J Gynecol Oncol.  2010 Sep;21(3):203-206. 10.3802/jgo.2010.21.3.203.

Co-occurrence of an adenoma malignum and an endocervical-type adenocarcinoma of the uterine cervix in a woman with Peutz-Jeghers syndrome

• Koo YJ
• Lee JE
• Hong SR
• Kwon YS

• KMID: 1980860
• DOI: http://doi.org/10.3802/jgo.2010.21.3.203

Abstract

We report a rare co-occurrence of an adenoma malignum and an adenocarcinoma in a 30-year-old woman with Peutz-Jeghers syndrome. The woman was diagnosed with Peutz-Jeghers syndrome based on an endoscopic biopsy after vaginal bleeding. A pelvic examination and an MRI revealed the co-occurrence of a 4x5 cm protruding adenocarcinoma of FIGO stage Ib2 based on a punch biopsy and a 4.5x5.7 cm multilocular cystic mass above the solid cancer. The patient received two courses of neoadjuvant chemotherapy, followed by a laparoscopic radical hysterectomy with pelvic lymph node dissection. Pathologic findings were consistent with adenocarcinoma (40%) and adenoma malignum (60%) confined to the cervix. Three courses of adjuvant chemotherapy were performed and no clinical evidence of recurrence was seen during a 12 month follow-up period. This study will contribute to defining the best diagnosis and treatment for these rare complicating tumors.

Keyword

Peutz-Jeghers syndrome; Adenoma malignum; Adenocarcinoma

MeSH Terms

Adenocarcinoma
Adenoma
Adult
Biopsy
Cervix Uteri
Chemotherapy, Adjuvant
Female
Follow-Up Studies
Gynecological Examination
Humans
Hysterectomy
Lymph Node Excision
Peutz-Jeghers Syndrome
Recurrence
Uterine Hemorrhage

Figure

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  Fig. 1 Peutz-Jeghers polyp of the colon. An arborizing smooth muscle core separates colonic glands into vague lobules (×12.5) (A). A band of smooth muscle fibers between lobules of colonic glands can be seen at higher magnification (×40) (B) (H&E).

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  Fig. 2 Enhanced T1-weighted MRI showing septal enhancement of a multiloculated cystic mass (long arrow, adenoma malignum) and enhancement of the solid portion (short arrow, endocervical type adenocarcinoma).

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  Fig. 3 Histopathologic findings (B-D: H&E, ×200). (A) Gross features showing a large exophytic tumor (short arrow: adenoma malignum) arising from the transformation zone, and a diffuse, bulging mass (long arrow: endocervical type adenocarcinoma) involving the endocervix. (B) The region composed of an adenoma malignum (left) and an endocervical type (right) adenocarcinoma. (C) An adenoma malignum showing irregular glands lined by deceptively bland, mucin rich columnar cells with basal nuclei and occasional mitosis. (D) An endocervical type adenocarcinoma showing cellular stratification, high nuclear atypism, and increased mitosis.

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  Fig. 4 Immunohistochemistry findings (×200). (A) P16 staining is diffuse positive in endocervical typed adenocarcinoma.(B) P16 staining is focal positive in minimal deviation variant. (C) Ki-67 staining shows diffuse proliferation in endocervical typed adenocarcinoma. (D) Ki-67 staining is focal in minimal deviation variant.

Reference

1. Dozois RR, Judd ES, Dahlin DC, Bartholomew LG. The Peutz-Jeghers syndrome: is there a predisposition to the development of intestinal malignancy? Arch Surg. 1969. 98:509–517.

2. Payson BA, Moumgis B. Metastasizing carcinoma of the stomach in Peutz-Jeghers syndrome. Ann Surg. 1967. 165:145–151.

3. Lin JI, Caracta PF, Lindner A, Guzman LG. Peutz-Jeghers polyposis with metastasizing duodenal carcinoma. South Med J. 1977. 70:882–884.

4. Cordts AE, Chabot JR. Jejunal carcinoma in a child. J Pediatr Surg. 1983. 18:180–181.

5. Hsu SD, Zaharopoulos P, May JT, Costanzi JJ. Peutz-Jeghers syndrome with intestinal carcinoma: report of the association in one family. Cancer. 1979. 44:1527–1532.

6. Miller LJ, Bartholomew LG, Dozois RR, Dahlin DC. Adenocarcinoma of the rectum arising in a hamartomatous polyp in a patient with Peutz-Jeghers syndrome. Dig Dis Sci. 1983. 28:1047–1051.

7. Srivatsa PJ, Keeney GL, Podratz KC. Disseminated cervical adenoma malignum and bilateral ovarian sex cord tumors with annular tubules associated with Peutz-Jeghers syndrome. Gynecol Oncol. 1994. 53:256–264.

8. Aideyan UO, Kao SC. Gastric adenocarcinoma metastatic to the testes in Peutz-Jeghers syndrome. Pediatr Radiol. 1994. 24:496–497.

9. Giardiello FM, Welsh SB, Hamilton SR, Offerhaus GJ, Gittelsohn AM, Booker SV, et al. Increased risk of cancer in the Peutz-Jeghers syndrome. N Engl J Med. 1987. 316:1511–1514.

10. Entius MM, Keller JJ, Westerman AM, van Rees BP, van Velthuysen ML, de Goeij AF, et al. Molecular genetic alterations in hamartomatous polyps and carcinomas of patients with Peutz-Jeghers syndrome. J Clin Pathol. 2001. 54:126–131.

11. Kaminski PF, Norris HJ. Minimal deviation carcinoma (adenoma malignum) of the cervix. Int J Gynecol Pathol. 1983. 2:141–152.