My daughter was diagnosed with an extremly rare disese called Pancreatoblastoma in July of 2007. There seems to be very little information about Pancreatoblastoma and what little there is doesn't seem to get circulated.
I AM NOT A DOCTOR BUT I DO KNOW WHAT MY FAMILY'S EXPERIENCE HAS BEEN AND I AM WILLING TO PUT IT OUT THERE IN THE EVENT THAT THERE ARE OTHER FAMILIES DEALING WITH PANCREATOBLASTOMA (sorry for the redundancy of the pancreatoblastomas but I know how google searches work.
Pancreatoblastoma (infantile pancreatic carcinoma) is an extremely rare pancreatic tumor in children.
It mainly presents during childhood but can also occur in adults. It tends to be less aggressive in infants and children compared to adults. Children with pancreatoblastoma usually present late with upper abdominal pain. Obstruction of the upper duodenum and gastric outlet by tumor in the head of the pancreas may be associated with vomiting, jaundice and gastrointestinal bleeding.
The majority of these tumors arise in the head of the pancreas. Alpha-fetoprotein may be elevated. Ultrasound and CT scan may be useful but preoperative diagnosis is often quite difficult.
The treatment of choice is complete resection, that may often be curative. The role of adjuvant chemotherapy or radiotherapy is still under discussion due to small number of patients treated as yet.
Chemotherapy regimens consisting of cyclophosphamide, etoposide, doxorubicin, and cisplatin have been used with anecdotal benefit. Prognosis of this rare tumor is good, when resected completely.
Prognosis is poorer, when there is metastasis or when it is inoperable.
WHAT IS CONSIDERED INOPERABLE IS CHANGING!
On the whole, PB is regarded to be a curable tumor; hence the clinical diagnosis should be made early. Awareness of this rare tumor of pancreas is essential for early detection and proper management
Sunday, July 27, 2008
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