Pseudomyxoma peritonei overview: Difference between revisions
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==Overview== | ==Overview== | ||
Pseudomyxoma peritonei is a very rare [[tumor]] characterized by the presence of abundant [[mucus]] in the abdominal cavity, consisting of intraperitoneal mucinous tumors and [[ascites]], most commonly arising from mucinous [[Tumor|tumors]] of the [[appendix]], however, the [[ovary]] or the [[colon]] may be the primary site of origin. The [[tumor]] is not harmful by itself, but the [[mucus]] accumulation in [[abdominal]] cavity compresses vital organs such as [[spleen]], [[kidney]], [[colon]]. On 1842, Carl F. Rokitansky described the first case of pseudomyxoma peritonei. Pseudomyxoma peritonei may be divided into two [[pathological]] subtypes with different [[etiology]] and [[prognosis]], these two subtypes are peritoneal adenomucinosis and [[Mucinous carcinoma|peritoneal mucinous carcinoma]]. The ''[[Ras|K-Ras]]'' and [[P53 gene|''p53'' genes]] are involved in the [[oncogenesis]]. Pseudomyxoma peritonei must be differentiated from peritoneal carcinomatosis without mucinous [[ascites]], [[peritoneal]] sarcomatosis, and [[tuberculosis]] peritonitis. The [[Incidence rate|incidence]] of pseudomyxoma peritonei is approximately 0.1 person per 100,000 individuals worldwide. Females are more commonly affected with pseudomyxoma peritonei than males. The median age at diagnosis is 50 years. There are no established risk factors for pseudomyxoma peritonei. Patients with pseudomyxoma peritonei may develop [[abdominal]] or [[pelvic]] pain, [[bloating]], [[abdominal distension]], weight change, and [[infertility]] symptoms if left untreated. Common [[physical examination]] findings of pseudomyxoma peritonei include [[abdominal tenderness]], [[Abdominal distension|abdominal distention]]. On abdominal [[CT scan]], pseudomyxoma peritonei is characterized by low attenuation, loculated [[ascitic]] mucinous fluid in the [[peritoneum]], [[omentum]] and [[mesentery]] with scalloping of visceral surfaces of the [[liver]]. On abdominal MRI, pseudomyxoma peritonei is characterized by a mass which is hypointense on T1-weighted [[MRI]] and hyperintense on T2-weighted [[MRI]]. Finding on ultrasound scan suggestive of pseudomyxoma peritonei are echogenic peritoneal masses or [[ascites]] which is not mobile. Cytoreductive [[Surgery operation|surgery]] combined with [[hyperthermic intraperitoneal chemoperfusion]] is the optimal way of treatment for pseudomyxoma peritonei. | Pseudomyxoma peritonei is a very rare [[tumor]] characterized by the presence of abundant [[mucus]] in the [[abdominal cavity]], consisting of [[intraperitoneal]] [[mucinous]] [[Tumor|tumors]] and [[ascites]], most commonly arising from [[mucinous]] [[Tumor|tumors]] of the [[appendix]], however, the [[ovary]] or the [[colon]] may be the primary site of origin. The [[tumor]] is not harmful by itself, but the [[mucus]] accumulation in [[abdominal]] cavity compresses vital organs such as [[spleen]], [[kidney]], [[colon]]. On 1842, Carl F. Rokitansky described the first case of pseudomyxoma peritonei. Pseudomyxoma peritonei may be divided into two [[pathological]] subtypes with different [[etiology]] and [[prognosis]], these two subtypes are peritoneal adenomucinosis and [[Mucinous carcinoma|peritoneal mucinous carcinoma]]. The ''[[Ras|K-Ras]]'' and [[P53 gene|''p53'' genes]] are involved in the [[oncogenesis]]. Pseudomyxoma peritonei must be differentiated from peritoneal carcinomatosis without mucinous [[ascites]], [[peritoneal]] sarcomatosis, and [[tuberculosis]] peritonitis. The [[Incidence rate|incidence]] of pseudomyxoma peritonei is approximately 0.1 person per 100,000 individuals worldwide. Females are more commonly affected with pseudomyxoma peritonei than males. The median age at diagnosis is 50 years. There are no established risk factors for pseudomyxoma peritonei. Patients with pseudomyxoma peritonei may develop [[abdominal]] or [[pelvic]] pain, [[bloating]], [[abdominal distension]], weight change, and [[infertility]] symptoms if left untreated. Common [[physical examination]] findings of pseudomyxoma peritonei include [[abdominal tenderness]], [[Abdominal distension|abdominal distention]]. On abdominal [[CT scan]], pseudomyxoma peritonei is characterized by low attenuation, loculated [[ascitic]] mucinous fluid in the [[peritoneum]], [[omentum]] and [[mesentery]] with scalloping of visceral surfaces of the [[liver]]. On abdominal MRI, pseudomyxoma peritonei is characterized by a [[mass]] which is hypointense on T1-weighted [[MRI]] and hyperintense on T2-weighted [[MRI]]. Finding on [[ultrasound scan]] suggestive of pseudomyxoma peritonei are echogenic [[Peritoneal|peritoneal masses]] or [[ascites]] which is not mobile. Cytoreductive [[Surgery operation|surgery]] combined with [[hyperthermic intraperitoneal chemoperfusion]] is the optimal way of treatment for pseudomyxoma peritonei. | ||
