Yonago Acta medica 2000;43:87–92

Multicentric Epithelioid Hemangioendothelioma of the Bone: Histologicand Radiographic Features
Haruhiko Yoshida, Mari Watanabe*, Takeshi Minamizaki† and NobuyukiTakasu§
Department of Pathological Technology Science, *First Department of Pathology and†Department of Orthopedic Surgery, Faculty of Medicine, Tottori University, Yonago 683-0826
and §Clinic of Orthopedic Surgery, Tottori Red Cross Hospital, Tottori 680-0017, Japan
A multicentric epithelioid hemangioendothelioma (EHE) of the bone is reported which
affected the right femur, the right patella and the right fibula of a 56-year-old man.
Plain radiographs demonstrated purely lytic multicentric lesions with well-definedsclerotic borders. Computed tomography (CT) scans showed multiple osteolytic lesions
in a honeycomb pattern at the epiphyseal lesion of the right femur; the other lesions of
the right femoral shaft, the right patella and the right fibula were punched out sharply.
Magnetic resonance (MR) images showed that the tumors contained mixed signal
intensities: low intensity for T1-weighted images and high intensity for T2-weighted
images. Histologically, the tumor was discerned as EHE with a spectrum of endothelial
tumor, with the added feature of epithelioid hemangioma in a limited area. As for theinitial treatment, curettage and bone cementing were performed for the epiphyseal
tumor of the right femur, followed by surgical resections of the other tumors. Neither
recurrence nor metastasis was observed 3 years after resection.
Key words: bone tumor; epithelioid hemangioendothelioma; histology; multiple; radiography

polygonal cells with intracytoplasmic vacuolization, fibromyxoid stroma and well-formed
vascular channels at varying degrees (Tsuneyoshiet al., 1986; Weiss et al., 1986). EHE of the soft
tissue takes various clinical courses from benign to malignant; the malignancy is defined asintermediate by WHO classification (WorldHealth Organization, 1993). EHE should be
distinguished from benign vascular tumors
though difficulty exists in differentiation
among epithelioid tumors (Keel and Rosenberg,1999). Local recurrence and metastasis werereported to be 13% and 31% in incidence, respectively (Weiss et al., 1986).
We here report a case of multicentric bonetumor that fits the category of EHE, with special reference to radiographic features.

Weiss and Enzinger (1982) first described epithelioid hemangioendothelioma (EHE) as aunique tumor of the soft tissue, and proposed it
as a counterpart to the vascular tumor associated with epithelioid or histiocytoid cells: thetumors are well known as lesions in the liver,the lung and other organs. EHE of the bone,
however, rarely occurs and reported cases havetotaled up to less than 80 (Hartmen and Stewart,
1962; Otis et al., 1968; Maruyama et al., 1985;
Mirra and Kameda, 1985; Tsuneyoshi et al., 1986;
Lyon et al., 1992; Abati et al., 1994; Bollinger etal., 1994; Boutin et al., 1996), including 29 cases
reported by Tsuneyoshi et al. (1986) and a seriesof 40 cases by Kleer et al. (1996).
EHE appears in a unilocular or multicentricmanner histologically with features of large,

Abbreviations: CT, computed tomography; EHE, epithelioid hemangioendothelioma; MR, magnetic
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H. Yoshida et al.

1B

1AFig. 1. Plain radiographs. A: Lesions in a honeycomb pattern in the epiphysis of the right femur and in theright fibula. B: In the lateral view, lesions of the right femoral shaft, the right epiphysis and the right patella
are punched out.

signal intensities in magnetic resonance (MR)images: the intensity was low in T1-weighted
images, and high in T2-weighted images (Figs.
3A and B). Technetium bone scan revealed
increased uptakes of isotopes at the lesions ofthe right femur, the right patella and the rightfibula (Fig. 4). Histologic examination was carried out for biopsy specimens tentatively suspected of having an EHE. For the initial treatment, curettage and bone cementing were employed for the epiphyseal tumor of the rightfemur, and then the other lesions of the patella
and the fibula were surgically resected.
The surgically obtained specimens wereexamined and histologically defined as multicentric bone tumor which satisfies the categoryof EHE. The tumors forming nests in thegrowth pattern consisted of accumulated epithelioid cells, with various-sized vasculaturesof the capillary type (Fig. 5). Epithelioid cells

Patient Report
A 56-year-old man visited Tottori Red Cross
Hospital with a 1-month history of a spontaneous pain in his right knee joint in December
1996. No history of trauma to the thigh wasnoted. Physical examination on admission revealed tenderness to palpation without anymass. In plain radiographs, anteroposterior andlateral views demonstrated multiple radiolucent
lesions in a honeycomb pattern in the distal
epiphysis of the right femur, the right fibula andthe right femoral shaft (Figs. 1A and B). Computed tomography (CT) scans showed an osteolytic area with marginal sclerosis in the epiphyseal lesion of the right femur; the other lesionsof the right femoral shaft, the right patella andthe right fibula were all sharply punched out(Figs. 2A and B). The tumors contained mixed
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Epithelioid hemangioendothelioma of bone

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Fig. 2. Computed tomography (CT) scans. A: Well-defined, osteolytic lesions with a thin sclerosing margin
in the right femur and the patella. B: Intracortical lesion of the right fibula.
Fig. 3. Magnetic resonance (MR) images. A: T1-weighted image shows low signal intensity in the rightfemoral tumor, which is close to the skeletal muscle. B: In T2-weighted image, the intensity of theright femoral tumor is high.

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4Fig. 4. Technetium bone scan shows increased uptakes of isotopes in the lesions of the right femur, theright patella and the right fibula.

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