Medullary Thyroid Cancer MCT 101: Why is the Diagnosis Difficult for Community Based physicians and Pathologists?
Four cases illustrate the problem.
The answer is that it is rare, the evidence based system says it is too costly to screen all thyroid nodules for calcitonin ( MCT cancer markers). However, it can look like Hurthle cell or follicular carcinoma to the average pathologist.
Whats the big deal when it will be found at surgery anyway?
Wrong as the first surgery for follicular based thyroid cancer is inadequate for MCT. Total thyroid or partial thyroidectomy is fine for thyroid cancers but grossly inadequate for MCT.
MCT needs total and modified radical neck surgery as the initial treatment due to high incidence of neck lymph node involvement. Screening for MEA syndromes and and pheochromocytoma can save the patient from a hypertensive crisis on the operating table. Being rare is no excuse for missing MCT on the first needle biopsy or even on the on-site adequacy sample.
Examples of missed MCT.
- Case #1 Pathologist at a community hospital called it a Hurthle neoplasm and recommended a lobectomy. At the surgery it was still called Hurthle cell carcinoma. The endocrinologist treated her for 17 years with radioiodine and multiple neck dissections until it was finally diagnosed just before she died. The very first needle biopsy slide from 17 years before was obvious MCT.
- This patient died and her estate collected 1.4 million malpractice judgement.
- The “Salt and pepper” nuclei of MCT and no nucleoli usually seen in Hurthle cell neoplasm was diagnostic of MCT.
- The “tilted fry pan” effect of MCT is shown here. As the yolk moves to the edge of the egg white so does the nuclei in MCT. Also the spindle cells with nuclei in an eccentric location.
- Case#2 Rapid on-site adequacy DifQuik slide on second case was diagnosed with the first eROSE air dried smear.Note the spindle cells and pink granules of calcitonin in the cytoplasm and the eccentric nuclei of MCT.
- The second case was called a follicular thyroid carcinoma and told to have a her thyroid out and Radioiodine. She was smart and asked for an outside opinion. The ultrasound found abnormal lymph nodes that was positive for MCT on biopsy.
- Case #2 because she asked for outside opinion she had the proper total TX and bilateral neck dissection and is alive today.
- Case #3 was just pure malpractice as failure to diagnose this serious cancer in a timely manner to protect her life. 2012 to 2018 she was called a simple benign nodular goiter as her nodule grew from 1.5 she never was sent for a biopsy until it was very large (5 CM ). She came for an opinion about alternative to surgery for what she thought benign nodule. The biopsy was not benign but another MCT. Her calcitonin was 5000 and CEA 150. Molecular markers showed expression on microRNA associated with MCT. The delay in initial diagnosis could have a negative effect on her survival as it haf spread to her neck lymph nodes. She was referred to a thyroid cancer surgeon, and to a malpractice lawyer. Classic MCT cells seen on first FNA pap smears from 2012.
Case 4. This is a case of failure to take a biopsy serious with atypical cells. The pathologists missed to classic cell pattern of MCT until the last biopsy .The endocrinologist did not folow up on multiple atypical inconclusive biopsies with a collection of molecular markers or even sending the slides for outside opinion.A positive calcitonin stain on the last biopsy and blood calcitonin was elevated.
51 Y/O F followed for 4 years by an endocrinologist with a palpable 1.5 cm nodule with normal TFT’s. 3 biopsies over 3 years were called atypical inconclusive. No molecular markers were ever obtained from the nodule. She told it is “most likely benign but even if thyroid cancer you would live forever”. The fourth biopsy again had atypical cells but the pathologist suggested it could be MCT and did calcitonin stain on the cells and found it was not benign or even thyroid cancer but the more dangerous MCT cancer. The delay of 4 years caused her to develop metastatic MCT to 2/15 lymph nodes at surgery.The first FNA had classic MCT cells missed by the pathologist fours earlier.
- ALWAYS GET AN OUTSIDE OPINION BEFORE SURGERY OR RADIOIODINE FOR THYROID CANCER AS THEY MAY BE MISSING MCT and GIVING YOU THE WRONG TREATMENT FOR REGULAR THYROID CANCER. Also possible surgery hypertensive crisis from undiagnosed pheochromocytoma.
- 310-393-8860 or [email protected] for details.
- Richard Guttler MD,FACE,ECNU
- Clinical Thyroiologist