Malignant Fibrous Histiocytoma Arising From Descending Thoracic Aorta John R. Busby, MD, John L. Ochsner, MD, W. Brooks E m o r y , MD, Morris Flaum, MD, William Mitchell, MD, M a r k A. Farber, N e w Orleans, Louisiana
Cases of malignant fibrous histiocytoma arising from the aorta are rare and have a dismal outlook despite treatment. The longest reported survival period following resection is only 28 months. A patient with malignant fibrous histiocytoma of the descending thoracic aorta was successfully treated with resection and reconstruction with a prosthetic graft. The patient developed metastatic disease eight months postoperatively. With aggressive triple regimen chemotherapy, complete remission has been obtained. The patient is alive and free of disease six years postoperatively. (Ann Vasc Surg 1990;4:185-188). KEY WORDS:
Histiocytoma; aorta; arteries.
Primary sarcomatous tumors of the major blood vessels are rare. When they occur, primary growth elsewhere must be excluded before an artery or vein can be considered as the site of origin. Presentation varies from a simple tumor mass to acute vascular occlusion, false aneurysm formation, or peripheral embolization. Sarcomatous vascular tumors are found in both the arterial and venous systems. In the order of decreasing frequency, they occur in the inferior vena cava, other large veins, the p u l m o n a r y artery, and large systemic arteries [1]. T h e r e are three main t u m o r types: polypoid and intraluminal, intimal, and adventitial [I]. T h e s e tumors have a very high incidence of local recurrence and distant metastasis, and patients with these tumors have an extremely low three-year survival. Primary treatment of vascular s a r c o m a has been limited to wide local
From the Departments of Cardiovascular Surgery, Pulmonary Medicine, Hematology-Oncology, and Pathology, Ochsner Clinic and Alton Ochsner Medical Foundation, New Orleans, Louisiana. Reprints requests: John L. Ochsner, MD, Ochsner Clinic, 1514 Jefferson Itighway, New Orleans, Louisiana 70121.
excision. Adjunctive therapy with radiation or chem o t h e r a p y has not been helpful. We report a case of malignant fibrous histiocyt o m a arising f r o m the thoracic aorta. Our patient is one of the longest reported survivors following surgical resection who was successfully treated with c h e m o t h e r a p y following a recurrence.
CASE REPORT A 71-year-old white man was found to have an abnormal chest roentgenogram while being evaluated for atypical chest pains. Past medical history included hypertension and coronary artery disease with an ancient anteroseptal infarction. He denied any history of shortness of breath, dyspnea on exertion, hemoptysis, or weight loss. There was no past history of tobacco use. Physical examination was essentially normal except for mild hypertension. Blood pressure was 150/95 mmHg in the right and 150/90 mmHg in the left arm. Admission complete blood count, sequential multiple analyzer computer 20, urinalysis, and clotting studies were normal. Pulmonary functions showed a FVC of 4.0 liters and a FEV 1 of 3.1 liters. Arterial blood gas levels were normal. Chest x-ray films showed a lesion in the posterior aspect of the left chest (Fig. 1). A C T scan revealed a mass 185
186
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Fig. 3. Cross section through segment of resected aorta encased by neoplasm. Fig. 1. Lateral chest x-ray showing lesion in posterior position.
surrounding the aorta with lung fields clear of disease (Fig. 2). The diagnosis of carcinoma of the lung was entertained, and surgical removal was recommended. At surgery on May 18, 1982, a large mass was noted arising from the descending thoracic aorta. An en bloc resection of the mass and aorta was performed which required removal of an 8 cm segment of aorta. The patient received 10,000 units of heparin prior to placement of cross clamps. The aorta was replaced with a tightly woven Dacron graft in an end-to-end fashion. The cross clamp time was 18 minutes, and the patient received a heparin reversal dose of protamine upon completion of the anastomosis. He was discharged on postoperative day 10 after an uneventful postoPerative course. The pathological diagnosis was
Fig. 2. CT scan of chest showing lesion surrounding aorta.
