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Telangiectatic osteosarcoma: an easily misdiagnosed lesion

Wei Chena1,  Yaling Liua1, Yanling Su; Yang Dingb, Jianling Cuic, Yingze Zhanga

a.Department of Orthopedics, 3rd Hospital, Hebei Medical University, China

b.Department of Pathology, 3rd Hospital, Hebei Medical University, China

c.Department of Radiology, 3rd Hospital, Hebei Medical University, China

a1.These two authors contributed equally to this work

Address for Correspondence:
Yingze Zhang
Department of Orthopedics, 3rd Hospital, Hebei Medical University, Shijiazhuang, Hebei, 050051, P.R. China
Fax    :  +86-311-87023626



Telangiectatic osteosarcoma is a rare subtype of osteosarcoma. The commonly existed delay in diagnosis or misdiagnosis potentially impairs the outcome and the prognosis. We experienced a case of telangiectatic osteosarcoma presenting as rare refractory hemorrhage, who suffered poor prognosis. Thereafter, we present the case of the telangiectatic osteosarcoma with rare manifestation to avoid further misdiagnosis and improve the outcome and diagnosis.

J.Orthopaedics 2009;6(3)e13


osteosarcoma; telangiectatic osteosarcoma; refractory hemorrhage; misdiagnosis

Telangiectatic osteosarcoma (TOS) represents 2.5%–12.0% of all osteosarcomas.1-6 The combination of operation and chemotherapy has improved the outcome and prognosis of telangiectatic osteosarcoma. Prompt diagnosis is essential for effective treatment. Delay in diagnosis will potentially impair the treatment and outcome. The case, presenting with rare refractory hemorrhage after right femoral shaft fracture treated with internal fixation, was addressed in the paper to avoid further misdiagnosis.


Materials and Methods:

Case presentation
Telangiectatic osteosarcoma presented with refractory hemorrhage is extremely scarce in the literature. We present a patient with right femoral shaft fracture. The patient was initially misdiagnosed as conventional fracture and treated by open reduction and internal fixation in the local hospital. The patients suffered progressive swelling at the distal femur ensuing refractory hemorrhage. Final pathologic examination confirmed a rare variant of osteosarcoma of the telangiectatic type.

Case presentation
An 18-year old boy suffered right femoral shaft fracture with significant pain and swelling when he skipped in the farm and fell down onto the ground. The patient was admitted into the local hospital and the fracture was confirmed on the radiographs (Figure 1). Open reduction and internal fixation was performed (Figure 2). The initial recovery course was uneventful, however, the patient suffered fever and progressive swelling in the right thigh one month later postoperatively. Physical examination found significant swelling and high temperature in the region of incision without other evidence of infection, effusion or pus(Figure 3). Aspiration in the swelling revealed uncoagulated hemorrhage and biopsy found no tumor cells. The patient was transferred to our hospital, a level one trauma center, for definite diagnosis and effective treatment. The stainless steel internal fixation made the MR imaging scan impossible. CT scans with contrast media demonstrated leakage of the contrast medium around the femur, swelling muscle and massive hematoma with apparent compression on the right femoral artery and right deep femoral artery (Figure 4). Arterial injury was suspected and angiograph was conducted from the left femoral artery. The contrast media was found to overflow from the penetrating branches of the deep femoral artery and the above-knee branches of the femoral artery. The injured branches of the deep femoral artery were embolized by gelatin sponge (Figure 5). However, the swelling went on and the blood routine examination showed severe anemia (RBC 2.02*1012/L, HGB 61g/L). Repeat blood transfusion was conducted which could not reverse the condition of anemia. The swelling of the right thigh became so severe that the patient complained of progressive pain and anesthesia which implied osteo-fascial compartment syndrome. Exploration into the swelling was performed. 2000ml unaggregated blood and approximate 400ml necrotic tissue was cleaned out in the right thigh(Figure 6A and 6B). The muscle and other soft tissue were dropsical, fragile and compressed significantly. No apparent injured arteries were identified, however, errhysis was fully filled in the wound which could not be stopped by electric coagulation or ligation due to the crisp muscle. The incision was finally closed with sterile package to stop bleeding(Figure 6C). During operation, 2400ml whole blood and 1000ml plasma was transfused to maintain stable blood pressure.

Figure 1 The comminution fracture of the right femur (A, the anterioposterior view and B, the lateral view).

Figure 2 The fracture was treated with open reduction and internal fixation (A, the anterioposterior view and B, the lateral view).

Figure 3 The right thigh appeared significant swelling and high temperature (A and B).

Figure 4 CT scan with contrast administration demonstrated leakage of the contrast medium around the femur, massive hematoma with apparent compression on the right femoral artery and right deep femoral artery (A) and swelling muscle(B).

Figure 5 During angiograph, the contrast media overflow extensively from the penetrating branches of the deep femoral artery and the above-knee branches of the femoral artery(A). The injured branches of the deep femoral artery were embolized by gelatin sponge (B).

