Abstract
Purpose
Carcinosarcoma is a rare malignant tumor of mixed epithelial and mesenchymal origin. In the head and neck, carcinosarcoma most commonly affects the salivary glands. Primary sinonasal carcinosarcoma (SN-CS) is exceedingly rare.
Methods
We performed a retrospective analysis of 15 cases of SN-CS obtained from the Surveillance, Epidemiology and End Results (SEER) database from 1973 to 2010. Case-matched cohorts of non-sinonasal carcinosarcoma (NS-CS), salivary gland carcinosarcoma (SG-CS) and carcinosarcoma at all other head and neck sites (NonSNSG-CS) were used for comparison.
Results
Women made up 60.0% of the SN-CS cohort and whites 73.3%. Tumors originated in the nasal cavity in 46.7% of cases, and from the maxillary sinus in 33.3%. In 66.7% of cases, tumors were poorly differentiated (histologic grades III and IV). Surgery with radiotherapy was the primary treatment modality in 46.7% of cases. Five-year disease-specific survival (DSS) was 48.5% for SN-CS compared to 65.5% for the case-matched SG-CS cohort (p = 0.2950), whereas it was 76.9% for the case-matched NonSNSG-CS cohort (p = 0.0406).
Conclusion
SN-CS is a rare tumor. Here we present the largest known cohort of SN-CS and report on its demographic, clinicopathologic and survival features. Our results suggest that patients with SN-CS have DSS comparable to the case-matched cohort of SG-CS patients. However, SN-CS patients have significantly poor survival outcomes compared to the case-matched cohort of NonSNSG-CS patients.
1
Introduction
Carcinosarcoma is a very rare malignant neoplasm involving dual histologic components: epithelial and mesenchymal. The epithelial component consists of a focal carcinoma, which can have any type of differentiation but typically takes the form of high grade or undifferentiated squamous cell carcinoma . The mesenchymal component comprises a sarcomatoid stroma, such as a chondrosarcoma, or osteosarcoma , but fibrosarcoma, leiomyosarcoma , or even liposarcoma is possible. Carcinosarcoma may arise in various anatomic sites, such as the salivary glands, respiratory tract, upper gastrointestinal tract, and female reproductive organs . Carcinosarcomas of the salivary glands (the most common head and neck sites) are aggressive with a tendency for local recurrence and commonly, distant metastasis to the lungs or brain, usually within a period of 30 months . Its occurrence is extremely rare in the nasal cavity and the paranasal sinuses, as indicated by the few cases reported in the literature .
Sinonasal carcinosarcoma (SN-CS), like other malignancies of the sinonasal tract, often presents with nonspecific symptoms, including facial pain, unilateral nasal obstruction and epistaxis . SN-CS tends to present in the sixth decade of life and has been reported to be more common in males (3.5:1) . The treatment of choice for SN-CS is wide local excision with adjuvant radiotherapy, although limited data exist for adequate comparison of treatment alternatives.
Due to the very rare nature of this tumor, to date, the reports of SN-CS in the literature have been limited to mostly case reports . Here we use data from the Surveillance, Epidemiology, and End Results (SEER) database from 1973 to 2010 to present the largest known cohort of SN-CS and compare survival features to case-matched cohorts of salivary gland carcinosarcoma (SG-SC), non-sinonasal carcinosarcoma (NonSN-CS), and all other head and neck carcinosarcomas (NonSNSG-CSs).
2
Materials and methods
Survival data were obtained from the SEER Program database using SEER*Stat 8.1.2 software (National Cancer Institute, Bethesda, MD); specifically, the SEER18 dataset (consisting of 18 registries, covering the years 1973–2010) was used. Institutional Review Board approval was not required, as SEER does not uncover sensitive patient information. Using the International Classification of Diseases for Oncology, 3rd edition (ICD-O-3) codes, we extracted data for the histologic subcategory of “Carcinosarcoma, NOS” (ICD-O-3 code: 8980/3). We then used anatomic site codes to identify cases of carcinosarcoma in “sinonasal,” “non-sinonasal,” “salivary gland,” and “all other head and neck” regions. The “sinonasal” category (SN-CS) included those structures comprising the sinonasal tract, namely the nasal cavity (C30.0) and paranasal sinuses (C31.0, C31.1, C31.2, C31.3, C31.8 and C31.9). The “non-sinonasal” category (NonSN-CS) included sites in the head and neck region excluding the sinonasal tract. The sites included in this category were the salivary glands (C07.9–C08.9), oral cavity (C00.0–C06.9, C09.0–C09.9), oropharynx (C10.0–C10.9), nasopharynx (C11.0–11.9), hypopharynx (C12.9, C13.0–C13.9), pharynx (C14.0, C14.2–C14.8), larynx (C32.0–C32.9), and trachea (C33.9). The “salivary gland” category (SG-CS) was comprised of all codes for the major salivary glands, including the parotid gland (C07.9), submandibular gland (C08.0), sublingual gland (C08.1), and “other salivary tissue” (miscellaneous or unspecified major salivary glands under site codes C08.8 and C08.9). Minor salivary glands were not included in this category since the ICD-O-3 provides no precise, well-defined site code for these glands. The category of “All other head and neck” carcinosarcomas (NonSNSG-CSs) included cases of carcinosarcoma in all other head and neck regions that excluded sinonasal tract and salivary gland regions.
