Malignant Fibrous Histiocytoma of the Aorta Complicated by Anuria Juan Luis Fonseca, MD, In6s Fernandez-Valderrama, MD, Ricardo Gesto, MD, Paloma Laguna, MD, Rocio Merino, MD, Julio Rodriguez, MD, Guido Volo, MD, Teodoro Lazaro, MD, Madrid, Spain

Tumors of the aorta have been reported infrequently in the literature. We report a case of a 63-year-old woman diagnosed with malignant aortic fibrous histiocytoma (also known as fibroxanthosarcoma). She was referred to us with suspected occlusion of the right renal artery in a single functioning kidney, with a clinical picture of anuria during the previous 48 hours. We also review 31 previously published cases in the literature. (Ann Vasc Surg 1992; 6:164-167). KEY WORDS:

Fibrous histiocytoma; anuria; tumor.

Since Brodowski [1] described the first known case in 1873 only about 30 cases of primary tumors of the aorta have been published to date. Most of the diagnoses have been made after surgery [2] or necropsy [3]. Chemotherapy, radiotherapy or surgery have little effect on survival, although surgery Is often necessary to manage complications. Computed tomographic (CT) scan and arteriography images may suggest the diagnosis preoperatively and enable the surgeon to outline and appropriate plan or therapy.

CASE REPORT A 63-year-old woman presented with a two day history of general discomfort, nausea, and anuria+ Her past medical history was positive for diabetes mellitus, ischemic heart disease and arterial hypertension with chronic obstruction of her left renal artery. Previous renal func-

From the Servicio de Cirugia Vascular, Hospital Doce de Octubre, Madrid, Spain. Presented at the annual meeting of the Sociedad Espafiola de Angiologia y Cirugia Vascular--5th Congress of the European Chapter of the International Union of Angiology, May 30-31, June 1-2, 1990, Barcelona, Spain. Reprint requests: Teodoro Lazaro, MD, Servicio de Cirugia Vascular, Hospital Doce de Octubre, 28041 Madrid, Spain.

tion tests were normal. Physical examination revealed uremic fetor. Results from chest, heart, abdominal and vascular examinations were normal. The initial laboratory studies were: hemoglobin, 10 g/dl; white blood cell count, 13,000/cu mm; creatinine, 6.6 mg/dt; potassium, 7 mEq/L; sodium, 133 mEq/L Electrocardiogram showed sinus rhythm, Q wave in III and aVF, and pointed T waves in V2, V3 and V4. Chest and abdominal x-ray films were normal. Abdominal ultrasound ruled out obstructive uropathy and revealed normal kidneys, 10.5 cm (right) and 9 cm (left) in length. An arteriogram (Figs. 1, 2) showed changes in the wall of the infrarenal aorta, obstruction of the inferior mesenteric artery and a descending meandering artery. In addition, both renal arteries were occluded at their origins with distal opacification of their branches and preserved nephrograms. The patient underwent hemodialysis before being taken to the operating room. At laparotomy the periaortic retroperitoneal tissue showed minimal inflammatory reaction. The right renal artery was explored through a longitudinal arteriotomy, embolus-like material was removed from it, and the arteriotomy was closed by means of a vein patch angioplasty. When the infrarenal aorta was opened, the abnormal tissue was found to adhere intimately to the aortic wall, with no plane of cleavage and a broad implantation base: it was whitish in color and vegetative growth was observed. The infrarenal aorta, including the origin of the inferior mesenteric artery was resected en bloc and aortic continuity was established, using a straight Dacron graft. Exploration of the left renal

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Fig. 3. Histological detail showing infiltration of media and intima by cells growing in a disorderly fashion into lumen where they desquamate.

Fig. 1. Preoperative arteriogram showing obstruction of both renal arteries with vegetative-like repletion defect in infrarenal aorta.

artery confirmed patency 3 cm from its origin, and it was revascularized with a reverse saphenous vein bypass attached to the Dacron graft and then anastomosed endto-end to the distal left renal artery. Postoperatively, the patient remained anuric. A control arteriogram was performed showing patency of the left aortorenal bypass and thrombosis of the right renal artery. Given the patient's condition, revascularization was not considered advisable and hemodialysis was started. A thoracoabdominal CT scan ruled out significant proximal aortic disease. Recently, the patient suffered a significant stroke and, because of her terminal condition, voluntary discharge was requested by her family 29 days postoperatively. Histological analysis of the surgical samples obtained from the contents of the right renal artery and walls of the left renal and infrarenal aorta and inferior mesenteric arteries were provisionally reported as a mesodermal tumor and finally identified as a malignant fibrous histiocytoma (Figs. 3-5).

