Salivary Carcinosarcoma: An Extremely Rare and Highly Aggressive Malignancy

Abstract

Objectives/Hypothesis: Carcinosarcomas represent a rare entity of mixed malignant tumors of the salivary gland with limited evidence regarding management strategies. We aim to demonstrate the incidence, prognostic factors, and conduct a survival analysis for this aggressive malignancy.Study Design: Retrospective database review. Methods: The Surveillance, Epidemiology, and End Results database was queried for all cases of major salivary carcinosarcoma and its incidence from 1973 to 2015 to identify 66 patients.Results: The incidence of this tumor was 0.02 cases per 1 million, with a rising number of absolute cases in the past 2 decades. The parotid gland was the most common site (78.8%) of involvement. The 5-year overall survival was 37% and 5-year disease-specific survival was 62%. Surgery was most common management strategy (95.5%), with total parotidectomy and facial nerve sacrifice procedures for those with parotid disease. Radiotherapy was commonly performed (75.8%) and chemotherapy use was rare (18.2%). Patients with distant metastasis had a greater than threefold increase in mortality, and those with total parotidectomy surgery had decreased mortality.Conclusions: Carcinosarcomas of major salivary glands are extremely rare and highly aggressive tumors. We recommend prompt surgical management and postoperative radiation for this tumor with a poor prognosis.

Patterns of Care and Outcomes of Carcinosarcoma of the Major Salivary Glands

“…This is similar to previous reports of salivary gland carcinosarcoma, which suggest that 60% of tumors are locoregionally advanced, 38% have nodal involvement, and 17% have distant metastases. 12 Additionally, most tumors are interpreted as high grade, which is consistent with prior reports. 18 However, because the absolute number of cases is low, definitive conclusions cannot be drawn.…”

“…5 The only population-level article in the literature that describes the tumor's incidence, prognostic factors, and survival was published by Gupta et al in 2020, which used the SEER database and was limited by its small sample size (66 patients). 12 To the best of our knowledge, the present publication investigates a sample larger than all other previous reports to better define the tumor characteristics and patterns of care for carcinosarcoma. The demographic characteristics of patients in our cohort are consistent with those reported in the literature.…”

Successful Resection of Retrobulbar Carcinosarcoma without Recurrence: A Case Report

“…The primary treatment for ovarian carcinosarcoma is cytoreductive surgery, followed by platinum-based chemotherapy, usually carboplatin-paclitaxel [3]. Cases of carcinosarcoma in the paranasal sinus [10], salivary gland [30], esophagus [31], and urinary bladder [4] treated with surgical resection combined with radiotherapy or chemotherapy have also been reported, although there is still a lack of consensus on the mode of therapy owing to the limited number of cases. Orbital carcinosarcoma is a rare disease entity.…”

Carcinosarcoma EX Pleomorphic Adenoma of Parotid: An Unusual Entity, A Report of Two Cases with Brief Review of Literature

“…One of the oldest case series of CSXPA was presented by James Stephen, et al in 1986 comprising 12 cases, the majority of which were ductal carcinoma combined with osteosarcoma. In recent years, a population-level based demographic study on salivary gland carcinosarcoma was conducted by Avigeet Gupta, et al in 2019, however, the independent histomorphological features were not registered [26]. A brief review of CSXPA cases in the past two decades is presented Table 1.…”