Atrial Myxoma with Intellectual Decline and Cerebral Growths on CT Scan Robert A. Frank, MD, Philip R. Shalen, MD, Danny G. Harvey, MD, Leonard Berg, MD, Thomas B. Ferguson, MD, and Henry G . Schwartz, M D
A w o m a n w i t h left atrial myxoma had progressive disturbance of intellectual function, headache, and prominent constitutional symptoms. The absence of stroke is noteworthy. Multiple high-density lesions w i t h contrast enhancement were seen b y CT scan, suggesting metastatic neoplasms, hematomas, or hemorrhagic infarcts. Serial study showed progressive enlargement of t h e lesions. The only cerebral lesion examined histologically proved to be an organizing hematoma, originating most likely from adjacent small arteries f o u n d to b e p l u g g e d b y embolic myxoma. Frank RA, Shalen PR, Harvey DG, et al: Atrial myxoma with intellectual decline and cerebral growths on C T scan. Ann Neurol 5:396-400, 1979
D e s p i t e t h e rarity of primary cardiac tumors, the neurological literature has urged consideration of atrial m y x o m a as a “curable” cause of s t r o k e , unexplained syncope, or periodic multiple cerebral deficits. T h i s case study emphasizes that atrial m y x o m a also can appear as progressive deterioration in intellectual function w i t h o u t stroke. C o m p u t e r i z e d tomography showed multiple enlarging cerebral lesions, o n e of which was r e m o v e d and l e d eventually to t h e proper diagnosis. A 49-year-old right-handed black woman was admitted on September 24, 1977, with a six-month history of progressive defects in memory and concentration. For three months she had had throbbing bifrontal headaches and was told she was “anemic.” A tender morbilliform rash was present on the soles for a month, then disappeared. Two months before admission, she awakened once with vertigo and vomiting. In August, she developed unsteadiness of gait and ceased driving because she found herself repeatedly crossing the center line. During September, she was intermittently febrile and intellectually unable to per~~
~~~
~
From the Departments of Neurology and Neurological Surgery, Radiology, Pathology, and Surgery (Cardiothoracic Surgery), Washington University Medical Center, St. Louis, MO. Accepted for publication Sept 19, 1978. Address reprint requests to Dr Frank, Department of Neurology and Neurosurgery, Washington University School of Medicine, Box 8111, 600 S Euclid, St. Louis, MO 631 10.
form her duties as a school teacher. She had noted two episodes of visual disturbance described as “a gray veil coming over the eyes,” lasting about 5 minutes. She had lost 9 kg. O n admission the patient was alert but slow in her responses. She was disoriented for dates and was unable to name presidents or perform calculations. She interpreted proverbs concretely. There were no aphasic or apraxic errors. H e r gait was shuffling and broad based. A right inferior homonymous quadrantanopia was found. Cardiac examination, including electrocardiogram, was normal. Laboratory results demonstrated microcytic, hypochromic anemia (hemoglobin, 8.5 to 9.0 g d d l ) and leukocytosis ( 14,000 to 22,000/mm3). The erythrocyte sedimentation rate (ESR) was elevated at 65 to 123 (normal. to 20 mm per hour). The following laboratory results were all negative or within normal limits: tests for hemolysis, lupus erythematosus preparations, antinuclear antibody, rheumatoid factor, serum protein electrophoresis, anti-DNA antibody, purified protein derivative, and multiple blood cultures. A C T scan showed multiple wellcircumscribed high-density contrast-enhancing lesions in the posterior regions of both cerebral hemispheres (Fig lA,B), varying in size from 0.3 to 2.0 cm. The larger masses showed diminished attenuation in their central portions. Extensive areas of low density (interpreted as edema) were present in the white matter of both cerebral hemispheres. The left lateral ventricle was compressed. The radionuclide brain scan showed areas of increased uptake in the right posterior parietal and left occipital