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

We describe a new case of heart osteosarcoma manifesting as a large polypoid mass of the left atrium. Clinically, the lesion presented with dyspnea, syncopal attacks, and fever. Ultrasound scans of the heart were suggestive of a cardiac myxoma, although some imaging features could retrospectively indicate a malignant neoplasm. In particular, the lesion was relatively immobile, did not show the characteristic myxoma motion during the heart cycle, and extended into the left pulmonary veins. Exploratory thoracotomy showed a nonresectable polypoid mass of the left atrium widely infiltrating the myocardium and the epicardium, histological features of a polymorphic sarcoma. The patient died 5 months after presentation with diffuse metastases. At autopsy, a 5-cm polypoid tumor was seen protruding into the left atrium. Neoplastic infiltration of the atrial myocardium, pericardium, and pulmonary veins was evident. Extensive metastatic deposits were observed in numerous sites, including the skin, lung, liver, and brain. Microscopically, a spindle cell sarcoma forming malignant osteoid was seen, admixed with areas indistinguishable from myxoid and pleomorphic malignant fibrous histiocytoma. This case provides further evidence that although rare, osteosarcoma of the heart usually presents as a polypoid mass of the left atrium and is histologically characterized by conspicuous polymorphism. It is associated with a poor prognosis and rapid appearance of distant metastases. Although at echocardiography it may mimic a myxoma, subtle features such as tumor extension into pulmonary veins and main origin from nonseptal atrial walls suggest the presence of a nonmyxomatous tumor and a preoperatory diagnosis of aggressive malignant disease.
 ...
PMID:Polypoid osteosarcoma of the left atrium: report of a new case with autopsy confirmation and review of the literature. 984 36

A 29 year old white man presented to the emergency room with new onset pleuritic chest pain and shortness of breath. He was initially diagnosed as having viral pericarditis and was treated with non-steroidal anti-inflammatory drugs. A few weeks later he developed recurrent chest pain with cough and haemoptysis. Chest radiography, cardiac examination, transthoracic and transoesophageal echocardiography pointed to a mass that arose from the posterior wall of the right atrium, not attached to the interatrial septum, which protruded into the lumen of the right atrium causing intermittent obstruction of inflow across the tricuspid valve. Contrast computed tomography of the chest showed a right atrial mass extending to the anterior chest wall. The lung fields were studded with numerous pulmonary nodules suggestive of metastases. A fine needle aspiration of the pulmonary nodule revealed histopathology consistent with spindle cell sarcoma thought to originate in the right atrium. Immunohistochemical stains confirmed that this was an angiosarcoma. There was no evidence of extracardiac origin of the tumour. The patient was treated with chemotherapy and radiation. This case highlights the clinical presentation, rapid and aggressive course of cardiac angiosarcomas, and the diagnostic modalities available for accurate diagnosis.
 ...
PMID:Primary right atrial angiosarcoma mimicking acute pericarditis, pulmonary embolism, and tricuspid stenosis. 1021 78

We report a case of carcinosarcoma of the urinary bladder. A 68-year-old man visited our hospital with complaints of asymptomatic macroscopic hematuria, cold sweat and general malaise. Excretory urography revealed a filling defect in the left wall of the bladder, and subsequent cystoscopy revealed a non-papillary sessile tumor. The tumor was transurethrally resected and its histology showed carcinosarcoma which was characteristics of a mixture of transitional cell carcinoma with spindle cell sarcoma. The patient underwent total cystectomy and his bladder was reconstructed with the ileum. Nevertheless, he died of multiple organ metastases 3 months after the surgery.
 ...
PMID:[A case of carcinosarcoma of the urinary bladder]. 1240 82

Malignant tumors composed of myofibroblasts are increasingly being recognized, but their existence remains controversial. Currently accepted examples within this category represent spindle cell or pleomorphic neoplasms of the soft tissues with a spectrum of histological grades. Low- and intermediate-grade myofibrosarcomas are fascicular spindle cell neoplasms resembling fibrosarcoma or leiomyosarcoma. They infiltrate deep soft tissue with disproportionate involvement of head and neck sites and can recur locally but infrequently metastasize. They variably express myoid immunohistochemical markers, and their differential diagnosis includes benign myofibroblastic proliferations such as fasciitis and fibromatosis as well as other types of spindle cell sarcoma. High-grade (pleomorphic) myofibrosarcomas are an ultrastructurally defined subset of malignant fibrous histiocytoma, which they resemble in morphology and behavior. Inflammatory myofibroblastic tumor and infantile fibrosarcoma are neoplasms that have myofibroblastic features and have been included in this category, but they have distinctive genetic findings. This article reviews the concept of myofibrosarcoma and describes its variants.
 ...
PMID:Myofibroblastic malignancies. 1522 Aug 22

