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Year 2020

Volume: 7 , Issue: 2

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Gupta, Choraria, Tiwari, Tikeykar, and Chaudhary: Pyloric gland adenoma of gallbladder with squamoid morules in pediatric age


Case Report

A 7 year old girl presented at our center with complaints of pain in upper abdomen on and off since 2 months. She was being treated with pain killers, but with no relief. An ultrasonography of the abdomen was performed which revealed a 2 x 1.3 x 0.83 cm echogenic polypoidal mass which had a vascular pedicle [Figure 1]. A low impedance flow was observed in the mass. An MRCP was performed which revealed an endophytic polypoidal T2 hypotense lesion measuring approximately 1.8 x 1.3 x 1.2 cm in size. It was found to be arising from the anterior fundal wall showing mild homogenous post contrast enhancement [Figure 2]. The pericholecystic fat planes were maintained. There was no evidence of biliary obstruction. A likely diagnosis of gall bladder adenoma was made.

A standard four port Laparoscopic Cholecystectomy was performed and the specimen was retrieved in plastic bag through the umbilical port. It was sent for histopathological examination and immunohistochemistry. No peritoneal drain was used. The patient was discharged on 2nd post operative day. No post operative complications were observed.

On gross examination the gall bladder measured 4.5 cm x 1.5 cm x 1 cm with a wall thickness of 0.3 to 0.4 cm. A polypoidal superficial growth was identified 2 cm away from the cystic duct margin measuring 1.5 x 1 x 1 cm with a tiny 0.2 to 0.3 cm stump (attachment to wall).

Microscopic examination of the specimen showed a benign appearing papillary-cystic glandular growth with minimal atypia situated superficial to the wall of gall bladder. No wall invasion or high grade dysplasia could be identified. Also present were several “squamoid morules” which consisted of vague spindle cell whorls without keratinization [Figure 3]. As per these features it was labeled as adenoma.

Immunohistochemical studies showed positive CK 7, CK 19, CEA, Beta- Catenin. CDX2 was positive in few cells and Ki-67 was also positive (8-10%). CK20 and p53 were negative [Figure 4]. As per these features it was labeled as Pyloric Gland Adenoma with squamoid morules with no evidence of malignancy.

Figure 1

Ultrasonography showing gall bladder polyp with vascular pedicle

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Figure 2

MRCP transverse and coronal views, white arrows showing polyp

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Figure 3

A–D): H&E sections show morphology of a gall bladder pyloric gland like tubular adenoma with squamoid morules. The poypoidal lesion displays tallcolumnar epithelium with very low grade adenomatous features and incospicuous mitotic activity. There are few prominent squamoid morules and no evidence of necrosis; E): KI67 displays proliferation index of 8- 10%; F): P53 is negative excluding malignancy

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Figure 4

A): Beta Catenin –nuclear positive; B): CDX2 –few cells positive; C): CEA- negative; D): CK7 – positive; E): CK19- negative; F): CK20- negative

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Discussion

Gall bladder polyps are seen in 5% adult population of the world but rarely seen in children. Incidence has greatly increased by widespread use of ultrasonography.1, 2 Investigators have observed malignant change on follow up of some of these adenomas and consider them as precancerous lesions.3 Adenocarcinomas of gall bladder have been known to have poor survival. There are no specific signs and symptoms for its early diagnosis and hence it is necessary to differentiate between benign and malignant polyps.1, 4

Kurt Elster first described Pyloric gland adenoma, but since 1990 pyloric gland adenoma has been categorized in WHO classification of gastric tumors as a distinct neoplastic entity.5, 6, 7

Previous literature reports pyloric gland adenomas are mostly seen in the stomach (69%), followed by gallbladder (14%), duodenum (12%), oesophagus, gastroesophageal junction, bile duct, pancreatic duct and rectum (together <5%).6, 7, 8, 9, 10, 11, 12, 13, 14, 15, 16, 17, 18

Most of the patients with gall bladder adenomas are adult females. The mean age group is approximately 70 years. More than half (58%) of them present with gall stones. Majority adenomas (91%) are single.19 The reported mean tumour size is 0.6-3.5 cm.6, 7, 8, 9, 17, 18, 10, 11, 20, 12, 13, 14, 15, 16

