Obstet Gynecol Sci.  2019 Nov;62(6):474-477. 10.5468/ogs.2019.62.6.474.

A rare case of gastric-type mucinous adenocarcinoma in a woman with Peutz-Jeghers syndrome

Affiliations
  • 1Department of Obstetrics and Gynecology, Cheil General Hospital & Women's Healthcare Center, College of Medicine, Dankook University, Seoul, Korea.
  • 2Department of Obstetrics and Gynecology, Seoul National University Bundang Hospital, Seoul, Korea.
  • 3Department of Obstetrics and Gynecology, Konkuk University School of Medicine, Seoul, Korea. joyfulplace@hanmail.net
  • 4Department of Obstetrics and Gynecology, Hallym University Kangdong Sacred Heart Hospital, Seoul, Korea.
  • 5CHA Gangnam Medical Center, CHA University, Seoul, Korea.
  • 6Department of Pathology, Cheil General Hospital & Women's Healthcare Center, College of Medicine, Dankook University, Seoul, Korea.

Abstract

Adenocarcinoma of the cervix is less common than squamous cell carcinoma. Minimal deviation adenocarcinoma (adenoma malignum) is considered an extremely well-differentiated variant of GAS. An association exists between GAS and Peutz-Jeghers syndrome, which is a rare autosomal dominant disorder characterized by mucocutaneous pigmentation and multiple hamartomatous polyps in the gastrointestinal tracts. The incidence of GAS in patients with Peutz-Jeghers syndrome is estimated to be 11-17%. We present a rare case of adenoma malignum, diagnosed using colposcopic biopsy in a woman with Peutz-Jeghers syndrome, which was histopathologically confirmed to be GAS after surgery.

Keyword

Adenocarcinoma; Uterine cervical neoplasms; Peutz-Jeghers syndrome

MeSH Terms

Adenocarcinoma
Adenocarcinoma, Mucinous*
Adenoma
Biopsy
Carcinoma, Squamous Cell
Cervix Uteri
Female
Gastrointestinal Tract
Humans
Incidence
Mucins*
Peutz-Jeghers Syndrome*
Pigmentation
Polyps
Uterine Cervical Neoplasms
Mucins

Figure

  • Fig. 1 (A) The cervix on colposcopy. (B, C) Pelvic magnetic resonance image showing an enlargement of the cervix with multiple cystic lesions (arrow). The cervical parametrium is intact.

  • Fig. 2 Histopathological results of colposcopic biopsy (A, B) and cervical conization (C-E). (A) The tumor consists of a mucinous epithelium infiltrating the cervical wall with little or no stromal reaction (original magnification ×4). (B) Cells lining these malignant glands have abundant clear cytoplasm and slightly enlarged nuclei with small nucleoli. Mitotic figures are rarely present (original magnification ×20). (C) Mucinous carcinoma of the gastric type with a more poorly differentiated component (original magnification ×20). (D) Immunohistochemical staining is positive for MUC6, a marker of pyloric gland mucin. (E) Immunohistochemical staining is focal positive but block pattern negative for p16.


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