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Surgical treatment of primary malignant fibrous histiocytoma of the left atrium
463
Surgical treatment of primary malignant fibrous histiocytoma of the left atrium Manuel Concha ‘, Javier Pasalodos 2 and Fernando L. Rubio 3 I Department of Cardiovascular Surgery, ’ Department of Cardiology, and 3 Department of Pathology, Ciudad Sanitaria “Reina Sofia”, Cordoba, Spain (Received 1 February 1983; revision received 27 June 1983; accepted 6 July 1983)
A 30-year-old patient was operated on as an emergency because of a left atrial tumor. Microscopic and electron microscopfc examination of the tumor revealed it to be a myxoid variant of a malignant fibrous histiocytoma. The tumor subsequently recurred, and the recurrence was recognized by echocardiography. The patient died 21 months after initial operation. Case report A 30-year-old patient was admitted to our hospital in severe congestive heart failure. He had a history of progressive dyspnea for the 4 months before admission. The echocardiogram (Fig. 1A) showed, during diastole, a mass of compact and dense ethos between the mitral valve leaflets. The ethos moved posteriorly during systole. The presumptive diagnosis was an atria1 tumor, although most likely to be a myxoma, and the patient was sent for emergency operation. Opening the left atrium revealed a white mass, elastic in consistency, which was attached to the left atria1 wall. It occupied the entire atrial cavity without infiltrating the mitral valve. However, the tumor did infiltrate the posterior atria1 wall and the interatrial septum, having a multilobed appearance. The patient did well in the post-operative period, and the post-operative echocardiogram showed the mitral valve to be free of ethos. Pathologic findings
Microscopic. The tumor presented a peripheral zone with high cellular density and a central myxoid zone with low cellular density. The peripheral zone (Fig. 2A) bad a fibrohistiocytic appearance with marked pleomorphism. Electron microscopic. The ultrastructural images showed the presence of pleomorphic cells. Fibroblasts were the main component of the tumor. In the intercellular space we saw abundant collagen fibers with typical periods of 640 A (Fig. 2B). The histiocytes contained lipidic vacuoles, primary and secondary lysosomes, abundant Go@ apparatus and limited rough endoplasmic reticulum. The tumor was diagnosed as a malignant fibrous histiocytoma.
Reprint requests ro: Manuel Concha, M.D., Ciudad Sanitaria “Reina Sofia”, Avda. Menendez Pidal. 1, Cordoba, Spain. Inrernurronai Journal of Cardiology, 4 (1983) 463-466 0 Eisevier Science Publishers B.V.
464
Fig. 1A. Preoperative diastole.
echocardiogram.
The arrow
Fig. 1B. Follow-up echocardiography in the ninth systole (open arrow) in diastole (black arrow).
shows
month.
a mass
between
the mitral
It is very suggestive
leaflets
of recurrence
during
both
in
Fig. 2A. Microscopic
appearance
of the peripheral
zone of the tumor.
Fig. 2B. When examined with the electron microscope tumor. Collagen fibers (CF) are also seen. ( x 25 000)
fibroblasts
(Hematoxylin-eosin,
constitute
X 125)
the main component
of the
466
Follow-up Because of the malignant nature of the tumor, after a short period of convalescence, the patient was begun on chemotherapy with Adriamycin, DTIC-DOME and Vincristine. Echocardiographic follow-up was performed monthly. At the ninth month, a recurrence was observed (Fig. 1B). This was treated with cobalt therapy. Reoperation was not considered because of the malignant nature of the initial tumor and its infiltration of the atria1 wall. The patient died 21 months after operation. Discussion Primary cardiac tumors are rare and their clinical presentation variable according to their location. Between 17.8 and 26% of primary cardiac tumors are malignant [1,2]. Echocardiography is perhaps the most diagnostic technique and is the major reason for the increasing accuracy in their preoperative diagnosis [3]. Serial echocardiography is invaluable in assessing possible recurrence. Although there are no exact light microscopic criteria of the malignant character of fibrohistiocytic tumors [4], the large irregularities in nuclear shape, the size, number and shape of the nucleolus and the morphology of the cytoplasm are ultrastructural features which permit a firm diagnosis of malignancy. The predominance of myxoid areas permit us to include this tumor in the myxoid variant of fibrohistiocytomas. The surgical management of intracardiac tumors is well established. There are many reports of successful treatment of benign atria1 tumors. Success is less frequent when the tumor involves the right or the left ventricle, particularly if it is malignant. Indeed, the whole management of a primary malignant tumor of the heart is less clear. Gabelman et al. [5] reported repeated resections of a recurrent primary malignant fibrous histiocytoma. The patient died 3 years later. In our case surgical excision, chemotherapy and radiation therapy failed to control local recurrence, and he died 21 months after the initial operation. Acknowledgement The authors
thank Dr. Vaamonde
for the ultrastructural
study.
References Yater WM. Tumors of left heart and pericardium. Arch Intern Med 1931;48:627-637. Pritchard RW. Tumors of the heart. Arch Path01 1951;51:98-128. Bulkley BH, Hutchins GM. Atrial myxomas: a fifty year review. Am Heart J 1979;97:639-643. Steven I, Haidu MD. Pathology of soft tissue. Philadelphia: Lea and Febiger, 1979;105-121. Gabebnan CH, Al-Sadir J, Lamberti J, Foazard HA, Laufer E, Reploge RL, Myerowitz PD. Surgical treatment of recurrent primary malignant tumor of the left atrium. J Thorac Cardiovasc Surg 1979;77:914-921.