A large study of 165 cases of pyloric gland adenomas showed 28% contained squamoid morules and 2% contained columnar oxyphil cells. 27% of them carried high-grade dysplasia/carcinoma in situ and low-grade dysplasia was found in 15% of the cases. However, only 1% invasive adenocarcinomas, both of intestinal type were diagnosed in pyloric gland adenomas. Their results indicate minor role in pathway of gallbladder carcinogenesis.19 Literature suggests the risk of malignant transformation into adenocarcinoma in up to 47% of cases of all locations.7 The pyloric gland adenoma may lead to the sequence of metaplasia-dysplasia-carcinoma in gallbladder and bile duct carcinogenesis.21, 22 Literature suggests gastric metaplasia in gallbladder, pancreas, rectum and duodenum are associated with pyloric gland adenomas.8, 17, 15, 16, 22 In a study of 3 cases of adenocarcinomas of extrahepatic bile ducts, 2 cases had adjacent pyloric gland metaplasia which suggests its precancerous nature.19 Comparative genomic hybridization analyses suggest pyloric gland adenoma has a precancerous nature explained by adenoma-adenocarcinoma sequence with a great potential for invasive malignancy.23 Pathologically PGA of GB is low grade in 70.8% and high grade/carcinomas in 29.2% of cases. Immunohistochemically, MUC6 is diffusely positive whereas MUC2, MUC5AC, and CDX2 are only focally positive. A study suggested unique histology, phenotype and molecular status of PGA of GB when compared with other sites like stomach, duodenum and pancreas. The mutation of CTNNB1 was seen in 100% and KRAS in 4.2% cases.24 Ki67 expression and p53 mutations also suggest malignant transformation.7

For symptomatic patients who have pain and dyspepsia, cholecystectomy is the recommended treatment. For asymptomatic or incidentally detected patients, the indications for cholecystectomy should be age more than 50 years, solitary polyp greater than 10mm in largest dimension, accompanying gall stones and increase in size on serial sonographies. If the polyp shows no increase in size, no signs of malignancy and is 6-9mm in size, it should be followed up 6 monthly for at least a year.25

Conclusion

As per our knowledge the case we report is the first case of gallbladder pyloric adenoma with squamoid morules in pediatric age group. Treatment with simple cholecystectomy is recommended in view of literature suggesting pre malignant lesion.

Compliance with ethical standards

This work is in compliance with ethical standards with following below mentioned details

Source of Funding

The authors declare no funding was obtained.

Conflict of Interest

The authors declare that they have no conflict of interest.

Informed Consent

Written informed consent was taken from participant of the study for publication of details and photographs regarding her case.

References

1 

Robert P Myers Eldon A Shaffer Paul L Beck Gallbladder Polyps: Epidemiology, Natural History and ManagementCan J Gastroenterol200216318794

2 

H Ito L E Hann D Angelica M Polypoid lesions of the gallbladder: diagnosis and followupJ Am Coll Surg2009208570575

3 

Sadao Kozuka Mikio Tsubone Akihiro Yasui Kitao Hachisuka Relation of adenoma to carcinoma in the gallbladderCancer19825010222634

4 

A Gupta A Choraria S Tiwari Laparoscopic radical cholecystectomy for carcinoma gallbladder: A case seriesInt J Hepatobiliary Pancreat Dis201771822

5 

F T Bosman F Carneiro R H Hruban WHO classification of tumours of the digestive systemInternational Agency for Research on CancerLyon2010

6 

Z M Chen J R Scudiere S C Abraham Pyloric gland adenoma: an entity distinct from gastric foveolar type adenomaAm J Surg Pathol20093318693

7 

Michael Vieth Ryoji Kushima Ken-ichi Mukaisho Rie Sakai T. Kasami Takanori Hattori Immunohistochemical analysis of pyloric gland adenomas using a series of Mucin 2, Mucin 5AC, Mucin 6, CD10, Ki67 and p53Virchows Archiv2010457552936

