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Glucagonoma syndrome is a rare entity defined by the existence of necrolytic migratory
erythema (NME), elevated serum glucagon levels, and an underlying alpha-cell secreting
tumor of the pancreas [
]. We present the case of a female with clinical manifestations of this syndrome. In
this paper, we describe the case of a 38-year-old woman referred to the dermatology
department to investigate pruritic lesions located on the lower extremity, with subsequent
extension to the axillae, groin, and intergluteal region, and pubis (Fig. 1). After several attempts, a skin biopsy specimen revealed histopathologic features
suggestive of NME. Further evaluations revealed the presence of 8 centimeters nonmetastatic
glucagonoma, located at the tail of the pancreas. 68Ga-DOTA TOC and an 18F-FDG PET
identified a mild uptake of the lesion (Fig. 1). The glucagon level was >500 pg/mL. The patient was diagnosed with glucagonoma syndrome,
and a caudal pancreatectomy was subsequently performed. Microscopy and the immunohistochemical
study were consistent with a glucagonoma (Fig. 2). After surgery, the cutaneous manifestation was resolved, with normalization of
glucagon levels. This uncommon case report adds information about an infrequent neuroendocrine
tumor with cutaneous expression. Early recognition and diagnosis must support by radiological
and nuclear medicine imaging tests [
Fig. 1Necrolytic migratory erythema in a patient with a glucagonoma. A close-up of waxing
and waning erythematous plaques, partly scaling with crusting areas (A). Sites affected
of the body: the dorsum of the foot (B) and axillary region (C). Computed Tomography
(D) shows a 8cm solid mass in the tail of the pancreas. The 18FFDG PET (E) and 68Ga-DOTA
TOC (F) revealed intense uptake of the lesion.
Fig. 2Skin biopsy revealing irregular acanthosis of the epidermis with loss of the stratum
granulosum and edema of the superficial portion of the epidermis (A). Hematic extravasation
and presence of abundant focal polynuclear cells alternating with areas of parakeratosis
(B). Histological study reveals a nodular tumor of 8 × 4.5 × 3.5 cm, located in the
tail of the pancreas with an intact capsular surface surrounded by adipose tissue
(C). In the sections performed, a proliferation of neuroendocrine cells is identified,
growing in an anastomosing, gyriform, pseudorosette trabecular pattern (D). Cells
with ample eosinophilic cytoplasm and enlarged nuclei with central macronucleolus.
Two mitoses are identified in 42 high magnification fields corresponding to less than
one mitosis per 2 mm2 (E). Cells show intense positivity for chromogranin (F) and
glucagon (G).
