2014, Volume 30, Number 2, Page(s) 137-141
Osteosarcoma Arising from a Haemangioma: Case Report and Review of the Literature
Paul MALLƯNSON1, Tyler COUPAL2, Malcolm HAYES3, Paul CLARKSON4, Peter MUNK1, Hugue OUELLETTE1
1Department of Radiology, Vancouver General Hospital/University of British Columbia, VANCOUVER/BC, CANADA
2McMaster University, De Groote Medical School, HAMILTON/ONTARIO, CANADA
3Departments of Pathology, BC Cancer Agency, VANCOUVER BC, CANADA
4Departments of Surgery, BC Cancer Agency, VANCOUVER BC, CANADA
Keywords: Osteosarcoma, Neoplastic cell transformation, Hemangioma
To create awareness of the benign lesions from which osteosarcoma
Osteosarcoma is a rare tumour of bone the etiology of which is
poorly understood, but it may arise from benign lesions. Malignant
transformation in hemangiomas, in the absence of prior radiation,
is exceedingly rare and the resulting neoplasm is usually an
angiosarcoma. We report the case of a 30-year-old woman where
investigation for thigh pain revealed a distal femoral hemangioma.
She represented with pain and mass 18 years later, leading to a
confirmed diagnosis of osteosarcoma at the same site.
Osteosarcomas may arise from a variety of benign lesions. In this article
we report the case of a histologically confirmed hemangioma which
subsequently underwent malignant change into an osteosarcoma.
Osteosarcoma is a rare tumour of bone the etiology of
which is poorly understood. Malignant transformation
of hemangiomas, in the absence of prior radiation, is
exceedingly rare, with only a handful of reported cases1
Reports in the literature describe osteosarcomas arising from
a variety of benign lesions and conditions, but the authors
found no reports of transformation from a hemangioma. In
this article we report the case of a histologically confirmed
haemangioma which underwent malignant change into an
osteosarcoma 18 years after the original diagnosis.
A 30-year-old woman was investigated for distal thigh pain.
An x-ray was performed and showed an area of bone sclerosis in the distal lateral femoral metaphysis measuring 3.5
cm in maximum dimension. The anterior margin was well
delineated but the other margins were indistinct. Diagnoses
of fibrous dysplasia and sarcoma were considered. A repeat
x-ray a month later again demonstrated the same sclerotic
lesion without evidence of cortical destruction or periosteal
reaction. A bone scan demonstrated uptake in the region
of the lesion with no evidence of metastatic disease. MRI
showed that the lesion extended to the epiphysis but showed
no evidence of cortical perforation or joint involvement.
Some surrounding bone marrow edema was noted.
An initial core needle biopsy was non-diagnostic and a
large open trephine biopsy was therefore performed. The
sections showed dense sclerotic bone lesion exhibiting remodelling
of lamellar bone trabeculae. This was associated with prominent activity of both osteoblasts and osteoclasts,
but no osteoblastic cytological atypia was seen. The intertrabecular
tissue showed a proliferation of crowded thinwalled
blood vessels lined by flat endothelial cells, again
lacking atypia, and associated with interspersed erythrocytes. The features were those of an intraosseous hemangioma
(Figure 1A,E). There was no evidence of malignancy.
The slides were reviewed by 2 experienced pathologists who
concurred with this interpretation.
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|Figure 1: A) Biopsy from 1994 showing a proliferation of
crowded thin-walled blood vessels lined by flat endothelial cells,
lacking atypia, and associated with interspersed erythrocytes,
diagnostic of a benign hemangioma (H&E x100). B) Clusters
of vessels surrounded by normal bone in keeping with a benign
hemangioma (H&E x200). C) Multiple vessels, spindle cells and
a capillary lumen in keeping with a benign hemangioma (H&E
x400). D) A mass of red cells and blood vessels in keeping with
a benign hemangioma (H&E x200). E) Spongy appearance with
proliferative abnormal vessels interspersed between reactive bone
trabeculae, in keeping with a intraosseous benign hemangioma
Seventeen years later, the patient re-presented with an
enlarging painful mass of the left knee that was associated
with dysesthesia at the anterior aspect of the left leg and foot. Physical examination was otherwise normal. The
patient was otherwise healthy, took simple analgesics for
shoulder pain and no other medication.
