Pseudomyxoma peritonei

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Classification according to ICD-10
C78.6 Secondary malignant neoplasm of the retroperitoneum and peritoneum
ICD-10 online (WHO version 2019)
Computed tomography pseudomyxoma peritonei: extensive hypodense masses between the intestinal loops

As Pseudomyxoma the peritoneum (PMP, Syn .: gelatinous carcinoma , Gallertcarcinom , jelly belly ) a massive tumor cell poor mucus production throughout is Intraperitonealraum (abdominal cavity), respectively. It is a very slowly progressing clinical picture with tumor formation on the peritoneum (peritoneum) and on the omentum and associated with large amounts of slimy (mucinous) ascites ( ascites ) that eventually fills the entire abdomen.

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

etiology

It is a clinical syndrome; H. several diseases can be hidden behind it. Rupture , leak and metastasis of an intraperitoneal mucinous neoplasm are considered to be the cause of a pseudomyxoma peritonei . The origin of the tumor has been controversial for years, the appendix and ovary are discussed. However, benign diseases of these organs, such as a mucocele of the appendix vermiformis or pseudomucinic cystomas of the ovaries, also come into consideration.

Symptoms

The most common symptoms are abdominal distension and pain. Other possible symptoms are nausea, vomiting, urinary tract problems , fatigue and masses of tissue in the scrotum or inguinal canal . Ultimately, inguinal hernias that are filled with the jelly can develop. When the mucus is in large quantities, the diaphragm is pushed up, making it harder to breathe.

Diagnosis

Sonography reveals an anechoic, intraperitoneal, slimy mass in the entire abdominal cavity and between the intestinal loops, in which characteristic, delicate, streaky reflexes are embedded. The jelly is mobile and malleable, but reacts more slowly than a classic ascites. Due to the viscosity, grotesque, tumor-like shapes appear. A computed tomography shows the expansion of the Pseudomyxoms. Depending on the extent of endoscopic examinations of the stomach or colon, as in addition possibly colon and gastroscopy , urological examinations or investigations of heart and lung and blood samples necessary. The extracellular production of mucus in the abdominal cavity is a decisive diagnostic feature.

therapy

For a long time, surgery with the aim of removing the pseudomyxoma from the abdominal cavity as much as possible was the only therapeutic option. Often parts of the organs are also removed (e.g. sections of the small or large intestine , (partial) stomach removal, removal of the gall bladder , removal of the spleen , the large network, etc.). Often pseudomyxoma cells remain, which leads to renewed tumor growth ( relapse ) and re-operations under difficult conditions (e.g. due to cicatricial adhesions).

A chemotherapy through the bloodstream appears to have the Pseudomyxoma the peritoneum no particular benefit.

In 1999, Paul Sugarbaker from the Washington Cancer Institute was able to demonstrate with his work on 385 patients that cytoreductive surgery in combination with hyperthermic intraoperative intraperitoneal chemotherapy currently achieves the best results in the treatment of pseudomyxoma. Five years after treatment, 86% of all patients are still living with this procedure. These results were confirmed by other scientists in 2001 and 2004.

forecast

Repeated recurrence after operations in pseudomyxoma peritonei with expansion of the multiple septate cystoid hypodense (myxoid) masses into the abdominal wall.

The prognosis depends on the one hand on the surgically removed tumor mass and on the other hand on the malignancy of the tumor cells. Sugarbaker gives the 5-year survival rate with a complete pseudomyxoma removal with approx. 80 percent and without complete removal with approx. 20 percent. Other authors describe 5-year survival rates between approx. 30 percent for the highly aggressive ("high grade") tumor and almost 100 percent for the less aggressive ("low grade") tumor from the point of view of malignancy.

history

The clinical picture was first described by the pathologist Carl von Rokitansky in 1842 . The term Pseudomyxoma peritonei , however, goes back to the German gynecologist Richard Werth , who published it for the first time in 1884.

