Adenosarcoma (also Müllerian adenosarcoma) is a rare

malignant Malignancy () is the tendency of a medical condition to become progressively worse; the term is most familiar as a characterization of cancer. A ''malignant'' tumor contrasts with a non-cancerous benign tumor, ''benign'' tumor in that a malig ...

tumor that occurs in women of all age groups, but most commonly post-

menopause Menopause, also known as the climacteric, is the time when Menstruation, menstrual periods permanently stop, marking the end of the Human reproduction, reproductive stage for the female human. It typically occurs between the ages of 45 and 5 ...

. Adenosarcoma arises from mesenchymal tissue and has a mixture of the tumoral components of an

adenoma An adenoma is a benign tumor of epithelium, epithelial tissue with glandular origin, glandular characteristics, or both. Adenomas can grow from many glandular organ (anatomy), organs, including the adrenal glands, pituitary gland, thyroid, prosta ...

, a tumor of

epithelial Epithelium or epithelial tissue is a thin, continuous, protective layer of cells with little extracellular matrix. An example is the epidermis, the outermost layer of the skin. Epithelial ( mesothelial) tissues line the outer surfaces of man ...

origin, and a

sarcoma A sarcoma is a rare type of cancer that arises from cells of mesenchymal origin. Originating from mesenchymal cells means that sarcomas are cancers of connective tissues such as bone, cartilage, muscle, fat, or vascular tissues. Sarcom ...

, a tumor originating from

connective tissue Connective tissue is one of the four primary types of animal tissue, a group of cells that are similar in structure, along with epithelial tissue, muscle tissue, and nervous tissue. It develops mostly from the mesenchyme, derived from the mesod ...

.NCI Dictionary of Cancer Terms: Adenosarcoma." National Cancer Institute, National Institutes of Health, www.cancer.gov/publications/dictionaries/cancer-terms/def/adenosarcoma. Carroll, A., Ramirez, P. T., Westin, S. N., Soliman, P. T., Munsell, M. F., Nick, A. M., ... & Fleming, N. D. (2014). Uterine adenosarcoma: an analysis on management, outcomes, and risk factors for recurrence. Gynecologic oncology, 135(3), 455-461. The adenoma, or epithelial component of the tumor, is

benign Malignancy () is the tendency of a medical condition to become progressively worse; the term is most familiar as a characterization of cancer. A ''malignant'' tumor contrasts with a non-cancerous benign tumor, ''benign'' tumor in that a malig ...

, while the sarcomatous stroma is malignant.Podduturi, V., & Pinto, K. R. (2016, January). Mullerian adenosarcoma of the cervix with heterologous elements and sarcomatous overgrowth. In Baylor University Medical Center Proceedings (Vol. 29, No. 1, pp. 65-67). Taylor & Francis. The most common site of adenosarcoma formation is the

uterus The uterus (from Latin ''uterus'', : uteri or uteruses) or womb () is the hollow organ, organ in the reproductive system of most female mammals, including humans, that accommodates the embryonic development, embryonic and prenatal development, f ...

, but it can also occur in the

cervix The cervix (: cervices) or cervix uteri is a dynamic fibromuscular sexual organ of the female reproductive system that connects the vagina with the uterine cavity. The human female cervix has been documented anatomically since at least the time ...

and

ovaries The ovary () is a gonad in the female reproductive system that produces ova; when released, an ovum travels through the fallopian tube/oviduct into the uterus. There is an ovary on the left and the right side of the body. The ovaries are endocr ...

. It more rarely arises in the

vagina In mammals and other animals, the vagina (: vaginas or vaginae) is the elastic, muscular sex organ, reproductive organ of the female genital tract. In humans, it extends from the vulval vestibule to the cervix (neck of the uterus). The #Vag ...

and

fallopian tubes The fallopian tubes, also known as uterine tubes, oviducts or salpinges (: salpinx), are paired tubular sex organs in the human female body that stretch from the ovaries to the uterus. The fallopian tubes are part of the female reproductive sy ...

as well as primary pelvic or peritoneal sites, such as the omentum, especially in those with a history of

endometriosis Endometriosis is a disease in which Tissue (biology), tissue similar to the endometrium, the lining of the uterus, grows in other places in the body, outside the uterus. It occurs in women and a limited number of other female mammals. Endomet ...

