Transcript What is the diagnosis

Commenatry
case
By
Prof Dr /Fawzy Megahed
Asst lec /Rafaat Saied
• A 29-year-old a highly trained athlete man was
admitted to this hospital because of severe
dyspnea and chest pain on the right side.
• 2.5 weeks before admission, when he was hit
in the chest while he was at work; headache
and nausea occurred for 3 days thereafter.
• 2 weeks before admission, he awoke at night
with pain in the right mid axillary region that
he described as a “ping-pong ball”; the pain
radiated to and from the right scapula and
was associated with shortness of breath.
• a diagnosis of muscle spasm was made.
muscle relaxants and ibuprofen were
administered, without improvement.
• During the following days, increasing dyspnea
occurred with minimal exertion; the pain
became localized to the right anterior chest
and had a sharp, stabbing quality, and a non
productive cough developed.
• Three days before admission, during a 1.5hour flight, the patient noted having leg
cramps, which was a usual occurrence for him
on flights.
• On the morning of admission, he felt well on
awakening, but sudden stabbing chest pain in
the sternal area later developed, with
associated severe pressure in his chest and
transient palpitations.
• He also noted severe shortness of breath, with
increasing tachypnea and difficulty talking.
Chest pain increased with coughing, laughing,
and sneezing.
• In the evening, the patient went to another
hospital for evaluation, where he reported
sharp chest pain on the right side that
worsened with deep breaths.
• he rated the pain at 8 on a scale of 0 to 10,
with 10 indicating the most severe pain. He
attributed some of his symptoms to his earlier
chest injury
• he noted decreased appetite, with associated
weight loss of approximately 1.5 kg.
• He had had traumatic injury with a cleat to
the left calf approximately 3 months before
admission; the calf had had minor bruising,
without pain or swelling.
• Sixteen years earlier, the patient had had a
Staphylococcus aureus skin infection involving
his elbow that required débridement.
• Otherwise, he was healthy and physically
active, regularly participating in
semiprofessional sports.
• He had no known allergies.
• He drank alcohol occasionally and had never
smoked cigarettes or used illicit drugs.
• He reported no sick contacts or recent
hospitalizations.
• His maternal grandmother had a history of
systemic lupus erythematosus, hypertension,
strokes, myocardial infarction, and a deep
venous thrombosis, and his paternal
grandparents had had hypertension.
• His parents and siblings were healthy.
Examination
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the blood pressure was 137/93 mm Hg,
the pulse 86 beats per minute,
the respiratory rate 20 breaths per minute,
the oxygen saturation 98% while he was
breathing oxygen through a nasal cannula at a
rate of 2 liters per minute
• The carotid arteries had no bruits, and the
jugular venous pressure was 5 cm of water,
with a normal waveform.
• The lung sounds were slightly diminished
bilaterally, with crackles involving the lower
half of the right lung field but no dullness on
percussion.
• Auscultation revealed a normal first heart
sound (S1), a second heart sound with a loud
P2 component, a faint low-pitched diastolic
sound, and a systolic murmur (grade 2/6) at
the base.
• The heart rate and rhythm were regular.
• The left ventricular impulse was discrete and
nondisplaced, and there was a right
ventricular heave.
• There was no swelling, palpable mass or cord,
erythema, tenderness, or edema in the legs.
The remainder of the examination was
normal.
• Heparin was administered intravenously.
Approximately 4 hours after the patient’s
arrival at the other hospital, he was
transferred by ambulance to this hospital for
consideration of thrombolysis.
• On admission to this hospital, the patient
reported pleuritic chest pain that had
improved since earlier in the day. While he
had been taking ibuprofen for the muscle
spasms,
Investigation
• The hematocrit, hemoglobin level, red-cell
indexes, and blood levels of electrolytes,
glucose, calcium, total protein, albumin, total
bilirubin, alanine aminotransferase, and
alkaline phosphatase were normal; other test
results are shown in Table 1
• Urinalysis revealed trace albumin and was
otherwise normal.
• An ECG revealed normal sinus rhythm at a
rate of 78 beats per minute, with nonspecific
intraventricular conduction delay, evidence of
right-axis deviation, and no evidence of
ischemia.
