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Juvenile Polyposis Syndrome Might Be Gao et al. BMC Gastroenterology (2020) 20:167 https://doi.org/10.1186/s12876-020-01238-7 CASE REPORT Open Access Juvenile polyposis syndrome might be misdiagnosed as familial adenomatous polyposis: a case report and literature review Xian Hua Gao1,2†, Juan Li3†, Zi Ye Zhao1,2†, Xiao Dong Xu1,2,YiQiDu2,4, Hong Li Yan2,5, Lian Jie Liu1*, Chen Guang Bai2,6* and Wei Zhang1,2* Abstract Background: Juvenile polyposis syndrome (JPS) is a rare disorder characterized by the presence of multiple juvenile polyps in the gastrointestinal tract, and germline mutations in SMAD4 or BMPR1A. Due to its rarity and complex clinical manifestation, misdiagnosis often occurs in clinical practice. Case presentation: A 42-year-old man with multiple pedunculated colorectal polyps and concomitant rectal adenocarcinoma was admitted to our hospital. His mother had died of colon cancer. He was diagnosed with familial adenomatous polyposis (FAP) and underwent total proctocolectomy and ileal pouch anal anastomosis. Two polyps were selected for pathological examination. One polyp had cystically dilated glands with slight dysplasia. The other polyp displayed severe dysplasia and was diagnosed as adenoma. Three years later, his 21-year-old son underwent a colonoscopy that revealed more than 50 pedunculated colorectal juvenile polyps. Both patients harbored a germline pathogenic mutation in BMPR1A. Endoscopic resection of all polyps was attempted but failed. Finally, the son received endoscopic resection of polyps in the rectum and sigmoid colon, and laparoscopic subtotal colectomy. Ten polyps were selected for pathological examination. All were revealed to be typical juvenile polyps, with cystically dilated glands filled with mucus. Thus, the diagnosis of JPS was confirmed in the son. A review of the literatures revealed that patients with JPS can sometimes have adenomatous change. Most polyps in patients with JPS are benign hamartomatous polyps with no dysplasia. A review of 767 colorectal JPS polyps demonstrated that 8.5% of the polyps contained mild to moderate dysplasia, and only 0.3% had severe dysplasia or cancer. It is difficult to differentiate juvenile polyps with dysplasia from adenoma, which could explain why juvenile polyps have been reported to have adenomatous changes in patients with JPS. Therefore, patients with JPS, especially those with concomitant dysplasia and adenocarcinoma, might be easily diagnosed as FAP in clinical practice. (Continued on next page) * Correspondence: [email protected]; [email protected]; [email protected] †Xian Hua Gao, Juan Li and Zi Ye Zhao contributed equally to this work. 1Department of Colorectal Surgery, Changhai Hospital, 168 Changhai Road, Shanghai 200433, China 2Hereditary Colorectal Cancer Center and Genetic Block Center of Familial Cancer, Changhai Hospital, Shanghai, China Full list of author information is available at the end of the article © The Author(s). 2020 Open Access This article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or other third party material in this article are included in the article's Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article's Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this licence, visit http://creativecommons.org/licenses/by/4.0/. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated in a credit line to the data. Gao et al. BMC Gastroenterology (2020) 20:167 Page 2 of 9 (Continued from previous page) Conclusions: Juvenile polyp with dysplasia is often diagnosed as adenoma, which might lead to the misdiagnosis of JPS as FAP. The differential diagnosis of JPS versus FAP, should be based on comprehensive evaluation of clinical presentation, endoscopic appearance and genetic investigations; not on the presence or absence of adenoma. Keywords: Juvenile polyposis syndrome, Familial adenomatous polyposis, Misdiagnosis, Dysplasia, Adenoma Background the central nervous system and pulmonary circulation have Juvenile polyposis syndrome (JPS) was first described in also been reported in JPS cases [2]. 1964 [1]. It is a rare (approximately one in every 100,000 Due to its rarity and complex clinical manifestation, mis- individuals) autosomal dominant disease that is character- diagnosis of JPS may occur in clinical practice. Here, we ized by the occurrence of several juvenile polyps in the report a father-son case of JPS misdiagnosed as familial ad- gastrointestinal tract [2, 3]. Juvenile polyp is a specific type enomatous polyposis (FAP). The literature was reviewed of hamartomatous polyp. The term “juvenile” refers to the to elucidate the possible reason for the misdiagnosis and polyp histology rather than the age of onset of the polyp to determine how a more accurate diagnosis can be made [3]. An isolated juvenile polyp is not diagnostic