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Use of a somatostatin analog (SMS 201-995) in the glucagonoma syndrome
Abstract:
A long-acting somatostatin analog, SMS 201-995, is now available to treat the hormonal manifestations of islet cell tumors. We report its use in a patient with a metastatic glucagonoma refractory to conventional therapy. This patient, who was severely disabled by the rash of necrolytic migratory erythema and brittle diabetes mellitus, allowed us to evaluate the therapeutic efficacy of SMS 201-995 and to gain insight into the origin of the rash. SMS 201-995 was administered subcutaneously (.05 mg twice a day). The rash improved markedly within 48 hours and was completely resolved within 1 week of treatment. Insulin requirements decreased from 90 U/day to zero during the first week of treatment. Corresponding to improvement in clinical symptoms circulating glucagon levels showed a marked decrease. There was no substantial change in plasma or urinary levels of zinc or in plasma amino acid levels. When SMS 201-995 was stopped, the rash recurred within 36 hours and it improved within 48 hours of readministration. The rash and diabetes have remained well controlled during 8 months of therapy but no change in tumor size has been seen on CT scan. The rapid changes in the rash related to the administration of SMS 201-995 indicate that the pathogenesis of necrolytic migratory erythema is probably due to circulating hyperglucagonemia or some other hormonal substance produced by the tumor.
Insights
SMS 201-995, a somatostatin analog, effectively treated a metastatic glucagonoma patient refractory to conventional therapy. Treatment rapidly resolved necrolytic migratory erythema and brittle diabetes, suggesting hyperglucagonemia as the rash
Area of Science:
- Endocrinology
- Oncology
- Dermatology
Background:
- Islet cell tumors, particularly glucagonomas, can cause severe hormonal manifestations.
- Metastatic glucagonoma presents challenges in conventional treatment, often leading to debilitating symptoms like necrolytic migratory erythema and brittle diabetes mellitus.
Purpose of the Study:
- To evaluate the therapeutic efficacy of SMS 201-995 (a somatostatin analog) in a patient with metastatic glucagonoma.
- To gain insight into the pathogenesis of necrolytic migratory erythema associated with glucagonoma.
Main Methods:
- Subcutaneous administration of SMS 201-995 (0.05 mg twice daily) to a patient with metastatic glucagonoma.
- Monitoring of clinical symptoms (rash, diabetes), insulin requirements, circulating glucagon levels, and tumor size via CT scan.
- Assessing the impact of drug administration and withdrawal on symptoms.
Main Results:
- Marked improvement and complete resolution of necrolytic migratory erythema within 1 week of SMS 201-995 treatment.
- Reduction of insulin requirements from 90 U/day to zero within the first week.
- Significant decrease in circulating glucagon levels correlating with clinical improvement.
- Recurrence of rash upon drug cessation and rapid improvement upon readministration.
- No change in tumor size observed on CT scan during 8 months of therapy.
Conclusions:
- SMS 201-995 is effective in managing hormonal manifestations of metastatic glucagonoma, including necrolytic migratory erythema and brittle diabetes.
- The rapid response of the rash to SMS 201-995 suggests that hyperglucagonemia is a likely cause of necrolytic migratory erythema in glucagonoma.
- SMS 201-995 offers a therapeutic option for patients with glucagonoma refractory to conventional treatments.