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T H E AMERICAN JOURNAL OF CLINICAL PATHOLOGY Vol. 39, No. 4, pp. 374-382 April, 1963 Copyright © 1963 by The Williams & Wilkins Co. Printed in U.S.A. INTRAMURAL FIBROMA OF THE HEART JAMES A. FREEMAN, M.D., JACK C. GEEE, M.D., W. S. RANDALL, JK., M.D., AND WILLIAM G. PALFREY, M.D. Department of Pathology, Louisiana State University School of Medicine, New Orleans, Louisiana, and the Departments of Pathology and Pediatrics, Baton Rouge General Hospital, Baton Rouge, Louisiana Because of the rarity of primary cardiac tumors in general, and fibromas in particular, recording their occurrence is desirable in order to assist in early clinical recognition and possible surgical treatment of future cases. Documentation is also necessary to the study of the biologic behavior of these tumors. The interventricular cardiac fibromas have histologic features and behavioral characteristics similar to those of the fibromatoses in general. With this in mind, a case is added to the short list of interventricular fibromas (Table 1), and an attempt is made to relate these fibromas to the general group of fibromatoses. R E P O R T OF CASE Clinical History The patient was a Caucasian female born in December 1960 in Baton Rouge, Louisiana. The birth weight was 7 lb. V/i oz. No perinatal difficulties were encountered. The infant was blood type B, Rh-positive, Coombs' test negative. With the exception of a milk allergy, growth and development were within normal limits. A complete physical examination performed March 20, 1962, during an office visit to a pediatrician for conjunctivitis, was normal. No murmurs were heard, and the heart rate and rhythm were normal. On May 8, 1962, the child's mother noted she was sleeping late and tried to arouse her without success. Upon arrival of a pediatrician she was pronounced dead. Gross Necropsy Report The body was that of a normally developed, fairly well nourished, 17-month-old Caucasian female. With the exception of the lungs, heart, and liver, there were no lesions. The liver was moderately congested, and the lungs were severely congested. Approximately 100 ml. of clear fluid were in the pericardial sac. The heart was twice the normal size, and a gray-yellow, lobulated, soft mass occupied practically all of the musculature of the anterior left ventricle and extended into the interventricular septum. There was encroachment of the tumor upon both ventricular chambers. No endocardial or atrial involvement was found. There were no valvular defects. The heart weighed 170 Gm. (wet weight, after 10 per cent formalin fixation; normal dry heart weight for age and sex is 48 to 52 Gm.). The tumor mass measured 8 by 8 by 5% cm. Received, July 13, 1962; revision received, August 29; accepted for publication December 5. Dr. Freeman is Instructor of Pathology, and Dr. Geer is Associate Professor of Pathology, Louisiana State University School of Medicine. Dr. Randall is Pathologist, Baton Rouge General Hospital, and Clinical Assistant Professor, Louisiana State University School of Medicine. Dr. Palfrey is Visiting Pediatrician, Baton Rouge General Hospital. This study was supported in part by grants from the Louisiana Heart Association, the New Orleans Cancer Association, and the National Institutes of Health (H-2549). 374 Microscopic Features of Tumor The tumor was an unencapsulated but well delineated mass of abundant, dense, fibrous connective tissue bands interlaced with bundles of collagen and some elastic fibrils (Figs. 2 to 5). An interstitial proliferative infiltration of fibrous tissue was evident in the muscle; it enclosed muscle fibers (Fig. 4). No "primitive" cardiac muscle cells were recognized. Groups of young fibroblasts were seen in selected areas. Numerous small capillaries were present (Fig. 4). Masson's trichrome stain revealed the bundles of collagen, some spiralling through the mass (Figs. 3 and 4). The characteristic unit fibrils of collagen with the 640 A pe- April 1968 