Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0027651 (tumor)
 685,946 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Sclerosing hemangioma of the lung remains poorly understood, and it is still unclear whether this lesion is neoplastic or not. It consists of two major cell types, pale cells and cuboidal cells. We analyzed the clonality of each cell types from six female cases of surgically resected sclerosing hemangioma. The pale cells and cuboidal cells were separated by microdissection from methanol-fixed sections, and DNA was extracted for clonal analysis based on an X-chromosome-linked polymorphic marker, the human androgen receptor (HUMARA) gene or the phosphoglycerate kinase (PGK) gene. The HUMARA and PGK genes were found to be amplified with or without digestion by the methylation-sensitive restrictive endonuclease HpaII. Five of six cases were informative. Pale cells and cuboidal cells showed the same monoclonality in all of the informative cases, whereas the control cells showed a polyclonal pattern. Our results demonstrated that sclerosing hemangioma is caused by monoclonal expansion of cells, confirming that it is a neoplasia. Moreover, the present data indicate that both pale cells and cuboidal cells are derived from the same cell.
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PMID:Monoclonality of both pale cells and cuboidal cells of sclerosing hemangioma of the lung. 954 67

A 3-year-old boy presented with persistent abdominal pain, hematuria and facial pallor with progressive anemia. A computed tomography scan showed a massive intraperitoneal hemorrhage and tumor mass in the bladder. Histological examinations of the resected tumor revealed findings of pseudosarcomatous myofibroblastic tumor (PMT). The clinico-pathological features of this case warn us that PMT can cause acute abdomen syndrome with massive intraperitoneal hemorrhage.
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PMID:Pseudosarcomatous myofibroblastic tumor of the urinary bladder with massive intraperitoneal hemorrhage in a child. 982 9

A retrospective clinical and pathological study of 4 patients who developed the syndrome of radiation induced dementia was performed. All patients fulfilled the following criteria: (1) a history of supratentorial irradiation; (2) no evidence of symptomatic recurrent tumor; (3) no other cause of progressive cerebral dysfunction and dementia. The clinical picture consisted of a progressive "subcortical" dementia occurring 3-12 months after a course of cerebral radiotherapy. Examination revealed early bilateral corticospinal tract involvement in all patients and dopa-resistant Parkinsonian syndrome in two. On CT scan and MRI of the brain, the main features consisted of progressive enlargement of the ventricles associated with a diffuse hypodensity/hyperintensity of the white matter best seen on T2 weighted images on MRI. The course was progressive over 8-48 months in 3 patients while one patient had stabilization of his condition for about 28 years. Treatment with corticosteroids or shunting did not produce sustained improvement and all patients eventually died. Pathological examination revealed diffuse white matter pallor with sparing of the arcuate fibers in all patients. Despite a common pattern on gross examination, microscopic studies revealed a variety of lesions that took two basic forms: (1) a diffuse axonal and myelin loss in the white matter associated with tissue necrosis, particularly multiple small foci of necrosis disseminated in the white matter which appeared different from the usual "radionecrosis"; (2) diffuse spongiosis of the white matter characterized by the presence of vacuoles that displaced the normally-stained myelin sheets and axons. Despite a rather stereotyped clinical and radiological course, the pathological substratum of radiation-induced dementia is not uniform. Whether the different types of white matter lesions represent the spectrum of a single pathological process or indicate that the pathogenesis of this syndrome is multifactorial with different target cells, remains to be seen.
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PMID:Dementia following treatment of brain tumors with radiotherapy administered alone or in combination with nitrosourea-based chemotherapy: a clinical and pathological study. 1022 34

To study the distribution of presenting features and their prognostic significance in neuroblastoma treated in a single institution in Malaysia. A retrospective study was made of 78 neuroblastoma cases diagnosed and treated in the University Hospital, Kuala Lumpur, Malaysia between June 1982 and February 1997. Diagnosis was established by standard histological criteria. The presenting features were evaluated for their distribution and prognostic influence. Disease-free survival from diagnosis was the outcome variable of interest. The ages ranged from 0.1 to 11 years old (median: 3 years old). The tumor originated from the adrenal glands in 83% and the majority of cases presented in advanced stage (stage III 22%, stage IV 66%). Bone marrow was the commonest site of distant metastasis occurring in 45% of patients. The main presenting signs and symptoms in decreasing order were pallor, fever, abdominal mass, weight loss, and bone/joint pain. Univariate analysis conferred age, initial stage and Hb level as significant prognostic factors. No influence in disease-free survival was found for sex, race, primary site, urinary vanillylmandelic acid level, white cell count and platelet count. Overall 2-year disease-free survival was achieved in 27 (39%) patients. Four patients underwent bone marrow transplant, three of whom achieved 2-year disease-free survival. The results suggest that age, initial stage and hemoglobin level are significant prognostic factors based on univariate analysis. In addition, more Malaysian children presented with adrenal primary site and advanced disease compared to previous reported studies.
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PMID:Presenting features and treatment outcome of 78 Malaysian children with neuroblastoma. 1069 3

