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'''For patient information, click [[Ovarian cancer (patient information)|here]]'''
{{Mucinous cystadenocarcinoma}}
{{Mucinous cystadenocarcinoma}}
{{CMG}}; {{AE}} {{Ammu}}
{{CMG}}; {{AE}} {{Qurrat}}, {{Ammu}}
 
==Overview==
==Overview==
[[Mucinous cystadenocarcinoma]] of the [[renal pelvis]] was first described in 1960 by Hasebe et al. [[Mucinous cystadenocarcinoma]] is one of the most aggressive forms of [[cancer]]. ''KRAS'' [[mutations]] are found in [[Mucinous carcinoma|mucinous carcinomas]]. The [[organs]] involved in [[Mucinous cystadenocarcinoma pathophysiology|pathogenesis of mucinous cystadenocarcinoma]] are [[ovary]], [[appendix]], [[pancreas]], [[colon]], [[rectum]], [[retroperitoneal]] [[organs]], [[testes]], [[salivary gland]], [[lung]], [[bladder]], and [[breast]]. On [[gross pathology]], multiloculated, smooth grey surface, and multilocular [[mass]] with thin walls and [[mucinous]] material are [[Mucinous cystadenocarcinoma pathophysiology|characteristic findings of mucinous cystadenocarcinoma]]. On [[Mucinous cystadenocarcinoma biopsy|microscopic histopathological analysis]], [[mucinous]] differentiation, [[Atypia|nuclear atypia]], and [[necrosis]] are characteristic findings of [[mucinous cystadenocarcinoma]]. [[Mucinous cystadenocarcinoma epidemiology and demographics|Mucinous cystadenocarcinoma commonly affects individuals older than forty years of age]]. [[Mucinous cystadenocarcinoma epidemiology and demographics|Females are more commonly affected with mucinous cystadenocarcinoma of pancreas than males]]. [[Mucinous cystadenocarcinoma risk factors|Common risk factors in the development of mucinous cystadenocarcinoma]] are [[obesity]] and [[Menopausal|post-menopausal]] women on [[hormone replacement therapy]]. According to the [[American Joint Committee on Cancer]] (AJCC), there are [[Mucinous cystadenocarcinoma staging|4 stages of mucinous cystadenocarcinoma]] based on the [[Mucinous cystadenocarcinoma physical examination|clinica]]<nowiki/>[[Mucinous cystadenocarcinoma physical examination|l features]] and [[Mucinous cystadenocarcinoma CT|findings on imaging]]. Each stage is assigned a [[Mucinous cystadenocarcinoma staging|letter and a number that designate the tumor size, number of involved lymph node regions, and metastasis]]. Findings on [[Mucinous cystadenocarcinoma CT|CT]] suggestive of [[mucinous cystadenocarcinoma]] include rounded or [[Mucinous cystadenocarcinoma CT|ovoid tumor, internal septations, and calcification]]. The main [[Mucinous cystadenocarcinoma surgery|mode of treatment of mucinous cystadenocarcinoma]] is [[chemotherapy]] and [[radiation]]. The most effective [[Mucinous cystadenocarcinoma surgery|treatment for mucinous cystadenocarcinoma is surgical resection]].


