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Ectopic pancreas, gastric, duodenal and colonic tissue in a case of persistent umbilical discharge: Report of two patients with review of literature


1 Department of Pathology and Lab Medicine, All India Institute of Medical Sciences (AIIMS), Bhubaneswar, Odisha, India
2 Department of Pediatric Surgery, All India Institute of Medical Sciences (AIIMS), Bhubaneswar, Odisha, India

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Date of Submission28-May-2021
Date of Decision12-Jul-2021
Date of Acceptance13-Jul-2021
Date of Web Publication26-May-2022
 

   Abstract 


One of the typical complaints in the pediatric population is umbilical discharge. Among the congenital causes, remnants of omphalomesenteric duct or patent urachus are often detected. On a few occasions, multiple types of ectopic tissue are present. We describe histopathologic findings of two cases reported recently at our center as pediatric umbilical lesions with associated ectopic tissue. Histopathology of the excised mass confirmed the patent omphalomesenteric duct with ectopic gastric, duodenal, and colonic mucosa and pancreatic tissue in two patients with the clinical presentation of umbilical discharge. There were no associated congenital anomalies in these patients. The presence of multiple ectopic gastrointestinal mucosa and pancreas in the umbilical mass is unusual. Herein, we report these cases because of its rarity, multiple ectopic tissues, and reviewing the literature of the reported cases of multiple ectopic tissues.

Keywords: Colonic mucosa, ectopic tissue, gastric tissue, omphalomesentric duct, pancreatic tissue


How to cite this URL:
Ayyanar P, Tripathy BB, Pati AB, Mohanty MK, Sable M. Ectopic pancreas, gastric, duodenal and colonic tissue in a case of persistent umbilical discharge: Report of two patients with review of literature. Indian J Pathol Microbiol [Epub ahead of print] [cited 2023 Feb 5]. Available from: https://www.ijpmonline.org/preprintarticle.asp?id=345878





   Introduction Top


Umbilical discharge is one of the common complaints among the pediatric population, and the common causes are omphalitis, umbilical granuloma, and congenital anomalies, which include remnants of the omphalomesenteric duct (OMD) or vitellointestine duct and patent urachus. Among these causes, remnants of OMD account for 2% in infants.[1] OMD cyst containing gastric mucosa has been reported.[2],[3] Ectopic pancreas at the umbilicus in the OMD remnant has also been reported in the literature.[4],[5] Bromberg et al.[6] have reported a total of 18 cases of the heterotopic pancreas with umbilical discharge in the adult age group, including five cases of the associated ectopic small intestine and/or gastric mucosa. Ectopic colonic mucosa in cases of the umbilical lesion has not yet been reported to date. The presence of gastrointestinal tissues (gastric and/or small intestinal mucosa) and pancreas is an unusual finding which was described previously in only two cases.[7],[8] We report another two cases of multiple ectopic tissues, which were presented as an umbilical mass with discharge.


   Case Details Top


Case 1

A three-year-old female child presented to the outpatient department with intermittent watery and thick purulent discharge from the umbilicus since birth. The baby was otherwise asymptomatic. On examination, a 2 × 2 cm size reddish polypoidal lesion at the umbilicus with the serous discharge was detected. Intraoperatively there was a 2 × 2 cm sub umbilical cyst, without any obvious intraabdominal connection, and it was excised. Grossly we received a single fragment of tissue, measuring 2 × 1.8 × 0.6 cm. There was no sinus tract. Microscopic examination showed a fragment of tissue lined by stratified squamous epithelium [Figure 1]a. The underlying tract was lined by gastric mucosa containing surface epithelium, lamina propria with fundic type glands, and muscularis mucosae [Figure 1]b and [Figure 1]c. The fibrocollagenous stromal tissue showed lobules of pancreatic acini [Figure 1]d with ductules [Figure 1]e and islets of Langerhans [Figure 1]f. Marked fibrosis was present. No granuloma or any evidence of malignancy was identified. We reported it as umbilical sinus with ectopic gastric and pancreatic tissue.
Figure 1: Scanning magnification of umbilical lesion covered by stratified squamous epithelium (1a, H and E 40×), the underlying tract was lined by gastric mucosa containing surface epithelium, lamina propria and thickened muscularis mucosae (1b, H and E 40×), edema of the lamina propria and gastric fundic-type glands containing parietal cells (black arrow) and chief cells (1c, H and E 400×), the underlying stroma showed lobules of pancreatic acini (1d, H and E 40×), lobules of acini with central ductules (black arrow) (1e, H and E 400×). Islets of Langerhans (black arrow) (1f, H and E 400×)

