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Necrolytic migratory erythema as a cutaneous manifestation of a pancreatic neuroendocrine tumor

      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 [
      • John A.M.
      • Schwartz R.A.
      Glucagonoma syndrome: a review and update on treatment.
      ]. 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 [
      • Jensen R.T.
      • Cadiot G.
      • Brandi M.L.
      • et al.
      ENETS Consensus Guidelines for the management of patients with digestive neuroendocrine neoplasms: functional pancreatic endocrine tumor syndromes.
      ]. Surgery is the initial treatment of choice.
      Fig. 1
      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. 2
      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).
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      References

        • John A.M.
        • Schwartz R.A.
        Glucagonoma syndrome: a review and update on treatment.
        J Eur Acad Dermatology Venereol. 2016; 30: 2016-2022
        • Jensen R.T.
        • Cadiot G.
        • Brandi M.L.
        • et al.
        ENETS Consensus Guidelines for the management of patients with digestive neuroendocrine neoplasms: functional pancreatic endocrine tumor syndromes.
        Neuroendocrinology. 2012; 95: 98