Pleomorphic liposarcoma is an uncommon form of liposarcoma that only recently has been properly characterized. A series of 57 cases is presented. Patient age at presentation ranged from 27 to 95 years (median, 54 years), and there was a slight male predilection (male/female ratio = 1.2:1). Tumors most frequently involved the lower limb (47% of cases) or upper limb (18%). Other anatomic sites, including trunk (14%), retroperitoneum (7%), head and neck (5%), abdomen/pelvis (5%), and spermatic cord (4%), were less frequently involved. Tumor size ranged from 1.5 to 21 cm (median, 8 cm), with deep (subfascial) locations (39 cases) being more frequent than subcutaneous (11 cases) or dermal sites (5 cases). All lesions showed features of pleomorphic sarcoma and at least focally contained typical multivacuolated lipoblasts. Although there was considerable overlap, tumors fell into three broad categories: high-grade pleomorphic/spindle cell sarcoma with scattered lipoblasts or sheets of lipoblasts (60%), high-grade pleomorphic sarcoma with epithelioid areas and scattered lipoblasts (28%), and intermediate- to high-grade sarcoma predominantly resembling myxofibrosarcoma except for the presence of lipoblasts (12%). Immunohistochemistry revealed focal staining for smooth muscle actin in 13 of 29 cases (45%), S-100 protein positivity in lipoblasts in 15 of 45 cases (33%), focal staining for keratin in 6 of 28 cases (21%), including 5 of 13 (38%) with epithelioid morphology, and focal staining for desmin in 4 of 30 cases (13%). Follow-up data, available in 50 patients (88%) (median, 33 months), showed local recurrence in 34% of patients, systemic metastases in 32%, and tumor-related death in 32%. Only 2 of the 16 superficial (dermal or subcutaneous) lesions metastasized. Five-year overall, local recurrence-free, metastasis-free, and disease-free survivals were 63%, 58%, 58%, and 39%, respectively. By univariate analysis, central (nonextremity) location, deep situation, tumor size > or =10 cm, mitotic rate > or =10 per 10 HPF, necrosis, and epithelioid morphology were associated with a worse prognosis. However, by multivariate analysis, only age > or =60 years, central location, tumor size, and mitotic rate remained independent predictors for an adverse outcome. By multivariate analysis, wide local excision or amputation and postoperative radiotherapy protected against local recurrence.
 ...
PMID:Pleomorphic liposarcoma: clinicopathologic analysis of 57 cases. 1537 41

There are limited data that define the role of chemotherapy in the treatment of high-grade spindle cell sarcomas of bone, other than osteosarcoma or malignant fibrous histiocytoma (MFH-B). This prospective study evaluates the effect of doxorubicin and cisplatin on these tumours. Thirty-seven patients, age 65 years, with spindle cell sarcoma of bone, except osteosarcoma or MFH-B, were included. Chemotherapy consisted of doxorubicin and cisplatin every 3 weeks for six cycles. Resection was performed after three cycles. In 15 patients with metastases, response assessment showed three complete responses (CR), four stable disease (SD), five progression; three were not evaluable. Median time to progression was 30 months (95% Confidence Interval (CI), 8-51 months) for the operable non-metastatic patients; median survival 41 months (95% CI, 16-82 months). Median time to progression in the metastatic group was 10 months (95% CI, 0-18 months) and median survival was 14 months (95% CI, 4-45 months). This study suggests a limited role for doxorubicin and cisplatin in metastatic high-grade spindle cell sarcoma of bone, other than osteosarcoma or MFH-B cases.
 ...
PMID:Doxorubicin and cisplatin chemotherapy in high-grade spindle cell sarcomas of the bone, other than osteosarcoma or malignant fibrous histiocytoma: a European Osteosarcoma Intergroup Study. 1566 41