of JPS and in the future. occurs in approximately 2% of children and adolescents [3]. In contrast to Peutz-Jeghers syndrome (PJS), in which Case presentation small bowel polyps are common, patients with JPS de- Clinical manifestation and treatment process of the father velop polyps primarily in the stomach and colon [2, 4]. A 42-year-old Chinese man with colorectal polyps and con- JPS is associated with an increased risk of gastrointestinal comitant rectal cancer was admitted to our department. cancer. The cumulative risks of colorectal and gastric can- His mother had died of colorectal polyposis and colon can- – cers are estimated to be approximately 40 50 and 20%, re- cer at 53 years old. Digital rectal examination showed a cir- – spectively [2 5]. Pancreatic cancer and duodenal cancer cular mass located 4 cm above the anal verge. No other – have also been reported [4 6]. Malignant tumor has not abnormality was observed by physical examination. Labora- been found in other parts of the small intestinal bowel. tory data also revealed no abnormalities. Colonoscopy re- Germline mutations in SMAD family member 4 vealed an ulcerative mass occupying a circle of the intestine (SMAD4) or bone morphogenetic protein receptor type 1A in the rectum, located 4 cm from the anal verge. Multiple – (BMPR1A), have been found in 50 60% of patients with polyps (40–50, diameter 0.5–3 cm) were also observed – JPS [4, 7 9]. Both genes are involved in the bone morpho- throughout the colorectum, and some were pedunculated BMP – genetic protein ( ) transforming growth factor-beta polyps (Fig. 1). Pathological examination of a biopsy sample TGF-β ( ) signaling pathway [5]. Most of the variants are identified the rectal mass as adenocarcinoma (Fig. 2). Fol- point mutations or small base pair deletions in the coding lowing a diagnosis of FAP, total proctocolectomy (TPC) regions, and approximately 15% of the variants are large de- and ileal pouch anal anastomosis (IPAA) was performed. ENG letions or recombinations. Mutations in related with The rectal mass was revealed to be an adenocarcinoma. TGF-β the signaling pathway have also been reported in Only two colorectal polyps were selected from the resected some patients [2, 6]. However, in a large proportion of JPS colon for pathological examination. One polyp had cysti- cases, the responsible gene mutation was not identified [2]. cally dilated glands with slight dysplasia. The other polyp – Approximately 20 50% of JPS cases have no family history displayed severe dysplasia and was diagnosed as adenoma. and they may harbor de novo mutations [3, 4, 6]. Recovery was uneventful and the ileostoma was closed 3 JPS often presents in childhood before puberty. Typical month later. After ileostoma closure, he experienced severe symptoms include hematochezia, anemia, diarrhea, abdom- fecal urgency, with a daily frequency of more than 20 times inal pain, intussusception, intestinal obstruction, and polyp even after medication with loperamide. Multiple lung me- – SMAD4 prolapse [2 6]. Patients with JPS with mutations tastases were detected one year after the initial surgery. can have markedly higher risks of gastric polyps and gastric BMPR1A cancer than patients with the mutation [3, 5, 10, Clinical manifestation and treatment process of the son SMAD4 11]. Most patients with JPS who harbor variants After the patient was misdiagnosed as FAP, a colonoscopy might have concomitant hereditary hemorrhagic telangi- was suggested for his 21-year-old son. The colonoscopy – ectasia (HHT) or Rendu-Osler-Weber syndrome [2 4], performed at our hospital revealed more than 50 polyps which is characterized by vascular malformations with epi- measuring 0.5–2.5 cm in diameter in the colon and rec- staxis, mucocutaneous telangiectasia, and arteriovenous tum (Fig. 3). Esophagogastroduodenoscopy (EGD) re- malformations of the lungs, liver, and brain [5, 12, 13]. Car- vealed no polyps in the stomach. He was also suggested to diac atrial and ventricular septal defects and aneurysms of undergo TPC + IPAA. He declined and came to our Gao et al. BMC Gastroenterology (2020) 20:167 Page 3 of 9 Fig. 1 Colonoscopic findings of the father. a: rectal adenocarcinoma; b, c: pedunculated polyps; d: sessile polyps with mulberry shape hereditary colorectal cancer counseling clinic. Gene muta- in the stroma. No dysplasia or cancer was evident in these tion testing indicated that both he and his father had a polyps (Fig. 5).Allexaminedpolypswereprovedtobetyp- germline mutation in BMPR1A (c.949_952delCTCT). This ical juvenile polyps, so the diagnosis of JPS was confirmed variant was classified as pathogenic. Finally, the son and in the son. He remains in excellent condition with normal his father were diagnosed with JPS. Endoscopic resection sexual and urinary functions.
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