INTRAMURAL CARDIAC FIBROMA 375 FIG. 1. Gross photograph of the heart riodicity were demonstrable by means of electron microscopy (Fig. 6). The PTAH stain and trichrome stain demonstrated the cardiac muscle fibers incarcerated in the infiltrating fibrous tissue (Fig. 4). There was a small area of dystrophic calcification. No prominent xanthomatous element was present. Mitoses, anaplasia, and giant cells were not present. DISCUSSION 7 Occurrence and Incidence Although primary tumors of the heart are rare, Prichard20 (1951) and Bigelow and associates2 (1954) have demonstrated that they are more frequent than is generally believed. Whereas metastatic tumors represent approximately 3.9 per cent (there have been 326 metastases in a total of 8414 cases in published reviews) of cardiac tumors (Jernstrom and Cremin," 1959), primary tumors represent approximately 0.15 per cent of 4.80,000 autopsies, i.e., 716 instances of tumor (Straus and Merliss,24 1945). To date, there have been approximately 27 published cases of intramural fibroma (or fibrous hamartoma) of the heart (Table 1), excluding those occurring in the atrium. Although Mahaim 16 (1954) attributes the first recognition of a cardiac fibroma to Colombo in 1559, the first documented bona fide case is that of Luschka14 (1885). Author Unknown Sudden death Cardiomegaly; harsh m u r m u r Unknown None Left ventricle Left ventricle Left ventricle Left ventricle Left ventricle F F F F F 1 yr. 8 mo. 3 da. 2yr. 9 mo. 23 1940 Vukan 13 1949 Kulka 1954 Bigelow and associates 2 9 1955 Froboese 19 1955 Naeve cleft Unknown None Diphtheria; polio Glomerulonephritis ( ± a m y loidosis) Unknown None None Unknown Harelip, palate None None Sudden d e a t h Sudden d e a t h Unknown Left ventricle Interventricular None septum Newborn A recapitulation of Mono keberg's case 15 mo. M Left ventricle (pos- Sudden d e a t h (in hospital) terior wall) Uremia 53 yr. M Left ventricle M M F M None None Unknown Diphtheria None Clinical Diagnosis Sudden d e a t h Murmur Unknown None Sudden d e a t h Presenting or Cardiac Symptoms, or Both 65 yr. 9 mo. 2yr. 3 mo. Left ventricle Interventricular septum Interventricular septum and left ventricle Interventricular septum Left ventricle Left ventricle Location of Tumor 1937 Macherey 1 6 1937 Fidler and associates' 1938 Symeonidis and Linzbaeh 2 6 1927 T e u s c h e r " 4 1930 Brown and Gray Newborn M 1924 Monckeberg 1 8 F M F Sex 30 yr. 6 yr. 3 mo. Age Patient 1880 Zander 3 1 1559 Colombo 14 1855 Luschka 1871 Wagstaffe 30 Year TABLE 1 Fibroma Fibroelastic hamartoma Fibroma Fibroma Fibroma Incidental Hamartoma finding Incidental finding (Plastic) surgical d e a t h ; incidental finding Description and p h o t o s compatible with fibroma (No photos in a r t i c l e ) ; died in hospital (Cited by M a h a i m ) Incidental finding Remarks Hamartoma Fibroma Fibrosarcoma Fibroma Rhabdomyoma Fibroma Fibroma Fibroma Fibroma Fibroma Pathologic Diagnosis R E S U M E O F C A S E S O F INTRAMURAL V E N T R I C U L A R CARDIAC F I B R O M A S CQ ••a % CO - ^ « Left ventricle M 4yr. VA yr. M M F 51 yr. 42 hr. 17 mo. 1960 Boyette and Foushee 3 1962 Freeman and Spurlock 8 F M 1960 Svejda and Tomasek 2 5 8 mo. Left ventricle F 43 yr. 1958 Aiiriol and associates 1 1958 Krueger and Knoll 1 2 and 1959 Jernstrom Cremin 1 1 1960 Valledor and associates 2 8 Left ventricle F 19 mo. * Interventricular septum and left ventricle Interventricular septum Interventricular septum and left ventricle Interventricular septum Left ventricle Left ventricle 1957 Edlund and Holmdahl 6 M 7 mo. Right ventricle Left ventricle Left ventricle » 1956 Conlon 5 F 4 yr. F F , 47 mo. » 5 mo. " 1956 Radnai 2 1 1955 McCue and associates" 1955 James and Stanfield10 < , Sudden death Air hunger Angina-like pain Congestive failu r e ; syncope Congestive failure Sudden death None Sudden death (in hospital) M u r m u r (age 3 mo.) Unknown M u r m u r ; cardiomegaly Tachycardia » » » None None Ventricular aneurysm Fibroelastosis Subaortic stenosis None