A thirty-five year-old woman sought help from several physicians for pain in the right side of her neck. It had started insidiously as a mild discomfort but over a nine-month period had increased to the point where she was taking strong pain medicine several times a day.
Her primary care physician (PCP) had ordered neck X-rays that showed only very early degenerative changes in her spine – typical of what one would see in someone her age. Not knowing what to make of this pain, her PCP referred her to a specialist in pain management. The consultant physician had started her career as a physiatrist – specialist in physical medicine and rehabilitation – but had taken on more and more responsibility in managing chronic pain, as other physicians referred her cases they did not wish to manage because narcotics were involved.
The pain management doctor (PMD) examined the lady and found slight asymmetry in the neck exam: the right side of the neck seemed to bulge a bit. She attributed this to muscle spasm and prescribed a muscle relaxant. This strategy provided some pain relief but the swelling did not improve. She next injected the area with a steroid medication designed to reduce the swelling associated with the muscle spasm. There was no objective benefit and only brief pain relief. After about four months, when the pain had failed to resolve, she ordered at CT scan because the asymmetry persisted and might even have become more prominent.
The radiologist on call the day of the scan looked at the requisition and noticed that the PMD had not specified that the scan be done with intravenous iodinated contrast. When looking at the soft tissues of the neck, iodine contrast can help bring out subtle abnormalities because tumors and areas of inflammation will preferentially take up the iodine, and the area in question will then stand out from surrounding structures. The radiologist called the office of the PMD but failed to reach her because it was her day off.
He proceeded to do the scan without contrast, saw only the vague asymmetry that had been noted on physical exam and in his report recommended that the scan be repeated with iodine contrast if the diagnosis remained in doubt. A copy of the CT report was sent to the PCP as well. Despite an absence of diagnosis, neither the PCP nor the PMD ordered a repeat scan with contrast.
The PMD continued to see the lady for another six months during which time she continued the muscle relaxants and periodic steroid injections. The pain waxed and waned. During this time the neck asymmetry increased to the point where the PMD realized that the lady probably had a mass in her neck. She referred her to an ear nose and throat (ENT) specialist.
The ENT immediately ordered a contrast-enhanced CT, as had been suggested months ago, and saw that there was a cluster of abnormal lymph nodes in the middle of the right neck (see figure at right) and a mass in the thyroid about 3 cm in diameter.
He did a needle biopsy of the lymph nodes and found her to have a rare condition called medullary carcinoma of the thyroid. Based on that diagnosis he performed total removal of her thyroid gland together with the lymph nodes in the right side of the neck. Final pathology revealed medullary carcinoma in the right lobe of the thyroid with metastasis to eleven of the twenty lymph nodes removed.
The Patient’s Outcome
The patient was placed on thyroid hormones to replace what her body no longer made. Since the cells that make this tumor also make a hormone called calcitonin, that hormone was measured every few months as well. If one’s entire thyroid is removed, the calcitonin level in the blood after surgery should fall to zero. Fortunately, one can live comfortably without any calcitonin. Our patient’s level never fell to zero, and starting about six months later her calcitonin level began to rise. Additional scans showed new lymph node enlargement in the upper part of the chest and she underwent a large operation designed to remove all of the lymph nodes in that area. Her calcitonin level fell after the surgery but not to zero and has started to rise again.
More surgery is contemplated, but, at some point, she is likely to die of metastatic medullary carcinoma. There is no medicine such as chemotherapy known to control reliably the spread of this disease.
Our patient filed a lawsuit against the PCP and the PMD. The insurance carrier for the PCP settled out of court for a modest sum and the case is being continued against the PMD. The patient’s attorney recognized that most of the liability rested with the PMD and was willing to settle with the PCP for a small amount of money. From a practical point of view that settlement pays his expenses as the fight against the PMD rages on. I was asked by the PMD’s lawyer to look at this case to gauge his client’s vulnerability.
My role as an expert herein goes to the issue of “causation,” a legal term that means that there is a connection between alleged delay in diagnosis and this lady’s poor prognosis. Put bluntly: was she doomed from the beginning or will the delay cause her to die? My job in essence was to recreate the extent of her disease at the time she started seeing the PMD and thereby calculate her prognosis at that time.
