Introduction

Head and neck osteosarcoma is a unusual primary malignant bone tumor (< 10% of all osteosarcoma) with aggressive behaviour which often requires multimodal management for better outcome [1]. It has got wide range of age distribution even though it occurs slightly at younger age than extremity variant, males are most commonly affected. Very few patients gives history of radiation exposure in the development of head and neck osteosarcoma before the diagnosis which says that radiation is one of the aetiologies explained [2]. Surgical excision forms the most important variable in the management of head and neck osteosarcoma [3]. We have reviewed 25 patients of head and neck OS in our tertiary cancer care center (Table 1).

Table 1 Study design characteristics
Full size table

Materials and Methods

Retrospective study of all the patients visited our head and neck OPD with the diagnosis of Osteosarcoma who are further evaluated and treated in Gujarat Cancer Research Institute, India during the period between 2013 and 2019 done after taking the ethical committee clearance.

All the patients were assessed by detailed documented clinical history and physical examination, relevant blood investigations, biopsy and imaging study. Contrast enhanced computed tomography is used to evaluate the local extent of the lesion. Treatment plan was decided on individual basis in multidisciplinary tumor board meeting (Table 2).

Table 2 Comparison of similar studies
Full size table

Results

Total 25 patients with diagnosis of head and neck osteosarcoma were further evaluated and treated in our tertiary cancer care center between 2013 and 2019 are included in our study. Gender distribution in our study shows 14 male and 11 female patients with age range from 10 to 55 years. Mean age of the study is 27.5 years.

The site of primary tumor is mandible in 18 patients, maxilla in 3 and 2 in nasal cavity, one in sphenoid sinus and infratemporal fossa each. None of the patients had previous history of radiation exposure in the past.

20 patients received surgery as the primary curative treatment out of 25 patients. 5 patients received curative chemoradiotherapy as these patients were medically unfit for surgery/unwilling for surgery. Among surgically treated patients, 6 patients received neoadjuvant chemotherapy which was decided on the basis of advanced size of the lesion with local soft tissue extent. Regimen of the same was minimum 2 cycles of cisplatin and adriamycin. Response assessment after neoadjuvant chemotherapy was done with contrast enhanced computed tomography of head and neck region.

Curative surgery was done in the form of mandibulectomy (segmental, hemi or extended) or maxillectomy. Two patients underwent craniofacial resection.

Reconstruction was done either with free fibula, locoregional flaps such as temporalis or pectoralis major myocutaneous flap. Neck dissection was selectively done in 4 patients either level 1a and 1b lymph node dissection in free flap, and supraomohyoid neck dissection in other three patients but none of the patients had nodal metastasis in final histopathological specimen. Osteoblastic variant was the most common subtype found in our study followed by chondroblastic type.

About 18 patients received adjuvant treatment which includes both chemotherapy and radiotherapy in 10 patients, only chemotherapy in 5 patients, whereas 3 patients received only radiotherapy.

Recurrence was noted in 14 patients (local—8, distant—6) out of 25 patients.

Two patients underwent surgical resection after neoadjuvant chemotherapy for local recurrences whereas all other patients of local recurrences were managed with either chemotherapy and/or radiotherapy in palliative setting. Lung, brain were the site of distant recurrences. All distant recurrences were managed with palliative chemotherapy.

Median DFS of our study was 16 months and median OS was 36 months, irrespective of the modality of the treatment. All five patients who did not underwent surgery were died and median OS was only 11 months. 2 yr survival in our study is 60%and 5 year overall survival is 42%.

Discussion

Osteosarcomas are primary malignant tumors of bone that are characterized by the production of osteoid or immature bone by the malignant cells. It is the most common primary malignant tumor of bone with usual occurrence in extremities and almost about 10 percent of cases arise in the head and neck region [4]. The median age at presentation is 36 years that is at least 10 years older than patients with non-head and neck osteosarcomas [2], although for non-head and neck osteosarcomas, there is a second peak in incidence in later adulthood that corresponds to secondary Paget disease-related osteosarcoma. Mean age of our study population is 27.5 years which is slightly younger age group than the usual presentation.

Head and neck osteosarcoma most commonly arise in the upper and lower jaws. In comparison to previous studies, our study also showed that the mandible is the most common site involved (72%) followed by maxilla [1]. The classical presentation is that of a mass or swelling in the cheek or jaw, sometimes associated with pain, and occasionally with dental complaints [5, 6]. All of our patients presented with complaints of mass, and local pain experienced by few patients. Local symptoms like diplopia, nasal discharge, epistaxis also noted in patients with paranasal sinus site as tumor. Gender distribution showed slight male predominance (M: F 1.08:1) which is similar to other previous studies [7].

