Extrauterine Adenosarcoma: A Clinicopathologic Study of 24 Cases
Nella C Fernandez, Elizabeth Euscher, Anais Malpica. University of Texas MD Anderson Cancer Center, Houston, TX
Background: Extrauterine adenosarcoma (EuAds) is a rare neoplasm occurring mostly in the ovaries and pelvis of women over a wide age range. In this study, we present the clinicopathologic features of 24 such cases seen in our institution over a period of 26 years.
Design: 24 cases of EuAds from 1985 to 2010 were retrieved from our files. Cases with questionable origin in the uterus were excluded. The following parameters were recorded: patients'(pts) age, clinical presentation, history of adenomyosis or endometriosis, initial diagnosis, site of involvement, gross appearance, tumor size, the presence of sarcomatous overgrowth (SO), treatment, and outcome.
Results: Pts ages ranged from 42-79 yrs (median, 51.5).The most common clinical signs/symptoms were: abdominal pain or fullness(37.5%), vaginal bleeding (18.7%), and leg edema(18.7%). 3 cases were incidentally detected (18.7%). History of adenomyosis or endometriosis was present in 35% and 61.9% of the cases, respectively. EuAds involved the following sites: pelvis(11 cases), ovaries(7 cases) uterine ligaments and adjacent soft tissue(2 cases), vagina(2 cases), lumbar plexus (1 case) and colon(1 case).Tumors ranged from 3-23.5 cm(mean 11.9 cm). SO was detected in 10/24 cases (41.6%). 2 cases with SO had heterologous elements: rhabdomyosarcoma, 1 case; rhabdomyosarcoma+chondrosarcoma, 1 case. 21 cases were received in consultation with a diagnosis other than EuAds in 10 (42%) cases:malignant mixed Mullerian tumor(4), endometriosis(2), adenomyoma(1), atypical mesenchymal component of a borderline tumor(1), malignant tumor of Mullerian origin(1) and high grade sarcoma(1).Primary treatment included: surgery/chemotherapy(9/24); surgery only(6/24); surgery/chemoradiation(2/24); surgery/radiotherapy(1/24). 13 pts (54.2%)had known recurrence 3 to 58 mos following diagnosis: pelvis(9/13), omentum(2/13), retroperitoneum(2/13). 1 pt never cleared her disease. 2 pts had metastasis: liver, 1 and lung, 1. Recurrence was unknown in 10 pts.Survival data was available for 22 pts: 9 pts died (DOD) 3 to 185 months after initial diagnosis. Of these 5 had tumor with SO (DOD 3-51 months) and 4 had no SO(DOD 26-185 months).
Conclusions: EuAds is rare and mostly seen in women in the 5th and 6th decades. EuAds is often associated with adenomyosis/endometriosis and is most commonly seen in the pelvis followed by the ovaries. EuAds can represent a diagnostic challenge as seen in 42% of our cases. SO is seen in almost half of the cases. EuAds tends to recur more frequently than their uterine counterpart due to the lack of anatomic barriers for the spread of disease.
Category: Gynecologic & Obstetrics
Tuesday, March 5, 2013 9:15 AM
Proffered Papers: Section E, Tuesday Morning