There is a fundamental irony in cancer malpractice litigation. The information I need as an expert to formulate an opinion is denied me by the very negligence in question. The prognosis for patients with medullary carcinoma of the thyroid is largely determined by the number of lymph nodes in the neck involved with tumor. At the time of the first CT scan it would have been nice to know how many nodes were involved. Since the scan was incomplete – no iodine contrast – and no surgery was done we cannot know how many nodes were involved. What we do know is that there were enough nodes full of cancer to cause obvious asymmetry of the neck and pain. It is unusual for patients with medullary carcinoma metastatic to neck nodes to experience pain, so she must have had invasion of the nerves responsible for sensing pain; it is also unusual for neck metastases from medullary carcinoma to be so large as to cause obvious swelling. So I concluded, with ample support in the medical literature, that it was likely that our patient had incurable cancer at the time she first developed neck pain.
This case is still pending in the court system. Numerous depositions of fact witnesses and experts have been taken. No one seriously disputes my conclusions. Nonetheless the facts as they have unfolded are so unfavorable to the actions of the PMD that I will be surprised if this case goes to trial. Even though I have the facts on my side, I frankly cannot imagine a jury agreeing with me that the PMD’s actions did not contribute to a bad outcome. The attorney for the PMD will want to settle out of court; the only obstacle could be how much money is demanded by the plaintiff.
What Lessons Can We Learn From This, Frankly, Egregious Case?
The primary care physician did the right thing by referring the patient to a specialist but unfortunately probably picked the wrong specialty. That can only be concluded with hindsight, however.
In order for the PCP’s attorney to have mounted a vigorous defense on her behalf he would have had to join the chorus of criticism against the PMD. Defense lawyers are averse to criticizing their colleagues’ clients, so this was not considered a viable option.
The pain management doctor was negligent by virtue of refusing to reconsider her original diagnosis of muscle spasm when the patient did not improve quickly and of not ordering a follow-up contrast enhanced CT scan. Her decision to inject the mass of cancerous lymph nodes with steroids likely did not aggravate an already bad situation but makes her look very foolish. Jurors will wince when they find out that the defendant stuck a needle into the tumor and injected it. I wince, even though intellectually I know that by then it didn’t matter what she did.
A Tragedy of Errors
As we have seen in previous cases, not just one error was made. Had the original CT scan been done with contrast, the diagnosis would have been made much sooner. Had the PMD not stuck to her original diagnosis in spite of mounting evidence that it was incorrect the diagnosis would have been obvious much sooner.
What about medullary carcinoma (MCT)? It is a rare condition representing only 4% of all thyroid neoplasms but the mortality rate is much higher than that of conventional thyroid cancer. MCT is in a minority of cases inherited in kindreds harboring a mutation in the RET gene, diagnosable with a blood test, in which many family members develop a variety of tumors of the endocrine system. Unless the affected family knows of this condition and its children are tested before they get cancer, MCT is usually diagnosed late, only after the tumor mass is big enough to feel. When children discover they have the abnormal gene, they undergo prophylactic removal of the thyroid gland. Occasionally a small focus of medullary carcinoma can be found even then, but the cure rate is very high. Overall, however, MCT is a dangerous and often lethal cancer.
This patient was unfortunate to develop a rare serious cancer that caught her physicians off guard. Her outlook was arguably further compromised by failure of her doctors to follow each others’ recommendations and by failure to abandon a program that was obviously not working. I do not believe that her ultimate survival was affected, but that opinion will not assuage her anger nor likely cause a jury to exonerate her doctor.
Although I will probably never meet the PMD I wonder whether this case will be sufficient cause for her to change her practice patterns. One can only hope so.
Dr. James Stark is the founder of StarkOncology, where he practiced Oncology in Hampton Roads for thirty-four years. He is now a health-care consultant specializing in topics such as breast cancer, colon cancer, lung cancer, chemotherapy complications, and failure to screen. In addition to this new venture he continues to serve as Professor of Medicine at Eastern Virginia Medical School.