THYROID Volume 2, Number 2, 1992 Mary Ann Liebert, Inc., Publishers
Familial
Papillary Carcinoma of the Thyroid DAVID GORSON
ABSTRACT A
38-year-old woman had bilateral papillary thyroid carcinoma. Her brother and mother also had papillary thyroid carcinoma. The literature on familial papillary thyroid carcinoma is reviewed briefly.
INTRODUCTION
FAMILIAL only rarely.
has been reported with this condition is here
PAPILLARY THYROID CARCINOMA
Another
family
described.
CASE REPORT A 38-year-old female went to her family doctor for treatment of an upper respiratory infection. An enlarged thyroid was noted. Thyroid function tests were normal. A thyroid scan revealed bilateral cold nodules. She was referred for evaluation. She had not noted any change in her neck. She denied dyspnea, dysphagia, or change in her voice. There was no history of iodine or kelp ingestion and no history of radiation to the head or the neck. Family history was pertinent for papillary thyroid carcinoma in her brother, who had had a thyroidectomy at age 37 and was well at age 4L Her mother also had had a thyroidectomy in her 30s for papillary thyroid cancer and was now alive and well at age 66. The brother is a physician and reported the pathologic findings on these tumors. Furthermore, none of the affected family members had received radiation to the head or the neck, and the family did not live in proximity to a nuclear processing facility. On examination, the patient had two stony hard nodules, one on the right and one on the left, that moved with swallowing. The gland was about two times enlarged. There was no palpable adenopathy in the neck. The patient had a fine needle aspiration biopsy of each nodule performed, and both returned as "suggestive of papillary carcinoma." The patient had a near total thyroidectomy. The rightside nodule measured 0.8 cm and the left-side nodule measured 3.2 cm. Seven of nine lymph nodes were positive for metastatic
disease. Flow cytometry was done and was diploid. Approximately 6 weeks later, a thyroid scan revealed uptake in the right neck at the superior aspect of the thyroid bed. She was treated with 100 mCi of l3,I. No uptake outside the neck was seen on a body scan after the treatment dose. She was given 0.175 mg of Synthroid daily afterward. A serum thyroglobulin level both before and after the 131I was 5 ng/mL, though antithyroglobulin antibodies were positive at 1:640.
DISCUSSION Familial papillary thyroid carcinoma has been described in association with Gardener's syndrome and familial polyposis coli (1-3), as well as independent of these syndromes (4-10). There may indeed be an overlap between these groups, since four of the parents of the propositi in the report by Stoffer et al. (6) had died of colon carcinoma. Although one group thought the appearance of papillary thyroid carcinoma occurring in two sisters to be coincidental (9), most authors conclude that the entity of familial papillary thyroid carcinoma does exist. The case presented here, in addition to previously reported cases, illustrates the importance of obtaining a family history when examining a patient with a thyroid nodule. When a family history of papillary thyroid carcinoma is obtained, a high index of suspicion for papillary cancer in the nodule being examined must be maintained.
REFERENCES 1. Camiel MC, Mule JE, Alexander LL, Benninghoff DL 1968 Association of thyroid carcinoma with Gardener's syndrome in siblings. N Engl J Med 278:1056-1058.
Department of Medicine, Division of Endocrinology, University of Miami School of Medicine, Aventura, 131
Florida.
GORSON
132
Bussy HJ, Glazer G, Thompson JP 1987 Adenomatous polyposis: An association with carcinoma of the thyroid. Br J Surg
2. Plail RO,
74:377-380. 3. Delamarre J, Capron JP, Armand A, et al 1988 Thyroid carcinoma in two sisters with familial polyposis of the colon. J Clin Gastroenterol 10:659-662. 4. Lote K, Andersen K., Nordal E, Brennhovd IO 1980 Familial occurrence of papillary thyroid carcinoma. Cancer 46:1291-1297. 5. Phade VR, Lawrence WR, Max MH 1981 Familial papillary carcinoma of the thyroid. Arch Surg 116:836-837. 6. Stoffer SS, Van Dyke DL, Bach JV, et al 1986 Familial papillary carcinoma of the thyroid. Am J Med Genet 25:775-782. 7. Fischer DK, Groves MD, Thomas SJ, et al 1989 Papillary carcinoma of the thyroid: Additional evidence in support of a familial component. Cancer Invest 7:323-325.
8. Samaan NA 1989 Papillary carcinoma of the thyroid: Hereditary or radiation induced? Cancer Invest 7:399-400. 9. Szanto J, Gundy C, Toth K, Kasler M 1990 Coincidental papillary carcinoma of the thyroid in two sisters. Oncology 47:92-94. 10. Ozaki O, Kunihiko 1, Kobayashi K, et al 1988 Familial occurrence of differentiated, nonmedullary thyroid carcinoma. World J Surg 12:565-571.