Leptomeningitis Diarrhea and emesis Cardiac t u m o r None Subaortic stenosis Fibroelastosis " ^ Fibroma Fibroma Fibroma Fibroma Fibroma Fibroma Fibroma Fibroma Fibroelastic hamartoma Mesenchymoma Fibroma Fibroma ' r T fibroma Subject of present report E K G : paroxysmal auricular t a c h y c a r d i a , r i g h t bundle branch block Died during surgery; E K G : left ventricular h y p e r t r o p h y ; strain E K G : old infarct; surgical diagnosis; died postoperative day 3 Gross diagnosis a t surgeo'. EKG: bundle branch block ± left ventricular hypertrophy Incidental finding Histologically E K G : left ventricular hypertrophy, strain EKG: sinoventricular versus ventricular tachycardia T 378 F R E E M A N ET AL. Vol. 39 FIG. 2. Gross photograph of a horizontal section of the heart, illustrating the relative size of the interventricular fibroma and the ventricular walls. The lacelike bundles of fibrous connective tissue are evident grossly. There is a remarkable gross resemblance to uterine leiomyomata. Of these 27 cases, 21 have occurred in children (78 per cent). No sex predisposition is noted; 12 of the 27 cases were in males. Two cases have been recorded as the cause of death in a newborn (Monckeberg,18 1924; Boyette and Foushee,3 1960), and 15 cases in infancy (Table 1). In 9 instances (including the present case), the tumor has been invoked as the cause of sudden death. Only 2 cases have been diagnosed premortem, both at operative surgery (Edlund and Holmdahl, 0 1957; Svejda and Tomasek,25 1960). Only 1 case in the childhood group was an incidental finding (Auriol and colleagues,1 1958). Most intramural fibromas are located in the interventricular septum or anterior part of the left ventricular wall, only 1 being recorded on the posterior left ventricular wall (Symeonidis and Linzbach,26 1938) and 1 infiltrating the right ventricle (Radnai,21 1956). Terminology and Pathogenesis Fibromas and fibrous hamartomas are grouped together for the purposes of this report. Only interventricular fibromas are included, inasmuch as fibromas of the atrium and heart valves may be related to myxomas, or they have been suspected of being nonneoplastic growths of inflammatory origin or Lambl's excrescences (Prichard,20 1951). Other terms have been applied to these infiltrating fibrous growths, such as fibroelastic hamartoma and desmoid. Considerable controversy has arisen regarding the pathogenesis of primary benign cardiac fibromas. Indeed, some authors will not accept fibroma as a true diagnosis, but group the lesions instead with hamartomas in general (Prichard, 20 1951). Conlon5 (1956) described a fibrous intramural growth as an embryonic mesenchymal tumor. Inasmuch as fibromas develop from mesenchyme, with an exaggeration of the fibrous element, and Conlon's report resembles the case herein presented, his case is included in the table. The designation of these tumors as embryonic mesenchymal tumors adds nothing to an understanding of the group to which they belong, inasmuch as all cardiac tumors may be said to arise from mesenchyme. The Fio. 3. Microscopic section, with the well demarcated, but unencapsulated fibrous mass. Unaffected cardiac muscle is at the top of the micrograph. Paraffin embedding. Masson's triclirome. X 240. Fio. 4. The proliferating, interstitial infiltration of fibrous connective tissue is incarcerating muscle fibers (M). Capillaries are prominent. Paraffin embedding. Masson's triclirome. X 240. 