Telangiectasia of Pembroke Welsh Corgi Dogs
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Vet. Pathol. 20: 203-208 (1983) Telangiectasia of Pembroke Welsh Corgi Dogs F. M. MOORE and G. W. THORNTON The Department of Pathology and the Department of Internal Medicine, Angell Memorial Animal Hospital, Boston, Mass Abstract. Multiple vascular lesions involving kidneys and various other organs consisting of cavernous, blood-filled spaces lined by endothelial cells with various amounts of mural collagen are described in eight Pembroke Welsh Corgi dogs. The most common clinical sign associated with this condition is hematuria. Hemangiomas are relatively common in dogs [14]. The differentiation between these benign neoplasms of endothelial cells, and the developmental anomalies of vessels (so-called hamartomas) is difficult [10, 17]. Multiple hemangiomatous syn- dromes have been reported in man [5, 6, 8, 17, 19], and several hemangioma-like conditions have been recognized in domestic animals [4, 11, 14, 15, 18]. No animal syndromes have been reported with multiple organ system involvement, however. This paper describes the occurrence of multiple vascular lesions in a group of Pembroke Welsh Corgi dogs. Case Report This disease entity has been diagnosed definitively by histological evaluation in eight red Pembroke Welsh Corgi dogs. Six dogs were necropsied and surgical renal biopsies alone were examined from the other two dogs. No geneological information was available, but none of the Corgies were known to be related. Five males and three females were affected and all except one had intermittent gross hematuria, often with several months between episodes. In addition to bloody urine, blood clots were voided occasionally. Other clinical signs reported were splinting of the abdomen, whining, vomiting, and occasional dysuria. In one dog, no clinical signs were reported. The age at onset of the clinical signs varied from two to eight years; however in one dog, the age at onset was unknown. Physical examination frequently revealed no abnormal findings. Occasionally, renal pain could be elicited upon palpation, or a small, irregular kidney could be felt. The only consistent laboratory finding was the presence of numerous red blood cells in the urine even when gross hematuria was absent. Most dogs had bacterial urinary tract infection during some episodes of hematuria. Radiographic evaluation of dogs in the acute phase of the disease commonly revealed no abnormalities. Renal calculi and renal calcification sometimes were seen in dogs that had 203 Downloaded from vet.sagepub.com by guest on October 12, 2015
204 Moore and Thornton Table I. Telangiectasia in Pembroke Welsh Corgi dogs Dog number Age (years) Sex Sites of involvement 1 5 M Kidneys 2 13 M Kidneys, subcutis midbrain 3 9 F/S Kidneys, spleen 4 II F Kidneys, subcutis 5 13 M Kidneys, subcutis, duodenal serosa 6 13 F/S Kidneys, subcutis, anterior mediastinum, spleen, retro- peritoneum 7* 6 M 8* 9 M * Only kidneys were examined microscopically. hematuria for several years. Intravenous pyelograms revealedpyelorenal backflow and dis- torted renal pelves in chronically affected dogs. The renal angiograms that were done on two dogs were not diagnostic. Treatment was directed at prevention of bacterial urinary tract infection, renal calculi, and anemia due to blood loss. The age range at death or euthanasia was from five to 13 years (table I). Three dogs died or were euthanatized due to anemia; one was euthanatized because of hydronephrosis secondary to obstructing ureteral blood clots; three died or were euthanatized for unrelated conditions, and in one dog the circumstances of death were unknown. Two corgies having this condition were not autopsied, and the extent of disease involvement other than the renal lesions was not determined. Therefore, the following discussion will refer to the six dogs which were necropsied. Tiss~es for histologic examination were obtained at surgery or necropsy, fixed in 10% formalin, embedded in paraffin, sectioned at 6 urn, and stained with hematoxylin and eosin (HE); and selected tissues were stained with Masson's trichrome, Verhoeff's elastic, and