A 54-year-old man presented with a recurrent swelling on the right occipital region of the scalp. Two months earlier, the patient had undergone an initial local excision of the lesion which had enlarged progressively over the previous 2 years on a hairless patch which had been present since birth. On examination, a 5 x 4-cm, pinkish, firm, ulcerated swelling was seen on the right occipital region with a scar running over it. The lesion was not fixed to the underlying bone and there was no regional lymphadenopathy. X-Ray of the skull was normal and no evidence of metastatic disease was identified. Wide local excision of the tumor was performed and it was sent for histopathologic examination. Specimens and slides of the earlier surgery performed elsewhere were also studied. The specimen of the initial surgery consisted of skin-covered tissue with an exophytic firm growth measuring 6 x 5 x 4 cm. The skin surface was rough with areas of ulceration. No necrosis was noted grossly. Microscopically, three distinct lesions were seen. One was a well-circumscribed tumor located in the superficial dermis with lobules of basaloid cell aggregates with peripheral palisading and with no epidermal connection. The lobules were surrounded by cellular fibrous tissue (Fig. 1). Unlike basal cell carcinoma, however, no cleft between the cellular aggregates and stroma was noted. Foci of pigmentation were seen within cellular lobules and these features were consistent with a diagnosis of tricho-blastoma. The second tumor was seen adjacent to the first, and consisted of duct-like structures and cystic spaces with papillary projections. These were lined by double-layered epithelium with stromal infiltration by plasma cells, which are features of syringocystadenoma papilliferum (Fig. 2). The third lesion was a spindle cell sarcoma which formed the major part of the lesion, diffusely infiltrating the dermis and subcutaneous tissue, elevating and ulcerating the overlying skin. The tumor consisted of interlacing fascicles of spindle cells with oval to elongated nuclei having finely dispersed chromatin and inconspicuous nucleoli. The tumor cells were seen encircling the sweat glands, without destroying them (Fig. 3). Nuclear pleomorphism was minimal, with a mitotic rate of 9-10 per high-power field. A small area of epidermal hyperplasia with acanthosis and papillomatosis overlying malformed highly placed sebaceous glands was the only evidence of a pre-existing nevus sebaceus. The status of the surgical margins was not clearly discernible. The wide excision specimen of the recurrent swelling consisted of a skin-covered nodule with ulceration, measuring 3 x 4 x 3 cm, with a gray-white whorled cut surface. No necrosis was noted grossly. Multiple sections showed only spindle cell sarcoma infiltrating the skin and subcutaneous tissue, morphologically similar to the earlier tumor, with ulceration of the overlying skin. The surgical margins were free from tumor. Immunohistochemical stains on the spindle cell sarcoma showed positive staining for smooth muscle actin (SMA) (Fig. 4), vimentin, epithelial membrane antigen (EMA), and S100. The tumor cells were negative for cytokeratin (CK), HMB45, desmin, glial fibrillary acidic protein (GFAP), CD34, and CD68. Correlating the histomorphology and immunohistochemical findings, a diagnosis of cutaneous leiomyosarcoma with tricho-blastoma and syringocystadenoma papilliferum arising on nevus sebaceus was made. The patient received postoperative radiotherapy and is disease free 8 months after diagnosis.
 ...
PMID:Cutaneous leiomyosarcoma, trichoblastoma, and syringocystadenoma papilliferum arising from nevus sebaceus. 1734 92

Primary spindle cell sarcoma of the left atrium is a rare tumour. Optimal treatment is to obtain complete surgical clearance of the tumour. The anatomic location of the tumour, infiltration into vital structures and difficult access provides a surgical challenge for resection of the lesion and reconstruction of the defect. The prognosis of patients with a primary cardiac sarcoma is very poor because of their resistance to treatment with chemotherapy and radiotherapy. Metastases and local recurrences are common despite optimal multimodality treatment. This report describes a 48-year-old gentleman who underwent multiple surgeries to achieve an 11-year survival since the diagnosis. The operative techniques have been described.
 ...
PMID:Prolonged survival with left atrial spindle cell sarcoma. 1930 72

A case of hypertrophic osteopathy secondary to a pulmonary spindle cell sarcoma is described. The 9-year-old male cat presented with a 1-month history of decreased appetite, decreased activity and progressive lameness with swelling and pain of all four limbs. Thoracic radiographs showed a soft tissue opaque mass in the left caudal lung lobe. Radiographs of all limbs showed extensive periosteal new bone formation of uniform opacity demonstrating a 'palisading' pattern. The lung mass was removed at exploratory thoracotomy; histopathological examination diagnosed a low-grade spindle cell sarcoma. Prior to surgery, the cat had a non-specific conjunctivitis that resolved spontaneously following lobectomy raising the possibility of a paraneoplastic association. The lameness also resolved; six months after surgery, the periosteal palisading of new bone on the long bones had remodelled, and there was no evidence of pulmonary metastases.
 ...
PMID:Hypertrophic osteopathy secondary to pulmonary sarcoma in a cat. 1937 84

Primary cardiac sarcomas are exceptionally rare tumours. A brain metastasis of a primary cardiac sarcoma has never been reported before. Although we know lots of primary malignomas spreading to the brain, we never observed cerebral metastases of an atrial spindle cell sarcoma. Cardiac sarcomas are more likely to haematogenously metastasize to the lung or the liver. Here, we describe the case of a young man, who suffered from a cerebral metastasis of a spindle cell sarcoma in the left heart atrium nine years ago. Postoperative whole brain irradiation with 30 Gy was performed. Later on, the patient experienced a local recurrence within the left atrium accompanied by cardiac arrhythmia and mitral valve insufficiency. This case is the very first description of a true cerebral metastasis from a primary heart sarcoma. Therefore, clear treatment paradigms are not established. Further case illustrations and the publication of larger patient series are mandatory, whenever possible.
 ...
PMID:Cerebral metastasis of a primary heart sarcoma. 2049 89


<< Previous 1 2 3 4 5 Next >>