Surface epithelial-stromal tumor

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Maria Fernanda Villarreal, M.D. [2]

Synonyms and keywords: Synonym 1; Synonym 2; Synonym 3

Overview

Surface epithelial-stromal tumors are a class of ovarian neoplasms that may be benign or malignant. Neoplasms in this group are thought to be derived from the ovarian surface epithelium (modified peritoneum) or from ectopic endometrial or Fallopian tube (tubal) tissue. This group of tumors accounts for 90% to 95% of all cases of ovarian cancer.[1][2]

Historical Perspective

  • Surface epithelial-stromal tumor was first discovered by [scientist name], a [nationality + occupation], in [year] during/following [event].
  • In [year], [gene] mutations were first identified in the pathogenesis of surface epithelial-stromal tumor.
  • In [year], the first [discovery] was developed by [scientist] to treat/diagnose surface epithelial-stromal tumor.

Classification

  • Surface epithelial-stromal tumor may be classified according to the World Health Organization (WHO) into 5 subtypes:
  • Mucinous tumors
  • Endometroid tumors
  • Clear cell tumors
  • Brenner tumors
  • Small cell tumors

Pathophysiology

  • The pathogenesis of surface epithelial-stromal tumor is characterized by the overgrowth of the ovarian surface epithelium.
  • The HYAL1-3 mutation has been associated with the development of surface epithelial-stromal tumor.
  • On gross pathology, characteristic findings of surface epithelial-stromal tumor, include:
  • Complex multiloculated mass with mucin.
  • Often large - may > 30 cm.
  • On microscopic histopathological analysis, characteristic findings of surface epithelial-stromal tumor, include:
  • Lined by tall, columnar, ciliated epithelial cells
  • Filled with clear serous fluid
  • The presence of psammoma bodies
  • May involve the surface of the ovary
  • The division between benign, borderline, and malignant is ascertained by assessing:
  • Cellular atypia (whether or not individual cells look abnormal)
  • Invasion of surrounding ovarian stroma (whether or not cells are infiltrating surrounding tissue)
  • Borderline tumors may have cellular atypia but do not have evidence of invasion

Causes

  • Causes of surface epithelial-stromal tumor, include:
  • Mucinous tumors
  • Endometroid tumors
  • Clear cell tumors
  • Brenner tumors
  • Small cell tumors

Differentiating Surface Epithelial-Stromal Tumor from Other Diseases

  • Surface epithelial-stromal tumor must be differentiated from other diseases that cause abdominal distension, pelvic or abdominal pain, and nausea, such as:
  • Ovarian dysgerminoma
  • Ovarian yolk sac tumor
  • Ovarian embryonal carcinoma

Epidemiology and Demographics

  • The prevalence of surface epithelial-stromal tumor is approximately 3 per 100,000 individuals worldwide.
  • In 2011, the delay-adjusted incidence of surface epithelial-stromal tumor was estimated to be 12.46 per 100,000 persons in the United States.[3]
  • In the United States, the age-adjusted prevalence of surface epithelial-stromal tumor is 71.3 per 100,000 in 2011.[3]

Age

  • Patients of all age groups may develop surface epithelial-stromal tumor.
  • Surface epithelial-stromal tumor is more commonly observed among patients aged 55 to 75 years old.
  • Surface epithelial-stromal tumor is more commonly observed among postmenopausal women.

Gender

  • Surface epithelial-stromal tumor only affects women.

Race

  • There is no racial predilection for surface epithelial-stromal tumor.

