Pseudomyxoma peritonei

Pseudomyxoma peritonei As ( PMP, Syn: gelatinous carcinoma, Gallertcarcinom, jelly belly ) a massive tumor cell-poor mucus throughout Intraperitonealraum ( abdominal cavity) is called. It is a very slowly progressive disease with tumor formation in the peritoneum ( ) and on the omentum and accompanied by large amounts of mucous ( mucinous ) ascites (abdominal fluid ), which eventually fills the entire abdominal cavity.

Pseudomyxoma peritonei is very rare. It is found in only about one in 5,000 laparotomies ( abdominal incision ). In the medical literature, a frequency of about one disease to 1.4 million people per year is given.

Etiology

It is a clinical syndrome that is several diseases can hide behind it. As the cause of the peritoneum for a pseudomyxoma rupture, leak and metastasis of an intraperitoneal mucinous neoplasm are considered. The origin of the tumor has been controversial for years, appendicitis ( appendix ) and ovary ( ovary) are discussed. However, it also come benign diseases of these organs, such as a mucocele of the vermiform appendix or Pseudomuzinkystome the ovaries into consideration.

Symptoms

The most common symptoms are swelling of the abdomen and pain. Other possible symptoms include nausea, vomiting, urinary problems, fatigue and tissue masses in the scrotum or inguinal canal. Ultimately, then also arise inguinal hernias, which are filled with the jelly. If the mucus present in large amounts, the diaphragm is pushed upwards, so that the breathing schwererfällt.

Diagnostics

On ultrasonography, an echo-free, intraperitoneal, slimy mass shows throughout the abdominal cavity and between the bowel loops, in which characteristic, delicate, striated reflexes are incorporated. The jelly is flexible and deformable, but reacts slower than a classic ascites. Due to the viscosity appear grotesque, tumor-like forms. A computed tomography shows the extent of Pseudomyxoms. Depending on the extent of any endoscopic examination of the stomach or colon, such as colon and gastroscopy, urological investigations or examinations of the heart and lungs, and blood samples are also needed. As a key diagnostic feature applies the extracellular mucus production in the abdominal cavity.

Therapy

Surgery with the aim to remove the Pseudomyxom as far as possible from the abdominal cavity, has long been regarded as the only treatment option. Here are also frequently organ components are removed (eg sections of the small or large intestine, stomach (partial ) removal, gallbladder removal, removal of the spleen, the greater omentum, etc.). Often this Pseudomyxomzellen remain, resulting in renewed tumor growth (relapse ) and reoperations under difficult conditions (eg cicatricial adhesions ).

Chemotherapy through the bloodstream appears to have the pseudomyxoma peritonei no particular benefit.

In 1999, Paul was able Sugarbaker from the Washington Cancer Institute demonstrated with his work on 385 patients that the so-called cytoreductive surgery combined with hyperthermic intraoperative intraperitoneal chemotherapy currently achieved the best results in the treatment of Pseudomyxoms. With this method, five years to live after treatment still 86 % of all patients. These results were confirmed in 2001 and 2004 by other scientists.

Forecast

The prognosis depends on the one hand, on the tumor mass surgically removed and on the other by the malignancy ( malignancy ) of the tumor cells. Sugarbaker is the 5-year survival rate for a complete Pseudomyxomentfernung with about 80 percent and without complete removal of about 20 percent. Other authors describe the perspective of malignancy 5 - year survival rates of about 30 percent for the highly aggressive ( "high grade " ) tumor and almost 100 percent ( " low grade ") for the less aggressive tumor.

History

The disease was first described in 1842 by the pathologist Carl von Rokitansky. However, the term pseudomyxoma peritonei goes back to the German gynecologist Richard Werth, who first published this in 1884.