Primary cardiac rhabdomyosarcoma presenting as pulmonary stenosis

Case Reports Primary Cardiac Rhabdomyosarcoma Presenting as Pulmonary

Stenosis*

ERNEST E. PUND, JR., M.D.,~ TERRY M. COLLIER, M.D., JOHN E. CUNNINGHAM,JR., M.D. and JUDE R. HAYES, M.D. San Diego, California

of a heart murmur even though he had had a number of thorough physical examinations for submarine duty. An adamantinoma had been excised from his The family history right mandible in 1960 and 1961. was noncontributory. On physical examination, the patient was a well developed, well nourished white man appearing mildly apprehensive. The pulse was 76,/min.; blood pressure, 120/80 mm. Hg; respiration, 16,‘min. and temperature 37’ c. There was no edema of the limts.

RIMARY rhabdomyosarcoma of the heart is a rare lesion, only 31 cases having been reBecause of their rarity and their ported.’ tendency to masquerade as more common lesions, primary tumors of the heart are seldom diagnosed ante mortem. We have recently encountered a case which presented as mild pulmonary stenosis. The presence of a murmur of probable recent onset, associated pulmonary lesions and a progressively deteriorating course led to the suspicion of a To our cardiac neoplasm prior to death. knowledge there has been only one other case which presented as primary involvement of the pulmonary valve.2

P

and the peripheral pulses were all palpable and normal. The jugular venous pressure was normal. The Inlungs were clear to percussion and auscultation. spection and palpation showed the precordium was normal. Auscultation at the pulmonary area revealed that the second sound was spIit, the second The component being less intense than the first. A systolic split did not fully close with expiration.

CASE REPORT

ejection click was heard. Beginning with this click, there was a grade 316 harsh. high pitched, ejection type systolic murmur which was well transmitted into the left infraclavicular area and the neck and was loudest at the pulmonary area. A third heart sound was easily heard at the lower left sternal border. r\;o diastolic murmur was heard. Ko organs nor masses were palpable in the abdomen. The hematocrit was Laboratory Studips on Admission: 42 per cent, white blood cell count, lO,OOO/cu. mm.: \vith a differential count of 75 per cent polymorphonuclear leukocytes, 22 per cent lymphocytes, 2 per cent monocytes and 1 per cent eosinophils; rrythroc)-te sedimentation rate (Wintrobe method) was 8 mm. hr. ; C-reactive protein 3 plus; antistreptolysin titer, 250 Todd units. Routine urinalysis, two-hour postprandial blood sugar and blood urea nitrogen were normal. The electrocardiogram showed sinus tachycardia at 115/min. and a mean QRS electrical

A 32 year old white male submariner

was admitted to the U. S. Naval Hospital on Jan. 16, 1962, with the chief complaint of dizziness and shortness of breath of one month duration. He had been completely well until two months prior to admission when he developed a mild, dry, hacking cough and coryza. The symptoms did not become more severe but the) persisted, and one month later he had anorexia. intermittent dizziness. shortness of breath and a single The dizziness was frequentl!. episode of hemoptysis. related to exertion. ?‘wo weeks before admission he was admitted to another facility where a heart murmur was detected, and he was referred to this hospital for further evaluation. The patient denied having orthopnea, paroxysmal nocturnal dyspnea. ankle edema or frank syncope. There was no past history of heart disease or rheumatic fever. He had no knowledge of the presence

* From the Departments of Medicine and Pathology, U. S. Naval Hospital, San Diego, Calif. The opinions or assertions contained in this article are private ones of the writers and are not to be construed as official or reflecting the views of the Navy Department or the Naval Service at large. t Present address: Dc Anza Medical Clinic, San Diego, Calif. AUGUST

1963

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250

Pund,

Collier,

Cunning-ham

’ January L7, 1962 II I

AVR

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and

Hayes

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* AVF

FIG. 1. Electrocardiograms taken during the hospital course. Note the shift of the electrical axis to the right and slight prolongation of the QRS duration. axis of plus 90 degrees (Fig. 1). The chest roentgenograms were interpreted as showing several nodular densities 1 cm. in diameter in the second intercostal space on the right and superimposed on the third rib on the left anteriorly. This latter density was The size of the heart was at considered an artifact. the upper limits of normal, and there was no prominence of the main pulmonary artery (Fig. 2). impression was viral Course: The initial clinical and congenital valvular pulmonary pneumonitis

