ABSTRACT
This report describes three patients with both multiple intestinal polyps and tumors of neural crest origin. This combination of findings may represent a new clinical syndrome. The embryologic relationships between tumors derived from endoderm and tumors derived from neurocrest are described. An inherent defect in tissue proliferation or repair is postulated to explain the abnormal growth in these two different cell lines.
Subject(s)
Colonic Neoplasms/embryology , Endoderm , Neoplasms, Multiple Primary/embryology , Neural Crest , Pheochromocytoma/embryology , Adenoma/embryology , Adrenal Gland Neoplasms/embryology , Adult , Carcinoid Tumor/embryology , Carcinoid Tumor/secondary , Carcinoma/embryology , Carcinoma/secondary , Humans , Intestinal Polyps/embryology , Liver Neoplasms/secondary , Lung Neoplasms/secondary , Male , Middle Aged , Pheochromocytoma/secondary , Syndrome , Thyroid Neoplasms/embryologyABSTRACT
Endocrine manifestations were absent in a patient presenting with pain in the left peri-umbilical region from a paraganglioma of the organs of Zuckerkandl. The authors describe the embryology of paragangliomas and review the published literature concerning the noradrenaline-secreting forms of these tumors. Only 86 references to those of Zuckerkandl, situated on the side of the aorta in relation to the inferior mesenteric artery, were found. Diagnosis of non-secreting forms is confirmed by arteriography which in this present case demonstrated three pedicles arising from the inferior mesenteric, celiac, and one of the lumbar arteries respectively. The only evidence of malignancy is the presence of metastases. Their diagnosis is therefore difficult but essential as one-third of the localisations of malignant pheochromocytomas (10 p. cent of pheochromocytomas) outside of the adrenals affect the organs of Zuckerkandl.
Subject(s)
Chromaffin System/diagnostic imaging , Para-Aortic Bodies/diagnostic imaging , Paraganglioma/diagnostic imaging , Pheochromocytoma/diagnostic imaging , Angiography , Humans , Male , Middle Aged , Paraganglioma/embryology , Pheochromocytoma/embryology , PrognosisABSTRACT
Aberrations in the metabolic pathways of catecholamines in patients with neural crest tumors result in characteristic urinary excretion patterns of their catabolites. Tumors such as pheochromocytoma, neuroblastoma and ganglioneuroma usually defy clinical diagnosis because of their rarity, small size, intraabdominal position and clinical symptoms similar to those of essential hypertension. Quantitative determination of catecholamine metabolites such as vanillylmandelic acid (VMA) and 3-methoxy-4-hydroxyphenylethyleneglycol (MHPG) offers possibilities for reliable confirmation of diagnosis. However, previous techniques for the assessment of catabolite levels suffered from inadequate sensitivity, reproducibility or specificity, which seriously diminished their usefulness as biochemical determinants in the prognosis of these life-threatening tumors. Reported in this paper is the analysis of urinary levels of VMA and MHPG using reversed-phase high-performance liquid chromatography with electrochemical and sectrophotometric detection. We present the excretion patterns showing these metabolites in 15 control subjects, 15 patients with pheochromocytoma and 5 patients with neuroblastoma.