Table 1.

Papers about lymphangiomatosis-related disease.

Reference	Age and gender	GI tract organs involved	Type of lesions	Histopathologic findings	Clinical features	Diagnostic workup	Treatment	Follow-up and outcome
Valakada J, Madhusudhan KS et al. [1]	59-years-old woman	duodenum, jejunum, mesentery and retroperitoneum	marked thickening of the small bowel loops in the duodenum and jejunum and multiple tubular channels in the mesentery and retroperitoneum hyperintense on T2-weighted images	lymphangectasia	recurrent abdominal pain, multiple episodes of melena, pedal edema, pallor and mild hepatosplenomegaly	abdominal magnetic resonance imaging (MRI), abdominal ultrasonography, double-balloon enteroscopy and biopsy	conservative management: low-fat and high-protein diet
Lin RJ, Zou H et al. [2]	38- year-old female	fundus of the stomach, peripancreatic area, mesenteric area, retroperitoneal space of the spleen, right upper quadrant of the greater omentum	multiple small cystic lesions without enhancement (TC), multiple cystic dark areas (abdominal ultrasonography)	submucosal microscopic cysts of lymphatic channels with walls composed of thin fibrous tissue	melena for 3 months, weakness for 10 days, hemoptysis for 4 months	computed tomography, abdominal ultrasonography, biopsy	distal gastric resection and Billroth II-type anastomosis	she continued to present melena, iron deficiency anemia and hypoproteinemia after the surgery
Jung SW, Cha JM et al. [7]	31-years-old woman	ascending colon, from the cecum to the hepatic flexure	multiple thumbprint-like lesions on the air contrast barium enema; clusters of round submucosal tumors with smooth surface, without ulcerations or erosions and positive to the cushion sign on the colonscopy; the EUS showed echo free cysts with a clear border and septal walls in the sbmucosal layer	submucosal cysts lined by endothelial cells, serous liquid resembling lymphatic fluid, with occasional multinucleated cells and without fat or blood cell components		air contrast barium enema, colonscopy, EUS and endoscopic biopsy	the patient was not treated with invasive treatment because she was asymptomatic
Rai P, Rao RN et al. [10]	31-years-old man	small bowel and small bowel mesentery starting from mid-jejunum to ileocecal junction	protruding submucosal lesions on the colonscopy, cystic lesions on the CT	multiple irregular dilated space lined by endothelial cells with lymphoid aggregates, filled with acellular proteinacious material and no evidence of malignant cells	recurrent melena for the last 8 years and iron deficiency	colonscopy, capsule andoscopy, contrast-enhanced CT, laparatomy with intraoperative endoscopy and endoscopic biopsy	limited ileocecal resection, end ileostomy and distal mucus fistula. After few days continuity was restored.	no gastrointestinal bleed, haemoglobin and albumin were normalised
Hwang SS, Choi HJ et al. [11]	71-year-old man	jejunal and adjacent mesentery	multiple nodular mesenteric masses infiltrating into the jejunum and adjacent mesentery; multiloculated cystic lesion from the mucosa to the subsierosa	numerous multiloculated, cystically dilated spaces lined by attenuated endothelium that appeared to dissect through the muscolaris propria of the small intestine with inside fluid containing lymphocytes		computed tomography, 18FDG PET/CT, biopsy	complete surgical resection of the segment involving the lesions
Ilhan M, Oner G et al. [12]	43-years-old woman	ileum and jejunum	diffuse wall thickness (CT)	expanded cystic vascular lesions, partly extending to the intestinal mucosa and subserosa	weakness, swelling in leg, weight loss, pretibial edema and recurrent upper respiratory infections	colonscopy, abdominal ultrasound, computed tomography, PET-CT and biopsy	resection of the affected part of ileum and end-to-end anastomosis; lymph node in the mesentery of 35–45 cm to the proximal terminal ileum were excised	after 1 month surgery pretibial edema was non seen, protein and albumin increased
Chung WC, Kim HK et al. [13]	48-years-old man	proximal transverse colon	several protruding mucosal lesions covered with normal mucosa on the colonscopy	cystic lesions with a lumen covered by a single layer of flat endothelial cells	abdominal discomfort and anemia	colonscopy, abdomen CT, biopsy	endoscopic mucosectomy	the patient had abdominal pain and anemia when he was followed up 3 month after musectomy
Lee JS, Kim GW et al. [14]	38 year-old man for a general check-up	mid-portion of the ascending colon up to the proximal portion of the tansverse colon	variably sized cystic mass lesions	normal colonic mucosa and markedly dilated lymphatic vessels in the submucosa positive at immunohistochemical staining for CD34 and D2-40 (marker of vascular endothelium and lymphatic endothelium)		chest and abdominal radiography, esophagogastroduodenoscopy, colonscopy, abdominal ultrasonography, CT and biopsy	several incisions and excisional biopsies	no complications such as bleeding or protein-losing enteropathy were noticed
Fang JF, Qiu LF et al. [15]	57-years-old woman	small intestine, 30 cm distal to the flexor tendon	mass with ulcers and erosion approximately of 5 cm × 4 cm	intrinsic layer of dilated lymphatic vessels and a small amount of interstitial neutrophil, eosinophil, plasma cell infiltration	recurrent melena for more than 2 months	gastroscopy, enteroscopy, and biopsy	partial resection of the small intestine	during the follow-up no recurrence was observed
Dong A, Zhang L et al. [16]	22-years-old female	mesentery and ileum	mass involving mesentery and ileum with nodules in the mass	proliferation and dilation of the mucosal lymphatic, containing a large amount of red blood cells. The cells were positive for CD31, CD34 and D2-40. Ki-67 was about 1%.	9-month history of intermittent melena, weakness and palpitation	abdominal MR, abdominal CT, PET-CT and biopsy	resection of the abdominal mass and a segment of 60 cm of the ileum invaded by the abdominal mass	after surgery symptoms improved and follow-up laboratory tests showed normal red blood cell count and hemoglobin level
Lu G, Li H et al. [17]	79-year-old man	sigmoid colon	multiple cystic masses (colonscopy), with spetal walls in the submucosal layer	cysts located in the submucosal layer surrounded by flat endothelial cells that were positive for D2-40 at the immunoistochemistry	intermittent attacks of bowel bleeding and abdominal discomforts for 3 months	colonscopy, endoscopic ultrasound and biopsy	laparoscopy-assisted partial sigmoid colon resection	in the 2-year follow-up after the operation, no bleeding or other complications were noticed
Xue L, Guo WG et al. [18]	58-year-old man	lower esophagus	longitudinally protruding mass covered with normal esophageal mucosa and a lesion outside but adjacent to the wall of the esophagus	multiple dilated lymphatic vessels of a different sizes filled with pink beneath squamous epithelium	dysphagia of 7 months	esophagogastroscopy, esophageal ultrasonography, chest CT and biopsy	a right lateral thoracotomy was performed fot the resection of the cysts, first the lesion outside and than that protruding in to the esophageal lumen	the postoperative course was uneventful and at the patient was discharged on th 10th postoperative day