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A simplistic representation of each tumour type for better understanding of the disease and the latest standards in treatment available

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Tumours

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Pseudomyxoma peritonei

Introduction:

Pseudomyxoma peritonei (PMP) is a condition caused by the excessive production of mucus or gelatinous ascites by tumor cells which fills the abdominal and the pelvis. The commonest cause of pseudomyxoma peritonei is primary cancer of appendix. It was thought to be a rare disease but it is a grossly under-diagnosed condition. The actual number of person suffering from this state is alarmingly more than the existing data. Previously, this condition was often misdiagnosed as colonic cancer or ovarian cancer; resulting in insufficient treatment and high mortality rate. PMP has recently received a lot of attention because of advances in medicine and surgical techniques, and the conceptualization of CRS and HIPEC. CRS and HIPEC has greatly improved the outcomes and the quality of life of PMP patients. Research in PMP treatment has paved way for treatment of many other cancers which was initially incurable and life-threatening.
By definition, PMP refers to the radiological apppearance that accompanies the accumalation of mucinous material inside the peritoneal cavity. This mucinous ascitis can be because of low-grade or clinically indolent mucinous adenomas; or highly malignant poorly different mucinous carcinomas, and just the term PMP does not differentiate these vastly different outcomes.
Sometimes neither a primary appendiceal tumour nor a normal appendix is apparent. In these cases it may be that the appendix has ruptured and has been obliterated by the developing fibrosis. DPAM is often referred to as being a 'borderline malignant' condition or a Non-malignant condition with a malignant clinical course. The tumour is not biologically aggressive because it does not metastasize via the lymphatics or blood stream like gastrointestinal adenocarcinomas, however, it is still a fatal process. The space required within the abdomen and pelvis for nutritional function eventually becomes replaced by mucinous tumour.
Most of these tumour cells are surrounded by fluid of varying consistency. Bulky cellular deposits are usually found within the omentum and beneath the right hemidiaphragm. Gravity creates a further accumulation of adenomucinous cells within the pelvis where the peritoneum reflects over the pelvic organs.

Types

Pseudomyxoma Peritonei is generally classified into two types :

  • low-grade mucinous carcinoma peritonei (previously called as diffuse peritoneal adeno-mucinosis or DPAM) – these have a relatively indolent course and have excellent outcomes with CRS and HIPEC

  • high-grade mucinous carcinoma peritonei (previously referred to as peritoneal mucinous carcinomatosis or PMCA) – these are aggressive tumors and although CRS and HIPEC is still the treatment of choice, the results may not be as good as in low-grade mucinous carcinoma peritonei.

Causes:

It is usually associated with cancer of appendix. The primary tumour appears to arise from the MUC2 expressing goblet cells and most commonly from these cells in the appendix. The K-Ras gene may be involved in the oncogenesis.

Signs and symptoms:

Abdominal or pelvic pain, bloating sensation, distension, digestive disorders, weight changes, increased girth and infertility.

Diagnosis:

Diagnostic tests may include CT scans, and the evaluation of tumor markers. PET scans are used for higher-grade cancers. MRI is also helpful. Confirmation of diagnosis is done only through histopathological examination. Sometimes incidental diagnosis happen while performing surgery for other condition like hernia, laparoscopy for infertility, accute appendicitis, etc

Treatment:

The treatment consist of CRS i.e Cytoreductive surgery which involves removal of all visible tumors and affected tissues and organs. It may involve resection of part or parts of intestines, hysterectomy, omentectomy, peritoneal resections, etc. The aim is to have no visible residual tumor in the peritoneal cavity. Performing this surgery safely is a function of adequate training and experience. Studies have shown that the outcomes of centers performing this surgery on a regular basis have better outcomes. It is the followed by heated or hypertermic intra-peritoneal chemotherapy ( HIPEC) which involves washing of peritoneum and abdominal cavity with heated (42 to 43 degree Celsius) chemotherapy drugs like Mitomycin- C, adriamycin or oxaliplatin. This constant washing for about 90 minutes kills all cancer cells that are not visible to eyes. Essentially, although it is a form of chemotherapy, what it essentially does is extends the CRS to a microscopic level.
This combination of CRS and HIPEC is useful in treatment of pseudomyxoma peritonei.

Whom to approach?

