Non oncologic elevated levels of chromogranin A in a symptomatic patient without a carcinoid tumor

Abstract

The diagnosis of neuroendocrine tumors requires a highly suspicious level due to its variable symptoms and clinical presentation. Early diagnosis, prompt and appropriate treatments are clues to success preventing the eventual disease progression and the devastating consequences of the carcinoid syndrome. Measurement of the chromogranin A, plays an important role in the diagnosis of carcinoid tumors, during the clinical evolution and even more to evaluate the treatment response to somatostatin analogues. However, in some situations like our case, the serum levels evaluation could be a confounding factor imitating the clinical scenario of the disease especially when the chromogranin level is elevated but not in the typical oncologic range and moreover when the symptoms magnitude is really convincing and similar to the tumor disease instances. Case ReportA 58-year-old Hispanic woman with non-past medical history and negative history of risk factors presented to the emergency department with one-day history of lower extremity edema, shortness of breath and tachycardia. She has a history of mammary prosthesis replacement 28 days ago. An initial diagnosis of pulmonary embolism was made; a pulmonary angio computed tomography (CT) was performed showing no vascular occlusion. Transthoracic echocardiogram has shown normal EF of 60% and no valvular disease. Coronary pharmacologic perfusion echocardiogram showed normal heart function and finally renal function was normal. The patient was treated for acute heart failure and discharged with ambulatory control. Six months later, upper gastric endoscopy was performed evidencing multiple polyps. Immediately after the procedure, the patient presented an episode of upper body flushing and an intense diffuse dull abdominal pain without rebound and tenderness. She concern that she had experienced explosive episodes of non bloody diarrhea, self limited episodes of dry cough, frequent episodes of flushing in the upper trunk and face with profuse sweating and intense pruritus lasting 15 minutes associated with significant unintentional weight loss over the last 6 month. Further workup included negative brain MRI, negative chest CT and abdominal MRI that only showed multiple benign liver cysts, (Figure 1) pancreatic and rectal endosonography were also normal. Somatostatin receptor scintigraphy (SRS) with Indium-111-DPA-octreotide was negative, capsule endoscopy negative for masses, 24-hour urine level of 5-HIAA appeared borderline 9.8 mg/24 hr (range levels 1.9-10.0 mg/24 hr) and plasma chromogranin A were above the reference range 92.0 ng/mL (range levels 1.9-15.0 ng/mL by electrochemilumiscent method) ( Table 1). An endoscopic polypectomy was done and histopathology reported hyperplasic polyps. Three months later, due to the persistent and more intense symptoms, a SRS with Indium-111-DPA-octreotide was repeated showing positive reactivity at the epigastric area and chromogranin A control persists elevated. The patient was placed on monthly intramuscular octreotide with dramati...