Meta-Analysis of 75 Cases of Sinonasal Teratocarcinosarcoma: A Rare Neoplasm of the Anterior Skull Base

Object: Sinonasal teratocarcinosarcoma (SNTCS) is a rare and highly malignant neoplasm that often involves the anterior skull base. Although the optimal treatment of these tumors is not well defined, the most common treatment method has included surgical resection and adjuvant radiotherapy. In this study, the authors perform a meta-analysis of SNTCS cases reported in the literature.

Methods: A MEDLINE/PubMed search revealed 74 cases of SNTCS in the English literature. The authors report an additional case in the study, making a total of 75 cases. Each case was analyzed for patient demographics, clinical presentation, tumor location, diagnosis, treatment, and outcome.

Results: The average age of the patients was 54.3 years, with a strong male predilection (6.5:1). The most common presenting symptoms were nasal obstruction (73.3%) and epistaxis (61.3%). Tumors presented mostly in the nasal cavity (64%) and ethmoid sinus (53.3%), followed by the maxillary sinus (22.7%). Eleven patients (14.7%) presented with intracranial extension, including our reported case. The most common treatment method was surgery with adjuvant radiation therapy (56%). Other treatments were surgery alone (14.7%), surgery with radiation and chemotherapy (9.3%), radiation alone (6.7%), surgery and chemotherapy (5.3%), and radiation and chemotherapy (2.7%). Of the 42 patients treated with surgery and radiation therapy, 18 (42.9%) showed no evidence of disease at the latest follow-up, and 10 (23.8%) patients showed tumor recurrence. Of the11 patients treated with surgery alone, 3 (27.3%) showed no evidence of disease at latest follow-up, while 5 (45.5%) experienced recurrence or metastasis. Seven patients were treated with a combination of surgery, radiation, and chemotherapy. Four of these patients (57.1%) showed no evidence of disease at latest follow-up, while three (42.9%) experienced tumor recurrence. Of the five patients who were treated with radiation alone, four (80%) developed recurrence. Treatment with surgery and chemotherapy was reported in four patients. Two remained disease free and two were alive with disease or with recurrence. Combination radiotherapy and chemotherapy was used in two patients. One showed no evidence of disease, the other developed metastases. Overall, surgery was used in 85.3% of cases, radiotherapy in 74.7%, and chemotherapy in 17.3%. At the most recent available reported follow-up (average, 40 months; range, 2 to 372 months), 40% were alive without disease, 32% were alive with disease (recurrence or metastasis), and 28% were dead.

Conclusions: SNTCS is a rare and aggressive malignant skull base tumor. Although a variety of treatment paradigms have been reported in the literature, radical surgical resection followed by radiation therapy appears to be the best treatment option. The role of chemotherapy is not well defined due to the limited number of cases.