Abstract
Objective
The objective of the study was to discuss the optimal management and treatment outcomes for patients with head and neck osteosarcomas.
Study Design
Review article.
Methods
Review of the pertinent literature.
Results
Osteosarcomas account for approximately 1% or less of all head and neck cancers. The vast majority occur in the mandible and maxilla. The median age is in the fourth decade, with a wide range. They are more likely to recur locally after treatment and distant metastases are observed less often than with the more common osteosarcomas arising in the long bones. The optimal treatment is complete resection. The role of adjuvant chemotherapy is ill-defined. The vast majority of recurrences are observed within 5 years. The 5-year disease-specific and overall survival rates are approximately 60% to 70%.
Conclusions
Osteosarcoma of the head and neck is a rare entity that occurs primarily in the mandible and maxilla. The optimal treatment is surgery. Adjuvant radiotherapy should be considered for those with close or positive margins. The role of adjuvant chemotherapy is ill-defined. The likelihood of cure is approximately 60% to 70%.
1
Introduction
Head and neck osteosarcoma is a rare entity that accounts for 10% or less of all osteosarcomas and less than 1% of all head and neck cancers . The incidence is approximately 2 to 3 per 1 million persons per year . The median age is in the fourth decade, with a wide range . Kassir et al reported on 173 patients included in a meta-analysis of nonrandomized studies published between 1980 and 1994; the median age was 36 years (range, 5–78 years). The male to female ratio is approximately 1:1 . Guadagnolo and coworkers reported on 119 patients treated at the MD Anderson Cancer Center (Houston, TX) between 1960 and 2007; 62 patients (52%) were male, and the remainder were female. The vast majority of head and neck osteosarcomas arise in either the mandible or maxilla . Guadagnolo and colleagues observed the following site distribution: mandible, 54 patients (45%); maxilla, 48 patients (40%); calvarium, 6 patients (5%); paranasal sinuses, 3 patients (2%); hard palate, 2 patients (2%); mastoid, 2 patients (2%); skull base, 1 patient (1%); zygoma, 1 patient (1%); infratemporal fossa, 1 patient (1%); and cervical soft tissues, 1 patient (1%) . A small subset of patients present with a history of prior radiotherapy (RT), and it is likely that the osteosarcoma is radiation induced in these individuals . There is a genetic link between retinoblastoma and osteosarcoma on the 13q 14 chromosome; and although retinoblastoma patients have an increased risk of developing a posttreatment sarcoma without RT, the addition of RT increases the risk . Huber et al reported on 4 patients who had a median latency of 9 years (range, 3–15 years).
Presenting symptoms depend on the location of the tumor. Patel et al reported on 44 patients treated at the Memorial Sloan-Kettering Cancer Center (New York, NY) between 1981 and 1998: 37 patients (84%) presented with a mass and 12 patients (27%) presented with a paresis of the infraorbital (V2) nerve. The median duration of symptoms was 4 months (range, 1–46 months) . Tumor size at diagnosis is variable. Guadagnolo et al reported a median size of 5.5 cm (range, 1.2–15 cm).
Histologic subtypes include chondroblastic, osteoblastic, fibroblastic, and telangiectatic . The majority of head and neck osteosarcomas are high grade . Grade was reported in 60 of 119 patients treated at MD Anderson Cancer Center: low, 13 patients (22%); intermediate, 9 patients (15%); and high, 38 patients (63%) . In contrast to classic central osteosarcomas, a small subset of patients may have juxtacortical intermediate-grade periosteal and parosteal variants that may have a better prognosis after surgery alone.
2
Materials and methods
Following a thorough head and neck examination, tissue diagnosis is obtained by fine needle aspiration (FNA), core needle biopsy, or open biopsy . Fleshman et al reported on 98 patients with high-grade sarcomas who underwent 107 FNAs at Ohio State University (Columbus, OH) between 2001 and 2007. FNAs were obtained from the primary tumor (71%), recurrent tumors (23%), or metastases (7%). Ninety-nine (93%) of 107 FNAs were followed by a core needle biopsy, open biopsy, or excision. The overall accuracy of FNA was approximately 91%.
Plain radiographs and computed tomography are used to define the extent of the lesion in bone . Magnetic resonance imaging is used to define the intramedullary and extramedullary extent of disease . Chest computed tomography is used to detect lung metastases. Fluorodeoxyglucose positron emission tomography is useful to determine whether suspicious lung lesions are metastatic tumors or benign .
Patients are staged according to the guidelines of the American Joint Commission on Cancer (AJCC) staging system ( Table 1 ) .
| Primary tumor (T) | ||||
| TX | Primary tumor cannot be assessed | |||
| T0 | No evidence of primary tumor | |||
| T1 | Tumor ≤8 cm in greatest dimension | |||
| T2 | Tumor >8 cm in greatest dimension | |||
| T3 | Discontinuous tumors in the primary bone site | |||
| Regional lymph nodes (N) | ||||
| NX ⁎ | Regional lymph nodes cannot be assessed | |||
| N0 | No regional lymph node metastasis | |||
| N1 | Regional lymph node metastasis | |||
| Distant metastasis (M) | ||||
| M0 | No distant metastasis | |||
| M1 | Distant metastasis | |||
| M1a | Lung | |||
| M1b | Other distant sites | |||
| Anatomical stage/prognostic groups | ||||
| Stage IA | T1 | N0 | M0 | G1,2 low grade, GX |
| Stage IB | T2 | N0 | M0 | G1,2, low grade, GX |
| T3 | N0 | M0 | G1, 2 low grade, GX | |
| Stage IIA | T1 | N0 | M0 | G3, 4 high grade |
| Stage IIB | T2 | N0 | M0 | G3, 4 high grade |
| Stage III | T3 | N0 | M0 | G3, 4 |
| Stage IVA | Any T | N0 | M1a | Any G |
| Stage IVB | Any T | N1 | Any M | Any G |
| Any T | Any N | M1b | Any G | |
2.1
Treatment
The mainstay of treatment of head and neck osteosarcoma is surgery . Adjuvant postoperative RT is indicated for those with close or positive margins . The role of adjuvant chemotherapy is unclear .
Proton beam RT may be useful to treat osteosarcomas that involve the skull base to reduce the dose to the visual apparatus and central nervous system and thus reduce the risk of late complications.
2
Materials and methods
Following a thorough head and neck examination, tissue diagnosis is obtained by fine needle aspiration (FNA), core needle biopsy, or open biopsy . Fleshman et al reported on 98 patients with high-grade sarcomas who underwent 107 FNAs at Ohio State University (Columbus, OH) between 2001 and 2007. FNAs were obtained from the primary tumor (71%), recurrent tumors (23%), or metastases (7%). Ninety-nine (93%) of 107 FNAs were followed by a core needle biopsy, open biopsy, or excision. The overall accuracy of FNA was approximately 91%.
Plain radiographs and computed tomography are used to define the extent of the lesion in bone . Magnetic resonance imaging is used to define the intramedullary and extramedullary extent of disease . Chest computed tomography is used to detect lung metastases. Fluorodeoxyglucose positron emission tomography is useful to determine whether suspicious lung lesions are metastatic tumors or benign .
Patients are staged according to the guidelines of the American Joint Commission on Cancer (AJCC) staging system ( Table 1 ) .