==Historical Perspective==
==Historical Perspective==
Mucinous adenocarcinoma of the renal pelvis was first described in 1960 by Hasebe et al.<ref name="pmid13712095">{{cite journal| author=HASEBE M, SERIZAWA S, CHINO S| title=[On a case of papillary cystadenocarcinoma following malignant degeneration of a papillary adenoma in the kidney pelvis]. | journal=Yokohama Med Bull | year= 1960 | volume= 11 | issue=  | pages= 491-500 | pmid=13712095 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=13712095  }} </ref>
Mucinous adenocarcinoma of the [[renal pelvis]] was first described in 1960 by Hasebe et al.
==Pathophysiology==
==Pathophysiology==
Mucinous adenocarcinoma is one of the most agrressive form of cancer. KRAS mutations are found in mucinous carcinomas.The organs involved in pathogenesis of mucinous cystadenoma are ovary, appendix, pancreas, colon, rectum, retroperitoneal organs, testes, salivary gland, lung, bladder, and breast. On gross pathology, multiloculated, smooth grey surface, and multilocular mass with thin walls and mucinous material are characteristic findings of mucinous adenocarcinoma. On microscopic histopathological analysis, mucinous differentiation, nuclear atypia, and necrosis are characteristic findings of mucinous adenocarcinoma.
[[Mucinous cystadenocarcinoma pathophysiology|Mucinous adenocarcinoma is one of the most aggressive forms of cancer]]. ''[[KRAS]]'' [[mutations]] are found in [[Mucinous cystadenocarcinoma pathophysiology|mucinous cystadenocarcinomas]]. T[[Mucinous cystadenocarcinoma pathophysiology|he organs involved in pathogenesis of mucinous cystadenoma]] are [[ovary]], [[appendix]], [[pancreas]], [[colon]], [[rectum]], [[retroperitoneal]] organs, [[testes]], [[salivary gland]], [[lung]], [[bladder]], and [[breast]]. On [[gross pathology]], [[Mucinous cystadenocarcinoma pathophysiology|multiloculated, smooth grey surface, and multilocular mass with thin walls and mucinous material are characteristic findings of mucinous adenocarcinoma]]. On [[microscopic]] [[histopathological]] analysis, [[Mucinous cystadenocarcinoma biopsy|mucinous differentiation, nuclear atypia, and necrosis are characteristic findings of mucinous adenocarcinoma.]]
==Causes==
==Causes==
Mutations in the KRAS gene cause mucinous cystadenocarcinoma.
[[Mutations]] in the ''[[KRAS]]'' gene cause [[mucinous cystadenocarcinoma]].
==Differentiating Mucinous Cystadenocarcinoma from other Diseases==
==Differentiating Mucinous Cystadenocarcinoma from other Diseases==
Mucinous cystadenocarcinoma must be differentiated from mucinous cystadenoma, serous cystadenoma, and pseudocyst.
[[Mucinous cystadenocarcinoma differential diagnosis|Mucinous cystadenocarcinoma]] must be differentiated from [[mucinous cystadenoma]], serous cystadenoma, and [[pseudocyst]].
==Epidemiology and Demographics==
==Epidemiology and Demographics==
Mucinous cystadenocarcinoma commonly affects individuals older than forty years of age. Females are more commonly affected with mucinous cystadenoma of pancreas than males.
[[Mucinous cystadenocarcinoma epidemiology and demographics|Mucinous cystadenocarcinoma]] commonly affects individuals older than forty years of age. Females are more commonly affected with [[mucinous cystadenocarcinoma]] of [[pancreas]] than males.
==Risk Factors==
==Risk Factors==
Common risk factors in the development of mucinous cystadenocarcinoma are obesity and post menopausal women on hormone replacement therapy.
[[Mucinous cystadenocarcinoma risk factors|Common risk factors]] in the development of [[mucinous cystadenocarcinoma]] are [[obesity]] and post [[menopausal]] women on [[hormone replacement therapy]].
==Natural history, Complications and Prognosis==
==Natural history, Complications and Prognosis==
If left untreated, most of patients with mucinous cytoadenocarcinoma may be confined to the organ itself. Common complications of mucinous cystadenocarcinoma include metastasis and inguinal hernia. The presence of metastasis is associated with a particularly poor prognosis among patients with mucinous cystadenocarcinoma.
If left untreated, most of the patients with [[Mucinous cystadenocarcinoma natural history|mucinous cystadenocarcinomas]] may be confined to the [[Organ (biology)|organ]] itself. Common [[Mucinous cystadenocarcinoma natural history|complications of mucinous cystadenocarcinoma]] include [[metastasis]] and [[inguinal hernia]]. The presence of [[metastasis]] is associated with a particularly [[Prognosis|poor prognosis]] among [[patients]] with [[mucinous cystadenocarcinoma]].
==Staging==
 
