대한흉부영상의학회 Korean Society of Thoracic Radiology

Discussion

Diagnosis With Brief Discussion

Diagnosis

Epithelioid hemangioendothelioma

Radiologic Findings

Chest PA shows multiple, well demarcated rounded nodules in the both lung fields. Some of them are definitely radioopaque. Chest CT shows multifocal nodules in the periphery with lower lobar predominancy. Measurable nodules are less than 20 in number. One-third of nodules have relatively central radioopaque nodules. Three nodules are totally calcific. Most of nodules have pleural tail or subsegmental atelectasis. In the largest nodule (2.4 cm) in the right lower lobe, there are stippled calcific densities and closely attached to a pleura. It is seldom enhanced (about 5~19 HU on enhanced CT). Tiny nodular thickenings are noted in right major fissure. PET-CT shows nodules of faint or mild FDG uptake. Cut section (Fig. 1) shows a soft yellowish nodule with central calcification. Pleural invasion is noted (arrow). Low power view (Fig. 2) shows central necrosis with calcification and peripheral cellular proliferation (H/Ex20). H/Ex100 shows central necrosis and ossification. High power view of peripheral area (Fig. 3) shows small nests or cords of epitheloid tumor cells within the myxoid background (H/EX400). Medium sized tumor cells with eosinophilic cytoplasm and inconspicuous nucleoli are noted. The immunohistochemistry shows the tumor cells are positive for CD31 antibody (x200).

Fig.1

Fig.2

Fig.3

Brief Review

Epitheloid hemangioendothelioma is an uncommon, typically multifocal pulmonary neoplasm first recognized in 1975. Approximately 80% of cases occur in women, and may younger than 40 years of age, a relation to a hormonal effect. The explanation for the multifocality of the neoplasm is unclear. The typical absence of both a dominant pulmonary tumor and an extrapulmonary primary tumor suggest that the tumor originates in a multifocal fashion within the lung. Some authors, however, have reported evidence for a primary extrapulmonary origin. The usual pathologic manifestations consist of multiple well-demarcated parenchymal nodules ranging in diameter from 0.3 to 3.0 cm. Light microscopic appearances are characteristic. A hypocellular sclerotic central portion is surrounded by a somewhat more cellular periphery. Dystrophic calcification and ossification may occur in the acellular region, although it is only occasionally recognizable radiographically. Radiographic manifestations simulate metastases or infarcts. They can also enlarge slowly and eventually cause respiratory insufficiency. In one patient, the nodules were situated close to a pleural surface. The tumors are best considered low-grade sarcoma.

References

1. Fraser. RS, Muller NL, Colman N, Pare PD. Epitheloid hemangioendothelioma. In. Diagnosis of Diseases of the Chest. 4ed. 1999;1339-1343.
2. Yousem SA, Hochholzer L: Unusual thoracic manifestations of epitheloid hemangioendothelioma. Arch Pathol Lab Med 1987;111:459
3. Luburich P, Ayuso MC, Picado C, et al: CT of pulmonary epitheloid hemangioendothelioma. J Comput Assist Tomogr 1994;18:562
4. Kim EY, Kim TS, Han J, Choi JY, Kwon OJ, Kim J. Thoracic epitheloid hemangioendothelioma: imaging and pathologic features. Acta Radiologica 2011;52:161-166

Keywords

lung, malignant tumor,