==Historical Perspective== | ==Historical Perspective== | ||
The first case of pseudomyxoma peritonei was described by Carl F. Rokitansky in 1842. | The first case of pseudomyxoma peritonei was described by Carl F. Rokitansky in 1842. | ||
==Pathophysiology== | ==Pathophysiology== | ||
Pseudomyxoma peritonei is a rare poorly understood neoplasm which may be divided into two pathological subtypes: [[Disseminated peritoneal adenomucinosis]] (DPAM), and [[Peritoneal mucinous carcinomatosis]] (PMCA). | Pseudomyxoma peritonei is a rare poorly understood [[neoplasm]] which may be divided into two pathological subtypes: [[Disseminated peritoneal adenomucinosis]] (DPAM), and [[Peritoneal mucinous carcinomatosis]] (PMCA). | ||
==Causes== | ==Causes== | ||
Pseudomyxoma peritonei is caused by a pre-existing intraperitoneal mucinous neoplasm. The ''K-Ras'' and [[P53 gene|''p53'' genes]] may be involved in the oncogenesis. | Pseudomyxoma peritonei is caused by a pre-existing [[intraperitoneal]] [[mucinous]] [[neoplasm]]. The ''K-Ras'' and [[P53 gene|''p53'' genes]] may be involved in the [[oncogenesis]]. | ||
==Differentiating Pseudomyxoma peritonei from other Diseases== | ==Differentiating Pseudomyxoma peritonei from other Diseases== | ||
Differential diagnosis of pseudomyxoma peritonei include peritoneal carcinomatosis without mucinous ascites, peritoneal sarcomatosis, [[Peritonitis]], peritoneal [[mesothelioma|mesothelioma.]] | Differential diagnosis of pseudomyxoma peritonei include peritoneal carcinomatosis without mucinous ascites, peritoneal sarcomatosis, [[Peritonitis]], peritoneal [[mesothelioma|mesothelioma.]] | ||
==Epidemiology and Demographics== | ==Epidemiology and Demographics== | ||
The [[incidence]] of pseudomyxoma peritonei is approximately 0.1 per 100,000 individuals worldwide. Females are more commonly affected with pseudomyxoma peritonei than males. The median age at diagnosis is 50 years. | The [[incidence]] of pseudomyxoma peritonei is approximately 0.1 per 100,000 individuals worldwide. [[Female|Females]] are more commonly affected with pseudomyxoma peritonei than [[Male|males]]. The median age at diagnosis is 50 years. | ||
==Risk Factors== | ==Risk Factors== | ||
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==Natural History, Complications and Prognosis== | ==Natural History, Complications and Prognosis== | ||
Patients with pseudomyxoma peritonei may develop abdominal or pelvic pain, [[bloating]], [[abdominal distension]], [[weight change]], and [[infertility]]. [[Prognosis]] is generally good and the 10-year [[Survival rates|survival rate]] of patients with pseudomyxoma peritonei is approximately 63%.Overall risk of complications after [[surgery]] such as [[urinary tract]] involvement due to [[tumor]] seeding in pelvis is very low. | Patients with pseudomyxoma peritonei may develop [[Abdominal pain|abdominal or pelvic pain]], [[bloating]], [[abdominal distension]], [[weight change]], and [[infertility]]. [[Prognosis]] is generally good and the 10-year [[Survival rates|survival rate]] of patients with pseudomyxoma peritonei is approximately 63%.Overall risk of [[complications]] after [[surgery]] such as [[urinary tract]] involvement due to [[tumor]] seeding in [[pelvis]] is very low. | ||
==History and Symptoms== | ==History and Symptoms== | ||
Symptoms of pseudomyxoma peritonei include:[[abdominal pain]], [[bloating]], [[abdominal distention]], [[Shortness of breath]], [[Weight change]], [[Menstrual irregularities]] in women. | Symptoms of pseudomyxoma peritonei include: [[abdominal pain]], [[bloating]], [[abdominal distention]], [[Shortness of breath]], [[Weight change]], [[Menstrual irregularities]] in women. | ||
==Physical Examination== | ==Physical Examination== | ||
Common physical examination findings of pseudomyxoma peritonei include: [[Abdominal tenderness]], [[Abdominal distention]]. | Common physical examination findings of pseudomyxoma peritonei include: [[Abdominal tenderness]], [[Abdominal distention]]. | ||
