Thyroid Papillary Adenocarcinoma in a Woman with Peutz-Jeghers Syndrome
We present a rare case of Peutz-Jeghers (P-J) syndrome in a 29-year-old woman who developed adenocarcinoma of the thyroid and polyps in the gastrointestinal tract. Polyps removed from the stomach, colon and ileum showed features typical of hamartomas. Histological examination indicated that the tumo...
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Published in | Internal Medicine Vol. 31; no. 9; pp. 1117 - 1119 |
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Main Authors | , , , , , |
Format | Journal Article |
Language | English |
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The Japanese Society of Internal Medicine
1992
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Abstract | We present a rare case of Peutz-Jeghers (P-J) syndrome in a 29-year-old woman who developed adenocarcinoma of the thyroid and polyps in the gastrointestinal tract. Polyps removed from the stomach, colon and ileum showed features typical of hamartomas. Histological examination indicated that the tumor resected from the right lobe of the thyroid was a papillary adenocarcinoma. This appears to be the first precise report of a P-J syndrome associated with thyroid carcinoma. This case adds an example of predisposition of P-J syndrome to extraintestinal malignancy and indicates the importance of long-term observation of malignancy. (Internal Medicine 31 : 1117-1119, 1992) |
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AbstractList | We present a rare case of Peutz-Jeghers(P-J) syndrome in a 29-year-old woman who developed adenocarcinoma of the thyroid and polyps in the gastrointestinal tract. Polyps removed from the stomach, colon and ileum showed features typical of hamartomas. Histological examination indicated that the tumor resected from the right lobe of the thyroid was a papillary adenocarcinoma. This appears to be the first precise report of a P-J syndrome associated with thyroid carcinoma. This case adds an example of predisposition of P-J syndrome to extra-intestinal malignancy and indicates the importance of long-term observation of malignancy. We present a rare case of Peutz-Jeghers (P-J) syndrome in a 29-year-old woman who developed adenocarcinoma of the thyroid and polyps in the gastrointestinal tract. Polyps removed from the stomach, colon and ileum showed features typical of hamartomas. Histological examination indicated that the tumor resected from the right lobe of the thyroid was a papillary adenocarcinoma. This appears to be the first precise report of a P-J syndrome associated with thyroid carcinoma. This case adds an example of predisposition of P-J syndrome to extraintestinal malignancy and indicates the importance of long-term observation of malignancy. (Internal Medicine 31 : 1117-1119, 1992) |
Author | ICHIKAWA, Masafumi HOSHINO, Hiroshi HAYAKAWA, Seijun YAMAMOTO, Masahiro ONIZUKA, Toshio KAWAKUBO, Akitoshi |
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References | 3) Dormandy TL. Gastrointestinal polyposis with mucocutaneous pigmentation (Peutz-Jeghers syndrome). N Engl J Med 256 : 1093, 1141, 1186, 1957. 4) Spiegelman AD, Murday V, Phillips RKS. Cancer and the Peutz-Jeghers syndrome. Gut 30 : 1588, 1989. 28) Burt RW, Bishop, HT, Lynch P. Risk and surveillance of individuals with heritable factors for colorectal cancer. Bull WHO 68 : 655, 1990. 14) Foley TR, McGarrity TJ, Abt AB. Peutz-Jeghers syndrome : a clinicopathologic survey of the "Harrisburg family" with a 49-year follow-up. Gastroenterology 95 : 1535, 1988. 23) Soeters R, Tiltman A, Learmonth G, et al. Peutz-Jeghers syndrome in association with adenoma malignum (minimal deviation adenocarcinoma) of the cervix. Case report. Br J Obstet Gynaecol 96 : 1101, 1989. 1) Bailey D, Chir M. Polyposis of gastro-intestinal tract : the Peutz syndrome. Br Med J 24 : 433, 1957. 