The Great Masquerade

It wasn't pregnancy and it wasn't cancer. What was it?

By Stewart Massad|Monday, July 01, 2002
RELATED TAGS: INFECTIOUS DISEASES

AlmaGuzman had been in Chicago's Cook County Hospital for almost a week.Her children had brought her to the emergency room because her abdomenwas growing, and they thought that at the age of 48 she was too old tobe pregnant.

Her evaluation had been intensive. She was notpregnant. A CT scan had shown that her abdomen was filled with fluid.The list of potential causes for such a condition can be long, but herinternists had ruled out the easy ones: Alma did not have any of themetabolic disorders that cause cirrhosis of the liver; she did not havelong-standing hepatitis, though her blood tests revealed she had beenexposed to hepatitis years before; and her heart, kidneys, thyroid, andblood vessels were quite healthy. Plus, her chest X ray was normal.Scanning through her test results on the hospital computer before goingto see her, I thought everything seemed fine.

But one glanceat her told me otherwise. She looked 58, not 48, her face was pinchedand sallow, and her huge abdomen seemed to spill away from her andcover the mattress. She tried to sit up, but the volume of her bellystopped her. She struggled just to turn to me and shake my hand.

We spoke in Spanish. She was depressed because her doctors believed shehad ovarian cancer. I had been called in because I'm a gynecologiconcologist, trained to examine and diagnose women with pelvic cancers.Alma was trying to cope with the idea she might be dying. Her answersto my questions were terse, toneless, and barely audible. Her breathwas short because of the fluid weighing against her diaphragm. She saidshe had not lost weight, but while her body had grown, the flesh hadfallen away from the rest of her. Her appetite had disappeared overseveral months, and she had cramps after she ate, although she did notvomit. No one in her family had ever had cancer.

My exam wasunrevealing, but even large tumors can remain hidden in a massiveabdomen. Although her lab tests were mostly normal, a tumor marker inher blood, known as CA-125, was almost 400, a high level oftenassociated with ovarian cancer.

Her scans were puzzling.Usually, ovarian cancer begins as a mass in the ovary that sheds cellsinto the abdomen. Fluid accumulates in the abdomen when those cast-offcells choke lymph vessels, preventing normal drainage. But Alma's scanshowed no mass. And she didn't fit the profile: Ovarian cancer is adisease more common in developed countries, and until three years agoAlma had lived in a Sonoran village in Mexico. Most women with ovariancancer have few or no children and thus have ovulated more than womenwho have carried pregnancies. One theory suggests that when eggs burstthrough the ovarian surface month after month, mutations can occur asthe ovary repairs its surface. Alma was married at 16 and had raisedsix children, including the four daughters who hovered around me as Iworked with her. There was another puzzling aspect of her case: alow-grade fever.

As Alma suspected, a diagnosis of advancedovarian cancer is usually hopeless. The disease is especially insidiousbecause symptoms are few and vague until the cancer is advanced. Theabsence of an ovarian mass didn't rule out cancer in this case. Manywomen who present with findings like Alma's have peritoneal cancer, atumor related to ovarian cancer that arises from the abdominal liningadjacent to the ovaries rather than from the lining of the ovariesthemselves. Peritoneal cancer also can produce a rise in CA-125. Theonly certain way to establish the diagnosis is to open the abdomensurgically and remove the ovaries and any visible tumor.

Fewpatients want surgery if they can avoid it. And there was still apossible explanation for her condition that we couldn't rule out yet.

"Have you known anyone with tuberculosis?" I asked Alma.

"¿Como no?"she responded. "In my village, everyone knows somebody withtuberculosis." Her husband had died from it. She had tested positivefor TB exposure years before, and she had even taken medicines for it.

"For how long did you take the medicine?" I asked, knowing that eventhe shortest courses of antituberculosis therapy take months.

"A few weeks," she said, shrugging. "My husband was sick. The childrenwere growing up. There was no money. I felt fine. I've always feltfine, until now."

The residents were intrigued by my suggestion. They drew off quarts of Alma's fluid and sent it for testing. Unfortunately, Mycobacterium tuberculosis,the TB-causing bacterium, takes weeks to grow on special agar plates.And more-sensitive DNA tests for the bacterium take up to two weeks.The old-fashioned microscopic evaluation of the fluid sample failed toturn up either tubercle bacilli or cancer cells. That was not asurprise because both are often too scarce to detect. If Alma had TB,the bacteria could be infecting the peritoneal lining. The resultingirritation would cause fluid to accumulate. Peritoneal tuberculosis canfollow a lung infection, so we tried to collect sputum. But Alma wasn'tcoughing, and even putting a tube into her lung to collect bronchialsecretions didn't turn up any tubercle bacilli. A specimen of urinecentrifuged to concentrate the sediment also proved negative.

Cancer remained the likely diagnosis, so we scheduled Alma for surgery.Her face clouded over as I described the procedure—not only theincision and the organs we might remove but also the substantial riskin a weakened patient. There are waiting lists for all but emergencysurgery at Cook County Hospital, so while we waited for Alma's turn, wetried one last test: a biopsy of the uterine lining. In it, under themicroscope, we saw the classic granulomas—nodules filled with immunecells—that are the hallmark of tuberculosis.

In women, pelvicand peritoneal tuberculosis are uncommon infections. They are thoughtto develop when a TB lung infection allows tubercle bacilli into theblood, and blood-borne bacteria implant in the fallopian tube anduterus. Infertility may be the only symptom, and the infection mayprogress silently for years.

Fortunately, treatment forperitoneal tuberculosis is less radical than ovarian cancer therapy.Unless the diagnosis is in question, opening the abdomen is notrecommended. A peritoneal infection cannot be removed surgically, andmembers of the operating team may become infected by bacilli in fluiddroplets aerosolized during the procedure. Instead, treatment consistsof combinations of antituberculosis drugs for a year or more. Alma'stherapy began immediately, and her swelling slowly diminished. Now,several months later, she can walk again, and her face has filled in.When she meets me in the hallway on her way back to the medicineclinic, she clutches my hands, her face bearing a smile as broad andbeaming as the sun of her homeland.
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