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Case 2

A one-year-old female child presented to us with a swelling at the umbilicus associated with discharge since birth. It was thin watery, and was odorless. There was a history of occasional bleeding from the swelling on trauma. A clinical diagnosis of umbilical granuloma was made, and it was excised under anesthesia [Figure 2]a. There was no intraperitoneal extension from the swelling. Grossly it was a single tissue of size 2 × 1 × 0.8 cm, attached to the skin, and the entire tissue was processed. Microscopically, we noticed a fragment of tissue lined by small bowel mucosa containing Brunner gland (duodenal mucosa) [Figure 2]b and [Figure 2]e and colonic type mucosa [Figure 2]c and [Figure 2]d. There was ulceration of the surface epithelium, and underlying lamina propria showed a moderate degree of chronic inflammation [Figure 2]c. The underlying stroma showed lobules of pancreatic acini, ducts, and the islet of the pancreas [Figure 2]f. There was no granuloma or malignancy. We reported it as a patent vitellointestinal duct with the ectopic pancreatic, duodenal and colonic tissue.
Figure 2: Intraoperative image showing whitish tissue fragment at the umbilicus (2a), scanning magnification of umbilical lesion covered by small intestinal mucosa and underlying stroma showed pancreatic parenchyma (black arrow) (2b, H and E 40×), remaining areas covered by colonic type mucosa which showed surface epithelial ulceration moderate degree of chronic inflammation, lymphoid follicle in the lamina propria (2c, H and E 40×), Colonic mucosa containing enterocytes, goblet cells and Paneth cells (black arrow) (2d, H and E 400×), Brunner glands (2e, H and E 200×), lobules of pancreatic acini, Islets of Langerhans (black arrow), and ductules (black arrow) (2f, H and E 200×)

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   Discussion Top


The prevalence of ectopic pancreas has been reported to be 0.5 to 13.7% at autopsy.[9],[10] 70 to 90% of the heterotopic pancreas occurs in the upper gastrointestinal tract.[11],[12] The Heinrich system is frequently used to classify the heterotopic pancreas. Depending upon the presence of structures of pancreatic tissue, i.e., acini, islets, and ducts, the ectopic pancreatic tissue is classified into three types.[13] Type 1 contains all three structures (acini, islets, and ducts), Type 2 contains acini and ducts only, but no islets, and Type 3 contains ducts alone. There are various theories explained for the presence of ectopic pancreatic tissue.

  1. Misplacement of embryonic tissue during organogenesis, e.g., the transplantation of embryonic pancreatic cells to adjacent structures,
  2. Metaplastic changes in one type of tissue from the endodermal origin, and
  3. Trans-differentiation of totipotent endodermal cells lining the intestine or OMD into pancreatic tissue.[14],[15],[16]


It is most often discovered in the fifth and sixth decades of life. The occurrence of heterotopic pancreatic tissue in umbilical lesions in the pediatric age group is uncommon. Only 18 pediatric cases of ectopic pancreatic tissue in umbilical lesions were found in the English literature. Six cases (33.33%), including our cases, noticed similar histomorphological features with Heinrich system I, which is the most common type. However, the rest of the cases did not mention the tissue details with specific Heinrich system typing.

The OMD or vitelline duct is the primitive connection between the yolk sac and embryonic midgut. During normal development, the duct undergoes involution between the seventh and ninth weeks of fetal development. Failure of involution or obliteration of the OMD may lead to various anomalies that present as umbilical nodule or cyst with or without discharge. The clinical presentations of symptomatic OMD remnants in children have been evaluated in a retrospective study. Those are gastrointestinal tract obstruction, acute abdomen, and umbilical anomalies. Out of these, 29% of patients presented with umbilical abnormalities characterized by prolapsed OMD, patent OMD with fecal drainage, umbilical polypoid masses, and umbilical cord hernias that contained Meckel's diverticulum. It also described that 25% of cases showed OMD cyst with the heterotopic gastric mucosa.[9] None of those cases showed umbilical discharge. Sharma et al. have also described the same. We observed ectopic gastric and pancreatic tissue in one case, and another case had ectopic duodenal and colonic mucosa with heterotopic pancreatic tissue, suggesting the remnant of the OMD. As per our search, the presence of ectopic colonic tissue in cases of umbilical mass has not yet been reported. Our second case showed colonic mucosa, along with duodenal and pancreatic tissue.

The clinicodemographic profile of multiple ectopic gastrointestinal and pancreatic tissues in cases of the umbilical lesion is shown in [Table 1], including our two cases. The majority of the patients were below two years (83.33%), and 13/18 (72.22%) of them had umbilical discharge as a presenting complaint. There are two different causes of skin ulceration (chemical dermatitis in the periumbilical area) in OMD ectopic remnants. First is the presence of ectopic gastric tissue in OMD.[2] In contrast, Sharma et al.[8] has mentioned that ectopic pancreatic secretion was the cause of skin excoriation. Our cases did not show skin ulceration, although both gastric and pancreatic tissues in OMD.
Table 1: Clinico-demographic profile of cases of umbilical lesion with pancreatic parenchyma, gastric, small intestinal and colonic epithelium reported in the English literature

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Preoperatively umbilical granuloma and discharge in the pediatric age group pose a diagnostic challenge. Umbilical discharge is widespread in children and is due to either infective or congenital causes. The congenital causes of umbilical discharge like patent urachus and omphalomesenteric duct remnant should be investigated for underlying communication with the intestine or urinary bladder. Excision of the lesion is the mainstay of treatment after a thorough radiological investigation. The excised lesion should always be examined histopathologically for the detection of causes of the umbilical lesion. Heterotopic pancreas is liable to manifest as diseases of pancreas like pancreatitis, neuroendocrine tumors, and pancreatic carcinoma. The incidence of malignant transformation has been reported as high as 12.7% in a Japanese series.[17] Hence complete clear margins excision of the heterotopic tissues should always be looked for the excised specimen.