regions. The cerebrospinal fluid was entirely normal (pressure, 180 mm CSF). Radiological examinations showed no primary malignancy. Cerebral angiography vaguely outlined multiple mass lesions by displaced arterial and venous structures. O n e or two smaller branches in the middle cerebral tree on each side appeared partially obstructed as though encased by tumor. These branches emptied slowly. One neuroradiologist interpreted the study as showing arterial occlusions, but the consensus favored mass effect alone. No tumor vasculature or stain was demonstrated. There was neither aneurysm formation nor evidence for vasculitis. A left posterior parietal craniotomy was performed on October 12. Gyral flattening and yellow discoloration were noted. A subcortical mass 1.5 cm in diameter was removed; it was nearly black and grossly reminiscent of melanoma. Frozen section of a tissue sample was interpreted as containing neoplasm, consistent with metastatic carcinoma. Permanent section of one of the tissue blocs had the gross and microscopic appearance of an organizing blood clot. Its wall was composed of dense fibrous tissue containing many hemosiderin-laden macrophages and mineralized vessels. Numerous blood and fibrin lakes were identified within the center. Actively proliferating vascular endorhelial cells were conspicuous. Three small vessels at the periphery of the clot were occluded by “myxoid” tissue containing nests of cells with pleomorphic nuclei and variable amounts of cytoplasm (Fig 2). The rnyxoid tissue invaded the muscularis of two vessels. The hisropathological interpretation was “hematoma in varying stages of organization”; it was suggested that the myxoid tissue represented one pattern
396 0364-5 134/79/040396-05$01.25 @ 1978 by Leonard Berg
F i g I . Senul CT scans (A) September 27, 1977, before contrust; IB) September 27, contrast-enbanred. (C) November 12, poJtoperutrte, rontrat-enhanied; note t b u t one Ierron b a s been exrijed. ( 0 )Mrlrrh 21 1978, contrrlst-enhanced. I
that an organizing thrombus may assume 1101. The nests of cells with pleomorphic nuclei had been misinterpreted on frozen section as metastatic carcinoma. Postoperatively, the patient had a persistent right homonymous hemianopia and a transient right hemiparesis. A repeat C T scan (Fig 1C) showed that o n e of the lesions had been excised, but it also showed an increase in the size of the adjacent lesions. Because of the myxoid tissue seen in the biopsy specimen, an echocardiogram was performed which demonstrated a decrease in the E-F slope and a group of echos behind the anterior leaflet of the mitral valve, findings classic for atrial myxoma. O n October 27 a 7 X 4 cm tumor was removed with its pedicle, which w d S attached to the superior rim of the foramen ovale. Histological examination revealed cords and nests of cells set in a pale, delicate stroma that was faintly positive with periodic acid-Schiff staining (Fig 3 ) . Numerous thin-walled vessels were present, surrounded by nests of cells identical to those seen in the specimen from the brain. Nuclear
pleomorphism was conspicuous, and several mitotic figures were seen. These findings fulfilled the histological criteria of cardiac myxoma. The patient was discharged o n November 16 on a warfarin regimen. In February she experienced three focal motor seizures involving the left side of her face. In early March, headaches reappeared, the right hemiparesis increased, and her mental status deteriorated. When the patient entered the hospital again o n March 14, a repeat C T scan showed six high-density lesions with intense contrast enhancement. T h e previous lesions had continued to enlarge, and new lesions were recognized in the right hemisphere. Pertinent studies included normal hemoglobin, ESR, and echocardiogram. Warfarin was discontinued and dexamethasone was given. Nevertheless, on March 2 1 another C T scan (Fig 1D) demonstrated marked mass effect with left-to-right shift. T h e patient died o n March 2 3 . Permission for postmortem examination was denied.
