Review Article

An Update on Clinicopathological and Molecular Features of Plexiform Fibromyxoma

Table 1

Clinical features of cases reported as PF or PAMT in the literature.

NoYearAuthorPopulationAgeSexClinical PresentationUlcerLocationSize (cm)DiagnosisTreatmentPrognosis

1[1]2007TakahashiJapan50Macute abdominal pain (perforation)+pyloric antrum4 × 4 × 2.5PAMTdistal gastrectomyNA

2[1]2007TakahashiJapan68Mincidental-pyloric antrum4.5 × 3.5 × 3.0PAMTpartial gastrectomyuneventful for 12 months

3[17]2008GalantBelgium61Mhematemesiserosionantrum3.7PAMTpartial gastrectomyuneventful for 6 months

4[18]2008RauGermany50Fmorning nausea+antrum1.9 × 1.8 × 0.8PAMTMedian laparotomy and local excisionNo recurrence for 3 months

5[9]2008YoshidaUSA19Fmass in the stomach-antrum4.5 × 3.5 × 3.0PAMTdistal gastrectomyuneventful for 9 months

6[9]2008YoshidaUSA46Mupper gastrointestinal bleeding+antrum3.5PAMTdistal gastrectomyuneventful for 4 months

7[11]2009MiettinenUSA38Fupper gastrointestinal bleeding, ulcer+antrum, lesser curvature3 × 2PFdistal gastrectomylost f/u

8[11]2009MiettinenUSA62Mprogressive weight loss for months-antrum, pyloric4 × 4PFpartial gastrectomy, omentectomylost f/u

9[11]2009MiettinenUSA75Funknown+antrum, serosa5PFsubtotal gastrectomydied of unknown cause, 2 months

10[11]2009MiettinenUSA65Fweight loss, gastric ulcer+antrum, lesser curvature, duodenal bulb5 × 4.5 × 2.5PFpartial gastrectomydied of unknown cause, 14.5 years

11[11]2009MiettinenUSA33Manemia, weakness+antrum, anterior wall5.5 × 3.5 × 3.5PF50% distal gastrectomyalive without disease for 19.7 years

12[11]2009MiettinenUSA43Mgastrointestinal bleeding+antrum, near pylorus5.5 × 4.5 × 4.5PFpartial gastrectomyalive without disease for 18.4 years

13[11]2009MiettinenUSA56Funknown-pylorus, duodenal bulb5.5+3PFpartial gastrectomyalive without disease for 19.9 years

14[11]2009MiettinenUSA50Mgastric outlet obstruction-antrum, duodenal bulb7 × 6 × 6PFdistal gastrectomydied of unknown cause, 25.5 years

15[11]2009MiettinenUSA21Msyncope, anemia+antrum, prepyloric, duodenal bulb9 × 6 × 5PFantrectomy, B1-reconstructionalive with unknown status for 22 years

16[11]2009MiettinenUSA16Fhematemesis+antrum, pylorus10 × 9 × 6PFdistal gastrectomyalive with unknown status for 3 years

17[11]2009MiettinenUSA30Fnonhealing gastric ulcer+antrum10 × 9 × 6PFdistal gastrectomyalive without disease for 9 years, alive with unknown status for 24 years

18[11]2009MiettinenUSA7Femesis, diarrhea, protruding abdominal mass-antrum, pylorus, duodenal bulb15 × 11 × 8PFexcision of tumor, gastric wall resection at the tumor attachmentlost f/u

19[19]2009PailoorMalaysia23Fmelena+antrum8.0 × 4.0PAMTpartial gastrectomyuneventful for 2 months

20[20]2010SingSouth Africa (Indian)35Fcushingoid appearance, amenorrhea-pyloro-antral region, anterior wall of the greater curvature4 × 3 × 2PAMTexploratory laparotomy and wide local excisionuneventful for 12 months

21[10]2010TakahashiJapan23Mabdominal pain, abdominal discomfort, melena+antrum, duodenal bulb14 × 14 × 7PAMTpartial gastrectomyuneventful for 12 months

22[21]2010TanAustralia (Filipino)34Mabdominal mass, abdominal discomfort, decreased appetite (perforation)+antrum3.5 × 3.4 × 2.5PAMTdistal gastrectomyuneventful for 2 months

23[22]2010WangChina54Fabdominal distension, heartburn, hiccup, regurgitation, loss of appetiteerosionfundus1.5 × 1.2PAMTendoscopic resectionuneventful for 6 months

24[23]2010CooperUSA64Mepigastric painNAantrum4.0 × 2.0PAMTlaparoscopic excisionuneventful for 3 years

