TY - JOUR
T1 - Malignant ovarian sex cord tumor with annular tubules in a patient with Peutz-Jeghers syndrome
T2 - A case report
AU - Lele, Subodh M.
AU - Savvh, Ravindranauth N.
AU - Zaharopoulos, Paul
AU - Adesokan, Adekunle
AU - Smith, Michael
AU - Linhart, Jessica M.
AU - Arrastia, Concepcion D.
AU - Krigman, Hannah R.
PY - 2000/4
Y1 - 2000/4
N2 - The majority of ovarian sex cord tumors with annular tubules (SCTAT) are benign neoplasms that arise sporadically. In patients who have Peutz-Jeghers syndrome (PJS), ovarian SCTAT is often an incidental finding. Malignant behavior in SCTAT has heretofore been reported only in sporadic cases. We report a case of bilateral, malignant SCTAT developing in a 47-year-old woman who had PJS, originally diagnosed as adenocarcinoma on cervicovaginal cytology. Cervicovaginal and peritoneal fluid cytologic preparations were characterized by pseudopapillary clusters and three-dimensional tubes of tumor cells with scanty cytoplasm and high nuclear: cytoplasmic ratio. Examination of surgical resection specimens revealed bilateral, solid ovarian tumors composed of simple and complex annular tubules with hyaline cores, typical of SCTAT. Tumor emboli were present within salpingeal lymphovascular spaces and in both right and left pelvic lymph nodes. Flow cytometry of tumor cells demonstrated a diploid phenotype. This case represents the first documented example of bilateral, malignant SCTAT arising in a patient who had PJS, presenting with an atypical cervicovaginal smear.
AB - The majority of ovarian sex cord tumors with annular tubules (SCTAT) are benign neoplasms that arise sporadically. In patients who have Peutz-Jeghers syndrome (PJS), ovarian SCTAT is often an incidental finding. Malignant behavior in SCTAT has heretofore been reported only in sporadic cases. We report a case of bilateral, malignant SCTAT developing in a 47-year-old woman who had PJS, originally diagnosed as adenocarcinoma on cervicovaginal cytology. Cervicovaginal and peritoneal fluid cytologic preparations were characterized by pseudopapillary clusters and three-dimensional tubes of tumor cells with scanty cytoplasm and high nuclear: cytoplasmic ratio. Examination of surgical resection specimens revealed bilateral, solid ovarian tumors composed of simple and complex annular tubules with hyaline cores, typical of SCTAT. Tumor emboli were present within salpingeal lymphovascular spaces and in both right and left pelvic lymph nodes. Flow cytometry of tumor cells demonstrated a diploid phenotype. This case represents the first documented example of bilateral, malignant SCTAT arising in a patient who had PJS, presenting with an atypical cervicovaginal smear.
KW - Cytology
KW - Ovarian neoplasms
KW - Peutz-Jeghers syndrome
KW - Ploidy
KW - Sex cord tumor with annular tubules
UR - http://www.scopus.com/inward/record.url?scp=0034005013&partnerID=8YFLogxK
U2 - 10.1038/modpathol.3880079
DO - 10.1038/modpathol.3880079
M3 - Article
C2 - 10786816
AN - SCOPUS:0034005013
SN - 0893-3952
VL - 13
SP - 466
EP - 470
JO - Modern Pathology
JF - Modern Pathology
IS - 4
ER -