Surface epithelial-stromal tumor: Difference between revisions

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==Overview==
==[[Surface epithelial-stromal tumor overview|Overview]]==
'''Surface epithelial-stromal [[tumor]]s''' are a class of [[ovarian neoplasm]]s that may be [[benign]] or [[malignant]]. [[Neoplasm]]s in this group are thought to be derived from the [[ovary|ovarian]] surface [[epithelium]] (modified [[peritoneum]]) or from [[ectopic]] [[endometrial]] tissue. This group of tumors accounts for the majority of all ovarian tumors. Serum [[CA-125]] is often elevated but is only 50% accurate so it is not a useful tumour marker to assess the progress of treatment.


==Classification==
==[[Surface epithelial-stromal tumor historical perspective|Historical Perspective]]==
Epithelial-stromal tumors are classified on the basis of the [[epithelial cell]] type, the relative amounts of epithelium and [[stroma]], the presence of [[papillary]] processes, and the location of the epithelial elements. [[Microscope|Microscopic]] [[pathology|pathological]] features determine whether a surface epithelial-stromal tumor is [[benign]], borderline, or [[malignant]] (evidence of malignancy and stromal invasion). Borderline tumors are of uncertain malignant potential.


This group consists of [[serous tumor|serous]], [[mucinous tumor|mucinous]], [[endometrioid tumor|endometrioid]], [[clear cell tumor|clear cell]], and [[brenner tumor|brenner]] (transitional cell) tumors, though there are a few mixed, undifferentiated and unclassified types.
==[[Surface epithelial-stromal tumor classification|Classification]]==


[[Image:lmp_dry.jpg|thumb|left|Ovarian surface papillary serous tumor of low malignant potential.Courtesy of Ed Uthman, MD]]
==[[Surface epithelial-stromal tumor pathophysiology|Pathophysiology]]==
[[Image:ovary_serous_ca.jpg|thumb|left|Serous cystadenocarcinoma of the ovary.  Courtesy of Ed Uthman, MD]]


==[[Surface epithelial-stromal tumor causes|Causes]]==


==[[Surface epithelial-stromal tumor differential diagnosis|Differentiating Surface epithelial-stromal tumor from other Diseases]]==


===Serous tumors===
==[[Surface epithelial-stromal tumor epidemiology and demographics|Epidemiology and Demographics]]==
*These tumors vary in size from small and nearly imperceptible to large, filling the [[abdominal]] cavity. 
*[[Benign]], borderline, and [[malignant]] types of serous tumors account for about 30% of all ovarian tumors.
*75% are [[benign]] or of borderline malignancy, and 25% are [[malignant]]
*The malignant form of this tumor, serous cystadenocarcinoma, accounts for approximately 40% of all carcinomas of the ovary and are the most common malignant ovarian tumors.
*Benign and borderline tumors are most common between the ages of 20 and 50 years.
*Malignant serous tumors occur later in life on average, although somewhat earlier in familial cases.
*20% of benign, 30% of borderline, and 66% of malignant tumors are bilateral (affect both ovaries).


Components can include:
==[[Surface epithelial-stromal tumor risk factors|Risk Factors]]==
#cystic areas
#cystic and fibrous areas
#predominantly fibrous areas


The chance of [[malignancy]] of the tumor increases with the amount of solid areas present, including both papillary structures and any necrotic tissue present.
==[[Surface epithelial-stromal tumor screening|Screening]]==


====Pathology====
==[[Surface epithelial-stromal tumor natural history, complications and prognosis|Natural History, Complications and Prognosis]]==
*lined by tall, columnar, [[ciliated]] [[epithelial]] cells
*filled with clear [[serous]] fluid
*the term [[serous]] which originated as a description of the [[cyst]] fluid has come to be describe the particular type of [[epithelial]] [[cell (biology)|cell]] seen in these tumors
*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 my have cellular atypia but do NOT have evidence of invasion
**the presence of [[psammoma body|psammoma bodies]] are a characteristic microscopic finding of cystadenocarcinomas<ref>Kumar: Robbins and Cotran: Pathologic Basis of Disease, 7th ed.</ref>


====Prognosis====
==Diagnosis==
The prognosis of a serous tumor, like most neoplasms, depends on
[[Surface epithelial-stromal tumor staging|Staging]] | [[Surface epithelial-stromal tumor history and symptoms|History and Symptoms]] | [[Surface epithelial-stromal tumor physical examination|Physical Examination]] | [[Surface epithelial-stromal tumor laboratory findings|Laboratory Findings]] | [[Surface epithelial-stromal tumor chest x ray|Chest X Ray]] | [[Surface epithelial-stromal tumor CT|CT]] | [[Surface epithelial-stromal tumor MRI|MRI]] | [[Surface epithelial-stromal tumor other imaging findings|Other Imaging Findings]] | [[Surface epithelial-stromal tumor other diagnostic studies|Other Diagnostic Studies]]
*degree of diffentiation
**this is how closely the tumor cells resemble benign cells
**a well-differentiated tumor closely resembles benign tumors
**a poorly differentiated tumor may not resemble the cell type of origin at all
**a moderately differentiated tumor usually resembles the cell type of origin, but appears frankly malignant
*extension of tumor to other structures
**in particular with serous malignancies, the presence of malignant spread to the peritoneum is important with regard to prognosis.


