Malignant vascular endothelial tumor with distal embolization: A new entity

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  • Malignant Vascular Endothelial Tumor with Distal Embolization

    A New Entity

    R. K. WINKELMANN, M.D., Ph.D.

    JONATHAN A. VAN HEERDEN,, IM.B.,

    Ch.B. (Cape Town), F.R.C.S. (C)*

    PHILIP E. BERNATZ, M.D.

    Rochester, Minnesota

    From the Mayo Clinic and Mayo Founda- tion, Department of Dermatology and Anatomy, and of Surgery, and from the M.ayo Graduate School of Medicine, Uni-

    versity of Minnesota, Rochester, Minne- sota. Requests for reprints should be ad- dressed to Dr. R. K. Winkelmann, Mayo Clinic, Rochester, Minnesota 55901. Man- uscript received October 26, 1970.

    * Present address: Winchester Surgical Clinic, P.O. Box 854, Winchester, Virginia 22601.

    An unusual cause of major arterial obstruction-a vascular endothelial tumor causing aorto-iliac stenosis and distal vascu- lar insufficiency by presumed embolization-is described. In the case reported, there are three distinctive features which imply that this rare syndrome should be recognized as a pattern of vascular disease. (1) There was focal proliferation of cells, similar to neoplastic reticular cells, associated with the endo- thelium of a large vessel. (2) Multiple embolization occurred to peripheral arteries, resulting in gangrene and cutaneous in- flammation. (3) The lesions were recognized in the two levels of the vascular system by use of angiographic technics and skin biopsy.

    The usual causes of obstructive disease at the aortic bifurca- tion are advanced atherosclerosis, with or without superimposed thrombosis, and saddle embolus, which is usually of intra- cardiac origin.

    Distal embolization to the extremities is not uncommon. Its basis is usually atherosclerotic vascular disease, cholesterol emboli, mural thrombi and rarely, fragmentation of intracardiac tumors. Intravascular foreign bodies (for example, bullets) have also, on occasion, embolized distally after entering a large central vessel.

    We recently saw a patient who had a massive reticular or endothelial cell proliferation of the abdominal aorta from which multiple emboli to the extremities induced gangrene; we be- lieve that this is a distinct clinical entity related to other intra- vascular reticuloendotheliomas. Among the rare cases of pri- mary aortic tumor are two similar to ours: Sladdens Case 1 [l] and the case of Staemmler [2]. We believe this syndrome is distinctive and deserves clinical recognition.

    692 The American Journal of Medicine

  • MALIGNANT VASCULAR ENDOTHELIAL TUMOR - WINKEL MANN IET AL.

    CASE REPORT

    A fifty-six year old whtte man was seen at the Mayo

    Clinic in June 1969, having been referred with the diagnosis of Kaposis sarcoma of the lower limbs. For a year prior ?o his admission the patient had noticed painful purple spots on both legs. After a cutaneous biopsy performed six months before admission, a diag- nosis of Kaposis sarcoma was made and both lower limbs were subjected to irradiation (1,125 rads (R) to the left leg and 375 R to the right leg), with some fad. ing of the lesions. Four weeks prior to admission the patient experienced severe pain in his feet and toes; the pain grew worse with exercise but was present at all times.

    Figure 1. A, multiple linear inflammatory and infarcted lesions OR the feet at initial examination. B, healing of lesions after bed rest, bypass surgery and cortisone ther-

    apy.

    Upon admission, abnormal physical findlngs were confined to the lower extremities. Fran: the middle of the thighs distally, a livedo reticular:s pattern and scattered erythematous macular lesio.ls (about 0.5 cm in diameter) were present. There were severe ischemic lesions on the toes of both feet iFigure IA), and peripheral pulses were decreased (Tab:{? I).

    A translumbar aortogram revealed a high degree of aorto-iliac obstruction. The aorta above ti:!s obstruc- tion was smooth, as were the distal iliac and fernoral arteries. A small popliteal aneurysm was present on the left, and there was occlusion (possibly embolic) of the left posterior tibia1 artery (Figure 2).

    Biopsy specimens of the skin from the left foot

    TABLE I Peripheral Pulses in Patient With Vascular Endothelial Tumor and Distal Embolization

    Grade*

    Pulse Right Side Left Side

    Aorta 4 Femoral 1 3 Popliteal 0 4

    Dorsalis pedis 0 0

    Posterior tibia1 0 0

    * Scale of 0 to 4, 4 being normal.

    Figure 2. Aortogram shows marked aorto-iliac obstruc-

    tion.

