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Pseudomyxoma peritonei

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Pseudomyxoma peritonei

Pseudomyxoma Peritonei
Computed tomographic scan of an abdomen showing pseudomyxoma peritonei with multiple peritoneal masses (arrow) with "scalloping effect" seen.
Classification and external resources
ICD-10 C78.6
ICD-9-CM 197.6
ICD-O M8480
DiseasesDB 31498
eMedicine med/1795
MeSH D011553

Pseudomyxoma peritonei (PMP) is a clinical condition caused by cancerous cells (mucinous adenocarcinoma) that produce abundant

External links

  1. ^ Qu Z, Liu L (2006). "Management of pseudomyxoma peritonei". World J Gastroenterol 12 (38): 6124–7.  
  2. ^ Chua; et al. (2012). "Early- and Long-Term Outcome Data of Patients With Pseudomyxoma Peritonei From Appendiceal Origin Treated by a Strategy of Cytoreductive Surgery and Hyperthermic Intraperitoneal Chemotherapy". Journal of Clinical Oncol 30 (20): 2449–2456.  
  3. ^ Misdraji, Joseph (2010). "Appendiceal mucinous neoplasms: Controversial issues" (PDF). Arch Pathol Lab Med. (Pathology Portal) 134 (6): 864–870. 
  4. ^ Ronnett BM, Zahn CM, Kurman RJ, Sugarbaker PH,Shmookler BM (1995). "Disseminated peritoneal adenomucinosis and peritoneal mucinous carcinomatosis. A clinicopathologic analysis of 109 cases with emphasis on distinguishing pathologic features, site of origin, prognosis, and relationship to 'pseudomyxoma peritonei'". Am J Surg Pathol. 19 (12): 1390–408.  
  5. ^ Bradley, R.F.; Cortina, G.; Geisinger, K.R. (October 2007). "Pseudomyxoma peritonei: Review of the controversy". Current Diagnostic Pathology 13 (5): 410–416.  
  6. ^ e.g., http://surgpathcriteria.stanford.edu/gitumors/appendix-adenocarcinoma/
  7. ^ Panarelli, Nicole C; Yantiss, Rhonda K (October 2011). "Mucinous Neoplasms of the Appendix and Peritoneum". Archives of Pathology & Laboratory Medicine 135 (10): 1261–1268.  
  8. ^ Young R (2004). "Pseudomyxoma peritonei and selected other aspects of the spread of appendiceal neoplasms". Semin Diagn Pathol 21 (2): 134–50.  
  9. ^ Jacquemin G, Laloux P (2005). "Pseudomyxoma peritonei: review on a cluster of peritoneal mucinous diseases". Acta Chir Belg 105 (2): 127–33.  
  10. ^ Sugarbaker P (2006). "New standard of care for appendiceal epithelial neoplasms and pseudomyxoma peritonei syndrome?". Lancet Oncol 7 (1): 69–76.  
  11. ^ Muhamod F, et al. (2011). "A new standard of care for the management of peritoneal surface malignancy". Curr Oncol 18 (2): e84–e96.  
  12. ^ Harmon R, Sugarbaker P (2005). "Prognostic indicators in peritoneal carcinomatosis from gastrointestinal cancer". Int Semin Surg Oncol 2 (1): 3.  
  13. ^ Culliford AT, Paty PB (2001). "Surgical debulking and intrapertioneal chemotherapy for established peritoneal metastases from colon and appendix cancer". Ann Surg Oncol 8 (10): 787–95.  
  14. ^ Marmor S1, Portschy PR, Tuttle TM, Virnig BA., J Gastrointest Surg. 2015 Apr;19(4):743-50. doi: 10.1007/s11605-014-2726-7.

References

  • Pseudomyxomasurvivor a global non-profit organization which provides patient advice and support and raises awareness of the condition in the medical community.
  • PMP Research Foundation who fund promising research to find a cure for Pseudomyxoma Peritonei (PMP), Appendix Cancer, and related Peritoneal Surface Malignancies (PSM),

Support organizations relating to this specific condition include:

See also

The first case was described by Carl F. Rokitansky in 1842. Werth in 1884 coined the term pseudomyxoma peritonei, describing it in association with a mucinous ovarian tumor. In 1901 Frankel described the first case associated with a cyst of the appendix.

History

The overall incidence is 0.5 to 1 cases per 100,000 people per year.[14] It is slightly more common in women than men (male:female ratio = 9:11 - needs citation). The median age at presentation is typically about 50 years with a range of 20–25 years.

Epidemiology

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.

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.

Chemotherapy (typically the agent Mitomycin C) may be infused directly into the abdominal cavity after cytoreductive surgery to kill remaining microscopic cancerous tumors and free floating 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, known as early postoperative intraperitoneal chemotherapy (EPIC). EPIC is may be given in multiple cycles for several months after surgery.[13]

Chemotherapy

In debulking, the surgeon attempts to remove as much tumor as possible. CRS or cytoreductive surgery involves surgical removal of the liver), the surgeon strips off the tumor from the surface.[12]

The standard of care for mucinous adenocarcinoma with clinical condition PMP[11] involves cytoreductive surgery (CRS) with hyperthermic intraperitoneal chemotherapy (HIPEC), by surgical oncologists who specialize in treating PMP. Some surgeons also apply early post-operative intraperitonial chemotherapy (EPIC), adjunct to surgical cytoreduction and HIPEC. 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.

