A heart gripping case: carcinoid heart disease.
Her symptoms worsened with new complaints of dysphagia along with continued vasomotor symptoms. She underwent a barium swallow showing a delay at the aortic arch and distal esophagus; repeat upper endoscopy and abdominal CT were both normal. Esophageal dilatation was performed and she was started on empiric medical treatment for esophageal spasms with a calcium channel blocker and as needed sublingual nitroglycerin. Her symptoms improved significantly with this medication regimen, but never totally resolved. Approximately one year later, she had a syncopal episode with a repeat unremarkable cardiac workup including electrocardiogram, transthoracic echocardiogram, and holter monitor, and the event was attributed to vasovagal syncope. Her hot-flashes, chest pain, and mid-epigastric pain continued intermittently with further workup unremarkable including a negative HIDA scan and negative repeat upper endoscopy. A gastric emptying scan did reveal significant delayed gastric emptying, but pro-motility agents failed to improve her symptoms.
Four years after her initial complaints of intermittent atypical chest pain, abdominal pain, and vasomotor symptoms, a repeat abdominal CT revealed an enlarging 2.3cm by 2.3 cm soft tissue mass noted within the mesentery adjacent to the duodenum with internal calcifications. Given the location of the tumor and concomitant vasomotor symptoms, the diagnosis of carcinoid syndrome was entertained. A subsequent serum chromogranin A was elevated at 52 ng/ml (reference normal <34 ng/ml) and a 24-hour urine 5-HIAA was elevated at 15 mg/24 hrs (reference range 0-6 mg/24 hrs). A PETCT showed uptake in the lesion of concern, as well as focal hypermetabolic activity at the cecum without a clear anatomic correlate. An octreoscan confirmed radiotracer uptake in the soft tissue mass adjacent to the duodenum, a new liver lesion with uptake, and a single focus of uptake within the mediastinum without a clear anatomic correlate. She underwent a right hemicolectomy with liver wedge resection. Pathology confirmed metastatic carcinoid tumor in the distal ileum, mesoappendix, and liver.
Within one month after resection, she developed recurrent chest pain, diarrhea, and hot-flashes. Repeat chromogranin A levels were 159.2 ng/ml. Further workup with a postoperative octreoscan showed a new liver lesion with radiotracer uptake and continued mediastinal uptake without a clear anatomic correlate. A gated cardiac CT showed a 1.5 cm soft tissue mass splaying the left anterior descending and the first diagonal branch coronary arteries, shown in Figures 1 and 2, with no discernible tissue plane between the mass and the ventricle. Cardiac MRI confirmed the mass at the bifurcation of these coronary arteries as well, with apparent continuity with the myocardium. She was evaluated by cardiothoracic surgery at our institution and this lesion was felt to be unresectable. She sought a second opinion at an outside institution and surgical resection of the tumor was performed. The patient noted immediate relief of her chest pain, flushing and diarrhea after the surgery and her chromogranin levels declined. She has been followed for one year since with serial PET scans and chromogranin levels without signs of definitive recurrence.
Carcinoid tumors are a heterogeneous group of neuroendocrine tumors whose clinical characteristics and behavior vary based on the primary site of origin, as each is derived from different precursor cells. Based on SEER data, most carcinoid tumors originate from the small bowel or appendix, and 80% of small bowel primary carcinoids are found in the ileum, as in our patient. (1) Given their location, small bowel carcinoid tumors commonly present with vague abdominal complaints as they must grow quite large to cause obstructive symptoms, and diagnosis is often delayed for years. Hence, the majority of patients present with metastases at diagnosis, most commonly to the liver and regional lymph nodes. Our patient presented in a similar fashion, with epigastric abdominal pain for several years prior to being diagnosed with metastatic disease.
Carcinoid syndrome, characterized by intermittent flushing, diarrhea and wheezing, is present in 5% to 7% of carcinoid tumors originating in the small bowel. (2) Carcinoid heart disease, occurring in more than 50% of patients with carcinoid syndrome, (3,4) usually manifests as right heart failure and valvular regurgitation secondary to valve and endocardial fibrosis. However, it is important to note cardiac metastases are included in the spectrum of carcinoid heart disease. Cardiac metastases are rare, though when they occur, carcinoid syndrome or metastatic disease to the liver is present a majority of the time. In our review of the literature, only slightly more than 20 cases of cardiac metastases were reported since 1980, the majority originating from the ileum. A case series of 74 patients with carcinoid by Pellika et al. found 3 (4%) patients had metastases to the heart, all of which originated from the ileum and were associated with carcinoid syndrome. (5) In another case series by Pandya et al, of 11 patients with myocardial metastases, 9 (82%) had ileal primary tumors, and all 11 had hepatic metastases and carcinoid syndrome. (6) Two other cases of cardiac metastases were found, both presenting as carcinoid syndrome and found to have small bowel primary tumors in the presence of extensive metastasis, including the heart. (7,8) Several cases of carcinoid heart disease without valvular involvement have been documented. Overall, these cases are similar to our case with respect to the extensive metastases and ileal primary, though this is one of only 2 cases presenting with angina.