Data were analyzed on the basis of age, gender, race, histologic grade, and treatment (including surgery, radiotherapy, or both) for SN-CS. Race was divided into white, black, and “other” (including American Indian, Alaskan Native, Asian/Pacific Islander, unspecified, or unknown). The case-matched NonSN-CS, SG-CS and NonSNSG-CS cohorts were obtained by matching patients in these categories with SN-CS patients for high histologic grade (III and IV), N0 nodal classification, and M0 metastasis classification. Overall survival rates and disease-specific survival (DSS) rates for unmatched and case-matched cohorts were estimated by Kaplan–Meier analysis using JMP Statistical Discovery 10 (SAS Institute, Cary, NC) for SN-CS, NonSN-CS, SG-CS and NonSNSG-CS. The survival curves were compared using the log-rank test. Microsoft Office Excel 2007 (Microsoft Corporation, Redmond, WA) was used for additional data processing. A probability value (p-value) of < 0.05 was considered statistically significant for all tests.
2
Materials and methods
Survival data were obtained from the SEER Program database using SEER*Stat 8.1.2 software (National Cancer Institute, Bethesda, MD); specifically, the SEER18 dataset (consisting of 18 registries, covering the years 1973–2010) was used. Institutional Review Board approval was not required, as SEER does not uncover sensitive patient information. Using the International Classification of Diseases for Oncology, 3rd edition (ICD-O-3) codes, we extracted data for the histologic subcategory of “Carcinosarcoma, NOS” (ICD-O-3 code: 8980/3). We then used anatomic site codes to identify cases of carcinosarcoma in “sinonasal,” “non-sinonasal,” “salivary gland,” and “all other head and neck” regions. The “sinonasal” category (SN-CS) included those structures comprising the sinonasal tract, namely the nasal cavity (C30.0) and paranasal sinuses (C31.0, C31.1, C31.2, C31.3, C31.8 and C31.9). The “non-sinonasal” category (NonSN-CS) included sites in the head and neck region excluding the sinonasal tract. The sites included in this category were the salivary glands (C07.9–C08.9), oral cavity (C00.0–C06.9, C09.0–C09.9), oropharynx (C10.0–C10.9), nasopharynx (C11.0–11.9), hypopharynx (C12.9, C13.0–C13.9), pharynx (C14.0, C14.2–C14.8), larynx (C32.0–C32.9), and trachea (C33.9). The “salivary gland” category (SG-CS) was comprised of all codes for the major salivary glands, including the parotid gland (C07.9), submandibular gland (C08.0), sublingual gland (C08.1), and “other salivary tissue” (miscellaneous or unspecified major salivary glands under site codes C08.8 and C08.9). Minor salivary glands were not included in this category since the ICD-O-3 provides no precise, well-defined site code for these glands. The category of “All other head and neck” carcinosarcomas (NonSNSG-CSs) included cases of carcinosarcoma in all other head and neck regions that excluded sinonasal tract and salivary gland regions.
Data were analyzed on the basis of age, gender, race, histologic grade, and treatment (including surgery, radiotherapy, or both) for SN-CS. Race was divided into white, black, and “other” (including American Indian, Alaskan Native, Asian/Pacific Islander, unspecified, or unknown). The case-matched NonSN-CS, SG-CS and NonSNSG-CS cohorts were obtained by matching patients in these categories with SN-CS patients for high histologic grade (III and IV), N0 nodal classification, and M0 metastasis classification. Overall survival rates and disease-specific survival (DSS) rates for unmatched and case-matched cohorts were estimated by Kaplan–Meier analysis using JMP Statistical Discovery 10 (SAS Institute, Cary, NC) for SN-CS, NonSN-CS, SG-CS and NonSNSG-CS. The survival curves were compared using the log-rank test. Microsoft Office Excel 2007 (Microsoft Corporation, Redmond, WA) was used for additional data processing. A probability value (p-value) of < 0.05 was considered statistically significant for all tests.
