Pulmonary epithelioid hemangioendothelioma accompanied by bilateral multiple calcified nodules in lung

Xu Jinghong,Chen Lirong

doi:10.1186/1746-1596-6-21

Abstract

Pulmonary epithelioid hemangioendothelioma (PEH) is a rare vascular tumor. It can present either as one solitary nodule or bilateral multiple nodules, usually without calcification. We describe here an unusual case of PEH in a 42-year-old female with a 6.0 cm dominant mass along with bilateral multiple calcified small nodules measuring 0.2-1.0 cm in diameter with a 25-year plus followup history. Overall histologic findings of the solitary tumor accorded with conventional PEH. While multiple calcified small nodules were composed predominantly of intra-alveolar homogeneously eosinophilic matrix, and only a few bland small cells were embedded in it. This lesion has never been reported in the literature. After comprehensive analysis of morphology, radiography, histochemistry, immunohistochemistry and differential diagnoses, PEH presenting multiple calcified small nodules was confirmed.

Highlights

Epithelioid hemangioendothelioma is an uncommon tumor of vascular endothelial origin, with an intermediate course between hemangioma and conventional angiosarcoma [1,2,3]
We describe here a case of Pulmonary epithelioid hemangioendothelioma (PEH) presenting as a 6.0 cm dominant mass along with multiple calcified small nodules in lung
The eosinophilic matrix in the satellite nodules and the way it filled and extended to adjacent alveolar spaces was the same as the main mass and fitted the description of PEH in that the neoplastic micronest spread through alveolar pores without destruction of the alveolar epithelium and interalveolar septum

Summary

Background

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 [1,2,3]. We describe here a case of PEH presenting as a 6.0 cm dominant mass along with multiple calcified small nodules in lung. The multiple small nodules were composed predominantly of intra-alveolar, homogeneously eosinophilic matrix with a few small cells embedded. These cells were cytologically not as atypical as the cells in the main mass (Figure 4A). Congo Red and Crystal violet stains were negative in both the main mass and small nodules In both the main tumor mass and the small nodules, cells were strongly and diffusely positive for vimentin, and the acellular matrix was positive for factor VIII-related antigen. The cells in all lesions were negative for cytokeratin, cytokeratin, cytokeratin, cytokeratin, cytokeratin, S-100 protein, smooth muscle actin, epithelial membrane antigen and thyroid transcription factor-1

Discussion

Conclusions