Surgical treatment of primary malignant fibrous histiocytoma of the left atrium Manuel Concha ‘, Javier Pasalodos 2 and Fernando L. Rubio 3 I Department of Cardiovascular Surgery, ’ Department of Cardiology, and 3 Department of Pathology, Ciudad Sanitaria “Reina Sofia”, Cordoba, Spain (Received 1 February 1983; revision received 27 June 1983; accepted 6 July 1983)
A 30-year-old patient was operated on as an emergency because of a left atrial tumor. Microscopic and electron microscopfc examination of the tumor revealed it to be a myxoid variant of a malignant fibrous histiocytoma. The tumor subsequently recurred, and the recurrence was recognized by echocardiography. The patient died 21 months after initial operation. Case report A 30-year-old patient was admitted to our hospital in severe congestive heart failure. He had a history of progressive dyspnea for the 4 months before admission. The echocardiogram (Fig. 1A) showed, during diastole, a mass of compact and dense ethos between the mitral valve leaflets. The ethos moved posteriorly during systole. The presumptive diagnosis was an atria1 tumor, although most likely to be a myxoma, and the patient was sent for emergency operation. Opening the left atrium revealed a white mass, elastic in consistency, which was attached to the left atria1 wall. It occupied the entire atrial cavity without infiltrating the mitral valve. However, the tumor did infiltrate the posterior atria1 wall and the interatrial septum, having a multilobed appearance. The patient did well in the post-operative period, and the post-operative echocardiogram showed the mitral valve to be free of ethos. Pathologic findings
Microscopic. The tumor presented a peripheral zone with high cellular density and a central myxoid zone with low cellular density. The peripheral zone (Fig. 2A) bad a fibrohistiocytic appearance with marked pleomorphism. Electron microscopic. The ultrastructural images showed the presence of pleomorphic cells. Fibroblasts were the main component of the tumor. In the intercellular space we saw abundant collagen fibers with typical periods of 640 A (Fig. 2B). The histiocytes contained lipidic vacuoles, primary and secondary lysosomes, abundant Go@ apparatus and limited rough endoplasmic reticulum. The tumor was diagnosed as a malignant fibrous histiocytoma.
Reprint requests ro: Manuel Concha, M.D., Ciudad Sanitaria “Reina Sofia”, Avda. Menendez Pidal. 1, Cordoba, Spain. Inrernurronai Journal of Cardiology, 4 (1983) 463-466 0 Eisevier Science Publishers B.V.
464
Fig. 1A. Preoperative diastole.
echocardiogram.
The arrow
Fig. 1B. Follow-up echocardiography in the ninth systole (open arrow) in diastole (black arrow).
shows
month.
a mass
between
the mitral
It is very suggestive
leaflets
of recurrence
during
both
in
Fig. 2A. Microscopic
appearance
of the peripheral
zone of the tumor.
Fig. 2B. When examined with the electron microscope tumor. Collagen fibers (CF) are also seen. ( x 25 000)
fibroblasts
(Hematoxylin-eosin,
constitute
X 125)
the main component
of the
466
Follow-up Because of the malignant nature of the tumor, after a short period of convalescence, the patient was begun on chemotherapy with Adriamycin, DTIC-DOME and Vincristine. Echocardiographic follow-up was performed monthly. At the ninth month, a recurrence was observed (Fig. 1B). This was treated with cobalt therapy. Reoperation was not considered because of the malignant nature of the initial tumor and its infiltration of the atria1 wall. The patient died 21 months after operation. Discussion Primary cardiac tumors are rare and their clinical presentation variable according to their location. Between 17.8 and 26% of primary cardiac tumors are malignant [1,2]. Echocardiography is perhaps the most diagnostic technique and is the major reason for the increasing accuracy in their preoperative diagnosis [3]. Serial echocardiography is invaluable in assessing possible recurrence. Although there are no exact light microscopic criteria of the malignant character of fibrohistiocytic tumors [4], the large irregularities in nuclear shape, the size, number and shape of the nucleolus and the morphology of the cytoplasm are ultrastructural features which permit a firm diagnosis of malignancy. The predominance of myxoid areas permit us to include this tumor in the myxoid variant of fibrohistiocytomas. The surgical management of intracardiac tumors is well established. There are many reports of successful treatment of benign atria1 tumors. Success is less frequent when the tumor involves the right or the left ventricle, particularly if it is malignant. Indeed, the whole management of a primary malignant tumor of the heart is less clear. Gabelman et al. [5] reported repeated resections of a recurrent primary malignant fibrous histiocytoma. The patient died 3 years later. In our case surgical excision, chemotherapy and radiation therapy failed to control local recurrence, and he died 21 months after the initial operation. Acknowledgement The authors
thank Dr. Vaamonde
for the ultrastructural
study.
References Yater WM. Tumors of left heart and pericardium. Arch Intern Med 1931;48:627-637. Pritchard RW. Tumors of the heart. Arch Path01 1951;51:98-128. Bulkley BH, Hutchins GM. Atrial myxomas: a fifty year review. Am Heart J 1979;97:639-643. Steven I, Haidu MD. Pathology of soft tissue. Philadelphia: Lea and Febiger, 1979;105-121. Gabebnan CH, Al-Sadir J, Lamberti J, Foazard HA, Laufer E, Reploge RL, Myerowitz PD. Surgical treatment of recurrent primary malignant tumor of the left atrium. J Thorac Cardiovasc Surg 1979;77:914-921.