.Mullerian Adenosarcoma of the Female Genital Tract. McCluggage, W. Glenn MD eviewAdvances in Anatomic Pathology. 17(2):122-129, March 2010. The rare cases of adenosarcoma outside the female genital tract usually occur in the

liver The liver is a major metabolic organ (anatomy), organ exclusively found in vertebrates, which performs many essential biological Function (biology), functions such as detoxification of the organism, and the Protein biosynthesis, synthesis of var ...

,Volkov VP, Lazdin OA, Sadikov ID. Adenosarcoma of the liver in patient with liver cirrhosis. Klin Med (Mosk). 1979;57(3):105–7.Nathenson, M. J., Ravi, V., Fleming, N., Wang, W. L., & Conley, A. (2016). Uterine adenosarcoma: a review. Current oncology reports, 18(11), 68.

bladder The bladder () is a hollow organ in humans and other vertebrates that stores urine from the kidneys. In placental mammals, urine enters the bladder via the ureters and exits via the urethra during urination. In humans, the bladder is a distens ...

kidney In humans, the kidneys are two reddish-brown bean-shaped blood-filtering organ (anatomy), organs that are a multilobar, multipapillary form of mammalian kidneys, usually without signs of external lobulation. They are located on the left and rig ...

,Sameshima N, Marutsuka K, Tsukino H, Kamoto T, Kono S, Asada Y. So-called 'adenosarcoma' of the kidney a novel adult renal tumor with a cystic appearance. Pathol Int. 2011;61(5):313–8. as well as the

intestine The gastrointestinal tract (GI tract, digestive tract, alimentary canal) is the tract or passageway of the digestive system that leads from the mouth to the anus. The tract is the largest of the body's systems, after the cardiovascular system. T ...

and are typically associated with endometriosis.Kondi-Pafiti A, Spanidou-Carvouni H, Papadias K, Hatzistamou-Kiari I, Kontogianni K, Liapis A, et al. Malignant neoplasms arising in endometriosis: clinicopathological study of 14 cases. Clin Exp Obstet Gynecol. 2004;31(4):302–4. Müllerian adenosarcoma with sarcomatous overgrowth is a very aggressive form of adenosarcoma that is characterized by post-operative recurrence and

metastases Metastasis is a pathogenic agent's spreading from an initial or primary site to a different or secondary site within the host's body; the term is typically used when referring to metastasis by a cancerous tumor. The newly pathological sites, ...

even when diagnosed at an early stage.Patrelli, T. S., Gizzo, S., Di Gangi, S., Guidi, G., Rondinelli, M., & Nardelli, G. B. (2011). Cervical Mullerian adenosarcoma with heterologous sarcomatous overgrowth: a fourth case and review of literature. BMC cancer, 11(1), 236. Sarcomatous overgrowth is diagnosed when the sarcomatous portion of the adenosarcoma makes up more than 25% of the tumor. Adenosarcomas do not typically have distant metastases, but they have a propensity for local recurrence.Clement PB, Scully RE. Mullerian adenosarcoma of the uterus: a clinicopathologic analysis of 100 cases with a review of the literature. Hum Pathol. 1990;21(4):363–381Verschraegen CF, Vasuratna A, Edwards C, Freedman R, Kudelka AP, Tornos C, Kavanagh JJ. Clinicopathologic analysis of mullerian adenosarcoma: the M.D. Anderson Cancer Center experience. Oncol Rep. 1998;5(4):939–944

Uterine Adenosarcoma

Uterine adenosarcoma Uterine adenosarcoma is an uncommon form of cancer that arises from mesenchymal tissue of the uterus and has a benign glandular component. Signs and symptoms The most common presentation is vaginal bleeding. Other presentations include pelvic ma ...