Figure 6 Massive unaggregated blood and necrotic tissue was cleaned out in the right thigh (A and B) and the incision was closed with sterile package to stop errhysis(C)

The package took effect during the first few days after operation, and swelling relieved and the blood routine examination demonstrated rising red cell and hematoglobin. The drainage of errhysis decreased from 400ml per day to 200ml per day. An extensive consultation was performed among the experts in Orthopedics, Hematopathy and Traumatology. They arrived at a conclusion that the patient suffered coagulation disorder-acquired combined deficiency of coagulation factor (deficiency of blood coagulation factors ,, and ). 400ml of fresh frozen plasma and 600U of prothrombin complexity were transfused once a day for two days. The hemoglobin rose as high as 110g/L. The package was dislodged and the incision was closed with a drain pipe on the third day after operation. However, the drainage of errhysis grew more from 400ml/day to 1360ml/day. The red blood cell and the hemoglobin re-decreased to a worse level than the former examination five days later even with persistent application of the fresh frozen plasma, cryoprecipitation, and prothrombin complexity. The patient was transferred to another level I trauma center in Beijing. However the cause of the refractory hemorrhage was still uncertain and the hemorrhage was still uncontrolled. The hemoglobin and the red blood cell continued decrease. The drainage of the errhysis could not decrease. The general condition of the patient became worse and worse. The amputation was finally performed due to high fever and significantly severe anemia. Pathological examination was performed. Characteristically, the lesion is primarily composed of multiple aneurysmal dilated cavities that contained blood and necrotic tissue, with high-grade sarcomatous cells and irregular osteoid in the peripheral rim and septations around these spaces(Figure 7). Some of the malignant cells showed typical mitoses. This confirmed the diagnosis of telangiectatic osteosarcoma. Follow-up studies showed local recurrence and pulmonary metastasis 16 months after surgery.

Figure 7 The thin and irregular osteoid(A) and the typical osteosarcomous cells(B) lied unevenly in the septations of the cysts.

Discussion :

Multiple histologic subtypes of osteosarcoma have been identified, the osteoblastic osteosarcomas, chondroblastic osteosarcomas, fibroblastic osteosarcoma and telangiectatic osteosarcoma. Telangiectatic osteosarcoma typically occurs in the extremities. Its greatest predilection is for the metaphysic of long bones, most often in bones adjacent to the knee joint and shoulder. Like conventional osteosarcoma, pain and swelling are the usual initial symptoms.7, 8 Another common symptom is pathological fracture. TOS was associated with a high rate of pathologic fracture and patients with TOS are at a higher risk of pathologic fractures than patients with conventional OS.1, 9 The rate of pathologic fracture among patients with TOS (17–32%) was significantly higher than that in conventional OS (6-13%).7, 9-12 Weiss report 9 of 21 patients (43%) had pathologic fractures in their study of TOS.13 A even high rate of pathologic fracture (61%) also was noted in a Murphy’s retrospective review of radiologic features in 36 patients with TOS.14 One possible explanation for the high rate of pathologic fractures in patients with TOS is the extensively lytic and cystic nature of the tumor, which may make the bone more prone to fracture.15

The patient suffered pathological fracture during a lower energy activity- skipping in the farm and falling down onto the ground. The review of the lateral and anteroposterior radiographs of the right femur demonstrated osteolytic lesion in the metaphysic with blotches of high density. Radiographs of this typical lesion showed geographic bone lysis, a wide zone of transition, and matrix mineralization. This subtle osteoid was recognized on only 58% of radiographs.13,16 However, the radiological feature was ignored by the local orthopedic surgeon. During the ensuing treatment, the swelling and the refractory hemorrhage confused us. The coagulation disorder was primarily considered as the diagnosis and the therapeutic protocols were mainly aimed to control the refractory hemorrhage. Characteristically, telangiectatic osteosarcoma is primarily (>90%) composed of multiple aneurysmally dilated cavities that contain blood and necrotic tissue, with viable high-grade sarcomatous cells around the periphery and septations of these spaces.1-6,16-20 The telangiectatic osteosarcoma may be more aggressive clinically than conventional osteosarcoma.21, 22 The refractory hemorrhage is the manifestation of the histological features of TOS since the internal fixation has connected the blood-filled cavity with the muscles outside of the periosteum. Definitive diagnosis on the basis of pathological examination was delayed until amputation was conducted. Delay diagnosis resulted in incorrect treatment and poor outcome. Local recurrence and pulmonary metastasis was noticed 16 months after surgery.


Results of the recent studies related TOS indicate that survival has improved apparently which is similar or even better than that of conventional high-grade intramedullary osteosarcoma. The presence of a pathologic fracture was not associated significantly with the type of surgery or patient outcome either. Therefore, it is important to provide accurate diagnosis promptly with ensuing effective treatment. The aim of presenting the case is to demonstrate the rare refractory hemorrhage of TOS. All-round acknowledgment of TOS can confirm the prompt diagnosis of the lesion to avoid misdiagnosis and guarantee the outcome and the prognosis.

Reference :

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This is a peer reviewed paper 

Please cite as: Yingze Zhang: Telangiectatic osteosarcoma: an easily misdiagnosed lesion

J.Orthopaedics 2009;6(3)e13





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