Neuroblastoma adrenal

FIGURE 10.34 A, Extra-adrenal neuroblastoma. Immunoperoxidase stain for chromogranin A reveals a granular staining pattern in areas of process formation. B, Extra-adrenal neuroblastoma. Immunoperoxidase stain for S-100 protein shows positivity restricted to sustentacular cells. 

Figure 5 - Muscle pH recording during and following excision of large right adrenal neuroblastoma which extended into the liver in a boy weighing 22 ka. (Patient h.m.) the firct operation bcLween...

An adrenal neuroblastoma may invade the upper pole of the kidney (Fig. 24.26). 

Fig. 24.25a,b. Adrenal neuroblastoma in an 11-month-old boy. On enhanced transverse CT scan, transverse images (a) may be misleading. Coronal reconstruction (b) easily confirms the extrarenal origin of the tumor... 

Fig. 24.26a,b. Adrenal neuroblastoma in a 10-month-old boy invading the upper pole of left kidney, a On unenhanced transverse CT scan the mass is almost totally extra-renal on enhanced scan (b) the renal artery arrow) is encased by the tumor. These features are suggestive of neuroblastoma and rarely observed in WT... 

Extrahypothalamic OX-B-like immunoreactivity, reminiscent to that of CRF, has been described in clustered GABAergic neuronal populations, in the lateral division of central nucleus ofthe amygdala, the bednucleus of the stria terminalis, and in the hippocampus. Moreover, ectopic expression of preproorexin mRNA in the gut, ependymal cells, neuroblastomas, and of orexin receptors in adrenal gland, cancer and hematopietic stem cells suggests yet unexplored roles of orexins as paracrine factors controlling blood-brain barrier, and tumor or stem cell function. 

The catecholamines are a group of hormones secreted by the adrenal medulla. The major urinary metabolite of norepinephrine and epinephrine is vanillylmandelic acid (VMA). Urinary levels of VMA are considerably higher than those of total catecholamine. From the standpoint of laboratory methodology, VMA estimation is preferable to total catecholamine estimation, although it is not a simple procedure. VMA has been shown to be elevated in some patients who had phenochromocytoma and normal urinary catecholamines, even though patients with neuroblastoma have a normal VMA level and elevated catecholamine levels. 

Neuroblastoma Liver metastases develop with extreme rapidity within the first 6-9 months of life. The general condition is not compromised until later. This metastatic spread is a result of a sympathogonioma which has its origin in the adrenal medulla or autonomic ganglia. Such a clinical picture is also termed Pepper s syndrome (W. Pepper, 1901). 

Fig. 1. Pertussis toxin-mediated ADP ribosylation of membrane G proteins. Isolated cell membranes (50 ng of protein) from N1E 115 cells (mouse neuroblastoma cell line), N2A cells (mouse neuroblastoma cell line), S49-1 eye cells (S49(-) mutated mouse lymphoma cell line deficient in Ga ), 549 wt cells (wild-type mouse lymphoma cell line), RBL (RBL 2H3 rat basophilic leukemia cell line), GH3 cells (GH3 rat hypophyseal tumor cell line), PC-12 (rat pheochromocytoma cell line), HIT-T15 cells (hamster insulinoma cell line), Y-1 cells (mouse adrenal cortex tumor cell line), 108 cc 15 cells (mouse/rat neuroblastoma x glioma hybrid cell line), HL-60 cells (DMSO-differentiated human leukemia cell line), HL-60 (+PT) cells (HL-60 cells pretreated with 25 ng/ml of pertussis toxin for 24 h prior to preparation of membranes), RINm5F cells (rat insulinoma cell line), and C6-2 cells (rat glioma cell line) were subjected to P-ADP-ribosylation as described in section 4.3.3. Samples were precipitated as outlined in section 4.3.5 and subjected to SDS-PAGE with separating gels containing 8% acrylamide (w/v). An autoradiogram of the dried gel is shown. Molecular masses of marker proteins are indicated (kDa). Modified Ga proteins migrate at approximately 40 kDa. Radioactivity running in front of the 30 kDa marker protein comigrates with the dye front...

