Malignant tumors associated with Peutz-Jeghers syndrome: Five cases from a single surgical unit

Table 1 Operative clinical data, diagnosis and treatment methods, and follow-up of five patients with Peutz-Jeghers syndrome

Number	Age	Diagnostic criteria	Diagnostic method1	Clinical manifestation	Operation method	Postoperative pathology2	Follow-up time
1	42	Pigmentation	Gastroscopy(+)4 gene detection(+)4	Intussusception	Total gastrectomy	Stomach: Multiple PJS polyps3 and highly differentiated adenocarcinoma in gastric fundus and body	25 mo
		Gastrointestinal polyps	Colonoscopy(+)4	Incomplete intestinal obstruction	Hepatic metastasis resection	Intestine: Multiple PJS polyps3
		Family history of PJS3	Upper digestive tract radiography(+)4	Abdominal pain	Small intestinal polyp resection	Liver: Infiltrating adenocarcinoma
			CT(+)4	Anti-acid, heartburn	Intussusception reduction	Gene detection: STK11 mutation
						T4bN3M1
2	29	Gastrointestinal polyps	Gastroscopy(+)4	Abdominal pain	Subtotal gastrectomy	Stomach: Multiple PJS polyps3, with most tumors developing into highly differentiated adenocarcinoma	20 mo
		Family history of PJS3	Colonoscopy(+)4	Abdominal distension	Resection of small intestinal polyps	Intestine: Multiple PJS polypsc
			CT(+)4			T2N0M0
			Upper digestive tract radiography(+)4
3	67	Gastrointestinal polyps	Gastroscopy(+)4	Intussusception	Gastrojejunostomy	Greater omentum: Infiltrating adenocarcinoma	The patient died of liver metastasis, abdominal lymph node metastasis, and cachexia 6 mo after surgery
		Pigmentation	Colonoscopy(+)4	Incomplete intestinal obstruction	Intussusception reduction	Meso-ileum: Infiltrating adenocarcinoma
			Upper digestive tract radiography(+)d	Abdominal distension	Resection of small intestinal polyps	Abdominal wall nodule: Infiltrating adenocarcinoma
			CT(+)d	Nausea and vomit		Intestine: Adenocarcinoma infiltrating intestinal wall; PJSc polyps of intestine
						T4bNxM1
4	29	Gastrointestinal polyps	Gastroscopy(+)4	Abdominal pain	Colonic polyp ESD	PJS3 polyps, with some high-grade intraepithelial neoplasia and clean margins	42 mo
		Family history of PJS3		Abdominal distension		T1aN0M0
5	51	Gastrointestinal polyps	Gastroscopy(+)4	Incomplete intestinal obstruction	Colonic polyp ESD	Stomach: Fundic gland polyp	47 mo
		Family history of PJS3	Colonoscopy(+)4	Abdominal distension	Gastric polyp ESD	Intestine: PJS3 polyps, with some high-grade intraepithelial neoplasia and clean margins
			MRI(-)5			T1aN0M0

¹Positive diagnosis of PJS.

² Staging criteria for gastric and colorectal cancers by the AJCC/UICC version 8 TNM staging system.

³PJS: Peutz-Jeghers syndrome.

⁴(+): Positive.

⁵(-): Negative. CT: Computed tomography; ESD: Endoscopic submucosal dissection.