malignant fibrous histiocytoma (Figs. 3 and 4). The Surgical Oncology Service suggested adjunctive therapy, but the patient refused. Six months following operation, he was evaluated because of persistent cough and left sided chest pain. Physical examination was normal, but a chest x-ray was abnormal (Fig. 5). Needle aspiration of the lung lesion revealed metastatic malignant fibrous histiocytoma. Complete blood count, sequential multiple analyzer 20, liver scan, and bone scan were normal. He began a triple drug regimen of chemotherapy consisting of dacarbazine 150 mg/m 2, doxyrubicin (Adriamycin) 30 mg/m 2, and cyclophosphamide (Cytoxan) 300 mg/m 2 and received a total of 15 cycles. The total dose of doxyrubicin was 435 mg/m 2. The chemotherapy was considered maximal and was administered over a year. The patient has been seen regularly in the Oncoiogy Clinic on a six-month basis. At his last visit on June 29, 1988, he was doing well with no evidence of recurrent disease (Fig. 6).
Fig. 4. Malignant fibrous histiocytoma showing pleomorphic cell population including multinucleated tumor cells (hematoxylin and eosin stain x 400).
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Fig. 5. Coned-down view of left chest showing metastatic lesion.
DISCUSSION O'Brien and Stout [2] first demonstrated malignant fibrous histiocytoma in 1964. In an extensive review by Weiss and Enzinger in 1978 o f o v e r 200 patients with malignant fibrous histiocytoma, not one case o f arterial involvement is mentioned [3]. There have been two subsequent reports o f cervical
carotid involvement with this tumor [4,5]. Malignant fibrous histiocytoma has been reported to involve the pulmonary artery [6-8]. In one patient, the tumor was noted to arise from the pulmonic valve with embolization resulting in pulmonary infarction [9]. T o date, six cases of malignant fibrous histiocytoma involving the aorta have been documented. Undoubtedly, some o f the sarcomatous lesions of the aorta reported prior to 1964 were malignant histiocytomas. Although the majority of patients with any type of primary tumor of the aorta are male [18], five o f the six patients reported with malignant fibrous histioc y t o m a o f the aorta are female. The age distribution is from 44 to 66 years, the same as that for other primary tumors o f the aorta. The location o f these tumors tends to favor the thoracic aorta with four o f six noted in this region. The apparently longest known survivor following resection o f an aortic tumor is described by Parussis [10]. This 44-year-old woman died 28 months following surgical resection. She had received radiation therapy 18 months after resection as an adjunctive therapy but later had metastatic disease. Guvendik and associates [13] reported a 48-year-old man free of disease, but his follow-up was only seven months. The malignant potential of malignant fibrous histiocytoma is very well documented. The rate of local recurrence is 44% and metastasis is 42%. Because there are no long-term survivors with malignant fibrous histiocytoma involving the aorta, surgical resection alone cannot be considered the sole therapy. Due to the paucity of these cases, the effectiveness of radiation or chemotherapy in the treatment of sarcomas of the aorta cannot be confirmed. More recent data suggest a significant increase in survival in patients with soft tissue sarcomas treated with combination therapy (surgery, radiation and chemotherapy) compared with single therapy alone [19]. Our experience with this single patient lends credence to the idea that combination treatment should be applicable to sarcomas o f the aorta and hopefully will improve long-term survival.
REFERENCES
Fig. 6. Follow-up chest x-ray showing resolution of metastatic disease.
1. SALM R. Primary fibrosarcoma of aorta. Cancer 1972; 29:73. 2. O'BRIEN JE, STOUT AP. Malignant fibrous xanthomas. Cancer 1964;17:1145-1148. 3. WEISS SW, ENZINGER FM. Malignant fibrous histiocytoma: an analysis of 200 cases. Cancer 1978;41:2250-2255. 4. KAPLAN BJ, DAY AL. Cervical carotid artery dissection secondary to malignant fibrous histiocytoma. Neurosurgery 1987;21:571-574. 5. HOSEMAN W, SCHEPKE P. Malignant fibrous histiocytoma as pulsating neck tumor---diagnosis using intraarterial digital subtraction angiography. H N O (Nurnberg) 1985;33: 380-382. 6. CARLIN BW, MOSER KM. Pulmonary artery obstruction
188
7.
8. 9.
10. 11. t2.
