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Table of Contents    
Year : 2011  |  Volume : 59  |  Issue : 1  |  Page : 117-119

Primary telangiectatic osteosarcoma of occipital bone: A case report and review of literature

1 Department of Neurosurgery, Nizam's Institute of Medical Sciences, Hyderabad, India
2 Department of Pathology, Nizam's Institute of Medical Sciences, Hyderabad, India
3 Department of Neurosurgery, NRI Medical College, Mangalagiri, Hyderabad, India
4 Department of Neurosurgery, Krishna Institute of Medical Sciences, Hyderabad, India

Date of Submission23-Jun-2010
Date of Decision19-Jul-2010
Date of Acceptance06-Aug-2010
Date of Web Publication18-Feb-2011

Correspondence Address:
Manas K Panigrahi
Department of Neurosurgery, Krishna Institute of Medical Sciences, Hyderabad, Andhra Pradesh
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0028-3886.76891

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 » Abstract 

Telangiectatic osteosarcoma (TOS), an uncommon variant of osteosarcoma, involving skull bones is extremely rare. We present clinico-pathological, imageological and treatment outcome of a primary TOS of occipital bone in a 30-year-old woman and review the previously reported skull bone TOS. We suggest that TOS should be included in the differential diagnosis of destructive lytic lesions involving the skull bones. As radical surgical procedures are not applicable to skull bones, the outcome is poor even with adjuvant chemotherapy.

Keywords: Lytic lesion, occipital bone, osteosarcoma, posterior fossa, telangiectatic

How to cite this article:
Patibandla MR, Uppin SG, Thotakura AK, Panigrahi MK, Challa S. Primary telangiectatic osteosarcoma of occipital bone: A case report and review of literature. Neurol India 2011;59:117-9

How to cite this URL:
Patibandla MR, Uppin SG, Thotakura AK, Panigrahi MK, Challa S. Primary telangiectatic osteosarcoma of occipital bone: A case report and review of literature. Neurol India [serial online] 2011 [cited 2023 Nov 29];59:117-9. Available from:

 » Introduction Top

Extragnathic osteosarcoma involving skull bones is uncommon. Telangiectatic osteosarcoma (TOS), an uncommon variant of osteosarcoma, involving skull bones has been rarely reported and to date, only 5 cases of TOS involving the skull bone have been reported in the world literature. [1],[2],[3],[4]

 » Case Report Top

A 30-year-old woman was brought to the Emergency Department with a 15-day history of occipital headache and 2-day history of nausea and two episodes of vomiting. Clinical examination revealed fine nystagmus and bilateral papilledema. Computerized tomogram (CT) scan of skull (bone window) showed a lytic lesion causing destruction of left occipital condyle with surrounding soft tissue mass with extension into the posterior fossa up to petrous bone [Figure 1]. On magnetic resonance imaging (MRI), it was a well-defined, extra-axial mass measuring 4 × 3 × 3.5 cm, involving the left occipital condyle with extension into posterior fossa causing mass effect on fourth ventricle. The mass was hyperintense on T2WI and predominantly hypointense on T1WI with heterogenous enhancement after administration of contrast [Figure 2]a and b.
Figure 1: Computerized tomogram scan (bone window) showing lytic lesion causing destruction of left occipital condyle with surrounding soft tissue mass

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Figure 2: Magnetic resonance imaging (with contrast) — (a) axial and (b) sagittal showing well-defined, relatively heterogenously enhancing extra-axial mass arising from the left occipital condyle extending into posterior fossa up to petrous bone

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Near-total excision of the tumor was done by left far lateral approach with placement of left frontal reservoir. Peroperatively, the tumor was found to be reddish, hemorrhagic, highly vascular and non-suckable. It was well demarcated from cerebellum and showed areas of necrosis and calcification. The tumor was seen eroding occipital bone with extension up to lateral border of C2 vertebra and infiltration into sub-occipital muscles. The patient had an uneventful postoperative period and was discharged on seventh postoperative day.

Histopathological examination revealed multiple blood-filled spaces of variable sizes lined by spindle cells and giant cells [Figure 3]a. The intervening area showed spindly-to-polygonal tumor cells with oval-to-elongated pleomorphic, hyperchromatic nuclei. Mitotic figures were frequent, including the atypical mitotic figures [Figure 3]b. Admixed with these cells, there were tumor giant cells and osteoclastic giant cells. Focal areas showed thin lacy osteoid traversing the tumor cells [Figure 3]c. Immunohistochemistry with CD 31 was negative in tumor cells, ruling out the possibility of angiosarcoma. The above histological features were consistent with the diagnosis of TOS.
Figure 3: Histopathological section showing (a) multiple blood-filled spaces separated by septae containing tumor cells (H and E, ×40). (b) Tumor cells showing nuclear pleomorphism and hyperchromasia and increased mitotic activity (H and E, ×400). (c) Fine lacy osteoid traversing the tumor cells (H and E, ×400)

