Thursday, January 5, 2017

Rare tumor

In September 2016, a 50 year old Hispanic female was referred to our clinic for an encapsulated mass to the left side of the Vulva.  On CT, the mass seems to contain fat, encapsulated with solid content extending into the labia.   The radiologist felt it could represent a Liposarcoma.   Tissue diagnosis however suggested an Angiomyofibroblastoma.
Surgery was recommended, and on December, 01,2016 she underwent
a resection of the mass which was 22 x8.5x4.0 cm
the tumor was weekly positive for Desmin
strongly positive for Vimentin
ER positive
PR positive
 all other tested immunochemical stains were negative

The case was referred to Massachusset General Pathology for review
there was no  other metastatic lesion
and surgery confirmed the diagnosis

what to do now for this patient
concerns:   The disease is benign- Observation was advised
                   The tumor is too large, and no mention of full removal despite the encapsulation seen on CT.   should she have Radiation to reduce local recurrence.
ER and PR were positive, should we give Tamoxifen now or wait for a recurrence?
The disease had some "Angio" component, will it benefit from anti-EGFR treatment in case it recurs?

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