Carcinoma of esophagus confirmed by upper endoscopy who presented with difficulty in swallowing and progressive weight loss

Presentation 2 (Fig. 1)
This image shows the classical ultrasound appearance of myxoma with it gelatinous appearance and cystic echolucencies. Myxoma is the commonest primary benign intracardiac tumor encountered. 87% of myxoma is located in the left atrium. Diagnosis almost assures if attachment site to the interatrial septum can be established. Myxoma is not a "benign" tumor in the sense that tumor fragmentation and thromboembolism from the tumor surface may occur. Damage to the mitral valve can occur when the tumor adheres to it. Systemic symptoms (fever and weight loss) result from secretion of interleukin-6. Once diagnosed, it should be removed surgically as soon as possible due to risk of systemic embolization. Post operatively, patients should be followed up by serial echo screening for possible tumor recurrence. A familial pattern with autosomal dominance inheritance is well described. Family screening should be offered with the following atypical presentations 1) multiple myxomas 2) atypical location 3) young age (< 30 year old).

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Left atrial myxoma who presented with systemic embolization and left sided stroke

Presentation 3 (Fig. 2)
This image shows the intracardiac extension of soft tissue tumor through the left upper pulmonary vein into the left atrium. Thrombus is not a differential diagnosis since it will not occur inside the pulmonary vein. Once an intracardiac tumor is identified, the following information should be obtained: 1) anatomical location & attachment site of the tumor 2) surrounding venous inflow tracts extension – superior & inferior vena cava, coronary sinus for right sided tumors; all pulmonary veins for left sided tumors 3) pericardial effusion which is a common occurrence in primary or metastatic malignant cardiac tumors 4) any involvement of adjacent cardiac structures e.g. cardiac valves, coronary artery ostium, encasement of great vessels which may affect surgical planning. For this patient, histological confirmation of pleomorphic large cell carcinoma was obtained by bronchoscopy and the intracardiac extension of tumor precluded him from curative surgery.

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Bronchogenic carcinoma with a large soft tissue shadow over the left upper lobe on CXR who presented with hoarseness of voice. The cardiac ultrasound examination confirmed intracardiac extension of tumor

Presentation 1 (Fig. 3)
This image is the classical appearance of a posterior mediastinal tumor when seen from transthoracic echo examination. The posterior left atrial border was being compressed externally by the large soft tissue tumor. Carcinoma of the esophagus is the commonest cause of posterior mediastinal tumor in our locality. Transesophageal examination before an upper endoscopy is a definite contraindication in patients with posterior mediastinal tumor. Esophageal rupture or perforation will be a serious and fatal complication in "blind" transesophageal procedures.

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Primary malignant angiosarcoma of the heart with the histology confirmed by open heart surgical debulking procedure

Presentation 4 (Fig. 4)
Though extremely rare, angiosarcoma is still the commonest primary malignant cardiac tumor encountered. The malignant nature of this primary intracardiac tumor can be seen from the image – infiltration of the whole coronary sinus, the transverse sinus between the left pulmonary artery and left atrium by soft tissue tumor. All primary malignant cardiac tumors have very grim prognosis. This patient died 8 months after the diagnosis despite multiple courses of aggressive chemotherapy.

The malignant nature of cardiac tumor can usually be established by echo, but the histology cannot be ascertained by whatever imaging modality. Therefore a management approach to obtain histological proof is always warranted. We have seen patients with intracardiac malignant lymphoma who presented in a similar manner, histological proof can only be obtained during surgery and the disease is cured by subsequent chemotherapy.

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What are the clinical differential diagnoses?

The clinical differential diagnoses include Becker's naevus, congenital melanocytic naevus, epidermal naevus, postinflammatory hyperpigmentation and large lentigos.

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What is the most likely clinical diagnosis in this patient?

Becker's naevus. It is characterized by unilateral irregularly pigmented macular lesion and often associated with hypertrichosis. The lesion usually develops and first noted in puberty.

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What is the investigation that confirms the clinical diagnosis?

A skin biopsy for histopathology and/or electron microscopic study can confirm the clinical diagnosis. The lesion is characterised by increased basal pigmentation without significant increase of melanocytes. Hair follicles are usually normal but can be increased and enlarged. Increased smooth muscle is almost always found in deep dermis.

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What are the other clinical associations with this condition?

Associated features include unilateral hypoplasia of breast, shoulder girdle, pectoralis major muscle, or upper limb. There may also be vertebral defects, multiple leiomymas, and localised lipoatrophy.

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What is the treatment for the lesion?

Treatment aims at reduction of hyperpigmentation and excessive hair. Laser therapy, such as Q-switched ruby has been reported to be successful in treating the hyperpigmentation and hypertrichosis.

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