379 380 FREEMAN ET Vol. 39 AL. FIG. 5. Bands of collagen (C) and fibrous connective tissue infiltrating mature cardiac tissue. Maraglas epoxy embedding. (Freeman and Spurlock,81962.) Toluidine blue. X 500. predominant element of these tumors is fibrous tissue with abundant collagen and young fibroblasts. Some, but little, elastic tissue component is present. Therefore, although it is perhaps semantically correct to call these tumors fibroelastic hamartomas, this designation serves no useful purpose. Hence, the present authors prefer to call these tumors fibromas. Symptomatology and Manifestations As previously mentioned, these growths may produce sudden death (9 of 27 cases) without giving rise to previous symptoms. Although the tumors are histologically benign, they are liable, by virtue of then- location, to interfere with the conduction system and, therefore, to cause death. They may, however, induce a variety of clinical manifestations, the most notable of which are bizarre electrocardiographic tracings. Two of the 27 cases manifested the findings of subaortic stenosis, whereas 2 other cases were suggestive of fibroelastosis (in children). Only 2 were diagnosed pre-mortem, both during cardiac surgery, and a cardiac tumor was suspected clinically in 1 case, whereas a ventricular aneurysm was suspected in the other. Relation to Fibromatosis Fibrous growths of other parts of the body (desmoid fibromatoses, Dupuytren's palmar fibromata, juvenile aponeurotic fibroma, the well differentiated fibroma-sarcomas, keloids, and others) are not uncommon (Stout, 22,23 1953 and 1954). The common characteristic of many of these growths is the unencapsulated, but well circumscribed, infiltrating, proliferating, dense, fibrous connective tissue pattern. These fibromatoses are regarded as benign, and metastasis is rare. Inasmuch as behavioral characteristics differ with the various diagnoses, their subclassification is well justified; however, they have all been interrelated as the fibromatoses April 1963 I N T R A M U R A L CARDIAC 381 FIBROMA SUMMARFO I N 1NTERLINGUA F I G . 6. An electron micrograph illustrating t h e G'10 A periodic banding characteristic of mature collagen. Maraglas epoxy embedding. Unstained. X 16,000. (Stout, 22,2:i ). Inasmuch as the intramural fibroma of the heart has the same benign, diffuse, interstitially proliferating, unencapsulated, but well circumscribed, fibrous growth pattern, it is suggested that this tumor should be in the same category and thus related to the desmoid fibromatosis and the other fibromatoses. Despite the benign character of the fibromatoses, especially the intramural cardiac fibroma, the anatomic site of occurrence precludes longevity. Iste articulo describe un fibroma interventricular que causava le morte subitanee de un previemente normal feminina de racia blanc de 17 menses de etate. Le litteratura pertinente a fibromas cardiac (hamartoma fibrose, desmoide, hamartoma fibroelastic) es revistate. Ex le 27 exemplos trovate, 21 occurreva in juveniles, e 2 esseva registrate como causa de morte in neomatos. II non existe un apparente predisposition sexual. In 9 casos, le tumor esseva incriminate como le causa de morte. Histologicamente, le tumores es proliferationes benigne de fibrose tissu conjunctive e non es incapsulate. Illos es simile in characteristicas histologic e in lor crescentia a fibromatoses in general. Acknowledgments. We express our appreciation to M r . Ben 0 . Spurlock, who did the fine sectioning for electron microscopy, and to Mr. Eugene Wolfe for t h e photography. REFERENCES 1. A U R I O L , M., PRADB, M., AND C E S A R I , 2. B I O E L O W , N . H . , K L I N G E R , S., AND W R I G H T , A. W . : P r i m a r y tumors of t h e heart in infancy and early childhood. Cancer, 7: 549-563, 1954. 3. B O Y E T T E , D . P . , AND F O U S H E E , J . H . : C a r d i a c fibroma of t h e interventricular septum in a newborn infant. Case Report. N o r t h Carolina M . J . , 2 1 : 544-546, 1960. 4. B R O W N , G., AND G R A Y , J . : A case of SUMMARY This paper deals with the description of an interventricular fibroma that caused sudden death in a previously healthy, 17-monthold Caucasian female. The literature pertaining to cardiac fibromas (fibrous hamartoma, desmoid, fibroelastic hamartoma) is reviewed. Of the 27 examples found, 21 occurred in children, and 2 were recorded as the cause of death in newborn infants. There is no apparent sex predisposition. In 9 cases, the tumor was invoked as a cause of sudden death. The tumors are histologically benign proliferations of fibrous connective tissue, and are not encapsulated. They are similar in histologic features and growth characteristics to fibromatoses in general. J.: Fibrome intramural du coeur. Bull. Assoc, franc, etude cancer, 46: 70-76, 1958. con- genital rhabdomyoma of the heart. Lancet, 1: 915-916, 1930. 