© 2014, Wiley Periodicals, Inc. DOI: 10.1111/echo.12789
Echocardiography
CASE REPORTS Section Editor: Brian D. Hoit, M.D.
Collagenous Fibroma of the Right Coronary Cusp: A Case Report and Literature Review Yanbo Zhu, M.S.,* Xiuhong Zhang, B.N.,† Xin Guan, M.S.,* and Lianqun Wang, M.D.‡ *Division of Ultrasound, Tianjin Chest Hospital, Tianjin, China; †Division of Nursing Management, Tianjin Children’s Hospital, Tianjin, China; and ‡Division of Cardiac Surgery, Tianjin Chest Hospital, Tianjin, China
Collagenous fibroma (desmoplastic fibroblastoma) is a rare benign tumor. This type of tumor mostly occurs in the subcutaneous tissues or skeletal muscle, and to the best of our knowledge, there is no unique case report of it occurring in the cardiac valves. This is the first description of collagenous fibroma in a human heart. (Echocardiography 2015;32:390–392) Key words: collagenous fibroma, aortic valve, coronary cusp, echocardiography Case Presentation: A 75-year-old female patient, who had been suffering from worsening intermittent precordium pain 3 days before admission, was referred to our hospital. She had a 6-year history of hypertension, a 15-year history of diabetes, and a 6-year history of cataract. On physical examination, her consciousness was clear, her heart rate was 86 beats/min with a regular rhythm and without murmur, and her blood pressure was 150/ 100 mmHg (1 mmHg = 0.133 kPa). Her electrocardiogram (ECG-1350C/P, Nihon Kohden Corporation, Tokyo, Japan) showed a QS wave in lead III, lowering of the T-wave in lead AVL, Twave inversion in lead V1, and ST segment depression for 0.1–0.2 mV in leads V2–V5. Her blood work was as follows: glutamic-oxalacetic transaminase 18.5 U/L, lactate dehydrogenase 277 U/L, creatine kinase 228 U/L, creatine kinase-MB 3.0 U/L, and a-hydroxybutyric dehydrogenase 180 U/L. Preoperative two-dimensional transthoracic (iE Elite, Philips Healthcare, Bothell, WA, USA) and intraoperative two-dimensional and real time three-dimensional transesophageal echocardiography (CX50, Philips Healthcare) examinations revealed an approximately 9 mm 9 7 mm medium echo mass on the right coronary cusp of the aortic valve that was inactively mobile with the opening and closing of the aortic valve; this echo was considered to be a fibrous mass on echocardiography (Fig. 1A–D). Cardiac-enhanced computed Address for correspondence and reprint requests: Xin Guan, M.S., Division of Ultrasound, Tianjin Chest Hospital, No. 261, Tai’erzhuang South Road, Jinnan District, Tianjin 300222, China. Fax: 0086-022-88185338; E-mail: [email protected]
390
tomography (CT) (Somatom Definition Flash, Siemens, Erlangen, Germany) showed a 6 mm 9 6 mm 9 7 mm soft tissue density nodule on the right coronary cusp tip without enhancement (Fig. 2). The coronary angiography showed triple vessel disease, with left coronary artery stenosis of 98%, right coronary artery stenosis of 80%, and left circumflex artery stenosis of 95%. The patient was prepared for coronary artery bypass graft and aortic tumor resection. The patient underwent a two-part surgery. In the first part (transaortic tumor resection), the operation was performed under normal temperature with the establishment of cardiopulmonary bypass. After dissection of the ascending aorta, the tumor was resected from the right coronary cusp and sent for histopathological examination (Fig. 3). The second part followed the first surgical procedure immediately by establishing three vein bypass grafts after her heart began rebeating automatically. Subsequently, the histopathological examination confirmed the diagnosis of collagenous fibroma (Fig. 4). Discussion: Collagenous fibroma is a rare, slowly progressive benign fibrous soft tissue tumor that usually presents without evidence of local infiltration. In 1995, Evans1 reported 7 cases, at which time this tumor type was called desmoplastic fibroblastoma. In 1996, Nielsen et al.2 renamed it as collagenous fibroma. Thus far, fewer than 130 cases have been reported in the literature. The disease can occur at any age; in the reported literature, the youngest patient was 5 years old and the oldest patient was 88 years old. It occurs in men more often than in women. The tumor’s
Cardiac Collagenous Fibroma
Figure 1. A. Preoperative two-dimensional transthoracic echocardiography (2DTTE) great artery short-axis view showing an approximately 9 mm 9 7 mm medium echo mass on the right coronary cusp of the aortic valve (white arrow). B. Preoperative 2DTTE left ventricular long-axis view. C. Intraoperative 2DTTE aortic root long-axis view. D. Intraoperative real time three-dimensional transesophageal echocardiography. LA = left atrium; ROVT = right ventricular outflow tract; AO = aorta; LV = left ventricle; RV = right ventricle; AAO = ascending aorta.
