Neuroblastoma: natural history and results of treating 133 cases.
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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.
Carney's Triad comprises a triad of neoplasms: gastric stromal tumor, extra-adrenal paraganglioma (usually functional), and pulmonary chondroma. At least two of these are needed for the presumptive diagnosis of the Triad. This report presents a patient who had resected a gastric tumor and nonfunctional extra-adrenal paraganglioma. The gastric tumor resembled a gastric leiomyosarcoma by light microscopy, but electron microscopy revealed it to be a gastric autonomic nerve (GAN) tumor. Based on this evidence it appears that both the gastric lesions and the paragangliomata of Carney's Triad are tumors of the autonomic nervous system. Thus, the Triad may be a disorder of the autonomic nervous system rather than a multiple endocrine neoplasia syndrome or multiple hamartoma syndrome.
The computed tomographic (CT) body scans of 59 patients and ultrasonograms of 31 patients, all with malignant disease, were reviewed to determine the effect of ascites on the ability to image the retroperitoneum. In 95% of cases evaluated by CT, the retroperitoneal structures were well seen. In 35% of the cases evaluated by ultrasonography, the retroperitoneum was completely obscured by bowel gas and partially obscured in an additional 55%. As a result of this study, it is felt that CT should be the procedure of choice when a thorough evaluation of the retroperitoneum is necessary in patients with large amounts of abdominal ascites.
A case of retroperitoneal hematoma is presented in which the characteristic density changes of the hematoma as seen on serial computed tomography permitted an accurate preoperative diagnosis.
Twenty-five patients were found to have retroperitoneal pathology involving the psoas muscle including nine patients with inflammatory lesions, three with hematomas, seven with adenopathy, one patient with leukemic infiltration of the psoas, and five with retroperitoneal tumors. Computed tomography (CT) offers a unique method to investigate pathological processes that affect the psoas. In addition, the abdominal films on 24 of the patients were analyzed to correlate the value of radiography with that of CT. Computed tomography confirms the belief that the mere presence of a retroperitoneal mass will not necessarily obliterate the psoas.
A case is presented in which a malpositioned spleen mimics a suprarenal mass. The relationship between renal agenesis or malposition and variance in the position of the pancreatic tail and spleen are explained on an embryologic basis.
One-thousand computed tomograms of the abdomen, chest, and pelvis in oncologic patients were reviewed to determine the incidence of serendipitous findings and their impact on clinical management. The incidence of serendipitous findings was 30% in thorax, 24% in both genitourinary and osseous structures, 23% in the abdomen, and 21% in the retroperitoneum. These serendipitous abnormalities changed clinical management or required further diagnostic procedures (thus, they were considered significant) in less than 1% of the abdominal to 12% in osseous findings.
In 50% of 84 ultrasonographic (US) examinations ordered for staging and restaging of non-Hodgkin lymphoma (NHL) patients, the abdomen and pelvis were not sufficiently visualized to allow diagnostic conclusions, whereas nondiagnostic studies were found in only 3% of the 102 computed tomographic (CT) examinations. Discrepancy about the presence and extent of disease below the diaphragm between US and CT occurred in 74%. The US underestimated the presence or extent of disease in nodal stations caudal to the pancreas more commonly than in other areas evaluated. Because of increased cost, patient inconvenience, and delay in therapeutic intervention due to repeat examinations, it is recommended that CT be used as the initial examination for staging, restaging, or following infradiaphragmatic disease in NHL patients.
A 74-year-old woman had multiple enlarged periaortic nodes with inhomogeneous enhancement on CT. Although a malignancy such as lymphoma was considered, amyloidosis involving retroperitoneal lymph nodes was confirmed on biopsy.
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Endometrial stromal sarcoma (ESS) of the uterus is a rare uterine malignancy that has not been characterized in detail. To characterize the phenotype of ESS of the uterus, we extracted RNA from ESS and the stroma of normal endometrium using a tissue microdissection system and compared the expression profiles in the two tissues. After suppression subtractive hybridization and differential screening, we detected the metastasis-associated lung adenocarcinoma transcript 1 (MALAT-1) gene as one of the major genes upregulated in ESS, and a full-length placental cDNA clone (CS0DI066YJ10) as one of the major genes downregulated. The results were confirmed by in situ hybridization in four resected specimens of ESS and 36 biopsy specimens of normal endometrial tissue. All ESS (4/4) and all cases of endometrial stromal cells in the proliferative phase (13/13) were positive for MALAT-1, but samples of normal stroma in the secretory phase and menopausal state included some that were negative or weakly positive for MALAT-1 (5/13 and 3/10, respectively). In contrast, all ESS and 12 of 13 cases of stromal cells in the proliferative phase were negative for the full-length placental cDNA clone but 10 of 13 cases of endometrial stromal cells in the secretory phase were positive for transcripts of the gene (P < 0.05). These results indicated that endometrial stromal cells have different phenotypic characteristics between proliferative and secretory phases and the tumor cells of ESS have the phenotypic character of endometrial stromal cells in the proliferative phase.
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A mucinous cystadenoma of the mesentery and two borderline mucinous cystadenocarcinomas of the mesentery and retroperitoneum are reported. The patients were females, aged 38, 47 and 58 years. The cysts showed identical features to those commonly seen in the appendix and ovary. One of our cases, with 'borderline' histology, developed metastases to mediastinal lymph nodes, 4 years after diagnosis. We suggest that these tumours develop through mucinous metaplasia in pre-existing mesothelium-lined cysts, the latter being the commonest cysts in this location.
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