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 51-year-old man was admitted because of complaints of cough and bloody sputa. A chest CT scan revealed a giant mass lesion in the right middle and lower lobes of the lung and mediastinal lymphadenopathy. Bronchoscopic findings showed a tumor which almost completely obstructed the intermediate bronchus. Histopathological examination of a biopsy specimen demonstrated malignant hemangiopericytoma. Two courses of chemotherapy that combined cisplatin, ifosfamide and gemcitabine were performed every 3 weeks. Both primary lesion and mediastinal lymph node metastases showed marked reduction and toxicity was manageable.
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PMID:[A case of primary malignant hemangiopericytoma of the lung with marked response to combination chemotherapy with cisplatin, ifosfamide and gemcitabine]. 1126 7

Most hemangiopericytomas (HPCs) are located in the musculoskeletal system and the skin, while the intracranial location is rare. They represent 2 to 4% in large series of meningeal tumours, thus accounting for less than 1% of all intracranial tumours. Many authors have argued about the true origin of this tumour. The current World Health Organization classification of Central Nervous System tumours distinguishes HPC as an entity of its own, and classified it into the group of "mesenchymal, non-meningothelial tumours". Radical surgery is the treatment of choice, but must be completed with postoperative radiotherapy, which has proved to be the therapy most strongly related to the final prognosis. HPCs have a relentless tendency for local recurrence and metastases outside the central nervous system which can appear even many years after diagnosis and adequate treatment of the primary tumour. Twelve patients with intracranial HPC were treated at our Unit between 1978 and 1999. There were 10 women and 2 men. Ten tumours were supratentorial and most located at frontoparietal parasagittal level. The most common manner of presentation was a focal motor deficit. All tumours were hyperdense in the basal Computed Tomography scans and most enhanced homogeneously following intravenous contrast injection. In 50% of cases, tumour margins were irregular or lobulated. Seven tumours were studied with Magnetic Resonance Imaging, being six of them iso-intense with the cortical gray matter on T1-weighted and T2-weighted images. Twenty operations were performed in the 12 patients. In 10 cases radical excision could be achieved with no operative mortality. Total recurrence rate was 33.3%. Eight patients were treated with external radiotherapy at some time through the course of their disease. Eight out of the 12 patients in this series are disease-free (Glasgow Outcome Scale categories 1 and 2) after a mean follow up of 52 months.
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PMID:Intracranial hemangiopericytoma: study of 12 cases. 1153 74

The authors report on a rare case of malignant hemangiopericytoma. A 56-year old female patient had developed a painless node on her left gluteal region 20 years ago. Years later, the resistance became tender and was subsequently surgically removed. On histology, the lesion was identified as malignant hemangiopericytoma. After a symptom-free period of 8 years distant pulmonary metastases arose, which were removed by surgery again. Shortly afterwards the patient manifested with further metastasis involving the lung again, as well as the abdomen, the skin and the breast, leading to the patient's death 2 years later. The authors review the literature, clinical course, diagnostic possibilities, prognosis and therapeutic options of malignant hemangiopericytomas. Special attention is drawn to the unexceptionally long clinical course at this particular malignancy, as well as to the late occurrence of metastases and their unusual breast localisation.
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PMID:[Malignant hemangiopericytoma with breast metastasis]. 1157 9

