Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0027627 (metastases)
 103,950 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A series of 30 myxofibrosarcomas is described. These malignant soft tissue tumours are characterized by a mucoid and nodular appearance, a coarse plexiform capillary pattern, and they are mostly seen subcutaneously (26 out of 30) in the extremities (24 out of 30) and trunk (4 out of 30) elderly people. Histochemical studies, comprising staining with Alcian blue and toluidine blue at different pH's with and without preceding digestion with testicular hyaluronidase and with the Scott technique, indicated the presence of hyaluronic acid but not sulphated glycosaminoglycans as chondroitinsulphates. Myxofibrosarcoma is believed to belong to the general category of fibroblastic and histiocytic malignant soft tissue tumours. The median diameter of the tumours was 7 cm. They were divided into 4 grades according to cellularity, cell atypia and mitotic activity. The grade III and IV tumours showed pronounced atypia, often with the bi- and multinucleated giant tumour cells and occasionally with giant cells of Touton's type, suggesting a relationship to malignant fibroxanthoma. All of the patients were treated surgically and one received also pre- and post-operative irradiation. None of the 2 grade I myxofibrosarcomas recurred, while 2 out of 7 grade II tumours, 6 out of 10 grade III tumours, and 7 out of 11 grade IV tumours recurred once and up to 9 times. Metastasis appeared in 7 out of 30 patients; grade I tumours were not seen in any of these cases. By the time of follow-up after intervals ranging from 1 month up to 27 years, 14 patients had died; 6 of these had died post-operatively or of intercurrent disease. The differential diagnosis between myxofibrosarcoma and other myxoid soft tissue tumours is discussed.
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PMID:Myxofibrosarcoma. A study of 30 cases. 1 96

Myxoid malignant fibrous histiocytoma (MFH) is an intermediate grade tumor with a definite metastatic potential but a relatively indolent natural history compared to the pleiomorphic variant of MFH. Little is known about its sensitivity to chemotherapy. We reviewed our experience with chemotherapy in myxoid MFH between 1986 and 1992. The patient population was identified through a search of the database maintained by the Departments of Melanoma-Sarcoma Medical Oncology and Pathology: 55 patients with histologically confirmed diagnosis of myxoid MFH were identified. Chemotherapy was administered to 18 of these patients (10 females, 8 males). The median age was 65 (range: 30-76). Ten patients had an extremity primary, seven had a trunk or retroperitoneal primary, and one patient had head and neck as the site of primary tumor. The median size of the primary tumor was 11 cm (range: 5-23 cm) in maximum dimension. Seven patients received chemotherapy in the neoadjuvant setting, eight received it for recurrent or metastatic disease, and three received it postoperatively after complete resection of the tumor. All patients received doxorubicin and dacarbazine with or without cyclophosphamide. Of the 15 patients evaluable for response, 4 achieved an objective response (one CR, 3 PRs, RR = 27%) to a median of 3 cycles (range: 1-7 cycles). At the time of last follow-up, eight patients are alive with no evidence of disease, two patients are alive with disease, and eight patients have expired. The median follow-up is 51 months (range: 26-216 months) from diagnosis. The relatively small sample precludes any definitive conclusions; however, it seems that doxorubicin- and dacarbazine-based chemotherapy has modest activity in myxoid MFH.
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PMID:Myxoid malignant fibrous histiocytoma: experience with chemotherapy. 852 98