==Laboratory Findings== | ==Laboratory Findings== | ||
The laboratory findings associated with pseudomyxoma peritonei include: increased levels of [[tumor markers]] such as [[carcinoembryonic antigen]] (CEA), carbohydrate antigen 19-9 (CA19-9), carbohydrate antigen 125 ([[CA125]]). | The laboratory findings associated with pseudomyxoma peritonei include: increased levels of [[tumor markers]] such as [[carcinoembryonic antigen]] (CEA), [[CA 19-9|carbohydrate antigen 19-9 (CA19-9)]], [[CA-125|carbohydrate antigen 125]] ([[CA125]]). | ||
==CT== | ==CT== | ||
Findings on abdominal [[CT scan]] of patients with pseudomyxoma peritonei include: accumulation of fluid throughout the [[peritoneum]], [[omentum]] and [[mesentery]] seen as low attenuation [[mucin]] depositis on [[CT scan]], scalloping of [[visceral]] organs. | Findings on abdominal [[CT scan]] of patients with pseudomyxoma peritonei include: accumulation of [[fluid]] throughout the [[peritoneum]], [[omentum]] and [[mesentery]] seen as low [[attenuation]] [[mucin]] depositis on [[CT scan]], scalloping of [[visceral]] organs. | ||
==MRI== | ==MRI== | ||
On abdominal [[MRI]], pseudomyxoma peritonei is characterized by a mass which is hypointense on T1-weighted [[MRI]] and hyperintense on T2-weighted MRI, it can further clarify the [[cystic]] structure of mass. | On abdominal [[MRI]], pseudomyxoma peritonei is characterized by a [[mass]] which is hypointense on T1-weighted [[MRI]] and hyperintense on T2-weighted [[MRI]], it can further clarify the [[cystic]] structure of [[mass]]. | ||
==Ultrasound== | ==Ultrasound== | ||
Abdominal [[ultrasound]] scan may be helpful in the diagnosis of pseudomyxoma peritonei. Finding on [[ultrasound]] scan suggestive of pseudomyxoma peritonei are echogenic peritoneal masses or [[ascites]] with echogenic particles which do not move. | Abdominal [[ultrasound]] scan may be helpful in the diagnosis of pseudomyxoma peritonei. Finding on [[ultrasound]] scan suggestive of pseudomyxoma peritonei are [[echogenic]] [[Peritoneal|peritoneal masses]] or [[ascites]] with echogenic particles which do not move. | ||
==Medical Therapy== | ==Medical Therapy== | ||
The predominant [[therapy]] for pseudomyxoma peritonei is [[Resection|surgical resection]] and [[debulking]]. Supportive therapy for pseudomyxoma peritonei includes [[chemotherapy]] which can be used as [[Adjuvant|adjuvant treatment]]. The treatment of choice is complete tumor excision named cytoreductive surgery (CRS) combined with [[Hyperthermic intraperitoneal chemoperfusion]] (HIPEC). | The predominant [[therapy]] for pseudomyxoma peritonei is [[Resection|surgical resection]] and [[debulking]]. Supportive therapy for pseudomyxoma peritonei includes [[chemotherapy]] which can be used as [[Adjuvant|adjuvant treatment]]. The treatment of choice is [[Excision|complete tumor excision]] named cytoreductive surgery (CRS) combined with [[Hyperthermic intraperitoneal chemoperfusion]] (HIPEC). | ||
==Surgery== | ==Surgery== | ||
[[Surgery]] is the mainstay of treatment for pseudomyxoma peritonei. The feasibility of [[surgery]] depends on the stage of pseudomyxoma peritonei at diagnosis. | [[Surgery]] is the mainstay of treatment for pseudomyxoma peritonei. The feasibility of [[surgery]] depends on the stage of pseudomyxoma peritonei at diagnosis. | ||
[[Category:Types of cancer]] | [[Category:Types of cancer]] | ||
Latest revision as of 20:14, 1 April 2019
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Nima Nasiri, M.D.[2]
Overview
Pseudomyxoma peritonei is a very rare tumor characterized by the presence of abundant mucus in the abdominal cavity, consisting of intraperitoneal mucinous tumors and ascites, most commonly arising from mucinous tumors of the appendix, however, the ovary or the colon may be the primary site of origin. The tumor is not harmful by itself, but the mucus accumulation in abdominal cavity compresses vital organs such as spleen, kidney, colon. On 1842, Carl F. Rokitansky described the first case of pseudomyxoma peritonei. Pseudomyxoma peritonei may be divided into two pathological subtypes with different etiology and prognosis, these two subtypes are peritoneal adenomucinosis and peritoneal mucinous carcinoma. The K-Ras and p53 genes are involved in the oncogenesis. Pseudomyxoma peritonei must be differentiated from peritoneal carcinomatosis without mucinous ascites, peritoneal