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Uterine Tumor Resembling Ovarian Sex Cord Tumors (UTROSCT)

Uterine Tumor Resembling Ovarian Sex Cord Tumors (UTROSCT) are rare tumors, with only 67 cases being reported worldwide. The cause and cell of origin of the tumor is currently unknown.

What are the other Names for this Condition? (Also known as/Synonyms)

  • Uterine Neoplasm Resembling Ovarian Sex Cord Tumors
  • UTROSCT (Uterine Tumor Resembling Ovarian Sex Cord Tumors)

What are Uterine Tumor Resembling Ovarian Sex Cord Tumors? (Definition/Background Information)

  • Uterine Tumor Resembling Ovarian Sex Cord Tumors (UTROSCT) are rare tumors, with only 67 cases being reported worldwide. The cause and cell of origin of the tumor is currently unknown
  • Signs and symptoms of UTROSCT include abnormal uterine bleeding, abdominal pain, increased size of the uterus, etc. Occasionally, upon palpation (physical exam using hands) of the abdomen, an enlarged uterus may be felt
  • Radiological imaging studies, such as ultrasound/CT scan of abdomen, do not reveal specific radiological findings.  In a majority of the cases, the diagnosis is made after a pathologist examines the tissue under a microscope
  • The treatment of choice for UTROSCT is the removal of the uterus. In addition to this, the uterus, bilateral fallopian tubes and ovaries may also be removed
  • Majority of the tumors behave in a benign fashion; rarely, they do recur. Hence, a close follow-up is important. Since, there is a rare chance of recurrence; the tumor is often classified as a tumor of low malignant potential

Who gets Uterine Tumor Resembling Ovarian Sex Cord Tumors? (Age and Sex Distribution)

  • Uterine Tumor Resembling Ovarian Sex Cord Tumors usually occurs in premenopausal or postmenopausal women (age group 40-60 years)
  • No racial or ethnic predisposition is seen

What are the Risk Factors for Uterine Tumor Resembling Ovarian Sex Cord Tumors? (Predisposing Factors)

Risk factors for the development of Uterine Tumor Resembling Ovarian Sex Cord Tumors are currently unknown.

It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases ones chances of getting a condition compared to an individual without the risk factors. Some risk factors are more important than others.

Also, not having a risk factor does not mean that an individual will not get the condition. It is always important to discuss the effect of risk factors with your healthcare provider.

What are the Causes of Uterine Tumor Resembling Ovarian Sex Cord Tumors? (Etiology)

  • Currently, the cause of occurrence of Uterine Tumor Resembling Ovarian Sex Cord Tumors is unknown
  • No specific genetic or chromosomal abnormalities are noted in this tumor

What are the Signs and Symptoms of Uterine Tumor Resembling Ovarian Sex Cord Tumors?

The presence of Uterine Tumor Resembling Ovarian Sex Cord Tumors may lead to signs and symptoms that include, but are not limited to:

  • Abdominal pain, discomfort and/or feeling of pressure
  • Abnormal uterine bleeding
  • Increased size of the uterus
  • In some cases, upon palpation of the abdomen, an enlarged uterus may be felt

How is Uterine Tumor Resembling Ovarian Sex Cord Tumors Diagnosed?

Uterine Tumor Resembling Ovarian Sex Cord Tumors are rare tumors; hence, these tumors can cause diagnostic difficulties. They may be confused with other tumors, such as:

  • Epithelioid leiomyoma
  • Low-grade endometrial stromal sarcoma with sex cord elements
  • Endometrioid carcinoma showing sex cord-like features
  • Plexiform tumorlet
  • Plexiform vascular leiomyoma
  • Metastatic ovarian sex cord stromal tumors

A differential diagnosis of UTROSCT may include other tumors, such as carcinosarcoma, perivascular epithelioid cell tumor, adenomatoid tumor, and adenosarcoma.

The physician may perform a physical evaluation and other diagnostic lab tests to detect and establish the diagnosis. A diagnosis of UTROSCT is usually (definitively) made after examination of the hysterectomy specimen in a laboratory. Other diagnostic tests include:

  • Physical exam with medical history
  • CT scan of the abdominal and pelvic region
  • Ultrasound scan of abdomen and pelvis
  • X-ray studies of the pelvic, lower abdominal region
  • Radiological imaging studies, such as ultrasound of abdomen and CT scan of abdomen, do not reveal specific radiological findings
  • Most of the times, a diagnosis is made after a pathologist examines the tissue under a microscope. No specific genetic abnormalities or chromosomal abnormalities are noted with UTROSCT

Many clinical conditions may have similar signs and symptoms. Your healthcare provider may perform additional tests to rule out other clinical conditions to arrive at a definitive diagnosis

What are the possible Complications of Uterine Tumor Resembling Ovarian Sex Cord Tumors?

Complications with Uterine Tumor Resembling Ovarian Sex Cord Tumors are mostly due to a lack of proper treatment. The complications include:

  • Infection of the uterus
  • Uterine bleeding
  • Complications related to surgical removal of the tumor, such as surgical site infection

How is Uterine Tumor Resembling Ovarian Sex Cord Tumors Treated?

Early diagnosis and appropriate treatment is the key to a speedy recovery from Uterine Tumor Resembling Ovarian Sex Cord Tumors (UTROSCT):

  • The treatment of choice for these tumors is the removal of the uterus. Additionally, the bilateral fallopian tubes and ovaries may also be removed; a procedure termed - hysterectomy, with or without bilateral salpingo-oophorectomy
  • A majority of the medical scientists believe that these tumors have low malignancy potential, and that they may occasionally recur

How can Uterine Tumor Resembling Ovarian Sex Cord Tumors be Prevented?

