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)

We present 22 (0.9%) cases of rhabdoid tumour of the kidney diagnosed amongst 2392 renal tumours in children. The patients ages ranged from 3 weeks to 94 months (median 7 months) and the female:male ration was 1.2:1. Clinically, they presented with an abdominal mass but four (18%) children also had hypercalcaemia and one (4.5%) developed a brain tumour (primitive neuroectodermal tumour). None of the children presented with stage I disease, five (23%) had stage II, ten (46%) stage III, and five (23%) stage IV disease. Two (9%) patients had bilateral tumours. Histologically, the vast majority (20/22) of the tumours exhibited a classical pattern but other histological patterns were also noted. Immunohistochemical studies performed in 12 cases showed vimentin positivity in all cases, CAM 5.2 in eight, epithelial membrane antigen in six, neuron specific enolase in four, S-100 protein in eight, and desmin in one case. In only 12 of the 22 tumours was there agreement between the reporting pathologist and the panel on a diagnosis of rhabdoid tumour of the kidney. Eight tumours originally diagnosed as rhabdoid tumour of the kidney were found to be other renal tumours and in another ten cases the initial diagnosis was changed by the panel to rhabdoid tumour. Metastases developed in 18 (82%) of the children--in eight they were present at the time of diagnosis and in 10 they developed from 2 weeks to 9 months after initial diagnosis. Metastases were found in the lung, abdomen, lymph nodes, liver, bone and brain. Of the 19 children with adequate follow-up, only two (10.5%) with stage II disease are alive, while 17 (89.5%) died 2 weeks to 20 months after the diagnosis.
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PMID:Rhabdoid tumour of the kidney: a clinicopathological study of 22 patients from the International Society of Paediatric Oncology (SIOP) nephroblastoma file. 873 42

Rhabdomyosarcoma (RMS), a high-grade, malignant, skeletal muscle tumor, represents approximately 5% of neoplasms in children. The poorly differentiated forms of RMS are often not easily diagnosed and classified. Among the four histologic variants, alveolar RMS is the least frequently reported subtype. A poorly differentiated solid variant of alveolar RMS occurred on the right hand of a 16-year-old girl. Because of the tumor size, local invasiveness, and occurrence of cutaneous and breast metastases at presentation, the clinical staging was group IV (T2/NO/M1). Surgical excisions of the primary and metastatic locations were performed and chemotherapy with vincristine, dactinomycin, cyclophosphamide, and doxorubicin was administered. Light and electron microscopy studies revealed a solid proliferation with a focal alveolar pattern of monomorphous, small, round neoplastic cells without easily detectable muscular morphologic features. The skeletal muscle origin was revealed by the positive immunostaining for desmin, alpha-sarcomeric actin, muscle-specific actins, and enolase, and confirmed by immunoblotting for desmin. Despite the age of our patient, which is considered by some authors an independent predictor of outcome, all prognostic variables were unfavorable. However, a disease-free interval during three years of follow-up underlines the importance of multidisciplinary regimens for the treatment of this rare solid tumor of childhood and adolescence.
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PMID:Solid alveolar rhabdomyosarcoma of the hand in adolescence: a clinical, histologic, immunologic, and ultrastructural study. 874 83

Primary hepatic carcinoid tumors are extremely rare; conversely, the liver is the most frequent site of metastases from gastrointestinal carcinoids. Clinically, primary lesions are characterized, in most cases, by the absence of an overt endocrine syndrome. Histologic findings and immunohistochemical demonstrations of chromogranin and neuron specific enolase, generally, enable the neuroendocrine origin of these neoplasms to be established. Prognosis after surgical treatment of primary hepatic carcinoids seems to be more favorable when compared with other hepatic carcinomas.
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PMID:[A case of primary hepatic carcinoid. A report of its surgical resolution]. 926 29