The [[five year survival rate]] of borderline and malignant tumors confined to the ovaries are 100% and 70% respectively. If the peritoneum is involved, these rates become 90% and 25%.
==Treatment==
[[Surface epithelial-stromal tumor medical therapy|Medical Therapy]] | [[Surface epithelial-stromal tumor surgery|Surgery]] | [[Surface epithelial-stromal tumor primary prevention|Primary Prevention]] | [[Surface epithelial-stromal tumor secondary prevention|Secondary Prevention]] | [[Surface epithelial-stromal tumor cost-effectiveness of therapy|Cost-Effectiveness of Therapy]] | [[Surface epithelial-stromal tumor future or investigational therapies|Future or Investigational Therapies]]


While the 5-year survival rates of borderline tumors are excellent, this should not be seen as evidence of cure, as recurrences can occur many years later.
==Case Studies==


===Mucinous tumors===
[[Surface epithelial-stromal tumor case study one|Case #1]]
[[Mucinous tumor]]s:
*Closely resemble their serous counterparts
*Somewhat less common, accounting for about 25% of all ovarian neoplasms
*Occur principally in middle adult life and are rare before puberty and after menopause
*80% are benign or borderline and about 15% are malignant
*Mucinous cystadenocarcinomas (the malignant form of this tumor) are relatively uncommon and account for only 10% of all ovarian cancers
*Mucinous tumors are characterized by more cysts of variable size and a rarity of surface involvement as compared to serous tumors
*Also in comparison to serous tumors, mucinous tumorsare less frequently bilateral, approximately 5% of primary mucinous tumors are bilateral.
*May form very large cystic masses, with recorded weights exceeding 25kg
*Appear as multiloculated tumors filled with sticky, gelatinous fluid 
 
====Pathology====
Benign mucinous tumors are characterized by a lining of tall columnar epithelial cells with apical mucin and the absence of cilia, similar in appearance with benign cervical or intestinal epithelia. Cystadenocarcinomas (malignant tumors) contain a more solid growth pattern with the hallmarks of malignancy:  cellular atypia and stratification, loss of the normal architecture of the tissu, and necrosis.  The appearance can look similar to colonic cancer. Clear stromal invasion is used to differentiate borderline tumors from malignant tumors.
 
====Prognosis====
10-year survival rates for borderline tumors contained within the ovary, malignant tumors without invasion, and invasive malignant tumors are greater than 95%, 90%, and 66%, respectively. One rare but noteworthy condition associated with mucinous ovarian neoplasms is [[pseudomyxoma peritonei]]. As primary ovarian mucinous tumors are usually unilateral (in one ovary), the presentation of bilateral mucinous tumors requires exclusion of a non-ovarian origin.
 
===Endometrioid tumors===
[[Endometrioid tumor]]s account for approximately 20% of all ovarian cancers and are mostly malignant (endometroid carcinomas). They are made of tubular glands bearing a close resemblance to benign or malignant endometrium. 15-30% of endometrioid carcinomas occur in individuals with carcinoma of the endometrium, and these patients have a better prognosis. They appear similar to other surface epithelial-stromal tumors, with solid and cystic areas. 40% of these tumors are bilateral, when bilateral, metastases is often present.
 
====Pathology====
*Glands bearing a strong resemblance to endometrial-type glands
**Benign tumors have mature-appearing glands in a fibrous stroma
**Borderline tumors have a complex branching pattern without stromal invasion
**Carcinomas (malignant tumors) have invasive glands with crowded, atypical cells, frequent mitoses.  With poorer differentiation, the tumor becomes more solid.
 
====Prognosis====
Prognosis again is dependent on the spread of the tumor, as well as how differentiated the tumor appears.  The overall prognosis is somewhat worse than for serous or mucinous tumors, and the 5-year survival rate for patients with tumors confined to the ovary is approximately 75%.
 
===Clear cell tumors===
[[Clear cell tumor]]s are characterized by large epithelial cells with abundant clear cytoplasm and may be seen in association with endometriosis or endometrioid carcinoma of the ovary, bearing a resemblance to clear cell carcinoma of the endometrium. They may be predominantly solid or cystic. If solid, the clear cells tend to be arranged in sheets or tubules. In the cystic variety, the neoplastic cells make up the cyst lining.
 
====Prognosis====
These tumors tend to be aggressive, the five year survival rate for tumors confined to the ovaries in approximately 65%.  If the tumor has spread beyond the ovary at diagnosis, the prognosis is poor
 
===Brenner tumor===
[[Brenner tumor]]s are uncommon surface-epithelial stromal cell tumors in which the epithelial cell (which defines these tumors) is a transitional cell.  These are similar in appearance to bladder epithelia.  The tumors may be very small to very large, and may be solid or cystic.  Histologically, the tumor consists of nests of the aforementioned transitional cells within surrounding tissue that resembles normal ovary.  Brenner tumors may be benign or malignant, depending on whether or not the tumor cells invade the surrounding tissue.
 
==Treatment of ovarian cancer==
Surgical treatment may be sufficient for malignant tumors that are well-differentiated and confined to the ovary. Addition of chemotherapy may be required for more aggressive tumors that are confined to the ovary. For patients with advanced disease a combination of surgical reduction with a combination chemotherapy regimen is standard. Borderline tumors, even following spread outside of the ovary, are managed well with surgery, and chemotherapy is not seen as useful.
 
==Sources==
* Kumar, et al, ed. Robbins and Cotran Pathologic Basis of Disease, 7th Edition, Elsevier-Saunders, 2005.
* Braunwald, et al, ed. Harrison's Principles of Internal Medicine, 15th Edition, McGraw-Hill, 2001.
* Haber, et al, Differential Diagnosis in Surgical Pathology, Saunders, 2002.
 
==References==
{{Reflist}}
 
==External links==
* [http://ovariancancer.jhmi.edu/epithelial.cfm Johns Hopkins: "Surface Epithelial Tumors"]
* [http://www.netterimages.com/image/3561.htm Diagram: "Epithelial Stromal Ovarian Tumors"]


[[Category:Gynecology]]
[[Category:Gynecology]]

Revision as of 20:35, 18 September 2012