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Peutz-Jeghers Syndrome - JMI ArticlesArticles in our collection about Peutz-Jeghers Syndrome and Cancer/Tumors/CarcinomaTitle: Celiac disease and other precursors to small-bowel malignancy Authors: Green PH;Jabri B; Journal: Gastroenterol Clin North Am Date: 2002 Jun Abstract: Small-intestinal malignancies are rare. Major risk factors for the development of these malignancies include celiac disease, which predisposes to both carcinoma and lymphoma. Crohn's disease patients have an increased risk of the development of adenocarcinoma, as do the inherited polyposis syndromes, FAP, and Peutz-Jehgers syndrome. Each of these conditions provides unique models for the development of malignancy Link: Click for Pubmed record Title: Peutz-Jeghers syndrome associated with adenocarcinoma and protein-losing enteropathy Authors: Ianniccillo H;Varsky C;Rizzolo M;Dutack A;Frider N;Villafane V; Journal: Gastrointest Endosc Date: 2000 Oct Abstract: Link: Click for Pubmed record Title: Extraintestinal polyps in Peutz-Jeghers syndrome: presentation of four cases and review of the literature. Deutsche Peutz-Jeghers-Studiengruppe Authors: Vogel T;Schumacher V;Saleh A;Trojan J;Moslein G; Journal: Int J Colorectal Dis Date: 2000 Apr Abstract: Peutz-Jeghers syndrome (PJS) is a rare hereditary disorder characterized by hamartomatous polyps in the gastrointestinal tract and typical pigment lesions. Extraintestinal polyps have rarely been reported. Possible sites include the respiratory tract, urogenital tract, and gallbladder. We here describe four cases of extraintestinal polyps in PJS patients and review the literature on the need for operative therapy of extraintestinal polyps in PJS. Three nonrelated patients were examined who had PJS and polyps in the gallbladder; the fourth patient had PJS and recurrent choanal polyps. Surgery has so far been performed only for symptomatic polyps: one laparoscopic cholecystectomy and removal of the choanal polyps for recurrent infections of the respiratory tract. The remaining two patients reported no symptoms from the extraintestinal polyps. No malignant transformation was found in these patients, nor has such been reported in the literature on PJS. The frequent observation of this manifestation in our patients raises the question of clinical management: Is prophylactic surgery indicated? Since malignant transformation of PJS polyps in the intestine is extremely rare we see no reason for operative therapy as long as the polyps are small and asymptomatic. Regular sonographic controls are recommended since the risk of malignant transformation cannot be ruled out at present Link: Click for Pubmed record Title: Antigens recognized by autologous antibody in patients with renal-cell carcinoma Authors: Scanlan MJ;Gordan JD;Williamson B;Stockert E;Bander NH;Jongeneel V;Gure AO;Jager D;Jager E;Knuth A;Chen YT;Old LJ; Journal: Int J Cancer Date: 1999 Nov 12 Abstract: The screening of cDNA expression libraries derived from human tumors with autologous antibody (SEREX) is a powerful method for defining the structure of tumor antigens recognized by the humoral immune system. Sixty-five distinct antigens (NY-REN-1 to NY-REN-65) reactive with autologous IgG were identified by SEREX analysis of 4 renal cancer patients and were characterized in terms of cDNA sequence, mRNA expression pattern, and reactivity with allogeneic sera. REN-9, -10, -19, and -26 have a known association with human cancer. REN-9 (LUCA-15) and REN-10 (gene 21) map to the small cell lung cancer tumor suppressor gene locus on chromosome 3p21.3. REN-19 is equivalent to LKB1/STK11, a gene that