pseudomyxoma peritoneii

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…..