ARTICLE
Left atrial myxomas are the most frequent tumors of the heart in adults.
The clinical features of these tumors are principally intracardiac obstruction,
extracardiac embolism and general symptoms. We report a case of a woman
who presented successively left hemiplegia with bilateral hyperdensity
in parieto-occipital areas, splenic and renal infarction and constitutional
disturbances initially evoking tumor metastases. The appearance of cutaneous
lesions related to embolism phenomenon permitted us to diagnose this heart
tumor.
Case report
An obese 53-year-old woman (95 kg/1.60 m) was hospitalized in January
1996 for left hemiplegia. Cerebral computed tomography showed bilateral
hyperdensity in parieto-occipital areas with focal oedema with enhancing
of the lesions after contrast injection. Haemorrhagic brain metastases
were initially suspected. A search for primary neoplasia was negative
and corticosteroid treatment improved her symptoms. Four months later,
she presented with fatigue, fever, arthralgia, myalgia, weight loss and
laboratory abnormalities. White blood cell count was 15,0000/cu mm (10,000
granulocytes/cu mm), hemoglobin was 8.5 g/dl, erythrocyte sedimentation
rate (ESR) was 109 mm/hr, and CRP level was 266 mg/ml (normal < 15).
Thoracic and abdominal computed tomography revealed renal and splenic
infarction features. She also complained of painful violaceous papulosis
on the fingers and toes from which she had been suffering for the last
month (Fig. 1); there
was no evidence of livedo reticularis nor Raynaud's phenomenon. A cutaneous
biopsy specimen showed thrombosis of dermal vessels with wall destruction
and neo-angiogenesis. All these features led to a suspicion of endocarditis
but transoesophageal echocardiography showed a left atrial tumor evoking
myxoma : this diagnosis was confirmed by surgical removal and histopathological
examination. So, we diagnosed a left atrial myxoma complicated by extracardiac
embolism (cerebral, renal, splenic, cutaneous) and general disturbances.
Multiple slides of the cutaneous biopsy specimen could not find myxoid
intravascular deposit but the cutaneous lesions were quite old (15 days)
and thrombosis and neoangiogenesis were predominant. Cutaneous lesions
completely disappeared in 3 days after removal of the tumor. For the cerebral
lesions, the computed tomography aspect of the lesions (hyperdensities
without contrast injection and enhancing of the lesions after contrast
injection) suggested multiple cerebral aneurysms related to myxoma fragments
embolism. We didn't find any symptoms of more complex syndromes such as
NAME syndrome, LAMB syndrome or Carney syndrome.
Discussion
Left atrial myxomas are the most frequent tumors of the heart in adults
and occur most frequently in the left atrium (75%) between the third and
sixth decades of life. The clinical features of these tumors are principally
intracardiac obstruction, extracardiac embolism and general symptoms [1].
Embolism occurs in about 35 percent of patients with myxomas. The cerebral
arteries are principally affected with ischemia manifestations but multiple
cerebral aneurysms and metastases have also been described [2]. Involvement
of the retinal arteries and embolization into peripheral (renal, splenic,
coronary...) arteries can occur [1, 2].
General disturbances (fatigue, fever, weight
loss) and laboratory abnormalities (anemia, elevation in ESR, CRP and
globulin levels) are often observed suggesting an infection such as endocarditis,
immunological disorders (vasculitis or collagen disease) or malignant
disease as in our case. Recent findings suggest that the production of
interleukin-6 (IL-6) by the tumor itself may be responsible for the inflammatory
and auto-immune manifestations [3, 9]. In our case, measurement out of
IL-6 levels in the sera before and after surgical removal of the tumor
was not done and immuno-histochemistry of the heart tumor using mouse
monoclonal antibody against human IL-6 (Immunotech) was negative.
The cutaneous manifestions of myxomas are often reported and can be
classified in three types:
First, cutaneous manifestations of emboli include
more frequently acral erythematous papular eruption, splinter hemorrhages,
necrosis of the toes and livedo reticularis. Cutaneous biopsy usually
shows thrombosis and neoangiogenesis in the dermis. Myxoid intravascular
deposit within the vessels of the dermis is not always found. This is
perhaps particularly the case when the cutaneous biopsy is performed late.
These manifestations disappear when the tumor is removed [4, 7].
Second, cutaneous manifestations may be related to auto-immune symptoms
like Raynaud's phenomenon, malar erythematous, vasculitis: this can be
partially explained by IL-6 secretion of the tumor [3, 9]. IL-6 acts on
a wide variety of cells, regulating immune response, acute phase reaction
and hematopoieisis and can be involved in several diseases including autoimmunities,
lymphoid malignancies and inflammations [8]. Regression of these cutaneous
and general symtoms is usual after removal of the heart tumor but a prolonged
elevation of antinuclear antibodies has been described in a patient after
extirpation of the myxoma [10]. Moreover, a case of cutaneous vasculitis
with circulating antiphospholipid antibody has been reported, revealing
left atrial myxoma [11].
Third, specific cutaneous findings suggest the diagnosis of atrial myxoma
as part of a more complex syndrome that can be sporadic or hereditary.
Different syndromes have been described such as NAME syndrome (Naevus,
Atrial Myxoma, Ephelides), LAMB syndrome (Lentigines, Atrial Myxoma, Blue
nevi) [4, 12]. Finally, the Carney complex is characterized by myxomas,
schwannomas, mottled pigmentation and endocrine hyperfunction: a linkage
to the short arm of chromosome 2 and cytogenetic and microsatellite alterations
in tumors from patients have been recently reported [13, 15]. However,
alternative interpretations for NAME syndrome have been proposed and a
new term "familial endocrine myxolentiginosis" has been proposed, which
is descriptive of the major components of the syndrome that we did not
find in our patient [16].
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