A RARE NONENDOCRINE TUMOR FROM ALPHA-2 CELLS OF PANCREATS WITH HIGH RATE OF THROMBOEMBOLIC PHENOMENON. NECROLYTIC MIGRATING ERYTHEMA IS THE MOST CHARACTERISTIC CLINICAL SIGN.
Medical Care
Currently, active drugs used to treat glucagonoma do not exist, although some drugs can cause partial regression of a neoplastic mass or improvements in the symptoms of NME.
" In the literature, good results are obtained with doxorubicin and streptozotocin (5-fluorouracil [5-FU] and streptozotocin) via selective damage of islets cells.
"
" Long-acting octreotide, analogous to human somatostatin, causes NME symptom regression in some patients and not in other patients.
"
" Remission of glucagonoma through treatment with dacarbazine is described in a single patient.
Surgical Care
Once a glucagonoma is identified, optimal management is surgical resection, which is the only curative therapy.
" In some patients, removal of the tumor may reverse symptoms.
"
" Patients with liver metastases and severe symptoms caused by tumor bulk or hormone-release syndromes may benefit from procedures that reduce hepatic arterial blood flow to metastases, including hepatic arterial occlusion with embolization or with chemoembolization that causes a necrosis of the metastases without damaging the healthy hepatic parenchyma, which is supplied from the portal circulation. This treatment may also be combined with systemic chemotherapy in selected patients.
"
" Beyond neoplasm removal, resect healthy surrounding parenchyma and locoregional lymph nodes, which can occasionally be metastatic or, more rarely, the primary site of the tumor.
"
" Several authors have reported clinical palliation of symptoms from surgical debulking of the tumor.
Diet
In patients with glucagonoma, providing a supplemental protein supply in order to furnish amino acids is useful. This can be administrated intravenously in the more severe cases. Essential fatty acids (ie, administering olive oil), zinc, vitamins, and minerals are also helpful.
Activity
Mild exercise is usually not harmful in patients with glucagonoma.
DRUG TREATMENT :
1. ANTISECRETORY AGENTS :
- OCTREOTIDE
2. ANTINEOPLASTIC AGENTS :
- DOXORUBICIN
- FLUOROURACIL ( 5-FLUOROURACIL )
- STREPTOZOCIN
- DACARBAZINE