1/9. Malignant myxoid endobronchial tumour: a report of two cases with a unique histological pattern.AIMS: To present two cases of malignant endobronchial myxoid tumours with a highly distinctive sarcomatoid pattern not previously described at this site, and discuss their histogenesis in relation to previously documented endobronchial neoplasms. methods AND RESULTS: Both tumours presented in young adult females and were purely sarcomatoid with interweaving cords of small uniform, rounded or slightly elongated cells lying within a myxoid stroma. The stroma was alcian blue positive, but sensitive to hyaluronidase in both cases. The tumour cells contained a small volume of periodic acid-Schiff-positive eosinophilic cytoplasm and stained positively for vimentin only, but there also was a prominent background population of CD68-positive dendritic cells. Ultrastructural studies showed that the tumour cells contained an excess of rough endoplasmic reticulum, with some of the cisternae appearing dilated, and scalloping of the cell surfaces, although no intracisternal tubules were identified. CONCLUSIONS: Although the histological pattern was most reminiscent of extraskeletal myxoid chondrosarcoma, the sensitivity of the stroma to pretreatment with hyaluronidase precluded the diagnosis. However, there were similarities with the sarcomatoid component of malignant salivary gland-type mixed tumours of the lung and this tumour possibly represents a variant of a bronchial gland tumour. Despite this uncertainty over origin, this pattern should be recognized as part of the differential diagnosis of myxoid tumours in the lung, as an apparently indolent type of malignant endobronchial neoplasm.- - - - - - - - - - ranking = 1keywords = neoplasm (Clic here for more details about this article) |
2/9. Low-grade pulmonary myxoid sarcoma of uncertain histogenesis.The present report describes an unusual case of low-grade pulmonary sarcoma with extensive myxoid change in a 60-year-old man. During 30 months, the tumor enlarged gradually and thereafter rapidly to 9 cm. Preoperative biopsy and cytology gave negative results. The resected mass, located at the periphery of the right upper lobe, was well circumscribed and showed gelatinous without necrosis or hemorrhage. Histologically, the tumor was composed of slightly atypical, spindle-shaped or stellate cells, which were loosely distributed within a prominent myxoid stroma. Epithelial differentiation was not seen. Lacunar structures were occasionally evident, but no cartilaginous matrix was seen. Mitotic figures were infrequent. immunohistochemistry failed to clarify the nature of the neoplastic cells except vimentin positivity. Histochemically, the myxoid ground substance was composed of hyaluronic acid and acid mucopolysaccharide. Electron microscopy revealed no specific differentiation other than aggregates of filaments, which were seen in a number of neoplastic cells. Flow cytometric analysis of the neoplastic cells revealed a diploid pattern. These findings indicated that the neoplasm was a low-grade myxoid sarcoma; however, a definite diagnosis could not be made. The tumor might have been a variant of extraskeletal myxoid chondrosarcoma, especially considering the histochemical results.- - - - - - - - - - ranking = 0.5keywords = neoplasm (Clic here for more details about this article) |
3/9. Myxoinflammatory fibroblastic sarcoma with complex supernumerary ring chromosomes composed of chromosome 3 segments.Myxoinflammatory fibroblastic sarcoma is a rare, recently described, and distinctive low-grade tumor of soft tissue. To our knowledge, there is only one previous report on the cytogenetics of this tumor. That case showed complex structural abnormalities, including a reciprocal translocation between chromosomes 1 and 10 [t(1;10)(p22;q24)] with loss of chromosomes 3 and 13. We describe here a second case showing supernumerary ring chromosomes, and a derivative chromosome 13, with additional material on the short arm. We conclude that the presence of chromosomal abnormalities supports the neoplastic nature of this tumor and aids in its diagnosis. Furthermore, we also postulate that the finding of ring chromosomes, which have been identified in other low-grade soft-tissue tumors, may have important prognostic implications regarding the aggressiveness of this neoplasm.- - - - - - - - - - ranking = 0.5keywords = neoplasm (Clic here for more details about this article) |
4/9. Myxoid monophasic synovial sarcoma: case report of an unusual histological variant.We report the case of a 61-year-old woman who presented a recurrent myxoid synovial sarcoma involving the right ankle and foot. This tumor, defined as a synovial sarcoma showing more than 50% myxoid change in the stroma, has only recently been described as a rare histological variant. The histological diagnosis is particularly difficult in such cases, as the one we are describing, where the tumor is entirely myxoid and monophasic raising the possibility of other myxoid soft tissue neoplasms, such as extraskeletal myxoid chondrosarcoma, malignant peripheral nerve sheath tumor or leiomyosarcoma. On the basis of morphological and immunophenotypical findings, the diagnosis of myxoid synovial sarcoma should be properly established, especially in view of its unusual clinical course and treatment. Furthermore, we will discuss the clinicopathological and immunohistochemical features observed in our case.- - - - - - - - - - ranking = 0.5keywords = neoplasm (Clic here for more details about this article) |