We monitored both chromogranin A (CgA) and neuron specific enolase (NSE) in serial serum specimens from 14 patients with prostate cancer (CAP patients) showing resistance to hormonal treatment. Elevated serum CgA was detected in 10 out of these 14 patients (71%) during treatment, and an early appearance of elevated serum CgA was found in 6 of 14 (43%) of these patients when serum tPSA levels were still in the normal range. If patients with radical prostatectomy were not included, the percentage of patients showing an early appearance of elevated serum CgA would have been much higher. Elevated serum CgA levels also were found in patients not subject to hormonal therapy. Serial specimens from two out of three prostate cancer patients, randomly selected, contained elevated serum CgA. Serum NSE was not detectable in any of the serial specimens we studied, suggesting that CgA, not NSE, should be used as a marker for neuroendocrine differentiation. We also compared the serum CgA in random serum specimens between patients with BPH (benign prostate hyperplasia) and with prostate cancer in the concentration range of serum tPSA between 3-15 ng/mL. Although serum CgA concentrations in BPH patients overlapped considerably with those levels in patients with prostate cancer, levels > 100 ng/mL should suggest prostate cancer. The early appearance of elevated serum CgA allows an early change of therapy to be made and can lead to the effective prevention of any further development of metastases.
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PMID:Serum chromogranin A: early detection of hormonal resistance in prostate cancer patients. 948 65

Oncocytic neoplasms of the adrenal gland are rare. We describe the clinicopathologic and immunohistochemical findings of seven oncocytic adrenocortical neoplasms, five oncocytomas, and two oncocytic neoplasms of uncertain malignant potential. Three tumors were studied using electron microscopy. These neoplasms occurred in five women and two men (median age, 55 years) with no clinical evidence that the neoplasms were functional. The size of the neoplasms varied from 5.0 cm to 13.5 cm. Histologically, each neoplasm was composed exclusively of oncocytes. The oncocytomas had very low or absent mitotic activity and no evidence of necrosis. The two oncocytic neoplasms of uncertain malignant potential had increased mitotic activity and necrosis but no evidence of invasion or metastases. Nuclear atypia, either focal or generalized, was found in all neoplasms. Immunohistochemical studies performed using fixed, paraffin-embedded sections showed strong reactivity with the mitochondrial antibody mES-13 in all neoplasms. Four of five oncocytomas and one oncocytic neoplasm of uncertain malignant potential expressed keratin, predominantly keratin 18, as shown using the CAM 5.2 and AE3 antibodies. Two neuroendocrine-associated markers, neuron specific enolase and synaptophysin, were positive in seven and five neoplasms, respectively. However, all neoplasms were negative for the other neuroendocrine markers tested, including chromogranin A, tyrosine hydroxylase, and dopamine beta-hydroxylase, as well as for epithelial membrane antigen, S100, and p53. Using the MIB-1 (Ki-67) antibody, proliferative activity was increased in both oncocytic neoplasms of uncertain malignant potential. All six patients with available clinical follow-up data are alive without evidence disease, although the follow-up interval is relatively short (< 2 years) for the two patients with oncocytic neoplasms of uncertain malignant potential. We conclude that oncocytic adrenocortical neoplasms are nonfunctional tumors that can become large before they are detected by radiologic studies. The majority of neoplasms are benign and should not be misdiagnosed as carcinoma.
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PMID:Oncocytic adrenocortical neoplasms: a report of seven cases and review of the literature. 959 31

A group of biphasic low-grade thymic epithelial tumors is presented that we suggest calling low-grade metaplastic carcinoma of the thymus [37]. Four of the patients were men, their age ranging from 44 to 71 years. Three tumors invaded mediastinal fat or pleura. No metastases were present. Histologically, the tumors showed a biphasic pattern with solid carcinomatous areas merging with a spindle cell component. Only few lymphocytes were present. Cytological atypia and mitotic activity were variable in the solid areas, but low in the spindle cell component. The tumors showed expression of cytokeratin, vimentin and/or epithelial membrane antigen (EMA), both in the carcinomatous and in spindle cell components. In two cases, actin expression was also present in both components. In one case, chromogranin, S100 protein, glial fibrillary acidic protein, and neuron specific enolase were expressed in a minority of cells of both components. None of the patients had myasthenia gravis. All patients are alive without distant metastasis, but meanwhile one patient suffers from local recurrence. We conclude that metaplastic carcinoma of the thymus is a clinicopathological entity that is probably distinct from the recently described "thymoma with pseudosarcomatous stroma", and should be distinguished from the usually benign medullary thymomas and the highly aggressive carcinosarcomas and sarcomatoid carcinomas.
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PMID:Low-grade metaplastic carcinomas of the thymus: biphasic thymic epithelial tumors with mesenchymal metaplasia--an update. 1048 86

