UTROSCT (Uterine Tumor Resembling Ovarian Sex Cord Tumor)

Question:
Your opinion for the management of a case of UTROSCT (Uterine Tumor Resembling Ovarian Sex Cord Tumor):
28 year old woman with a history of intermenstrual spotting was examined by means of SIS this January and a 12x12 mm intracavitary polyp visualized. February the 7th, a hysteroscopic resection was performed and fibroid-like tissue removed from the upper posterior aspect of the cavity, the tissue 60% intracavitary positioned.

The histology specimen showed UTROSCT uterine tumor resembling ovarian sex cord tumor. It was not possible to evaluate the margins of the lesion, whether there should be residues of the tumor in the uterine cavity. A control in the outpatient clinic is scheduled this month.

The patient has not been pregnant.

The literature about the prognosis seems very limited, as most cases refers to histology from removed uteri.

The patient had in September 2011 surgery for a Clark level 2 malignant melanoma from the back, re-excision showed no residual tumor.

Your opinion for further management is much appreciated.

Kind regards
Mansoor Raza Mirza, MD

 
Response # 1:  
Uterine tumors resembling ovarian sex cord tumors (UTROSCT) are distinguished into two separate groups: endometrial stromal tumors with sex cord-like elements (Group I), which have an unfavorable prognosis ; and UTROSCT proper (group II), with more than 40% sex cord-like differentiation and less endometrial component, which are biologically less aggressive than the tumors of the other group. Few patients from the group II received conservative surgery but were treated conservatively due to the better prognosis.

In the case reported below, this could be discussed with the patient if the diagnosis of UTROSCT is clearly confirmed by IHC and expert pathologist (a panel of 4 including calretinin, inhibin, CD99, and Melan A seemed to be the most characteristic sex cord markers. Positivity for calretinin and at least for 1 of the other above-mentioned markers may thus confirm the diagnosis of UTROSCT. Endometrial stromal tumors with sex cord-like elements, on the other hand, usually express only 1 sex cord marker, mostly calretinin) ; however, this was not confirmed today by many publications.
 
Kind regards
 
Isabelle

Isabelle Ray-Coquard, MD, PhD

======================================================================
Dear Isabelle,

Our pathologist has described it as type II UTROSCT, and we are following her without any futher intervention.

Kind regards,

Mansoor

======================================================================
Very good news for the patient.

Kind regards,

Isabelle

 
Response # 2:  
Hi Mansoor – am looking to see what people suggest as I think I am seeing one in 2 weeks- will let you know .

Amit M. Oza

 
Response # 3:  
Dear colleague,

You mention that 60% was intracavitary, so this means that the rest is in the uterine wall? Is there indication for lesions outside the uterus? What is the proliferation rate of this malignancy, any markers done (IHC)?

Kind regards

Jan

Jan B. Vermorken, MD, PhD
======================================================================
Dear Jan,

There are not extra uterine lesions. There is not evidence of intra uterine leftovers. Our pathologist describe it as type II UTROSCT, and we are following her up without any further investigation.

Kindest regards,

Mansoor