Routine stenosis of little physiologic significance. sputum cultures and cultures for tuberculosis and The fungi were negative for pathogenic organisms. patient was treated with aspirin and expectorants, and his symptoms improved slightly until February 20 when he had two syncopal episodes associated with On physical examipain in the left side of the chest. nation there was a sinus tachycardia of llO/min.; a paradoxic pulse of 16 mm. Hg ; and decreased breath sounds, dullness to percussion and rales in the right THE

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CARDIOLOGY

Cardiac

Rhabdomyosarcoma

.\dmission chest roentgenogram with barium Fro. 2. Note the 1 cm. density overlying the left third swallow. rib anteriorly, which was interpreted as artifact.

lung base. The only- change in the cardiac examinaThis was tion was the addition of a gallop cadence. attributed to the previously noted third heart sound The liver was palin the presence of a tachycardia. The vepable 2 cm. below the right costal margin. nous pressure was 220 mm. saline, and the arm-totongue circulation time (Decholin”) was 12 sec. A chest roentgenogram showed an increase in size of the densities described on the admission roentgenograms, and the cardiac silhouette was markedly enlarged, typical of a pericardial effusion [Fig. 3). ‘The electrocardiogram showed sinus tachycardia at 11 O/min. with a mean QRS electrical axis of plus 110 degrees and an RSR ’ in Vi (Fig. 1). A prricardiocentesis was performed, and 310 cc. of A gram stain serosanguinous fluid was removed. The cell count was 10,000, on the fluid was negative. cu. mm. with 90 per cent polymorphonuclear leukocytes. Microscopic examination of block sections At this timr revealed cells suspicious for malignancy. serious consideration was given to the possibility that a pleural mesothelioma with extension into the main pulmonary arterial region was causing the murmur. The patient continued to do poorly and repeated pericardiocenteses were performed on February 21 and March 1 with results similar to the first. Examination of cell block sections from pleural fluid obtained at the time of an unsuccessful needle biopsy of the pleura showed cells suspicious for malignancy. Chest roentgenograms on March 2 showed a marked AUGUST

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FIG. 3. Chest roentgenogram showing enlargement of thr nodular density overlying the left third rib anteriorly and thr cardiac silhouette enlargement typical of perirardial effusion. Notr the appcarancr of ksions in the right lateral iung Iicld and the pleural rffllsion.

pleural effusion on the right with almost complete obliteration of the lung field, and the previously described density of the left upper lobe had more than doubled in size. The cardiac silhouette remained markedly enlarged, and it was still typical of a pericardial effusion (Fig. 4). On March 8 the patient was transferred to the thoracic surgery division in preparation for a thoraTwo days later he suddenly cotomy and open biopsy. died during a syncopal episode. i2n electrocardiogram one day before death showed a slight prolongation of the QRS duration and further shift of the mean QRS electrical axis to the right in comparison with previous tracings (Fig. 1). During the entire hospital course the body temThe entire illness, perature did not exceed 37.8’ c. including the hospitalization, was 90 days. The pertinent findings were Findings at Autopsy: The pleural and perilimited to the thoracic cavity. cardial spaces were almost completely obliterated by fibrous adhesions. Multiple umbilicated, confluent tumor nodules studded the parietal pleura and pericardium. The lungs weighed 1,950 gm. and contained multiple subpleural and intrapulmonary tumor masses separated by a congested parenchyma. ‘The tracheobronchial tree was filled with foamy, bloody secretions. The heart \\-eighed 750 gm. The tricuspid valve

Pund, Collier,

Cunningham

and Hayes

Fro. 4. Chest rotmtgenogram showing pleural and pericardial effusion with progrcssivc Aarq’mc’nt of densitic,s in tht- right and left lung fields.

‘Ike epicardium was cncasrd in a mrasurrd 13 cm. nodular tumor mass which superficially infiltrated the myocardium but not the coronary arteries. Within the pulmonary artery- a protuberant mass of tumor filled the sinuses of the pulmonar>, valve and virtually occluded the lumen (Fig. 5). ‘l’hr tumor. grossly similar throughout. \vas tan and rubbery. and the larger masses contained central Congestive hepatosplenomegaly areas of necrosis. was present. Grossly, no involvement of lymph Dissection of the mandibular surnodes was found. gical site and adjacent cervical area failed to reveal Review of all biopsied recurrent adamantinoma. material from the adamantinoma coniirmed that diagnosis, and no histologic similarity to thr thoracic tumor was noted. In rni~osco~~ics&ionJ: the tumor was identical in all areas. Masses of eosinophilic necrotic tumor contained sheets of viable cells with large oval-to-elongate nuclei with vesicular chromatin pattern and a promiMitotic activity was nent eosinophilic nucleolus. ‘l‘he eosinorare: but a tripolar mitosis was seen. philic cytoplasm contained areas of periodic granuThe bipolar cytoplasmic projections showed laritv. bifurcation and fusion with adjacent cells terknal creating a pattern such as is seen in tissue culture of rmbryonal rhabdomyoblasts. Typical racquet, banjo and strap forms were seen, but cross striations were rarely found (Fig. 6.).

