Multimodality management of a case of primary osteogenic sarcoma of the zygoma.
SV Deo, NK Shukla, RK Khazanchi, GK Rath, L Chandy, MB Prakash
Department of Surgical Oncology, Surgery Radiation Oncology, Medical Oncology and Pathology, Institute Rotary Cancer Hospital (IRCH), All India Institute of Medical Sciences (AIIMS), New Delhi., India
S V Deo
Department of Surgical Oncology, Surgery Radiation Oncology, Medical Oncology and Pathology, Institute Rotary Cancer Hospital (IRCH), All India Institute of Medical Sciences (AIIMS), New Delhi.
Craniofacial osteogenic sarcomas are rare primary malignant bone tumors and very few cases involving zygomatic bone were reported in literature. We present our experience of multimodality management of a case of primary osteogenic sarcoma of zygoma. Wide radical excision of the tumor including the parotid gland was done followed by three cycles of adjuvant chemotherapy and fifty Gy of external radiotherapy. The patient is disease-free at two years follow-up. Till 1970s, craniofacial osteogenic sarcomas were managed mainly by radical surgery with a high local failure rate. With the advances made in the field of radiotherapy and chemotherapy, multimodality therapy is playing a major role in the treatment of these aggressive tumors with better overall and disease-free survival.
|How to cite this article:|
Deo S V, Shukla N K, Khazanchi R K, Rath G K, Chandy L, Prakash M B. Multimodality management of a case of primary osteogenic sarcoma of the zygoma. J Postgrad Med 1995;41:13-5
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Deo S V, Shukla N K, Khazanchi R K, Rath G K, Chandy L, Prakash M B. Multimodality management of a case of primary osteogenic sarcoma of the zygoma. J Postgrad Med [serial online] 1995 [cited 2021 Apr 21 ];41:13-5
Available from: https://www.jpgmonline.com/text.asp?1995/41/1/13/476
Craniofacial osteogenic sarcomas (CFOGS) are exceedingly rare primary malignant bone Tumours. Their incidence varies from 6 to 9% of all osteogenic sarcomas,,. GFOGS commonly affect the patients in younger group. The frequently involved sites are mandible, maxilla, hard palate and vault of the skull. Zygoma is a very rare site of involvement ,. CFOGS are very aggressive Tumours and difficult to treat as radical surgical removal may not be feasible in all cases. Multimodality management of a case of primary osteogenic sarcoma involving the zygomatic bone presented to our clinic and review of literature regarding the incidence and management of CFOGS in general are discussed in this article.
A 22 year old man presented with a recurrent 6 cm x 4 cm hard painless, swelling, 4 cm lateral to the outer can thus of left eye [Figure:1a] and [Figure:1b] to Institute Rotary Cancer Hospital in January 1992. A simple excision was done by a private practitioner in June 1991 and no details were available with the patient at the time of presentation to our centre. There was no clinical or radiological evidence of intraorbital or intracranial extension. Plain X Ray and CT scan revealed areas of destruction with new bone formation in the left zygomatic region with a large soft tissue component. Trucut biopsy of the lesion revealed features of osteogenic sarcoma. Chest Xray, haemogram and liver function tests were normal. The patient was taken up for surgery and a radical excision of the Tumour sacrificing the left parotid gland was done.
Histopathological examination of the specimen showed large areas of cartilaginous tissue surrounded by spindle cell stroma and areas of tumour osteoid production [Figure:2] consistent with chondrosarcomatous type of osteogenic sarcoma. Resected margins of the surgical specimen were free of tumour.
Subsequently, the patient received 3 cycles of adjuvant chemotherapy consisting of cisplatinum (120 mg/m2) +. adriamycin (30 mg/m2) alternating with bleomycin (12 mg/m2) + cyclophosphamide (600 mg/ m2) + dactinomycin (450 mg/m2). This was followed by 50 Gy of external radiotherapy in 25 fractions over a period of 5 years. The patient is under regular follow up at monthly intervals and alive and diseasefree at 2 years followup.
Osteogenic sarcoma is the most common primary malignant bone tumour excluding plasma cell tumours. It commonly involves the appendicular skeleton. Osteogenic sarcoma arising from craniofacial bones is well known but a rare entity. Mayo Clinic reported an incidence of 9.7 % and Memorial Sloan Kettering Cancer Centre 7% of all osteogenic sarcomas. The commonly involved bones in order of frequency are jaw bones, palate and skull vault. Involvement of zygomatic bone is very rare,. CI`GOS are classified into primary and secondary types. Secondary CFOGS occur in patients of skeletal Paget's disease, fibrous dysplasia of bone and as a late sequela to craniofacial irradiation. Patients of primary CFGOS are younger (mean age 48 years). Majority of CFOGS occur in skeletally mature patients in contrast to those that affect the appendicular skeleton. CFGOS are very aggressive tumours and 412 50% develop distant metastasis within 2 years of diagnosis. Imaging plays a very crucial role in the diagnosis and treatment planning of CFOGS. Computed tomography is routinely recommended to know the extent of bone involvement, extraosseous soft tissue extension and for precise definition of invasion into pterygopalatine fossa, infratemporal fossa and cranial cavity. Four distinct histological varieties (1) fibrous sarcomatous (2) chondrosarcomatous (3) osteoblastic and (4) Poorly differentiated type are described in literature. Evolution of therapy of CFOGS has slowly progressed from single modality therapy till 1 970s to multimodality therapy in 1990s. Till 1970s radical surgery played a major role, its main disadvantage being a high local failure rate (50% to 60%). But with the better understanding of radiobiology of bone tumours and advances made in radiotherapy techniques, high dose of radiotherapy can be delivered in the form of external beam radiotherapy or interstitial radiotherapy sparing adjacent important structures like eye and brain. By giving preoperative operative or post-operative radiotherapy, the local failure rate has come down significantly from 60% to 30%,.
With the success achieved in the management of extremity osteogenic sarcomas with combination chemotherapy, few centres have started giving combination chemotherapy in CFOGS patients to improve the, overall survival. Sundaresan et al have reported a 10 yr. survival of 30% in CFOGS patients treated with multimodality therapy. Recently, more and more centres, are using multimodality therapy in treating CFOGS.
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