left upper arm. The cases demonstrate a special tumour-entity of low-grade malignancy, which show a good prognosis and a wide spectrum of biological behaviour.
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We report a 67 years old male with a history of pulmonary tuberculosis at the age of 15, that consults for malaise, weight loss and productive cough. Chest X ray examination showed a left pleural effusion. A pleural tap obtained a sterile exudate. A thorax CAT scan showed a proliferating mass in the splenic angle of the colon, with left lung and diaphragmatic invasion. Endoscopic biopsies confirmed the diagnosis of adenocarcinoma. The patient underwent a subtotal colectomy with partial excision of diaphragm and left lung. The pathological report showed a mucosecretory adenocarcinoma, infiltrating the pericolonic adipose tissue without adjacent organ infiltration and a chronic inflammatory process involving colonic serosa, diaphragm pleura, and lung. Adjuvant chemotherapy was indicated and the patient is asymptomatic and without evidences of tumor recurrence after a 24 months follow up.
A 38-year-old patient presented with an anterior mediastinal mass after chemotherapeutic and surgical treatment for lung metastases from a malignant histiocytoma. Because of the risk for tumour recurrence the thymic mass was resected. Thymic hyperplasia was found on pathological examination. In this case thymic hyperplasia is a rebound phenomenon aflcer chemotherapy. It appears to atrophy during the administration of chemotherapy and regrow afterwards. Surgical resection provides the definitive diagnosis and treatment.
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The Authors report a case of extraskeletal Ewing's sarcoma of thoracic wall in a 15-year-old sport-man, who complained about pain in the left upper extremity. The diagnosis was performed by radiology (ultrasound study, TC, RM) and pre-operative needle-aspiration biopsy. The patient was treated by neoadjuvant chemotherapy, wide surgical resection and, then, radiotherapy. Neither local recurrence nor metastases have developed for 8 months.
A case of intranodal myofibroblastoma with amianthoid fibres was studied by histology, immunohistochemistry and electron microscopy, in a Canadian Caucasian male presenting with an inguinal mass which appeared following a sports injury. Tumour cells were spindled and formed haphazard interlacing fascicles with intervening areas of haemorrhage. They were positive for vimentin, alpha-smooth-muscle actin and HHF35. By electron microscopy, they displayed moderate numbers of rough endoplasmic reticulum cisternae, fine actin-sized filaments in cell processes, and discrete stretches of unambiguous lamina ('external' lamina), sometimes in association with short attachment plaques. The fibronectin fibrils and fibronexus junctions characteristic of myofibroblasts were not seen. The absence of fibronexus junctions and the presence of surface features typical of smooth-muscle cells (attachment plaques with overlying lamina) suggest that this tumour is not myofibroblastic but is exhibiting a degree of smooth-muscle differentiation. The findings confirm earlier observations suggesting that some intranodal myofibroblastomas are not myofibroblastic, but show a form of smooth-muscle differentiation.
GIST have still today controversial aspects of their histogenesis that are reflected on the classification and prognosis. It's showed a particular case with mixed muscular-neuronal histogenesis that supposes totipotent cell origin of these neoplasms. Diagnosis, classification and prognosis are very difficult to evaluate; surgery at the moment is the only useful treatment modality.
The authors report a case of benign tumor of the psoas muscle occurring in a 36-year-old man. The diagnosis was based on computed tomography and the patient underwent tumorectomy by an extrapoeritoneal approach. The authors emphasize the ratity f psoas tumors that are usually malignant and the advantage of computed tomography and percutaneous biopsy. Treatment must be incorporated into a multidisciplinary approach in which surgery plays a leading role and must be based on the treatment of soft tissue tumors.
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Electron microscopy in the diagnosis and academic study of myofibroblastic lesions is discussed. Myofibroblasts from granulation tissue and tumor stroma are regarded as the nearest equivalent to a "normal" myofibroblast population with which to define myofibroblastic differentiation in tumoral and pseudotumoral lesions. Histological features include a plump-spindle-cell morphology, with an ill-defined cytoplasm paler and less fibrillar than in smooth-muscle cells, and matrix collagen. Myofibroblasts stain for alpha-smooth-muscle actin, fibronectin, and vimentin. Desmin is found in some lesional myofibroblasts. The main ultrastructural features are prominent rough endoplasmic reticulum, modestly developed myofilaments with focal densities ("stress fibers"), and fibronexus junctions. The latter are foci on the cell surface where intracellular myofilaments and extracellular fibronectin filaments converge. Myofibroblastic lesions vary in the extent to which they mirror this overall phenotype. Hypertrophic scar, Dupuytren's disease, nodular fasciitis, the fibromatoses, and inflammatory myofibroblastic tumors have the most developed myofibroblastic features. Keloid, postoperative spindle-cell nodule, and fibroma of tendon sheath are less well differentiated. Myofibroblastoma is among many lesions described as myofibroblastic which, however, appear to show a kind of smooth-muscle differentiation. Some spindle-cell malignancies express myofibroblastic features.
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