25[24]2010CuiChina54Fabdominal distension, decreased appetiteerosionfundus0.8PAMTNANA

26[14]2011KimKorea52Mdyspepsia+antrum3.5 × 2.3PAMTlaparoscopic wedge resectionuneventful for 5 months

27[25]2012KangKorea47Mincidental+mid body, posterior wall and greater curvature3 × 2PAMTwedge resectionuneventful for 6 years

28[25]2012KangKorea63Fincidental+lower body, greater curvature2.2 × 1.6PAMTendoscopic resectionuneventful for 1 month

29[26]2012SchulzGermany59Mheartburn, upper abdominal pain+pylorus1.5PAMTcombined laparoscopic endoscopic local resectionNA

30[27]2012CaiChina32MincidentalNAantrumPFdistal gastrectomyNA

31[27]2012CaiChina47Fupper abdominal painNAantrumPFradical distal gastrectomyNA

32[28]2012WangChina12Mgastrointestinal bleedingNAantrumPFpartial gastrectomyuneventful for 84 months

33[29]2012MiettinenUSA49FNANAantrum3.5 × 2.5 × 2.5PFwedge resectionNA

34[30]2012LiChina47Fepigastric discomfort, abdominal pain-antrum5 × 3 × 2PAMTlaparoscopic distal gastrectomyuneventful for 1.5 years

35[31]2012BiChina31MAbdominal pain-Antrum8.0PAMTNANA

36[31]2012BiChina47FAbdominal pain+Antrum4.5PAMTNANA

37[31]2012BiChina42FAbdominal distension-Antrum4.6PAMTNANA

38[32]2013KaoChina53FAbdominal distension, abdominal pain, nausea, acid regurgitationNANANAPAMTNANA

39[33]2014BaekKorea38Fincidental+upper body, posterior wall and greater curvature3.5 × 2.3PAMTwedge resectionuneventful for 6 months

40[34]2014DuckworthUSA16Fchest pain, shortness of breath, finger numbnessNAposterior mediastinum near the esophagus at the level of the carina3.2PFthoracoscopic resectionuneventful for 14 months

41[34]2014DuckworthUSA11Fsevere iron-deficiency anemia+pylorus3.5PFlaparoscopic distal gastrectomyuneventful for 15 months

42[35]2014IkemuraJapan27Fepigastric pain, melena, anemia+antrum of the lesser curvature side4.6 × 3.0 × 2.8PAMTpartial gastrectomyuneventful for 40 months

43[36]2014LeeHong Kong (Filipino)42Fabdominal pain, fever, anemia (fistulating abscess formation)+antrum12.9 × 11.9 × 10.6PFdistal gastrectomyuneventful for 3 weeks

44[37]2014LiChinese32Mincidental-antrum, anterior wall3.4 × 3.0PAMTpartial gastrectomyuneventful for 3 years

45[38]2014SakamotoJapan60Mepigastralgia+antrum2PFlaparoscopic partial gastrectomyuneventful for 12 years

46[39]2014LiChina73Fupper abdominal painNAantrumPFpartial gastrectomyNA

47[40]2014TianChina64Mupper abdominal discomfort-antrum3.3 × 2.5PFdistal gastrectomyuneventful for 6 months

48[41]2015BanerjeeIndia19Fdull upper abdominal pain, lump in the right hypochondrium+duodenum (D1), posterior13.8 × 8.6PAMTdistal gastrectomy, proximal duodenectomy, Billroth II gastrojejunostomyuneventful for 6 months

49[42]2015FassanItaly (Caucasian)55FcholelithiasisNAgallbladder1PFlaparoscopic cholecystectomyno postsurgical complication

50[43]2015LuChina26Fprogressive abdominal distension, nausea, vomiting, melenaNAantrumNAPAMTdistal gastrectomyNA

51[44]2015NiChina21Fmelena, dizziness, anemia+antrum, greater curvature4 × 3 × 3PFdistal gastrectomyuneventful for 3 years

52[45]2015WeiChina50Fupper abdominal painNAantrumPFsubtotal gastrectomyuneventful for 3 months

53[46]2015YueChina34Mupper abdominal painNAantrumPFdistal gastrectomyuneventful for 24 months

54[46]2015YueChina50Fupper abdominal painNAantrumPFsubtotal gastrectomyuneventful for 3 months

55[47]2015XuChina50Fabdominal painNAgastric body3 × 2PAMTTotal gastrectomyuneventful for 6 months

56[15]2016DixitIndia51Fabdominal pain, vomiting, weight loss (with synchronous ovarian neoplasms)+pylorus8.4 × 6.1 × 5PAMTdistal gastrectomyno postsurgical complication

