Glucagonoma and the glucagonoma syndrome (Review)

Abstract

Abstract. Glucagonoma is an extremely rare pancreatic α-islet cell tumor and is often accompanied by certain clinical symptoms including necrotizing migratory erythema (NME), diabetes, weight loss and anemia. The objectives of the current review were to discern the clinical features, diagnosis, treatment and prognosis of glucagonoma by evaluating 623 reported cases. A 1998 study reviewed 407 cases and 216 cases were reported in studies published after 1998. The current review consisted of 268 males and 339 females, with an average age of 52.4 years. The male-to-female ratio was 0.79. The incidence of typical clinical findings were as follows: NME, 82.4% (350/425); diabetes, 68.5% (291/425); weight loss, 60.2% (256/425); anemia, 49.6% (211/425); and glossitis or stomatitis or cheilitis, 41.2% (175/425). A total of 499 cases reported the location of the tumor as the pancreas and 64.1% (320/499) involved the pancreatic tail. Tumor size was recorded in 58.3% (126/216) cases reported after 1998 and average tumor size was 5.0 cm. Metastasis was detected in 49.2% of patients (293/595 for whom metastasis or no metastasis were recorded) upon diagnosis. These patients were older than those without metastasis (average age, 54.0 years old vs. 50.8 years old).

Manifestations of Glucagonoma Syndrome

“…The treatment of choice for glucagonoma is surgery given the poor response to chemotherapy. In cases of early diagnosis, surgical resection could be curative (6)(7)(8). The morbidity of GS is mostly due to NME, which, as it did in our case, frequently produces extensive lesions that affect the quality of life of the patient.…”

“…NME is the most characteristic clinical manifestation of the disease and is present in up to 70% of cases (4,6,8,9). Often, as in this case, NME is the first manifestation of the disease.…”

“…Necrolytic erythema can improve up to 50% with somatostatin analogs, amino acid infusion, and antibiotics (6,7). There are other treatment options such as chemoembolization and radiotherapy (8). In 2 case series with >20 patients in each, the response to chemotherapy was up to 50% (10,11).…”

Glucagonoma: From skin lesions to the neuroendocrine component (Review)

“…A study published in 2018 on 623 cases of glucagonoma revealed a mean age of 52.4 years and men to women ratio of 0.79 (27). Their presentation included most frequently NME of 82%, followed by diabetes mellitus 68%, weight loss 60%, anaemia in almost half of the subjects, and glositis/stomatitis/cheilitis of 41% (27).…”

“…A study published in 2018 on 623 cases of glucagonoma revealed a mean age of 52.4 years and men to women ratio of 0.79 (27). Their presentation included most frequently NME of 82%, followed by diabetes mellitus 68%, weight loss 60%, anaemia in almost half of the subjects, and glositis/stomatitis/cheilitis of 41% (27). Almost two thirds of the glucagonomas were located at pancreas tail while a mean tumour diameter was 5 cm, and almost half of the patients had liver metastasis at first diagnosis (especially in older persons) (27).…”