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Papers of the Week

2022 Apr 08

Am J Case Rep


Pancreatic Carcinoid Tumor in a Pediatric Patient.


Musalli D I, Binafif Y A, Mirdad AM, Moshref LH, Moshref RH
Am J Case Rep. 2022 Apr 08; 23:e936029.
PMID: 35393387.


BACKGROUND Carcinoid tumors are well-differentiated tumors that develop from neuroendocrine cells. They are rare tumors and occur most commonly in the gastrointestinal tract, followed by the pulmonary system. They usually present with abdominal pain or cough or persistent pneumonia. They are usually diagnosed with computed tomography (CT) or magnetic resonance imaging (MRI) of the chest or abdomen. The cornerstone of treatment is surgical resection. There are rare reported cases of carcinoid tumor presenting in the pancreas as gastrinoma or insulinoma and are associated with multiple endocrine neoplasia type 1 (MEN1). CASE REPORT We report a case of an otherwise healthy 9-year-old girl who presented with manifestations of Cushing syndrome (easy fatigability, weakness, back pain, easy bruising, hirsutism, acne, skin discoloration [pigmentation], and blurred vision). She was diagnosed with incidental carcinoid tumor in the pancreas based on hypertension and typical stigmata. She underwent distal pancreatectomy and splenectomy. The histopathology showed a well-differentiated neuroendocrine tumor with G2. The diagnosis of concurrent Cushing syndrome and carcinoid syndrome can be challenging, as it is rare. It is important to screen for MEN syndrome when Cushing syndrome occurs in a child, as there is a high rate of transition to malignancy. They are usually diagnosed with ACTH, cortisol, and imaging. CONCLUSIONS Incidental hypertension in children is not common and mandates further investigation and clinical work-up to look for endocrinopathies such as Cushing syndrome and carcinoid syndrome. As the literature on such cases is scant, further reporting of cases is needed.