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  • MALIGNANT VASCULAR ENDOTHELIAL TUMOR - WINKELMANN ET AL.

    showed a mass obstructing the muscular vessels of the deep dermis and the connections to the subpapil- lary vessels. The proximal parts of the vessels were filled with large reticular cells that had little cytoplasm and homogeneous nuclei (Figure 3A). The distal parts of the vessels were filled with red blood cells and fibrin clots (Figure 38).

    Exploratory surgery was undertaken on June 27, 1969, at which time the diseased aorto-iliac segment was bypassed with a bifurcated, knitted, Dacron graft. The distal aorta and iliac arteries were filled with malig- nant-appearing tissue that seemed more embolic than aortic in origin. Histologically, this tissue was a malig- nant, grade 4 neoplasm of reticulohistiocytic type (Figure 3C). The cells were identical with thDSe ob- served in the lumen of the skin vessels (Figure 3D).

    The patients postoperative course was essentially uneventful, with continued ischemia of the toes of the

    left foot. Angiography undertaken in search of intra- cardiac neoplasia revealed no abnormality. The pa- tient was discharged in the care of his home physician; no specific treatment was prescribed.

    On August 11, 1969, prednisone therapy, 5 mg four times daily, was instituted. After one month of therapy, the patients condition had improved dra- matically. The mottled rubor had largely disappeared, and although some toenails on the left foot were lost, the pain was gone and the ischemic lesions were sub- stantially healed. The patient continued on this regi- men and by the end of December 1969, he was free of symptoms and had returned full time to his previous occupation. The condition of his feet at this time was much improved (Figure 1B).

    In February 1970, eight months after the operation, the patient had an acute relapse and died. Autopsy revealed an extensive intravascular angiosarcoma that

    694

    Figure 3. A, specimen of muscular ves- sel from deep dermis obtained by punch biopsy of skin shows syncytium of large reticular cells filling the lumen. Hematoxylin and eosin stain, original magnification x 500. 6, specimen of cutaneous vessel distal to that shown in Figure 2 contains a fibrin and red

    blood cell thrombus. Hematoxylin and eosin stain, original magnification X 150. C, specimen of tumor mass is composed of layers of fibrin, red blood cells with areas of surrounding necrosis and large reticular cells. Hematoxylin and eosin stain, original magnification x 100. D, close examination of syncyt- ium of cells reveals pale cytoplasm and homogeneous nuclei with prominent nu- cleoli, suggestive of reticulum cell neo- plasia. Hematoxylin and eosin stain, original magnification x 285.

    The American Journal of Medicine

  • MALIGNANT VASCULAR ENDOTHELIAL TUMOR - WINKELMANN ET AL.

    completely occluded the distal portion of the aorta and the right hypogastric artery. In addition, metas- tasrs had occurred to the vessels of both the terminal part of the ileum (Figure 4A and 4B) and the rectum, causing mucosal ulceration. A skin nodule in the left inguinal region showed extensive intradermal neo- plastic involvement (Figure 4C and 4D).

    COMMENTS

    Three distinctive features of this case imply that this rare syndrome should be a recognized pat- tern of vascular disease: (1) There was focal pro- liferation of cells, similar to neoplastic reticular cells, associated with the endothelium of a large vessel. (2) Multiple emboli occurred to peripheral arteries, resulting in gangrene and cutaneous in-

    flammation. (3) The lesions were recognrzed in the two levels of the vascular system by ist: of angio- graphic technics and skin biopsy.

    Abulafia and associates [3] desc!-rhed a rnaie patient in whom embolism to both upper and lower extremities developed in two abrupt episodes of inflammation and gangrene. No an;:iographic studies were performed, but because of the loca- tion of the emboli, either there were multiple tu- mors in the aorta or there was involvement of the ascending aorta or the arch of the aorta. Biopsy

    specimens of the skin of infarcted areas of both

    arms and legs revealed masses of intravascular

    reticular cells that produced complete occlusion

    of the vessels, identical with that seen in the biopsy specimens from the foot of our patient.

    Figure 4. A, specimen shows infiltra- tion of intestinal wall with angiosar-

    coma. Hematoxylin and eosin stain, original magnification >< 30. B, speci-

    men shows angiosarcoma with mixtures

    of large neomorphic cells and poorly

    formed vascular spaces, fibrosis and

    inflammation. Hematoxylin and eosin stain, original magnification x 170. C, specimen shows angiosarcoma meta- static to inguinal region. Hematoxylin and eosin stain, original magnification

    X 30. D, specimen shows ill-defined vascular spaces with large hyperchro- matic cells. Hematoxylin and eosin

    stain, original magnification x 130.

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    TABLE II Types of Primary Tumors of the Aortic lntima Recorded in the Literature

    Type Reference Year

    Benign (fibromyxoma) Karhoff [5] 1952

    Kattus et al. 16) 1960 Ma