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Pseudomyxoma peritonei

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Pseudomyxoma peritonei
SpecialtyOncology Edit this on Wikidata

Pseudomyxoma Peritonei is an uncommon tumor known for its production of mucus in the abdominal cavity.[1] If left untreated, mucin will eventually build up to the point where it compresses vital structures: the colon, the liver, kidneys, and pancreas.

Unlike most cancers, this disease rarely spreads through the lymphatic system or through the bloodstream. Therefore it is characterized by mucin and scattered cancer cells in the abdominal cavity but not with liver or other sites of metastasis.This disease is most commonly associated with cancer of the appendix; mucinous tumors of the ovary have also been implicated. There have been efforts to rename or reclassify this as a condition that begins in the appendix as low grade mucinous neoplasm of the appendix with disseminated peritoneal adenomucinosis.

Clinical

Pseudomyxoma Peritonei may be diagnosed with a range of conditions. While the majority of these cases are associated with appendiceal carcinomas,[2] other conditions may also be found, including disseminated peritoneal adenomucinosis (DPAM), peritoneal carcinomas, several mucinous cancers (mucinous adenocarcinoma, mucinous cystadenoma, and mucinous cystadenocarcinoma), as well as other disease states.[3] Symptoms may include abdominal or pelvic pain and/or bloating, distension, digestive disorders, weight changes, increased girth and infertility. Diagnosis is confirmed through pathology. Diagnostic tests may include CT scans, and the evaluation of tumor markers.

This disease is most often discovered during surgery for other conditions, e.g., hernia repair, following which an experienced pathologist can confirm the diagnosis. Due to the rarity of this disease, it is important to obtain an accurate diagnosis so that appropriate treatment may be obtained.

Treatment

Treatment is variable, both due to its rarity and to its frequently slow-growing nature. Treatment ranges from watchful waiting to debulking and cytoreductive surgery.[4]

Surgical

Most commonly, treatment involves surgery performed by specific specialists trained in treating this disease.

When appropriate, surgery may include hyperthermic intra-operative peritoneal chemotherapy (HIPEC), or early post-operative intraperitoneal chemotherapy (EPIC).

In situations where surgery is not required immediately, patients can be monitored via CT scans, tumor marker laboratory tests, and physical symptoms, to determine when, and if, surgery is warranted.

Although some surgical procedures may be rather extensive, patients can and do recover from surgery, and the majority of these patients can and do live productive lives.

In debulking, the surgeon attempts to remove as much tumor as possible. CRS or cytoreductive surgery involves surgical removal of the peritoneum and any adjacent organs which appear to have tumor seeding. Since the mucus tends to pool at the bottom of the abdominal cavity, it is common to remove the ovaries, fallopian tubes, uterus, and parts of the large intestine. Depending upon the spread of the tumor, other organs might be removed, including but not limited to the gallbladder, spleen, and all or portions of the small intestine and/or stomach. For organs that cannot be removed safely (like the liver), the surgeon strips off the tumor from the surface. [5]

Chemotherapy

Chemotherapy (typically the agent Mitomycin C) may be infused directly into the abdominal cavity to kill remaining microscopic cancerous cells. The heated chemotherapy (HIPEC) is perfused throughout the abdominal cavity for an hour or two as the last step in the surgery, or ports are installed to allow circulation and/or drainage of the chemicals for one to five days after surgery (EPIC). EPIC is typically given in multiple cycles for several months after surgery. [6]

Systemic chemotherapy may be administered as additional or adjuvant treatment. Due to the increased availability of new chemotherapies developed for colon and colorectal cancer patients, some patients have experienced stability in tumor growth with systemic chemotherapy. Systemic chemotherapy is reserved for patients with advanced disease, recurrent disease, or disease that has spread to the lymph nodes or distant sites.

This disease may recur following surgery and chemotherapy. Periodic post operative CT scans and tumor marker laboratory tests are used to monitor the disease for any tumor regrowth.

