PMC3325741_01
Gross surgical specimen. A) Primary tumor measured 22x17x10.5 cm mass described as a large, mulitnodular infiltrating mass; B) cut surfaces were described as hemorrhagic, tan/yellow, softened surfaces; C) evidence of diffuse invasion of vascular structures on gross evaluation.
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F1
On gross examination, the mass was described as having hemorrhagic, tan/yellow, softened cut surfaces with multiple finger-like projections into the myometrium (Figure 1).
A) Uterine PEComa showing infiltrative growth into the myometrium; B) nests of perivascular epithelioid cells clear to slightly eosinophilic cytoplasm, moderate nuclear variability and small nucleoli.
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F2
Extensive areas of hemorrhage, coagulative necrosis and prominent vascular invasion were also noted; the mitotic index was 2/50 high powered fields (HPF) (Figure 2).
Immunohistochemistry demonstrating co-expression of melan-A (A) and smooth muscle actin (B). Immunostain for S-100 was negative (C).
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F3
The neoplastic cells were positive for HMB-45, melan A, smooth muscle actin, desmin, and estrogen receptor, while they were negative for S100, pankeratin, inhib-in alpha and tyrosinase (Figure 3).
Postoperative imaging studies. A) Computed tomography (CT) scan of the pelvis performed 34 days post surgical resection revealing persistent right gonadal vein thrombosis (arrow); B) positron emission tomography/computed tomography performed 56 days post surgical resection demonstrating mild FDG avidity in right gonadal vein region (arrow). Adjacent FDG avidity felt to be most consistent with postoperative changes; C) CT scan 12 months following surgical resection showing complete resolution of gonadal vein thrombosis following 6 month course of anticoagulation therapy.
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F4
The patient made a full recovery, and CT of the chest, abdomen and pelvis 5 weeks following surgery revealed a small, subsegmental pulmonary embolus (PE), multiple bilateral sub-cm pulmonary nodules and a tubular 9.0x2.4 cm mass within the right gonadal vein (Figure 4).