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Pseudomyxoma peritoneii is a rare entity. It is good for medical students as tutorial session
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PSEUDOMYXOMA PERITONEII
‘jelly belly’—a case
Dr Rekha KhareMD Radiology
It is a clinical condition caused by cancerous cells that produce diffuse intra peritoneal gelatinous ASCITIS in abdomen and pelvis
What is PMP ?
Ascites is recurrent, voluminous and mucinous
Ascites is due to surface growth on the peritoneum without invading the underlying tissue
contd…..
Carl F. Rokitansky reported first case in 1842
Werth coined the term pseudomyxoma peritoneii in 1884, that was in association with mucinous ovarian tumour
Frankel reported first case of PMP in 1901 that was in association with cyst of appendix
History of PMP…..
Over all incidence- 1-2/million/year
Male :Female ratio= 9:11
Median age at presentation is about 50years (range 20-25years)
Epidemiology…..
Ruptured mucinous tumour of appendix/ appendiceal mucocele
Most common cause…..
Mucinous tumour of large & small bowel, Lung, Breast, Pancreas, Stomach, Bile duct, Gall bladder and Fallopian tube / Ovary, urachal tumour are implicated
Other uncommon causes…..
Patient may present with:
-abdominal or pelvic pain -bloating/ distension -digestive disorder -weight loss -increasing abdominal girth -infertility
Clinical Presentation…..
In male: patient may come with Inguinal hernia
In female: presentation could be with uni/ bilateral ovarian disease
Clinical presentation…..
Ultrasound CT scan
History, clinical exam. & imaging studies often lead to the diagnosis
Sometimes diagnostic paracentesis under U/S confirmation by cytological exam.
Often discovered during surgery
How to diagnose PMP?
PET scan may be used to evaluate high grade mucinous adenocarcinoma
New MRI procedures are being developed for disease monitoring
Recent modality…..
Non mobile Ascites with septations or loculated ascites with echogenic particle
Scalloping of liver, spleen & other organs
Ultrasound findings…..
Loculated low attenuated ascites
Multiple complex cystic masses of fat density or variable density
CT findings.....modality of choice
Scalloping of visceral surface of liver, spleen & other organs
Compression or central displacement of small bowels
CT findings…..
Any of the above finding with:
- mass in Rt. lower quadrant/appendicial mucocele
-pelvic mass/ mucinous ovarian mass
- any other underlying cause of PMP
CT findings…..
Peritoneal carcinomatosis without mucinous ascites
Peritoneal sarcomatosis
Peritonitis
Differential Diagnosis…..
Peritoneal Adenomucinosis Primary tumour: ADENOMA
Peritoneal mucinous carcinoma Primary tumour: MUCINOUS ADENOCARCINOMA Intermediate or discordant feature
Pathological subtypes…..
Recurrent bowel obstruction due to fibrosis or adhesion
Mucus build up, filling the abdominal cavity, compression of organ will impede digestive or organ function
Increased abdominal pressure
Complications…..
Good with treatment
Lethal if untreated with death by -cachexia, -renal failure -other types of complication
Prognosis…..
A middle aged lady was referred to Deptt. Of Radiology for ultrasound exam.
She had problem of progressively increasing abdominal girth, pain in
abdomen and digestive disorders
Case to present…..
Multiloculated ascites with no particles (not moving like blood or pus)
Scalloping effect over the border of liver and spleen
(spleen is significantly reduced in size)
Ultrasound exam…..
Multi loculated ascites with well enhanced rim of peritoneum
Pocket of fluid in right iliac fossa, pelvic cavity and para colic gutter
CECT findings…..
Scalloping effect over the border of liver and spleen
Small bowel seems to be gathered in the center
Right pleural effusion r
CT findings….. Contd.
Finally…..
Long history of slow growing illness, clinical exam. and Imaging findings lead to the diagnosis of: Pseudomyxoma -Peritoneii
Nothing much could be done for her, as she has left the hospital against medical advice
Conclusion…..
PMP is rare, slow growing disease & may recur after surgery or chemotherapy
It is important to obtain an accurate diagnosis as treatment ranges from watchful waiting to: -debulking -hyper thermic intraperitoneal chemotherapy -cytoreductive surgery
References…..
Pseudomyxoma Peritoneii, Alexandra Stanislavsky. Radiology Reference article
Radiopaedia.org
Pseudomyxoma peritoneii(PMP), Diagnosis and Imaging finding www.medscape.com/view article/506509_2
Pseudomyxoma peritoneii. Submitted by Paurush shah MSIV www.learning radiology.com
Peritoneum & Mesentery PartII Pathology Angela Levy, The Radiology Assistant
References contd…..
Gray-scale sonographic finding in a patient with pseudomyxoma , C Lersch etal Journal of Clinical ultrasound vol 29 Issue3 page186-191March/April 2001
References contd…..