is defective in Peutz-Jeghers syndrome and cancer. REN-26 is encoded by the bcr gene involved in the [t(9:22)] bcr/abl translocation. Genes encoding 3 of the antigens in the series showed differential mRNA expression; REN-3 displays a pattern of tissue-specific isoforms, and REN-21 and REN-43 are expressed at a high level in testis in comparison to 15 other normal tissues. The other 62 antigens were broadly expressed in normal tissues. With regard to immunogenicity, 20 of the 65 antigens reacted only with autologous sera. Thirty-three antigens reacted with sera from normal donors, indicating that their immunogenicity is not restricted to cancer. The remaining 12 antigens reacted with sera from 5-25% of the cancer patients but not with sera from normal donors. Seventy percent of the renal cancer patients had antibodies directed against one or more of these 12 antigens. Our results demonstrate the potential of the SEREX approach for the analysis of the humoral immune response against human cancer Link: Click for Pubmed record Title: The hamartoma-adenoma-carcinoma sequence Authors: Bosman FT; Journal: J Pathol Date: 1999 May Abstract: Carcinomas develop, although at low frequency, in hamartomatous polyps in the Peutz-Jeghers' syndrome. With the identification of at least one of the genes involved (LKB1/STK11), attempts can be made to unravel the molecular events responsible for this hamartoma-adenoma-carcinoma sequence. Recent data indicate that this sequence is real, that the molecular pathways involved are different from those in other hereditary colorectal cancer syndromes, and that, possibly in contrast to the hamartomatous polyps in the juvenile polyposis syndrome, the genetic abnormalities occur in the epithelial compartment of the lesions Link: Click for Pubmed record Title: Intracellular distribution of beta-catenin in colorectal adenomas, carcinomas and Peutz-Jeghers polyps Authors: Herter P;Kuhnen C;Muller KM;Wittinghofer A;Muller O; Journal: J Cancer Res Clin Oncol Date: 1999 Abstract: The interaction of the adenomatous polyposis coli (APC) tumor-suppressor protein and the intracellular cell-adhesion protein beta-catenin is crucial for the development of colorectal tumors. Since functional nuclear complexes of beta-catenin with transcription factors have been identified recently, the knowledge of level and distribution of beta-catenin in sporadic colorectal tumors will give important insights into the intracellular mechanism of sporadic colorectal tumor initiation and progression. In contrast to the familiar adenomatous polyposis syndrome and to the majority of sporadic colorectal tumors, Peutz-Jeghers (PJ) syndrome is not caused by mutations in the APC gene. Since PJ syndrome is an inherited disease with an increased risk for gastrointestinal adenocarcinoma, whether beta-catenin plays a similarly important role for the development of PJ polyps should be further investigated. For these reasons we analyzed the distribution of beta-catenin in a total of 60 sporadic colorectal tumors at different stages of progression and in 6 PJ polyps. In addition to the localization at the cell-to-cell border membranes, fluorescence immunohistochemistry revealed a nuclear accumulation of beta-catenin in single tumor cells of 10/14 small adenomas with mild dysplasia and in 14/16 adenomas with moderate dysplasia. Further tumor progression is accompanied by an expansion of cells with increased level of nuclear and cytoplasmic beta-catenin. These cells were observed in 5/16 adenomas with moderate dysplasia and in 15/15 adenomas with severe dysplasia. In all adenocarcinomas investigated, as well as in the corresponding lymph