Cardiac metastases can also occur in the absence of hepatic metastases as noted in 4 case reports, though only one originated from the small bowel. In one case, a carcinoid tumor of the heart was discovered incidentally on a CT scan for suspected pancreatitis and was subsequently found to have a pancreatic mass 6 months later, which was thought most likely to be the primary. (9) There were no manifestations of carcinoid syndrome or evidence of liver metastases. Another presented with symptoms of carcinoid syndrome and was found to have cardiac metastases along with a mass in the pancreatic head. (10) No hepatic involvement was discovered based on imaging and laparotomy findings. A third case involved a patient with known bronchial carcinoid, status post resection, who presented with symptoms of heart failure and was found to have diffuse infiltration of the myocardium with carcinoid tumor in the absence of valvular disease or hepatic metastases. (11) Another case is one of a patient with appendiceal carcinoid who developed cardiac arrest and at autopsy was found to have carcinoid infiltration of the a patient with myocardium. (12) One case describes carcinoid heart disease involving the interventricular septum, presenting with palpitations and paroxysmal atrial fibrillation. (13) Finally, one case describes a patient with metastatic carcinoid inducing coronary vasospasm with associated inferior ST-segment elevations. (14)
Based on the results of our literature review, cardiac metastases are most common in the presence of small bowel carcinoid tumors and hepatic metastases, though cardiac metastases without hepatic involvement have been demonstrated, and they may be more common than what is reported. It is also important to recognize cardiac metastases can occur in the absence of carcinoid syndrome, and as the sole manifestation of carcinoid heart disease. Furthermore, these metastases can cause symptoms of heart failure in the absence of valvular disease, cardiac arrest, arrhythmias, or angina by mass effect, as noted in our case. Hence, while cardiac metastases are rare in patients with carcinoid tumor, they should be considered in the differential for any patient presenting with cardiac-related complaints and a history of carcinoid tumor.
(1.) Modlin IM, Sandor A. An analysis of 8305 cases of carcinoid tumors. Cancer. 1997; 79:813-829.
(2.) Moertel CG, Sauer WG, Dockerty MB, Baggenstoss AH. Life history of the carcinoid tumor of the small intestine. Cancer. 1961; 14:901-912.
(3.) Lundin L, Norheim I, Landelius J, Oberg K, Theodorsson-Norheim E. Carcinoid heart disease: relationship of circulating vasoactive substances to ultrasound-detectable cardiac abnormalities. Circulation. 1988; 77(2):264-269.
(4.) Patel C, Moses M, Escarcega RO, Bove AA. Carcinoid heart disease: current understanding and future directions. Am Heart J. 2014; 167(6):789-795.
(5.) Pellikka PA, Tajik AJ, Khandheria BK, et al. Carcinoid heart disease. Clinical and echocardiographic spectrum in 74 patients. Circulation. 1993; 87:1188-1196.
(6.) Pandya UH, Pellikka PA, Enriquez-Sarano M, Edwards WD, Schaff HV, Connolly HM. Metastatic carcinoid tumor to the heart: echocardiographicpathologic study of 11 patients. J Am Coll Cardiol. 2002; 40:1328-1332.
(7.) Sivasankaran S, Sonn AT, Venesy DM, et al. Metastatic Cardiac Carcinoid. Tex Heart Inst J. 2007; 34:132-133.
(8.) Davis G, Birbeck K, Roberts D, Nagvi N. Nonvalvular myocardial involvement in metastatic carcinoid disease. Postgrad Med J. 1996; 72:751-752.
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(10.) Penz M, Kurtaran A, Vorbeck F, Oberhuber G, Raderer M. Case 2: myocardial metastases from a carcinoid tumor. J Clin Oncol. 2000; 18(7):1596-1597.
(11.) Goddard MJ, Atkinson C. Cardiac metastasis from a bronchial carcinoid: report of a case presenting with diffuse thickening of the left ventricular wall. J Clin Pathol. 2004; 57:778-779.
(12.) Patel S, Heetun M, Gurjar SV, Szakacs S. A rare case of intra-cardiac metastasis from an appendiceal carcinoid tumour without liver metastases. Int J Colorectal Dis. 2009; 24:993-994.
(13.) Yan AT, Gupta P. Deva D, Choi R, Kirpalani A. an unusual case of metastatic carcinoid tumor in the interventricular septum. J Cardiovascular Med. 2014 (Epub ahead of print).
(14.) Eapen D, Clements S, Block P, Sperling L. metastatic carcinoid disease inducing coronary vasospasm. Tex Heart Inst J. 2012; 39(1):76-78.
Capt Magulick is a Gastroenterology Fellow, San Antonio Uniformed Services Health Education Consortium, Brooke Army Medical Center, San Antonio, Texas.
Maj Flynt is Associate Program Director, Hematology/ Medical Oncology, San Antonio Uniformed Services Health Education Consortium, Brooke Army Medical Center, San Antonio, Texas.
LtCol Steel is Program Director, Cardiology, San Antonio Uniformed Services Health Education Consortium, Brooke Army Medical Center, San Antonio, Texas.
LTC Shumway is Program Director, Hematology/Medical Oncology, San Antonio Uniformed Services Health Education Consortium, Brooke Army Medical Center, San Antonio, Texas.
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|Author:||Magulick, John P.; Flynt, Frederick L.; Steel, Kevin E.; Shumway, Nathan M.|
|Publication:||U.S. Army Medical Department Journal|
|Article Type:||Case study|
|Date:||Jan 1, 2015|
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