Endoscopy 2006; 38: E32-E33
DOI: 10.1055/s-2006-944647
Unusual cases and technical notes

© Georg Thieme Verlag KG Stuttgart · New York

Two cases of solitary Peutz-Jeghers-type hamartoma of the duodenum

S.  Itaba1 , M.  Namoto1 , S.  Somada1 , K.  Nakamura2 , Y.  Kumashiro3 , N.  Nakamura3 , T.  Yao3
  • 1Department of Gastroenterology, Beppu Medical Center, Beppu, Oita, Japan
  • 2Department of Medicine and Bioregulatory Science, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan
  • 3Department of Anatomic Pathology, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan
Further Information

K. Nakamura, M. D.

Department of Medicine and Bioregulatory Science
Graduate School of Medical Sciences
Kyushu University

Fukuoka 812-8582
Japan

Fax: +81-92-642-5287

Email: knakamur@intmed3.med.kyushu-u.ac.jp

Publication History

Publication Date:
22 January 2007 (online)

Table of Contents

Peutz-Jeghers syndrome is a rare autosomal dominant disease that is characterized by hamartomatous gastrointestinal polyposis and mucocutaneous pigmentation [1]. One report proposed that solitary Peutz-Jeghers-type hamartomatous polyps in patients with no other features of Peutz-Jeghers syndrome were likely to represent a separate disease entity from classic Peutz-Jeghers syndrome [2]. The solitary Peutz-Jeghers-type hamartoma of the duodenum is extremely rare in comparison with Peutz-Jeghers syndrome. We describe two such cases.

Patient 1 was an 87-year-old Japanese woman. She presented at our hospital complaining of epigastric discomfort. Upper gastrointestinal endoscopy revealed a pedunculated polyp, 1.8 cm in diameter and with a long stalk, in the second portion of the duodenum (Figure [1]). Patient 2 was a 56-year-old Japanese man. He was noted to have a high serum level of carcinoembryonic antigen. Upper gastrointestinal endoscopy was performed to screen for gastrointestinal malignancy, and this examination revealed a pedunculated and lobulated polyp in the second portion of the duodenum, 1.2 cm in diameter (Figure [2]).

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Figure 1 Upper gastrointestinal endoscopy in patient 1 revealed a pedunculated polyp with a long stalk in the second portion of the duodenum.

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Figure 2 Upper gastrointestinal endoscopy in patient 2 revealed a pedunculated and lobulated polyp in the second portion of the duodenum.

Neither patient showed cutaneous pigmentation and we found no other gastrointestinal polyps on colonoscopy or on small-bowel follow-through examination in either patient; both patients had an unremarkable familial medical history. The surface of these polyps was normal in colour. Biopsy specimens revealed normal duodenal mucosa in both cases. These polyps were resected by endosopic polypectomy. Histologically, both the polyps consisted of branching bundles of smooth-muscle fibers covered by hyperplastic duodenal mucosa (Figure [3]).

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Figure 3 A low-power view of the resected duodenal polyp from patient 1 (a) and a similar view of the polyp from patient 2 (b), showing branching bundles of smooth-muscle fibers covered by hyperplastic duodenal mucosa in both cases (hematoxylin & eosin stain).

We only found eight well-documented cases of solitary Peutz-Jeghers-type hamartoma of the duodenum in a review of the English-language literature [2] [3] [4] [5]. The features we describe are characteristic of a Peutz-Jeghers polyp. The possibility of a solitary Peutz-Jeghers-type hamartoma should be considered in the differential diagnosis of a duodenal polyp.

Endoscopy_UCTN_Code_CCL_1AB_2AZ_3AB

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References

  • 1 Jeghers H, McKusick V A, Katz K H. Generalized intestinal polyposis and melanin spots of the oral mucosa, lips, and digits.  N Engl J Med. 1949;  241 993-1005
  • 2 Acea Nebril B, Taboada Filgueira L, Parajo Calvo A. et al . Solitary hamartomatous duodenal polyp; a different entity: report of a case and review of the literature.  Surg Today. 1993;  23 1074-1077
  • 3 Ichiyoshi Y, Yao T, Nagasaki S. et al . Solitary Peutz-Jeghers type polyp of the duodenum containing a focus of adenocarcinoma.  Ital J Gastroenterol. 1996;  28 95-97
  • 4 Oncel M, Remzi F H, Church J M. et al . Course and follow up of solitary Peutz-Jeghers polyps: a case series.  Int J Colorect Dis. 2003;  18 33-35
  • 5 Kitaoka F, Shiogama T, Mizutani A. et al . A solitary Peutz-Jeghers-type hamartomatous polyp in the duodenum: a case report including results of mutation analysis.  Digestion. 2004;  69 79-82

K. Nakamura, M. D.

Department of Medicine and Bioregulatory Science
Graduate School of Medical Sciences
Kyushu University

Fukuoka 812-8582
Japan

Fax: +81-92-642-5287

Email: knakamur@intmed3.med.kyushu-u.ac.jp

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References

  • 1 Jeghers H, McKusick V A, Katz K H. Generalized intestinal polyposis and melanin spots of the oral mucosa, lips, and digits.  N Engl J Med. 1949;  241 993-1005
  • 2 Acea Nebril B, Taboada Filgueira L, Parajo Calvo A. et al . Solitary hamartomatous duodenal polyp; a different entity: report of a case and review of the literature.  Surg Today. 1993;  23 1074-1077
  • 3 Ichiyoshi Y, Yao T, Nagasaki S. et al . Solitary Peutz-Jeghers type polyp of the duodenum containing a focus of adenocarcinoma.  Ital J Gastroenterol. 1996;  28 95-97
  • 4 Oncel M, Remzi F H, Church J M. et al . Course and follow up of solitary Peutz-Jeghers polyps: a case series.  Int J Colorect Dis. 2003;  18 33-35
  • 5 Kitaoka F, Shiogama T, Mizutani A. et al . A solitary Peutz-Jeghers-type hamartomatous polyp in the duodenum: a case report including results of mutation analysis.  Digestion. 2004;  69 79-82

K. Nakamura, M. D.

Department of Medicine and Bioregulatory Science
Graduate School of Medical Sciences
Kyushu University

Fukuoka 812-8582
Japan

Fax: +81-92-642-5287

Email: knakamur@intmed3.med.kyushu-u.ac.jp

Zoom Image

Figure 1 Upper gastrointestinal endoscopy in patient 1 revealed a pedunculated polyp with a long stalk in the second portion of the duodenum.

Zoom Image

Figure 2 Upper gastrointestinal endoscopy in patient 2 revealed a pedunculated and lobulated polyp in the second portion of the duodenum.

Zoom Image
Zoom Image

Figure 3 A low-power view of the resected duodenal polyp from patient 1 (a) and a similar view of the polyp from patient 2 (b), showing branching bundles of smooth-muscle fibers covered by hyperplastic duodenal mucosa in both cases (hematoxylin & eosin stain).