Ganglioneuroblastoma, say that ten times fast.

Today we had the biopsy and the surgery went well. Luke is recovering and will hopefully be home sometime within the week. Not so soon for the pathology reports. Some in-house, some sent out and some from the national cancer institute and data base. We were told probably early next week. We were told he has a ganglioneuroblastoma type tumor. Not the best and not the worst. Basically still a mystery. From what I’ve read, it affects about 5 cases out of 1,000,000 children. I;ve pasted some discriptions of the tumor here, kind of vague on treatment due to needing the pathology reports back. Chemotherapy it turns out is very specialized and the protocol to be followed needs to be based on the exact cell type that the tumor turns out to be. Luke also had a broviac http://en.wikipedia.org/wiki/Hickman_line inserted today, so no more “pokes”
Again, thanks for your prayers for this strong little boy.

Ganglioneuromas and ganglioneuroblastomas are tumors of the sympathetic nervous system that originate from neural crest sympathogonia, which are completely undifferentiated cells of the sympathetic nervous system. Along with neuroblastomas, ganglioneuromas and ganglioneuroblastomas are collectively known as neuroblastic or neurogenic tumors.1
Most frequently occurring in the abdomen, these tumors can grow wherever sympathetic nervous tissue is found. Common locations for ganglioneuromas and ganglioneuroblastomas include the adrenal gland, paraspinal retroperitoneum (sympathetic ganglia), posterior mediastinum, head, and neck; it is uncommon to find them in the urinary bladder, bowel wall, abdominal wall, and gallbladder.

Ganglioneuroblastomas (Luke’s diagnosis at this point)
Ganglioneuroblastomas are a mix of malignant neuroblastoma and benign ganglioneuroma tissues; they are sometimes called transitional tumors. These lesions also originate from sympathetic cells. Histologically, they are considered malignant because they contain primitive neuroblasts along with mature ganglion cells.
Ganglioneuroblastomas have a propensity for secreting catecholamines; approximately 90-95% actively secrete vanillylmandelic acid (VMA) and homovanillic acid (HVA). Catecholamine toxicity rarely results. HVA tends to be secreted by more mature and differentiated tumors, whereas VMA is usually a product of less differentiated tumors. In fact, the ratio of VMA to HVA secreted can be used as a prognostic factor to assess tumor maturity. In addition, more mature tumors may contain vasoactive intestinal peptide (VIP)–producing ganglion cells. Elevated levels of VIP can produce diarrhea, hypokalemia, and acidosis.
Despite these possible comorbidities, the prognosis for patients with ganglioneuroblastomas is relatively good. These tumors may spontaneously regress or mature. Maturation occurs at an unknown rate that eventually stops at the ganglioneuroma stage. In fact, all ganglioneuromas are thought to have once been, at an earlier stage of their development, ganglioneuroblastomas or neuroblastomas. Regression occurs in 1-2% of tumors; the cause of ganglioneuroblastoma regression is unknown.