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 Table of Contents  
CASE REPORT
Year : 2021  |  Volume : 54  |  Issue : 1  |  Page : 28-31

Atypical telangiectatic osteosarcoma


1 Faculty of Medicine, University of Indonesia, Jakarta, Indonesia
2 Department of Orthopedic and Traumatology, Faculty of Medicine, Cipto Mangunkusumo General Hospital, University of Indonesia, Jakarta, Indonesia
3 Division of Orthopedic Oncology, Department of Orthopedic and Traumatology, Faculty of Medicine, Cipto Mangunkusumo General Hospital, University of Indonesia, Jakarta, Indonesia

Date of Submission04-May-2020
Date of Decision19-Jun-2020
Date of Acceptance01-Sep-2020
Date of Web Publication22-Jan-2021

Correspondence Address:
Kevin Jonathan Adhimulia
Department of Orthopedic and Traumatology, Cipto Mangunkusumo General Hospital, Pangeran Diponegoro No.71, 10430, Jakarta
Indonesia
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/fjs.fjs_63_20

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  Abstract 


Telangiectatic osteosarcoma (TOS) is a rare malignant bone tumor. The main clinical and radiological significance of the case presentation is TOS can be easily misdiagnosed as a benign lesion such as an aneurysmal bone cyst (ABC). In this case, we present 63-year-old male with a large mass in the left leg. Imaging modalities were obtained in this patient to define the diagnosis. In this atypical case of TOS, relatively old age presentation, slow-progressing lesion, well-demarcated lesion, and lack of periosteal reaction further accentuate the similarity between TOS and ABC or other benign lesion causing high rate misdiagnosis of TOS. The histopathological examination will finally differentiate TOS from ABC.

Keywords: Aneurysmal bone cyst, atypical presentation, telangiectatic osteosarcoma


How to cite this article:
Adhimulia KJ, Alfariq L, Kamal AF. Atypical telangiectatic osteosarcoma. Formos J Surg 2021;54:28-31

How to cite this URL:
Adhimulia KJ, Alfariq L, Kamal AF. Atypical telangiectatic osteosarcoma. Formos J Surg [serial online] 2021 [cited 2021 Apr 13];54:28-31. Available from: https://www.e-fjs.org/text.asp?2021/54/1/28/307624




  Introduction Top


Telangiectatic osteosarcoma (TOS) is a rare type of osteosarcoma that comprises around 3% of all osteosarcoma and 2%–12% of all appendicular skeletal tumors.[1],[2] Osteosarcoma itself, is the commonly found bone tumor with an incidence of 4–5/1,000,000 of all population.[3] The most common site for TOS is the femur, followed by tibia and humerus while being less common in pelvis, fibula, skull, and ribs. Historically, this type of aggressive lesion is described as “malignant bone aneurism” in 1903 by Gaylord and “hemorrhagic osteosarcoma” in 1971 by Campanacci.[4] This type of aggressive osteosarcoma commonly occurs in children, adolescence, and young adults.[1]

TOS is distinguished from classic osteosarcoma by the histopathological features that show spaces that commonly filled with blood and separated by septum containing highly malignant cells.[1],[4] These radiological finding shows a highly lytic lesion of the bone with absence or minimal sclerotic lesion.[2] These clinical and radiological appearances of TOS are similar to another benign lesion especially aneurysmal bone cyst (ABC). In some cases, the clinical and radiological appearance of these two conditions is very similar, causing high rate misdiagnosis of TOS.[1] This malignant tumor is commonly treated by limb salvage surgery (LSS) or amputation with chemotherapy before the surgery.[3]

This case report presents the finding of TOS in the left proximal tibia of a 63-year-old male with unusual radiographic findings. Our target in assessing the case is to have a better understanding in interpreting the radiological finding to diagnose TOS more accurately.


  Case Report Top


A 63-year-old male was referred to our hospital with large mass in the left leg since 7 months before admission. At first, the mass was about the size of marble and located below the left knee with progressive enlargement within the next 7 months. The patient felt neither pain in palpation and movement nor limitation in the range of movement of the knee joint. The patient decline any history of trauma to the left knee and any previous treatment to the lesion what so ever. Four months before admission, continuous pain began to occur in the area of the mass which increased by activity. The pain is not alleviated by rest.

