Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0677930 (primary tumor)
 20,210 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The clinical, light microscopic, and ultrastructural features of four malignant carcinoid tumors in children, three boys and one girl ages 8--14 years, are described. Extensive metastases to multiple organs were present in three, and in the fourth child there was diffuse local infiltration of the bowel wall, which resembled a lymphoma. The primary tumor arose in the ileum in one child and in the transverse colon in another. In two children, the primary sites could not be determined; one patient is still alive and in the other, permission for autopsy was refused. Electron microscopy showed moderate numbers of neurosecretory granules in some cells in all cases. One patient with extensive metastases showed repeated partial response to radiotherapy and chemotherapy. Eight benign appendiceal carcinoids were seen at the same hospital over the same period, suggesting malignant carcinoids may be more common in children than often assumed.
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PMID:Malignant carcinoid tumors in children. 705 88

Although patients with bronchial and ovarian carcinoid tumors can develop the carcinoid syndrome (diarrhea and/or flushing) in the absence of hepatic metastasis, it is believed that development of the carcinoid syndrome in patients with carcinoid tumors of gastrointestinal origin occurs only after the patient has hepatic metastasis. This is explained by hepatic inactivation of most of the serotonin in the portal circulation or by the fact that hepatic metastases are larger than the primary tumor in the gastrointestinal tract. Three patients with ileal and jejunal carcinoid tumors who developed the carcinoid syndrome without obvious hepatic metastasis are described. Two of the patients had intra-abdominal, but extrahepatic, metastasis that probably drained directly into the systemic circulation. The third patient had an ileal carcinoid with clinical involvement limited to adjacent mesenteric lymph nodes. Following resection of her tumor, her urinary 5-HIAA excretion and platelet serotonin level returned to normal, and her attacks of carcinoid flushing virtually ceased. She has occasional spells of "blushing" that are thought to be benign; however, further close follow-up study will be needed to be certain that she is free of disease. It is suggested that each patient with the carcinoid syndrome be evaluated with CT and technetium-99 pertechnetate liver scans. If there is no liver involvement detected with these studies, one should consider hepatic arteriogram or laparotomy to determine if the patient's tumor might be totally resectable.
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PMID:Carcinoid syndrome from gastrointestinal carcinoids without liver metastasis. 709 50

A case of pure carcinoid of the testis is reported. The patient presented with metastatic carcinoid and the carcinoid syndrome 17 years after removal of the primary tumor. He died 2 years later. The long interval from initial orchiectomy to presentation with metastases strongly suggests that the original lesion was primary rather than metastatic. Only 2 cases have been reported to date in which an apparently pure primary testicular carcinoid developed metastases. In both of these cases the possibility that the testicular lesion itself was metastatic was not excluded.
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PMID:Primary carcinoid of the testis with metastases. 720 68

Twenty-six cases of carcinoid-related mesenteric angiopathy and intestinal infarction (three from our institution and 23 previously reported cases) were reviewed. Twenty patients presented with acute abdominal findings, including peritonitis (13 cases), intestinal obstruction (five cases), and bleeding per rectum (two cases). Fifteen patients (75%) experienced antecedent symptoms of abdominal pain and/or diarrhea, averaging 2.5 years in duration. Twelve patients (46%) exhibited symptoms of carcinoid syndrome. Mesenteric angiography in three cases demonstrated encasement and segmental branch narrowing or occlusion of major mesenteric vessels. Eleven patients underwent resection and primary bowel anastomosis with an early survival rate of 91%. Four additional patients who underwent lesser surgical procedures and five patients who did not undergo operation all died. Elastic vascular sclerosis (EVS) was identified in 19 of 22 cases with available histologic material (86%). These changes were observed in proximity to as well as distant to the primary tumor. In general, the severity of EVS did not correlate with the likelihood of gut ischemia. Although not the sole cause of intestinal gangrene in patients with midgut carcinoids, EVS may contribute significantly to the evolution of these ischemic changes.
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PMID:Mesenteric angiopathy, intestinal gangrene, and midgut carcinoids. 728 Oct 10

The accuracy of stage I lung cancer assessment achieved by traditional clinico-diagnostic staging was retrospectively evaluated in 164 consecutive patients who underwent thoracotomy. The diagnostic conversion rate was 6.7% (1 carcinoid and 10 innocent pulmonary lesions) and occurred only in the subset of patients lacking preoperative pathologic confirmation (15%). The conversion rate to unresectable tumor extent was 8% (11/153), and local spread was the main cause of unresectability (5.5%). The staging conversion rate was 29% (43/153): the conversion rate for nodal evaluation was double that of primary tumor evaluation (24% versus 12%), but conversion to anatomically unresectable nodal diffusion occurred in only one patient (0.6%). The ability of the surgeon to convert the wrong diagnosis was scanty without extemporary biopsy, and 7 patients with innocent lesions underwent standard resection for primary cancer. Surgical staging was a precise as pathological staging in primary tumor evaluation, but was faulty in nodal evaluation (15% error in sN- and sN1-2 assessment). It is concluded that following stage I lung cancer assessment by traditional means, supplementary examinations are requested for a better sensitivity of pathological confirmation and a better refinement of local spread. Better nodal evaluation has less value until a biologic limit to surgery for anatomically resectable nodal diffusion is universally accepted.
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PMID:Post-thoracotomy diagnostic and staging conversion rates of clinically staged I lung cancer. 728 Dec 43