Microscopic section of the tumor showing FIG. 6. rhabdomyoblasts with granular cytoplasm and periHematoxylin and rosin stain. magninuclear striations. fication X970. THE

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Cardiac DISCUSSION

Several aspects of the case should have suggested a cardiac neoplasm. The patient had several thorough examinations for submarine duty and no murmur was heard. This fact was not given serious consideration at the time of admission. The case clearly demonstrated pulmonary stenosis on physical examination, but there was nothing to indicate the lesion was physiologically significant. In retrospect, it probably was at least transiently significant, since the valvular stenosis is the best explanation for the dizziness associated with exertion that the patient experienced prior to admission. The pulmonary lesions were not thought to be related to the cardiac lesion and, at first, were so unimpressive that the possibility of artifact was relentless deterioration entertained. The following admission was characteristic of a malignant disease. The case reported by Mannix and Lukash” is intriguing because of the similarity of site of origin and the almost complete obliteration of the pulmonary valve in both cases. They also detected a heart murmur for the first time in a patient who had had a number of negative physical examinations prior to the onset of symptoms. The most common site of origin of rhabdomyosarcoma of the heart in 31 cases was one or both atria. The atrioventricular valves were more cominonly involved than the semilunar valves. Supraventricular arrhythmias were common.2 7’he diagnosis of rhabdomyosarcoma has been reported to depend on the finding of cross striations in the muscle fibers.4 However, recent studies employing immunohistochemicai technics have demonstrated myosin in three soft tissue embryonal rhabdomyosarcomas which did not show cross striations.3 Tissue culture studies of cmbryonal cardiac rhabdomyoblasts have shown that myofibril formation without cross striation precedes contraction, which occurs only in striated myofibrils. The development

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Rhabdomyosarcoma

of contractile (striated) myofibrils is thought require c!-totension, which would be difficult obtain in tumor e;rowth.‘j

to to

SUMMARY A 32 year old man presented with signs of pulmonary stenosis. The murmur was not present in previous physical examinations as recent as one year prior to admission. Parenchyma1 lung lesions and pleural and pericardial effusions developed in the course of the disease which was rapid and fatal. The pathologic specimens revealed a primary rhabdomyosarcoma of the heart growing across the pulmonary valve and metastasizing to the lungs. This represents the thirty-second case report of primary rhabdomyosarcoma of the heart and the second presenting as primar)- involvement of the pnlmonar)- val1.e. ACKNOWLEDGMENT The authors gratefully acknowledge the assistance of Sheldon C. Sommcrs, M.D., of Scripps Memorial Hospital, La Jolla, California, in confirming the pathologic diagnosis.

KEFERENCES 1. PORTER, G. A., BERROTH, M. and BRISTOW, J. D.

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4.

5.

6.

Primary rhabdomyosarcoma of the heart and complete atrioventricular block. .4m. J. ivied., 31: 820. 1961. MANNIX, E. P., JR. and LUKASH, L. Primary rhabclomyosarcoma of the heart producing marked obstruction of the pulmonary valve. Bull. St. Francis Hosp.. 15: 14, 1958. SAPHIR, 0. Neoplasma of the pericardium and heart. In: Pathology of the Heart, cd. 2, p. 859. Edited by GOULD, S. E. Springfield, Ill.. 1960. Charles C Thomas. \VashSTOUT. il.P. Tumors of Soft ‘I’issue, p. 93. ington, D. C., 1953. Armed Forws Institutr of Pathology. HIRAMOTO. R., JURANDOWSKI, J.. BERNECKY, J. and PRESSMAN. D. Immunochemical differentiation of rhabdomyosarcoma. Cuzr~r Rcs.. 21 : 383. 1961. RUMERY. R. E.. BLANDAU. R. J. and HAGEY, P. Observations on living myocardial ~11s from culturcd forty-tight hour chick hearts. .4 nnt Record. 141: 253, 1961.