Extremity osteosarcoma is ideally managed with neoadjuvant chemotherapy followed by surgery. Unlike extremity variant, head and neck subsite is usually managed by upfront surgery as shown in many previous studies. We also believe that chemotherapy in neoadjuvant setting is not beneficial in the management of head and neck osteosarcoma as shown in meta-analysis since we also did not see any advantage in 6 patients who had received neoadjuvant chemotherapy. Few authors in contrast says that neo-adjuvant chemotherapy helps by improving local control, by decreasing the incidence of lung metastases and also by prolonging the time to development of lung metastases [5, 8, 9].

The principle of surgery for head and neck OS is wide excision with adequate margins which is much more than other usual histology like squamous cell carcinoma. Adequate margin for head and neck osteosarcoma considered to be minimum of 2 cm of bony margins with soft tissue margins of 0.5 cm as the risk of local recurrences increases if margin is less than 1 cm [8, 10]. But due to the complex anatomy of the head and neck, adequate wide margins are not able to achieve in all cases [11], reflect the higher incidence of margin positivity and local recurrences in head and neck subsite osteosarcoma whereas extremity type usually display higher chance of distant recurrences [12, 13]. Margin positivity in our study group is 20% which is slightly lesser than other studies.

Lymph node involvement is very unusual in all the variants of osteosarcoma, hence routinely lymph node dissection is not required during surgery even though selective neck dissection can be done if clinically indicated [13,14,15]. Most of these tumors are high grade which often represents high local recurrence requiring adjuvant treatment like chemotherapy [16]. Low grade tumors can be managed with surgery alone if adequate margins can be achieved. Adjuvant radiotherapy is only indicated in positive margins cases which helps in decreasing the local recurrences [1, 14].

We had considered several factors to decide about adjuvant treatment like grade of the tumor, margin positivity, advanced size of the lesion (> 7 cm) and adverse histopathological features like perineural invasion [2]. Mandible site tumors have better results compared to maxilla as it is easy to get adequate anatomical margins for mandible and due to high vascularity of maxilla, they tend to have more distant metastasis [7]. Only 2 patients who underwent surgery did not receive adjuvant treatment who had no adverse features on histopathology. Similar to previous studies, our results also showed higher local recurrences than distant metastasis which were managed either with surgery if feasible or palliative chemotherapy or radiotherapy. 5 year survival of head and neck osteosarcoma cases in various studies ranges from 30 to 63%, with our study demonstrating considerable 5 year overall survival of 42% [2].

Outcome of our study design clearly demonstrated that surgery as the principle curative modality of treatment which stands tall among multimodality management of head and neck osteosarcoma [1, 2, 7, 17,18,19] as surgery with positive margins decreases the survival (5 year survival 25% vs 62%). Surgery alone or with chemotherapy has similar survival (mean DFS 44 months vs 42 months) even though few differ in this. Adjuvant treatment especially, radiotherapy has a survival advantage when it is given either alone or with chemotherapy by decreasing the local recurrence (mean DFS 58 months vs 43 months). Similar outcomes shown by various previous studies as well [1, 2, 20] (Figs. 1, 2, 3).

Fig. 1
figure 1

Kaplan–Meir chart showing the superiority of surgery over nonsurgical management

Full size image
Fig. 2
figure 2

Kaplan–Meir chart showng the role of adjuvant radiotherapy in survival

Full size image
Fig. 3
figure 3

Kaplan–Meir chart showing no additional benefit with adjuvant chemotherapy

Full size image

Conclusion

Head and neck osteosarcoma, in our experience primarily affecting the jaw bone which has got aggressive clinicopathological features. Surgical wide excision played a pivotal role in the management of these cases. Adjuvant treatment like chemotherapy and/or radiotherapy is needed as surgically free margins are difficult to achieve due to complex anatomy of the head and neck with preservation of aesthetic and functional outcome. Among the various factors studied for the prognosis, nonsurgical therapy as a primary modality of treatment and margin positivity affected the survival (Fig. 4).

Fig. 4
figure 4

Section shows sheets and singly dispersed malignant spindle cells having hyperchromatic nuclei, moderate cytoplasm and osteoid production

Full size image