Currently, no measures are currently available to prevent Uterine Tumor Resembling Ovarian Sex Cord Tumors formation.

What is the Prognosis of Uterine Tumor Resembling Ovarian Sex Cord Tumors? (Outcomes/Resolutions)

  • A proper and timely treatment of Uterine Tumor Resembling Ovarian Sex Cord Tumors is helpful. The prognosis is good in such cases, where the tumor can be removed
  • Rarely, the tumor may recur and hence close follow-up visits are important

Additional and Relevant Useful Information for Uterine Tumor Resembling Ovarian Sex Cord Tumors:

  • Morehead and Bowman described UTROSCT in 1945. Since the cell of origin of the tumor is not definitively known, the tumor is currently classified under miscellaneous category of tumors of the uterine body, in the World Health Organization tumor classification
  • Uterine Tumor Resembling Ovarian Sex Cord Tumors, when examined grossly by a pathologist, the tumor usually has a well circumscribed, non-capsulated feature. Very often, infiltrative border or pushing edges are observed on the tumor
  • The size of the tumor can vary greatly from 2 cm to 15 cm; studies have shown that usually, the tumor is about 6 cm in average dimension
  • Special studies of this tumor in the laboratory show the tumor to be positive for immunohistochemical stains, such as calretinin, inhibin, CD44, and Melan-A
  • Rarely, electron microscopic studies have been performed on the tumor. However, electron microscopic studies are not performed routinely, to arrive at a diagnosis

What are some Useful Resources for Additional Information?

American Cancer Society (ACS)
1599 Clifton Road, NE Atlanta, GA 30329-4251
Toll-Free: (800) 227-2345
TTY: (866) 228-4327
Website: http://www.cancer.org

National Cancer Institute (NCI)
U.S. National Institutes of Health
Public Inquiries Office
Building 31, Room 10A03
31 Center Drive, MSC 8322 Bethesda, MD 20892-2580
Phone: (301) 435-3848
Toll-Free: (800) 422-6237
TTY: (800) 332-8615
Email: cancergovstaff@mail.nih.gov
Website: http://www.cancer.gov

References and Information Sources used for the Article:

http://www.ncbi.nlm.nih.gov/pubmed/16118629 (accessed on 12/06/2013)

http://www.nature.com/modpathol/journal/v19/n1/full/3800475a.html (accessed on 12/06/2013)

http://informahealthcare.com/doi/abs/10.3109/01913120903506603 (accessed on 12/06/2013)

http://www.ncbi.nlm.nih.gov/pubmed/18317219 (accessed on 12/06/2013)

Helpful Peer-Reviewed Medical Articles:

Czernobilsky, B. (2008). Uterine tumors resembling ovarian sex cord tumors: an update. Int J Gynecol Pathol, 27(2), 229-235. doi: 10.1097/PGP.0b013e3181569a21

Kunz, J., & Friedrich, T. (2007). [Uterine tumor resembling ovarian sex cord tumor: case report and review of literature]. Praxis (Bern 1994), 96(31-32), 1177-1181.

Oztekin, O., Soylu, F., Yigit, S., & Sarica, E. (2006). Uterine tumor resembling ovarian sex cord tumors in a patient using tamoxifen: report of a case and review of literature. Int J Gynecol Cancer, 16(4), 1694-1697. doi: 10.1111/j.1525-1438.2006.00647.x

Stefanovic, A., Jeremic, K., Kadija, S., Mitrovic, M., Filimonovic, D., Jankovic-Raznatovic, S., & Tavcar, J. (2013). Uterine tumor resembling ovarian sex cord tumor. Case report and review of literature. Eur J Gynaecol Oncol, 34(3), 275-277.

Clement, P. B., & Scully, R. E. (1976). Uterine tumors resembling ovarian sex-cord tumors: a clinicopathologic analysis of fourteen cases. American journal of clinical pathology, 66(3), 512-525.

Dede, M., Gezginç, K., Yenen, M. C., Ulubay, M., Safalı, M., & Başer, İ. (2008). Uterine tumour resembling ovarian sex cord tumour. Eur J Gen Med, 5(2), 118-20.

Irving, J. A., Carinelli, S., & Prat, J. (2006). Uterine tumors resembling ovarian sex cord tumors are polyphenotypic neoplasms with true sex cord differentiation. Modern Pathology, 19(1), 17.

Krishnamurthy, S., Jungbluth, A. A., Busam, K. J., & Rosai, J. (1998). Uterine tumors resembling ovarian sex-cord tumors have an immunophenotype consistent with true sex-cord differentiation. The American journal of surgical pathology, 22(9), 1078-1082.

Kantelip, B., Cloup, N., & Dechelotte, P. (1986). Uterine tumor resembling ovarian sex cord tumors: report of a case with ultrastructural study. Human pathology, 17(1), 91-94.

Hurrell, D. P., & McCluggage, W. G. (2007). Uterine tumour resembling ovarian sex cord tumour is an immunohistochemically polyphenotypic neoplasm which exhibits coexpression of epithelial, myoid and sex cord markers. Journal of clinical pathology, 60(10), 1148-1154.

Kabbani, W., Deavers, M. T., Malpica, A., Burke, T. W., Liu, J., Ordoñez, N. G., ... & Silva, E. G. (2003). Uterine tumor resembling ovarian sex-cord tumor: report of a case mimicking cervical adenocarcinoma. International journal of gynecological pathology, 22(3), 297-302.

Biermann, K., Heukamp, L. C., Büttner, R., & Zhou, H. (2008). Uterine tumor resembling an ovarian sex cord tumor associated with metastasis. International Journal of Gynecological Pathology, 27(1), 58-60.