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

• Kim Y
• Kim EY
• Kim TJ
• Lim KT
• Lee KH
• Chun Y
• So KA

• KMID: 2462127
• DOI: http://doi.org/10.5468/ogs.2019.62.6.474

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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