• The blood levels,glucose, creatine kinase,
creatine kinase MB isoenzymes, troponin T, Nterminal pro–B-type natriuretic peptide (NTproBNP), fibrinogen, and total homocysteine
were normal.
• Heparin therapy was continued, and
intravenous fluids were administered.
• During the first hospital day, the blood
creatinine level and estimated glomerular
filtration rate normalized.
• Posteroanterior and lateral chest radiographs
showed a mass like opacity with ill-defined
borders posterior to the right hilum, as well as
prominent pulmonary arteries (Fig. 1A).
• (CT) angiography revealed a filling defect in
the main and right pulmonary arteries and a
mottled, wedge-shaped air-space opacity in
the posterior right lower lobe, findings
consistent with pulmonary embolism and
infarction.
•The next
• Transthoracic echocardiography
revealed a highly mobile irregular mass, nearly
10 cm in length, with a frond like appearance
and heterogeneous echotexture.
• The mass moved back and forth between the
right atrium and the right ventricle, and there
was a suggestion of possible attachment near
the tricuspid valve.
• The right ventricle was mildly dilated and
mildly hypokinetic in a manner that was
suggestive of ventricular strain, but the left
ventricle was normal.
• There was trace tricuspid regurgitation, but
the other valves were normal.
• No mass was seen in the inferior vena cava..
Diagnosis
D:D
• Venous Thromboembolism
• Endocarditis
• OTHERS
Venous Thromboembolism
• The most common cause of pulmonary
embolism is venous thromboembolism,
• Occasionally, the migrating thrombus can be
visualized while it is moving in the right atrium
or right ventricle and tethered to the right
ventricular trabeculae.
• Several clues in this patient’s presentation
suggest a diagnosis other than a classic
venous thromboembolic pulmonary
embolism.
• The patient is a 29-year-old previously healthy
semiprofessional athlete without major
illnesses or coexisting conditions
• He had post flight leg cramps, but he had had
leg cramps during many previous flights,
He does not have the typical risk factors for
venous thromboembolism, which include
1. previous thromboembolism,
2. physical factors (e.g., sedentary behavior),
3. mechanical factors (e.g., stricture of the inferior vena
cava or iliac vein),
4. foreign-body placement (e.g., placement of a central
venous catheter or inferior vena cava filter),
5. biochemical and genetic factors (e.g., thrombophilia
or a known hypercoagulable condition).
• His grandmother had had lupus and a related
venous thromboembolism, but there is no
other family history of thrombophilia
• The most notable feature of this patient’s
presentation that is atypical for classic venous
thromboembolism is the echocardiographic
appearance the intracardiac mass.
• The large size, frond like appearance,
heterogeneous echotexture, mobility, and
possible fixation to the tricuspid valve are all
highly unusual for a clot related to venous
thromboembolism
• Thrombi associated with venous
thromboembolism usually have a solid or
sausage like appearance that represents a
cast of the vessel from which the thrombi
were derived.
• Also, the thrombi typically migrate into the
pulmonary artery rather than occupying most
of the cavity of the chambers on the right side
of the heart
Endocarditis
• An endocarditis- related vegetation that is
attached to the tricuspid valve is a
consideration in this case.
• However, the mass is larger than those
typically associated with endocarditis,
• and in the absence of fever, sweats, or other
constitutional symptoms, there is little else to
support this diagnosis.
THE NEXT
• Therefore, the mass should be removed
immediately by means of surgical
pulmonary embolectomy, regardless of
the cause.
• Removal and pathological examination of the
mass will most likely establish the diagnosis.
• The patient thus underwent surgical
pulmonary embolectomy. In the
operating room,
•
transesophageal echocardiography
revealed a large, mobile, highly irregular mass
that extended into the superior vena cava
• The resected mass consisted of tangled white,
firm, stringlike structures that ranged from
less than 1 mm to 5 mm in thickness .
• On histologic examination, the mass
was composed of individual cells in a myxoid
stroma. The cells appeared to be pleomorphic
and were spindle shaped to stellate, with
nuclear atypia and prominent nucleoli
.Scattered mitoses were visible
• Histologic examination of a separate portion
of tricuspid valve revealed attachment of the
tumor, although the tumor did not originate
from the valve itself.
