Solitary Peutz-Jeghers Type Polyp of Jejunum with Gastric Fundic and Antral Gland Lining Mucosa: A Case Report and Review of Literature

2021 ◽  
pp. 106689692110677
Author(s):  
Bella Lingjia Liu ◽  
Huifang Zhou ◽  
Martina Risech ◽  
Alex Ky ◽  
Jane Houldsworth ◽  
...  
Keyword(s):  
Family History ◽  
Small Bowel ◽  
Smooth Muscles ◽  
Fundic Gland ◽  
Muscularis Mucosa ◽  
Hamartomatous Polyp ◽  
Gi Tract ◽  
First Case ◽  
Mucocutaneous Pigmentation ◽  
Peutz Jeghers Syndrome

Solitary Peutz-Jeghers type polyps are characterized by a hamartomatous polyp of the gastrointestinal (GI) tract in a patient without mucocutaneous pigmentation, family history of Peutz-Jeghers syndrome, or STK11/LKB1 mutations. Histologically identical to the polyps in Peutz-Jeghers syndrome, these sporadic polyps can arise anywhere along the GI tract, with typical arborizing smooth muscles extending from the muscularis mucosa. While the lining mucosa is generally the same as the organ in which it arises, gastric pyloric and osseous metaplasia have been reported in intestinal polyps in Peutz-Jeghers syndrome. Herein, the authors report the first case of a small intestinal solitary Peutz-Jeghers type polyp with gastric antral and fundic gland lining mucosa. A 43-year-old male was admitted for small bowel obstruction. Diagnostic laparoscopy revealed jejuno-jejunal intussusception with an associated polyp measuring 7.2 cm. Histological examination showed a hamartomatous polyp with arborizing smooth muscle bundles extending from the muscularis mucosae. The polyp was lined by non-dysplastic gastric antral and fundic gland mucosa, and was sharply demarcated from the adjacent non-polypoid intestinal mucosa. Colonoscopy, esophagogastroduodenoscopy and small bowel enteroscopy revealed no additional polyps or masses. Thorough investigation of the patient's family history was negative for Peutz-Jeghers syndrome or mucocutaneous pigmentation. Molecular analysis of the lesion was negative for STK11/LKB1 mutations. A diagnosis of solitary Peutz-Jeghers type polyp of the small bowel with gastric antral and fundic gland mucosal lining was rendered.

scholarly journals Obstructing Hamartomatous Polyp in Peutz-Jeghers Syndrome

2013 ◽  
Vol 2013 ◽  
pp. 1-3
Author(s):  
Brian S. Bentley ◽  
Hassan M. Hal
Keyword(s):  
Weight Loss ◽  
Abdominal Pain ◽  
Soft Tissue ◽  
Oral Mucosa ◽  
Physical Exam ◽  
Hamartomatous Polyp ◽  
Hamartomatous Polyps ◽  
Mucocutaneous Pigmentation ◽  
Soft Tissue Masses ◽  
Peutz Jeghers Syndrome

A 53-year-old male presented with complaints of abdominal pain and weight loss. On physical exam he was noted to have mucocutaneous pigmentation around his lips and oral mucosa. Radiologic and endoscopic investigations demonstrated an obstructing mass in the second portion of the duodenum along with additional smaller soft tissue masses throughout the bowel. Histology of biopsied specimens revealed architectural disorganization without dysplasia, suggestive of Peutz-Jeghers hamartomatous polyps.

scholarly journals PEUTZ-JEGHERS SYNDROME IN RESOURCE-LIMITED SCENARIO

2020 ◽  
Vol 57 (3) ◽  
pp. 227-231 ◽  
Author(s):  
Natascha Silva SANDY ◽  
Elizete Aparecida LOMAZI ◽  
Maria de Fátima SERVIDONI ◽  
Maria Angela BELLOMO-BRANDÃO
Keyword(s):  
Family History ◽  
Wide Spectrum ◽  
Case Series ◽  
Retrospective Chart Review ◽  
Resource Limited ◽  
Mucocutaneous Pigmentation ◽  
Age Of Diagnosis ◽  
Dominant Disease ◽  
Peutz Jeghers Syndrome