Discussion This woman sought aid for progressive impairment of intellectual function, headache, and fever. There were a few minor episodes of transient focal neuro-
Case Report: Frank et al: Intellectual Loss and CT Scan in Myxoma
397
logical symptoms. Anemia, elevated ESR, and multiple lesions seen o n C T scan suggested disseminated malignancy, vasculitis, or subacute bacterial endocarditis. Only after lengthy study of the mass excised from the brain was the diagnosis of atrial myxoma suggested. Even then, clinical examination of the heart was normal. Three patterns of clinical presentation are observed in patients with atrial myxoma: obstructive, systemic, and ernbolic [2, 61. Cardiac murmurs, pulmonary hypertension, and severe dyspnea are common obstructive effects. Constitutional effects include fever, weight loss, clubbing of fingers, anemia, leukocytosis, increased ESR, and abnormal serum proteins (including hypergammaglobulinemia). Factors responsible for these constitutional disturbances are not established, but mechanical hemolysis, release of mucopolysaccharide from the myxoma, and tumor emboli to muscle have all been proposed as explanations [21. Neurologists have been particularly interested in the embolic manifestations of this neoplasm. U p to 50c4 of atrial myxornas give rise to clinical emboli, of which half affect the brain [2]. Other authors have emphasized the need to consider this “curable” entity in young persons with stroke [ 4 , 111. Neurological
398 Annals of Neurology Vol 5 No 4 April 1979
F i g 2 . Ocrlirsion of tmall cortical cessel by myxomatoiis tissue. Note pale, amorphoris stroma, rfirmps of myxoma cells, and per.tr~tenreof tniafflrrmcn. ( H 6 E . X250.j
manifestations other than stroke have also been documented: syncope, seizures with right atrial myxoma [ 3 ] , and spinal cord embolism [13]. Disturbances of thinking have been noted previously with myxoma [ 5 , 121, but their description has been fragmentary. T h e terms “impaired mental status,” “mental deterioration,” and “depression and confusion” have been employed to describe these disturbances. T h e prominence of our patient’s intellectual deterioration with little early evidence of other cerebral dysfunction is noteworthy. Angiographic features of cerebral emboli from atrial myxoma have been described [5]. These include nonspecific findings of multiple vascular occlusions, prolonged circulation time, collateral flow, and early venous filling. More specific angiographic changes are multiple fusiforrn or saccular aneurysmal dilatations, or both. These are thought to be caused by continued growth of viable tumor emboli in the vascular endothelium with progressive destruction of the arterial wall. Cerebral angiography in our case demonstrated only nonspecific findings.
Fig 3 . Myxomu remowdjrom feh atrium. Note striking similarity to myxomatous ti.r.rnein FiRure 2. (H&E, X250.1
Discovery of mass lesions by C T scan had not previously been reported in intracerebral myxoma, but during the preparation of this paper we learned of a case report in press [7]. That patient had a large but histologically benign myxoma attached to the choroid plexus of a lateral ventricle. The mass had a high density on CT scan and was enhanced by contrast material (Rankin LI: personal communication, 1978). It was successfully removed eight years after resection of a left atrial myxoma. In our patient, multiple high-density lesions with contrast enhancement were demonstrated by C T scan. The one mass that was excised proved to be an organizing hematoma. Numerous disorders can be included in the differential diagnosis of this C T appearance. Multiple hematomas, hemorrhagic metastases, and hemorrhagic infarcts are the most common; less likely possibilities are multiple meningiomas and multiple foci of cerebral calcification. It has been said [ 11 that metastasis may be distinguished from hemorrhagic infarct by its well-circumscribed contour and more homogeneous density, but these
are not absolute criteria. Metastases from certain primary lesions, including metastatic melanoma, choriocarcinoma, and colonic adenocarcinoma, are likely to appear denser than brain tissue on C T scan. However, other metastases may have a similar appearance. Our biopsy material demonstrated myxomatous emboli and adjacent hematoma in various stages of organization. Increase in size of lesions demonstrated by CT scan and the appearance of “new” masses are best explained by additional episodes of hemorrhage through arterial walls invaded by myxoma (a mechanism possibly promoted by postoperative warfarin). Less likely explanations for the rapidly enlarging lesions are malignant myxomatous growths or unrelated metastatic neoplasms. In the absence of postmortem evidence, we can only point to the excised organizing hematoma, which had a CT scan appearance identical to that of the other masses. Although most authors accept myxoma as a neoplastic lesion, there are cogent opinions that myxomas arise as organizing thrombi [9]. The neoplasms are usually judged benign, but recurrences at sites of previously excised myxoma have been documented on several occasions, and isolated reports establish that myxomas may be malignant [8].
Case Report: Frank et al: Intellectual Loss and CT Scan in Myxorna 399
Cerebral symptoms associated with atrial myxoma may result from cardiogenic syncope, bland or infected thromboemboli, or myxomatous emboli. Myxomatous emboli may invade arterial walls and give rise to progressive arterial changes or hemorrhages with progressive clinical deterioration, as in our patient. In other rare instances, a large intracerebra1 myxoma may result from subsequent growth of an embolus [71. A malignant cardiac myxoma may give rise to enlarging metastatic deposits elsewhere [81, but we have been unable to find in the literature documentation of malignant myxoma deposits growing in the brain.* A wide spectrum of clinical pictures can be expected, but the constitutional effects and CT appearance should be valuable clues to diagnosis. 'A case mentioned in the discussion of Read et a1 [8]as "a malignant tumor which metastasized to the brain" had only multiple infarcts in the brain (Sterns LP: personal communication, 1978). We thank James W. Walsh, MD, who referred the patient to us.