Address reprint requests to: David Gorson, M.D. Department of Medicine University of Miami School of Medicine 21110 Biscayne Blvd, Suite 203 Aventura, FL 33180
Familial
Papillary Carcinoma of the Thyroid DAVID GORSON
ABSTRACT A
38-year-old woman had bilateral papillary thyroid carcinoma. Her brother and mother also had papillary thyroid carcinoma. The literature on familial papillary thyroid carcinoma is reviewed briefly.
INTRODUCTION
FAMILIAL only rarely.
has been reported with this condition is here
PAPILLARY THYROID CARCINOMA
Another
family
described.
CASE REPORT A 38-year-old female went to her family doctor for treatment of an upper respiratory infection. An enlarged thyroid was noted. Thyroid function tests were normal. A thyroid scan revealed bilateral cold nodules. She was referred for evaluation. She had not noted any change in her neck. She denied dyspnea, dysphagia, or change in her voice. There was no history of iodine or kelp ingestion and no history of radiation to the head or the neck. Family history was pertinent for papillary thyroid carcinoma in her brother, who had had a thyroidectomy at age 37 and was well at age 4L Her mother also had had a thyroidectomy in her 30s for papillary thyroid cancer and was now alive and well at age 66. The brother is a physician and reported the pathologic findings on these tumors. Furthermore, none of the affected family members had received radiation to the head or the neck, and the family did not live in proximity to a nuclear processing facility. On examination, the patient had two stony hard nodules, one on the right and one on the left, that moved with swallowing. The gland was about two times enlarged. There was no palpable adenopathy in the neck. The patient had a fine needle aspiration biopsy of each nodule performed, and both returned as "suggestive of papillary carcinoma." The patient had a near total thyroidectomy. The rightside nodule measured 0.8 cm and the left-side nodule measured 3.2 cm. Seven of nine lymph nodes were positive for metastatic
disease. Flow cytometry was done and was diploid. Approximately 6 weeks later, a thyroid scan revealed uptake in the right neck at the superior aspect of the thyroid bed. She was treated with 100 mCi of l3,I. No uptake outside the neck was seen on a body scan after the treatment dose. She was given 0.175 mg of Synthroid daily afterward. A serum thyroglobulin level both before and after the 131I was 5 ng/mL, though antithyroglobulin antibodies were positive at 1:640.
DISCUSSION Familial papillary thyroid carcinoma has been described in association with Gardener's syndrome and familial polyposis coli (1-3), as well as independent of these syndromes (4-10). There may indeed be an overlap between these groups, since four of the parents of the propositi in the report by Stoffer et al. (6) had died of colon carcinoma. Although one group thought the appearance of papillary thyroid carcinoma occurring in two sisters to be coincidental (9), most authors conclude that the entity of familial papillary thyroid carcinoma does exist. The case presented here, in addition to previously reported cases, illustrates the importance of obtaining a family history when examining a patient with a thyroid nodule. When a family history of papillary thyroid carcinoma is obtained, a high index of suspicion for papillary cancer in the nodule being examined must be maintained.
REFERENCES 1. Camiel MC, Mule JE, Alexander LL, Benninghoff DL 1968 Association of thyroid carcinoma with Gardener's syndrome in siblings. N Engl J Med 278:1056-1058.
Department of Medicine, Division of Endocrinology, University of Miami School of Medicine, Aventura, 131
Florida.
GORSON
132
Bussy HJ, Glazer G, Thompson JP 1987 Adenomatous polyposis: An association with carcinoma of the thyroid. Br J Surg
2. Plail RO,
74:377-380. 3. Delamarre J, Capron JP, Armand A, et al 1988 Thyroid carcinoma in two sisters with familial polyposis of the colon. J Clin Gastroenterol 10:659-662. 4. Lote K, Andersen K., Nordal E, Brennhovd IO 1980 Familial occurrence of papillary thyroid carcinoma. Cancer 46:1291-1297. 5. Phade VR, Lawrence WR, Max MH 1981 Familial papillary carcinoma of the thyroid. Arch Surg 116:836-837. 6. Stoffer SS, Van Dyke DL, Bach JV, et al 1986 Familial papillary carcinoma of the thyroid. Am J Med Genet 25:775-782. 7. Fischer DK, Groves MD, Thomas SJ, et al 1989 Papillary carcinoma of the thyroid: Additional evidence in support of a familial component. Cancer Invest 7:323-325.
8. Samaan NA 1989 Papillary carcinoma of the thyroid: Hereditary or radiation induced? Cancer Invest 7:399-400. 9. Szanto J, Gundy C, Toth K, Kasler M 1990 Coincidental papillary carcinoma of the thyroid in two sisters. Oncology 47:92-94. 10. Ozaki O, Kunihiko 1, Kobayashi K, et al 1988 Familial occurrence of differentiated, nonmedullary thyroid carcinoma. World J Surg 12:565-571.
Address reprint requests to: David Gorson, M.D. Department of Medicine University of Miami School of Medicine 21110 Biscayne Blvd, Suite 203 Aventura, FL 33180