Myxofibrosarcoma is one of the most common sarcomas in the extremities of elderly patients. We analysed the clinicopathologic features in a series of 75 patients. All patients were adults (range, 22-91 years; median, 66 years) with an approximately equal incidence in men and women. Thirty-five tumors arose in the lower and 25 in the upper extremities, nine on the trunk, two each in the retroperitoneum and the head and neck region, and one each in the pelvis and penis. Forty-eight cases (69.5%) were located in dermal or subcutaneous tissues. Distinctive histologic features included the following: a commonly nodular growth pattern; a myxoid matrix containing elongated, curvilinear capillaries; and fusiform, round or stellate tumor cells with indistinct cell margins, slightly eosinophilic cytoplasm, and hyperchromatic atypical nuclei. These lesions varied from a hypocellular, mainly myxoid, and purely spindle-cell appearance (low-grade neoplasms) to high-grade, pleomorphic (malignant fibrous histiocytoma-like) lesions with multinucleated giant cells, high mitotic activity, and areas of necrosis. Immunohistochemistry in 44 cases revealed only vimentin and occasional actin positivity. Ultrastructurally, tumor cells had a fibroblastic phenotype. DNA flow cytometry and proliferation analysis showed an association between aneuploidy and histologic grade. An average follow-up of 45 months (range, 5-300 months) in 60 cases has revealed local recurrence in 33 cases (54%). Thirteen patients developed metastases, and 13 tumor-related deaths occurred. A short interval to first local recurrence was associated with poor clinical outcome. The rate of local recurrence was independent of histologic grade, but only intermediate and high-grade neoplasms metastasized. The depth of the primary lesion did not influence the incidence of local recurrence. However, in deep-seated neoplasms, the incidence of metastases was higher and the percentage of tumor-related deaths was twice as high as in superficially located lesions, reflecting the fact that deep-seated lesions tended to be higher-grade, larger tumors. Myxofibrosarcoma tends to become progressively higher grade in recurrences, as demonstrated in five cases in our series. The poorly recognized low-grade myxofibrosarcoma is emphasized, as proper diagnosis and treatment and scrupulous follow-up are mandatory to avoid local recurrence and gradual tumor progression to a higher-grade neoplasm that may then metastasize.
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PMID:Myxofibrosarcoma. Clinicopathologic analysis of 75 cases with emphasis on the low-grade variant. 860 5

Myxofibrosarcoma (MFS) is one of the most common soft tissue sarcomas of elderly patients and has a predilection for the limbs. Herein, we report a previously undescribed variant of MFS showing epithelioid morphology. Seventeen cases diagnosed as epithelioid MFS were retrieved from the authors' files from among 570 cases of MFS. Hematoxylin and eosin-stained sections were reexamined and immunostains for pan-keratin (15 cases), S-100 protein (15), desmin (15), and alpha-smooth muscle actin (13) were performed. Nine patients were men and 8 were women (age range 43 to 89 y; median 63.5). Fifteen patients presented with a mass, and in 2 of these there was also pain. Duration of symptoms varied from 1 to 24 months (median 3). Tumor size ranged from 2 to 15 cm (median 6.75). In 10 cases, the tumor was located in subcutaneous tissue and in 6 cases it was subfascial. The majority of the tumors were located on the limbs (8 lower extremities and 6 upper extremities) followed by neck (1), scalp (1), and trunk (1). Follow-up was available for 14 patients (range 2 to 240 mo; median 16). Twelve patients were treated by surgery followed by chemotherapy and/or radiation (8 cases). One patient received chemotherapy after an incisional biopsy and 1 patient was treated by surgery alone. Ten patients (71.4%) developed local recurrences. Seven patients (50%) developed metastases to lungs or retroperitoneum. Five patients (35.7%) have died of disease so far. Two patients were lost to follow-up. Morphologically, 14 cases were high grade, 2 were intermediate, and 1 was low grade. Tumors were characterized by a multinodular, infiltrating growth pattern with alternation of hypercellular and hypocellular myxoid areas; the latter showed prominent curvilinear vessels. Neoplastic cells were arranged singly and in small clusters in the myxoid areas or formed sheets in the hypercellular areas, where they showed epithelioid morphology with round nuclei, vesicular chromatin, prominent nucleoli, and moderate amounts of eosinophilic cytoplasm. The epithelioid areas were generally multifocal with admixed areas of conventional MFS. Immunostains were negative for all markers studied. Differential diagnosis included carcinoma, melanoma, myoepithelial carcinoma, pleomorphic liposarcoma, and pleomorphic rhabdomyosarcoma. In conclusion, epithelioid MFS is a rare variant of MFS, accounting for <3% of MFS in consultation material. Its natural history seems more aggressive than usual high-grade MFS, with approximately 70% local recurrence and 50% metastases, even within a relatively short follow-up period.
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PMID:Epithelioid variant of myxofibrosarcoma: expanding the clinicomorphologic spectrum of myxofibrosarcoma in a series of 17 cases. 1719 25

