The PJS/Thyroid Connection
This report is on the PJS/thyroid connection. For new readers, I've written several pieces on PJS from a patient's view. Some topics include: sex cord tumors, bowel preparations, a review of the article Very High Risk of Cancer in Familial PJS7 etc. Please remember these pieces are not medical advice, but reviews of the literature that you can take to your doctor. Some of you may want to have your thyroids screened, others may have thyroid troubles that weren't connected to PJS by doctors.
This topic was introduced last year by two of our support group members who visited Dr. Stratakis at the NIH in Bethesda, Maryland. Dr. Stratakis is an endocrinologist who works primarily on Carney Complex. His study1 is open to people with PJS. One member wrote that her thyroid tumors were found during her study participation. Another member wrote to us on September 4, 2000:
"The doctor gave me a couple of his articles. His latest, from Frontiers in Bioscience, discusses the overlap between disorders associated with multiple tumors and pigmentation defects. Among the non- GI neoplasms associated with PJS, endocrine tumors including thyroid cancer and ovarian and Sertoli cell tumors are the most frequent. My surgeon in RI was unaware of any thyroid association with PJS. When I needed bloodwork for my surgery in May, I asked to get my thyroid level checked. All came back normal. But, Dr. Stratakis told me that it is not sufficient to just get bloodwork. You also need a thyroid ultrasound. He said that there is an especially aggressive form of thyroid cancer associated with PJS."
The full article from Frontiers in Bioscience is available online2.
The news of potential thyroid cancer and screening for people with PJS was particularly startling since none of the screening guidelines from other doctors include the thyroid.3 I read Dr. Stratakis' article which included the following quote: "Among the nongastrointestinal neoplasms associated with PJS, endocrine tumors including thyroid cancer and ovarian and Sertoli cell tumors are the most frequent." He referenced three articles4-6, but when I read the articles, I found only one mention of thyroid cancer or tumors. I wrote to Dr. Stratakis requesting more information, but he didn't respond.
Dr. Giardiello in his article, Very High Risk of Cancer In Familial PJS7, showed no increased risk of thyroid cancer in PJS and doesn't even include it in his tables. I have to wonder what Dr. Stratakis knows that we don't know. If he sees an increased risk for thyroid cancer, it might be as a result of his studies or one-to-one communications with other doctors. Now that I have a large collection of PJS articles, I combed them for reports of thyroid tumors, malignant & benign, in people with PJS. You will see from my table, that there are reports of thyroid tumors in PJS folks.
|
Author (publication date) |
Gender, Age | Diagnosis |
| Spigelman (1989)6 | Female, 30 | Thyroid cancer |
| Burdick & Prior (1981)8 | Female, 61 | Thyroid colloid nodule & Hashimoto's thyroiditis |
| Yamamoto M (1992)9 | Female, 29 | Thyroid papillary adenocarcinoma |
| Reed MW (1990)10 | n/a | Papillary carcinoma of the thyroid |
| Boardman LA (1998)11 | Age 33 | Thyroid cancer |
| Srivatsa, PJ (1994)12 | Female, 91 | Grade 1 metastatic papillary cystic adenocarinoma of thyroid with squamous metaplasia 2 months after first operation (patient was 91 years old & in good health at time of article) |
| Srivatsa, PJ (1994)12 | Female, 21 | Graves' disease, subtotal thyroidectomy |
| Srivatsa, PJ (1994)12 | Female, 32 | Colloid adenoma of thyroid with small area of papillary carcinoma |
| Dormandy, TL (1957)13 | Female, 60 | Nodular goiter |
| Dormandy, TL (1957)13 | Male, 52 | Nodular goiter |
The most common type of thyroid cancer in PJS folks and the general population is papillary carcinoma. A search at Cancer Net revealed that thyroid papillary adenocarcinoma is a differentiated cancer that is highly treatable and curable.14 So Dr. Stratakis' comment on an aggressive thyroid cancer associated with PJS isn't substantiated from my readings of the literature.
Though I did find cases of PJS folks with thyroid problems, but I have no idea if these findings are significant. Since thyroid problems are fairly common in the general population, there may not be a connection to PJS. Hopefully we can get response from medical folks. I will also forward this to Dr. Stratakis and Dr. Giardiello, asking for their opinions.
If you or someone you know has thyroid problems that may or may not be connected to PJS, it will be interesting to hear your stories. I wish my findings were clearer, but maybe a response will come from more knowledgable doctors about the PJS/thyroid connection.
© 2004 by Stephanie Sugars, all rights reserved, used by permission. No part of this article may be reproduced in any form without written permission from the author.
Contact Stephanie Sugars at PJ4Steph@aol.com
Update: 10.May'04