ABSTRACT BACKGROUND: Peutz-Jeghers Syndrome (PJS) is a rare, autosomal dominant disease, caused by deletions in the chromosome 19p33.3/ gene LKB1/STK11. These mutations inactivate a serine/threonine kinase and predispose to carcinogenesis. In PJS, tumors of the gastrointestinal, testicles, pulmonary, breast, pancreas, uterus and ovaries can be found. OBJECTIVE: To evaluate demographics, clinical presentation and complication/outcomes of pediatric patients presenting with Peutz-Jeghers syndrome (PJS), as well as to present and discuss management in the context of limited resources. METHODS: We conducted a retrospective chart review of a cohort of six patients, who were diagnosed and/or followed at the Clinics Hospital, University of Campinas - Sao Paulo/Brazil, between 2000 and 2018. Data analyzed included gender, age of presentation, age of diagnosis, family history, PJS complications. RESULTS: Median age of diagnosis of 6.7 years, with a mean time of follow-up of 8.1 years. Mucocutaneous pigmentation was universally present. Half of the patients had a known family history at the time of diagnosis. On follow up, intestinal intussusception was documented in four out of six patients, in most (three), in different locations and in multiple occasions. The active investigation of siblings and parents of the index case led to the diagnosis of three first-degree relatives in the present case series. CONCLUSION: In this first pediatric PJS Brazilian case series, we report a wide spectrum of PJS manifestations and complications. In a resource limited scenario, despite limitations for the surveillance of complications, the relative frequency of complications was not higher than historically reported.

Giant Solitary Gastric Peutz-Jeghers Polyp Mimicking a Malignant Gastric Tumor: the Largest Described in Literature

2014 ◽  
Vol 23 (3) ◽  
pp. 321-324 ◽  
Author(s):  
Sorinel Lunca ◽  
Vlad Porumb ◽  
Natalia Velenciuc ◽  
Dan Ferariu ◽  
Gabriel Dimofte
Keyword(s):  
Upper Gastrointestinal Bleeding ◽  
Rare Event ◽  
Gastric Tumor ◽  
Node Metastasis ◽  
Mucocutaneous Pigmentation ◽  
History Of ◽  
Peutz Jeghers Syndrome ◽  
Upper Gastrointestinal ◽  
Gastric Malignancy ◽  
Polypoid Tumor

A solitary Peutz-Jeghers polyp is defined as a unique polyp occurring without associated mucocutaneous pigmentation or a family history of Peutz-Jeghers syndrome. Gastric solitary localization is a rare event, with only eight reported cases to date. We report herein the case of a 43-year old woman who presented with upper gastrointestinal bleeding, severe anemia, weight loss and asthenia. Endoscopy revealed a giant polypoid tumor with signs of neoplastic invasion of the cardia, with pathological aspect suggesting a Peutz-Jeghers hamartomatous polyp. Computed tomography suggested a malignant gastric tumor and a total gastrectomy was performed. The pathological specimen showed a giant 150/70/50 mm polypoid tumor and immunochemistry established the final diagnostic of a Peutz-Jegers type polyp. This is the largest solitary Peutz-Jeghers gastric polyp reported until now, and the second one mimicking a gastric malignancy with lymph node metastasis.

SMAD4 juvenile polyposis syndrome and hereditary haemorrhagic telangiectasia presenting in a middle-aged man as a large fungating gastric mass, polyposis in both upper and lower GI tract and iron deficiency anaemia, with no known family history

2020 ◽  
Vol 13 (12) ◽  
pp. e236855
Author(s):  
Wendy Chang ◽  
Patricia Renaut ◽  
Casper Pretorius
Keyword(s):  
Family History ◽  
Autosomal Dominant ◽  
Signs And Symptoms ◽  
Juvenile Polyposis ◽  
Hereditary Haemorrhagic Telangiectasia ◽  
Gi Tract ◽  
Juvenile Polyposis Syndrome ◽  
Polyposis Syndrome ◽  
History Of ◽  
Gastric Mass

Juvenile polyposis syndrome (JPS) and hereditary haemorrhagic telangiectasia (HHT) are rare autosomal dominant diseases, where symptoms manifest at childhood. A 32-year-old man with no family history of JPS or HHT with SMAD4 gene mutation who developed signs and symptoms only at the age of 32, when he was an adult. In this article, we highlight the steps taken to diagnose this rare pathology, explain its pathophysiology and management.

scholarly journals Bladder hamartoma in Peutz-Jeghers syndrome: a rare case report

2021 ◽  
Vol 27 (1) ◽  
Author(s):  
Jai Kumar ◽  
Mohammad Irfaan Albeerdy ◽  
Nadeem Ahmed Shaikh ◽  
Abdul Hafeez Qureshi
Keyword(s):  
Urinary Bladder ◽  
Filling Defect ◽  
Hamartomatous Polyps ◽  
Case Presentation ◽  
Rare Case Report ◽  
Mucocutaneous Pigmentation ◽  
Dominant Disease ◽  
Peutz Jeghers Syndrome ◽  
Comprehensive Compilation

Abstract Background Peutz-Jeghers syndrome is an autosomal dominant disease characterized by mucocutaneous pigmentation and hamartomatous polyps in the gastrointestinal tract (GIT). There have also been cases of extra GIT polyps such as the renal pelvis, urinary bladder, lungs and nares. Bladder hamartoma is an extremely rare finding, with only 12 cases described in the literature up to now. The rarity of the condition necessitates a comprehensive compilation of managements up to now so as to provide a better tool for the treatment of such conditions in the future. Case presentation A twenty-year-old male, known to have Peutz-Jeghers syndrome, presented to us complaining of obstructive urinary symptoms. A urethrogram done showed a filling defect at the base of the urinary bladder. The mass was resected transurethrally, and histopathology revealed a hamartoma of the bladder. The patient has since remained tumor-free on follow-up. Conclusions Transurethral resection of the bladder mass proved to be an effective therapy in this patient with no recurrence on the patient’s follow-up till now. There is still, however, a dearth of knowledge regarding the management of bladder hamartomas owing to the extreme rarity of the case.