6) Jeghers H, McCusick VA, Katz KH. Generalized intestinal polyposis and melanin spots of the oral mucosa, lips and digits : a syndrome of diagnostic significance. N Engl J Med 241 : 993, 1949. 10) Perzin KH, Bridge MF. Adenomatous and carcinomatous changes in hamartomatous polyps of the small intestine (Peutz-Jeghers syndrome) : report of a case and review of the literature. Cancer 49 : 971, 1982. 16) Trau H, Schewach-Millet M, Fisher B, Tsur H. Peutz-Jeghers syndrome and bilateral breast carcinoma. Cancer 50 : 788, 1982. 7) Thatcher BS, May ES, Taxier MS, Bonta JA, Murthy L. Pancreatic adenocarcinoma in a patient with Peutz-Jeghers syndrome. A case report and literature review. JAMA 81 : 594, 1986. 24) MacGowanL, Young RH, Scully RE. Peutz-Jeghers syndrome with "Adenoma Malignum" of the cervix. A report of two cases. Gynecol Oncol 10 : 125, 1980. 2) Utsunomiya J, Gocho H, Miyanaga T, Hamaguchi A, Kashimure A. Peutz-Jeghers syndrome : its natural course and management. Johns Hopkins Med J 136 : 71, 1975. 5) Peutz JLA. Very remarkable case of familial polyposis of the membrane of the intestinal tract and nasopharynx accompanied by peculiar pigmentations of the skin and mucous membrane. Ned Maandschr Geneeskd 10 : 134, 1921. 9) Giardiello FM, Welsh SB, Hamilton SR, et al. Increased risk of cancer in the Peutz-Jeghers syndrome. N Engl J Med 316 : 1511, 1987. 20) Kese VG. Adenocarcinoma cervicis uteri bei einer 18 jahrigen Frau mit Peutz-Jeghers Syndrome. Zentral Gynaekol 91 : 215, 1969 (in German). 25) Bodmar WF, Bailey CJ, Bodmer J, et al. Localization of the gene for familial adenomatous polyposis on chromosome 5. Nature 328 : 1411, 1987. 15) Bowlby LS. Pancreatic adenocarcinoma in an adolescent male with Peutz-Jeghers syndrome. Hum Pathol 17 : 97, 1986. 17) Riley E, Swift M. A family with Peutz-Jeghers syndrome and bilateral breast cancer. Cancer 46 : 815, 1980. 18) Gloor E. Un cas de syndrome de Peutz-Jeghers associe un carcinome mammary bilatéral, à un adénocarcinome ducol untérin et à des tumeurs des cordons sexuels à tubules annelés bilatérales dans les ovaries. Schweiz Med Wochenschr 108 : 717, 1978 (in French). 26) Olschwang S, Weiffenbach B, Laurent-Puig P, et al. Genetic characterization of the APC locus involved in familial adenomatous polyposis. Gastroenterology 101 : 154, 1991. 8) Tavar JA, Eizaguirre I, Albert A, Jimenez J. Peutz-Jeghers syndrome in children : report of two cases and review of the literature. J Pediatr Surg 18 : 1, 1983. 22) Siverberg SG, Hurt WG. Minimal deviation adenocarcinoma ("adenoma malignum") of the cervix : a reappraisal. Am J Obstet Gynecol 121 : 971, 1975. 21) Costa J. Peutz-Jeghers syndrome, case presentation. Obstet Gynecol 50 (suppl) : 15s, 1977. 11) McGowan L, Young RH, Scully RE. Peutz-Jeghers syndrome with "Adenoma Malignum" of the cervix. Gynecol Oncol 10 : 125, 1980. 27) Miyaki M, Seki M, Okamoto M, et al. Genetic changes and histopathological types in colorectal tumors from patients with familial adenomatous polyposis. Cancer Res 50 : 7166, 1990. 12) Bolwell JS, James PD. Peutz-Jeghers syndrome with pseudoinvasion of hamartomatous polyps and multiple epithelial neoplasm. Histopathology 3 : 39, 1979. 13) Wada K, Tanaka M, Yamaguchi K, Wada K. Carcinoma and polyps of the gallbladder associated with Peutz-Jeghers syndrome. Dig Dis Sci 32 : 943, 1987. 19) Dozois RR, Judd ES, Dahlin DC, et al. The Peutz-Jeghers syndrome. Is there a predisposition to the development of intestinal malignancy? Arch Surg 98 : 509, 1969. |
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SubjectTerms | Adenocarcinoma, Papillary Adult autosomal dominant inheritance extraintestinal malignancy Female gastrointestinal polyps Hamartoma Humans Neoplasms, Multiple Primary Peutz-Jeghers Syndrome Thyroid Neoplasms |
Title | Thyroid Papillary Adenocarcinoma in a Woman with Peutz-Jeghers Syndrome |
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