M A L I G N A N T FIBR 0 US HIS TIOC YTOMA
due to malignant fibrous histiocytoma. Chest 1987;92:173175. MISRA DP, SUNDERRAJAN EV, ROSENHOLTZ MJ, HURST DJ. Malignant fibrous histiocytoma in the lung masquerading as recurrent pulmonary thromboembolism. Cancer 1983;51:538-541. VAN DAMME H, VANDEERDEWEG W, SCHOOFS E. Malignant fibrous histiocytoma of the pulmonary artery. Ann Surg 1987;205:203-207. KERN SE, COWEN ME, ABRAMS GD. Malignant fibrous histiocytoma of the heart presenting as unilateral pulmonary thromboembolism and infarct. Hum Pathol 1985;16:12791281. PARUSSIS E. Primares malignes fibroses polymorphes histiozytom der aorta. Pathologe 1985;6:282-285. H E R M A N E K P, GENTSCH HH, SCHEELE J. Maligne tumoren der groBen GefaBe. Chirurg 1985;56:120-122. H E R M A N E K P, LANG M, RAITHEL D. Primares malignes fibroses histiozytoma der aorta. Deutsch Med Wochenschr 1983;42:1616-1617.
mmm
ANNALS OF VASCULAR SURGERY
13. G U V E N D I K L, ROSS JK, MARSHALL ILl. Primary aortic malignant fibrous histiocytoma: a successfully treated case by surgical excision. Ann Thorac Surg 1986;42:578-580. 14. WENKER JC, BECKER GJ, REILLY MK, TEJADA E, COCKERILL EM. Malignant myxoid emboli in a patient with a primary tumor of the aorta. Virch Invest Radiol 1986;21:928-931. 15. WEINBERG DS, MAINI BS. Primary sarcoma of the aorta associated with a vascular prosthesis. A case report. Cancer 1980;46:398-399. 16. CHEN KTK. Primary malignant fibrous histiocytoma of the aorta. Cancer 1981 ;48:840-844. 17. CRUM CP, FELDMAN PS, NOLAN SP. Primary fibroxanthosarcoma of the thoracic aorta. Virch Arch Pathot Anat 1978;1:37%381. 18. CRAWFORD ES. Diseases of the Aorta. Baltimore, Williams & Wilkins, 1984, pp. 387-394. 19. DEVITA JT, HELLMAN S, ROSENBERG SA. Cancer Principles and Practice of Oncology. Philadelphia, J.B. Lippincott Company, Vol. 2. 1985, pp. 1244-1291.
Cases of malignant fibrous histiocytoma arising from the aorta are rare and have a dismal outlook despite treatment. The longest reported survival period following resection is only 28 months. A patient with malignant fibrous histiocytoma of the descending thoracic aorta was successfully treated with resection and reconstruction with a prosthetic graft. The patient developed metastatic disease eight months postoperatively. With aggressive triple regimen chemotherapy, complete remission has been obtained. The patient is alive and free of disease six years postoperatively. (Ann Vasc Surg 1990;4:185-188). KEY WORDS:
Histiocytoma; aorta; arteries.
Primary sarcomatous tumors of the major blood vessels are rare. When they occur, primary growth elsewhere must be excluded before an artery or vein can be considered as the site of origin. Presentation varies from a simple tumor mass to acute vascular occlusion, false aneurysm formation, or peripheral embolization. Sarcomatous vascular tumors are found in both the arterial and venous systems. In the order of decreasing frequency, they occur in the inferior vena cava, other large veins, the p u l m o n a r y artery, and large systemic arteries [1]. T h e r e are three main t u m o r types: polypoid and intraluminal, intimal, and adventitial [I]. T h e s e tumors have a very high incidence of local recurrence and distant metastasis, and patients with these tumors have an extremely low three-year survival. Primary treatment of vascular s a r c o m a has been limited to wide local
From the Departments of Cardiovascular Surgery, Pulmonary Medicine, Hematology-Oncology, and Pathology, Ochsner Clinic and Alton Ochsner Medical Foundation, New Orleans, Louisiana. Reprints requests: John L. Ochsner, MD, Ochsner Clinic, 1514 Jefferson Itighway, New Orleans, Louisiana 70121.
excision. Adjunctive therapy with radiation or chem o t h e r a p y has not been helpful. We report a case of malignant fibrous histiocyt o m a arising f r o m the thoracic aorta. Our patient is one of the longest reported survivors following surgical resection who was successfully treated with c h e m o t h e r a p y following a recurrence.