References 1. Davis KR, Taveras JM, New PFJ, et al: Cerebral infarction diagnosis by computerized tomography. Am J Roentgen01 Radium Ther Nucl Med 124:643-660, 1975
400 Annals of Neurology Vol 5 N o 4 April 1979
2. Goodwin JF: The spectrum of cardiac tumors. Am J Cardiol 21:307-314, 1968 3. Kendall D, Symonds B: Epileptiform attacks due to myxoma of the right auricle. Br Heart J 14:139-143, 1952 4. Maroon JC, Campbell R L Atrial myxoma: a treatable cause of stroke. J Neurol Neurosurg Psychiatry 32: 129-133, 1969 5. New PFJ, Price DL, Carter B:Cerebral angiography in cardiac myxoma: correlation of angiographic and histopathological findings. Radiology 96:335-345, 1970 6. Peters MN, Hall RJ, Cooley DA, et al: The clinical syndrome of atrial myxoma. JAMA 230:695-701, 1974 7. Rankin LI, DeSousa A L Metastatic atrial myxoma presenting as an intracranial mass lesion. Chest 74:451-452, 1978 8. Read RC, White HJ, Murphy ML, et al: The malignant potentiality of left atrial myxoma. J Thorac Cardiovasc Surg 68:857-868, 1974 9. Salyer WR, Page DL, Hutchins GM: The development of cardiac myxomas and papillary endocardial lesions from mural thrombus. Am Heart J 894-17, 1975 10. Salyer WR, Salyer DC: Myxoma-like features of organizing thrombi in arteries and veins. Arch Pathol 99:307-311, 1975 11. Schwarz GA, Schwartzman RJ, Joyner CR: Atrial myxoma: cause of embolic stroke. Neurology (Minneap) 22:11121121, 1972 12. Steinmetz EF, Calanchine PR, Aguilar MJ: Left atrial myxoma as a neurological problem: a case report and review. Stroke 4:451-458, 1973 13. Wolman L, Bradshaw P: Spinal cord embolism. J Neurol Neurosurg Psychiatry 30:446-454, 1967
A w o m a n w i t h left atrial myxoma had progressive disturbance of intellectual function, headache, and prominent constitutional symptoms. The absence of stroke is noteworthy. Multiple high-density lesions w i t h contrast enhancement were seen b y CT scan, suggesting metastatic neoplasms, hematomas, or hemorrhagic infarcts. Serial study showed progressive enlargement of t h e lesions. The only cerebral lesion examined histologically proved to be an organizing hematoma, originating most likely from adjacent small arteries f o u n d to b e p l u g g e d b y embolic myxoma. Frank RA, Shalen PR, Harvey DG, et al: Atrial myxoma with intellectual decline and cerebral growths on C T scan. Ann Neurol 5:396-400, 1979
D e s p i t e t h e rarity of primary cardiac tumors, the neurological literature has urged consideration of atrial m y x o m a as a “curable” cause of s t r o k e , unexplained syncope, or periodic multiple cerebral deficits. T h i s case study emphasizes that atrial m y x o m a also can appear as progressive deterioration in intellectual function w i t h o u t stroke. C o m p u t e r i z e d tomography showed multiple enlarging cerebral lesions, o n e of which was r e m o v e d and l e d eventually to t h e proper diagnosis. A 49-year-old right-handed black woman was admitted on September 24, 1977, with a six-month history of progressive defects in memory and concentration. For three months she had had throbbing bifrontal headaches and was told she was “anemic.” A tender morbilliform rash was present on the soles for a month, then disappeared. Two months before admission, she awakened once with vertigo and vomiting. In August, she developed unsteadiness of gait and ceased driving because she found herself repeatedly crossing the center line. During September, she was intermittently febrile and intellectually unable to per~~
~~~
~
From the Departments of Neurology and Neurological Surgery, Radiology, Pathology, and Surgery (Cardiothoracic Surgery), Washington University Medical Center, St. Louis, MO. Accepted for publication Sept 19, 1978. Address reprint requests to Dr Frank, Department of Neurology and Neurosurgery, Washington University School of Medicine, Box 8111, 600 S Euclid, St. Louis, MO 631 10.