According to the American Joint Committee on Cancer (AJCC)[1] there are 4 stages of mucinous cystadenocarcinoma based on the clinical features and findings on imaging. Each stage is assigned a letter and a number that designate the tumor size, number of involved lymph node regions, and metastasis.
== Diagnosis ==
==History and Symptoms==
 
Symptoms of mucinous cystadenocarcinoma of ovary include mass in the abdomen, increase in abdominal size, bloating, weightloss, shortness of breath, and pain abdomen.
=== Diagnostic study of choice ===
==Physical Examination==
According to the [[American Joint Committee on Cancer]] ([[AJCC]]) there are 4 stages of mucinous cystadenocarcinoma based on the [[Clinical|clinical features]] (pattern recongnition) and findings on [[imaging]]. Each stage is assigned a letter and a number that designate the [[tumor]] size, number of involved [[lymph node]] regions, and [[metastasis]].
[edit | edit source]
 
Patients with mucinous cystadenocarcinoma usually appear normal. Physical examination of patients with mucinous cystadenocarcinoma is usually remarkable for abdominal distention, shifting dullness, a palpable abdominal mass, and coarse crackles upon auscultation of the lung bases.
===Staging===
==CT==
According to the [[American Joint Committee on Cancer|American Joint Committee on Cancer (AJCC)]], there are [[Mucinous cystadenocarcinoma staging|4 stages of mucinous cystadenocarcinoma]] based on the [[Mucinous cystadenocarcinoma physical examination|clinical features]] and [[Mucinous cystadenocarcinoma MRI|findings on imaging]]. Each stage is assigned a letter and a number that designate the [[tumor]] size, number of involved [[lymph node]] regions, and [[metastasis]].
Findings on CT suggestive of mucinous cystadenocarcinoma include rounded or ovoid tumor, internal septations, and calcification.
 
==MRI==
===History and Symptoms===
Findings on MRI suggestive of mucinous cystadenocarcinoma include lower signal intensity for loculi with watery mucin on T1-weighted images.
[[Mucinous cystadenocarcinoma history and symptoms|Symptoms of mucinous cystadenocarcinoma]] of [[ovary]] include [[mass]] in the [[abdomen]], increase in [[abdominal]] size, [[bloating]], [[weight loss]], [[shortness of breath]], and [[pain]] [[abdomen]].
==Ultrasound==
===Physical Examination===
Ultrasound may be helpful in the diagnosis of mucinous cystadenocarcinoma. Findings on ultrasound suggestive of mucinous cystadenocarcinoma include mural thickening and solid components.
[[Patients]] with [[Mucinous cystadenocarcinoma physical examination|mucinous cystadenocarcinoma]] usually appear normal. [[Physical examination]] of [[Mucinous cystadenocarcinoma physical examination|patients with mucinous cystadenocarcinoma]] is usually remarkable for [[abdominal distention]], [[shifting dullness]], a [[Abdominal mass|palpable abdominal mass]], and [[Crackles|coarse crackles]] upon [[auscultation]] of the [[Lung|lung bases]].
==Biopsy==
 
On microscopic histopathological analysis, mucinous differentiation, nuclear atypia, and necrosis are characteristic findings of mucinous adenocarcinoma.
=== Electrocardiogram ===
==Medical Therapy==
[[Electrocardiogram]] findings in patients with mucinous cystadenocarcinoma are within normal limits.
The main mode of treatment of mucinous cystadenocarcinoma is chemotherapy and radiation.
 
==Surgery==
=== X-Ray ===
The most effective treatment for mucinous cystadenocarcinoma is surgical resection.
[[X-ray]] is not used in the [[diagnosis]] of mucinous cystadenocarcinoma. Instead, [[ultrasound]], [[CT scan]] and [[Magnetic resonance imaging|MRI]] are used in the [[diagnosis]] and staging of the [[tumor]].
 
