Surface epithelial-stromal tumor: Difference between revisions
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==Overview== | ==Overview== | ||
'''Surface epithelial-stromal tumors''' are a class of [[Ovarian cancer|ovarian neoplasm]]s that may be [[benign]] or [[malignant]]. [[Neoplasm]]s in this group are thought to be derived from the [[ovarian surface epithelium]] (modified [[peritoneum]]) or from [[:wikt:ectopic|ectopic]] [[endometrial]] or [[Fallopian tube]] (tubal) tissue. This group of tumors accounts for 90% to 95% of all cases of [[ovarian cancer]].<ref>{{cite book |author=Bradshaw, Karen D.; Schorge, John O.; Schaffer, Joseph; Lisa M. Halvorson; Hoffman, Barbara G. |title=Williams' Gynecology |publisher=McGraw-Hill Professional |location= |year=2008 |pages= |isbn=0-07-147257-6 |oclc= |doi= |accessdate=}}</ref><ref name=SEER6215ch16>{{Cite book|contribution=Chapter 16: Cancers of the Ovary|first=Carol L.|last=Kosary|pages=133–144|publisher=National Cancer Institute|title=SEER Survival Monograph: Cancer Survival Among Adults: US SEER Program, 1988-2001, Patient and Tumor Characteristics|editor1-last=Baguio|editor1-first=RNL|editor2-last=Young|editor2-first=JL|editor3-last=Keel|editor3-first=GE|editor4-last=Eisner|editor4-first=MP|editor5-last=Lin|editor5-first=YD|editor6-last=Horner|editor6-first=M-J|series=SEER Program|volume=NIH Pub. No. 07-6215|place=Bethesda, MD|year=2007|chapterurl=http://seer.cancer.gov/publications/survival/surv_ovary.pdf|url=http://seer.cancer.gov/publications/survival/}}</ref> The pathogenesis of surface epithelial-stromal tumor is characterized by the overgrowth of the ovarian surface epithelium. 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, and endometrial or colon cancer (Lynch II). The prevalence of surface epithelial-stromal tumor is approximately 3 per 100,000 individuals worldwide. Surface epithelial-stromal tumor is more commonly observed among postmenopausal women. Early clinical features of surface epithelial-stromal tumor include pelvic fullness, abdominal distension, and abdominal pain. The mainstay of therapy for surface epithelial-stromal tumor is platinum-based chemotherapy. 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. | '''Surface epithelial-stromal tumors''' are a class of [[Ovarian cancer|ovarian neoplasm]]s that may be [[benign]] or [[malignant]]. [[Neoplasm]]s in this group are thought to be derived from the [[ovarian surface epithelium]] (modified [[peritoneum]]) or from [[:wikt:ectopic|ectopic]] [[endometrial]] or [[Fallopian tube]] (tubal) tissue. This group of tumors accounts for 90% to 95% of all cases of [[ovarian cancer]].<ref>{{cite book |author=Bradshaw, Karen D.; Schorge, John O.; Schaffer, Joseph; Lisa M. Halvorson; Hoffman, Barbara G. |title=Williams' Gynecology |publisher=McGraw-Hill Professional |location= |year=2008 |pages= |isbn=0-07-147257-6 |oclc= |doi= |accessdate=}}</ref><ref name="SEER6215ch16">{{Cite book|contribution=Chapter 16: Cancers of the Ovary|first=Carol L.|last=Kosary|pages=133–144|publisher=National Cancer Institute|title=SEER Survival Monograph: Cancer Survival Among Adults: US SEER Program, 1988-2001, Patient and Tumor Characteristics|editor1-last=Baguio|editor1-first=RNL|editor2-last=Young|editor2-first=JL|editor3-last=Keel|editor3-first=GE|editor4-last=Eisner|editor4-first=MP|editor5-last=Lin|editor5-first=YD|editor6-last=Horner|editor6-first=M-J|series=SEER Program|volume=NIH Pub. No. 07-6215|place=Bethesda, MD|year=2007|chapterurl=http://seer.cancer.gov/publications/survival/surv_ovary.pdf|url=http://seer.cancer.gov/publications/survival/}}</ref> The pathogenesis of surface epithelial-stromal tumor is characterized by the overgrowth of the ovarian surface epithelium. Common risk factors in the development of surface epithelial-stromal tumor, include: nulliparity, [[Menopause|early menopause]], [[Gonadal dysgenesis, XY female type|gonadal dysgenesis]], family history (e.g. [[BRCA1|BRCA1/BRCA2]] mutations), smoking, previous history of breast, and endometrial or colon cancer ([[Hereditary nonpolyposis colorectal cancer|Lynch]] II). The prevalence of surface epithelial-stromal tumor is approximately 3 per 100,000 individuals worldwide. Surface epithelial-stromal tumor is more commonly observed among postmenopausal women. Early clinical features of surface epithelial-stromal tumor include pelvic fullness, abdominal distension, and abdominal pain. The mainstay of therapy for surface epithelial-stromal tumor is [[Chemotherapy|platinum-based chemotherapy]]. 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. | ||