Gomori's reticulin stains. Results Bilateral renal involvement was evident in each dog, but the kidneys were unequal in size in four. Despite these findings, the weight (as expressed in combined renal weights/Hx) g body weight) was considered normal. Red-black nodules, ranging from several millimeters to several centimeters in diameter, were visible on the capsular surface (fig. I). Some nodules were soft and fluctuant and others were firm. Occasionally, a single nodule would comprise one pole of the kidney. The intervening capsular surface was roughened with multiple depressed foci, I to 3 mm in diameter. On cross section, multiple, soft, red-black regions were visible; the larger of which corresponded to the capsular nodules. In addition, soft red foci (0.2 to 0.7 em diameter), many of which were cystic, were located within the medulla. These foci clustered subjacent to the corticomedullary junction consistently (fig. 2). Clotted blood was present in these cysts and frequently in the renal pelvis as well. Hydro- Downloaded from vet.sagepub.com by guest on October 12, 2015
Telangiectasia in Corgi Dogs 205 Fig. I: Kidneys of dog 6 with raised black masses and irregular capsular surface. Fig. 2: Longitudinal section of kidney. dog 6, with numerous black foci; some appear cystic. Clustering of foci in outer medullary region. Blood clot fills renal pelvis. nephrosis was evident in three dogs, and severe hydronephrosis and hydroureter were associated with a blood clot in dog 1. Subcutaneous lesions were well circumscribed, soft to firm, red and 0.4 to 2.5 em in diameter. Splenic lesions occurred in two dogs (table I), and gross abnormalities were evident in one of these. Multiple, raised, red-purple, soft masses 0.4 to 0.8 ern in diameter occurred in dog 6. In dog 5, a firm and red-black duodenal mass (0.3 X 0.2 x 0.1 em) was located on the serosal surface along the antimesenteric border. The anterior mediastinal masses of dog 6 were well-delineated, soft, red, and 0.1 to 0.3 cm in diameter. A similar lesion, 0.8 em in diameter, was located ventral to the fifth thoracic vertebra. In this dog, a 2.0-cm diameter, soft, red, nodular mass also occurred in the retroperitoneal space ventral to the fourth lumbar vertebra. In dog 2, a 0.7 X 0.6 x O.5-cm black, irregular, granular mass was located at the left ventrolateral edge of the thalamus, immediately rostral to the cerebellar cortex. The lesions were characterized by multiple, cavernous, blood-filled spaces with simple endothelial Downloaded from vet.sagepub.com by guest on October 12, 2015
206 Moore and Thornton 3 4 5 6 Fig.3: Histological section of kidney, dog 6, with multiple, cavernous, blood-filled spaces subjacent to cortico-medullary junction. HE. Fig. 4: High power magnification of renal vascular lesion with simple endothelial lining. HE. Fig.5: Duodenum, dog 5. Cavernous, blood-filled space located with tunica muscularis. Masson's trichrome. Fig. 6: Midbrain lesion, dog 2. HE. Downloaded from vet.sagepub.com by guest on October 12, 2015
Telangiectasia in Corgi Dogs 207 linings and various amounts of mural collagen. Frequently, thrombosis and hemor- rhage were evident [fig. 3, 4]. In the kidney, the lesions were located primarily in the medulla adjacent to the corticomedullary junction. Often, the width of the overlying cortex was decreased due to a loss of tubules and glomeruli, with interstitial fibrosis and mononuclear interstitial nephritis. In several kidneys, the overlying cortex was thinned so that the larger lesions extended close to the capsular surface. No tubules were found inter- posed between vascular spaces. The duodenal lesion of dog 5 was located within the inner portion of the muscularis externa [fig. 5]. A leiomyoma occupied a similar position in an adjacent portion of the duodenum. No neural tissue was found interposed between the blood-filled spaces within the midbrain lesion [fig. 6]. Discussion Renal hemangiomas have been reported in the dog [3, 12], and in both of these dogs a solitary, discrete lesion involving one kidney was associated with hematuria. Similarly, unilateral involvement is the most frequent finding in humans with renal hemangiomas [I, 2, 