sarcomatosis, and tuberculosis peritonitis. The incidence of pseudomyxoma peritonei is approximately 0.1 person per 100,000 individuals worldwide. Females are more commonly affected with pseudomyxoma peritonei than males. The median age at diagnosis is 50 years. There are no established risk factors for pseudomyxoma peritonei. Patients with pseudomyxoma peritonei may develop abdominal or pelvic pain, bloating, abdominal distension, weight change, and infertility symptoms if left untreated. Common physical examination findings of pseudomyxoma peritonei include abdominal tenderness, abdominal distention. On abdominal CT scan, pseudomyxoma peritonei is characterized by low attenuation, loculated ascitic mucinous fluid in the peritoneum, omentum and mesentery with scalloping of visceral surfaces of the liver. On abdominal MRI, pseudomyxoma peritonei is characterized by a mass which is hypointense on T1-weighted MRI and hyperintense on T2-weighted MRI. Finding on ultrasound scan suggestive of pseudomyxoma peritonei are echogenic peritoneal masses or ascites which is not mobile. Cytoreductive surgery combined with hyperthermic intraperitoneal chemoperfusion is the optimal way of treatment for pseudomyxoma peritonei.
Historical Perspective
The first case of pseudomyxoma peritonei was described by Carl F. Rokitansky in 1842.
Pathophysiology
Pseudomyxoma peritonei is a rare poorly understood neoplasm which may be divided into two pathological subtypes: Disseminated peritoneal adenomucinosis (DPAM), and Peritoneal mucinous carcinomatosis (PMCA).
Causes
Pseudomyxoma peritonei is caused by a pre-existing intraperitoneal mucinous neoplasm. The K-Ras and p53 genes may be involved in the oncogenesis.
Differentiating Pseudomyxoma peritonei from other Diseases
Differential diagnosis of pseudomyxoma peritonei include peritoneal carcinomatosis without mucinous ascites, peritoneal sarcomatosis, Peritonitis, peritoneal mesothelioma.
Epidemiology and Demographics
The incidence of pseudomyxoma peritonei is approximately 0.1 per 100,000 individuals worldwide. Females are more commonly affected with pseudomyxoma peritonei than males. The median age at diagnosis is 50 years.
Risk Factors
There are no established risk factors for pseudomyxoma peritonei.
Natural History, Complications and Prognosis
Patients with pseudomyxoma peritonei may develop abdominal or pelvic pain, bloating, abdominal distension, weight change, and infertility. Prognosis is generally good and the 10-year survival rate of patients with pseudomyxoma peritonei is approximately 63%.Overall risk of complications after surgery such as urinary tract involvement due to tumor seeding in pelvis is very low.
History and Symptoms
Symptoms of pseudomyxoma peritonei include: abdominal pain, bloating, abdominal distention, Shortness of breath, Weight change, Menstrual irregularities in women.
Physical Examination
Common physical examination findings of pseudomyxoma peritonei include: Abdominal tenderness, Abdominal distention.
Laboratory Findings
The laboratory findings associated with pseudomyxoma peritonei include: increased levels of tumor markers such as carcinoembryonic antigen (CEA), carbohydrate antigen 19-9 (CA19-9), carbohydrate antigen 125 (CA125).
CT
Findings on abdominal CT scan of patients with pseudomyxoma peritonei include: accumulation of fluid throughout the peritoneum, omentum and mesentery seen as low attenuation mucin depositis on CT scan, scalloping of visceral organs.
MRI
On abdominal MRI, pseudomyxoma peritonei is characterized by a mass which is hypointense on T1-weighted MRI and hyperintense on T2-weighted MRI, it can further clarify the cystic structure of mass.
Ultrasound
Abdominal ultrasound scan may be helpful in the diagnosis of pseudomyxoma peritonei. Finding on ultrasound scan suggestive of pseudomyxoma peritonei are echogenic peritoneal masses or ascites with echogenic particles which do not move.
Medical Therapy
The predominant therapy for pseudomyxoma peritonei is surgical resection and debulking. Supportive therapy for pseudomyxoma peritonei includes chemotherapy which can be used as adjuvant treatment. The treatment of choice is complete tumor excision named cytoreductive surgery (CRS) combined with Hyperthermic intraperitoneal chemoperfusion (HIPEC).
Surgery
Surgery is the mainstay of treatment for pseudomyxoma peritonei. The feasibility of surgery depends on the stage of pseudomyxoma peritonei at diagnosis.