==Historical Perspective== | ==Historical Perspective== | ||
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==Classification== | ==Classification== | ||
*Surface epithelial-stromal tumor may be classified according to the World Health Organization (WHO) into 5 subtypes: | *Surface epithelial-stromal tumor may be classified according to the World Health Organization (WHO) into 5 subtypes: | ||
:*Mucinous tumors | :*[[Mucinous tumor|Mucinous tumors]] | ||
:*Endometroid tumors | :*Endometroid tumors | ||
:*Clear cell tumors | :*[[Clear cell tumor|Clear cell tumors]] | ||
:*Brenner tumors | :*[[Brenner tumor|Brenner tumors]] | ||
:*Small cell tumors | :*Small cell tumors | ||
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:*Lined by tall, columnar, ciliated epithelial cells | :*Lined by tall, columnar, ciliated epithelial cells | ||
:*Filled with clear serous fluid | :*Filled with clear serous fluid | ||
:*The presence of psammoma bodies | :*The presence of '''[[Psammoma body|psammoma bodies]]''' | ||
:*May involve the surface of the ovary | :*May involve the surface of the ovary | ||
:*The division between benign, borderline, and malignant is ascertained by assessing: | :*The division between benign, borderline, and malignant is ascertained by assessing: | ||
:*Cellular atypia (whether or not individual cells look abnormal) | :*[[Atypia|Cellular atypia]] (whether or not individual cells look abnormal) | ||
:*Invasion of surrounding ovarian stroma (whether or not cells are infiltrating surrounding tissue) | :*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 | :*Borderline tumors may have cellular atypia but do '''not''' have evidence of invasion | ||
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==Differentiating Surface Epithelial-Stromal Tumor from Other Diseases== | ==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: | *Surface epithelial-stromal tumor must be differentiated from other diseases that cause abdominal distension, pelvic or abdominal pain, and nausea, such as: | ||
:*Ovarian dysgerminoma | :*[[Dysgerminoma|Ovarian dysgerminoma]] | ||
:*Ovarian yolk sac tumor | :*[[Yolk sac tumor|Ovarian yolk sac tumor]] | ||
:*Ovarian embryonal carcinoma | :*Ovarian embryonal carcinoma | ||
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*The diagnosis of surface epithelial-stromal tumor when the following diagnostic criteria are met: | *The diagnosis of surface epithelial-stromal tumor when the following diagnostic criteria are met: | ||
:*Imaging findings compatible with ovarian mass. | :*Imaging findings compatible with ovarian mass. | ||
:*Elevated levels of CA-125 | :*Elevated levels of [[CA-125]] | ||
:*Present clinical criteria | :*Present clinical criteria | ||
::*Increased abdominal distension | ::*Increased abdominal distension | ||
Revision as of 16:00, 18 April 2016
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: Borderline ovarian tumors; Ovarian epithelial neoplasms
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] The pathogenesis of surface epithelial-stromal tumor is characterized by the overgrowth of the ovarian surface epithelium. 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, and endometrial or colon cancer (Lynch II). The prevalence of surface epithelial-stromal tumor is approximately 3 per 100,000 individuals worldwide. Surface epithelial-stromal tumor is more commonly observed among postmenopausal women. Early clinical features of surface epithelial-stromal tumor include pelvic fullness, abdominal distension, and abdominal pain. The mainstay of therapy for surface epithelial-stromal tumor is platinum-based chemotherapy. 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.
Historical Perspective
- Surface epithelial-stromal tumor was first discovered by Taylor in 1929.
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 when the following diagnostic criteria are met:
- Imaging findings compatible with ovarian mass.
- Elevated levels of CA-125
- Present clinical criteria
- Increased abdominal distension
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.
- Pelvic and abdominal examination may be remarkable for:
- Increased abdominal distension
- "Wave sign" ascities
- Decreased breath sounds
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 therapy for surface epithelial-stromal tumor is platinum-based chemotherapy.
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
- ↑ 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.
- ↑ 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.
- ↑ 3.0 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.