===CT scan===
Findings on [[Computed tomography|CT]] suggestive of [[Mucinous cystadenocarcinoma CT|mucinous cystadenocarcinoma]] include rounded or [[Ovoid|ovoid tumor]], internal septations, and [[calcification]].
===MRI===
Findings on [[Magnetic resonance imaging|MRI]] suggestive of [[Mucinous cystadenocarcinoma MRI|mucinous cystadenocarcinoma]] include [[Magnetic resonance imaging|lower signal intensity for loculi]] with watery [[mucin]] on T1-weighted images.
===Ultrasound===
[[Ultrasound]] may be helpful in the [[diagnosis]] of [[Mucinous cystadenocarcinoma echocardiography or ultrasound|mucinous cystadenocarcinoma]]. Findings on [[ultrasound]] [[Mucinous cystadenocarcinoma echocardiography or ultrasound|suggestive of mucinous cystadenocarcinoma include mural thickening and solid components]].
===Biopsy===
On [[microscopic]] [[Histopathology|histopathological analysis]], [[Mucinous cystadenocarcinoma biopsy|mucinous differentiation, nuclear atypia, and necrosis are characteristic findings]] of [[mucinous cystadenocarcinoma]].
 
=== Other imaging findings ===
There are no other imaging findings associated with mucinous cystadenocarcinoma.
 
=== Other diagnostic studies ===
There are no other diagnostic studies associated with mucinous cystadenocarcinoma.
 
== Treatment ==
 
===Medical Therapy===
The main mode of [[Mucinous cystadenocarcinoma medical therapy|treatment of mucinous cystadenocarcinoma]] is [[chemotherapy]] and [[radiation]].
===Surgery===
The most effective [[Mucinous cystadenocarcinoma surgery|treatment for mucinous cystadenocarcinoma]] is [[surgical resection]].
 
== Prevention ==
 
=== Primary prevention ===
There are no established methods for [[primary prevention]] of mucinous cystadenocarcinoma.
 
==References==
==References==
{{reflist|2}}
{{reflist|2}}
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Latest revision as of 22:19, 24 May 2019


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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Qurrat-ul-ain Abid, M.D.[2], Ammu Susheela, M.D. [3]

Overview

Mucinous cystadenocarcinoma of the renal pelvis was first described in 1960 by Hasebe et al. Mucinous cystadenocarcinoma is one of the most aggressive forms of cancer. KRAS mutations are found in mucinous carcinomas. The organs involved in pathogenesis of mucinous cystadenocarcinoma are ovary, appendix, pancreas, colon, rectum, retroperitoneal organs, testes, salivary gland, lung, bladder, and breast. On gross pathology, multiloculated, smooth grey surface, and multilocular mass with thin walls and mucinous material are characteristic findings of mucinous cystadenocarcinoma. On microscopic histopathological analysis, mucinous differentiation, nuclear atypia, and necrosis are characteristic findings of mucinous cystadenocarcinoma. Mucinous cystadenocarcinoma commonly affects individuals older than forty years of age. Females are more commonly affected with mucinous cystadenocarcinoma of pancreas than males. Common risk factors in the development of mucinous cystadenocarcinoma are obesity and post-menopausal women on hormone replacement therapy. According to the American Joint Committee on Cancer (AJCC), there are 4 stages of mucinous cystadenocarcinoma based on the clinical features and findings on imaging. Each stage is assigned a letter and a number that designate the tumor size, number of involved lymph node regions, and metastasis. Findings on CT suggestive of mucinous cystadenocarcinoma include rounded or ovoid tumor, internal septations, and calcification. The main mode of treatment of mucinous cystadenocarcinoma is chemotherapy and radiation. The most effective treatment for mucinous cystadenocarcinoma is surgical resection.

Historical Perspective

Mucinous adenocarcinoma of the renal pelvis was first described in 1960 by Hasebe et al.