A 60-year-old male was referred to our hospital with a complaint of asymptomatic gross hematuria. Cystoscopic examination revealed a non-papillary broad-based tumor on the posterior wall of the urinary bladder. Computed tomography revealed no evidence of metastases. Transurethral resection of bladder tumor (TUR-BT) was performed and muscle invasion was detected by histological examination of the specimen. Total cystectomy and ileal conduit formation were performed at the preoperative diagnosis of T2-3N0M0. Hematoxylin-eosin staining of the specimen revealed small cancer cells with hyperchromatic nucleus and scanty cytoplasm growing in the muscle layer of the urinary bladder and in the left obturator lymph nodes. Immunohistochemistry for neurospecific enolase showed diffuse staining in the cytoplasm of cancer cells, and ultrastructural study showed dense core granules. From these findings, the patient was diagnosed with small cell neuroendocrine carcinoma of the urinary bladder at the stage of pT3bpN1M0. Three courses of adjuvant chemotherapies with cis-platinum (CDDP) and etoposide were administered. The patient is still alive with no evidence of any recurrence at 22 months after the operation. This case suggests that treatment with combined total cystectomy and adjuvant CDDP and etoposide chemotherapies is effective against neuroendocrine carcinoma of the urinary bladder with regional lymph node metastases.
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PMID:[Small cell neuroendocrine carcinoma of the urinary bladder: a case report]. 1054 Jul 13

Solid-pseudopapillary tumors are uncommon neoplasms of low malignant potential generally occurring in young women. They often cause few symptoms and may reach a large size by the time they are detected. Degenerative cystic changes are common, and the clinical presentation is often that of a cystic pancreatic tumor. Pathological features include solid, cellular, hypervascular regions without gland formation, and degenerative pseudopapillae. The cells contain eosinophilic granules rich in alpha-1-antitrypsin and the nuclei are typically grooved. Despite its characteristic microscopic appearance, the immunophenotype (positive for vimentin, alpha-1-antitrypsin, and neuron specific enolase) is not specific and does not define a line of differentiation corresponding to any normal pancreatic cell type. Ultrastructural studies have also failed to identify specific differentiated features. Nonetheless, the biological behavior of solid-pseudopapillary tumor is well established. The tumor is indolent, with infrequent metastases to liver or peritoneum and usually long survival, even in the presence of disseminated disease.
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PMID:Solid-pseudopapillary tumor of the pancreas: a typically cystic carcinoma of low malignant potential. 1072 8

A captive adult female muskrat (Ondatra zibethicus) was found dead without previous signs of disease. At necropsy, abdominal organs were infiltrated with a poorly demarcated, soft, tan tissue. Microscopically this tissue was composed of neoplastic cells assuming two distinct growth characteristics consistent with Antoni A and B patterns. Ultrastructurally, the neoplastic cells were pleomorphic, lacked junctional devices, had abundant mitochondria and ergastoplasm, and frequently were closely associated with extracellular collagen. Immunocytochemical examination of tumor cells demonstrated sporadic expression of neuron specific enolase. Microscopic tumor metastases to the myocardium, ascending aorta, lungs and visceral pleura were present. This is the first report of a sarcoma compatible with a malignant peripheral nerve sheath tumor in a muskrat.
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PMID:Sarcoma of possible nerve sheath origin in a captive muskrat. 1094 48

Gastrointestinal autonomic nerve sarcomas are rare tumours. A 36-year-old man presented with abdominal pain, distension and constipation. Abdominal ultrasound and CT scanning demonstrated a giant cystic intra-abdominal mass. Laparotomy confirmed a large cystic mass arising from the ileum with multiple metastases. Immunohistochemical staining was positive for vimentin, neuronal-specific enolase and PGP9.5. This is the first reported case to present as a giant intra-abdominal cyst. Specialist histopathological and immunohistochemical analysis is essential to establish the diagnosis of this rare tumour.
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PMID:Gastrointestinal autonomic nerve sarcoma presenting as a giant intra-abdominal cyst. 1105 53


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