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Pseudomyxoma peritonei

Pseudomyxoma peritonei (PMP, sometimes informally known as "jelly belly") is a very rare form of cancer, commonly known as "jelly belly" due to its production of mucus in the abdominal cavity. The tumor is not harmful by itself, but it has no place to go inside the abdominal cavity. more...

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If left untreated, it will eventually build up to the point where it compresses vital structures: the colon, the liver, kidneys, etc.

Unlike most cancers, PMP does not spread through the lymphatic system or through the bloodstream.

It is believed that most PMP starts as cancer of the appendix; the Helicobacter pylori bacterium also seems to be implicated.

Diagnosis

Because it is so rare, it is frequently either not diagnosed or misdiagnosed. Usually the only symptom is of the belly getting bigger, which doctors and patients alike can easily assume is from the patient getting fatter.

Frequently, PMP is diagnosed after the patient is operated on or gets a CT scan for some other problem. On a CT scan, the mucous shows up as a light grey area.

The mucous normally has the consistency and appearance of orange gelatin, but can cement to become much harder.

Treatment

Because PMP is very rare, there is variation in the treatment.

As the tumor grows very slowly, sometimes people choose to just watch and wait.

The most common treatments are debulking and cytoreductive surgery. With debulking, the surgeon attempts to remove as much tumor as possible.

With cytoreductive surgery, the surgeon takes out the peritoneum and any organs that appear to have tumor on them. If the organ is important, only part of it might be removed. 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 can not be removed safely (like the liver), the surgeon strips off the tumor from the surface.

It is very important to remove or kill every last cancer cell because the cancer cells reproduce quickly on scar tissue, and there is lots of scar tissue after surgery.

To kill the last few cells, chemotherapy drugs are put directly into the abdominal cavity. Either the drugs are swished around by hand 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.

Cytoreductive surgery usually takes between ten and thirteen hours, and is sometimes referred to by patients as MOAS (Mother Of All Surgeries) or as the Sugarbaker Procedure (after the doctor who pioneered this form of treatment).

Even with the most aggressive heated chemotherapy treatment, it is very common to have the tumor come back, so further surgeries are frequently needed. The patients usually get frequent CT scans for a while in order to spot any regrowth of the tumor.

Read more at Wikipedia.org


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Borderline ovarian tumors can mimic more aggressive malignancies - Pathology Report Critical
From OB/GYN News, 5/1/02 by Kate Johnson

LAKE TAHOE, NEV. -- Borderline ovarian tumors often present very similarly to frank ovarian cancer, but their treatment is more straightforward and survival rates are substantially better, according to one expert.

Symptoms and imaging may not give many clues to help differentiate these two kinds of tumors, Dr. Gary Leiserowitz said at an obstetrics and gynecology conference sponsored by the University of California, Davis.

In either case, ultrasound or CT scans will appear very abnormal, and the mass will often measure greater than 5-8 cm and demonstrate complex morphologic characteristics.

"It's not going to look reassuring, but it's a minority of these patients that actually have a malignancy. A high percentage of [reproductive-age women] will have something like endometriosis or a dermoid cyst, and then a certain proportion will have borderline tumors, said Dr. Leiserowitz, chief of gynecologic oncology at the University of California, Davis.

A patient with a borderline ovarian tumor may have pain, bloating, or have just a pressure sensation. There is a wide range of symptoms, and they are not remarkably different than what's associated with frank ovarian malignancies, he said.

Surgery is the primary treatment for borderline ovarian tumors, also known as ovarian tumors of low malignant potential. Although these tumors are not cancerous and survival rates are good, borderline ovarian tumors may occasionally recur, and rarely cause death.

Nearly all patients with early-stage borderline tumors survive, but there can be complications associated with advanced-stage tumors. "Deaths associated with these tumors are usually due to ascites or pseudomyxoma peritonei, or the tumor itself may cause a bowel obstruction or put pressure on a vital structure," he told this newspaper.

There is also an association between borderline tumors and the development of frank cancer. Although this is sometimes referred to as a "malignant transformation," it may not be a literal transformation.

"The literature suggests that this is a de novo cancer. It is not literally a borderline tumor that has somehow degenerated into cancer. We think that whatever puts a woman at risk for a borderline tumor also puts her at risk for an ovarian cancer," he said.

Ob.gyns. will find it difficult to differentiate between cancerous and borderline tumors until they see a pathology report. But this vital distinction, which should be made by an experienced pathologist, profoundly affects the course of treatment.

Whereas aggressive surgery and chemotherapy are considered standard for ovarian cancer, they often are not indicated for borderline tumors and especially not for early-stage disease.

Three-quarters of borderline tumors fall into the stage I classification, meaning they are associated with nearly 100% survival. These tumors are usually confined to one ovary, and although they can be large and grow for a long time, they metastasize less often than ovarian cancer.

More advanced stage borderline tumors can involve both ovaries and/or extraovarian tissue. The prognosis is not as good for these tumors due to the risks of recurrence. Nevertheless, even the majority of these patients survive, in contrast to those with invasive epithelial ovarian cancer.

"Maybe 25% of patients have extraovarian disease, and of that small group, no more than 2% have malignant transformation over a period of 10-20 years," he said.

For early-stage borderline ovarian tumors, conservative therapy such as a unilateral salpingo-oophorectomy or an ovarian cystectomy is appropriate. In either case, peritoneal washings and targeted peritoneal biopsies should be performed.

The risk of recurrence is about 20% if an ovarian cystectomy is performed, but almost all recurrences can be salvaged with a repeat surgery. "I prefer to treat conservatively and spare the ovary even if there is a risk of having to do a second surgery," he said.

Definitive therapy, which is indicated if childbearing is complete or in the presence of extraovarian disease, is a total abdominal hysterectomy; bilateral salpingo-oophorectomy and aggressive cytoreduction, with peritoneal washings, biopsies, and node sampling.

There is little benefit for adjuvant chemotherapy; because it has not been shown to increase the time to recurrence or survival rates, he said. In addition, some of the deaths associated with advanced-stage borderline tumors are actually due to chemotherapy complications.

COPYRIGHT 2002 International Medical News Group
COPYRIGHT 2002 Gale Group

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