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 1.5-hour 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• 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, N-terminal pro–B-type natriuretic peptide (NT-proBNP), 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. cardiac myxomas2. Papillary fibroelastoma3. Rhabdomyomas4. Malignant Primary Cardiac Tumors5. Malignant Secondary Cardiac Tumors6. 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.

• The possibilities of metastatic cancer and lymphoma were 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 alpha-fetoprotein 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