Jewish Holiday “Tu Bishvat” or Two Cases of Small Bowel Obstruction with Dried Apricot

2021 ◽  
Vol 6 (1) ◽  
Author(s):  
Kukeev I ◽  
◽  
Replyansky I ◽  
Czeiger D ◽  
Atias S ◽  
...  
Keyword(s):  
Small Bowel ◽  
Small Bowel Obstruction ◽  
Bowel Obstruction ◽  
Gastric Bypass Surgery ◽  
Surgical Removal ◽  
Cellulose Content ◽  
Lead Point ◽  
First Case ◽  
Dried Fruit ◽  
High Level

Introduction: Small bowel obstruction caused by bezoars is rare. One of the causes of phytobezoars is dried fruits. We present two cases of small bowel obstruction caused by dried apricots during Jewish holiday “Tu BiShvat”. Case Presentation: Two men, 54 and 86 years old hospitalized with acute abdomen attributed to small bowel obstruction. In the first case - intoxicated patient, due to suspicion of mesenteric ischemia underwent laparotomy. A lead point caused obstruction was found and after enterotomy whole dried apricot was removed. The patient swallowed it whole three days before hospitalization. In the second case, edentulous patient with small bowel obstruction and peritonitis underwent laparotomy. The cause of obstruction was a dried apricot swallowed whole by the patient. Discussion: Presentation of bezoar with features of acute surgical abdomen is extremely rare, accounting for only 1% of the patients. The expansion of phytobezoar that is high in cellulose content can absorb a large amount of fluid causing an obstruction of the small bowel. The treatment of small bowel obstruction caused by bezoars varies from dissolving with cellulase, papain and even Coca-Cola, followed by endoscopic and surgical removal. Conclusion: A high level of suspicion needs to exist in the presence of a history of eating dried fruit, which can cause gastrointestinal obstruction. Especially on background gastric bypass surgery and inadequate mastication.

Diagnostic yield of magnetically assisted capsule endoscopy versus gastroscopy in recurrent and refractory iron deficiency anemia

Endoscopy ◽  
2018 ◽  
Vol 51 (05) ◽  
pp. 409-418 ◽  
Author(s):  
Hey-Long Ching ◽  
Melissa F. Hale ◽  
Matthew Kurien ◽  
Jennifer A. Campbell ◽  
Stefania Chetcuti Zammit ◽  
...  
Keyword(s):  
Small Bowel ◽  
Iron Deficiency ◽  
Iron Deficiency Anemia ◽  
Capsule Endoscopy ◽  
Diagnostic Yield ◽  
Deficiency Anemia ◽  
Gi Tract ◽  
Upper Gi ◽  
Upper Gi Tract ◽  
Magnetically Assisted

Abstract Background Small-bowel capsule endoscopy is advocated and repeat upper gastrointestinal (GI) endoscopy should be considered for evaluation of recurrent or refractory iron deficiency anemia (IDA). A new device that allows magnetic steering of the capsule around the stomach (magnetically assisted capsule endoscopy [MACE]), followed by passive small-bowel examination might satisfy both requirements in a single procedure. Methods In this prospective cohort study, MACE and esophagogastroduodenoscopy (EGD) were performed in patients with recurrent or refractory IDA. Comparisons of total (upper GI and small bowel) and upper GI diagnostic yields, gastric mucosal visibility, and patient comfort scores were the primary end points. Results 49 patients were recruited (median age 64 years; 39 % male). Combined upper and small-bowel examination using the new capsule yielded more pathology than EGD alone (113 vs. 52; P < 0.001). In upper GI examination (proximal to the second part of the duodenum, D2), MACE identified more total lesions than EGD (88 vs. 52; P < 0.001). There was also a difference if only IDA-associated lesions (esophagitis, altered/fresh blood, angioectasia, ulcers, and villous atrophy) were included (20 vs. 10; P = 0.04). Pathology distal to D2 was identified in 17 patients (34.7 %). Median scores (0 – 10 for none – extreme) for pain (0 vs. 2), discomfort (0 vs. 3), and distress (0 vs. 4) were lower for MACE than for EGD (P < 0.001). Conclusion Combined examination of the upper GI tract and small bowel using the MACE capsule detected more pathology than EGD alone in patients with recurrent or refractory IDA. MACE also had a higher diagnostic yield than EGD in the upper GI tract and was better tolerated by patients.

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