We studied 4 new cases of juxtaglomerular cell tumor and compared their morphologic and immunohistochemicalfeatures with 2 renal hemangiopericytomas and 5 cutaneous glomus tumors. The juxtaglomerular tumors were resectedfrom 2 males and 2 females (mean age at diagnosis, 23 years). Three patients manifested with severe hypertension. Tumors ranged from 2.2 to 8.0 cm and were well circumscribed. The tumors consisted of solid sheets and nodules of variably sized tumor cells with round, oval, and spindled nuclei alternating with edematous microcystic foci. Nuclear atypia, present in all tumors, was a prominent feature in 2. Mitotic activity was not identified. All cases showed hemorrhage, numerous mast cells, and thick-walled blood vessels. Unusual features included coagulative tumor necrosis, a hemangiopericytoma-like vascular pattern, and hyalinized stroma. All tumors were immunoreactive for CD34 and actin. Ultrastructural analysis revealed the presence of rhomboid-shaped renin protogranules. Patients were treated by partial or radical nephrectomy and followed up for 14 to 48 months. There were no recurrences or metastases. The characteristic clinical and morphologic features of juxtaglomerular cell tumor permit distinction from renal hemangiopericytoma and other renal tumors.
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PMID:Juxtaglomerular cell tumor: a clinicopathologic study of four cases and review of the literature. 1204 49

We report our experiences of four cases with meningeal hemangiopericytoma treated with surgery and postoperative radiotherapy and survey the literature to elucidate the efficacy of radiotherapy. Patients were treated with surgical resection and 46-52 Gy postoperative radiotherapy. Three patients had local control for 30, 54 and 138 months, respectively and one patient had local recurrence after 49 months. Distant metastases were observed in two patients; one had multiple bone, liver and lung metastases and the other multiple bone and brain metastases. For bone and brain metastases, better tumor control was obtained with palliative radiotherapy and stereotactic radiotherapy. Literature analyses demonstrated that surgery and postoperative radiotherapy of 50 Gy or more resulted in significantly better local control than surgery alone (p = 0.02). Stereotactic radiosurgery was effective for intracranial recurrence or metastasis, especially when the tumor volume was <8 cm(3) and >15 Gy at the 50% isodose line was used. Radiotherapy for bone metastases was also effective for palliation.
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PMID:Four cases of meningeal hemangiopericytoma treated with surgery and radiotherapy. 1177 63

Pulmonary metastases of endometrial stromal sarcoma (ESS) are uncommon and can pose diagnostic problems. We reviewed lung specimens from 16 patients with metastatic ESS. Patients were 31-77 years of age at the time of lung biopsy. Uterine ESSs were diagnosed an average of 9.8 years before lung biopsy in 11 patients. Uterine ESSs were originally called smooth muscle tumors in three additional patients. Thirteen patients were evaluated for new pulmonary nodules, seven of whom were asymptomatic. Nodules were multiple in 14 and solitary in four, ranging from 1.0 to 8.0 cm in greatest dimension. One patient died of metastatic disease; 14 were alive and seven of these were without disease (mean follow-up 4.1 years). Diagnostic considerations in 12 consultation cases included ESS, sclerosing hemangioma, carcinoid tumor, lymphangioleiomyomatosis, endometriosis, hemangiopericytoma, and lymphoma. Tumors were well circumscribed and usually solid, composed of plump spindle cells arranged in short fascicles. Two tumors were predominantly cystic. Sex cord-like stromal differentiation was identified in three. Neoplastic cells stained for vimentin (93%), estrogen and progesterone receptor (100%), smooth muscle actin (57%), desmin (50%), and keratin (46%). Metastatic ESS should be included in the differential diagnosis of nonepithelial neoplasms in women.
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PMID:Endometrial stromal sarcoma metastatic to the lung: a detailed analysis of 16 patients. 1191 21

Haemangiopericytoma (HPC) of the orbit is a rare tumour occurring in all age groups and presenting with slowly progressive proptosis, ocular motility impairment and visual loss. Although most are benign tumours that are resectable at diagnosis, one-third of these tumours demonstrate malignant features and recur locally or metastasize, usually with a fatal outcome. In 1995, a morphologically unique variant, termed lipomatous haemangiopericytoma (L-HPC), was described. Only one case has been reported previously arising in the orbit. In this paper, a case is reported of L-HPC occurring in the orbit and consider the prognostic implications of this HPC variant.
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PMID:Orbital lipomatous haemangiopericytoma: an unusual variant. 1212 69