Figure 2. Cardiac-enhanced CT showing a 6 mm 9 6 mm 9 7 mm soft tissue density nodule on the right coronary cusp tip without enhancement (white arrow). LA = left atrium; AO = aorta; LV = left ventricle; RV = right ventricle.
anatomic distribution is wide, with head, neck, back, shoulders, arms, limbs, and dorsal foot being the most common affected areas,
Figure 3. Resected tumor from the right coronary cusp.
although it may also be found in glands.3 Nonetheless, to the best of our knowledge, it has not previously been found in the heart and the cardiac valves. The most common symptoms are 391
Zhu, et al.
Figure 4. Histopathological examination confirmed the collagenous fibroma.
local compression symptoms caused by the tumor. The clinical examination often includes xrays, CT, magnetic resonance imaging, and positron emission tomography,4,5 which are of great value in the diagnosis of the tumor’s location and type. Because this is a slow-growing, benign disease, operative excision is the best choice of treatment. There are currently no reports of metastasis or recurrence following surgical resection, and the longest reported follow-up period was 12 years. In our case, for a collagenous fibroma of the right coronary cusp in the aortic valve, echocardiography was the preferred method of examination. Following the echocardiography, which detected a medium echo mass on the aortic valve that was inactively mobile and small in volume, the color Doppler echocardiography determined that the tumor was not significantly effecting
392
cardiac hemodynamics. Three-dimensional echocardiography is helpful for observing the location and adjacent relations of the tumor; enhanced CT showed a low-density nodule in the aortic valve without apparent enhancement. Due to its special anatomic site, the differential diagnoses of the tumor included aortic valve aneurysm, aortic valve vegetation, aortic valve calcification and thrombosis, other types of tumors that typically occur in the aortic valve, and aortic sinus aneurysm. This was a benign tumor, and therefore, if the patient had shown no other cardiac diseases or obvious symptoms, and if there had been no valvular regurgitation or obstruction, the recommended management would have been a regular echocardiographic examination to observe the tumor size and to determine whether it has an impact on hemodynamics; there would have been no need for a surgical resection. Our patient was admitted to the hospital because of coronary atherosclerotic heart disease, and the tumor in the aortic valve was found by a routine echocardiographic examination; in this case, surgical removal was the best therapeutic option. References
1. Evans HL: Desmoplastic fibroblastoma. A report of seven cases. Am J Surg Pathol 1995;19:1077–1081. 2. Nielsen GP, O’Connell JX, Dickersin GR, et al: Collagenous fibroma (desmoplastic fibroblastoma): A report of seven cases. Mod Pathol 1996;9:781–785. 3. Zhang Z, Yang S, Xiu D: Unprecedented case of desmoplastic fibroblastoma in the pancreas. Pancreas 2011;40:313–315. 4. Okubo T, Saito T, Takagi T, et al: Desmoplastic fibroma of the rib with cystic change: A case report and literature review. Skeletal Radiol 2014;43:703–708. 5. Okubo T, Saito T, Takagi T, et al: Desmoplastic fibroma of the scapula with fluorodeoxyglucose uptake on positron emission tomography: A case report and literature review. Int J Clin Exp Pathol 2013;6:2230–2236.