5. CONLON, H . J . : Embryonic mesenchymal tumor of t h e heart. Am. J . Clin. P a t h . , 26: 297-300, 1956. 6. EDLTJND, S., AND HOLMDAHL, K.: Primary tumour of t h e heart. Report of a case. Acta paediat., 46: 59-63, 1957. 7. F I D L E R , R. S., K I S S A N E , R. W., AND K O O N S , R. A . : P r i m a r y fibrosarcoma of t h e heart. Am. H e a r t J . , 13: 736-740, 1937. S. F R E E M A N , J . , AND SPURLOCK, B . : A new epoxy embedment for electron microscopy. J . Cell Biol., 13: 437-443, 1962. 9. FROBOESE, C : Grosses angeboremes Elastofibrom des kindlichen Herzmuskels. Arch. Kinderh., 151: 59-6S, 1955. 10. J A M E S , U., AND S T A N F I E L D , M . H . : Case of fibroma of t h e left ventricle in a child of 4 years. Arch. D i s . Childhood, 30: 1S7-192, 1955. 11. J E R N S T R O M , P . , AND C R E M I N , J . H . : mural fibroma of t h e heart. P a t h . , 32: 250-256, 1959. Intra- Am. J . Clin. 12. K R U E G E R , N . L., AND K N O L L , W. V . : I n t r a - mural fibroma of the myocardium. Arch. P a t h . , 66: 590-593, 195S. A. M . A. 382 FREEMAN ET AL. Vol. 89 13. K U L K A , VV.: I n t r a m u r a l fibroma of t h e heart. Am. J . P a t h . , 25: 549-557, 1949. 14. LTJSCHKA, H . : E i n Fibroid in Hertzfieische. Virchows Arch. p a t h . Anat., 8: 343-347, 1855. 15. MACHEREY, W . : E i n Fibrom m i t Knockembildung in der M u s k u l a t u r des linken Ventrikels. Beitrag z u r Kasuistik primiirer Herztumoren. Beitr. p a t h . Anat., 98: 565-570, 1937. 16. M A H A I M , I . : Les tumeurs et les polj'ps du coeur. Etude anatomo-clinique. P a r i s : Mason et Cie, 1945. 21. RADNAI, B . : Uber das i n t r a m u r a l Herzfibrom. Zentralbl. allg. P a t h . , 95: 527-535, 1956. 22. STOUT, A. P . : Tumors of t h e soft tissues. A F I P Atlas of Tumor Pathology, Sec. 2, Fas. 5: 1953. 23. STOUT, A. P . : Juvenile fibromatosis. Cancer, 7: 953-978, 1954. 17. M C C U E , C. M . , H E N N I G A R , G. R., D A V I S , E . , die fibro-elastosen H a m a r t i e n des Myokards (sog. Herzfibrom). Virchows Arch. p a t h . Anat., 302: 383-404, 1938. 27. TEUSCHER, M . : Uber ein Herzfibrom. F r a n k furt. Ztschr. P a t h . , 35: 101-106, 1927. AND R A Y , J . : Congenital subaortic stenosis caused by a fibroma of left ventricle. Pediatrics , 16: 372-377, 1955. 18. M O N C K ^ B E R G , Myokards J.: Die Erkrankungen und des spezifischen des muskel s y s t e m s . In H E N K E , F . , AND LUBARSCH, 0 . : H a n d b u c h der speziellen pathologischen. II. Anatomie u n d Histologie. Berlin: J . Spring er-Verlag, 1924, p p . 482-502. 19. N A E V E , W - : Plotzlicher T o d eines Siiuglings bei fibro-elastosen H a m a r t i e des Myokards (sog. "Herzfibrom"). Kinderiirztl. Praxis, 23: 304-306, 1955. 20. PRICHARD, R . W . : Tumors of t h e heart. R e view of t h e subject and report of one hundred and fifty cases. Arch. P a t h . , 5 1 : 98-128, 1951. 24. STRAUS, R., AND M E R L I S S , R . : P r i m a r y t u m o r _ of t h e heart. Arch. P a t h . , 39: 74-78, 1945. 25. SVEJDA, J., AND T O I I A S E K , V.: Fibrous ham- artoma or so-called fibroma of t h e myocardium. J . P a t h . Bact., 80: 430-432, 1960. 26. SYMEONIDIS, A., AND LINZBACH, A. J . , U b e r 28. VALLEDOR, T . , BORBOLLA, L., SATANOWSKY, C , P R I E T O , E . , SANCHEZ, G., A G U I R R E , F . , J U N C O , J . , AND GARCIA PALACIO, A . : F i b r o m a of t h e heart. Case report. D i s . Chest, 37: 698-701, 1960. 29. VUKAN, J . : A case of tumor of t h e heart. Orvosi Hetilap, 84: 356-357, 1940. 30. WAGSTAFPE, W.: Fibrous tumour of t h e heart. T r . P a t h . Soc. London, 22: 121-124, 1871. 31. ZANDER, R . : Fibrom des Herzens. Virchows Arch. p a t h . Anat., 80: 507-510, 1SS0.