Pyloric gland metaplasia/differentiation in multiple organ systems in a patient with Peutz–Jegher's syndrome

Abstract

Peutz-Jegher's syndrome (PJS) involves multiple organ systems and the development of hamartomatous, metaplastic, or neoplastic lesions of different cell lineages. Among them, glandular lesions are the most common, but their properties are obscure. We report here a 53-year-old woman with PJS who developed multiple hamartomatous polyps in the jejunum and mucinous glandular lesions in multiple organ systems: glandular metaplasia in the urinary bladder; lobular endocervical glandular hyperplasia in the uterine cervix; mucinous metaplasia in the right fallopian tube; mucinous adenoma in the left ovary. Histological and immunohistochemical analyses disclosed that all of the intestinal and extra-intestinal lesions were associated with pyloric gland metaplasia/differentiation across the organ systems. In the general population, the organs described above rarely or infrequently show pyloric gland phenotype, to say nothing of trans-organ involvement. It is strongly suggested that commitment to pyloric gland metaplasia/differentiation is closely associated with PJS.

A Pyloric Gland-Phenotype Ovarian Mucinous Tumor Resembling Lobular Endocervical Glandular Hyperplasia in a Patient with Peutz-Jeghers Syndrome

“…Minimal deviation adenocarcinoma (an extremely well differentiated form of gastric type adenocarcinoma) of the cervix and ovarian sex cord tumor with annular tubules are the most well-known tumors of the female genital organs associated with PJS, but association with ovarian mucinous tumors has rarely been described [4]. Moreover, gastric phenotype of the mucinous epithelium in patients with PJS has not been clearly determined, and there is only one case briefly mentioning ovarian mucinous tumor with pyloric/gastric differentiation [4].…”

“…Moreover, gastric phenotype of the mucinous epithelium in patients with PJS has not been clearly determined, and there is only one case briefly mentioning ovarian mucinous tumor with pyloric/gastric differentiation [4]. In that case report, the pyloric gland differentiation involved the lesions of multiple organs, including the jejunum, urinary bladder, uterine cervix, fallopian tube, and ovary.…”

Fallopian tube metastases of non‐gynaecological origin: a series of 20 cases emphasizing patterns of involvement including intra‐epithelial spread

“…One possible exception to this generalization is in the context of mucinous lesions, as rare primary mucinous epithelial alterations of the tubal mucosa, ranging from simple mucinous metaplasia to benign and borderline‐like neoplasia, have been reported. Some of these cases have been associated with multifocal mucinous neoplasia within the gynaecological tract or have occurred in patients with Peutz–Jegher’s syndrome 34–40 . Recently, Wong et al.…”

“…Some of these cases have been associated with multifocal mucinous neoplasia within the gynaecological tract or have occurred in patients with Peutz-Jegher's syndrome. [34][35][36][37][38][39][40] Recently, Wong et al 41 reported a series of 23 mucinous epithelial lesions of the Fallopian tubes and considered that these represented an in-situ (metaplastic) process rather than mucosal colonization from an extratubal site. This interpretation was supported by the lack of cellular atypia and the absence in most cases of an E112 C J R Stewart et al…”

Cancer risk and management in a woman with Peutz-Jeghers syndrome

“…Therefore, this case is representative of SMMN-FGT, since the gastric-type mucinous lesions were identified in the cervix and in the fallopian tube. Furthermore, SMMN-FGT lesions frequently occur in women with PJS [15].…”