node metastases, a sub-population of tumor cells exhibited a remarkably increased level of beta-catenin within the entire cytoplasm and the nucleus. In contrast to the situation in sporadic colorectal tumors, nuclear and cytoplasmic beta-catenin was not increased in PJ polyps. These results point to an extensive redistribution of beta-catenin, which starts early in colorectal tumorigenesis. The nuclear accumulation in single cells of small adenomas can be considered as the first visible sign of the loss of APC function. Thus the immunohistochemical detection of beta-catenin distribution could serve as a criterion for estimating the malignant potential in the clinico-pathological evaluation of colon tumors during their early progression Link: Click for Pubmed record Title: Rectal carcinoid tumor associated with the Peutz-Jeghers syndrome Authors: Wada K;Asoh T;Imamura T;Wada K;Tanaka N;Yamaguchi K;Tanaka M; Journal: J Gastroenterol Date: 1998 Oct Abstract: A 16-year-old man who had been diagnosed with the Peutz-Jeghers syndrome at the age of 8 years presented with crampy abdominal pain. Thorough examinations revealed a large jejunal polyp causing intussusception, as well as multiple polyps in the small and large intestines. Preoperative proctoscopy demonstrated the coexistence of a submucosal tumor in the rectum. Proctoscopic mucosal resection was performed and histological and immunohistochemical examinations led to a diagnosis of carcinoid tumor. Additional transanal resection of the rectal wall showed no residual tumor and the patient has been well for 2 years to date. Although malignant tumors are increasingly reported in association with the Peutz-Jeghers syndrome, to our knowledge, there have been no previous reports of such an association in the English-language and Japanese literature Link: Click for Pubmed record Title: Cancer in Peutz-Jeghers syndrome Authors: Hizawa K;Iida M;Matsumoto T;Kohrogi N;Kinoshita H;Yao T;Fujishima M; Journal: Cancer Date: 1993 Nov 1 Abstract: BACKGROUND. Peutz-Jeghers (P-J) syndrome has been found to be associated with an increased risk of malignant neoplasia. METHODS. The authors reviewed the clinical courses of eight patients with P-J syndrome (four male and four female patients, ranging in age from 8 to 55 years), who had been followed up for as long as 12 years. The diagnosis of this syndrome was based on the evidence of characteristic mucocutaneous pigmentations and gastrointestinal hamartomatous polyposis. RESULTS. Four cases of malignant neoplasm among the eight patients were found. In a 25-year-old woman, an extremely well-differentiated adenocarcinoma of the uterus cervix was found at the initial examination. A gastric cancer in a 32-year-old woman, duodenal cancers in a 43-year-old man, and a pancreatic cancer in a 60-year-old man also were identified 12, 10, and 5 years after the time of initial examinations, respectively. Two of these patients died of the effects of the tumor soon after. CONCLUSION. The authors' experience confirms a veritable malignant potency in P-J syndrome and suggests that an intensive follow-up of gastrointestinal and extra-gastrointestinal sites is needed in patients with this syndrome Link: Click for Pubmed record Title: Association of congenital hypertrophy of the retinal pigment epithelium with familial adenomatous polyposis Authors: Iwama T;Mishima Y;Okamoto N;Inoue J; Journal: Br J Surg Date: 1990 Mar Abstract: Ophthalmological examinations were performed in 49 people (43 patients) from 24 families affected by familial adenomatous polyposis (FAP). No features of congenital hypertrophy of the retinal pigmented epithelium (CHRPE) were