CASE REPORT A 71-year-old white man was found to have an abnormal chest roentgenogram while being evaluated for atypical chest pains. Past medical history included hypertension and coronary artery disease with an ancient anteroseptal infarction. He denied any history of shortness of breath, dyspnea on exertion, hemoptysis, or weight loss. There was no past history of tobacco use. Physical examination was essentially normal except for mild hypertension. Blood pressure was 150/95 mmHg in the right and 150/90 mmHg in the left arm. Admission complete blood count, sequential multiple analyzer computer 20, urinalysis, and clotting studies were normal. Pulmonary functions showed a FVC of 4.0 liters and a FEV 1 of 3.1 liters. Arterial blood gas levels were normal. Chest x-ray films showed a lesion in the posterior aspect of the left chest (Fig. 1). A C T scan revealed a mass 185
186
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ANNALS OF VASCULAR SURGERY
Fig. 3. Cross section through segment of resected aorta encased by neoplasm. Fig. 1. Lateral chest x-ray showing lesion in posterior position.
surrounding the aorta with lung fields clear of disease (Fig. 2). The diagnosis of carcinoma of the lung was entertained, and surgical removal was recommended. At surgery on May 18, 1982, a large mass was noted arising from the descending thoracic aorta. An en bloc resection of the mass and aorta was performed which required removal of an 8 cm segment of aorta. The patient received 10,000 units of heparin prior to placement of cross clamps. The aorta was replaced with a tightly woven Dacron graft in an end-to-end fashion. The cross clamp time was 18 minutes, and the patient received a heparin reversal dose of protamine upon completion of the anastomosis. He was discharged on postoperative day 10 after an uneventful postoPerative course. The pathological diagnosis was
Fig. 2. CT scan of chest showing lesion surrounding aorta.
malignant fibrous histiocytoma (Figs. 3 and 4). The Surgical Oncology Service suggested adjunctive therapy, but the patient refused. Six months following operation, he was evaluated because of persistent cough and left sided chest pain. Physical examination was normal, but a chest x-ray was abnormal (Fig. 5). Needle aspiration of the lung lesion revealed metastatic malignant fibrous histiocytoma. Complete blood count, sequential multiple analyzer 20, liver scan, and bone scan were normal. He began a triple drug regimen of chemotherapy consisting of dacarbazine 150 mg/m 2, doxyrubicin (Adriamycin) 30 mg/m 2, and cyclophosphamide (Cytoxan) 300 mg/m 2 and received a total of 15 cycles. The total dose of doxyrubicin was 435 mg/m 2. The chemotherapy was considered maximal and was administered over a year. The patient has been seen regularly in the Oncoiogy Clinic on a six-month basis. At his last visit on June 29, 1988, he was doing well with no evidence of recurrent disease (Fig. 6).
Fig. 4. Malignant fibrous histiocytoma showing pleomorphic cell population including multinucleated tumor cells (hematoxylin and eosin stain x 400).
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Fig. 5. Coned-down view of left chest showing metastatic lesion.
DISCUSSION O'Brien and Stout [2] first demonstrated malignant fibrous histiocytoma in 1964. In an extensive review by Weiss and Enzinger in 1978 o f o v e r 200 patients with malignant fibrous histiocytoma, not one case o f arterial involvement is mentioned [3]. There have been two subsequent reports o f cervical
carotid involvement with this tumor [4,5]. Malignant fibrous histiocytoma has been reported to involve the pulmonary artery [6-8]. In one patient, the tumor was noted to arise from the pulmonic valve with embolization resulting in pulmonary infarction [9]. T o date, six cases of malignant fibrous histiocytoma involving the aorta have been documented. Undoubtedly, some o f the sarcomatous lesions of the aorta reported prior to 1964 were malignant histiocytomas. Although the majority of patients with any type of primary tumor of the aorta are male [18], five o f the six patients reported with malignant fibrous histioc y t o m a o f the aorta are female. The age distribution is from 44 to 66 years, the same as that for other primary tumors o f the aorta. The location o f these tumors tends to favor the thoracic aorta with four o f six noted in this region. The apparently longest known survivor following resection o f an aortic tumor is described by Parussis [10]. This 44-year-old woman died 28 months following surgical resection. She had received radiation therapy 18 months after resection as an adjunctive therapy but later had metastatic disease. Guvendik and associates [13] reported a 48-year-old man free of disease, but his follow-up was only seven months. The malignant potential of malignant fibrous histiocytoma is very well documented. The rate of local recurrence is 44% and metastasis is 42%. Because there are no long-term survivors with malignant fibrous histiocytoma involving the aorta, surgical resection alone cannot be considered the sole therapy. Due to the paucity of these cases, the effectiveness of radiation or chemotherapy in the treatment of sarcomas of the aorta cannot be confirmed. More recent data suggest a significant increase in survival in patients with soft tissue sarcomas treated with combination therapy (surgery, radiation and chemotherapy) compared with single therapy alone [19]. Our experience with this single patient lends credence to the idea that combination treatment should be applicable to sarcomas o f the aorta and hopefully will improve long-term survival.