Additionally recent (2003) publications linking the MUC2 enzyme overexpression to the cell reproduction has launched research efforts into additional drug treatments.

Research

Research for treatment is presently conducted at major institutions in the USA, Australia Italy, England, France, Germany, Japan, the Netherlands, and Spain. Hundreds of published studies may be found in medical libraries and in online resources throughout the internet.

In mid-2008, the NIH temporarily suspended clinical trials for this disease, however funds for clinical trials became available again in 2009.

In 2004 APCAN, designated in the United States as a 501c3 charity, was developed, by patients, to fund research for the treatment of appendiceal cancer, from which the condition of pseudomyxoma peritonei originates.

Recognizing that research into this disease is seriously underfunded, patients in the United States formed an IRS-designated 501(c)3 charity in late 2008 called PMP Research Foundation (PMPRF). PMPRF's focus is to fund promising research to improve treatment options and ultimately to find a cure for the disease.

In spring, 2009, PMPRF retained NORD (National Organization for Rare Disorders) to administer its first $100,000 in grants. NORD is currently reviewing research proposals and will award 2 grants of $50,000 each in Fall 2009.

Future research into the treatment will necessitate an improved understanding of the molecular structure of the disease and targeted drug therapies that may interfere with the growth of mucinous tumor.

During recent years,treatment research using laboratory animals has been conducted by Dr Wim Ceelen in Belgium, Dr Edward Levine in the US, and Prof David L Morris in Australia. In summer 2009 Dr. David Bartlett, at the University of Pittsburgh Medical Center, reported to the president of PMPRF that UPMC's lab presently has live mice actively growing Pseudomyxoma Peritonei. Dr. Bartlett is hopeful the studies conducted with these mice will lead to advancements in PMP treatment.:)

PMP Pals' Network

In 1998, patients, and family caregivers in the US, UK and Holland united to share resources including regarding medical options, research, clinical trials, preparation and recuperation from/for surgery and/or chemotherapy, assistance with health insurance and disability claims, and assisting patients with obtaining transportation and family lodging near cancer centers.

These patients, and family caregivers, found that their need for personal interaction was not being met via message boards, therefore they created the PMP Pals' Network providing opportunities for patients and families to communicate directly, meet personally, and communicate via email,telephone or via Skype.

During the past decade the PMP Pals' Network of volunteers has expanded to include 45 countries and assists patients and their families in 12 languages.

References

  1. ^ Qu Z, Liu L (2006). "Management of pseudomyxoma peritonei". World J Gastroenterol. 12 (38): 6124–7. PMID 17036382.
  2. ^ Young R (2004). "Pseudomyxoma peritonei and selected other aspects of the spread of appendiceal neoplasms". Semin Diagn Pathol. 21 (2): 134–50. doi:10.1053/j.semdp.2004.12.002. PMID 15807473.
  3. ^ Jacquemin G, Laloux P (2005). "Pseudomyxoma peritonei: review on a cluster of peritoneal mucinous diseases". Acta Chir Belg. 105 (2): 127–33. PMID 15906901.
  4. ^ Sugarbaker P (2006). "New standard of care for appendiceal epithelial neoplasms and pseudomyxoma peritonei syndrome?". Lancet Oncol. 7 (1): 69–76. doi:10.1016/S1470-2045(05)70539-8. PMID 16389186.
  5. ^ Harmon R, Sugarbaker P (2005). "Prognostic indicators in peritoneal carcinomatosis from gastrointestinal cancer". Int Semin Surg Oncol. 2 (1): 3. doi:10.1186/1477-7800-2-3. PMC 549516. PMID 15701175.{{cite journal}}: CS1 maint: unflagged free DOI (link)
  6. ^ Culliford AT, Paty PB (2001). "Surgical debulking and intrapertioneal chemotherapy for established peritoneal metastases from colon and appendix cancer". Ann Surg Oncol. 8 (10): 787. doi:10.1007/s10434-001-0787-9. PMID 11776492.


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