The physical examination revealed an irregular mass with a circumferential length of 65 cm compared with 28 cm in contralateral extremity [Figure 1]. Skin induration and distal edema were absent, while tenderness could be found with visual analog scale of 2–3. The movement of the local knee was within normal limit. On laboratory examination, erythrocyte sedimentation rate was 82 mm/h (normal: <10 mm) and lactic dehydrogenase was 381 U/L (normal: <225 U/L).
Figure 1: Clinical pictures with large mass in the left proximal lower leg with circumferential length 65 cm compared to 28 cm in the contralateral lower leg. There are neither sign of inflammation nor induration of the skin

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Radiography of the left tibia showed geographic and moth-eaten type of lytic lesion with a well-defined margin which extended from epiphysis to the mid-shaft of the tibia [Figure 2]. There was neither of sclerotic thickening of the cortex nor periosteal reaction around the lesion. The striation of calcification was shown inside the cystic lesion. Magnetic resonance imaging (MRI) showed destruction of the epiphysis, metaphysis, and diaphysis of the proximal tibia which soft-tissue mass was found within the lesion. The tumor size was 5.8 cm × 5.2 cm × 16.6 cm extending to the anterior tibial muscle, popliteal muscle, posterior tibial muscle, and anterior subcutaneous tissue. On short T1 inversion recovery (STIR) MRI, it demonstrated high signal intensity within the mass [Figure 3]a. On T1-weighted MRI, the mass showed homogeneous low-signal intensity [Figure 3]b. No fluid-fluid level within the lesion was demonstrated [Figure 3]a and [Figure 3]b. There were neither signs of intra-articular nor neurovascular involvement. A chest computed tomography (CT) scan showed no metastasis of the lungs. The histopathologic study from core biopsy was consistent with malignant sarcoma. The patient received a series of neoadjuvant chemotherapy and underwent LSS with en block excision and knee arthrodesis with metallic-bone cement composites [Figure 4]. The gross appearance of the tumor shows a cystic lesion filled with hemorrhage and calcified septa [Figure 5]. The histopathological examination from the excised mass showed an atypical stromal cell with multiple nuclei, nuclear hyperchromasia, atypical mitoses, and pleomorphism with patches of hemorrhage in the spaces of the tissue lesion which was consistent with TOS [Figure 6].
Figure 2: Antero-posterior and lateral radiographs of the left tibia showed geographic and moth-eaten lytic appearances extending from epiphyseal, metaphyseal, and diaphyseal without intra-articular extension. There is cortical destruction in the anterior side of tibia without any periosteal reaction. Striated calcification is shown inside of the lesion

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Figure 3: (a) Magnetic resonance imaging examination shows a multiloculated cystic lesion, some with thick lesion with extensive soft-tissue protrusion. Short T1 inversion recovery shows homogeneous high-signal intensity. (b) On T1-weighted imaging, the mass shows homogenous low-signal intensity and septation some with thickening. Both images show no fluid-fluid level in the lesion. The homogeneity of the signal intensity suggesting benign lesion

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Figure 4: Postoperative radiograph of limb salvage surgery with en block excision and knee arthrodesis with metallic-bone cement composites

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Figure 5: Gross appearance shows cystic lesion filled with hemorrhage and necrotic tissue. The space is separated by calcified septa across the cystic lesion. There are signs of cortical destruction and soft-tissue protrusion

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Figure 6: (a) A histological examination from the biopsy of the surgery. On × 400 magnification, the biopsy shows hemorrhage and necrotic debris within the cystic lesion without endothelial lining. Within the cystic lesion, septa containing atypical stromal cell were identified. (b) On × 1000 magnification, shows atypical stromal cell with multiple nuclei, nuclear hyperchromasia, atypical mitoses, and pleomorphism (black arrow) with patch of hemorrhage in the spaces of the tissue. Osteoid formation is clearer in this image. Both the pathological anatomy examination consistent with telangiectatic osteosarcoma

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  Discussion Top


TOS commonly occurs in children, adolescence, and young adults.[1] The median age of presentation in TOS is 17.5-year-old with a predominance of male patients.[2] TOS rarely occurs in elderly patients. Some tumors may have atypical clinical and radiological presentations when they occur outside their normal age group.[4] The most common location of the lesion is in the metaphyseal bone of the distal femur (42.6%), followed by proximal tibia (16.9%), and proximal humerus (9.2%). TOS is commonly found in the initial complaint of local mass in distal femur or proximal tibia with or without recent local pain.[2] In this case, we found that the patient is clearly outside the age group which TOS typically occurs. Some reports showed cases with patients with 51- and 78-year-old who had TOS.

It is stated that some of the tumors with onset outside of the age group have a quite unusual clinical and radiological presentation. Typically, TOS presented as recent onset of pain, soft-tissue lump, or both.[2] Radiological presentation usually shows a lytic lesion with a geographic pattern with minimal intralesional sclerosis.[2],[4] Both TOS and ABC present pain and/or soft-tissue lump as clinical presentation. However, the progression of lump size and pain is typically faster in TOS, similar with conventional osteosarcoma.[1],[2] It is clearly shown in this case which has an atypical clinical and radiological presentation.