Surgical

Treatment is variable, both due to its rarity and to its frequently slow-growing nature. Treatment ranges from watchful waiting to debulking and hyperthermic intraperitoneal chemotherapy (HIPEC, also called intraperitoneal hyperthermic chemotherapy, IPHC) with cytoreductive surgery.[10]

Treatment

This disease is often discovered during surgery for other conditions, e.g., hernia repair, following which an experienced pathologist can confirm the diagnosis. Advanced stages may present as tumors palpable on the abdomen or distention of the belly ("jelly belly" is sometimes used as a slang term for the condition). Due to the rarity of this disease, it is important to obtain an accurate diagnosis so that appropriate treatment may be obtained from a surgical oncologist who specializes in appendix cancer. Diagnostic tests may include CT scans, examination of tissue samples obtained through laparoscopy, and the evaluation of tumor markers. In most cases a colonoscopy is unsuitable as a diagnostic tool because in most cases appendix cancer invades the abdominal cavity but not the colon (however, spread inside the colon is occasionally reported). PET scans may be used to evaluate high-grade mucinous adenocarcinoma, but this test is not reliable for detecting low-grade tumors because those do not take up the dye which shows up on scans. New MRI procedures are being developed for disease monitoring, but standard MRIs are not typically used as a diagnostic tool. Diagnosis is confirmed through pathology.

Diagnosis

The primary tumor appears to arise from the MUC2 expressing goblet cells and most commonly from these cells in the appendix. The K-Ras and p53 genes may be involved in the oncogenesis. It may be diagnosed with a range of conditions. While the majority of these cases are associated with appendiceal carcinomas,[8] other conditions may also be found, including disseminated peritoneal adenomucinosis (DPAM), peritoneal carcinomas, several mucinous tumors (mucinous adenocarcinoma, mucinous cystadenoma, and mucinous cystadenocarcinoma), as well as other disease states.[9] Other primary sites that have been reported include colon, rectum, stomach, gallbladder, bile ducts, small intestine, urinary bladder, lung, breast, fallopian tubes, and the pancreas.

Cause

Signs and symptoms of pseudomyxoma peritonei may include abdominal or pelvic pain and/or bloating, distension, digestive disorders, weight changes, increased girth, and infertility.

Signs and symptoms

The term mucinous adenocarcinoma is used in different contexts depending on the reference material used by the pathologist for disease classification. For example, neoplasms characterized by high-grade features, invasive glands and or signet ring cells, are termed adenocarcinoma in pathology literature.[6] However, some pathologists (e.g., Odze and Goldblum, Surgical Pathology of the GI Tract, Liver, Biliary Tract and Pancreas, 2nd ed.) also use the term mucinous adenocarcinoma when referring to low-grade, well-differentiated tumors lacking high-grade features. Low-grade mucinous adenocarcinoma is used by the American Joint Committee on Cancer and World Health Organization and is nearly or completely synonymous with the DPAM designation.[7] For low-grade mucinous adenocarcinoma, disease may be designated as "benign" because tumors do not invade deeply into tissue and rarely metastasize to parenchyma of organs; this designation may be misleading and confusing to the layperson because pseudomyxoma peritonei is not a harmless condition, fatal if untreated. High-grade or poorly differentiated mucinous adenocarcinoma has a generally poorer prognosis, though surgical treatment with heated intra-peritoneal chemotherapy (HIPEC) is yielding promising outcomes (see surgical treatment).

There is substantial debate regarding histopathologic classification of pseudomyxoma peritonei.[3] In 1995, Ronnett et al.[4] proposed separating pseudomyxoma peritonei cases into two diagnostic categories: adenoma (disseminated peritoneal adenomucinosis, DPAM) or carcinoma (peritoneal mucinous carcinomatosis, PMCA) with a third category reserved for cases with intermediate features. In this classification system, cases of DPAM were characterized by peritoneal lesions composed of abundant extracellular mucin containing scant simple to focally proliferative mucinous epithelium with little cytologic atypia or mitotic activity (in other words, most cells looked fairly normal and there was no evidence of mitosis which would indicate that cells were rapidly dividing), with or without an associated appendiceal mucinous adenoma. Cases of PMCA were characterized by peritoneal lesions composed of more abundant mucinous epithelium with the architectural and cytologic features of carcinoma (irregular cells, evidence that cells were rapidly dividing, and other criteria), with or without an associated primary mucinous adenocarcinoma. Bradley et al. (2007)[5] argued that continued use of non-malignant terms, i.e., adenoma, for those frequent cases with low-grade features (such as DPAM), is misleading because pseudomyxoma peritonei is a disease state that results from invasion of the abdominal cavity by cells with uncontrolled growth. Bradley states that an adenoma, by definition, is a tumor confined to the appendiceal mucosa with absolutely no evidence of invasion beyond the muscularis mucosae.

Classification

Contents

  • Classification 1
  • Signs and symptoms 2
  • Cause 3
  • Diagnosis 4
  • Treatment 5
    • Surgical 5.1
    • Chemotherapy 5.2
  • Epidemiology 6
  • History 7
  • See also 8
  • References 9
  • External links 10

This disease is most commonly caused by an appendiceal primary cancer (cancer of the appendix); mucinous tumors of the ovary have also been implicated, although in most cases ovarian involvement is favored to be a metastasis from an appendiceal or other gastrointestinal source. Disease is typically classified as low- or high-grade (with signet ring cells). When disease presents with low-grade histologic features the cancer rarely spreads through the lymphatic system or through the bloodstream.

, bowel obstruction, or other types of complications. cachexia but the disease is lethal if untreated, with death by [2]

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