7, 9, 13, 16]. In contrast, the vascular lesions found in these Corgies consistently involved both kidneys, and frequently occurred in multiple organs. This finding, in association with the consistently simple endothelial lining and lack of endothelial cell proliferation suggests that these lesions represent malformations. The unique group of animals affected may evidence a possible congenital or hereditary nature of this disorder. The term telangiectasia was chosen to designate these lesions to avoid confusion with terms suggestive of neoplasia. Multiple hemangiomatous syndromes have been reported in humans, including Sturge-Weber's syndrome, von Hippel-Lindau disease, and Osler's disease. These syndromes are characterized by the development of lesions in the skin, mucous membranes, retina, leptomeninges, respiratory, urinary, and gastrointestinal tract. In von Hippel-Lindau disease, the vascular lesions are associated with cysts of the pancreas, liver, and kidneys. In Sturge-Weber's disease, a lesion within the lepto- meninges is associated with ipsilateral facial nevi [5, 6, 8, 17, 19]. In these corgies, the vascular abnormality was not found in association with other malformations. No syndromes of vascular anomalies with multiple system involve- ment have been reported previously in animals. Further investigation of these telangiectasias is necessary to define more accurately the abnormality, and to explore the possible use of these dogs as models for the development of vascular malformations. Acknowledgements This work was supported in part by National Institute of Health, National Research Service Award 5T32RR07000-07. Downloaded from vet.sagepub.com by guest on October 12, 2015
208 Moore and Thornton References ANDERSON, J.B.; RASMUSSEN, TH.: Renal hemangioma diagnosed preoperatively by select- ive renal angiography. Acta Radiol [Diagn] (Stockh) 2:201-204, 1964 2 BENNINGTON, J.L.; BECKWITH, J.B.: Tumors of the Kidney, Renal Pelvis and Ureter, p. 220. Armed Forces Institute of Pathology, Washington, D.C., 1975 3 CADWALLADER, J.A.; GOULDEN, B.E.; WYBURN, R.S.; JOLLY, R.D.: Renal hemangioma in a dog. NZ Vet J 21:48-51, 1973 4 COTCHIN, E.; SWARBRICK, 0.: Bovine cutaneous angiomatosis: A lesion resembling human "pyogenic granuloma" ("Granuloma telangiectaticum"). Vet Rec 75:437-444, 1963 5 CRAIG, J.M.: Encephalo-trigeminal angiomatosis (Sturge-Weber's disease). J Neuropathol Exp Neuro18:305-318, 1949 6 DAVIDOFF, L.M.: Hemangioblastoma of the cerebellum. Am J Patho15: 141-145. 1929 7 FRIEDMAN, P.S.; SOLIS-COHEN, L.: Hemangioma of the kidney-Its roentgen diagnosis. Am J Roentgenol60:408-41O, 1948 8 GOLDSTEIN, H.J.: Hereditary multiple telangiectasia. Archives of Dermatology and Syphilogy 26:282-308, 1932 9 HAMM. F.C.: Angioma of the kidney. J Uro155: 143-148.1946 10 LANDING, B.H.; FARBER, S.: Tumors of the Cardiovascular System, p. 45. Armed Forces Institute of Pathology, Washington, D.C., 1958 11 LEE, C.H.: Hemangioendotheliomata in a herd of pure bred middle white swine. J Malay Vet Med Assoc I: 164-167, 1957 12 LEE, R.; WEAVER, A.D.; ROBINSON, P.B.: Persistent hematuria in a dog due to a discrete renal hemangioma. J Small Anim Pract 15:621-626, 1974 13 MCCREA, L.E.: Hemangioma of the kidney: Review of the literature. Urologic and Cutaneous Review 55:670-680, 1951 14 MOULTON, J.E.: Tumors in Domestic Animals, pp. 33-35. University of California Press, Berkeley, 1978 15 NUMI, D.: Patho-histological studies on the characteristic hemangiomata in the scrotum of swine. J Jpn Soc Vet Sci 10:31-52.1931 16 PETERSON, N.E.; THOMPSON, H.T.: Renal hemangioma. J UrolI05:27-31, 1971 17 ROBBINS, S.L.; COTRAN, R.: Pathological Basis of Disease, p. 634. W. B. Saunders Company, Philadelphia, Pennsylvania. 1979 18 WEIPERS, W.L.; JARRETT, W.F.: Hemangiomas of the scrotum of dogs. Vet Rec 66: 106- 107, 1954 19 WILLIS, R.A.: The Borderland of Embryology and Pathology, pp. 355-357. Butterworth and Co., London, 1962 Request reprints from Frances M. Moore, Angell Memorial Animal Hospital, 350 S. Hunting- ton Ave, Boston, MA 02130 (USA). Downloaded from vet.sagepub.com by guest on October 12, 2015
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