A rare case of intra-cardiac metastasis from an appendiceal carcinoid tumour without liver metastases

Abstract

Metastatic tumours to the heart are more common than primary cardiac neoplasms and can occur in as many as 1.5% of patients with malignancies. The most common primary tumours that metastasise to the heart are breast, lung and malignant melanoma. The cardiac tissue most frequently affected is the myocardium, followed by the pericardium and then the endocardium. Metastatic tumours extending into the heart chambers and conducting system are less common. The most common route of metastasis is through lymphatic spread, followed by haematogenous dissemination. Carcinoid metastases to the heart are extremely rare, and only 19 cases have been described in the literature over the past 30 years. These invariably occur in the presence of liver metastases. We present a unique case of a patient who, at post-mortem, was found to have intra-cardiac carcinoid metastases involving a sensitive cardiac structure, in the absence of liver or any other metastases. A 51-year-old white woman presented with a 3-month history of central abdominal pain, nausea, lethargy and malaise. She had previously undergone lapararoscopic cholecystectomy and was prescribed hormone replacement therapy and anti-depressant medication. She denied a history of alcohol misuse, previous blood transfusions or risk factors for hepatitis. Clinical examination was unremarkable; liver function tests were shown to be abnormal (raised gamma-glutamyl transferase and alkaline phosphatase). Abdominal ultrasound showed an echogenic pattern suggestive of metastatic disease, with cirrhosis a less likely possibility. Abdominal and chest computed tomography scan revealed a small (<5 mm) lesion in the left lobe of the liver possibly representative of metastatic disease. Retro-peritoneal lymphadenopathy was also noted, with enlarged lymph nodes seen in the porta hepatis and para-aortic regions. A small right pleural effusion was also seen. No obvious primary lesion was identified. Upper and lower gastro-intestinal endoscopy were inconclusive for a primary neoplastic lesion. Her symptoms worsened with increased abdominal distension, shortness of breath and clinical ascites. Radiological biopsy of the paraaortic lesions was not feasible, and she proceeded to a diagnostic laparoscopy and biopsy. At laparoscopy, a mass lesion involving the appendix and terminal ileum with omental caking and peritoneal deposits was found. The procedure was converted to a laparotomy: An appendicectomy and palliative bypass were performed. A liver biopsy was also carried out. Histology revealed a carcinoid tumour in the appendix with infiltration into mesoappendiceal fat and evidence of perineural invasion. The liver wedge biopsy showed diffuse granulomatous inflammatory change but no evidence of malignancy. Two weeks after surgery, the patient suffered a sudden cardiac arrest. Cardiopulmonary resuscitation was performed, with restoration of pulse at 55 bpm but no blood pressure. An electrocardiogram showed left bundle branch block. The family declined any life-sustaining measures, and the patient passed away shortly afterwards. A post-mortem was carried out, which revealed the presence of Hodgkin’s lymphoma in both the liver and retroperitoneal nodes. No evidence of carcinoid involveInt J Colorectal Dis DOI 10.1007/s00384-009-0709-z