noted in six first-degree relatives without colonic polyps. Patients with Gardner's syndrome exhibited CHRPE more frequently than those without exostosis or desmoid tumour (91 per cent versus 45 per cent). In addition the prevalence of CHRPE was slightly higher in the patients with gastric or duodenal polyps (68 per cent compared with 50 per cent) in those who had a normal gastroduodenal examination. There were 11 families in which two or more patients underwent ophthalmological examinations. In five of these 11 families, all 12 patients were negative for CHRPE. In four of the remaining six families, all 11 patients had CHRPE. CHRPE is one of the pleiotropy of FAP and ophthalmological examination is an important screening test for the family of patients with CHRPE associated with FAP Link: Click for Pubmed record Title: Cancer and the Peutz-Jeghers syndrome Authors: Spigelman AD;Murday V;Phillips RK; Journal: Gut Date: 1989 Nov Abstract: Among 72 patients with the Peutz-Jeghers syndrome malignant tumours have developed in 16 (22%) of whom all but one have died. There were nine gastrointestinal and seven nongastrointestinal tumours. The relative risks of death from gastrointestinal cancer and all cancers were 13 (95% CI 2.7-38.1) and 9 (95% CI 4.2-17.3) respectively. The chance of dying of cancer by the age of 57 was 48%. There is evidence for a hamartoma/carcinoma sequence in the Peutz-Jeghers syndrome, suggesting that the gene locus involved is relevant to the development of malignancy in general Link: Click for Pubmed record Title: Increased risk of cancer in the Peutz-Jeghers syndrome Authors: Giardiello FM;Welsh SB;Hamilton SR;Offerhaus GJ;Gittelsohn AM;Booker SV;Krush AJ;Yardley JH;Luk GD; Journal: N Engl J Med Date: 1987 Jun 11 Abstract: The Peutz-Jeghers syndrome is an autosomal dominant hereditary disease characterized by hamartomatous polyps of the gastrointestinal tract and by mucocutaneous melanin deposits. The frequency of cancer in this syndrome has not been studied extensively. Therefore, we investigated 31 patients with the Peutz-Jeghers syndrome who were followed from 1973 to 1985. All cases of cancer were verified by histopathological review. Cancer developed in 15 of the 31 patients (48 percent)--gastrointestinal carcinomas in 4, nongastrointestinal carcinomas in 10, and multiple myeloma in 1. In addition, adenomatous polyps of the stomach and colon occurred in three other patients. The cancers were diagnosed when the patients were relatively young, but after the Peutz-Jeghers syndrome had been diagnosed (interval between diagnoses, 25 +/- 20 years; range, 1 to 64). According to relative-risk analysis, the observed development of cancer in the patients with the syndrome was 18 times greater than expected in the general population (P less than 0.0001). Our results suggest that patients with the Peutz-Jeghers syndrome have an increased risk for the development of cancer at gastrointestinal and nongastrointestinal sites Link: Click for Pubmed record Title: The use of indomethacin, sulindac, and tamoxifen for the treatment of desmoid tumors associated with familial polyposis Authors: Klein WA;Miller HH;Anderson M;DeCosse JJ; Journal: Cancer Date: 1987 Dec 15 Abstract: Seven familial polyposis patients with desmoid tumors were treated with indomethacin, sulindac, or tamoxifen either as single agents or in combination. Serial computed tomographic (CT) scan examination was employed for objective measurement of tumor size since physical examination alone was an inaccurate means to evaluate intraabdominal and retroperitoneal desmoids. Only one patient with minimal tumor burden demonstrated a favorable