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Bone scan; and CT scan of chest, abdomen and pelvis showed no evidence of systemic involvement. Postoperative CT scans done 2 weeks after surgery showed contrast enhancement at the surgical margins, suggestive of residual tumor. Postoperatively the patient received 53 Gy of radiation over 6 weeks in 30 fractions, followed by 6 cycles of cisplatin, adriamycin and ifosphamide-based chemotherapy. Follow-up CT and MRI scans at 4, 6, 9 and 12 months showed recurrence of tumor with progressively increasing size [Figure 4]. The patient succumbed to the disease at 16 months after surgery in spite of aggressive adjuvant radiotherapy and chemotherapy.
Figure 4: MRI scans done 4 months after surgery showing recurrent tumor

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 » Discussion Top

Osteosarcoma (OS) is the most common primary malignant bone tumor presenting in childhood and adolescence. The head and neck osteosarcomas account for 0.5% to 8.5% and 75% to 88% with location in jaws. [5] TOS accounts for 0.4% to 12% of all skeletal osteosarcomas. [2],[6] These tumors commonly (75%) occur in the metaphyseal region of long bones [7] , but are known to arise from mandible, vertebrae, scapula and innominate bone. [7] Extragnathic TOSs have been rarely reported at unusual sites such as sphenoid bone [4] and frontal bone. [3]

The hallmark of TOS is expansile lytic lesion without sclerosis [6] and often exhibits heterogenous high signal intensity on T2WI and fast spin T2WI and may also show zones of high signal intensity on T1WI and proton density (PDWI) corresponding to areas of hemorrhage. These tumors show prominent gadolinium enhancement. [4],[7] Fluid-fluid level is a nonspecific feature, which represents the hemorrhage. [8] Radiologically the closest possible differential diagnosis for TOS is aneurysmal bone cyst, giant cell tumor or angiosarcoma and histopathology examination is essential to diagnose these tumors. [4],[8] TOS should be considered as a diagnostic possibility when either CT or MR studies show a lytic lesion with fluid-fluid levels. [4],[6],[8] However, our patient did not show the fluid-fluid levels. All cases of skull bone TOS, so far reported including our case, have shown characteristic radiological features. [3],[4]

Histopathological studies in correlation with imageological findings are essential for the diagnosis of TOS. [8] The histological and radiological diagnostic criteria proposed by the World Health Organization include: 1) predominantly lytic bone mass with minimal sclerosis; 2) grossly cystic medullary mass with no or minimal solid or sclerotic component; and 3) histologic features consisting of bone-forming tumor with notable blood-filled spaces separated by septae lined by, and/ or containing, malignant tumor cells with prominent nuclear atypia and limited osteoid deposition. [9] Histological findings of all the previously reported cases of skull bone TOS, including the present case, were characteristic. [1],[2],[3],[4]

Treatment modality of TOS involves a combination of aggressive surgery with good margins, radiotherapy and multi-agent chemotherapy. [4] Though TOS is highly chemo-sensitive compared to conventional OS, [10] at present, using multi-modality therapy, the outcomes of TOS and conventional type are considered similar. [2],[8] Neoadjuvant chemotherapy can be administered to these patients if diagnosis can be achieved before surgery on a biopsy, which is not possible in all skull base tumors. Local recurrence is the major cause of death in skull bone osteosarcomas. The two important factors that are predictive of better outcome are (i) absence of local progression on chemotherapy and (ii) use of three or more chemotherapeutic agents. [8]

 » References Top

1.Donato G, Lavano A, Volpentesta G, Chirchiglia D, Veraldi A, De Rose F, et al. Telangiectatic osteosarcoma of the skull. A post-Paget case. Clin Neuropathol 1997;16:201-3.  Back to cited text no. 1
2.Huvos AG, Rosen G, Bretsky SS, Butler A Telangiectatic osteogenic sarcoma: A clinicopathologic study of 124 patients. Cancer 1982;49:1679-89.   Back to cited text no. 2
3.Merino S, Arrazola J, Saiz A, Blanco JA, Ortega L. Post-Paget telangiectatic osteosarcoma of the skull. Skeletal Radiol 1999;28:470-2.  Back to cited text no. 3
4.Whitehead RE, Melhem ER, Kasznica J, Eustace S. Telangiectatic osteosarcoma of the skull base. AJNR Am J Neuroradiol 1998;19:754-7.  Back to cited text no. 4
5.Defries CH, Perlin CE, Liebel SA. Treatment of osteogenic sarcoma of the mandible. Arch Otolaryngol Head Neck Surg 1979;105:538-59.  Back to cited text no. 5
6.Mervak TR, Unni KK, Pritchard DJ, McLeod RA. Telangiectatic osteosarcoma. Clin Orthop Relat Res 1991;270:135-9.  Back to cited text no. 6
7.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. 7
8.Weiss A, Khoury JD, Hoffer FA, Wu J, Billups CA, Heck RK, et al. Telangiectatic osteosarcoma: The St. Jude Children's Research Hospital's experience. Cancer 2007;109:1627937.  Back to cited text no. 8
9.Matsuno T, Okada K, Knuutila S. Telangiectatic osteosarcoma. In: Fletcher C, Unni K, Mertens F, editors. Tumors of Soft Tissue and Bone. Lyon, France: IARC Press; 2000. p. 271-2.  Back to cited text no. 9
10.Rosen G, Huvos AG, Marcove R, Nirenberg A. Telangiectatic osteogenic sarcoma. Improved survival with combination chemotherapy. Clin Orthop Relat Res 1986;207:164-73.  Back to cited text no. 10


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

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