57[48]2016InoueJapan36Fepigastric pain, anemia+antrum2.5 × 2.2 × 2.0PFradical resection with laparoscopic endoscopic cooperative surgeryuneventful for unknown period

58[49]2016JonaitisLithuania (Caucasian)28Fepigastric pain associated with meals, iron-deficiency anemia, weight loss+antrum, anterior wall3PAMTpartial gastrectomy of the Billroth I typefollow-up not reported; gastroduodenal anastomositis, resolved with conservative treatment

59[50]2016KaneUSA (Vietnamese)28Facute, severe abdominal pain and worsening anemia+antrum5.5 × 3.5PFdistal gastrectomy with a Roux-en-Y gastrojejunostomydyspepsia 23 months thereafter, otherwise uneventful for 23 months (only moderate stenosis of anastomosis)

60[51]2016MorrisUSA9Fintermittent abdominal pain, nausea, vomiting, weight loss+antrum, posterior5PFlaparotomic anterior gastrotomy with tumor resectionuneventful for 6 months

61[52]2016NagahisaJapan39Mepigastric pain+antrum, anterior wall3.5 × 3PAMTpartial gastrectomy with laparoscopic endoscopic cooperative surgeryuneventful for 9 months

62[53]2016QueroItaly47Msyncope, regurgitation, epigastric discomfort+antrum6.5PAMTdistal gastrectomyuneventful for 10 months

63[54]2016SpansBelgium19MNANAantrum5.5PFNANA

64[54]2016SpansBelgium65FNANAantrum4.3 × 3.0 × 1.7PFNANA

65[54]2016SpansBelgium58FNANAantrumNAPFNANA

66[54]2016SpansBelgium51FNANAantrum9.0 × 8.5 × 5.5PFNANA

67[54]2016SpansBelgium63FNANAjejunum, proximal3.5 × 3.0 × 2.0PFNANA

68[54]2016SpansBelgium76FNANAstomach4PFNANA

69[54]2016SpansBelgium62FNANAstomachNAPFNANA

70[54]2016SpansBelgium30FNANAstomach2PFNANA

71[54]2016SpansBelgium28MNANAgastric body10 × 6 × 3PFNANA

72[54]2016SpansBelgium44FNANAstomachNAPFNANA

73[54]2016SpansBelgium18FNANAantrum4.5 × 3.5 × 2.7PFNANA

74[54]2016SpansBelgium19FNANAstomach4.5PFNANA

75[54]2016SpansBelgium46MNANAantrum3.5PFNANA

76[54]2016SpansBelgium29MNANAantrum6.5 × 4.5 × 4.0PFNANA

77[54]2016SpansBelgium36FNANAantrum8PFNANA

78[54]2016SpansBelgium47FNANAantrum4.5 × 3.7PFNANA

79[55]2016LiChina11Mupper abdominal discomfortNAantrumPFpartial gastrectomyuneventful for 12 months

80[56]2016LiChinese44Fincidental-antrum0.8 × 0.8PAMTendoscopic submucosal dissectionuneventful for 6 months

81[57]2016ZhangChina48Mupper abdominal painNAantrumPFpartial gastrectomyuneventful for 12 months

82[16]2017AkaiJapan55MincidentalNAgastric angle1.7PAMTlaparoscopic partial gastrectomyNA

83[58]2017Gonzalez-CorderoSpain37Mupper gastrointestinal bleeding with hemodynamic instabilityNAantrum5.8 × 5 × 4PFantrectomyuneventful postoperatively

84[59]2017HuChina26Mabdominal distension+fundus1.8PFendoscopic submucosal dissectionuneventful for 32 months

85[59]2017HuChina31Fabdominal distension-antrum2.5PFendoscopic submucosal dissectionNA

86[59]2017HuChina72Fabdominal distension+antrum7PFdistal subtotal gastrectomyuneventful for 56 months

87[59]2017HuChina59Fabdominal distension+antrum2.5PFdistal subtotal gastrectomyuneventful for 68 months

88[59]2017HuChina52Mincidental+fundus1.2PFendoscopic submucosal dissectionuneventful for 24 months

89[59]2017HuChina59Fmelena, anemia+antrum3PFdistal subtotal gastrectomyuneventful for 40 months

90[59]2017HuChina48Mabdominal distension+antrum3PFdistal subtotal gastrectomyuneventful for 95 months

91[59]2017HuChina58Mabdominal pain+antrum3.5PFdistal subtotal gastrectomyuneventful for 34 months

92[59]2017HuChina46Mabdominal distension-antrum4PFdistal subtotal gastrectomyuneventful for 65 months