response with complete resolution of an abdominal wall desmoid Link: Click for Pubmed record Title: Peutz-Jeghers syndrome with metastasizing gastric adenocarcinoma. Report of a case Authors: Halbert RE; Journal: Arch Pathol Lab Med Date: 1982 Oct Abstract: A case of metastatic, poorly differentiated gastric adenocarcinoma in a 22-year-old woman with Peutz-Jeghers syndrome was studied. The young age of many patients who have gastrointestinal (GI) carcinoma associated with this syndrome, as well as the unusual locations of primary tumors (eg, duodenum), suggest an increased tendency toward malignant transformation in the upper Gl tract. Statistical support of this contention, however, is unavailable from presently available data Link: Click for Pubmed record Title: A family with Peutz-Jeghers syndrome and bilateral breast cancer Authors: Riley E;Swift M; Journal: Cancer Date: 1980 Aug 15 Abstract: The proband and her paternal grandmother had bilateral breast carcinoma and the Peutz-Jeghers syndrome. An ovarian sex cord tumor with annular tubules was an incidental finding when oophorectomy was performed as treatment of the proband's premenopausal breast cancer. Because there are previous reports of breast cancer in patients with this syndrome, the Peutz-Jeghers gene may be associated with an increased risk of breast tumors Link: Click for Pubmed record Title: Subungual malignant melanoma Authors: Braitman M; Journal: Cutis Date: 1979 May Abstract: A sixty year old white woman with a history of Peutz-Jeghers syndrome with nail involvement, presented with a marked hyperpigmentation of the terminal phalanx and nail of the right index finger. Two preceding biopsy specimens from the fingertip were interpreted as junction nevus. After amputation of the fingertip and terminal phalanx, the final diagnosis was subungual malignant melanoma. Further amputation of the entire finger and portion of the corresponding metacarpal bone was done as a successful follow-up procedure Link: Click for Pubmed record Title: Peutz-Jeghers syndrome associated with gastrointestinal carcinoma. Report of two cases in a family Authors: Cochet B;Carrel J;Desbaillets L;Widgren S; Journal: Gut Date: 1979 Feb Abstract: Patients with the Peutz-Jeghers syndrome carry a slight, though definite, increased risk of gastrointestinal carcinoma. The malignant potentiality of Peutz-Jeghers hamartomatous polyps, generally considered benign, is supported by this report. Two cases of metastasising gastrointestinal carcinoma associated with the Peutz-Jeghers syndrome are described in a 56 year old female and her 29 year old son. Both mother and son died from duodenal and gastric carcinomas respectively, which developed in hamartomatous polyps with extensive metastases. Both cases also showed dysplastic areas within hamartomatous polyps. These features indicate that hamartomatous polyps may, in some cases, be the precursors of digestive tract carcinomas Link: Click for Pubmed record Title: Malignant neoplasm in Peutz-Jeghers syndrome Authors: Major P; Journal: JAMA Date: 1978 Nov 10 Abstract: Link: Click for Pubmed record Title: Extensive metastases in Peutz-Jeghers syndrome Authors: Ryo UY;Roh SK;Balkin RB;Siedband LB;Sobel GW;Pinsky SM; Journal: JAMA Date: 1978 May 26 Abstract: A 27-year-old woman with Peutz-Jeghers syndrome since age 11 years was hospitalized with a sudden onset of weakness of the right extremity, an expressive aphasia, and a three-month history of back pain. Liver and whole-body scintigrams demonstrated multiple metastatic disease; the brain scintigraphic study was compatible with infarction of the left hemisphere. Postmoretem examination two weeks