form her duties as a school teacher. She had noted two episodes of visual disturbance described as “a gray veil coming over the eyes,” lasting about 5 minutes. She had lost 9 kg. O n admission the patient was alert but slow in her responses. She was disoriented for dates and was unable to name presidents or perform calculations. She interpreted proverbs concretely. There were no aphasic or apraxic errors. H e r gait was shuffling and broad based. A right inferior homonymous quadrantanopia was found. Cardiac examination, including electrocardiogram, was normal. Laboratory results demonstrated microcytic, hypochromic anemia (hemoglobin, 8.5 to 9.0 g d d l ) and leukocytosis ( 14,000 to 22,000/mm3). The erythrocyte sedimentation rate (ESR) was elevated at 65 to 123 (normal. to 20 mm per hour). The following laboratory results were all negative or within normal limits: tests for hemolysis, lupus erythematosus preparations, antinuclear antibody, rheumatoid factor, serum protein electrophoresis, anti-DNA antibody, purified protein derivative, and multiple blood cultures. A C T scan showed multiple wellcircumscribed high-density contrast-enhancing lesions in the posterior regions of both cerebral hemispheres (Fig lA,B), varying in size from 0.3 to 2.0 cm. The larger masses showed diminished attenuation in their central portions. Extensive areas of low density (interpreted as edema) were present in the white matter of both cerebral hemispheres. The left lateral ventricle was compressed. The radionuclide brain scan showed areas of increased uptake in the right posterior parietal and left occipital regions. The cerebrospinal fluid was entirely normal (pressure, 180 mm CSF). Radiological examinations showed no primary malignancy. Cerebral angiography vaguely outlined multiple mass lesions by displaced arterial and venous structures. O n e or two smaller branches in the middle cerebral tree on each side appeared partially obstructed as though encased by tumor. These branches emptied slowly. One neuroradiologist interpreted the study as showing arterial occlusions, but the consensus favored mass effect alone. No tumor vasculature or stain was demonstrated. There was neither aneurysm formation nor evidence for vasculitis. A left posterior parietal craniotomy was performed on October 12. Gyral flattening and yellow discoloration were noted. A subcortical mass 1.5 cm in diameter was removed; it was nearly black and grossly reminiscent of melanoma. Frozen section of a tissue sample was interpreted as containing neoplasm, consistent with metastatic carcinoma. Permanent section of one of the tissue blocs had the gross and microscopic appearance of an organizing blood clot. Its wall was composed of dense fibrous tissue containing many hemosiderin-laden macrophages and mineralized vessels. Numerous blood and fibrin lakes were identified within the center. Actively proliferating vascular endorhelial cells were conspicuous. Three small vessels at the periphery of the clot were occluded by “myxoid” tissue containing nests of cells with pleomorphic nuclei and variable amounts of cytoplasm (Fig 2). The rnyxoid tissue invaded the muscularis of two vessels. The hisropathological interpretation was “hematoma in varying stages of organization”; it was suggested that the myxoid tissue represented one pattern
396 0364-5 134/79/040396-05$01.25 @ 1978 by Leonard Berg
F i g I . Senul CT scans (A) September 27, 1977, before contrust; IB) September 27, contrast-enbanred. (C) November 12, poJtoperutrte, rontrat-enhanied; note t b u t one Ierron b a s been exrijed. ( 0 )Mrlrrh 21 1978, contrrlst-enhanced. I
that an organizing thrombus may assume 1101. The nests of cells with pleomorphic nuclei had been misinterpreted on frozen section as metastatic carcinoma. Postoperatively, the patient had a persistent right homonymous hemianopia and a transient right hemiparesis. A repeat C T scan (Fig 1C) showed that o n e of the lesions had been excised, but it also showed an increase in the size of the adjacent lesions. Because of the myxoid tissue seen in the biopsy specimen, an echocardiogram was performed which demonstrated a decrease in the E-F slope and a group of echos behind the anterior leaflet of the mitral valve, findings classic for atrial myxoma. O n October 27 a 7 X 4 cm tumor was removed with its pedicle, which w d S attached to the superior rim of the foramen ovale. Histological examination revealed cords and nests of cells set in a pale, delicate stroma that was faintly positive with periodic acid-Schiff staining (Fig 3 ) . Numerous thin-walled vessels were present, surrounded by nests of cells identical to those seen in the specimen from the brain. Nuclear
pleomorphism was conspicuous, and several mitotic figures were seen. These findings fulfilled the histological criteria of cardiac myxoma. The patient was discharged o n November 16 on a warfarin regimen. In February she experienced three focal motor seizures involving the left side of her face. In early March, headaches reappeared, the right hemiparesis increased, and her mental status deteriorated. When the patient entered the hospital again o n March 14, a repeat C T scan showed six high-density lesions with intense contrast enhancement. T h e previous lesions had continued to enlarge, and new lesions were recognized in the right hemisphere. Pertinent studies included normal hemoglobin, ESR, and echocardiogram. Warfarin was discontinued and dexamethasone was given. Nevertheless, on March 2 1 another C T scan (Fig 1D) demonstrated marked mass effect with left-to-right shift. T h e patient died o n March 2 3 . Permission for postmortem examination was denied.
