Carcinosarcomas
Carcinosarcomas, also known as malignant mixed Müllerian tumors, are a type of aggressive cancer characterized by a combination of epithelial (carcinoma) and connective tissue (sarcoma) components. These tumors can occur throughout the body, with the stromal component typically being high-grade and associated with rapid growth and spread. While carcinosarcomas are relatively rare compared to other cancers, they are notable, with an estimated 67,880 new cases expected in the U.S. in 2024. Risk factors include previous treatments such as tamoxifen, as well as exposure to radiation and certain chemicals.
Symptoms may be subtle in the early stages, often presenting as lumps or swelling, and can lead to pain or complications depending on their location. Diagnosis typically involves imaging techniques and biopsies to assess the tumor's characteristics. Treatment options usually include surgical removal, possibly followed by radiation, chemotherapy, or immunotherapy. The prognosis depends largely on the tumor's stage and invasion depth, with five-year survival rates varying significantly based on these factors. Awareness and early detection are crucial for improving outcomes for those affected by carcinosarcomas.
On this Page
Subject Terms
Carcinosarcomas
ALSO KNOWN AS: Mixed epithelial-stromal carcinomas, mixed epithelial-nonepithelial malignant tumors, collision tumors, malignant mixed mullerian tumors, sarcocarcinomas
RELATED CONDITIONS: Carcinoma, carcinoma of unknown primary origin, carcinoma in situ, carcinomatosis, carcinomatous meningitis
DEFINITION: Carcinosarcomas are malignant tumors, with are abnormal masses of tissue that can invade and destroy local tissue and spread to other regions. Carcinosarcomas are a combination of carcinoma (cancer of epithelial tissue, which is skin or tissue that lines organs) and sarcoma (cancer of connective tissue, such as bone) that occur throughout the body. The stromal, or connective tissue, component is usually high-grade and contains abnormal cells that will most likely grow and spread quickly. The mesenchymal component, the mass of tissue that develops mainly from the mesoderm, the middle layer of the trilaminar germ disc during development, is classified into two groupsthe homologous form composed of cell types that are normally found and the heterologous form composed of cell types that are not normally found.
Risk factors:Tamoxifen, a nonsteroidal triphenylethyl compound widely used as adjuvant therapy in the treatment of breast cancer, has been positively correlated with the development of uterine carcinosarcoma. Radiation and chemical exposure also have been associated with carcinosarcomas.
Etiology and the disease process: The cause of carcinosarcomas is poorly defined. For malignant mixed mullerian tumors (carcinoma of the endometrium), based on histological staining preparations, the epithelial and stromal components are presumed to originate from the same cell.
Incidence: Incidence of carcinosarcomas are small compared to other cancers, but the numbers remain notable. The American Cancer Society estimated that 67,880 new cases would emerge in 2024, and 13,250 women would die that year from the disease.
From the mid-2010s through the mid-2020s, a one percent increase has been noted in White women, and a two to three perent increase is noted in women of all other racial groups.
Symptoms: Carcinosarcomas can occur anywhere in the body. They often produce no signs and symptoms in their early stages. They may grow and create a lump or swell. A tumor may cause pain if it impinges on nerves or muscles. Depending on the location, the tumor may cause blockage, bleeding, or other pathological features.
Screening and diagnosis: Like many cancers, carcinosarcomas are staged according to their size and location, spread to lymph nodes, spread to other regions in the body, and grade. Imaging tests such as magnetic resonance imaging (MRI), computed tomography (CT), and ultrasound are frequently used for diagnosis. Biopsies are conducted to determine the malignancy and the grade, or aggressiveness, of the tumor.
Treatment and therapy: Treatment options for carcinosarcomas, like those for other cancers, depend on their size, type, location, and stage. The primary treatment is surgery to remove the uterus. Fallopian tubes and ovaries may also be excised. Other types of follow-on treatments may involve radiation, chemotherapy, and hormone therapy. A more targeted form of treatment is immunotherapy. This involves drugs that can make the body's immune system more adept at eliminating cancerous tissues. In many cases, a combination of methods may be employed.
Prognosis, prevention, and outcomes: Prognosis and outcome are determined primarily by depth of invasion and stage. Nonetheless, uterine sarcoma is curable if prevented from spreading. According to the National Cancer Institute, the five-year post-diagnosis survival rate ranges from 41 to 95 percent, depending on the type of sarcoma and the rate of spread.
Bibliography
Belkacemi, Yazid, René-Olivier Mirimanoff, and Mahmut Ozsahin. Management of Rare Adult Tumours. Paris, Springer-Verlag, 2009.
"Endometrial Cancer." American Cancer Society, 2024, www.cancer.org/cancer/types/endometrial-cancer.html. Accessed 27 June 2024.
Erickson, B. K., et al. "Black Race Independently Predicts Worse Survival in Uterine Carcinosarcoma." Gynecologic Oncology, vol. 133, no. 2, 2014, pp. 238–41, pubmed.ncbi.nlm.nih.gov/24613675. Accessed 27 June 2024.
Kanjeekal S., A.Chambers, M. F. Fung, and S. Verma. "Systemic Therapy for Advanced Uterine Sarcoma: A Systematic Review of the Literature." Gynecologic Oncology, vol. 97, no. 2, 2005, pp. 624–37.
"Key Statistics for Endometrial Cancer." American Cancer Society, 17 Jan. 2024, www.cancer.org/cancer/types/endometrial-cancer/about/key-statistics.html. Accessed 27 June 2024.
Raghavan, Derek, et al., eds. Textbook of Uncommon Cancer. 4th ed. Hoboken, Wiley, 2012.
"Treatment for Uterine Sarcoma, by Type and Stage." American Cancer Society, 20 Sept. 2022, www.cancer.org/cancer/types/uterine-sarcoma/treating/by-stage.html. Accessed 27 June 2024.
"Uterine Sarcoma." Cleveland Clinic, 31 Jan. 2022, my.clevelandclinic.org/health/diseases/16408-uterine-sarcoma. Accessed 27 June 2024.