Cyclic vomiting syndrome (CVS) remains a mysterious disorder despite our increasing knowledge since its classic description by Gee in 1882. Its hallmark feature of recurrent, explosive bouts of vomiting punctuating periods of normal health causes substantial medical morbidity (50% of patients require intravenous therapy), as well as significant time lost from school (20 school absences per year) and work. Limited epidemiologic data indicate that CVS may occur more commonly than previously thought, affecting as many as 1.9% of school-aged children. Besides the relentless vomiting, the child usually has pallor (87%), lethargy (91%), anorexia (74%), nausea (72%), and abdominal pain (80%). There is evidence of clinical and physiologic overlap among CVS, abdominal migraine, and migraine headaches. We propose revised criteria for abdominal migraine that include pain as the predominant and consistent symptom, lack of abnormal screening tests, and in retrospect, either subsequent development of migraines or positive response to antimigraine medication. Besides migraines, other etiologic possibilities include mitochondrial DNA mutations, ion channelopathies, excessive hypothalamic-pituitary-adrenal axis activation, and heightened autonomic reactivity. The differential diagnosis includes idiopathic CVS (88%); gastrointestinal disorders (7%), including serious surgical disorders (e.g., malrotation); and extraintestinal disorders (5%), including serious surgical (brain stem neoplasm) and metabolic disorders (e.g., fatty acid oxidation disorder). Within the idiopathic group, there may be migraine, Sato's neuroendocrine, mitochondrial, and other subgroups. Treatment includes avoidance of triggers, prophylactic medication, supportive care, abortive medication, and family support. In the future, investigation into mitochondrial DNA mutations, ion channel defects, corticotropin-releasing factor, and serotonin and tachykinin receptor physiology and pharmacology may help discover the etiology and pathogenesis of this disorder.
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PMID:Cyclic vomiting syndrome: evolution in our understanding of a brain-gut disorder. 1095 42

We report a case of cribriform-morular variant (C-MV) of papillary thyroid carcinoma (PTC) in a 27-year-old woman. In addition to conventional cytologic features of typical PTC, the fine-needle aspirate showed numerous epithelial cells with abundant, eosinophilic, very elongated cytoplasm. Microscopically, the tumor was encapsulated and highly cellular and exhibited a mixture of cribriform, follicular, papillary, trabecular, solid, and spindle cell patterns of growth, with morular foci showing peculiar nuclear clearing (biotin-rich nuclei). The cells were cuboidal or tall, with frequent nuclear pseudostratification and abundant eosinophilic cytoplasm. The nuclei were usually hyperchromatic, with grooving, pallor, and pseudoinclusions. Angioinvasion and foci of capsular invasion were observed. Immunohistochemically, the neoplastic cells showed reactivity for thyroglobulin, epithelial membrane antigen, low- and high-molecular-weight cytokeratins, vimentin, neuron-specific enolase, CD15, estrogen and progesterone receptors, and bcl-2 protein. Molecular genetic analysis of the APC gene revealed a mutation in exon 15 at codon 1309 in tumoral tissue but not in peripheral lymphocytes. These findings support a relationship between the morphologic pattern of the C-MV of PTC and the APC gene and the existence of this variant as a sporadic counterpart of familial adenomatous polyposis-associated thyroid carcinoma.
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PMID:Somatic but not germline mutation of the APC gene in a case of cribriform-morular variant of papillary thyroid carcinoma. 1129 95

The present retrospective study evaluated 180 patients with resectable (Group I ) and 128 patients with unresectable (Group II) gastric carcinoma at Belen Hospital, Trujillo, Peru, from 1966 to 1998, with the aim to identify the clinical and pathological features, actuarial survival rate and surgical morbidity and mortality rates of both groups. The mean age of the total series was of 58.3 + 14.8 years (range, 18 to 85 years). The most frequent symptoms in both groups were abdominal pain (89.4% and 94.5% respectively) and the most common sign was pallor (62.8% and 54.5% respectively). The unresectable cases presented a higher frequency of palpable mass (p<0.001), upper two thirds neoplasms (p=0.0032), T4 lesions (p<0.001), distant metastasis (p<0.001), stage IV (p<0.001), hepatic metastasis (p<0.001) and peritoneal metastasis (p<0.001), compared with resectable gastric cancer patients. The total surgical mortality rate was of 19.5% (Group I: 12.1%, Group II: 28.9%). The most frequent complications were pneumonia (Group I: 8.9%, Group II: 7.8%) and surgical wound infection (Group I: 10.6%, Group II: 3.9%). In Group II, the exploratory laparotomy was carried out in 82 cases, whilst 46 cases underwent gastroenterostomy (n=34), gastrostomy (n=6), gastrectomy by exclusion (n=5) and ileotransversoanastomosis (n=1). The 5-year survival rate in resectable patients was of 18.5% and in unresectable cases the survival rate at 12 and 36 months was of 9% and 0% respectively. The early diagnosis of this neoplasm, mainly in high risk patients, would offer better possibilities of an opportune treatment.
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PMID:[Clinic-pathologic pattern and survival range in resectable gastric carcinoma]. 1196 66

