Weekly Chest CasesArchive of Old Cases

Case No : 398 Date 2005-06-13

  • Courtesy of Hyo Lim Kim, M.D., Jung Im Jung, M.D. / St. Mary’s Hospital, College of Medicine, The Catholic Univesity of Korea
  • Age/Sex 52 / F
  • Chief ComplaintExcertional dyspnea for four months
  • Figure 1
  • Figure 2

Diagnosis With Brief Discussion

Diagnosis
Myxoma, Left Atrium
Radiologic Findings
Chest radiograph shows mild cardiomegaly and left cardiophrenic angle blunting, suggesting pleural effusion. Contrast?enhanced CT scan shows polypoid hypodense mass in the left atrium. The mass is attached to the interatrial septum. Small amount of pleural and pericardial effusions are combined.

Photograph of the gross specimen shows lobular pedunculated mass with multiple leaf like projections. Photomicrograph (HE stain) demonstrates nest of myxoma cells, and myxoid matrix. Immunohistochemical stain shows expression of calretinin of myxoma cells. Calretinin is expressed in about 75 % of cardiac myxoma.
Brief Review
Primary tumors of the heart are rare. Secondary malignant cardiac tumors, often arising in the lung and the breast, are at least 20 to 40 times more common than primary neoplasm. The majority of primary tumors are histologically benign.

Cardiac myxoma is the most common primary cardiac neoplasm, and accounts for approximately one-half of all primary cardiac tumors. Myxoma typically manifests as a polypoid, intracavitary left atrial mass (75 %) that arises from the interatrial septum, but it may originate in any cardiac chamber. Although most cases occur sporadically, familial lesions and lesions associated with a clinical complex have also been reported (carney complex).

Most affected patients present with at least one feature of a classically described triad that includes cardiac obstructive symptoms, constitutional symptoms (fever, malaise, weight loss, anemia, and elevated sedimentation rate), and embolic events.

Presenting complaints relate to tumor location, which predicts obstructive sequelae and influences embolic sites (pulmonary or systemic). Cardiac arrhythmias, atrial fibrillation, and atrial flutter reportedly occur in approximately 20% of patients with cardiac myxoma.

The histologic features of cardiac myxomas are distinctive but heterogeneous. The tumor cells form rings, nests, and linear syncytia. The amount of myxomatous stroma is widely variable. Hemorrhage, thrombus, and hemosiderin are common and calcification is also common.

Radiographic manifestations of 86 cases of cardiac myxoma were reviewed by Mary et al. Most patients with cardiac myxomas have abnormal but usually nonspecific radiographic findings. Radiographs of 38 patients with left atrial myxoma showed evidence of mitral valve obstruction in 53%; radiographs of 16 patients with right atrial myxoma demonstrated calcification and cardiomegaly in 56% and 50%. Other nonspecific manifestations such as cardiomegaly and pleural effusion occurred in 37% and 15% cases. One-third of all patients in this series had normal chest radiographic findings. CT demonstrates intracavitary heterogeneous, hypoattenuated (81%) lobular masses. MR imaging shows heterogeneous enhancing lesions and usually allows visualization of the point of attachment (78%). The differential diagnosis of an intracavitary cardiac mass includes thrombus and other much less frequent cardiac neoplasms, including sarcomas and papillary fibroelastomas. Left atrial thrombi are much more common than myxomas. They usually arise from the posterior and lateral atrial walls and from the atrial appendage. Left ventricular thrombi occur in regions of ventricular dyskinesia or aneurysm, and myxomas very rarely occur in this location.
References
1. Mary LG, Melissa LR, Curtis EG, Allen PB, and Jeffrey RG. From the Archives of the AFIP: Cardiac Myxoma: Imaging Features in 83 Patients. RadioGraphics 2002; 22: 673-689.

2. Michael JP, Samuel VL, G. Fraank OT. Primary Cardiac Tumor: Fourty Years’ Experience with 71 patients. Cancer 1997;79: 1809-1815.
Keywords
Vascular, heart, Benign tumor,

No. of Applicants : 38

▶ Correct Answer : 32/38,  84.2%
  • - Kosin University Bokum Hospital, Korea Seong Hyup Kim
  • - The Armed Forces Capital Hospital, Korea Seung Ho Kim
  • - Samsung Medical Center, Seoul, Korea Sun Young Jeong
  • - Kyungbook National University Hospital, Korea Kyung-Min Shin
  • - Yonsei University Hospital, Korea Yong Eun Chung
  • - Yonsei University Hospital, Korea Eun Hye Yoo
  • - Ewha Womans University Hospital, Korea Min-sun Lee
  • - Ewha Womans University Mokdong Hospital, Korea Yookyung Kim
  • - Seoul National University Hospital, Korea Dae Sik Kim
  • - Seoul National University Hospital, Korea Sang Min Lee
  • - Hangang SacredHeart Hospital, Korea Eil Seong Lee
  • - Soonchunhyang University Hospital, Cheonan, Korea Hae Seung Lee
  • - Hanyang University Hospital, Seoul, Korea Yo Won Choi
  • - Incheon Sarang Hospital, Incheon, Korea Jung Hee Kim
  • - Kuk-gun Jigu Hospital, Korea Joon Won Kang
  • - OBP, Russia Lepikhina Dasha
  • - Veterans General Hospital, Taipei, Taiwan Lin Wei-Chun
  • - Yonsei University Hospital, Korea Hye-Jeong Lee
  • - Ondokuz Mayis University, Samsun, Turkey Cetin Celenk
  • - Ross University, Portsmouth, Dominica Charles Ambelang
  • - Government Medical College, Vadodara, India Vijay Vaidya
  • - IRCCS S.Luca Hospital, Milano, Italy Filippo Casolo
  • - Annecy Hospital, France Gilles Genin
  • - Gwangmyoung Sungae Hospital, Korea Jiyong Rhee
  • - ASL Bologna, Maggiore Hospital, Bologna, Italy Marcellino Burzi
  • - Social Security Hospital, Ankara, Turkey Meric Tuzun
  • - Annemasse, Polyclinique de Savoie, France Gay-Depassier
  • - Homs National Hospital, Homs, Syria Rami Abou Zalaf
  • - CHU Besancon, France Sebastien Aubry
  • - Max Hospital, New Delhi, India Vickrant Malhotra
  • - Aditya Imaging Center, Gujarat, India Vivek Patel
  • - Ev. Krkhs. Hubertus, Berlin, Germany Michael Weber
▶ Correct Answer as Differential Diagnosis : 4/38,  10.5%
  • - Asan Medical Center, Seoul, Korea Hyun Ju Kim
  • - Busan Veterans Hospital, Korea Suhku Huh
  • - Ping Tung Christian Hospital,Taiwan Jun-Jun Yeh
  • - Radiologie Guiton, La Rochelle, France Denis Chabassiere
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