Glucagonoma with necrolytic migratory erythema exhibiting responsiveness to subcutaneous octreotide injections

Lo, Chang-Han; Ho, Ching‐Liang; Shih, Yu‐Lueng

doi:10.1093/qjmed/hct027

Cited by 8 publications

(6 citation statements)

References 2 publications

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“…However, first‐line tools are ultrasonography and computed tomography (CT) due to lower cost and widespread availability. Other methods to identify the tumour are positron emission tomography (PET), magnetic resonance imaging (MRI), octreotide scintigraphy and somatostatin receptor scintigraphy . Calcifications on pancreatic CT are commonly found with glucagonomas and can indicate the presence of lymph node metastases …”

Section: Differential Diagnosismentioning

confidence: 99%

Glucagonoma syndrome: a review and update on treatment

John

Schwartz

2016

Acad Dermatol Venereol

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Glucagonoma syndrome is defined by the presence of an alpha-cell secreting tumour of the pancreas, elevated levels of glucagon, and a characteristic rash called necrolytic migratory erythema (NME). NME is usually a specific and often initial finding of glucagonoma syndrome, but it may occur in other settings unassociated with an alpha-cell pancreatic tumour (pseudoglucagonoma syndrome). Glucagonoma syndrome must be distinguished from pseudoglucagonoma syndrome. Prompt recognition of NME and subsequent workup for a glucagonoma can allow for an earlier diagnosis and enhance the chances of a favourable outcome. In particular, metastases occur late, so early recognition of glucagonoma syndrome before liver metastases can be life-saving. Surgical resection is the definitive treatment for glucagonoma syndrome, although chemotherapeutic agents, somatostatin analogues and radionuclide therapy are also employed. Herein, we offer an approach to workup after identifying NME and an update on its current treatment modalities.

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Section: Differential Diagnosismentioning

confidence: 99%

Glucagonoma syndrome: a review and update on treatment

John

Schwartz

2016

Acad Dermatol Venereol

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“…Those with hormonal-based symptoms are often well controlled through somatostatin analog (SSA) administration. [20][21][22][23][24] Present efforts have also shown that octreotide…”

Section: Discussionmentioning

confidence: 98%

Diagnosis and treatment of pancreatic glucagonoma presenting with multiple metastases and recurring postoperatively: Report of two patients

Zhang

et al. 2022

Preprint

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Background: Pancreatic glucagonoma is a rare neuroendocrine neoplasm with malignant potential. Its etiology is unclear, and its course is protracted, often fraught by recurrences and greatly diminished quality of life. Case presentation: Herein, we report two patients with such tumors, one a 60-year-old woman hospitalized for blood glucose elevation and recurrent necrolytic migratory erythema (NME) of the trunk and limbs. Imaging studies showed an area (~3 cm) of reduced enhancement at head of pancreas. Examination of the subsequent pancreaticoduodenectomy specimen confirmed a pancreatic neuroendocrine tumor (G2). The patient’s blood glucose normalized postoperatively, and her skin erythema cleared. During follow-up, however, intramuscular injections (30 mg) of long-acting release (LAR) octreotide acetate microspheres were required at 28-day intervals for recurrent NME; and there was apparent hepatic and retroperitoneal nodal involvement after 36 months. The second patient was a 32-year-old woman hospitalized for recurrent glossitis, perioral dermatitis, and NME of both lower limbs. Imaging studies revealed a low-density nodule (~2 cm) in tail of pancreas, as well as multiple space-occupying lesions of liver. Distal pancreatectomy was undertaken, with splenectomy and palliative resection of hepatic nodules, confirmingmetastasis of a primary pancreatic neuroendocrine tumor (G2) to liver.The NME resolved postoperatively, but this patient also received intramuscular octreotide LAR injections (30 mg) at 28-day intervals. After 6 months, percutaneous radiofrequency ablation of residual liver lesions took place. Conclusions: The present accounts detail our diagnostic and therapeutic experiences with this rare type of tumor, especially the management of multiple metastases upfront and postoperative recurrences.

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“…Those with hormonal-based symptoms are often well controlled through somatostatin analog (SSA) administration. [37][38][39][40] Present efforts have also shown that octreotide LAR is an important and effective long-term treatment in this setting, although the scienti c merit attributed to so few cases may be debated.…”

Section: Discussionmentioning

confidence: 99%

Diagnosis and comprehensive treatment of pancreatic glucagonoma presenting with multiple metastases and recurring postoperatively

Zhang

et al. 2023

Preprint

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Background: Pancreatic glucagonoma is a rare neuroendocrine neoplasm with malignant potential. At present, clinicians have a poor understanding of the disease, and the current epidemiology, diagnosis and comprehensive treatment of the disease have not been fully established. Case presentation: Herein, we report two patients with such tumors, one a 60-year-old woman hospitalized for blood glucose elevation and recurrent necrolytic migratory erythema (NME) of the trunk and limbs. Imaging studies showed an area (~3 cm) of reduced enhancement at head of pancreas. Examination of the subsequent pancreaticoduodenectomy specimen confirmed a pancreatic neuroendocrine tumor (G2). The patient’s blood glucose normalized postoperatively, and her skin erythema cleared. During follow-up, however, intramuscular injections (30 mg) of long-acting release (LAR) octreotide acetate microspheres were required at 28-day intervals for recurrent NME, and there was apparent hepatic and retroperitioneal nodal involvement after 36 months. The second patient was a 32-year-old woman hospitalized for recurrent glossitis, perioral dermatitis, and NME of both lower limbs. Imaging studies revealed a low-density nodule (~2 cm) in tail of pancreas, as well as multiple space-occupying lesions of liver. Distal pancreatectomy was undertaken, with splenectomy and palliative resection of hepatic nodules, confirming metastasis of a primary pancreatic neuroendocrine tumor (G2) to liver. The NME resolved postoperatively, but this patient also received intramuscular octreotide LAR injections (30 mg) at 28-day intervals. After 6 months, percutaneous radiofrequency ablation of residual liver lesions took place. Conclusions： In patients with multiple liver metastases, palliative surgical treatment may confer patient benefits and prolong survival. Those with hormonal-based symptoms are often well controlled through somatostatin analog (SSA) administration. postoperative adjuvant therapy of metastatic pancreatic glucagonoma has also obvious effects on the prognosis.

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Glucagonoma with necrolytic migratory erythema exhibiting responsiveness to subcutaneous octreotide injections

Cited by 8 publications

References 2 publications

Glucagonoma syndrome: a review and update on treatment

Glucagonoma syndrome: a review and update on treatment

Diagnosis and treatment of pancreatic glucagonoma presenting with multiple metastases and recurring postoperatively: Report of two patients

Diagnosis and comprehensive treatment of pancreatic glucagonoma presenting with multiple metastases and recurring postoperatively

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