Risk Factors

  • Common risk factors in the development of surface epithelial-stromal tumor, include:
  • Nulliparity
  • Early menopause
  • Gonadal dysgenesis
  • Family history (e.g. BRCA1/BRCA2 mutations)
  • Smoking
  • Previous history of breast, endometrial or colon cancer (Lynch II)
  • Common protective factors in the development of surface epithelial-stromal tumor, include:
  • Oral contraceptives

Natural History, Complications and Prognosis

  • The majority of patients with surface epithelial-stromal tumor remain asymptomatic for years.
  • Early clinical features of surface epithelial-stromal tumor include pelvic fullness, abdominal distension, and abdominal pain.
  • If left untreated, the minority of patients with surface epithelial-stromal tumor may progress to develop local invasion, lymphadenopathy, ascites, or metastases.
  • Common complications of surface epithelial-stromal tumor include peritoneal metastases, or ovarian torsion.
  • Prognosis will depend on tumor histology. In general, the 10-year survival rate of patients with surface epithelial-stromal tumor is approximately 66% - 90%

Diagnosis

Diagnostic Criteria

  • The diagnosis of surface epithelial-stromal tumor is made when at least [number] of the following [number] diagnostic criteria are met:
  • [criterion 1]
  • [criterion 2]
  • [criterion 3]
  • [criterion 4]

Symptoms

  • Surface epithelial-stromal tumor is usually asymptomatic.
  • Symptoms of surface epithelial-stromal tumor may include the following:

Physical Examination

  • Patients with surface epithelial-stromal tumor usually are well-appearing.
  • Physical examination may be remarkable for:
  • Pelvic and abdominal examination may be remarkable for:

Laboratory Findings

  • Laboratory findings consistent with the diagnosis of surface epithelial-stromal tumor, include:
  • Elevated CA-125 level
  • Often unspecific
  • Useful for treatment response

Imaging Findings

  • Pelvic transabdominal/transvaginal ultrasound with or without Doppler is the initial imaging method of choice for surface epithelial-stromal tumor.
  • Enhanced CT is the imaging modality of choice for surface epithelial-stromal tumor.
  • On ultrasound, findings of surface epithelial-stromal tumor, include:
  • Hypoechoic/hyperechoic solid mass
  • Calcification
  • On CT, findings of surface epithelial-stromal tumor, include:
  • Calcifications
  • Solid component may show mild to moderate enhancement post contrast.
  • Large mass
  • On MRI, findings of surface epithelial-stromal tumor, include
  • Hypointense on T2 weighted sequences

Treatment

Medical Therapy

  • The mainstay of therapies for surface epithelial-stromal tumor, include:

Surgery

  • Surgery is the mainstay of therapy for surface epithelial-stromal tumor.

Prevention

  • There are no primary preventive measures available for surface epithelial-stromal tumor.
  • A controversial measure for the primary prevention of surface epithelial-stromal tumor may include oral contraceptives.
  • According to the American College of Radiology, secondary prevention of surface epithelial-stromal tumor may include periodical pelvis or transvaginal ultrasound with or without Doppler.

References

  1. Bradshaw, Karen D.; Schorge, John O.; Schaffer, Joseph; Lisa M. Halvorson; Hoffman, Barbara G. (2008). Williams' Gynecology. McGraw-Hill Professional. ISBN 0-07-147257-6.
  2. Kosary, Carol L. (2007). "Chapter 16: Cancers of the Ovary" (PDF). In Baguio, RNL; Young, JL; Keel, GE; Eisner, MP; Lin, YD; Horner, M-J. SEER Survival Monograph: Cancer Survival Among Adults: US SEER Program, 1988-2001, Patient and Tumor Characteristics. SEER Program. NIH Pub. No. 07-6215. Bethesda, MD: National Cancer Institute. pp. 133–144.
  3. 3.0 3.1 Howlader N, Noone AM, Krapcho M, Garshell J, Miller D, Altekruse SF, Kosary CL, Yu M, Ruhl J, Tatalovich Z,Mariotto A, Lewis DR, Chen HS, Feuer EJ, Cronin KA (eds). SEER Cancer Statistics Review, 1975-2011, National Cancer Institute. Bethesda, MD, http://seer.cancer.gov/csr/1975_2011/, based on November 2013 SEER data submission, posted to the SEER web site, April 2014.