BACKGROUND: Although a rare complication of ionizing radiation, radiation‐induced osteosarcoma is now more frequently recognized as radiation therapy has become common and cancer survival has increased. To date, publications on radiation‐induced osteosarcoma of the cranium are limited to a few small series and case reports.METHODS: Data from 175 patients with a history of sarcoma of the head at The University of Texas M. D. Anderson Cancer Center from 1975 to 2007 were reviewed to identify patients with radiation‐induced osteosarcoma. The diagnostic criteria were: 1) osteosarcoma arose within the previously irradiated field; 2) new sarcoma was histologically distinct from the original neoplasm; 3) no evidence of new sarcoma at the time of radiation; and 4) distinct latency period could be recognized. Frequencies and descriptive statistics were obtained for the various characteristics under study.RESULTS: The authors identified 16 patients with radiation‐induced osteosarcoma of the cranium at their institution. The average age at diagnosis was 35 years. The median latency period was 12.5 years. Nine patients had skull base tumors, and 7 had calvarial tumors. Of the 14 patients treated surgically, 86% developed local recurrence. The median survival time was 29 months, and the 5‐year survival rate was 29.6%.CONCLUSIONS: The authors report the largest series of cranial radiation‐induced osteosarcoma. Although radiation‐induced osteosarcoma is an uncommon but dire complication of radiotherapy, its incidence will probably increase in the future as the frequency of radiation treatment and cancer survival increase. These tumors are locally aggressive, and despite aggressive surgical and medical management, they have a high rate of local recurrence and mortality. Cancer 2011. © 2010 American Cancer Society.

Extensive clival tumors that involve both the midline and lateral skull base compartments, or those that extend inferiorly to the anterior cervical spine, are difficult to expose in a wide fashion using any of the transmaxillary, transoral, or transcervical routes. In the transmandibular, circumglossal, retropharyngeal (TCR) approach wide access of this region can be obtained, thus allowing for a more complete resection of tumor and infiltrated bone. It also provides for an improved ability to perform dural reconstruction, should it be necessary. Over the past 4 years four patients with extensive clival chordomas underwent resection via the TCR approach. Gross-total resection was achieved in two patients, a greater than 98% resection in one patient, and a greater than 95% resection in the fourth patient. The surgical technique, all approach-related complications and morbidity, and patient outcome are discussed. If an expanded exposure of the clivus is necessary, the TCR approach is a good choice as well as a useful surgical technique to have available.

Background: The impact of tumor differentiation on the behavior and response of sinonasal neuroendocrine carcinoma is unknown. Methods: We performed a retrospective review of the patients treated for neuroendocrine carcinoma (NEC) of the nasal cavity or paranasal sinuses from 1992 to 2008 at MDACC. Results: The results of our study suggest that pathologic differentiation may not be a critical factor in the clinical management of patients with NEC of the sinonasal tract. This is in contrast to laryngeal and lung NEC for which pathological differentiation has traditionally guided clinical management. Conclusion: Mutlimodality approach should be the cornerstone of treating sinonasal NEC regardless of their differentiation. Specifically, RT may provide durable local control for patients with moderately differentiated NEC if resection is not feasible or desirable, while surgical resection can benefit patients with chemo-resistant or radio-resistant disease.

OBJECT: Adenoid cystic carcinoma (ACC) is a locally aggressive tumor of salivary gland origin. Little data exist to guide treatment when this tumor extends to involve the structures of the skull base. METHODS: Fifty-one patients with a diagnosis of ACC affecting the skull base were identified from a prospective database at MD Anderson Cancer Center (from 1992 to 2010). RESULTS: Median follow-up for study patients was 6.75 years. The 5- and 10-year overall survival (OS) rates were 78% and 50%, respectively. Sixty-six percent of patients had progression of their disease. The 5- and 10-year progression-free survival (PFS) rates were 46.7% and 21.0%, respectively. Gross-total resection was achieved in 75% of patients, with 49% having microscopically negative margins at the time of first operation. On univariate analysis, resections with microscopically negative margins were associated with a significant OS advantage (20.1 ± 3.3 years) compared with resections that left residual disease, even if microscopic (10.3 ± 1.6 years, p = 0.035). In patients who underwent reoperation, the effect persisted, with improved OS in those with negative margins (21.4 ± 0.0 vs 16.7 ± 4.0 years, p = 0.06). The use of adjuvant radiotherapy was associated with an OS advantage (16.2 ± 2.5 vs 5.5 ± 2.2 years, p = 0.03) at initial diagnosis and improved PFS (7.8 ± 1.0 vs 2.1 ± 0.62 years, p = 0.005), whereas repeat irradiation provided no benefit. The use of adjuvant chemotherapy at diagnosis or at recurrence was not associated with any significant advantage. Multivariate analysis revealed margin-negative resection at initial operation and at recurrence retained OS significance, even after controlling for age, radiation therapy, and T stage. CONCLUSIONS: ACC of the skull base is best treated with a multidisciplinary approach aimed at maximal, safe resection. Adjuvant radiotherapy should be offered, whereas chemotherapy does not confer benefit.

Object. Partial resection of the orbital bones is not uncommon during the excision of anterior and anterolateral skull base tumors. Controversy exists regarding the need for and extent of reconstruction after this procedure. The authors studied this factor in a series of patients. Methods. The authors conducted a retrospective review of 56 patients in whom resection of 57 anterior or anterolateral skull base tumors and partial excision of the orbital bone were performed. Adverse ophthalmological outcomes were noted in 16 patients, in nine of whom adverse outcomes were believed to be directly related to resection of the orbital walls. Some degree of orbital reconstruction was performed during 23 of the 57 procedures. An adverse orbital outcome was strongly associated with resection of the orbital floor and resection of two thirds or more of two or more orbital walls, but not with the presence or absence of orbital reconstruction. The latter finding, however, is likely a function of selection bias. Conclusions. In most patients elaborate orbital reconstruction is not necessary after partial excision of the orbital bones. Isolated medial and lateral orbital wall defects, or combined superior and lateral orbital wall defects, especially in cases in which the periorbita is intact, probably do not require primary reconstruction. In cases of orbital floor defects, whether isolated or part of a multiple-wall resection, primary reconstruction is recommended.