Pulmonary epithelioid hemangioendothelioma accompanied by bilateral multiple calcified nodules in lung

Epithelioid hemangioendothelioma (formerly known as intravascular bronchioalveolar tumor, IVBAT) is an uncommon tumor of vascular endothelial origin, with an intermediate course between hemangioma and conventional angiosarcoma[1-3]. PEH typically occurs as bilateral multiple nodules among young women[1,4]. But some cases also develop as a solitary lung nodule or a single cavitary nodule[1,5]. We describe here a case of PEH presenting as a 6.0 cm dominant mass along with multiple calcified small nodules in lung. To our knowledge, this type of PEH manifestation has not been reported in the literature before.In 1982, a 20-year-old, non-smoking woman was incidentally found to have an abnormal chest X-ray during a routine medical examination. The X-ray radiograph showed diffusely scattered, high-density small nodular opacities in all the lung fields. Routine laboratory data were within normal limits at the time. She had no significant complaints, past illnesses, or family history. The multiple high-density small nodular opacities were slowly increasing in number and size in the serial follow-up chest radiographs. Twenty years later (May 2002), a follow-up X-ray film showed one approximately 3.0 cm sized, well-defined mass in the middle field of right lung. Because of the absence of symptoms, she had always rejected any medical interventions. In July 2004, she began to cough with sputum. A plain chest radiograph (Figure 1A)and computerized tomograph (CT) (Figure 1B) revealed the mass to be increased to 6.0 cm along with more calcified small nodules (all smaller than 1.0 cm). Physical examination and laboratory findings were still not unremarkable. In September 2004, the patient underwent a lobectomy of the right lower lobe.Macroscopically, the specimen measured 12.0 × 10.0 × 4.0 cm in size and was mostly replaced by a well-circumscribed, unencapsulated mass, up to 6.0 × 5.0 × 4.0 cm. The cut surface of the mass was whitish to brownish in color and solid, but focal cystic