Echocardiography
CASE REPORTS Section Editor: Brian D. Hoit, M.D.
Collagenous Fibroma of the Right Coronary Cusp: A Case Report and Literature Review Yanbo Zhu, M.S.,* Xiuhong Zhang, B.N.,† Xin Guan, M.S.,* and Lianqun Wang, M.D.‡ *Division of Ultrasound, Tianjin Chest Hospital, Tianjin, China; †Division of Nursing Management, Tianjin Children’s Hospital, Tianjin, China; and ‡Division of Cardiac Surgery, Tianjin Chest Hospital, Tianjin, China
Collagenous fibroma (desmoplastic fibroblastoma) is a rare benign tumor. This type of tumor mostly occurs in the subcutaneous tissues or skeletal muscle, and to the best of our knowledge, there is no unique case report of it occurring in the cardiac valves. This is the first description of collagenous fibroma in a human heart. (Echocardiography 2015;32:390–392) Key words: collagenous fibroma, aortic valve, coronary cusp, echocardiography Case Presentation: A 75-year-old female patient, who had been suffering from worsening intermittent precordium pain 3 days before admission, was referred to our hospital. She had a 6-year history of hypertension, a 15-year history of diabetes, and a 6-year history of cataract. On physical examination, her consciousness was clear, her heart rate was 86 beats/min with a regular rhythm and without murmur, and her blood pressure was 150/ 100 mmHg (1 mmHg = 0.133 kPa). Her electrocardiogram (ECG-1350C/P, Nihon Kohden Corporation, Tokyo, Japan) showed a QS wave in lead III, lowering of the T-wave in lead AVL, Twave inversion in lead V1, and ST segment depression for 0.1–0.2 mV in leads V2–V5. Her blood work was as follows: glutamic-oxalacetic transaminase 18.5 U/L, lactate dehydrogenase 277 U/L, creatine kinase 228 U/L, creatine kinase-MB 3.0 U/L, and a-hydroxybutyric dehydrogenase 180 U/L. Preoperative two-dimensional transthoracic (iE Elite, Philips Healthcare, Bothell, WA, USA) and intraoperative two-dimensional and real time three-dimensional transesophageal echocardiography (CX50, Philips Healthcare) examinations revealed an approximately 9 mm 9 7 mm medium echo mass on the right coronary cusp of the aortic valve that was inactively mobile with the opening and closing of the aortic valve; this echo was considered to be a fibrous mass on echocardiography (Fig. 1A–D). Cardiac-enhanced computed Address for correspondence and reprint requests: Xin Guan, M.S., Division of Ultrasound, Tianjin Chest Hospital, No. 261, Tai’erzhuang South Road, Jinnan District, Tianjin 300222, China. Fax: 0086-022-88185338; E-mail: [email protected]
390
tomography (CT) (Somatom Definition Flash, Siemens, Erlangen, Germany) showed a 6 mm 9 6 mm 9 7 mm soft tissue density nodule on the right coronary cusp tip without enhancement (Fig. 2). The coronary angiography showed triple vessel disease, with left coronary artery stenosis of 98%, right coronary artery stenosis of 80%, and left circumflex artery stenosis of 95%. The patient was prepared for coronary artery bypass graft and aortic tumor resection. The patient underwent a two-part surgery. In the first part (transaortic tumor resection), the operation was performed under normal temperature with the establishment of cardiopulmonary bypass. After dissection of the ascending aorta, the tumor was resected from the right coronary cusp and sent for histopathological examination (Fig. 3). The second part followed the first surgical procedure immediately by establishing three vein bypass grafts after her heart began rebeating automatically. Subsequently, the histopathological examination confirmed the diagnosis of collagenous fibroma (Fig. 4). Discussion: Collagenous fibroma is a rare, slowly progressive benign fibrous soft tissue tumor that usually presents without evidence of local infiltration. In 1995, Evans1 reported 7 cases, at which time this tumor type was called desmoplastic fibroblastoma. In 1996, Nielsen et al.2 renamed it as collagenous fibroma. Thus far, fewer than 130 cases have been reported in the literature. The disease can occur at any age; in the reported literature, the youngest patient was 5 years old and the oldest patient was 88 years old. It occurs in men more often than in women. The tumor’s
Cardiac Collagenous Fibroma
Figure 1. A. Preoperative two-dimensional transthoracic echocardiography (2DTTE) great artery short-axis view showing an approximately 9 mm 9 7 mm medium echo mass on the right coronary cusp of the aortic valve (white arrow). B. Preoperative 2DTTE left ventricular long-axis view. C. Intraoperative 2DTTE aortic root long-axis view. D. Intraoperative real time three-dimensional transesophageal echocardiography. LA = left atrium; ROVT = right ventricular outflow tract; AO = aorta; LV = left ventricle; RV = right ventricle; AAO = ascending aorta.