DISCUSSION

Fig. 2. Preoperative arteriogram showing distal revascularization of right renal artery and revascularization of lower mesenteric artery through the meandering artery.

To date, 32 cases of p r i m a r y aortic tumors have been published in addition to the case reported here. O f the 32 patients, 21 h a v e been males and 11 females, aged b e t w e e n 16 w e e k s and 75 years. Only three of t h e m had histological features of nonmalignancy (Table I). T h e r e was no particular affinity for any aortic segment, with the abdominal aorta affected in 14 cases, the thoracic aorta in 13 occasions, and the t h o r a c o a b d o m i n a l aorta in five. Tumors ( m e s o d e r m a l in all cases) were identified on the basis of their p r e d o m i n a n t cell type. I m m u n o h i s t o c h e m i c a l determinations p e r f o r m e d in our patient, obtained a positive reaction to Vi-

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ANNALS o r VASCULAR SURGERY

TABLE I.--Primary aortic tumors

Histological types Myxoma Fibromyxoma Fibromyosarcoma Myxosarcoma Sarcoma* Fusiform cell sarcoma Fibrosarcoma Angiosarcoma* Leiomyosarcoma Malignant fibrohistiocytoma*

No. of cases 1 2 3 2 4 1 6 4 2 7

References 4 5, 20 3, 21, 22 23, 24 15, 16, 26, 27 25 1, 10, 28-31 8, 9, 12, 16 13, 19 2, 6, 7, 11, 17, 32, present case

*In one c a s e associated with prosthesis

Fig. 4. Light microscopy showing polygonal cells mixed with fusiform cells with hypechromatic nuclei and some atypical mitoses, Hematoxylin-eosin stain (400x).

mentine antibodies and alpha-l-antitrypsin and alpha-l-antichemotrypsin type histiocyte markers. These showed anarchically distributed, polygonal "histiocyte-like" cells and fusiform cells, with nuclear atypias and images of atypical mitosis; cell groups were observed growing from the media and intima into the lumen and desquamating there while respecting the adventitia. Together with general cell morphology, the immunohistochemical findings enabled the tumor to be typed as a malignant fibrous histiocytoma. Irrespective of the histological features of malig-

Fig. 5. Stain using alpha-l-AT antibodies showing polygonal cells with nuclear atypias and atypical mitoses.

nancy or non-malignancy, the tendency towards intraluminal growth and subsequent fragmentation with seeding of emboli [4,5] makes these tumors particularly malignant. Pain, whether locally referable to the tumor mass [6,7], remote, as a result of obstructed blood flow (acute or chronic) to limbs and organs, or resulting from metastatic implants [8], has been the primary feature of the clinical picture in most cases. Other common forms of presentation have been constitutional syndrome [9,10] and arterial hypertension [5-11], which appeared in I0 cases. Skin nodes and distal ischemic lesions [12] indicating embolic metastatic processes have been described. Metastases at other levels are also frequent, mainly in bone, spleen, liver, pancreas, kidney, lungs and pleura [10-13]. Its extremely low incidence, unusual location in the aorta and varied forms of clinical presentation make malignant aortic fibrous histiocytoma very difficult to diagnose. However with increasing frequency it is possible to establish the diagnosis preoperatively, on the basis of the unique morphology demonstrated in the CT scan and arteriographic studies [6-8]. In our case, we observed the typical arteriographic findings in the form of filling defects. Computed tomographic scan performed in the postoperative period, once the histological diagnosis was known, has not enabled us to ascertain the origin of the material obtained from the right renal artery. We found no lesions at other levels which would lead us to suspect a primary tumor at a site other than that found at surgery. To the fact that no other tumor sites were found we must add the rarity of fibrous histiocytoma in territories that were not sufficiently examined [14]. The difficulty in establishing the diagnosis in certain cases is well known, although the biopsy was diagnostic in all the preoperative samples obtained from all the patients. Three cases of aortic tumors with nearby, previously implanted aortic prostheses have been diagnosed [11,15,16]. However, this association is of doubtful etiologic or pathogenic significance, partic-

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ularly if we consider the high number of prostheses implanted in the aorta and the few cases reported with this association. Chemotherapy and radiotherapy have been used in the treatment of the metastases [16,17] but have not been shown to be effective, although in one case a survival of two years was achieved [13]. Surgery was used to treat the primary tumor in 19 cases; the type of operation has varied according to tumor location and size, and to the preoperative suspicion of the diagnosis. The techniques used ranged from endarterectomy to bypass to excision en bloc and implantation of a prosthesis. However, irrespective of the technique, survival after surgery has been short with an operative mortality (first 30 days) exceeding 40%. All surviving patients, except one, died within the first year after surgery due to local recurrence of the tumor or metastasis [17-19]. One patient survived for four years, finally dying from metastatic disease.