Pheochromocytomas are catecholamine-producing tumors that arise from chromaffin cells of the adrenal medulla. Excluding neuroblastomas, about 10% of catecholamine-producing tumors arise from extraadrenal sympathochro-maffin tissue, usually in the abdomen, and are known as... 

The anatomic location of the primary tumor in neuroblastomas parallels the sympathetic nervous system, as predicted from its neuronal origin. The majority of tumors are intraabdominal, arising in the adrenal gland or the upper... 

The simplest in vitro model, the cell line, is a population of cells that can be maintained in culture for an extended period of time. Neuronal cell lines normally originate from a single common ancestor cell (clonal) and are often derived from tumors, e.g., pha-eochromocytomas (adrenal medullary tumor) (e.g., PC12 cell line) [13] and neuroblastomas [14] such as mouse N2a or human SH-SY5Y. However, a recently developed technique to introduce oncogenes into primary cultures through retroviruses has opened up new possibilities [15]. 

Neuroblastomas are small, round, blue cell tumors that may arise in the adrenal gland and a variety of extra-adrenal sites. The differential diagnosis is wide and includes rhabdomyosarcoma, Ewing s sarcoma-primitive neuroectodermal tumor (ES-PNET), medulloblastoma, small cell osteosarcoma, lymphoblastic lymphoma, blastematous Wilms tumor, and small cell desmoplastic tumor. Numerous markers have been used for the diagnosis of neuroblastomas including NE markers, cytoskeletal proteins, catecholamine-synthesizing enzymes, and neuroblastoma-"specific antibodies (Eig. 

Franquemont DW, Mills SE, Lack EE. Immunohistochemical detection of neuroblastomatous foci in composite adrenal pheochromocytoma-neuroblastoma. Am J Clin Pathol. 1994 102 163-170. 

The human Y1 mRNA is about 3.5-4 kb and was detected in the neuroblastoma cell line SK-N-MC by Northern hybridization (Larhammar et al., 1992). An in situ hybridization study with a riboprobe reported widespread distribution in human fetal and adult organs, e.g. colon, kidney, heart, placenta and adrenal (Wharton etal., 1993). However, a Northern hybridization in the same study detected a single mRNA of only 2.2 kb suggesting that the specificity of the probe for Y1 mRNA may be questioned. Curiously, in the same study the size for NPY mRNA was reported as 3.3 kb, which disagrees with the size seen in a human pheochromocytoma (0.8 kb) as well as the size of the human cDNA clone which ends with a poly(A) tract (Minth et al., 1984). 

Brevetoxins which bind to site 5, enhance the veratridine-activated Na+ influx in neuroblastoma cells (Catterall and Risk 1981) they increase Poo rather than reduce Ko.s (Catterall and Gainer 1985). However in vera-tridine-treated adrenal medullary cells the brevetoxin PbTx-3 increases both Poo and affinity, again determined by Na+ influx measurements (Wada et al. 1994). Brevetoxins also enhance batrachotoxin binding, and this effect is very much potentiated by certain pyrethroids, for example. 

Use of tricyclics in the presence of catecholamine-secreting tumors of the adrenal medulla (e.g., pheochromocytoma, neuroblastoma), may precipitate a hypertensive crisis, due to the increase in catecholamine production in the face of decreased adrenergic reuptake. 

Synaptophysin Neuroendocrine tumors pituitary adenomas, medullary thyroid carcinoma, pheochromocytoma, islet cell tumors, small cell carcinoma, carcinoid and neuroendocrine Medulloblastoma, retinoblastoma, neurocytoma, ependymoma, neuroblastoma, adrenocortical tumors. Merkel cell tumors Neuronal and neuroendocrine cells, carotid body cells, adrenal cortex and medulla... 

Fig. 7.13. A 1-year-old boy with a solid tumour arising from the adrenal gland. Biopsy was performed to confirm the diagnosis of neuroblastoma and to obtain specimen for genetic marker studies. The biopsy was done using freehand technique, the arrow delineates the hiopsy needle. S, spleen M, mass...

Tumours of nerve cells (neuroblastoma). These are mainly extra-adrenal. 

---