93[59]2017HuChina40Fmelena, anemia+antrum3.8PFdistal subtotal gastrectomyuneventful for 70 months

94[60]2017KawaraJapan66Mgastric tumor-antrum4 × 3PFendoscopic submucosal dissectionuneventful for 12 months

95[61]2017KimKorea51Mincidental-antrum, lesser curvature2.0 × 1.5PAMTlaparoscopic gastric wedge resectionuneventful for 3 years

96[62]2017LiangChina11Mright epigastric discomfort with episodic pain-pylorus, anterior17 × 10.5 × 5PAMTpartial gastrectomyuneventful for 12 months

97[63]2017MorisUSA63Mupper gastrointestinal bleeding, anemia, melenaNAduodenum3 × 3PFpancreas-preserving duodenectomy of the first 2 portionsuneventful for 20 months, complicated only by a low output pancreatic skin fistula totally drained after 4 months

98[64]2017QiChina42Mmelena+antrum, greater curvature3 × 2 × 1.8PFlaparoscopic partial gastrectomyuneventful for 12 months

99[64]2017QiChina66Mepigastric pain-antrum, greater curvature2 × 1 × 0.7PFendoscopic submucosal dissectionuneventful for 12 months

100[65]2017SzurianAustria (Caucasian)16Fanemia, nausea+antrum, anterior wall6.5PFdistal gastrectomy and retrocolic gastrojejunostomy (Billroth II)pulmonary embolism postoperatively and discharged on day 9, otherwise uneventful for 6 months

101[65]2017SzurianAustria34Mepigastric discomfort, flatulence+pylorus1.6PFdistal gastrectomy and retrocolic gastrojejunostomy (Billroth II)uneventful for 16 months

102[66]2017WamburaTanzania (Tanzanian)41Fanemia, epigastric discomfort, melena+antrum5.5 × 5PFdistal gastrectomy with Roux-en-Y gastrojejunostomyuneventful for 12 months

103[67]2017YangChina50Fabdominal pain-gastric upper body15 × 10PFlaparoscopic partial gastrectomyuneventful for 2 years

104[68]2017ZhouChina51Mepigastric discomfort, heartburnNAantrum, greater curvature4 × 3 × 1.8PFtotal resectionuneventful for 15 months

105[69]2017WangChina66FAbdominal pain, melena, dizziness, abdominal distensionerosionAntrum3PFNANA

106[70]2018DjurićSerbia14Miron-deficiency anemia, fatigue, epigastric painNAAntrumNAPFpartial gastrectomyNA

107[71]2018JangKorea47Fheartburn-pylorus2.5 × 2.0PFantrectomy with Billroth I anastomosisuneventful for 7 months

108[72]2018RohitUSA81Fweight loss of 3.6 kg in 3 monthsNAgastric body, lesser curvature2.7 × 2.4PFnon-surgical follow-upsymptom resolved 3 months after

109[73]2018WangChinese52Fupper abdominal pain+mid gastric body, greater curvature1.5 × 1.0 × 1.0PFgastroscope-assisted laparoscopic wedge resectionuneventful for 10 months

110[74]2018ZhangChina31Fhematochezia, syncope, upper gastrointestinal bleeding, anemia+upper segment of jejunum1.2 × 1.0PFexploratory laparotomy and resection of the upper jejunal tumor and local intestineuneventful for 3 years

1112019WuTaiwan (Taiwanese)59Facid regurgitation-antrum2 × 1.2 × 1.2PFpartial gastrectomyuneventful for 20 months

112[75]2019FukazawaJapan14Fabdominal pain, hematemesis+antrumPFpartial gastrectomyuneventful for 16 months

113[76]2019BanerjeeUSA65Manemia, early satiety, dyspepsia, hematemesis+antrum5 × 2.3PFwedge resectionNA

114[77]2019LaiUSA21FNANAgastric bodyPFpartial gastrectomy4 years

115[77]2019LaiUSA42FNA+antrumPFpartial gastrectomy4 years

116[77]2019LaiUSA79FNANAantrumPFpartial gastrectomy1 year

117[77]2019LaiUSA33MNANAantrumPFpartial gastrectomy11 years

118[77]2019LaiUSA60MNANAantrumPFpartial gastrectomy1 year

119[77]2019LaiUSA77MNANAgastric bodyPFpartial gastrectomy2 years

120[77]2019LaiUSA45MNANAantrumPFonly endoscopic biopsy0.2 years

121[78]2019LiChina5Mpale complexionNAantrum8.2 × 7.5 × 5.5PFdistal gastrectomy36 months