Peutz-Jeghers syndrome with ovarian sex cord tumor with annular tubules and cervical adenoma malignum.
A patient with Peutz-Jeghers syndrome, a sex cord tumor with annular tubules, and an initially unrecognized adenoma malignum of the cervix is described. The patient presented with a mucinous adenocarcinoma in the vaginal apex. Review of the hysterectomy slides demonstrated an adenoma malignum of the cervix. In addition to a microscopic sex cord tumor with annular tubules of the right ovary, the left ovary contained mucinous cystadenomas. Adenoma malignum remains a difficult diagnosis and is frequently made only after hysterectomy for a presumed benign indication; pathology frequently demonstrates a deeply invasive, unusually well-differentiated adenocarcinoma of the cervix. Patients with Peutz-Jeghers syndrome need careful clinical and cytologic follow-up to exclude such lesions.
Podczaski
E
E
Department of Obstetrics & Gynecology, M. S. Hershey Medical Center, Pennsylvania State University, Hershey 17033.
Kaminski
P F
PF
Pees
R C
RC
Singapuri
K
K
Sorosky
J I
JI
D000230:Adenocarcinoma / Q000150:complications* / Q000473:pathology / Q000532:radiotherapy; D000293:Adolescent; D005260:Female; D006801:Humans; D007044:Hysterectomy; D009364:Neoplasm Recurrence, Local; D009378:Neoplasms, Multiple Primary; D010051:Ovarian Neoplasms / Q000150:complications* / Q000473:pathology / Q000601:surgery; D010580:Peutz-Jeghers Syndrome / Q000150:complications* / Q000601:surgery; D002583:Uterine Cervical Neoplasms / Q000150:complications* / Q000473:pathology / Q000532:radiotherapy