TOS shows mostly lytic appearance of the bone with a minimal or complete absence of sclerotic appearance.[1] The image often shows a wide zone of transition and indefinite margin, with the most common lytic pattern being geographic (94%), while the rest are moth-eaten and permeative.[1],[5] Small dense area of calcification with curvilinear or lobular configuration are commonly present in the bone component, soft tissue, or both.[6] In some cases, osteoid production does not occur suggesting ABC.[6] There are soft-tissue extension with total destruction of the cortex.[1],[2]

Typically, periosteal reaction such as Codman's triangle and sunburst appearance is found in the result suggesting the malignant nature of the lesion.[5] However, in this patient's radiograph examination, the lesion demonstrates geographic and moth-eaten lytic appearance without any periosteal reactions. Radiopaque striation inside the lesion displayed extensive calcification and osteoid production. The appearance of intralesion opaque striation is rare and usually only appear in CT-scan examination.[2] It may be caused by hemorrhage and necrotic tumor dominated cystic lesion.[7]

MRI examination is considered as the main diagnostic modality to differentiate TOS from ABC. Typically, MRI shows heterogeneous signal intensity with the dominance of low-signal intensity in T1-weighted images and high-signal intensity in T2-weighted images.[7],[8] In this case, T1-weighted images shows low-signal intensity with high-signal intensity with STIR. Multiloculated cystic pattern is also seen in STIR with nodular appearance in the periphery of the lesion. High-signal intensity in STIR shows the present of hemorrhage inside the cystic lesion. Protrusion of lesion shows significant soft-tissue extension and cortical destruction. However, heterogeneous signal intensity and fluid-fluid level that typically present in TOS is not present in this MRI. Meanwhile, homogeneous signal with low-signal intensity in T1-weighted imaging display minimal septation and peripheral thickening of cystic lesion suggesting benign lesion such as ABC.[6]

On gross examination, both lesions TOS and ABC have cavity filled with blood with expanding tissue inside the bone.[4] Sometimes, solid mass and septation inside of the cyst are also found in ABC [Figure 5]. In TOS, there are nodular tissue around the cyst with finding of malignant stromal cells that are found in the septal and peripheral area of the lesion.[2] The histopathological examination finally confirms the diagnosis of TOS. The septal contains atypical stromal cells with multiple nuclei, nuclear hyperchromasia, atypical mitoses, and pleomorphism. Strings of osteoid matrix are found between the malignant cells showing an osteoid production and mineralization inside the septal which demonstrates as TOS.


  Conclusion Top


In atypical case of TOS, the clinical and radiological appearance of TOS is similar to other benign lesion ABC causing high rate misdiagnosis of TOS. The histopathological examination finally will-differentiate TOS from ABC. It is universally accepted that the management of patient with this case needs a team approach that works in close cooperation to confirm the diagnosis.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient has given his consent for his images and other clinical information to be reported in the journal. The patient understands that his name and initials will not be published and due efforts will be made to conceal the identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Liu JJ, Liu S, Wang JG, Zhu W, Hua YQ, Sun W, et al. Telangiectatic osteosarcoma: A review of literature. Onco Targets Ther 2013;6:593-602.  Back to cited text no. 1
    
2.
Sangle NA, Layfield LJ. Telangiectatic osteosarcoma. Arch Pathol Lab Med 2012;136:572-6.  Back to cited text no. 2
    
3.
Kamal AF, Widyawarman H, Husodo K, Hutagalung EU, Rajabdo W. Clinical outcome and survival of osteosarcoma patients in Ciptomangun Kusumo Hospital: Limb salvage surgery vs. amputation. Acta Med Indonesa 2016;48:175-83.  Back to cited text no. 3
    
4.
Greenspan A, Jundt G, Remagen W. Differential Diagnosis in Orthopedic Oncology. 2nd ed. Philadelphia: Lippincott Williams and Wilkins; 2007.  Back to cited text no. 4
    
5.
Murphey MD, wan Jaovisidha S, Temple HT, Gannon FH, Jelinek JS, Malawer MM. Telangiectatic osteosarcoma: Radiologic-pathologic comparison. Radiology 2003;229:545-53.  Back to cited text no. 5
    
6.
Gao ZH, Yin JQ, Liu DW, Meng QF, Li JP. Preoperative easily misdiagnosed telangiectatic osteosarcoma: Clinical-radiologic-pathologic correaltion. Cancer Imaging 2013;13:520-6.  Back to cited text no. 6
    
7.
Discepola F, Powell TI, Nahal A. Telangiectatic osteosarcoma: Radiologic and pathologic finding. Radiographics 2009;29:380-3.  Back to cited text no. 7
    
8.
Yarmish G, Klein MJ, Landa J, Lefkowitz RA, Hwang S. Imaging characteristics of primary osteosarcoma: Nonconventional subtypes. Radiographics 2010;30:1653-72.  Back to cited text no. 8
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6]



 

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