A 77-year-old man visited our department with a painless mass in the left scrotum. He underwent left high orchiectomy with clinical diagnosis of testicular tumor. The tumor penetrated deeply into the internal inguinal ring. The tumor was resected as proximally as possible with positive surgical margin at the internal ring. Macroscopically, the left testis was intact in the resected specimen. Microscopic examination revealed that the tumor was consisted of myxoid stroma with spindle cells, and there was a large variation in histological findings. Immunohistchemical staining was positive with vimentin, and negative with alphaSMA, S-100, Myo D1, EMA, keratin, HMB45, and LCA. Accordingly, the tumor was diagnosed as myxofibrosarcoma, high-grade, of the spermatic cord. Postoperative CT scanning revealed multiple retroperitoneal lymph node metastases. He underwent radiation therapy, but soon developed lung metastases and pleural dissemination, and died 2 months after the operation. Myxofibrosarcoma, which had been called myxoid MFH is one of the most common soft tissue sarcomas. Myxofibrosarcoma or myxoid MFH of the spermatic cord is a rare disease. To our knowledge, only 7 cases have been reported in Japan to date.
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PMID:[Myxofibrosarcoma of the spermatic cord: a case report]. 1840 85

Myxofibrosarcoma is a common connective tissue neoplasm of malignant fibrocytes in a myxoid matrix favoring the extremities. Like many other tumors of connective tissue, soft tissue sarcoma exhibits high recurrence rates but is rarely known to metastasize. We present a patient with myxofibrosarcoma of the hand with metastases to the lungs, pleura, and mediastinum. The mediastinal metastasis presented clinically with gastrointestinal symptoms due to compression of the gastroesophageal junction. To our knowledge, this is the first report of metastatic myxofibrosarcoma of the hand and also the first report of metastatic myxofibrosarcoma to mediastinal lymph nodes at the level of the gastroesophageal junction. We also performed a comprehensive literature review of metastatic myxofibrosarcoma.
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PMID:Myxofibrosarcoma with metastasis to the lungs, pleura, and mediastinum: a case report and review of literature. 2232 54

Myxofibrosarcoma is one of the most common sarcomas in the extremities of elderly people. It is characterized by a high frequency of local recurrence due to an infiltrative growth property. In contrast, the overall risk of distant metastases is generally low. This makes the prognosis for the patients with myxofibrosarcoma definitely good. In this paper, we will report the case of a 79-year-old female with very aggressive metastatic low-grade myxofibrosarcoma. The disease progression was really unexpected and misled every possible medical interpretation, leading to rapid worsening of the patient's clinical conditions and no chance for therapy. The tumor developed diffuse infiltration in lung, spine, skeletal bone, abdomen, paravertebral muscles, and liver. The patient died 8 months after the diagnosis of remote metastases due to rapid tumor progression.
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PMID:Rapid multiorgan dissemination of low-grade myxofibrosarcoma: a case report. 2297 15

Myxofibrosarcoma is a unique subtype of soft tissue sarcoma with a locally infiltrative behavior. High-quality MRI imaging is critical for preoperative planning. Wide surgical resection with a 2 cm soft tissue margin is the mainstay of treatment and can require complex vascular and plastic surgery reconstruction. Local recurrence is common, and a subset of patients with higher-grade lesions will develop distant metastases. Radiation may be beneficial in reducing local recurrence.
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PMID:Myxofibrosarcoma. 2759 98

Myxofibrosarcoma (MFS) is a malignant fibroblastic tumor that primarily affects the lower and upper extremities. It is usually described as a slow-growing tumor with high recurrence rates but low metastatic potential. The reported incidence of head and neck MFS is 2 to 4% and rarely presents with distant metastases. This report describes a case of maxillary MFS in a 72-year-old woman whose disease progression followed an atypical course with an extremely rapid rate of growth and early pulmonary and central nervous system lesions. The pulmonary symptoms at initial presentation made a final diagnosis a challenge. Various diagnostic modalities and multidisciplinary collaboration were required. The disease course and management are outlined. To the authors' knowledge, this is the first case of MFS originating in the maxillary alveolus with multiple metastases, including the brain and lungs, in the early course of the disease.
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PMID:Myxofibrosarcoma in Head and Neck: Case Report of Unusually Aggressive Presentation. 2889 44

Herein we describe the case of a young patient who presented with a recent history of epilepsy due to multiple brain lesions; he did not complain about any cardiopulmonary impairments. The patient died as a consequence of hemorrhagic progression of brain metastatic disease. Regardless of a thorough investigation, the heart tumor remained occult. Primary cardiac tumors are very rare entities. Most of these are benign, but approximately 25% are malignant, and the majority of these are sarcomas. Myxofibrosarcoma and osteosarcoma are exceptionally rare. To date, we find only small series of cardiac myxofibrosarcoma, and to our knowledge, this one exceptionally presented with multiple brain metastatic lesions without cardiopulmonary symptoms.
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PMID:Primary Cardiac High-grade Myxofibrosarcoma Presenting with Multiple Brain Metastases: A Case Report. 2937 52


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