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Peutz-Jeghers Syndrome: A Circumventable Emergency

Peutz-Jeghers syndrome (PJS) is characterized by multiple hamartomatous polyps in the gastrointestinal tract and mucocutaneous pigmentation. Here we present, a case of multiple gastrointestinal hamartomatous polyps in a 22-year-old male who had been operated for intestinal obstruction due to ileocol...

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Detalles Bibliográficos
Autores principales: Choudhury, Sourav, Das, Anupam, Misra, Priyankar, Ray, Udipta, Sarangi, Sujata
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Medknow Publications & Media Pvt Ltd 2018
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5903049/
https://www.ncbi.nlm.nih.gov/pubmed/29692461
http://dx.doi.org/10.4103/ijd.IJD_563_16
Descripción
Sumario:Peutz-Jeghers syndrome (PJS) is characterized by multiple hamartomatous polyps in the gastrointestinal tract and mucocutaneous pigmentation. Here we present, a case of multiple gastrointestinal hamartomatous polyps in a 22-year-old male who had been operated for intestinal obstruction due to ileocolic intussusception. Resection of the affected segment was done with proximal ileostomy and distal mucous fistula formation. Clinicopathological diagnosis of PJS was made. Later, during ileostomy closure, it was found that the patient had a transverse colonic mass which was resected. Histological examination with immunohistochemistry confirmed it to be a Mucosa-Associated Lymphoid Tissue Lymphoma (MALToma). Colonic MALToma in the background of PJS is a unique case for which it has been reported.