Discussion This woman sought aid for progressive impairment of intellectual function, headache, and fever. There were a few minor episodes of transient focal neuro-
Case Report: Frank et al: Intellectual Loss and CT Scan in Myxoma
397
logical symptoms. Anemia, elevated ESR, and multiple lesions seen o n C T scan suggested disseminated malignancy, vasculitis, or subacute bacterial endocarditis. Only after lengthy study of the mass excised from the brain was the diagnosis of atrial myxoma suggested. Even then, clinical examination of the heart was normal. Three patterns of clinical presentation are observed in patients with atrial myxoma: obstructive, systemic, and ernbolic [2, 61. Cardiac murmurs, pulmonary hypertension, and severe dyspnea are common obstructive effects. Constitutional effects include fever, weight loss, clubbing of fingers, anemia, leukocytosis, increased ESR, and abnormal serum proteins (including hypergammaglobulinemia). Factors responsible for these constitutional disturbances are not established, but mechanical hemolysis, release of mucopolysaccharide from the myxoma, and tumor emboli to muscle have all been proposed as explanations [21. Neurologists have been particularly interested in the embolic manifestations of this neoplasm. U p to 50c4 of atrial myxornas give rise to clinical emboli, of which half affect the brain [2]. Other authors have emphasized the need to consider this “curable” entity in young persons with stroke [ 4 , 111. Neurological
398 Annals of Neurology Vol 5 No 4 April 1979
F i g 2 . Ocrlirsion of tmall cortical cessel by myxomatoiis tissue. Note pale, amorphoris stroma, rfirmps of myxoma cells, and per.tr~tenreof tniafflrrmcn. ( H 6 E . X250.j
manifestations other than stroke have also been documented: syncope, seizures with right atrial myxoma [ 3 ] , and spinal cord embolism [13]. Disturbances of thinking have been noted previously with myxoma [ 5 , 121, but their description has been fragmentary. T h e terms “impaired mental status,” “mental deterioration,” and “depression and confusion” have been employed to describe these disturbances. T h e prominence of our patient’s intellectual deterioration with little early evidence of other cerebral dysfunction is noteworthy. Angiographic features of cerebral emboli from atrial myxoma have been described [5]. These include nonspecific findings of multiple vascular occlusions, prolonged circulation time, collateral flow, and early venous filling. More specific angiographic changes are multiple fusiforrn or saccular aneurysmal dilatations, or both. These are thought to be caused by continued growth of viable tumor emboli in the vascular endothelium with progressive destruction of the arterial wall. Cerebral angiography in our case demonstrated only nonspecific findings.