3
Results
3.1
Analysis of demographic and clinicopathologic factors
We queried the SEER 18 data set (1973–2010) for carcinosarcoma cases at various subsites including the sinonasal tract, salivary glands and other head and neck regions. Our dataset included a total of 135 cases of head and neck carcinosarcomas. Fifteen cases involved the sinonasal tract, while 39 cases involved the salivary glands, and the remaining 81 cases involved other head and neck regions. The demographic and clinicopathologic characteristics for SN-CS cases are reported in Table 1 . The mean age at diagnosis was 60.3 years (SD, ± 21.3 years). Females represented 60.0% of the SN-CS cases and the majority of the patients were white (73.3%). The most common subsite within the sinonasal tract was the nasal cavity (46.7%), followed by the maxillary sinus (33.3%). 66.7% of the SN-CS cases were of high histologic grade (grades III and IV). The grade was unknown for the remaining 33.3% of the cases. Of the cases for which histologic grade was available in the SEER database, none were of low grade (grades I and II). 66.7% of the patients had a T3 or T4 disease classification while 20.0% of the patients had T1 or T2 disease classification. T classification was not available for 2 patients. 60.0% of the patients had no nodal metastasis (N0), while the N classification was not available for the remaining 40.0% of the patients. The most common treatment modality was surgery with adjuvant radiotherapy (46.7%), followed by radiotherapy alone (26.7%) and surgery alone (20.0%).
| n | % | |
|---|---|---|
| Total | 15 | 100 |
| Gender | ||
| Male | 6 | 40.0 |
| Female | 9 | 60.0 |
| Race | ||
| White | 11 | 73.3 |
| Black | 2 | 13.3 |
| Other | 2 | 13.3 |
| Location | ||
| Nasal cavity | 7 | 46.7 |
| Maxillary sinus | 5 | 33.3 |
| Ethmoid sinus | 1 | 6.7 |
| Frontal sinus | 1 | 6.7 |
| Sphenoid sinus | 1 | 6.7 |
| Histologic grade | ||
| Low grade (grades I and II) | 0 | 0.0 |
| High grade (grades III and IV) | 10 | 66.7 |
| Unknown | 5 | 33.3 |
| T classification | ||
| T1/2 | 3 | 20.0 |
| T3/4 | 10 | 66.7 |
| TX | 2 | 13.3 |
| N classification | ||
| N0 | 9 | 60.0 |
| N + | 0 | 0.0 |
| NX | 6 | 40.0 |
| M classification | ||
| M0 | 14 | 93.3 |
| M1 | 0 | 0.0 |
| MX | 1 | 6.7 |
| Treatment | ||
| Surgery alone | 3 | 20.0 |
| Surgery and radiotherapy | 7 | 46.7 |
| Radiotherapy alone | 4 | 26.7 |
| No therapy | 1 | 6.7 |
3.2
Survival analysis
Kaplan–Meier estimates of overall survival and DSS in SN-CS patients (N = 15) were calculated. We also calculated the same for NonSN-CS (N = 120), SG-CS (N = 39), and NonSNSG-CS (N = 81) patients. The results for the 5-year survival analysis are shown in Table 2 . The 5-year overall survival rate for SN-CS patients (45.0%) was found to be similar to that for other categories: NonSN-CS patients (47.3%), SG-CS patients (47.8%), and NonSNSG-CS patients (47.2%). The 5-year DSS rates for SN-CS cohort and case-matched SG-CS cohort were 48.5% and 65.5%, respectively. Although DSS for SN-CS was lower than that for SG-CS, this difference was not statistically significant (p = 0.4471). The 5-year DSS for case-matched NonSN-CS (73.6%, p = 0.0784) and NonSNSG-CS (76.9%, p = 0.0406) was found to be higher than that for SN-CS. The p-value approached, but did not reach, statistical significance when comparing the DSS rates between SN-CS and NonSN-CS; whereas it was statistically significant (p < 0.05) when comparing the same rates between SN-CS and NonSNSG-CS. Fig. 1 shows 5-year DSS plots for SN-CS and case-matched cohorts in other categories. The results for 10-year survival analysis are depicted in Table 3 . The differences in survival rates in 10-year analysis were found to be similar to those found in 5-year analysis. Fig. 2 shows the 10-year DSS plots for SN-CS and case-matched cohorts in other categories.
| SN-CS a | NonSN-CS | SG-CS | NonSNSG-CS | ||||
|---|---|---|---|---|---|---|---|
| Survival | Survival | p -value | Survival | p -value | Survival | p -value | |
| Unmatched cohorts | |||||||
| Overall Survival | 45.0% | 47.3% | 0.7537 | 47.8% | 0.6756 | 47.2% | 0.8062 |
| DSS | 48.5% | 61.4% | 0.4151 | 59.2% | 0.5069 | 62.6% | 0.4133 |
| Matched cohorts b | |||||||
| Overall Survival | 45.0% | 53.5% | 0.4175 | 51.3% | 0.6457 | 54.6% | 0.3705 |
| DSS | 48.5% | 73.6% | 0.0784 ⁎ | 65.5% | 0.4471 | 76.9% | 0.0406 |
Stay updated, free articles. Join our Telegram channel
Full access? Get Clinical Tree
