Hemangioendothelioma of Bone

Path. Res. Pract. 180, 521-525 (1985)

Hemangioendothelioma of Bone A Report of an Unusual Case with Lymph Node Metastasis R. Volpe, A. Carbone and R. Manconi Division of Pathology (Head: Dr. A. Carbone), Centro Riferimento Oncologico, Aviano, Italy

L. Santi Istituto Nazionale per la Ricerca suI Cancro (Scientific Director: Prof. L. Santi), Genova, Italy

SUMMARY An unusual case of hemangioendothelioma of bone with regional lymph node metastasis in a 54-year-old woman is reported. The patient died nine weeks after admission of circulatory collapse. Six possibly similar cases were found in reviewing the literature. It is concluded that lymph node metastases from hemangioendothelioma of bone appear to be an extraordinary event and may represent an extremely unfavourable prognostic finding.

Introduction Hemangioendothelioma (angiosarcoma) of bone is a rare tumor which can metastasize through the blood stream and sometimes through the lymphatics9,1O. Either reports of single cases or largest series published on this subject have usually been devoted to the study of its nomenclature, pathogenesis, classification, clinical behaviour and treatment but little information is currently available in the literature about the frequency of lymph node metastases and their significance. A case of hemangioendothelioma of bone with regional nodal metastasis, in which both the primary tumor and the metastasis had a similar histologic appearance, is the subject of the present paper. This study was undertaken in order to review the frequency of nodal metastases in previously reported cases, and to evaluate the clinical implication of such an event. Case Report

a) History The patient was a 54-year-old woman, in otherwise good health, who was admitted to another hospital in March 1981 with a complaint of persistent pain in the left © 1985 by Gustav Fischer Verlag, Stuttgart

shoulder of three months duration. She was referred to the hospital with a diagnosis of arthritis. The past medical history was not relevant. Physical examination showed limitation of motion of the left arm with hemorrhagic suffusion in the subcutis of the shoulder and of the arm. X-ray examination (Fig. 1) revealed a fuzzily mottled large osteolytic lesion in the left scapula that extended to the articular surface with associated osteoporosis of the head and metaphysis of the humerus. On routine hematologic workup, significant data consisted of: hemoglobin 8.4%; RBC 2.68 x 106 ; WBC 4.9 x 103 ; and platelet count 160,000 cu mm. The serum-calcium and alkaline phosphatase values were within normal range. An arteriography revealed a pathological circle of anastomizing vessels (Fig. 2). In March 1981, the lesion was exposed and found to contain brownish-orange, friable tissue which was curetted. The postoperative course was characterized by hemorrhage at the site of the curettment with severe anemia. The patient underwent surgical reintervention with excision of a palpable left axillary lymph node. There was continued and rapid increase in size of the lytic lesion. The patient died nine weeks after admission of circulatory collapse. No autopsy was performed. 0344-0338/85/0180-0521$3.50/0

522 . R. Volpe, A. Carbone, R. Manconi and L. Santi

Fig. 1. Radiograph shows an osteolytic large lesion of the left scapula. Posterior view.

b) Histopathologic Findings Histologic sections of the primary operative specimen showed that the bone tissue was partially destroyed and replaced by a neoplasm composed of plump endothelial cells. They were arranged in cords, small aggregates and occasional small vessels containing red blood cells. The slightly hyperchromatic nuclei were considerably larger than those of normal endothelial cells. The lumina were of various size and often appeared stuffed with proliferating endothelial cells. Mitotic figures were uncommon. Lymphocytes and extravasated red blood cells were scattered diffusely between the aggregates. The diagnosis of hemangioendothelioma of bone was made (Fig. 3). Microscopically, in the axillary lymph node, the most conspicuous and diagnostically significant feature was the presence, in the paracortical area, of a neoplastic tissue constituted by a network of vessels which were extremely variable in size. There were free anastomoses of vascular channels and sometimes, papillary infolding of endothelial cells (Fig. 4).

Fig. 2. An arteriography of the left arm shows a pathological vascular circle.

Discussion Hemangioendothelioma of bone is a very rare tumor whose clinical behaviour appears to be unpredictable 14 • However, a generally accepted concept is that hemangioendothelioma of bone can metastasize, usually, by way of the blood stream with the lungs being an important site of metastatic deposits3. Other sites of metastatic involvement are the bones followed by other internal viscera. In a review of the old literature, Carter2 stated that up to 1955 only two of 16 collected cases were reported to be metastatic to lymph nodes 5• 11 . In the evaluation of following literature (Table 1), we have encountered other four similar cases; one of these, that occurred in a child, has

Table 1. Other Case Report in the Literature Total No. of Cases with Lymph node metastases Patients

Author Freilich and Coes Rohrl

ll

Hartmann and Stewarr7 Bundens and Brighton Gilbert et al. 6 Unni et al. B

1

Age (yrs) and sex

Site of Primary

Status

1

1

61 M

Scapula

Died 12 months

1

1

64M

Scapula

Died on 39th hospital day

10

1

54 F

Foot and tibia

Alive and well 3 yrs

2

1

34M

Left radius

Died 8 months

1

1

16 months M

12th dorsal vertebral body

Died 6 months

22

1

48 M

12th dorsal vertebral body

Died 5 months

Hemangioendothelioma of Bone . 523

b Fig. 3. Primary tumor of bone demonstrates a bone replacement by neoplastic tissue (A) consisting of atypical endothelial cells forming free anastomosing vessels (B). A) HE, X 250; B) Gomori silver reticulum stain, x 250.

