LETTER TO THE E D I T O R S The Editors invite readers to submit letters commenting on the contents of articles that appear in the Journal. Also welcome are brief communications in letter form reporting investigative or clinical observations without extensive documentation and with brief bibliography (five titles or less), not requiring peer review but open to critique by readers. Letters to the Editors should be no more than 500 words in length and they may have to be edited for publication.

Efficacy o f corticosteroids in suppression o f intimal

Malignant fibrous histiocytoma o f the aorta

hyperplasia: A possible antiangiogenic effect

To the Editors: We wish to bring to your attention a case that will be of interest to your readers. A 73-year-old woman with a history of left subscapular pain of 8 weeks in duration underwent a diagnostic workup including chest x-ray, digital subtraction angiography of the aorta, and CT scanning of the chest. Then she was transferred to our hospital for resection of a chronically dissecting type B aortic aneurysm. On admission the patient was found to be somewhat anemic, but otherwise in good general health. Physical examination revealed a 2 over 6 systolic murmur heard best in the left third intercostal space adjacent to the sternum. With the exception of an eosinophilic sedimentation rate of 90, all laboratory parameters were within normal limits. It was assumed that the aneurysm had been perforated because the patient was experiencing intense thoracic pain. Consequently, surgical exploration was decidcd on. A coarse whitish tumor the size of a large chicken egg originating from the wall of the aorta 8 cm distal t o the origin of subclavian artery was noted. Frozen section showed a sarcoma, and a 20 mm blood vessel prothesis (Braun Uni-Graft DV) was applied in a end-to-side fashion proximal and distal to the minor-carrying portion of the aorta, which was resected. After operation ultrasonography of the abdomen, bone scanning, and CT scanning of the entire body showed no metastases, and the patient was discharged in good general condition. Grossly, the spindle-shaped, 4.5 × 4.0 × 3.5 cm nonencapsulated mass was situated in the wall of the aorta and extended preponderantly from the adventitia through the partly destroyed media to the intima with polypoid areas on the inner surface, The origin of some intercostal arteries were also involved and partly destructed. The lobdated cut surface appeared greyish-white with areas of hemorraghes. On histologic examination the tumor was characterized by a proliferation of plump spindle and pleomorphic cells arranged in short fascicles showing focally a typical storiform pattern. Sparse amounts of collagen fibers could be seen between the tumor cells and a modest numbers of lymphocytes. The polymorphic nuclei showed various numbers o f mitotic figures including atypical ones. Immunohistologic investigations with the AAPAP-method on fresh frozen and parafin embeded sections according to Cordell et al? gave the following results: Vimentin (V9) was strongly positive in most tumor cells. Immunostains for desmin (DE-Rll), neurofilaments (NR4), S100 protein, lymphatic cells (LC), and pankeratin marker K_L1, were completely negative, the antibody LU5 showed a

To the Editors: I have read with pleasure the article about a corticosteroid suppression ofintimal hyperplasia by Chervu et al? The authors' results support further my proposition to use antiangiogenesis ~ as a preventive treatment of restenosis after angioplasty? This proposition is based on the following facts: (a) interstitial fibrosis is a consequence of protracted angiogenesis and may be prevented by antiangiogenesis with corticosteroids and heparin, 4'2 (b) there is an essential similarity between interstitial and vascular intimal fibroses. In both cases, undifferentiated mesenchymal cells prc(Serate and differentiate into fibroblastic cells, ~ (c) antiproliferative antiangiogenic effect of corticosteroids is responsible for their antifibrotic efficacy?,~ Consequently, a preventive treatment by corticosteroids and heparin, the antiangiogenesis, should be able to prevent restenosis after angioplasty and other vascular intimal fibroses. One interesting question remains: what is the origin of undifferentiated mesenchymal cells in the intima? Do they derive from arterial endothelium 7 or from the vasa Ivasorum capillaries, which grow into the intima, s Both processes, being well documented, may take place. Jiri 7". Beranek, M D Division of Cardiothoracic Surgery Department of Surgery Wayne State University School of Medicine Detroit, MI 48201

REFERENCES 1. Chervu A, Moore WS, Quifiones-Baldrich WJ, Henderson T. Efficacy of corticosteroids in suppression of intimal hyperplasia. J VASCSURG 1989;10:129-34. 2. Beranek JT. Antiangiogenesis comes out of its shell. Cancer J 1988;2:87-8. 3. Beranek JT. Possible and np to now not exploited treatment of restenosis. Circulation (In press). 4. Beranek JT, Masseyeff R, Desmet VJ. Hyperplastic capillaries and their possible involvement in the pathogenesis of fibrosis. Histopathology 1986;10:543-51. 5. Orekhov AN, Andreeva ER, Krushinsky AV, et al. Intimal cells and atherosclerosis. Am J Pathol 1986;125:402-15. 6. Voss R, Mueller IR, Matthias FR. Effect of monocytopenia on trauma induced atherosclerotic lesions in the rabbit ear artery. Exp Mol Pathol 1988;49:75-86. 7. Altschtfl R. Endothelium. Its development, morphology, function, and pathology. New York: Macmillan Co, 1954. 8. Sarkisov DS, Kolokol'chikova EG, Varava BN, Printseva OY, Tyurin AV. Morphogenesis of thickenings of the intima observed in nonspecific aortoarteritis. Byulleten' t~ksperimental'noi Biologii i Meditsiny 1986;102:233-5.

