Pulmonary epithelioid hemangioendothelioma

Pulmonary
epithelioid hemangioendothelioma accompanied by bilateral multiple calcified nodules in lung. Radiographs of chest. Chest radiograph (A) and computerized tomograph (B) show an approximately 6.0 cm sized well-demarcated mass in the right lung and multiple calcified small nodules in both lungs.
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Pulmonary
epithelioid hemangioendothelioma: a case report. Computed tomography scan showing tissular mass with peripheral calcification in the right lung.
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Pulmonary
epithelioid hemangioendothelioma misdiagnosed as a benign nodule. The initial chest computed tomography scan. (A) There is a peripheral small nodule with lobulation in the apicoposterior segment of the left upper lobe (arrow). (B, C) Two tiny nodules are noted in the posterobasal segment of the right lower lobe (arrows).
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Pazopanib for
metastatic pulmonary epithelioid hemangioendothelioma—a suitable treatment option: case report and review of anti-angiogenic treatment options. 18F-FDG PET-CT. a PET-CT (selected axial slice) performed at staging, demonstrates pathological FDG foci in a few lung nodules. b PET-CT (selected coronal slice) performed at staging, demonstrates pathological FDG foci in mediastinal lymph nodes and the liver. Additional findings were demonstrated in a few cervical lymph nodes (not shown). c PET-CT (selected axial slice) performed after treatment, demonstrates a few lung nodules with no FDG uptake. d PET-CT (selected coronal slice) performed after treatment, demonstrates pathological FDG foci in the liver. No mediastinal findings are shown
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Pulmonary epithelioid hemangioendothelioma is a rare vascular tumor of the lung and/or pleura with low malignant potential.

Epidemiology

It is a rare tumor, with ~50 cases reported. Patient age at presentation ranges from 25-54 years old, with a female predilection.

Clinical presentation

Pulmonary epithelioid hemangioendothelioma is often asymptomatic and discovered incidentally. It has been associated with chest tightness and shortness of breath.

Pathology

Etiology

The exact etiology of pulmonary epithelioid hemangioendothelioma is unclear. It may occur in male patients, which confuses the usual explanation of a role of estrogen in tumor formation.

Histology

Histology shows epithelioid or spindle-shaped cells with abundant eosinophilic cytoplasm. Lumina or vacuoles containing erythrocytes are present. Cells are arranged in nests and cords with degenerated interstitial mucoid.

Radiographic features

Usually discovered incidentally.

CT
  • multiple nodules in the lungs or pleura
    • bilateral, random distribution
    • nodules usually 0.3-2.0 cm (although up to 5 cm has been reported)
  • calcifications are occasionally seen within the nodules
  • if pleural invasion, then pleural thickening or effusion may occur

Treatment and prognosis

The prognosis of pulmonary epithelioid hemangioendothelioma is unpredictable due to variable estrogen effects on the tumor. Life expectancy has ranged from 1-25 years, depending on presentation and possible resectability of the lesions. If the lesions are unresectable, then chemotherapy is usually used.

Poor prognostic factors
  • male gender
  • hemoptysis
  • asymmetric distribution
  • pleural effusions

History and etymology

Originally described in 1975 as an "intravascular sclerosing bronchioloalveolar tumor (IVBAT) of the lung".

Differential diagnosis

The imaging differential is wide, including:

Siehe auch:
Assoziationen und Differentialdiagnosen zu pulmonary epithelioid haemangioendothelioma:
epitheloides
Hämangioendotheliom
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epitheloides
Hämangioendotheliom der Leber
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