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Year : 2004  |  Volume : 14  |  Issue : 4  |  Page : 440-442
Multicentric skeletal Hemangioendothelioma with pulmonary metastasis


Department of Radiology and Imaging, Vardhman Mahavir Medical College and Safdarjang Hospital, New Delhi, 110024, India

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How to cite this article:
Grover S B, Mahato S K, Gupta P. Multicentric skeletal Hemangioendothelioma with pulmonary metastasis. Indian J Radiol Imaging 2004;14:440-2

How to cite this URL:
Grover S B, Mahato S K, Gupta P. Multicentric skeletal Hemangioendothelioma with pulmonary metastasis. Indian J Radiol Imaging [serial online] 2004 [cited 2020 Feb 26];14:440-2. Available from: http://www.ijri.org/text.asp?2004/14/4/440/28692
Sir,

Hemangioendotheliomas are rare vascular tumors that can arise from soft tissue or bone [1]. In the skeleton,these neoplasms account for only 0.5 to 1% of malignantprimary bone tumors [1],[2]. These rare tumors, whichwere given the nomenclature by Mallory in 1903, occurdue to neoplastic proliferations of blood vessel endothelialcells and they characteristically infiltrate surroundingtissue and recur locally [1]. Hemangioendotheliomasoccur in the age group of 20 to 80 years and approximately25% are multicentric [2],[3]. The common sites ofoccurrence are the lower limb bones, less than 10% arisein the vertebrae and only a minority may occur in skull,pelvis and clavicle [1],[2]. These tumors rarely metastasize[2].

We share our experience of this bone tumor exhibitingmetastasis, in a 37 year old male, who presented withinsidious pain and swelling in left gluteal and hip regionfor five months. Local radiographic examination revealeda permeative destruction with ill defined margins, whichshowed a mixed pattern of honey comb lucencies andlinear sclerosis involving the left iliac bone includingacetabulum. The head, neck and greater trochanter ofipsilateral femur also showed multiple infiltrative lytic areas[Figure - 1]a & b. Skeletal survey showed collapse of 7thdorsal vertebra with a "soap bubble" appearance, theadjoining disc spaces were preserved [Figure - 2]a & b. A"cannon ball" metastasis in the right lower zone was seenin the chest radiograph [Figure - 3]. This radiologicalappearance of honey comb lucencies intermingled withlinear sclerotic foci and permeative infiltration, withindistinct margins, suggested a malignant tumor ofvascular origin. Fine needle aspiration and excision biopsyof the left iliac bone both confirmedhemangioendothelioma. Therefore, a final diagnosis ofmulticentric skeletal hemangioendothelioma involving leftiliac bone, left femur and 7th dorsal vertebra withpulmonary metastasis was made. The patient was advisedfurther imaging evaluation and necessity of surgicalmanagement, followed by radiotherapy, which was notaccepted and the patient was therefore lost to further followup.

Bone tumors of vascular origin may be benign ormalignant. The former comprise of hemangioma,epithelioid hemangioma, hemangiomatosis and massiveosteolysis or Gorham's disease (which mimics malignantneoplasm due to aggressive osteolytic destruction) [4].The rarer malignant counterparts are epithelioidhemangioendothelioma, hemangioendothelioma &angiosarcoma in increasing order of malignancy [3]. Themost common clinical manifestations ofhemangioendothelioma are localized pain and soft tissueswelling [3]. Radiographically, they present as single ormultiple lesions, with frequently a lytic pattern of bonedestruction. However, some are known to have mixed lyticand sclerotic pattern, as was seen in our patient. In tubularbones, these tumors manifest as osseous expansion,endosteal erosion and cortical thickening [3].

The radiographic differential diagnosis forhemangioendothelioma 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 amixed 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 andfibrosarcoma need to be considered [2],[3].

Histologically, hemangioendothelioma are vasoformativetumors with plump endothelial cells lining the vascularspaces and a frequently observed feature is the tendencyto permeate medullary bone. Tumors which show highgrade cytological atypia and brisk mitotic activity areclassified as angiosarcomas [3]. The well circumscribedtumor with mature vessels is the least malignantcounterpart known as the epithelioidhemangioendothelioma [3]. In our patient the histologyfeatures were consistent with hemangioendothelioma.

CT is advocated for superior anatomic delineation oftumors arising in pelvic bones, skull, spine and ribs. Askeletal survey or bone scan is recommended for diseasestaging as presence or absence of multicentricitydetermines the treatment options [2],[3]. MRI is helpful inprecise anatomic delineation of the extent of the lesionand for spinal cord status in vertebral lesions, but hasvirtually no contribution to the pre biopsy diagnosis [2],[3]. On the contrary conventional radiographs & CT maysuggest a tumor of vascular origin, if prominent trabeculaeand "soap bubble" appearance are observed, as was seenin our patient. Surgical excision is the preferred treatmentfor unifocal lesions, whereas multifocal lesions requireradiotherapy. Pre operative angiographic embolization isadvisable to reduce intra operative blood loss in patientsundergoing surgical management [1],[2]. So farchemotherapy is believed to have no role in the treatmentof hemangioendothelioma [1].

We report this case not only for the rarity of the tumorand its characteristic radiographic appearance, itsfrequent multicentricity as seen in our case, but also forits unusual primary site (which appeared to be iliac bonein our patient) and most importantly for its metastaticnature which is extremely rare. Moreover, skeletal surveyand bone scan have been emphasized by otherinvestigators, who have not however highlighted theimportance of screening the lungs for metastases [1],[2].Therefore, the role of a routine chest radiograph forexcluding lung metastases in the diagnostic work upprotocol for this tumor is recommended by us and is amplyhighlighted by our report.

 
   References Top

1.Ibarra RA, Kesava P, Hallet KK, Bogeaev C. Hemangioendothelioma of the Temporal Bone with Radiologic Findings Resembling Hemangioma. Am J Neuroradiol, 2001; 22:755-758.  Back to cited text no. 1    
2.Aflatoon K, Staals E, Bertoni F, et al . Hemangioendothelioma of the Spine. Clin Orthop 2004;418:191-197.  Back to cited text no. 2    
3.Wenger DE, Wold LE. Malignant Vascular Lesions of Bone : Radiologic and Pathologic Features. Skeletal Radiol 2000;29:619-631.  Back to cited text no. 3    
4.Wenger DE, Wold LE. Benign Vascular Lesions of Bone : Radiologic and Pathologic Features. Skeletal Radiol 2000;29:63-74.  Back to cited text no. 4    

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Correspondence Address:
S B Grover
Department of Radiology and Imaging, Vardhman Mahavir Medical College and Safdarjang Hospital, New Delhi, 110024
India
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Source of Support: None, Conflict of Interest: None


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[Figure - 1], [Figure - 2], [Figure - 3]

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