8 

Bradley W. Bakotic Morton J. Robinson Patrick D.J. Sturm Ralph H. Hruban G. Johan A. Offerhaus Pyloric Gland Adenoma of the Main Pancreatic DuctAm J Surg Pathol199923222731

9 

Noriko Kato Sunao Akiyama Teiichi Motoyama Pyloric gland-type tubular adenoma superimposed on intraductal papillary mucinous tumor of the pancreasVirchows Archiv200244022058

10 

Ryoji Kushima Michael Vieth Ken-ichi Mukaisho Rie Sakai Hidetoshi Okabe Takanori Hattori Pyloric gland adenoma arising in Barrett’s esophagus with mucin immunohistochemical and molecular cytogenetic evaluationVirchows Archiv2005446553741

11 

Yoji Wani Kenji Notohara Masayoshi Fujisawa Aberrant expression of an “intestinal marker” Cdx2 in pyloric gland adenoma of the gallbladderVirchows Archiv200845355217

12 

M G Oh S J Cho J H Lee A spongiform mass in the stomach: pyloric gland adenoma with a transition to adenocarcinomaKorean J Gastroenterol20105615

13 

Y Gutierrez-Grobe J Gavilanes-Espinar M Uribe Pyloric Gland Adenoma: Case ReportRev Gastroenterol Mex2010753602

14 

J Amaris Intraductal mucinous papillary tumor and pyloric gland adenoma of the pancreasGastrointest Endosc2002564414

15 

R. Kushima W. Remmele M. Stolte F. Borchard Pyloric gland type adenoma of the gallbladder with squamoid spindle cell metaplasiaPathol Res Pract199619299639

16 

R. Kushima H. J. Rüthlein M. Stolte M. Bamba T. Hattori F. Borchard ’Pyloric gland-type adenoma' arising in heterotopic gastric mucosa of the duodenum, with dysplastic progression of the gastric typeVirchows Archiv199943544527

17 

M. Vieth R. Kushima J. P. A. de Jonge F. Borchard F. Oellig M. Stolte Adenoma with gastric differentiation (so-called pyloric gland adenoma) in a heterotopic gastric corpus mucosa in the rectumVirchows Archiv200544655425

18 

M. Vieth R. Kushima F. Borchard M. Stolte Pyloric gland adenoma: a clinico-pathological analysis of 90 casesVirchows Archiv2003442431721

19 

Jorge Albores-Saavedra Fredy Chablé-Montero Marco Aurelio González-Romo Manuel Ramírez Jaramillo Donald E. Henson Adenomas of the gallbladder. Morphologic features, expression of gastric and intestinal mucins, and incidence of high-grade dysplasia/carcinoma in situ and invasive carcinomaHuman Pathol2012439150613

20 

D. Golger A. Probst T. Wagner H. Messmann Pyloric-gland adenoma of the stomach: case report of a rare tumor successfully treated with endoscopic submucosal dissectionEndosc200840S 02E110E1

21 

Jorge Albores-Saavedra Fredy Chablé-Montero Nahum Méndez-Sánchez Miguel Ángel Mercado Mario Vilatoba-Chapa Donald E. Henson Adenocarcinoma with pyloric gland phenotype of the extrahepatic bile ducts: a previously unrecognized and distinctive morphologic variant of extrahepatic bile duct carcinomaHuman Pathol2012431222928

22 

Ryoji Kushima Michael Vieth Franz Borchard Manfred Stolte Ken-ichi Mukaisho Takanori Hattori Gastric-type well-differentiated adenocarcinoma and pyloric gland adenoma of the stomachGastric Cancer20069317784

23 

Inga-Marie Schaefer Silke Cameron Peter Middel Kia Homayounfar Harald Schwörer Michael Vieth Pyloric gland adenoma of the cystic duct with malignant transformation: report of a case with a review of the literatureBMC Cancer2012121570

24 

C He Y Fukumura A Toriyama Pyloric Gland Adenoma (PGA) of the Gallbladder: A Unique and Distinct Tumor from PGAs of the Stomach, Duodenum, and PancreasAm J Surg Pathol2018429123745

25 

Åke Andrén-Sandberg Diagnosis and management of gallbladder polypsN Am J Med Sci20124520311



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