are a subtype of uterine sarcomas. Uterine sarcomas account for 3 to 9% of uterine cancers, and 5.5 to 9% of uterine sarcomas are adenosarcomas. The most common presenting symptom is abnormal vaginal bleeding. Other symptoms include pelvic pain, abdominal mass, or vaginal discharge.McCluggage, Glenn W. "Uterine Adenosarcoma." Uterine Adenosarcoma, Department of Pathology, Royal Group of Hospitals Trust, Belfast, Northern Ireland, 2009, uscapknowledgehub.org/site~/98th/pdf/companion12h05.pdf. Uterine adenosarcoma commonly arise from the endometrium. Uterine adenosarcomas have the highest incidence in perimenopausal and

postmenopausal Menopause, also known as the climacteric, is the time when menstrual periods permanently stop, marking the end of the reproductive stage for the female human. It typically occurs between the ages of 45 and 55, although the exact timing can ...

women with a mean age of 50 years, but some incidence among children. Survival is better compared to other types of uterine sarcomas. The prognosis of uterine adenosarcoma depends on the stage and if sarcomatous overgrowth is present.Andre Pinto and Brooke Howitt (2016) Uterine Adenosarcoma. Archives of Pathology & Laboratory Medicine: March 2016, Vol. 140, No. 3, pp. 286-290.

Risk Factors

No definitive causes of adenosarcoma have been identified. Potential risk factors include a medical history of endometriosis and use of estrogen modulating agents such as

tamoxifen Tamoxifen, sold under the brand name Nolvadex among others, is a selective estrogen receptor modulator used to prevent breast cancer in women and men. It is also being studied for other types of cancer. It has been used for Albright syndrome ...

. Other potential risk factors include previous pelvic irradiation and prolonged estrogen exposureStern RC, Dash R, Bentley RC, Snyder MJ, Haney AF, Robboy SJ. Malignancy in endometriosis: frequency and comparison of ovarian and extraovarian types. Int J Gynecol Pathol. 2001;20(2):133–9.

Treatment

The standard care of treatment is total abdominal

hysterectomy Hysterectomy is the surgical removal of the uterus and cervix. Supracervical hysterectomy refers to removal of the uterus while the cervix is spared. These procedures may also involve removal of the ovaries (oophorectomy), fallopian tubes ( salpi ...

with bilateral salpingo-oophorectomy. Lymphadenectomy is usually not performed as the incidence of lymph node metastasis is rare. There is no standardized

chemotherapy Chemotherapy (often abbreviated chemo, sometimes CTX and CTx) is the type of cancer treatment that uses one or more anti-cancer drugs (list of chemotherapeutic agents, chemotherapeutic agents or alkylating agents) in a standard chemotherapy re ...

hormone therapy Hormone therapy or hormonal therapy is the use of hormones in medical treatment. Treatment with hormone antagonists may also be referred to as hormonal therapy or antihormone therapy. The most general classes of hormone therapy are hormonal therap ...

, or

radiation therapy Radiation therapy or radiotherapy (RT, RTx, or XRT) is a therapy, treatment using ionizing radiation, generally provided as part of treatment of cancer, cancer therapy to either kill or control the growth of malignancy, malignant cell (biology), ...

.Özen Ö. Müllerian adenosarcoma. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/uterusadenosarcoma.html. Accessed March 20th, 2019Nathenson, M. J., Conley, A. P., Lin, H., Fleming, N., & Ravi, V. (2017). Treatment of recurrent or metastatic uterine adenosarcoma. Sarcoma, 2017. Because of the rarity of adenosarcoma, there is limited data to guide treatment decisions, particularly in regard to recurrent or metastatic tumors. Chemotherapy may be considered in patients with recurrence or tumors unable to be completely removed through surgery. It has been suggested that uterine adenosarcomas can respond to doxorubicin/ifosfamide and gemcitabine/docetaxel chemotherapy.M. J. Nathenson, V. Ravi, N. Fleming, W.-L. Wang, and A. Conley, "Uterine adenosarcoma: a review," Current Oncology Reports, vol. 18, no. 11, p. 68, 2016. The use of hormone therapy in recurrent or metastatic disease is limited to case reports.