Figure 2. Cardiac-enhanced CT showing a 6 mm 9 6 mm 9 7 mm soft tissue density nodule on the right coronary cusp tip without enhancement (white arrow). LA = left atrium; AO = aorta; LV = left ventricle; RV = right ventricle.
anatomic distribution is wide, with head, neck, back, shoulders, arms, limbs, and dorsal foot being the most common affected areas,
Figure 3. Resected tumor from the right coronary cusp.
although it may also be found in glands.3 Nonetheless, to the best of our knowledge, it has not previously been found in the heart and the cardiac valves. The most common symptoms are 391
Zhu, et al.
Figure 4. Histopathological examination confirmed the collagenous fibroma.
local compression symptoms caused by the tumor. The clinical examination often includes xrays, CT, magnetic resonance imaging, and positron emission tomography,4,5 which are of great value in the diagnosis of the tumor’s location and type. Because this is a slow-growing, benign disease, operative excision is the best choice of treatment. There are currently no reports of metastasis or recurrence following surgical resection, and the longest reported follow-up period was 12 years. In our case, for a collagenous fibroma of the right coronary cusp in the aortic valve, echocardiography was the preferred method of examination. Following the echocardiography, which detected a medium echo mass on the aortic valve that was inactively mobile and small in volume, the color Doppler echocardiography determined that the tumor was not significantly effecting
392
cardiac hemodynamics. Three-dimensional echocardiography is helpful for observing the location and adjacent relations of the tumor; enhanced CT showed a low-density nodule in the aortic valve without apparent enhancement. Due to its special anatomic site, the differential diagnoses of the tumor included aortic valve aneurysm, aortic valve vegetation, aortic valve calcification and thrombosis, other types of tumors that typically occur in the aortic valve, and aortic sinus aneurysm. This was a benign tumor, and therefore, if the patient had shown no other cardiac diseases or obvious symptoms, and if there had been no valvular regurgitation or obstruction, the recommended management would have been a regular echocardiographic examination to observe the tumor size and to determine whether it has an impact on hemodynamics; there would have been no need for a surgical resection. Our patient was admitted to the hospital because of coronary atherosclerotic heart disease, and the tumor in the aortic valve was found by a routine echocardiographic examination; in this case, surgical removal was the best therapeutic option. References
1. Evans HL: Desmoplastic fibroblastoma. A report of seven cases. Am J Surg Pathol 1995;19:1077–1081. 2. Nielsen GP, O’Connell JX, Dickersin GR, et al: Collagenous fibroma (desmoplastic fibroblastoma): A report of seven cases. Mod Pathol 1996;9:781–785. 3. Zhang Z, Yang S, Xiu D: Unprecedented case of desmoplastic fibroblastoma in the pancreas. Pancreas 2011;40:313–315. 4. Okubo T, Saito T, Takagi T, et al: Desmoplastic fibroma of the rib with cystic change: A case report and literature review. Skeletal Radiol 2014;43:703–708. 5. Okubo T, Saito T, Takagi T, et al: Desmoplastic fibroma of the scapula with fluorodeoxyglucose uptake on positron emission tomography: A case report and literature review. Int J Clin Exp Pathol 2013;6:2230–2236.
