scholarly journals A Case of Peutz-Jeghers Syndrome Presenting With an Inoperable Periampullary Tumor

Cureus ◽  
2021 ◽  
Author(s):  
Muhammer Ergenç ◽  
Tevfik Kıvılcım Uprak
Keyword(s):  
Periampullary Tumor ◽  
Peutz Jeghers Syndrome

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.

Cirurgia Paliativa em Pacientes com Tumor Periampular Irressecável: Estudo Retrospectivo de 5 Anos em um Hospital em São José dos Campos – SP/Surgical Palliation in Patients with Unresectable Periampullary Tumor: a Retrospective Study of 5 Years in a Hosp

1970 ◽  
Vol 3 (4) ◽  
pp. 9-20
Author(s):  
José Henrique Gomes Torres ◽  
Rosyane Rena De Freitas
Keyword(s):  
Pancreatic Cancer ◽  
Retrospective Study ◽  
Survival Rates ◽  
Periampullary Tumor ◽  
The Past ◽  
Palliative Procedure ◽  
Surgical Palliation ◽  
Head Of The Pancreas ◽  
Keywords Pancreatic Cancer ◽  
Main Complication

Objetivo: Avaliar diferentes métodos paliativos quanto a sua resolução, complicações e sobrevida em pacientes com tumor periampular irressecável. Materiais e métodos: Estudo retrospectivo com análise dos prontuários de pacientes com tumor periampular irressecável e que foram submetidos a procedimento paliativo no Hospital Municipal Dr José de Carvalho Florence nos últimos cinco anos. Resultados: O principal tumor periampular foi o de cabeça de pâncreas, com incidência de 94%, acometendo pacientes com média de 66 anos, sem preferência por sexo. Os procedimentos mais realizados foram derivação biliar e colocação de endoprótese através de colangiopancreatografia endoscópica retrógrada, apresentando sobrevidas de 586 e 56 dias, respectivamente. Conclusão: A coledocojejunostomia foi o procedimento mais realizado e apresentou menor tempo de internação e maiores sobrevida e tempo de permanência anictérico. Pneumonia foi a complicação mais frequente.  Palavras chave: Câncer pancreático, Colangiocarcinoma, Cuidados paliativos.  Objective: To evaluate different palliative methods concerning its resolution, complications and survival in patients with unresectable periampular tumor. Materials and methods: Retrospective study analysing records of patients with unresectable periampullary tumor and who underwent palliative procedure in the Hospital Municipal Dr José de Carvalho Florence in the past five years. Results: The main periampullary tumor was the head of the pancreas, with an incidence of 94%, affecting patients with an average of 66 years old, regardless of gender. The most common procedures were bypass and biliary stent, with survival rates of 586 and 56 days, respectively. Conclusion: Coledocojejunostomy was the procedure which was the most often performed and showed a shorter hospital stay and longer survival time and time without jaundice. Pneumonia was the main complication.  Keywords: Pancreatic cancer, Cholangiocarcinoma, Palliative care  

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.

scholarly journals Delayed diagnosis of Peutz–Jeghers syndrome due to pathological information loss or mistake in family/personal history

2021 ◽  
Vol 16 (1) ◽  
Author(s):  
Yu-Liang Jiang ◽  
Xiao-Dong Xu ◽  
Bai-Rong Li ◽  
En-Da Yu ◽  
Zi-Ye Zhao ◽  
...  
Keyword(s):  
Clinical Presentation ◽  
Novel Mutation ◽  
Delayed Diagnosis ◽  
Personal History ◽  
Pathological Examination ◽  
The Novel ◽  
Novel Mutations ◽  
The Family ◽  
Genetic Level ◽  
Peutz Jeghers Syndrome

Abstract Objective To report Peutz–Jeghers syndrome (PJS) cases with non-definitive clues in the family or personal history and finally diagnosed through pathological examination and STK11 gene mutation test. Clinical presentation and intervention PJS was suspected in 3 families with tortuous medical courses. Two of them had relatives departed due to polyposis or colon cancer without pathological results, and the other one had been diagnosed as hyperplastic polyposis before. Diagnosis of PJS was confirmed by endoscopy and repeated pathological examinations, and the STK11 mutation test finally confirmed the diagnosis at genetic level, during which 3 novel mutation were detected (536C > A, 373_374insA, 454_455insGGAGAAGCGTTTCCCAGTGTGCC). Conclusion Early diagnosis of PJS is important and may be based on a family history with selective features among family members, and the pathological information is the key. The novel mutations also expand the STK11 variant spectrum.

scholarly journals Peutz-Jeghers syndrome in a woman presenting as intussusception: A case report

2021 ◽  
Vol 79 ◽  
pp. 286-290
Author(s):  
Erwin Syarifuddin ◽  
Rina Masadah ◽  
Ronald Erasio Lusikooy ◽  
Warsinggih ◽  
Julianus Aboyaman Uwuratuw ◽  
...  
Keyword(s):  
Case Report ◽  
Peutz Jeghers Syndrome

scholarly journals Appendiceal cancer leading to intussusception detected incidentally during follow-up for Peutz–Jeghers syndrome

2020 ◽  
Vol 13 (6) ◽  
pp. 1136-1143
Author(s):  
Kazuhiro Kurihara ◽  
Takanori Suganuma
Keyword(s):  
Malignant Tumors ◽  
Endoscopic Examination ◽  
Autosomal Dominant Disorder ◽  
Tomography Image ◽  
Elevated Lesion ◽  
Poorly Differentiated ◽  
Lower Endoscopy ◽  
Peutz Jeghers Syndrome ◽  
Computed Tomography Image

AbstractPeutz–Jeghers syndrome is an autosomal dominant disorder characterized by hamartomatous polyposis, pigmentation, and malignant tumors. We report a case of ileocecal carcinoma that was incidentally detected during follow-up for Peutz–Jeghers syndrome. A 39-year-old man with solitary Peutz–Jeghers syndrome had undergone three abdominal surgeries. He had been followed up via upper and lower gastrointestinal endoscopy and small intestinal endoscopy. In the endoscopic examination of the lower gastrointestinal tract, a 35 mm large, bumpy, elevated lesion was observed in the cecum. This lesion was not observed 9 months earlier during lower endoscopy. Biopsy of the specimen confirmed tubulovillous adenoma and carcinoma. This lesion was judged to be an indication for operation, and we performed ileocecectomy + D3 lymph node dissection. From the excised specimen, poorly differentiated carcinoma and adenoma components in contact with Peutz–Jeghers-type polyps in the appendix were recognized. A review of the computed tomography image obtained 2 years ago confirmed appendiceal swelling. We suspect that the ileocecal carcinoma in the appendix may have rapidly developed within the 9 months, and was incidentally detected on lower endoscopic examination during follow-up. For the prevention of appendicular tumorigenesis, prophylactic appendectomy may be considered in certain cases during follow-up for Peutz–Jeghers syndrome.

Peutz-Jeghers Syndrome

2014 ◽  
Author(s):  
Barry H. Davison ◽  
Joan W. Mayfield
Keyword(s):  
Peutz Jeghers Syndrome

Jejunal Endoscopic Polypectomy in the Peutz-Jeghers Syndrome

Endoscopy ◽  
1983 ◽  
Vol 15 (04) ◽  
pp. 270-271 ◽  
Author(s):  
A. Paterlini ◽  
C. Huscher ◽  
A. Salmi
Keyword(s):  
Endoscopic Polypectomy ◽  
Peutz Jeghers Syndrome
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