after these studies showed extensive metastases in bone, liver, brain, lung, and left ovary. The source of the metastases was a malignant change from Peutz-Jeghers polyps Link: Click for Pubmed record Title: Peutz-Jeghers polyposis with metastasizing duodenal carcinoma Authors: Lin JI;Caracta PF;Lindner A;Guzman LG; Journal: South Med J Date: 1977 Jul Abstract: A case of Peutz-Jeghers polyposis associated with duodenal carcinoma but without cutaneous pigmentation was presented. Duodenal carcinoma is the most commonly associated neoplasm. The presence of vitiligo in this patient is coincidental. Of 14 reported cases of Peutz-Jeghers syndrome associated with intestinal carcinoma, in only two was the tumor shown to originate in the polyp. In our case, too, we believe the carcinoma developed independently Link: Click for Pubmed record Title: Intestinal carcinoma in the Peutz-Jeghers syndrome Authors: Reid JD; Journal: JAMA Date: 1974 Aug 12 Abstract: Link: Click for Pubmed record Title: Intestinal cancer in the Peutz-Jeghers syndrome. A re-evaluation Authors: Reid JD;Petrelli M; Journal: Ohio State Med J Date: 1974 Apr Abstract: Link: Click for Pubmed record Title: Malignant changes in Peutz-Jeghers syndrome Authors: Papaioannou A;Critselis A; Journal: N Engl J Med Date: 1973 Sep 27 Abstract: Link: Click for Pubmed record Title: Malignant changes in Peutz-Jeghers syndrome Authors: Papaioannou A;Critselis A; Journal: N Engl J Med Date: 1973 Sep 27 Abstract: Link: Click for Pubmed record Title: Peutz-Jeghers syndrome and gastrointestinal malignancy Authors: Dodds WJ;Schulte WJ;Hensley GT;Hogan WJ; Journal: Am J Roentgenol Radium Ther Nucl Med Date: 1972 Jun Abstract: Link: Click for Pubmed record Title: Peutz-Jeghers syndrome with jejunal and colonic adenocarcinomas Authors: Shibata HR;Phillips MJ; Journal: Can Med Assoc J Date: 1970 Aug 1 Abstract: Link: Click for Pubmed record Title: The problem of carcinoma in the Peutz-Jeghers syndrome Authors: Olansky S;Achord JL; Journal: South Med J Date: 1969 Jul Abstract: Link: Click here to read the article Title: The Peutz-Jeghers syndrome. Is there a predisposition to the development of intestinal malignancy? Authors: Dozois RR;Judd ES;Dahlin DC;Bartholomew LG; Journal: Arch Surg Date: 1969 Apr Abstract: Link: Click here to read the article Title: Metastasizing carcinoma of the stomach in Peutz-Jeghers syndrome Authors: Payson BA;Moumgis B; Journal: Ann Surg Date: 1967 Jan Abstract: Link: Click here to read the article Title: DUODENAL CARCINOMA IN THE PEUTZ-JEGHERS SYNDROME: REPORT OF A CASE Authors: J. D. REID Journal: CANCER Date: 1965 Abstract: THE ASSOCIATION OF MELANIN SPOTS OF THE ORAL MUCOSA, LIPS AND DIGITS WITH GENERALIZED INTESTINAL POLYPOSIS CONSTITUTES A WELLKNOWN SYNDROME DESIGNATED BY THE NAMES OF THE DUTCH AND AMERICAN AUTHORS, PEUTZ AND JEGHERS, WHO DESCRIBED IT IN 1921 AND IN 1944, RESPECTIVELY. Link: Click here to read the article Title: PEUTZ-JEGHERS SYNDROME WITH METASTASIZING DUODENAL CARCINOMA Authors: J. P. WILLIAMS, ANTHONY KNUDSEN Journal: GUT Date: 1965 Abstract: SINCE INTEREST WAS FIRST AROUSED IN THE PEUTZ-JEGHERS SYNDROME THERE HAVE BEEN CONFLICTING VIEWS AS TO THE FREQUENCY WITH WHICH MALIGNANT CHANGE OCCURS IN THE POLYPS FOUND IN THE SMALL INTESTINE, AND THIS HAS BEEN DUE TO THE DIFFICULTY OF INTERPRETING THE HISTOLOGICAL STRUCTURE OF THE POLYPI. MANY NOW FEEL THAT IN THIS SYNDROME THESE LESIONS ARE HAMARTOMAS AND THAT THE APPARENT EARLY MALIGNANT CHANGE IS DUE TO EPITHELIAL ELEMENTS BEING CAUGHT UP IN THE INTERLACING BANDS OF SMOOTH MUSCLE. Link: Click here to read the article Title: MALIGNANT DEGENERATION AND METASTASIS IN PEUTZ-JEGHERS