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Letters to the Editors

Fig. 1.Histologic appearance of the tumor: distinct storiform growth pattern with strands of oval to elongated cells interspersed with a large number of small vessels. Partial destruction of the aortic wall in the outer third of the media, adventitia, and paraaortic tissue. Note islands of tumor cells squeezed together within the intima (T, intimal surface: I). (Hematoxylin-eosin stain; original magnification x 18.)

Fig. 2. Higher magnification of an area of the tumor tissue showing a pleomorphic aspect: multinucleated giant cells (GC), increased numbers of mitotic figures (M), and plump fibroblastic and round to oval histiocytic cells. (Hematoxylin-eosin stain; original magnification x 380.) focal expression, which is related to unspecific cross reaction in the paraffin embedded material. Type IV collagen (using the polyclonal antibody obtained from Medac) could be demonstrated in sliflike vessels, the individual tumor cells were not surrounded by collagen type IV. By use of cryostate sections the antibody Ki 67 revealed a proliferation fraction of about 20%.

The histologic and immunohistochemical findings led to the diagnosis of a malignant fibrous histiocytoma, storiform-pleomorphic subtype of the aorta. Five months after surgery the patient experienced rapidly progressing thoracic pain. CT scanning of the chest showed a local recurrence, measuring 5 to 9 cm, extending from the peak of the aortic arch paravertebraly to the dist.~l

Volume 11 Number 4 April 1990

graft anastomosis. In spite o f radiation therapy the patient died 7 months after operation. Autopsy showed no other primary lesion in a different location. Malignant tumors o f the blood vessels are found only rarely. Bleisch and Fredereck2 in 1980 discussed 60 cases of primary tumors o f the pulmonary root that had been described in literature. Tumors originating in the aorta are even rarer, 3 about 30 such cases have been described.4 Malignant fibrous histiocytomas usually occur in the extremities and in retroperitoneum, 5 occasionally in the lungs and mediastinum. Although it is rare for histiocytomas to develop in the aorta, they represent just over a quarter o f all aortic tumors because o f the extreme rarity o f aortic tumors in general. In conclusion, primary tumors o f the aorta were diagnosed in half o f the cases at autopsy. 6 Patients with fibrous histiocytoma o f the aorta on the other hand develop significant thoracic pain for which they seek medical attention, r All known cases were operated on with a false preoperative diagnosis o f aortic aneurysm. Because o f the rarity o f these tumors no standard therapy has been establisF ~.. In the published cases the involved aorta was either bypassed or replaced with a graft. In the present case the graft was applied as a bypass to avoid the additional risk o f paraplegia associated with cross-clamping o f the aorta. The effectiveness o f radiation or chemotherapy was not demonstrated in the therapy o f malignant fibrous histiocytomas. 5 The long-term prognosis o f patients with malignant fibrous histiocytoma o f the aorta is poor, underlined by the present case. Nevertheless, reasonable palliation can be achieved, at least for a limited time, through tumor resec-

Letters to the Editors 611

tion and replacement or bypass o f the affected portion of the aorta with a vascular graft. Onnen Grauhan, M D Uwe Thalmann, ME) Clinic for Cardiovascular and Thoracic Surgery German Heart Center Berlin Augustenburger Platz 1 1000 Berlin 65 Federal Republic o f Germany REFERENCES

1. Cordell J L, Falini B, Erber WN, et al. Irnmuno-enzymatic labelling of monoclonal antibodies using immune complexes of alkaline phosphatase and monoclonal anti-alkaline phosphatase (APA AP). J Histochem Cytochem 1984; 32:219-29. 2. Bleisch VM, Fredereck TK. Polypoid sarcoma of the pulmonary mink: analyses of the literature and report of a case. Cancer 1980;46:314-24. 3. Crawford ES. Aortic rumors. In: Diseases of the aorta. Baltimore: Williams and Wilkins, 1984:387-94. 4. Wenker JC, Becket Gj', Reilly MK, Tejada E, Cockerill EM. Malignant myxoid emboli in a patient with a primary tumor of the aorta. Virch Invest Radiol 1986;21:928-31. 5. Weiss SW, Enzinger FM. Malignant fibrous histiocytoma: an analysis of 200 cases. Cancer 1978;41:2250-5. 6. Chen KTK. Primary malignant fibrous histiocytoma of the aorta. Cancer 1981;48:840-4. 7. Guvendik L, Ross JK, Marshall RJ. Primary aortic malignant fibrous histiocytoma: a successfully treated case by surgical excision. Ann Thorac Surg 1986;42:578-80.