| Abstract|| |
Hemangioendotheliomas are rare vascular tumors that can arise from soft tissue or bone. The former comprises hemangioma, epithelioidhemangioma, hemangiomatosis and massiveosteolysis or Gorham's disease (which mimics malignantneoplasm due to aggressive osteolytic destruction). The rarer malignant counterparts are epithelioidhemangioendothelioma, hemangioendothelioma and angiosarcoma in increasing order of malignancy. The diagnosis of these lesions is difficult both clinically and radiologically. Hereby we report an unusual case which was mistaken for metastasis and was diagnosed with the help of histopathology.
Keywords: bone tumors, vascular lesions, multifocal hemangioendothelioma
|How to cite this article:|
Narang V, Garg B, Sood N, Kaur H. Multifocal hemangioendotheliomas of bone mimicking metastases
. Indian J Pathol Microbiol 2014;57:609-10
| Introduction|| |
Hemangioendotheliomas are rare vascular tumors that can arise from soft tissue or bone. In the skeleton, these neoplasms account for only 0.5 to 1% of malignant primary bone tumors. These rare tumors, which were given the nomenclature by Mallory in 1903, occurdue to neoplastic proliferations of blood vessel endothelialcells and they characteristically infiltrate surrounding issue and recur locally.  Hemangioendotheliomasoccur in the age group of 20 to 80 years and approximately 25% are multicentric. , The common sites ofoccurrence are the lower limb bones, less than 10% arise in the vertebrae and only a minority may occur in skull, pelvis and clavicle. , These tumors rarely metastasize.  We hereby report an unusual case of mutifocalhemangioendothelioma mimicking metastasis both clinically and radiologically
| Case report|| |
A 68year-old male presented with closed fracture of left subtrochantric region. The possibility of pathological fracture was considered. 99m Tc MIDP bone scan revealed multiple hot spot in skull-frontal bone, right scapula, 8th rib, right sacroiliac joint, left trochanteric region femur, left shaft of femur giving a final impression of skeletal metastasis [Figure 1]a. The serum protein electrophoresis and serum PSA levels were within normal limits. Open reduction and internal fixation with proximal femoral nail were planned. The excision biopsy was performed. Histopathology revealed a definite vasoformative lesion with infiltrative growth pattern. The endothelial cell lining of the vessels showed mild hyperchromasia. The cells were larger and plumper than those seen in hemagioma. No mitosis or cellular anaplasia was noted. The immunohistochemistry showed the tumor cells to be positive for CD31, CD34 and vimentin. Cytokeratin S100 and SMA were negative [Figure 1]b-d. The patient was advised further imaging evaluation and necessity of surgical management, followed by radiotherapy, which was not accepted and the patient was therefore lost to further follow up.
|Figure 1: a: Bone scan showing multiple hot spots in the bone. (b) Vasoformative tumor with plump spindle shaped cells (100x, H and E). (c) High power of revealing plump shaped cells. (d) Tumor cells positive for CD 34|
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| Discussion|| |
Bone tumors of vascular origin may be benign or malignant. The former comprise of hemangioma, epithelioidhemangioma, hemangiomatosis and massiveosteolysis or Gorham's disease (which mimics malignantneoplasm due to aggressive osteolytic destruction).  The rarer malignant counterparts are epithelioidhemangioendothelioma, hemangioendotheliomaandangiosarcoma in increasing order of malignancy. , The most common clinical manifestations of hemangioendothelioma are localized pain and soft tissues welling. ,, Hemangioendothelioma may affect any portion of the skeleton but most often is the axial skeleton. It occurs at any age with slight male preponderance. Multicentric tumors are present in 25% of cases. Radiographically, they present as single or multiple lesions, with frequently a lytic pattern of bone destruction. ,, However, some are known to have mixed lytic and sclerotic pattern, as was seen in our patient. In tubular bones, these tumors manifest as osseous expansion, endostealerosion and cortical thickening. ,
The radiographic differential diagnosis for hemangioendothelioma varies with clinical and radiologicalprofile. In case of young patients with purely lytic, multifocallesions, histiocytosis, brown tumor and metastasesshould be considered; while in older patients, metastases, myeloma and lymphoma are likely. In cases where admixed lytic with sclerotic pattern is present in a solitarylesion, with well defined margins, fibrous dysplasia, aneurysmal bone cyst and fibrous cortical defect shouldbe excluded. If the latter pattern is seen with ill definedmargins, osteosarcoma, Ewing's sarcoma and fibrosarcoma need to be considered. ,
Histologically, hemangioendothelioma are vasoformative tumors with plump endothelial cells lining the vascularspaces and a frequently observed feature is the tendencyto permeate medullary bone. Tumors which show high grade cytological atypia and brisk mitotic activity are classified as angiosarcomas. ,, The well circumscribed tumor with mature vessels is the least malignant counterpart known as the epithelioid hemangioendothelioma. ,, In our patient the histology features were consistent with hemangioendothelioma. Unni et al., in a review of 22 cases of hemangioendothelioma of bone, divided 22 cases of hemngioendothelioma of bone into three distinct histologic grades and stated that most important indicator of prognosis was the grade of anaplasia. Three grades were delineated on the degree of vasoformative appearance, pleomorphism of neoplastic cells and mitotic figures. Although patients were treated with variety of modalities and comparisons are difficult, there was definite decrease in disease free survival with increasing grade (grade 1, 95%; grade 2, 62%; and grade 3;20%). ,,
| Conclusion|| |
The multicentric hemangioendothelioma of bone are rare tumors and can mimic metastatic disease both radiologically and clinically. Pathological evaluation is strongly recommended in such cases to reach a definite diagnosis.
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Department of Pathology, Dayanand Medical College and Hospital, Ludhiana, Punjab
Source of Support: None, Conflict of Interest: None