CONCLUSIONS It seems clear that only a careful examination of the CT scan and arteriographic findings, together with the medical history, will enable this rare aortic tumor to be diagnosed. Elective surgery should probably be offered to patients without metastases and to those in whom a preoperative biopsy is compatible with a nonmalignant tumor.

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9. MASON MS, W H E E L E R JR, GREGORY RT, et al. Primary tumors of the aorta: report of a case and review of the literature. Oncology 1982:39:167-172. 10. SALM R. Primary fibrosarcoma of aorta. Cancer 1972;29: 73-83. 11. WEINBERG DS, MAINI BS. Primary sarcoma of the aorta associated with a vascular prosthesis: a case report. Cancer 1980;46:398--402. 12. W l N K E L M A N N RK, VAN HEERDEN JA, BERNATZ PE. Malignant vascular endothelial tumor with distal embolization. A m J M e d 1971 ;51:692--694. 13. HERNANDEZ FJ, STANLEY TM, RANGANATH KA, et al. Primary leiomyosarcoma of the aorta. A m J Surg Pathol 1979 ;3:251-256. 14. WEISS SW, ENZINGER FM. Malignant fibrous histiocytoma. An analysis of 200 cases. Cancer 1978;41:250-255. 15. O ' C O N N E L L TX, FEE HY, GOLDING A. Sarcoma associated with Dacron prosthetic material: case report and review of the literature. J Thorac Cardiovasc Surg 1976;72: 94-96. 16. FEHRENBACHER JW, BOWERS N, STRATE R, et al. Angiosarcoma of the aorta associated with a Dacron graft. Ann Thorac Surg 1980"32(3):297-301. 17. GRAUHAN O, T H A L M A N N U. Malignant fibrous histiocytoma of the aorta. J Vasc Surg 1990;11:609-611. 18. CRAWFORD ES. Aortic tumors. In: Disease o f the Aorta. Baltimore: Williams and Wilkins, 1984: pp 387-394. 19. MILLILI J, LAFLARE RG, NEMIR P. Leiomyosarcoma of the abdominal aorta: a case report. SurgeD' 1981 ;89:631634. 20. DETRIE D. Tumeur primitive intravasculaire de Faorte. J Chir (Paris) 1960;80:666-668. 21. ZEITHOFER J, HOLZENR JH, KREPLER P. Prim~ires fibromyosarkom der aorta. Kredsarzi 1963;18:25%269. 22. SMELOFF EA, REECE JM, MASTER JH. Primary intraluminal malignant tumor of the aorta. A m J Cardiol 1965;15107. 23. KARHOFF B. Primartumor der aorta. Zbi Allg Pathol 1952 ;839-846. 24. SILVERMAN JF, WEXLER L. Primary intraluminal tumor of the aorta. Radiology 1972;12:512-518. 25. A U F F E R M A N N H. Prim~ire aortengeschwulst mit eigentfimlichen riesenzellen. Z Krebsfach 1911;11:294-301. 26. SLADDEN RA. Neoplasia of aortic intima. J Clin Pathol 1964; 17:602-607, 27. STEFFELAAR JW, VAN DER H E U L RO, BLACKSTONE E, et al. Primary sarcoma of the aorta. Arch Pathol 1975 ;99:139-142. 28. KAIGORODOVA RE, BEREZOVSKAIA EK. Endotelioma grundnogo otdela aorty. Grudn Khir 1963;5:88-90. 29. KOVALEVEA AN, PRESS BO. A case of primary sarcoma of the intima of the aorta. Arch Pathol 1959;21:62-65. 30. MIURA M. Das prim~ire riesenzellensarkom der aorta thoracica, lnt Beitr Wissenth Med Festschr R Virchows 1981 ;2: 24%255. 31. NENCKI L. Zur kenntnis des prim~irtumoren der grossen gef~il3st~imme. Cardiology 1946;10:1. 32. CRUM CP, FELDMAN PS, NOLAN SP. Primary fibroxanthosarcoma of the thoracic aorta. Virchows Arch (A) 1978;379:351-358.

Malignant fibrous histiocytoma of the aorta complicated by anuria.

Tumors of the aorta have been reported infrequently in the literature. We report a case of a 63-year-old woman diagnosed with malignant aortic fibrous...
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