SYNDROME Authors: JAMES L. ACHORD, HERBERT D. PROCTOR Journal: ARCHIVES OF INTERNAL MEDICINE Date: 1963 Abstract: SINCE PEUTZ IN 1921 RECOGNIZED THE HEREDITARY NATURE OF GASTROINTESTINAL POLYPOSIS ASSOCIATED WITH ORAL PIGMENTATION AND ITS REEMPHASIS BY JEGHERS IN 1949 THE SYNDROME OF PEUTZ-JEGHERS HAS BECOME A WELL-DOCUMENTED CLINICAL ENTITY. SEVERAL THOROUGH REVIEWS HAVE APPEARED ON THE CLINICAL FEATURES OF THIS SYNDROME. IT IS NOT OUR INTENTION HERE TO REITERATE THESE SIGNS AND SYMPTOMS. Link: Click here to read the article Title: THE PEUTZ-JEGHERS SYNDROME Authors: ROBERT C. HORN, JR., WALTER A. PAYNE, GERALD FINE Journal: ARCHIVES OF PATHOLOGY Date: 1963 Abstract: ATTENTION WAS FIRST CALLED TO THE SYNDROME, THAT IS THE TITLE OF THIS PAPER IN 1991 BY PEUTZ. IN 1949 JEGHERS AND HIS CO-WORKERS 8 WERE ABLE TO COLLECT 23 CASES, AND JEGHERS NAME BECAME LINKED WITH THAT OF PEUTZ TO FORM THE NOW FAMILIAR EPONYM. IT IS OF INTEREST THAT JEGHERS STATES THAT KEEFER NOTED THE SIMILARITY OF HIS (JEGHERS') FIRST CASE TO A PAIR OF 12-YEAR-OLD TWIN GIRLS DISCUSSED BY HUTCHINSON AS EARLY AS 1896. Link: Click here to read the article Title: PEUTZ-JEGHERS SYNDROME WITH JEJUNAL CARCINOMA Authors: SAMUEL DE LA PAVA, AURELIO CABRERA, E. R. STUDENSKI Journal: NEW YORK STATE JOURNAL OF MEDICINE Date: 1962 Abstract: IN CHILDREN ADENOMATOUS POLYPS OF THE SMALL INTESTINE ARE USUALLY ASSOCIATED WITH OTHER STIGMAS OF THE PEUTZ-JEGHERS SYNDROME. Link: Click here to read the article Title: PRECANCEROUS LESIONS OF UPPER GASTROINTESTINAL TRACT Authors: BASIL C. MORSON Journal: JAMA Date: 1962 Abstract: CANCER OF THE STOMACH IS ONE OF THE COMMONEST AND MOST SERIOUS FORMS OF MALIGNANT DISEASE BOTH IN BRITAIN AND IN THE UNITED STATES. THE ETIOLOGICAL POSSIBILITIES THAT HAVE ATTRACTED ATTENTION INCLUDE GENETICAL, DIETARY, GEOGRAPHICAL, AND RACIAL FACTORS, AS WELL AS THE OCCURRENCE OF PRECANCEROUS CHANGES IN THE GASTRIC MUCOSA. IT IS THESE MORPHOLOGICAL CHANGES PREDISPOSING TO CANCER AND THEIR RELATIONSHIP TO OTHER ETIOLOGICAL FACTORS WHICH ARE THE PARTICULAR CONCERN OF THIS MANUSCRIPT. Link: Click here to read the article Title: PEUTZ-JEGHERS SYNDROME: REPORT OF A CASE OF SMALL INTESTINAL POLYPOSIS AND CARCINOMA ASSOCIATED WITH MELANIN PIGMENTATION OF THE LIPS AND BUCCAL MUCOSA Authors: MALCOLM S. BEINFIELD, GEORGE W. CHANGUS Journal: GASTROENTEROLOGY Date: 1958 Abstract: THE FIRST CLEAR CUT ASSOCIATION OF MELANIN PIGMENTATION OF THE LIPS AND BUCCAL MUCOSA WITH INTESTINAL POLYPOSIS WAS REPORTED BY PEUTZ IN 1921. SINCE THAT TIME A FEW REPORTS HAVE APPEARED, INCLUDING ONE PUBLISHED BY JEGHERS IN 1944, BUT IT WAS NOT UNTIL JEGHERS AND CO-WORKERS REPORTED 10 CASES IN 1949 THAT THIS SYNDROME BECAME CLEARLY IDENTIFIED IN THE ENGLISH MEDICAL LITERATURE. Link: Click here to read the article Title: SYNDROME OF INTESTINAL POLYPOSIS WITH MELANOSIS OF THE LIPS AND BUCCAL MUCOSA: A STUDY OF THE INCIDENCE AND LOCATION OF MALIGNANCY Authors: STANLEY B. BERKOWITZ, MILTON J. PEARL, NEWTON H. SHAPIRO Journal: REPRINTED FROM ANNALS OF SURGERY Date: 1955 Abstract: POLYPS OF THE SMALL INTESTINE ARE RARE AND DIFFICULT TO DIAGNOSE. HOWEVER, 8 TO 9 PER CENT OF THE CASES MAY BE SUSPECTED BECAUSE OF ASSOCIATION WITH MELANOTIC SPOTTING OF THE LIPS AND BUCCAL MUCOSA. IN SPITE OF THE ILLUSTRATED, WELL-DOCUMENTED, CRITICAL REVIEW BY JEGHERS, ET AL., IN 1949, LESS THAN TEN SUBSEQUENT REPORTS OF THE SYNDROME HAVE APPEARED IN THE WORLD LITERATURE. Link: Click here to read the article
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Jeghers Medical Index, St. Elizabeth Health Center, 1044 Belmont Ave., Youngstown, OH 44501
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