Oncosurgeon who has experience in dealing with peritoneal malignancies and trained in advance technology of CRS and HIPEC would be ideal for consultation.
There are many oncosurgeons all around the world who are trained in dealing with such kind of cancer treatment by surgery and chemotherapy.
In India, Dr Sanket Mehta is the pioneer of HIPEC and CRS treatment and has been trained in France and United States. He is specially trained in laproscopical GI and gynaecological and uro onco- surgery . He is affiliated to the American Society of Peritoneal Surface Malignancy ( ASPSM). He has treated many patients with peritoneal surface malignancy from all over the country.

How long is the hospital stay post-surgery ?

When the surgery is successfully completed, the patient is shifted to Intensive Care Unit where the stay is for 3-7 days. The aim is to maintain a high quality monitoring over patient condition ; nutritional status and other parameter. Following the ICU stay, the stay in the hospital may be variable lasting from 5-15 days. The patient is usually discharged once he/she is able to take the required daily caloric intake orally and has no other major symptoms.

What are complications of HIPEC surgery?

Like any other abdominal surgeries, CRS and HIPEC has its own risks and complications. The risk of complications depends on the extent of surgery, the need for GI resections, the baseline general condition of the patient and several other factors. The HIPEC surgery complication include but are not limited to gastro-intestinal fistulas, hemorrhage, pancreatitis, respiratory complications, deep vein thrombosis, neutropenia and risk of infections, etc. Several studies as well as our experience have shown that the benefit afforded by the CRS and HIPEC far outweighs the risk involved. Majority of the patient come back to their pre-operative baseline health and resume work within 2 months of surgery. At the end of 6 months, most patients' quality of life is better than the pre-operative status.

In how many days patient will be back to normal life ?

It usually takes two months for patient to fully normalise from post-operative weakness.

What is the best treatment option for pseudomyxoma peritonei ?

The only effective treatment for pseudomyxoma peritonei is CRS and HIPEC. This procedure removes all the mucin producing cells and affected tissues /organ with a motive to achieve total disease free status. The hot chemotherapy wash given after CRS ensures that not even microscopic cells affected by cancer are left behind. No other treatment options are as effective as HIPEC in reducing the rate of mortality in person suffering from pseudomyxoma peritonei. This treatment is als proven to be beneficial in other forms of cancers like Ca of ovary, colon cancer, primary peritoneal cancer, peritoneal mesotheliomas, endometrial carcinoma recurrence, and certain other rare diseases.

How to know more about pseudomyxoma peritonei survivors?

There is a website www.pmppals.org which is excellent in providing any PMP related queries .This website helps in connecting with other cancer survivors and knowing their experience while they underwent HIPEC and CRS. It helps in sharing awareness and providing information related to best available treatment / surgery and rare forms of cancers. For medical literature, you can go to http://www.ncbi.nlm.nih.gov/pubmed and type pseudomyxoma peritonei in the search field. The most emphatic study establishing CRS and HIPEC as the treatment of choice for pseudomyxoma peritonei was published in Journal of Clinical Oncology (this journal is one of the highest rated oncology journal), and is available on this link. http://jco.ascopubs.org/content/30/20/2449.abstract?ijkey=ec964422dfd926d85a81ed173ff5df213cb8b2cf&keytype2=tf_ipsecsha
You can also get in touch with other patients treated by Dr. Sanket Mehta. Please ask for their contact details.

Is Pseudomyxoma Peritonei curable ?

It was initially considered as not treatable condition but introduction of HIPEC and CRS has been efficient in treating this disease. It has increased years of disease free as well as overall survival. Limited extent pseudomyxoma peritonei have a realisitc chance of cure.

How long does the CRS and HIPEC surgery last ?

CRS and HIPEC usually takes an average of 8-11 hours. Duration of surgery depends on extent of disease.

In which diseases except PMP is HIPEC beneficial ?

HIPEC sugery is beneficial in cases of colorectal cancers, cancer of Ovary, peritoneal mesothelioma, primary peritoneal cancers, selected cases of endometrial and gastric cancers, other rarer cancers like psammocarcinomas, desmoid small round cell tumors, etc.

What is the cost of HIPEC?

The average cost of the treatment is about 5-7 lac rupees. However, it is quite variable and depends on the extent of the disease and the general condition of the patient. The doctor will be able to give you an accurate estimate after examining the patient and going through the investigations.

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