Unusual filamentous inclusion bodies in the cytoplasm of metastatic tumor cells are described. Their presence (intermingled with zymogen granules) seems rather restricted to cells of primary or metastatic acinar cell carcinoma of the pancreas, acinic carcinoma of salivary gland (parotid gland) and Paneth cells (neoplastic or in zinc deficiency state). For the time being, the real nature of these inclusions (deranged zymogen granules?) is unclear. This case also emphasizes the value of electron microscopy in solving the problem of the occult primary tumor and avoiding the misdiagnosis of an endocrine tumor (e.g. islet cell tumor or carcinoid), or a duct cell tumor with eosinophilic granular cytoplasm or in recognizing the foci of acinar cells in a mixed variant of carcinoma of the pancreas.
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PMID:Unusual intracytoplasmic inclusions in metastatic carcinoma. Discussion of their possible significance. 747 75

The prognosis and the quality of life of patients with carcinoid tumors is related either to symptoms from the substances secreted or to progressive tumor growth. Medical treatment with cytotoxic agents is of marginal value for increasing life expectancy and reducing clinical symptoms. Recent studies with interferon have shown interesting results. In the present investigation, 22 patients with carcinoid tumors and syndrome were treated with recombinant interferon alpha-2a (r-IFN alpha-2a) at the dose of 6 x 10(6) IU intramuscularly daily for 8 weeks and three times weekly thereafter. The primary tumor was localized in the foregut (n = 11), midgut (n = 7), hindgut (n = 1), and unknown site (n = 3). Most cases had liver metastasis. Seventeen patients had elevated 5-hydroxyindoloacetic acid (5-HIAA) excretion and 5 had flushing and/or diarrhea as the only clinical manifestation. Six cases presented a complete syndrome (flushing, diarrhea and 5-HIAA excretion). Control of symptoms was obtained in 80% and a 5-HIAA level reduction in 58% of the patients. The interferon treatment was more effective for control of the carcinoid syndrome than for control of tumor growth. The treatment was well tolerated and fever, myalgia, anorexia and fatigue were the most frequent side-effects.
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PMID:Treatment of carcinoid syndrome with recombinant interferon alpha-2a. 768 66

High numbers of high-affinity somatostatin binding sites have been found on carcinoid tumors, gastrinomas, small cell lung cancers and the majority of medullary thyroid cancers, enabling in vivo visualization of these tumors with octreotide scintigraphy. A comparison of the results obtained at our institution and another 15 centers in Europe show a few remarkable similarities and differences. The overall sensitivity of octreotide receptor scintigraphy to detect the primary GEP tumor and its metastases is high, e.g. 80-90%. The main difference was found in gastrinomas and to a lesser extent in insulinomas. These differences might be attributed to different scanning protocols. Furthermore, octreotide scintigraphy also has a high sensitivity to localize the primary tumor and its metastases causing Cushing's syndrome by ectopic production of ACTH or CRH. Octreotide scintigraphy is a new, sensitive and noninvasive technique to localize somatostatin receptor expressing endocrine tumors and their metastases.
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PMID:Somatostatin receptor scintigraphy in carcinoids, gastrinomas and Cushing's syndrome. 769 38

Carcinoid tumors occur most frequently in the gastrointestinal tract. Most of these tumors are clinically silent and the diagnosis is not made before surgery. The surgical treatment depends on the localization and the size of the tumor. Small bowel carcinoid tumors metastasize in 20-30% of the cases if the tumor is smaller than 1 cm. Therefore, the primary tumor should always be resected widely including the regional lymph nodes. Carcinoid tumors of the appendix less than 1 cm in size do not metastasize. For such patients an appendectomy is the treatment of choice. For tumors larger than 2 cm, a right hemicolectomy should be performed. If the tumor is between 1 and 2 cm, the surgical treatment depends on several factors (positive lymph nodes, extension of the tumor into the mesoappendix or subserosal lymphatic invasion, age of the patient). In young patients, an aggressive treatment is preferred. Carcinoid tumors of the colon and rectum less than 2 cm in size rarely metastasize. The surgical treatment for patients with tumors less than 2 cm is local excision, whereas for patients with tumors larger than 2 cm a wide resection is advocated.
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PMID:Surgical treatment of carcinoid tumors of the small bowel, appendix, colon and rectum. 769 43

A 78-year old male came to our observation presenting and enlargement of bilateral inguinal lymph nodes and a tumor of the mesogastric abdominal wall. Three years before the patient had been operated on for a primary tumor of the umbilicus with concomitant longstanding diarrhea. No histological examination was performed at that time. We performed a lymph node biopsy which demonstrated carcinoid metastasis. We went on to perform radical resection of the abdominal wall, regional lymphadenectomy and right hemicolectomy for malignant villous adenoma of the right colon. The abdominal defect was repaired by using Goretex mesh. Cyclic adjuvant alpha-interferon therapy was continued for more than 1 year, followed by long term therapy with longastatin. Twenty months after the operation the patient is in good clinical conditions and disease-free. On the basis of literature review our case appears to be the first primary carcinoid of the umbilicus.
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PMID:[Primary carcinoid of the umbilicus. Description of the 1st case]. 770 43


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