404
2013 Wiley Periodicals, Inc.
CASE REPORT ______________________________________________________________________
Cardiac Angiofibroma: A Rare Primary Cardiac Tumor Omar M. Issa, D.O.,* Javier Reyna, M.D.,y Orlando Santana, M.D.,y Steven DeBeer, M.D.,z Angelo LaPietra, M.D.,z and Joseph Lamelas, M.D.z *Division of Internal Medicine at Mount Sinai Medical Center, Mount Sinai Heart Institute, Miami Beach, Florida; yColumbia University Division of Cardiology, Mount Sinai Heart Institute, Miami Beach, Florida; and zDivision of Cardiac Surgery, Mount Sinai Heart Institute, Miami Beach, Florida ABSTRACT Cardiac angiofibromas are rare tumors. We report a patient with an angiofibroma of the mitral valve and discuss the management of these tumors. doi: 10.1111/jocs.12081 (J Card Surg
2013;28:404–405) Primary cardiac tumors are fairly uncommon.1,2 Most of these cardiac tumors are benign, with the majority being myxomas or papillary fibroelastomas. Cardiac angiofibromas comprise an extremely rare subset of these tumors. We present a 23-year-old female with this type of rare tumor. CASE REPORT A 23-year-old Hispanic female presented with a chief complaint of palpitations. The physical exam was significant for a faint murmur consistent with mitral regurgitation. A 24-hour Holter monitor demonstrated transient episodes of supraventricular tachycardia. A transthoracic echocardiogram revealed a 1 cm 1 cm smooth, round mass attached to the A2 portion of the anterior leaflet of the mitral valve. The decision was made to remove the left atrial mass via a minimally invasive approach. An intraoperative transesophageal echocardiogram confirmed the presence of the mass on the anterior leaflet of the mitral valve (Fig. 1). A femoral platform was utilized to establish cardiopulmonary bypass. A 5 cm right infra-mammary
Conflict of Interest: The authors acknowledge no conflict of interest in the submission. Funding: None. Address for correspondence: Orlando Santana, M.D., Director, Echocardiography Laboratory, Columbia University Division of Cardiology, Mount Sinai Heart Institute, 4300 Alton Road, Miami Beach, Florida 33140. Fax: 305-674-2368; e-mail:
[email protected]
incision was then made lateral to the anterior axillary line and carried down through 4th intercostal space into chest cavity. A soft tissue retractor and rib spreader were utilized to improve visualization. The pericardium was incised above and parallel to the phrenic nerve. An aortic cross clamp was applied directly through the incision and thereafter antegrade cardioplegia was delivered and readministered at 20 minutes intervals. A left atriotomy was preformed and the septum retracted with a specialized atrial lift retractor. The mitral valve was visualized and noted to have a wide based mass adherent to the atrial side of the A2 portion of the anterior leaflet. The mass was resected along with a portion of the surrounding anterior leaflet of the mitral valve. The resected area was replaced with an autologous pericardial patch. The atriotomy was closed and the heart was de-aired in the standard fashion. The patient was subsequently weaned from cardiopulmonary bypass. Decannulation and closure of the chest were performed in the routine fashion. The total cross clamp and cardiopulmonary bypass times were 55 and 95 min, respectively. The patient was transferred to the intensive care unit and extubated the same day. Her hospital course was uneventful, and she was discharged home on the third postoperative day. On gross pathologic examination, the resected mass was white, firm, and ovoid in shape, measuring 1 cm 1 cm 0.6 cm. Cut sections of the tumor revealed multiple spaces within the parenchyma. The microscopic examination demonstrated areas of collagen and myxoid tissue composed of spindle cells, all surrounding dilated vessels (Fig. 2). A CD31 stain highlighted the endothelial cells of these vessels supportive of the vascular nature of the lesion. The mass did not stain for calretinin, which is usually expressed in cardiac myxoma, essentially ruling this type of pathology. This histology was consistent with the diagnosis of an angiofibroma. DISCUSSION Primary cardiac tumors are fairly uncommon, with an overall incidence of approximately 0.02%.1,2 Most of these tumors are benign, with the majority consisting of myxomas or papillary fibroelastomas. Overall, cardiac angiofibromas are extremely rare. The data on angiofibromas involving the heart are limited to a few isolated case reports,3–6 two of which are in the Russian literature.5,6 One case report was that of a 35-year-old Caucasian male who presented with atrial fibrillation and was incidentally found to have a 23 mm 13 mm 7 mm mass attached to the subvalvular apparatus of the tricuspid valve.3 Another case report involved a 47-year-old female who presented with shortness of breath and edema of the lower extremities.4 She was found to have a fairly extensive angiofibroma extending from the right atrium to the inferior vena cava and the left renal vein. The tumor was successfully debulked and removed.
J CARD SURG 2012;28:404–405
ISSA, ET AL. CARDIAC ANGIOFIBROMA: A RARE PRIMARY CARDIAC TUMOR
405
Figure 1. (A) Intraoperative transesophageal echocardiogram showing a modified mid-esophageal four-chamber view, revealing the mass on the A2 portion of the mitral valve. (B) Mid-esophageal commissural view, showing different view of mass on the anterior leaflet of the mitral valve.
Figure 2. (A) Low (20) power H&E stain of the pathology specimen shows a circumscribed nodule primarily composed of dense pink acellular collagen and vascular spaces filled with blood. (B) On higher (100) power examination the endothelial lining of the vascular channels are visible, surrounded by a myxoid matrix and spindle cells.
Overall, angiomas are the skin manifestation of patients with tuberous sclerosis, usually presenting as multiple papules or nodules on the face.4 These tumors are almost always composed of fibroblasts set invariably in a collagenous or myxoid stroma.7 The other prominent feature is their vascular component, consisting of subtle variations in the wall size and branching pattern of the vessels that further distinguish this entity. Since there are only four case reports of these tumors in the literature, their long-term prognosis is not known, nor is there any evidence for malignant degeneration or recurrences of these tumors.
REFERENCES 1. Silverman NA: Primary cardiac tumors. Ann Surg 1980;191: 127–138.
2. Elbardissi AW, Dearani JA, Daly RC, et al: Survival after resection of primary cardiac tumors: A 48-year experience. Circulation 2008;118 (Suppl):S7–S15. 3. Jutley RS, Janas R, Matuszewski M, et al: Angiofibroma of the tricuspid valve: A rare presentation of the tuberous sclerosis complex? J Cardiovasc Surg (Torino) 2006; 47: 481–482. 4. Cao DB, Yang SR, Gao YS, et al: Angiofibroma in the right atrium and inferior vena cava. Eur J Cardiothorac Surg 2012;41:962. 5. Volkolakov IaV, Blascuez DT, Latsis RIa, et al: Surgical treatment of primary tumors of the heart. Grud Serdechnososudistaia Khir 1990 (2): 28–35. Russian. 6. Kirillov MM, Arkhangel’ski AV, Ivanovski GI, et al: Endocardial angiofibroma simulating a mitral heart defect. Klin Med (Mosk) 1986;64:120–123. Russian. 7. Marin˜o-Enrı´quez A, Fletcher CD: Angiofibroma of soft tissue: Clinicopathologic characterization of a distinctive benign fibrovascular neoplasm in a series of 37 cases. Am J Surg Pathol 2012;36:500–508.
