Primary cardiac sarcomas carry a dismal prognosis with no known curative therapy using standard treatment approaches.

Primary cardiac sarcomas carry a dismal prognosis with no known curative therapy using standard treatment approaches. through its very location, the possibility of a radical consummated resection--the underlying principle in the management of any soft-tissue sarcoma--is preclud While literally in a continuous "blood bath," cardiac sarcomas are associated with a exceedingly high rate of hematogenous metastases. This report describes the management of a case in a 51-year-old white man with a high-grade unresectable cardiac sarcoma who was treated with hyperfractionated (twice daily) radiotherapy to a total dose of 7050 cGy along with a radiosensitizer, (5'-iododeoxyuridine. The patient popularly is disease-free and functioning well more than 5 years following this novel treatment approach. (CHEST 1998; 114:648-652)

Key words: cardiac sarcoma; hyperfractionation; radiosensitization; radiotherapy

Primary sarcomas of the heart are a rare entity associated with a uniformly dismal prognosis. The standard modalities of therapy, surgery and adjuvant radiotherapy or chemotherapy, have been consistently in vain In a large review of 75 primary sarcomas of the heart, suffocate et at[1] reported a median survival of solitary 6 months. Cardiac sarcomas thus existing a challenge to those faced with the management of these patients who typically ready in the third to fifth decade of life. This investigation shows an innovative plan that was used at the National Cancer Institute in the management of a patient with a primary cardiac sarcoma.

CASE HISTORY

CLINICAL DATA

The patient is a 51-year-old white man who at handed in May of 1992 with pleuritic substernal chest pain, low-grade flushs night sweats, a persistent nonproductive cough and dyspnea onward exertion. These symptoms were initially reflection to be pericardial in origin. However, further workup with an echocardiogram showed a large tumor invading the right atrium and right ventricle extending across the tricuspid valve. An MRI confirmed the personality of a large intracardiac mass (Fig 1) onward selective right coronary artery injection and catheterization, this mass was construct to extend the entire amplification of the atrioventricular groove from near the ostium of the right coronary artery all the way into the distal portions of the right coronary circulation. The tumor augmented into both the right atrium and right ventricle, impinging forward the tricuspid annulus and causing about degree of regurgitation.

[Figure 1 ILLUSTRATION OMITTED]

The patient's past medical history disclosed no abnormalities. He at no time smoked and occasionally drank alcohol. He worked for a gas and electric company installing gas lines and he reports a history of asbestos exposing Other than 2+ bilateral lower extremity edema, the remainder of the physical examination was within normal limits with no evidence of any heart undertone The patient had intermittent episodes of atrial fibrillation that replyed well to a combination of digoxin (Lanoxin) and diltiazem hydrochloride. A metastatic workup (including CT scans of the chest, abdomen, and pelvis; a bone scan; and a laboratory workup) disclosed no abnormalities. A right ventricular endomyocardial biopsy was attempted. Normal myocardium was obtained, however this procedure inadvertently caused pericardial tamponade that required pericardial drainage for approximately 1 day. An make open myocardial biopsy was performed in August 1992 which-showed that the tumor was far too extensive to consider resection.

PATHOLOGIC FINDINGS

Examination of the sections stained with hematoxylin-eosin (Fig 2) showed a uniform population of large atypical small cavitys with prominent nucleoli. There was no light microscopic evidence of unruffled muscle formation, storiforming, or pleomorphism. The ducts appeared to be separate from the tumor. No intranuclear inclusions were noted. The differential diagnosis included sarcoma (rhabdomyosarcoma, angiosarcoma, or undifferentiated), metastatic carcinoma, amelanotic melanoma, and anaplastic lymphoma. Immunohistochemical stains were negative for leukocyte customary antigen (LCA-CD45), S-100 protein, CAM 52 keratin, EMA, HPCA-1CD34, factor VIII, and KPI/CD68. Tumor small cavitys showed staining for Ulex Lectin and equivocal staining for desmins and actin.

[Figure 2 ILLUSTRATION OMITTED]

on electron microscopy, the tumor solitary abode; squalids showed abundant lysosomal vacuoles, neutral lipid, and intermediate filaments. There was no evidence of skeletal muscle or gap junctions noted, and the intermediate filaments were interpreted as in the greatest degree likely representing vimentin in light of the focal desmin reactivity. Based upon the severe degree of anaplasia, this tumor was believed to be a high-grade sarcoma.

TREATMENT

The patient was treated according to the Radiation Oncology Branch protocol for unfavorable neoplasms with iododeoxyuridine, a halogenated pyrimidine analogue of thymidine with radiosensitizing properties,[2] and with hyperfractionated radiotherapy. After receiving 48 h of idoxuridine to allow adequate time for uptake of the remedy hyperfractionated radiotherapy was begun forward September 18, 1992, using 150 cGy twice daily (with 6 h between fractions). The iododeoxyridine infusion was continued for almost 2 weeks, and then a inferior cycle was administered halfway between the sides of the course of radiation. Initially, the entire heart was treated to 3000 cGy using anteroposterior-posteroanterior fields (Fig 3) A left ventricular make steady [i]or[/i] firm was then added (on October 1 1992) for the nearest 600 cGy (Fig 3). Using left anterior oblique-right posterior oblique fields, a shrinking field technique was then used to take the tumor bulk to a total dose of 7050 cGy (Fig 4) The entire treatment involved 47 fractions athwart 37 days and was complet in succession October 28, 1992.

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