In conclusion, the presence of multiple ectopic gastrointestinal mucosa and pancreas in the umbilical mass is unusual. We report two such cases because of its rarity, multiple ectopic tissues, and reviewing the literature of the reported cases of multiple ectopic tissues. Our report highlights the need for awareness regarding these findings and proper histopathological examination to arrive at a correct diagnosis.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
   References Top

1.
Moore TC. Omphalomesenteric duct malformations. Semin Pediatr Surg 1996;5:116-23.  Back to cited text no. 1
    
2.
Tamilselvan K, Mohan A, Cheslyn-Curtis S, Eisenhut M. Persistent umbilical discharge from an omphalomesenteric duct cyst containing gastric mucosa. Case Rep Pediatr 2012;482185. doi: 10.1155/2012/482185.  Back to cited text no. 2
    
3.
Jagtap AB, Dhongade HS. Omphalomesenteric duct cyst with heterotopic gastric mucosa presenting as persistent umbilical discharge. Indian J Pathol Oncol 2016;3:110-4.  Back to cited text no. 3
    
4.
Park E, Kim H, Jung KW, Chung JH. Heterotopic pancreas in omphalomesenteric duct remnant results in persistent umbilical discharge. Korean J Pathol 2014;48:323-6.  Back to cited text no. 4
    
5.
Zhao Z, Sim CK, Mantoo S. Heterotopic pancreas in the omphalomesenteric duct remnant in a 9 month-old-girl: A case report and literature review. Diagn Pathol 2017;12:49.  Back to cited text no. 5
    
6.
Bromberg SH, CamiloNeto C, Borges AF, Franco MI, França LC, Yamaguchi N. Pancreatic heterotopias: Clinicopathological analysis of 18 patients. Rev Col Bras Cir 2010;37:413-9.  Back to cited text no. 6
    
7.
de Silva WD, Samarasinghe MC, Dias MN, Perera CS. Ectopic gastric and pancreatic tissue: A rare cause of umbilical discharge. Ann Trop Paediatr 2010;30:73-5.  Back to cited text no. 7
    
8.
Sharma S, Maheshwari U, Bansal N. Ectopic pancreatic, gastric, and small intestine tissue in an umbilical polyp, causing persistent umbilical discharge in a 2 year old child: A rare case report. J Evol Med Dent Sci 2013;2:447-51.  Back to cited text no. 8
    
9.
Durakbasa CU, Okur H, Mutus HM, Bas A, Ozen MA, Sehiralti V, et al. Symptomatic omphalomesenteric duct remnants in children. Pediatr Int 2010;52:480-4.  Back to cited text no. 9
    
10.
Dolan RV, Remine WH, Dockerty MB. The fate of heterotopic pancreatic tissue-a study of 212 cases. Arch Surg 1974;109:762-5.  Back to cited text no. 10
    
11.
Agale SV, Agale VG, Zode RR, Grover S, Joshi S. Heterotopic pancreas involving stomach and duodenum. J Assoc Physicians India 2009;57:653-4.  Back to cited text no. 11
    
12.
Yuan Z, Chen J, Zheng Q, Huang XY, Yang Z, Tang J. Heterotopic pancreas in the gastrointestinal tract. World J Gastroenterol 2009;15:3701-3.  Back to cited text no. 12
    
13.
Heinrich H. Ein Beitrag zur Histologie dessogen. Akzessorischen Pankreas Virchows Arch 1909;198:392-401.  Back to cited text no. 13
    
14.
Chandan VS, Wang W. Pancreatic heterotopia in the gastric antrum. Arch Pathol Lab Med 2004;128:111-2.  Back to cited text no. 14
    
15.
Gupta MK, Karlitz JJ, Raines DL, Florman SS, Lopez FA. Clinical case of the month. Heterotopic pancreas. J La State Med Soc 2010;162:310-13.  Back to cited text no. 15
    
16.
Baysoy G, Balamtekin N, Uslu N, Karavelioğlu A, Talim B, Ozen H. Double heterotopic pancreas and Meckel's diverticulum in a child: do they have a common origin? Turk J Pediatr 2010;52:336-8.  Back to cited text no. 16
    
17.
Nakao T, Yanoh K, Itoh A. Aberrant pancreas in Japan. Review of the literature and report of 12 surgical cases. Med J Osaka Univ 1980;30:57-63.  Back to cited text no. 17
    

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Correspondence Address:
Mukund Sable,
Department of Pathology and Lab Medicine, AIIMS, Bhubaneswar - 751 019, Odisha
India
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ijpm.ijpm_526_21



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