Fig 3 . Myxomu remowdjrom feh atrium. Note striking similarity to myxomatous ti.r.rnein FiRure 2. (H&E, X250.1
Discovery of mass lesions by C T scan had not previously been reported in intracerebral myxoma, but during the preparation of this paper we learned of a case report in press [7]. That patient had a large but histologically benign myxoma attached to the choroid plexus of a lateral ventricle. The mass had a high density on CT scan and was enhanced by contrast material (Rankin LI: personal communication, 1978). It was successfully removed eight years after resection of a left atrial myxoma. In our patient, multiple high-density lesions with contrast enhancement were demonstrated by C T scan. The one mass that was excised proved to be an organizing hematoma. Numerous disorders can be included in the differential diagnosis of this C T appearance. Multiple hematomas, hemorrhagic metastases, and hemorrhagic infarcts are the most common; less likely possibilities are multiple meningiomas and multiple foci of cerebral calcification. It has been said [ 11 that metastasis may be distinguished from hemorrhagic infarct by its well-circumscribed contour and more homogeneous density, but these
are not absolute criteria. Metastases from certain primary lesions, including metastatic melanoma, choriocarcinoma, and colonic adenocarcinoma, are likely to appear denser than brain tissue on C T scan. However, other metastases may have a similar appearance. Our biopsy material demonstrated myxomatous emboli and adjacent hematoma in various stages of organization. Increase in size of lesions demonstrated by CT scan and the appearance of “new” masses are best explained by additional episodes of hemorrhage through arterial walls invaded by myxoma (a mechanism possibly promoted by postoperative warfarin). Less likely explanations for the rapidly enlarging lesions are malignant myxomatous growths or unrelated metastatic neoplasms. In the absence of postmortem evidence, we can only point to the excised organizing hematoma, which had a CT scan appearance identical to that of the other masses. Although most authors accept myxoma as a neoplastic lesion, there are cogent opinions that myxomas arise as organizing thrombi [9]. The neoplasms are usually judged benign, but recurrences at sites of previously excised myxoma have been documented on several occasions, and isolated reports establish that myxomas may be malignant [8].
Case Report: Frank et al: Intellectual Loss and CT Scan in Myxorna 399
Cerebral symptoms associated with atrial myxoma may result from cardiogenic syncope, bland or infected thromboemboli, or myxomatous emboli. Myxomatous emboli may invade arterial walls and give rise to progressive arterial changes or hemorrhages with progressive clinical deterioration, as in our patient. In other rare instances, a large intracerebra1 myxoma may result from subsequent growth of an embolus [71. A malignant cardiac myxoma may give rise to enlarging metastatic deposits elsewhere [81, but we have been unable to find in the literature documentation of malignant myxoma deposits growing in the brain.* A wide spectrum of clinical pictures can be expected, but the constitutional effects and CT appearance should be valuable clues to diagnosis. 'A case mentioned in the discussion of Read et a1 [8]as "a malignant tumor which metastasized to the brain" had only multiple infarcts in the brain (Sterns LP: personal communication, 1978). We thank James W. Walsh, MD, who referred the patient to us.
References 1. Davis KR, Taveras JM, New PFJ, et al: Cerebral infarction diagnosis by computerized tomography. Am J Roentgen01 Radium Ther Nucl Med 124:643-660, 1975
400 Annals of Neurology Vol 5 N o 4 April 1979
2. Goodwin JF: The spectrum of cardiac tumors. Am J Cardiol 21:307-314, 1968 3. Kendall D, Symonds B: Epileptiform attacks due to myxoma of the right auricle. Br Heart J 14:139-143, 1952 4. Maroon JC, Campbell R L Atrial myxoma: a treatable cause of stroke. J Neurol Neurosurg Psychiatry 32: 129-133, 1969 5. New PFJ, Price DL, Carter B:Cerebral angiography in cardiac myxoma: correlation of angiographic and histopathological findings. Radiology 96:335-345, 1970 6. Peters MN, Hall RJ, Cooley DA, et al: The clinical syndrome of atrial myxoma. JAMA 230:695-701, 1974 7. Rankin LI, DeSousa A L Metastatic atrial myxoma presenting as an intracranial mass lesion. Chest 74:451-452, 1978 8. Read RC, White HJ, Murphy ML, et al: The malignant potentiality of left atrial myxoma. J Thorac Cardiovasc Surg 68:857-868, 1974 9. Salyer WR, Page DL, Hutchins GM: The development of cardiac myxomas and papillary endocardial lesions from mural thrombus. Am Heart J 894-17, 1975 10. Salyer WR, Salyer DC: Myxoma-like features of organizing thrombi in arteries and veins. Arch Pathol 99:307-311, 1975 11. Schwarz GA, Schwartzman RJ, Joyner CR: Atrial myxoma: cause of embolic stroke. Neurology (Minneap) 22:11121121, 1972 12. Steinmetz EF, Calanchine PR, Aguilar MJ: Left atrial myxoma as a neurological problem: a case report and review. Stroke 4:451-458, 1973 13. Wolman L, Bradshaw P: Spinal cord embolism. J Neurol Neurosurg Psychiatry 30:446-454, 1967