524 . R. Volpe, A. Carbone, R. Manconi and L. Santi

Fig. 4. Intra-Iymphnode metastatic hemangioendothelioma consists of a network of vessels variable in size. Lymphoid remnants are also observed HE, X 100.

been reported in 1971 by Gilbert et al. 6 who in a review of the pertinent literature, were not able to find previous cases of hemangioendothelioma in children with nodal metastases. Of the cases summarized in Table 1, the site of involved lymph nodes was not srecified in two casess, 13 whereas in the remaining cases l , ,7,11 the regional lymph nodes were involved; however it was not specified whether the tumor spread to the nodes was a classic embolic metastasis or involvement by direct extension of the tumor from destroyed cortical bone to the soft tissue and then to the adjacent nodes. In particular, the metastases to hilar and mediastinal lymph nodes in the case of Gilbert et al. 6 were probably due to the direct extension of the tumor from the lungs. In our case the metastasis was clearly of the embolic type: the capsule of the lymph node was not involved by the neoplastic tissue which replaced in part the lymphoid tissue. In reviewing the literature it appears evident that the lymph node metastasis may represent a worsening prognostic finding, analogously to our case. In fact in these cases, the median survival was seven months. A remarkable exception is represented by the case 4 of the series of Hartmann and Stewart7• This case concerns a 54-year-old female with multiple hemangioendothelioma of foot and tibia who had metastases to one popliteal lymph node. The patient had a good survival with a long-standing follow-up of 9 years as reported later by Otis et al. 8 • According to Rosai et al. 12, however, the examination of Fig. 6 of Hartmann's article which shows "a lymph node containing metastatic hemangioendothelioma and the adherent

popliteal artery" suggests that the involved structure may represent and extension of the neoplasm to the surrounding soft tissue. In conclusion, whereas in cutaneous angiosarcomas regional lymph node metastases occur in 15-20% of the cases\ from the literature reports it appears that true lymph node metastases from a malignant hemangioendothelioma of bone are an extraordinary event; however, due to the unfavourable outcome of such cases, a careful clinical evaluation of regional lymph nodes is mandatory in the staging of the disease.

References 1 Bundens WD, Brighton CT (1965) Malignant hemangioendothelioma of bone. Report of two cases and review of the literature. J. Bone and Joint Surg 47 A: 762-772 2 Carter JH, Dickerson R, Needy C (1956) Angiosarcoma of bone. A review of the literature and presentation of a case. Ann Surg 144: 107-117 3 Craver WL, Brown BS (1979) Hemangioendothelioma of bone with pulmonary metastases. A 25-year course. Cancer 43: 1917-1923 4 Enzinger FM, Weiss SW (1983) Soft Tissue Tumors. C W Mosby Comp., St. Louis 5 Freilich EB, Coe GC (1936) Angiosarcoma: case report and review of the literature. Am J Cancer 26: 269-275 6 Gilbert EF, Jones B, Majzoub HS (1971) Malignant hemangioendothelioma of bone in childhood. Report of a case and review of the literature. Amer J Dis Child 121: 410-414

Hemangioendothelioma of Bone . 525 7 Hartmann WH, Stewart FW (1962) Hemangioendothelioma of bone. Cancer 15: 846-854 8 Otis j, Hutter RVP, Foote FW, Marcove RC, Stewart FW (1968) Hemangioendothelioma of bone. Surg Gynecol Obstet 127: 295-305 9 Ramsey Hj (1966) Fine structure of hemangiopericytoma and hemangioendothelioma. Cancer 19: 2005-2017 10 Robbins SL (1968) Pathology. WB Saunders, Philadelphia 11 Rohrl W (1941) Angiosarcoma of bone. Multiple metastases. Rontgenpraxis 13: 389-391

12 Rosai j, Gold j, Landy R (1979) The histiocytoid hemangiomas. A unifying concept embracing several previously described entities of skin, soft tissue, large vessels, bone and heart. Hum Patholl0: 707-730 13 Unni KK, Ivins jC, Beabout jW, Dahlin DC (1971) Hemangioma, hemangiopericytoma and hemangioendothelioma (anrosarcoma) of bone. Cancer 27: 1403-1414 1 Volpe R, Mazabraud A (1982) Hemangioendothelioma (angiosarcoma) of bone. A distinct pathologic entity with an unpredictable course? Cancer 49: 727-736

Received August 21, 1984 . Accepted in revised form November 10, 1984

Key words: Hemangioendothelioma - Lymph node metastasis - Bone tumor - Angiosarcoma Dr. R. Volpe, Division of Pathology, Centro di Riferimento Oncologico, 33081 Aviano, Italy