Gary Mann, MD, FACS, Professor of Oncology in the Department of Surgical Oncology at Roswell Park Comprehensive Cancer Center, recounts an unexpectedly complicated series of surgeries performed on a 27-year-old patient, initially examined for hypercalcemia. The patient ultimately benefitted from the access to a multidisciplinary team of specialists available at Roswell Park, instead of needing to travel to multiple locations for diagnosis and treatment.
The initial surgery found only moderate enlargement of the patient’s parathyroid and, despite the expectation that his thyroid hormone levels would decrease after surgery from their very high levels in the 500 pg/mL range down to a more normal range of around 65 pg/mL, his levels actually increased a little. A lower left gland was removed and the patient was diagnosed with thyroid cancer, found in a node separate from the thyroid gland. However, no additional abnormal glands were found, leading Dr. Mann to order a CT scan of the patient’s chest. During that scan, the patient was found to have bilateral tumors on his kidneys, measuring 4.5 centimeters on the left adrenal gland and 3.5 centimeters on the right adrenal gland. Eventually the patient was diagnosed with hyperparathyroidism, medullary thyroid cancer, bilateral pheochromocytoma and multiple endocrine neoplasia symptom.
Hi, my name is Gary Mann. I'm one of the surgical oncologists at Roswell Park Comprehensive Cancer Center. I have a fairly broad practice. It includes uh colorectal surgery, including a robotic surgery, but I also do a lot of uh endocrine surgery. In fact, I'm the chief of endocrine surgery in the surgical oncology department. I recently in the last two years had an extremely interesting patient referred to me. He's a 27 year old gentleman. He was sent to me with hypercalcemia. Most people who have hypercalcemia in the community have it because of hyperparathyroidism. And his endocrinologist checked a parathyroid hormone level which was very, very high around the 500 range. Normally the upper limit of normal is about 65. Our protocol at Roswell Park is in patients with hyperparathyroidism is to get a high resolution contrast enhanced four D neck CT to look for the parathyroid adenoma so that we can do a more directed exploration. And he had a four XET which showed a parathyroid adenoma at the left upper location, no other nodes or abnormalities of the thyroid gland. And in this circumstance when I, when I have a patient such as this, we take them to surgery for a neck exploration and identification of the adenoma and resection of this, we also check parathyroid hormone levels while the patient is asleep on the table. So we send off samples both before resection of the parathyroid gland and then after resection of the parathyroid gland at 10 and 20 minutes after resection, and we should see a greater than 50% drop in the parathyroid hormone levels. If we take out the appropriate parathyroid gland on the day of surgery, with this gentleman, we took him to the operating room. We explored the left side of the neck. We found the left upper parathyroid gland, which was indeed enlarged but only modestly enlarged. It was not cancerous, not locally invasive, but his intraoperative parathyroid hormone measurements didn't come down at all. In fact, they increased a little bit. So we then changed from a uh directed exploration to a bilateral neck exploration and looked in all the locations of expected parathyroid gland, uh locations I took out a biopsy of what appeared to be a parathyroid gland in the lower left versus potentially a lymph node. And the pathologist told me on frozen section while the patient was asleep that this represented a thyroid cancer. So the patient had thyroid cancer in this nodule separate from the thyroid gland. In my mind, this was a lymph node metastasis from the thyroid cancer. So I went out and spoke to the gentleman's mother while the patient was asleep and I performed a total thyroidectomy because this patient most likely had a previously undiagnosed thyroid cancer. But at no time, did we actually ever find another abnormal parathyroid gland? In any event, the patient recovered very well spent a day in the hospital, no complications of that surgery. So I was wondering, you know, could it be somewhere in the chest or the mediastinum that has an ectopic parathyroid? When we get ac t of the chest, we scan through the entire chest, including the upper abdomen. And while we were doing that, often times you get cuts of the stomach, the liver, the spleen the upper aspects of the kidney. And on top of the kidney, I found the adrenal glands and he had really surprisingly bilateral left and right adrenal tumors. Uh 4.5 centimeters on the left, 3.5 centimeters on the right. So we had hyperparathyroidism, medullary thyroid cancer and bilateral pheochromocytoma, all of which were unknown at the time. And every time we did an evaluation or did something more, we peeled the onion back on this gentleman's multiple endocrine neoplasia syndrome. We sent him to our genetics department who did genetic analysis and in fact, he did have a germline mutation in the re proto oncogene, which is the archetypical genetic mutation in me. Two pheochromocytoma are overactive adrenal gland tumors and can be very, very dangerous because patients can have hypertensive crises have a stroke or a heart attack. So they need to be resected. So we appropriately gave him medication to block the effects of his cat colony access. And I took him to the operating room myself and we did a laparoscopic. So a minimally invasive left adrenalectomy. I decided to do the left side alone because that was the largest size side. And I decided only to do one resection because taking out both adrenal adrenal glands would leave him with hypoadrenalism, which is potentially an extremely dangerous uh physiologic state. So he recovered well from that left adrenalectomy. And then the adrenal gland uh was looked at by the pathologist and they thought this adrenal gland may have a higher likelihood of aggressive biologic behavior. It's very complicated, but he had a score of four. And after discussion, we have an endocrine multidisciplinary conference. Everyone felt like the safest thing for him. Given this concern for a more aggressive behavior of his adrenal gland. For Chrom Cyto was to go ahead and take out the right adrenal glands. So six months later, after appropriate blockade, the patient under went a robotic this time, right, Adrenalectomy and did very well from that, he was counseled as to the requirement for steroid and Manella corticoid supplementation uh which he was placed on about three months after that, we decided we would explore and resect the thymus and this possible mass in the anterior dynam because his calcium levels were still in the 13 to 14 range and his parathyroid normal levels were still 500. And together with one of my um expert thoracic colleagues who's also an expert robotic surgeon, we did a left sided robotic video assisted thyroids copic surgery and resected his thymus. And what turned out to be a 4 g parathyroid gland normal glands or a brown 30 to 50 mg. So his was 4000 mg very, very enlarged and we successfully resected this in a minimum invasive passion. His post-operative course was complicated by hypocalcemia because of his long standing, very significant hyperparathyroidism. He had hungry bone syndrome. He also had some hypoparathyroidism which uh resolved over time and currently, he's doing well free of disease both with his medullary thyroid cancer. His adrenal gland pio Chromos and his hyperparathyroidism which has not recurred. His tumor markers are all normal for his medary thyroid cancer C and calcitonin levels are normal and his calcium levels are normal and his metanephrines which are markers of adrenal medall hyperfunction, ie recurrence of vyto or so. Very, very interesting case originally presented with simple hyperparathyroidism and ended up not needing one operation, but ultimately needing four separate operations to render him free of his potentially life threatening endocrine tumors.
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