literature

  • Wim P. Ceelen (Ed.): Peritoneal Carcinomatosis: A Multidisciplinary Approach. 1st edition. Springer, Berlin 2007, ISBN 978-0-387-48991-9 .
  • Carlos Thomas (Ed.): Special Pathology . Schattauer, 1996, ISBN 3-7945-2110-2 ( limited preview in Google book search).
  • Stefan Feuerbach: Manual of diagnostic radiology: gastrointestinal system . Springer, 2007, ISBN 3-540-68472-7 ( limited preview in Google book search).
  • Michael Gnant, Peter M. Schlag (Ed.): Surgical Oncology: Strategies and Standards for Practice . Springer, 2008, ISBN 3-211-48612-7 ( limited preview in Google book search).
  • Werner Böcker, Helmut Denk, Philipp U. Heitz, Holger Moch: Pathology . Elsevier, Urban and Fischer, 2008, ISBN 3-437-42382-7 ( limited preview in Google book search).
  • Theodor Junginger, P. Hermanek, M. Klimpfinger: classification of malignant tumors of the gastrointestinal tract I . Springer, 2002, ISBN 3-540-42809-7 ( limited preview in Google book search).
  • Jörg Baltzer, Klaus Friese, Michael Graf, Friedrich Wolff (eds.): Practice of gynecology and obstetrics: the complete practical knowledge in one volume. Georg Thieme, 2006, ISBN 3-13-144261-1 ( limited preview in the Google book search).
  • Klaus Diedrich, Wolfgang Holzgreve (ed.): Gynecology and obstetrics . Springer, 2006, ISBN 3-540-32867-X ( limited preview in Google book search).
  • Vincent T. DeVita, Theodore S. Lawrence, Steven A. Rosenberg, Robert A Weinberg, Ronald A. DePinho: DeVita, Hellman, and Rosenberg's cancer: principles & practice of oncology, Volume 1 Cancer: Principles and Practice . Lippincott Williams and Wilkins, 2008, ISBN 0-7817-7207-9 ( limited preview in Google Book Search).

Individual evidence

  1. ↑ a b c d e f Information on Pseudomyxoma peritonei at Orphanet
  2. ↑ a b c d e f Information on Pseudomyxoma peritonei on the website of the St. Gallen Cantonal Hospital
  3. ↑ Pseudomyxoma peritonei, Gallertkarzinom, Gallertcarcinom ( Memento of February 9, 2007 in the Internet Archive ) by Herwart Müller
  4. ↑ Manfred Groß (Ed.): Sonography: Step by step to diagnosis. 1st edition. Elsevier, Munich 2006, ISBN 3-437-23630-X .
  5. ↑ PH Sugarbaker, D. Chang: Results of treatment of 385 patients with peritoneal surface spread of appendiceal malignancy. In: Annals of Surgical Oncology 8 (1999), pp. 727-731, PMID 10622499 .
  6. ^ AJ Witkamp, ​​E. de Bree, MM Kaag, GW van Slooten, F. van Coevorden, FA Zoetmulder: Extensive surgical cytoreduction and intraoperative hyperthermic intraperitoneal chemotherapy in patients with pseudomyxoma peritonei. In: British Journal of Surgery 88 (2001), pp. 458-463, PMID 11260116
  7. ↑ M. Deraco, D. Baratti, MG Inglese, B. Allaria, S. Andreola, C. Gavazzi, S. Kusamura: Peritonectomy and intraperitoneal hyperthermic perfusion (IPHP): a strategy that has confirmed its efficacy in patients with pseudomyxoma peritonei. In: Annals of Surgical Oncology. 11 (2004), pp. 393-398, PMID 15070599
  8. ↑ PH Sugarbaker: New standard of care for appendiceal epithelial neoplasms and pseudomyxoma peritonei syndrome? In: Lancet Oncology. 7 (2006), pp. 69-76, PMID 16389186
  9. ↑ R. Loungnarath, p Causeret, N. Bossard, M. Faheez, AC Sayag-Beaujard, C. Brigand, F. Gilly, O. Glehen: cytoreductive surgery with intraperitoneal chemohyperthermia for the treatment of pseudomyxoma inguinal hernia: a prospective study. In: Dis Colon Rectum . 48 (2005), pp. 1372-1379, PMID 15909071
  10. ↑ Jonathan A. Ledermann, William J. Hoskins, Stanley B. Kaye: Clinical management of ovarian cancer . Informa Health Care, 2001, ISBN 1-85317-704-0 ( limited preview in Google Book Search).
  11. ^ Richard Werth: Pseudomyxoma Peritonei . In: Archives for Gynecology. Volume 24 . August Hirschwald, Berlin 1884, p. 100 ff . ( limited preview in Google Book search).