Presentation
Known case of GIST, presented for re-evaluation after a course of treatment, to be compared with prior study done before 4 months .
Patient Data
There is a remarkable reduction in size and enhancement (HU density) of all target and non-target lesions with development of calcific clumps, indicating devascularization, overall impression is partial response according to Choi criteria.
Re-demonstration of unchanged polysplenia syndrome and multi-nodular goiter with retrosternal extension.
4 months earlier pre treatment
Multiple perigastric solid lesions with central necrosis and peripheral enhancement , nodular omental and peritoneal deposits mainly at left upper quadrant and umbilical seed (sister Mary Joseph nodule), small sub cm sized pulmonary nodules at right lung.
Absent morphological spleen replaced by multiple rounded spleneculi in normal anatomical left hypochondrial region combined with bilateral lung left sidedness in keeping with polysplenia syndrome.
Heterogeneous enlarged goiter with retrosternal extension, though no airway compromise.
Case Discussion
This case is a nice example of partial response (according to Choi criteria) of nonoperative GIST to medical treatment using Gleevec (Imatinib) as targeted therapy which is a tyrosine kinase inhibitor (TKI) also utilized in the management of different hematological diseases like leukemia, and certain types of cancer.
Our patient was a candidate for conservative therapeutic management since there was considerable peritoneal dissemination in addition to pulmonary deposition. Scanning 4 months post-therapy depicted a significant drop in the target and non-target lesions' attenuation value (HU) along with size shrinkage and calcification. These changes were observed even in the umbilical seed focus (Sister Mary Joseph nodule), which is a rare phenomenon, an indicator of intra-abdominal advanced malignant disease that carries a poor prognosis.
An incidental finding of polysplenia syndrome and a symptomatic mediastinal extended multi-nodular goiter.
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