5/9. Myxoid tumor of soft tissue.Focal myxoid change is a well-recognised feature of synovial sarcoma, but the presence of a predominantly myxoid stroma is rare. We describe a new case of myxoid synovial sarcoma in which marked myxoid change initially obscured the diagnosis leading to confusion with malignant peripheral nerve sheath tumor. The patient was a 16 year old man who presented with a left dorsal foot tumor. The diagnosis of synovial sarcoma was suspected on histological and immunohistochemical studies and confirmed with cytogenetic analysis. Recognition of this rare histologic variant of synovial sarcoma is important because it can easily be mistaken for other myxoid spindle cell neoplasms, potentially resulting in suboptimal therapy.- - - - - - - - - - ranking = 0.5keywords = neoplasm (Clic here for more details about this article) |
6/9. Primary pulmonary neoplasms of childhood: a review.Primary tumors of the lung rarely occur in children. However, 230 well-documented cases, including the 2 presented in this review, have been identified in the English-language literature. One hundred fifty-one tumors in these reports were classified as malignant lesions and 79 as benign neoplasms. Bronchial "adenomas" constituted the largest group; most of these lesions were of the carcinoid variety, and 8% were definitely malignant. Forty-seven cases of bronchogenic carcinoma were reported in children under 16 years of age, although there were few squamous cell tumors (12%). Fifty-six percent of the benign tumors were classified as inflammatory pseudotumor. Most of the children in this collective series were seen with symptoms related to bronchial irritation or obstruction, such as cough, hemoptysis, atelectasis, or pneumonitis. Respiratory distress was an unusual symptom that was often associated with large tumors seen in the neonatal period. Approximately 20% of the children were totally symptomatic. The limited survival data that are available indicate that leiomyosarcoma and mucoepidermoid carcinoma have a more favorable prognosis in children than in adults. survival with bronchial carcinoid tumors (90%), bronchogenic carcinoma (30%), and pulmonary blastoma (45%) appears to parallel that for adults. The experience with pulmonary rhabdomyosarcoma, as described in this review, emphasizes the importance of early diagnosis and the use of combined modes of therapy in the approach to these malignancies. Despite the rarity of primary pulmonary neoplasms in children, this diagnosis should be considered in young patients with solitary pulmonary masses or persistent, atypical pulmonary symptoms. It is hoped that early diagnosis will result in an improved prognosis and prevent life-threatening complications.- - - - - - - - - - ranking = 3keywords = neoplasm (Clic here for more details about this article) |
7/9. Primary left atrial myxosarcoma.A patient in whom rapidly progressive congestive heart failure developed due to the presence of a left atrial myxosarcoma is described. The histologic picture is consistent with the hypothesis that cardiac myxoid tumors are neoplasms derived from undifferentiated cells found in the endocardium. Unfortunately, most cardiac malignancies are detected too late for curative resection, and palliation remains the mainstay of therapy.- - - - - - - - - - ranking = 0.5keywords = neoplasm (Clic here for more details about this article) |
8/9. Primary cardiac malignancy presenting as left atrial myxoma. Clinical and surgical considerations.Primary heart neoplasms occur in 0.002-0.3% of autopsies: 30% are myxomas and 20-30% are malignancies, almost always sarcomas. Cardiac metastases are 10 to 40 times more frequent than primary heart cancer. We describe a case of a left atrial sarcoma erroneously diagnosed as myxomas preoperatively. Standard surgical indication for resection of cardiac myxomas is based on echocardiography. Because of the severity of cardiac malignant lesions than can mimic atrial myxomas at echocardiography, through preoperative investigation should be accomplished, best by magnetic resonance imaging. In case of suspected malignancy, total body computed tomography should be performed to avoid unnecessary cardiac operations in case of disseminated cancer. To date the only good medium and long-term results in the therapeutic management of heart sarcomas have been achieved by transplantation: the probable explanation is that criteria of surgical radicality should be those followed for soft tissue tumors located elsewhere in the organism.- - - - - - - - - - ranking = 0.5keywords = neoplasm (Clic here for more details about this article) |
9/9. Low-grade myxofibrosarcoma: progression in recurrence.A case of low-grade myxofibrosarcoma with histologic progression in recurrence occurring in a 51-year-old male is described. The patient had a well-circumscribed encapsulated myxoid mass, which measured 2.5 cm at its greatest diameter, in the subcutis of the left forearm. Microscopically, the tumor was characterized by a proliferation of sparsely distributed spindle or stellate cells, curvilinear small vessels and myxoid stroma. It demonstrated mild pleomorphism without mitotic figures. The patient had the first recurrence at 3 years, which was histologically identical to the primary tumor. The patient had a second recurrence at 11 years, which was predominantly composed of sheets of anaplastic, rounded or oval cells with focal osteoid formation. Immunohistochemically, the tumor cells in the primary and the first recurrent lesions were focally positive for vimentin. In the second recurrence, the tumor cells contained vimentin, alpha-smooth muscle actin and muscle specific actin (HHF35). The primary lesion had a diploid dna content with low S-phase fractions. The second recurrence showed a polyploidy. The patient was well with no evidence of disease 18 months after the second recurrence. These findings suggest that this neoplasm showed histological progression with an increasing risk of metastasis. Low-grade myxofibrosarcoma, which commonly is misinterpreted as benign, has a tendency for histological and biological progression in local recurrences, underlining the importance of accurate diagnosis and wide surgical excision of the primary lesion.- - - - - - - - - - ranking = 0.5keywords = neoplasm (Clic here for more details about this article) |