•What is the diagnosis ?
What is the diagnosis ?
1.
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6.
cardiac myxomas
Papillary fibroelastoma
Rhabdomyomas
Malignant Primary Cardiac Tumors
Malignant Secondary Cardiac Tumors
None of the above
cardiac myxomas
• cardiac myxomas, which represent
approximately 25% of all cardiac tumors;
these are usually tethered to the interatrial
septum, but 80% are located in the left atrium
and only 20% are located in the right atrium.
• Although the heterogeneous echotexture of
this patient’s mass could be consistent with a
friable myxoma, the attachment site is not
consistent with this diagnosis.
• These tumor cells were not reactive for
endothelial markers (CD31 and CD34), a
marker of cardiac myxoma and mesothelium
(calretinin), a general hematopoietic marker
(leukocyte common antigen).
Papillary fibroelastoma
• Papillary fibroelastoma is the most common
valvular tumor; the appearance is often
likened to a sea anemone and is somewhat
reminiscent of the mass seen in this patient.
However, this patient’s mass is larger and his
age younger than would be expected with a
fibroelastoma
Rhabdomyomas
• Rhabdomyomas are most commonly seen in
children. Leiomyomas, hemangiomas, and
teratomas are rare and not consistent with the
frond like appearance seen on
echocardiography in this case
Malignant Primary Cardiac
Tumors
• Malignant primary cardiac tumors are
extremely rare; they are predominantly
manifested by sarcomatous tumors and
occasionally by lymphoma.
• A primary sarcoma is possible in this case, but
any other primary cardiac tumors are unlikely
to produce a freely mobile mass.
Malignant Secondary Cardiac
Tumors
• Several types of cancer can migrate to the
heart and establish an intracardiac metastatic
focus of disease, although this is a rare
occurrence.
• On the basis of the heterogeneous and
irregular echocardiographic appearance of the
intracardiac mass, the attachment point of the
mass, and the overall clinical picture of this
patient,
• the large, space-occupying Intracardiac mass
seen on CT angiography and echocardiography
is not caused by venous thromboembolism
but rather by cardiac metastasis.
• This diagnosis would help to explain the
subacute presentation with recurrent
pulmonary embolism, scattered pulmonary
infarcts, and multiple nonspecific
abnormalities, including weight loss and
thrombocytopenia.
• CT of the head, neck, chest, abdomen, and
pelvis was ordered.
• Despite three negative preoperative scrotal
examinations, a scrotal ultrasound
examination was also ordered.
metastatic
cancer and lymphoma were
• The possibilities of
raised.
• The lactate dehydrogenase level declined
postoperatively,
• but the level of human chorionic
gonadotropin was elevated at 966 IU per liter
(normal, <0.7) and the level of alphafetoprotein was elevated at 374 ng per
milliliter (normal, <6.1).
• The testis contained a 1-cm tumor, which was
confined to the testicular parenchyma .
• On histologic examination, the
testicular tumor was a mixed germ-cell tumor
consisting of teratoma (25%), areas consistent
with calcified degenerated embryonal
carcinoma (20%), sarcoma (grade 2/3) (10%),
and fibrosis consistent with degenerated
burned-out tumor (45%).
• Having established the
diagnosis of
sarcomatous germ-cell tumor
of the testis,
• we next examined the tumor material
obtained from a cardiac resection specimen to
determine whether it was from the same
source
• The tumor cells in the cardiac mass were
diffusely reactive for cytokeratin and vimentin.
Approximately 20% of the tumor cells in the
cardiac mass showed strong staining for the
germ-cell marker human chorionic
gonadotropin
• The histologic and immunohistologic features
of the tumor cells in the cardiac mass are
indicative of a
metastatic
sarcomatous germcell tumor
and are similar to those of the
testicular tumor.
Management
• Orchiectomy.
• Chemotherapy
Diagnosis
• Pulmonary embolism and an
intracardiac mass due to a
metastatic germ-cell tumor.
•Thanks