Pathophysiology

Mucinous adenocarcinoma is one of the most aggressive forms of cancer. KRAS mutations are found in mucinous cystadenocarcinomas. The organs involved in pathogenesis of mucinous cystadenoma are ovary, appendix, pancreas, colon, rectum, retroperitoneal organs, testes, salivary gland, lung, bladder, and breast. On gross pathology, multiloculated, smooth grey surface, and multilocular mass with thin walls and mucinous material are characteristic findings of mucinous adenocarcinoma. On microscopic histopathological analysis, mucinous differentiation, nuclear atypia, and necrosis are characteristic findings of mucinous adenocarcinoma.

Causes

Mutations in the KRAS gene cause mucinous cystadenocarcinoma.

Differentiating Mucinous Cystadenocarcinoma from other Diseases

Mucinous cystadenocarcinoma must be differentiated from mucinous cystadenoma, serous cystadenoma, and pseudocyst.

Epidemiology and Demographics

Mucinous cystadenocarcinoma commonly affects individuals older than forty years of age. Females are more commonly affected with mucinous cystadenocarcinoma of pancreas than males.

Risk Factors

Common risk factors in the development of mucinous cystadenocarcinoma are obesity and post menopausal women on hormone replacement therapy.

Natural history, Complications and Prognosis

If left untreated, most of the patients with mucinous cystadenocarcinomas may be confined to the organ itself. Common complications of mucinous cystadenocarcinoma include metastasis and inguinal hernia. The presence of metastasis is associated with a particularly poor prognosis among patients with mucinous cystadenocarcinoma.

Diagnosis

Diagnostic study of choice

According to the American Joint Committee on Cancer (AJCC) there are 4 stages of mucinous cystadenocarcinoma based on the clinical features (pattern recongnition) and findings on imaging. Each stage is assigned a letter and a number that designate the tumor size, number of involved lymph node regions, and metastasis.

Staging

According to the American Joint Committee on Cancer (AJCC), there are 4 stages of mucinous cystadenocarcinoma based on the clinical features and findings on imaging. Each stage is assigned a letter and a number that designate the tumor size, number of involved lymph node regions, and metastasis.

History and Symptoms

Symptoms of mucinous cystadenocarcinoma of ovary include mass in the abdomen, increase in abdominal size, bloating, weight loss, shortness of breath, and pain abdomen.

Physical Examination

Patients with mucinous cystadenocarcinoma usually appear normal. Physical examination of patients with mucinous cystadenocarcinoma is usually remarkable for abdominal distention, shifting dullness, a palpable abdominal mass, and coarse crackles upon auscultation of the lung bases.

Electrocardiogram

Electrocardiogram findings in patients with mucinous cystadenocarcinoma are within normal limits.

X-Ray

X-ray is not used in the diagnosis of mucinous cystadenocarcinoma. Instead, ultrasound, CT scan and MRI are used in the diagnosis and staging of the tumor.

CT scan

Findings on CT suggestive of mucinous cystadenocarcinoma include rounded or ovoid tumor, internal septations, and calcification.

MRI

Findings on MRI suggestive of mucinous cystadenocarcinoma include lower signal intensity for loculi with watery mucin on T1-weighted images.

Ultrasound

Ultrasound may be helpful in the diagnosis of mucinous cystadenocarcinoma. Findings on ultrasound suggestive of mucinous cystadenocarcinoma include mural thickening and solid components.

Biopsy

On microscopic histopathological analysis, mucinous differentiation, nuclear atypia, and necrosis are characteristic findings of mucinous cystadenocarcinoma.

Other imaging findings

There are no other imaging findings associated with mucinous cystadenocarcinoma.

Other diagnostic studies

There are no other diagnostic studies associated with mucinous cystadenocarcinoma.

Treatment

Medical Therapy

The main mode of treatment of mucinous cystadenocarcinoma is chemotherapy and radiation.

Surgery

The most effective treatment for mucinous cystadenocarcinoma is surgical resection.

Prevention

Primary prevention

There are no established methods for primary prevention of mucinous cystadenocarcinoma.

References

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