Recurrence and Survival

Survival is influenced by the presence of myometrial invasion, sarcomatous overgrowth, lymphovascular invasion, necrosis, and the presence of heterologous elements, which are features in the tumor not native to the tissue of origin such as rhabdomyoblastic differentiation Post-operative recurrence is common in uterine adenosarcomas. Recurrence usually occurs in the vagina, pelvis, and abdomen, and is seen in up to 30% of cases resulting in a poor prognosis. The presence and depth of the sarcoma's myometrial invasion determines early staging diagnosis. The FIGO ( International Federation of Gynecology and Obstetrics) staging is IA: no myometrial invasion, IB: inner myometrial half, IC: outer myometrial half. If confined to the endometrium with no myometrial invasion (IA), the prognosis is good with 7-13% recurrence for noninvasive tumors. FIGO stage II or greater is considered advanced with overall survival of approximately 60% with myometrial invasion, but less than 50% if metastases are present. High grade adenosarcomas tend to have extrauterine spread and rapid recurrence. Adenosarcoma with myometrial invasion recurred in 36-46% of cases. Patients with sarcomatous overgrowth showed significantly increased risk of recurrence, around 70-77 %, a risk of metastases around 40%, and a decreased 5-year overall survival, 50 to 60%. This is comparable to other high grade uterine sarcomas.

Ovarian Adenosarcoma

Ovarian adenosarcoma is a very rare tumor effecting the ovaries. 97.5% of ovarian adenosarcomas are unilateral,Eichhorn JH, Young RH, Clement PB, Scully RE. Mesodermal (mullerian) adenosarcoma of the ovary: a clinicopathologic analysis of 40 cases and a review of the literature. Am J Surg Pathol. 2002;26(10):1243–58. affecting only one ovary. It mainly affects women aged 30–84, with a mean age of 54. Symptoms of ovarian adenosarcoma include abdominal or pelvic pain and abdominal swelling. Tumor may present as adnexal mass.Gupta N Müllerian adenosarcoma. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/ovarytumormullerianadeno.html. Accessed March 25th, 2019.

Risk Factors

Most of the cases reported have associated endometriosis or an adenosarcoma arising from an endometriotic area, but the direct relation between this tumor and endometriosis has not been made clear in the literature.Shakuntala, P. N., Umadevi, K., Usha, A., Abhilasha, N., & Bafna, U. D. (2012). Primary ovarian adenosarcoma with elevated Ca-125 levels and normal ascitic fluid cytology: a case report and review of literature. ecancermedicalscience, 6.

Treatment

Typically, ovarian adenosarcomas are surgically removed via salphingopherectomy or panhysterectomy. 67% of patients had tumor rupture at or before excision. There is no standardized chemotherapy, hormone therapy, or radiation therapy due to limited data.

Recurrence and Survival

Ovarian adenosarcomas have a worse prognosis than uterine adenosarcomas, presumably because of the greater ease of peritoneal spread. Many of these ovarian tumors have caused problems in differential diagnosis. Advanced stage ovarian adenosarcoma is characterized by extraovarian spreading, sarcomatous overgrowth, and tumor rupture. The presence of sarcomatous overgrowth is associated with increased risk of recurrence or extraovarian spreading. Recurrence poses more of a threat than metastases, which appear to be less prevalent. 5 year survival is 64%, 10 year survival is 46%.

Cervical Adenosarcoma

Cervical adenosarcoma is an extremely rare tumor that occurs most often in women of reproductive age. Typically, adenosarcoma found in the cervix is a result of extrauterine spreading of the tumor, though adenosarcoma can arise in the cervix. Symptoms of cervical adenosarcoma, like uterine adenosarcoma, are characterized by abnormal bleeding.Jones, M. W., & Lefkowitz, M. (1995). Adenosarcoma of the uterine cervix: a clinicopathological study of 12 cases. International journal of gynecological pathology: official journal of the International Society of Gynecological Pathologists, 14(3), 223-229. Treatment mainly consists of total abdominal

, occasionally with additional radiotherapy and chemotherapy. Prognosis of cervical adenosarcoma is usually favorable, with metastasis and recurrence of the tumor being less common. As with uterine adenosarcoma, the depth of myometrial invasion determines the prognosis, with deeper invasion being associated with metastases and tumor recurrence.

References

{{Reflist Types of cancer

Uterine Adenosarcoma

Risk Factors

Treatment

Recurrence and Survival

Ovarian Adenosarcoma

Risk Factors

Treatment

Recurrence and Survival

Cervical Adenosarcoma

See also

References