The repeat x-ray demonstrated an aggressive process,
featuring poorly marginated area of sclerosis/lysis occupying
the lateral femoral condyle with cortical destruction laterally
and ill-defined calcification in the adjacent soft tissues
(Figure 2). CT confirmed a lytic/sclerotic bony lesion with cortical perforation, periosteal reaction and calcified matrix
(Figure 3). MRI further affirmed the aggressive nature of
lesion that showed a soft tissue component seen extending
through the breached cortex into the adjacent muscle,
with surrounding edema (Figure 4). The lesion measured
6.6 x 5.1 x 5.3 cm and showed hypointensity on T1W
images and heterogeneous hyperintensity on T2W images.
The lesion was again positive on bone scan but with no
evidence of bone metastases. Diagnoses of osteosarcoma,
chondrosarcoma and calcified metastases from a mucinous
tumour were considered.
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|Figure 2: X-ray at time of re-presentation showing a lytic sclerotic
lesion in the lateral femoral condyle with cortical breach and ill
defined calcification in the adjacent soft tissue.
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|Figure 3: Axial CT showing a lytic sclerotic lesion in the lateral
femoral condyle with cortical breach and osteoid matrix.
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|Figure 4: MRI Coronal STIR and Axial T2W FS images demonstrating the lateral femoral condyle lesion breaching the cortex and
extending into the soft tissue with surrounding edema.
A CT-guided core biopsy was performed using an 11G coaxial
needle and a 14G biopsy needle via a lateral approach.
The histology sections demonstrated trabeculae of lamellar bone permeated by a high-grade malignant spindle and
polygonal cell neoplasm. This was associated with production
of osteoid matrix and irregular woven bone trabeculae,
characteristic of an osteosarcoma (Figure 5A-D). No
residual hemangioma was identified.
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|Figure 5: A) Biopsy from 2012 demonstrating lamellar bone permeated by a high grade malignant spindle and polygonal cell neoplasm,
with osteoid matrix and irregular woven bone trabeculae, diagnostic of a high grade osteosarcoma (H&E x200). B) Woven bone and
large malignant osteoblasts (H&E x200). C) Preexisting lamellar bone trabeculae permeated by sheets of tumour cells and woven bone in
keeping with osteosarcoma (H&E x200). D) Malignant osteoblastic tumoral cells admixed with woven bone in keeping with osteosarcoma
Malignant transformation in hemangiomas is most
commonly associated with radiation therapy and is
otherwise very rare. The malignant change has previously
been to an angiosarcoma1,2
. Our patient had no history of
prior radiation therapy to the distal femur. Osteosarcomas
usually arise de novo. Radiation has been implicated and
Paget's disease is a known cause in the older generations.
Associations have also been made with tall stature,
pubertal hormones, acromegaly, viruses and various cancer
predisposition syndromes including retinoblastoma3.
Osteosarcomas have been reported to arise from heterotopic
bone secondary to dermatomyositis and thermal injury,
giant cell tumors (GCT), irradiated aneurysmal bone cysts
(ABC), osteoblastomas, solitary osteochondromas and
fibrous dysplasia4-9. Malignant change in GCTs is most
commonly reported after radiation, but can be spontaneous
and has even occurred in a GCT lung metastasis6.
Difficulty in histological diagnosis of osteosarcoma has
been reported when the differential includes other osteoid
producing tumours, but distinction from hemangioma
is usually simple. Bertoni et al. described 17 cases of
osteosarcoma that resembled osteoblastoma on histological
analysis10. In the present case, the original histological
appearances were distinctly different from osteosarcoma
and the diagnosis of hemangioma was confirmed by 3
pathologists on the basis of a large open trephine biopsy.
Furthermore, the time interval was sufficiently long to
exclude a misdiagnosed indolent osteosarcoma due to a
Osteosarcoma is the most common primary sarcoma of
bone, but remains rare (estimated 1-2 cases per million/
population/year for 25-59 age group)3. The possibility
of a coincidental osteosarcoma arising at the site of a
hemangioma is statistically unlikely in the absence of other
known predisposing factors, but has to be considered. The
increased rate of bone turnover induced by the hemangioma
could have acted as a substrate for the development of
a secondary malignancy in a similar mechanism to the
development of osteosarcoma in Paget's disease.
The period of time taken for the malignant transformation
was 18 years. Previous reports of malignant transformation
to osteosarcoma in the literature cover a wide range of
timescales including 28 years for a case of dermatomyositis case5, 6 years for a GCT69 and 7 years for an ABC7.
Obana reported that the time taken for a hemangioma to
undergo malignant change to angiosarcoma as 2 years2.
Our case falls within this very wide spectrum.
In conclusion we present the unique case of an osteosarcoma
arising from a hemangioma 18 years after original diagnosis.
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