REFERENCES
Fig. 6. Follow-up chest x-ray showing resolution of metastatic disease.
1. SALM R. Primary fibrosarcoma of aorta. Cancer 1972; 29:73. 2. O'BRIEN JE, STOUT AP. Malignant fibrous xanthomas. Cancer 1964;17:1145-1148. 3. WEISS SW, ENZINGER FM. Malignant fibrous histiocytoma: an analysis of 200 cases. Cancer 1978;41:2250-2255. 4. KAPLAN BJ, DAY AL. Cervical carotid artery dissection secondary to malignant fibrous histiocytoma. Neurosurgery 1987;21:571-574. 5. HOSEMAN W, SCHEPKE P. Malignant fibrous histiocytoma as pulsating neck tumor---diagnosis using intraarterial digital subtraction angiography. H N O (Nurnberg) 1985;33: 380-382. 6. CARLIN BW, MOSER KM. Pulmonary artery obstruction
188
7.
8. 9.
10. 11. t2.
M A L I G N A N T FIBR 0 US HIS TIOC YTOMA
due to malignant fibrous histiocytoma. Chest 1987;92:173175. MISRA DP, SUNDERRAJAN EV, ROSENHOLTZ MJ, HURST DJ. Malignant fibrous histiocytoma in the lung masquerading as recurrent pulmonary thromboembolism. Cancer 1983;51:538-541. VAN DAMME H, VANDEERDEWEG W, SCHOOFS E. Malignant fibrous histiocytoma of the pulmonary artery. Ann Surg 1987;205:203-207. KERN SE, COWEN ME, ABRAMS GD. Malignant fibrous histiocytoma of the heart presenting as unilateral pulmonary thromboembolism and infarct. Hum Pathol 1985;16:12791281. PARUSSIS E. Primares malignes fibroses polymorphes histiozytom der aorta. Pathologe 1985;6:282-285. H E R M A N E K P, GENTSCH HH, SCHEELE J. Maligne tumoren der groBen GefaBe. Chirurg 1985;56:120-122. H E R M A N E K P, LANG M, RAITHEL D. Primares malignes fibroses histiozytoma der aorta. Deutsch Med Wochenschr 1983;42:1616-1617.
mmm
ANNALS OF VASCULAR SURGERY
13. G U V E N D I K L, ROSS JK, MARSHALL ILl. Primary aortic malignant fibrous histiocytoma: a successfully treated case by surgical excision. Ann Thorac Surg 1986;42:578-580. 14. WENKER JC, BECKER GJ, REILLY MK, TEJADA E, COCKERILL EM. Malignant myxoid emboli in a patient with a primary tumor of the aorta. Virch Invest Radiol 1986;21:928-931. 15. WEINBERG DS, MAINI BS. Primary sarcoma of the aorta associated with a vascular prosthesis. A case report. Cancer 1980;46:398-399. 16. CHEN KTK. Primary malignant fibrous histiocytoma of the aorta. Cancer 1981 ;48:840-844. 17. CRUM CP, FELDMAN PS, NOLAN SP. Primary fibroxanthosarcoma of the thoracic aorta. Virch Arch Pathot Anat 1978;1:37%381. 18. CRAWFORD ES. Diseases of the Aorta. Baltimore, Williams & Wilkins, 1984, pp. 387-394. 19. DEVITA JT, HELLMAN S, ROSENBERG SA. Cancer Principles and Practice of Oncology. Philadelphia, J.B. Lippincott Company, Vol. 2. 1985, pp. 1244-1291.