The authors present the methods and results of treatment of 20 epiphyseal and low-metaphysis pathological knee fractures treated at the Department of Orthopedics of the Lublin University of Medical Sciences between 1962-1999. The average age was 39 years. Malignant tumours (2 lymphomas, 1 plasmocytoma, 1 hemangiopericitoma, 2 adenocarcinoma metastases) and benign lesions (10 giant cell tumours, 3 solitary bone cysts, 1 intraosseous extension of pigmented villonodular synovitis) causes 18 femur fractures and 2 tibia fractures. The fractures caused by lymphomas in 2 patients and plasmocytoma in 1 patients were treated with either a plaster cast or skeletal traction and healed within 16 weeks. Tumour resection and reconstruction with Rush rods, Kuntscher nail, plate and methylmetacrylate were performed in 2 cases. In 1 case with hemangiopericytoma hip exarticulation was performed. In 5 cases pain relief and weight bearing capacity of the limb was not achieved because of neoplasm recurrences; 5 deaths occurred within 6 months to 7 years after femur fracture because of pulmonary metastases. Benign bone lesions were treated by curettage of pathological tissues, burring bony walls of the lesions and filling the cavities with bone grafts or methylmetacrylate (1 case). Displaced fragments were reduced whenever possible. Limbs were immobilised in plaster casts. The follow-up period for patients with benign lesions was 8 years. All fractures healed. Arthritis with persistent pain and knee contracture was noted in 1 patient who underwent surgery 3 times for giant cell tumour recurrences, complicated by infection and varus deformity.
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PMID:[Pathological fractures of the knee]. 1214 88

A 65-year-old man developed pancreatic and pubic tumors 22 years after craniotomy for a left sphenoid ridge tumor. The histological finding of the primary sphenoid ridge tumor was meningotheliomatous meningioma. The histological appearance of the biopsy specimen of the pubic tumor was hemangiopericytoma. The recurrent intracranial tumor was treated by radiosurgery, and the metastatic tumors were treated by conventional irradiation. Intracranial tumors rarely metastasize outside the central nervous system, except for meningeal hemangiopericytomas. This case indicates that meningeal hemangiopericytoma may metastasize many years after the initial onset and requires long-term follow up.
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PMID:Intracranial hemangiopericytoma with extracranial metastasis occurring after 22 years. 1216 Mar 9

Painless unilateral proptosis is a frequent manifestation of numerous orbital neoplastic and non-neoplastic processes. Various mesenchymal tumors of both fibrohistiocytic and vascular origin are well-described causes. Solitary fibrous tumors (SFTs) are rare spindle-cell neoplasms usually found associated with serosal surfaces, especially the pleura, but they have recently been described in a number of extrapleural sites including the orbit. The authors describe the case of an 18-year-old man who presented with a 6-month history of painless proptosis in the right eye. A visible nontender mass in the right supermedial orbit producing ptosis of the upper lid was present. Magnetic resonance imaging (MRI) showed a well-circumscribed soft tissue mass located above the right globe with no obvious invasion of adjacent orbital structures. Uneventful surgical excision through a right frontal-orbitotomy approach was performed. Histological evaluation showed a solid, highly vascular tumor mass composed of spindle cells arranged in short ill-defined fascicles. Intense immunohistochemistry staining for CD34 and B-cell lymphoma 2 (BCL-2) differentiated the lesion from the more common hemangiopericytoma. Though considered benign, local recurrence and extraorbital extension of orbital SFTs have been described. Malignant behavior, including distant metastases, has been documented in as many as 20% of pleural cases with mortality rates as high as 50%. The natural history of this tumor in the orbit is unclear. The authors report the 35th case of orbital solitary fibrous tumor and discuss the differential diagnosis, histopathology, radiological features, and clinical course.
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PMID:Solitary fibrous tumor of the orbit. 1221 91


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