A 25-year-old male student complained about episodic palpitations, dizziness, nausea and headache 5 years prior to presentation. No otorhinolaryngic, neurologic or gastrointestinal causes were identified. Several ECG recordings revealed sinus node dysfunction with intermittent sinus arrest and AV-nodal escape rhythm. The patient was given a permanent DDD-pacemaker. Six months later, the clinical symptoms were unchanged. During an attack, physical examination revealed paleness, diffuse sweating and an arterial blood pressure of 250/130 mmHg, which decreased to 120/80 mmHg within a few minutes. Abdominal ultrasound and abdominal computed tomographic scan demonstrated the presence of a large (6.4 x 5.5 cm) left-sided adrenal mass. Two 24-h-urinary collections demonstrated elevated noradrenaline (mean 315 micrograms/24 h, normal < 80 micrograms/24 h) and adrenaline (mean 268 micrograms/24 h, normal < 20 mg/24 h) levels. Blood samples, which were drawn during excessive blood pressure rise, revealed elevation of plasma catecholamines (6.793 pg/ml for adrenaline (normal 50-150 pg/ml) and 10.424 pg/ml for noradrenaline (normal 200-500 pg/ml), so that the diagnosis of pheochromocytoma was considered established. The tumor was successfully removed during laparascopic surgery. After surgery, the patient remained well and normotensive. Three months later, several long-term ECG recordings showed sinus arrhythmia with no evidence of sinus arrest or AV-nodal escape rhythm, so that the DDD pacemaker was turned off. This case underlines that sinus node dysfunction with intermittent sinus arrest and AV-nodal escape rhythm is a potential early manifestation of a pheochromocytoma. These changes seem to disappear after successful removal of the tumor.
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PMID:[Sinus node dysfunction with intermittent sinus arrest and AV-nodal escape rhythm as initial manifestation of pheochromocytoma]. 1196 12

In Peru, the Colorectal Cancer affects people of both sexes, making it necessary to know the clinical characteristics of this disease, in order to improve its diagnosis. With this purpose it was set a retrospective and descriptive study at E. Rebaglitati Martins Hospital. We included those patients that were attended between 1995 and 1999 and that had a pathologic diagnostic of colorectal cancer. The rectal tumor was the most frequent in males (40.9%) and females (31.3%). The kind of symptoms was related with the tumor localization, in rectum, it predominates hematochezia (29.13%), rectal bleeding (29.15%) and constipation (27.55%), while in the right colon there was abdominal pain (59.37%), lose of weight (53.12%) and pallor (46.87%). Between the fourth decades of age, there was a significant increase of the colorectal cancer prevalence. Likewise, 46.36% of the patients come to consult with an advance stage of disease (Dukes C,D). The carcinoembrionary antigen value was normal in 57.14% of patients. The colonoscopic exam associated with biopsy had a diagnostic success of 99.09%
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PMID:[CLINICAL CHARACTERISTICS IN COLON CANCER E. REBAGLIATI MARTINS HOSPITAL 1995-1999] 1214 May 75

A retrospective study was performed on patients diagnosed of gastric cancer, younger than 40 years old, in the Hospital Arzobispo Loayza, from January 1991 to December 1998.We found 81 patients younger than 40 years old, with confirmed diagnosis of gastric adenocarcinoma; we reviewed the records of 59 patients.There were 44 (74.6%) women and 15 (25.4%) men. The mean age was 31.644 +- 5.437 years and the mean duration of symptoms was 13.189 months.The most common symptoms were: epigastric pain (89.8%), weight loss (86.4%), dyspepsia (64.4%),vomiting (64.4%) and nausea (62.7%). The most frequent physical findings were pallor (62.7%), epigastric tenderness (40.7%) and emaciation (37.3%). 57.8% of patients had hemoglobine levels of less than 11 gr/dl.The most frequent location of the neoplasm was the antrum (31.1%) followed by the body (27.3%).On endoscopy, we observed that the most frequent lesion was advanced gastric cancer, Borrmann III (48%), followed by Borrmann IV (27.3%). The predominant histologic type was undifferentiated adenocarcinoma.
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PMID:[GASTRIC CANCER IN PATIENTS YOUNGER THAN FORTY YEARS OLD.HOSPITAL LOAYZA EXPERIENCE BETWEEN 1991 - 1998] 1214 May 86


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