Pleuropulmonary solitary fibrous tumour (SFT)
Introduction Solitary fibrous tumors (SFTs) are relatively rare neoplasms that commonly occur in the pleura. The tumor infrequently has been associated with paraneoplastic syndromes, most commonly reported is non-islet cell tumor hypoglycaemia (Doege Potter Syndrome). Case Report Case Report –...
Saved in:
| Main Authors: | , , , , , |
|---|---|
| Format: | Proceeding Paper |
| Language: | en en |
| Published: |
2019
|
| Subjects: | |
| Online Access: | http://irep.iium.edu.my/88384/1/SFT%20Acceptance%20Letter.pdf http://irep.iium.edu.my/88384/2/Solitary%20Fibrous%20Tumor.pdf http://irep.iium.edu.my/88384/ |
| Tags: |
Add Tag
No Tags, Be the first to tag this record!
|
| Summary: | Introduction
Solitary fibrous tumors (SFTs) are relatively rare neoplasms that commonly occur in the pleura. The tumor infrequently has been associated with paraneoplastic syndromes, most commonly reported is non-islet cell tumor hypoglycaemia (Doege Potter Syndrome).
Case Report
Case Report – A 63 year old female, non smoker, non diabetic presented to district hospital with history of cough for 2 weeks, difficulty breathing and constitutional symptoms. CXR noted homogenous opacity obscuring right hemothorax, mediastinal shifted with tracheal deviated to the left. CECT Thorax showed huge heterogeneously enhancing mass occupying the right hemithorax. It measures approximately 16.4 cm x 16.5 cm x 21.1 cm. Minimal residual collapsed lung noted at right apical region. Pleural tapping done at district hospital noted exudative picture. Patient also noted to have unexplained hypoglycaemia initially thought to be adrenal metastasis and was started on IV Hydrocortisone 100mg TDS however the hypoglycemia persist. Upon transferred to our care, patient was on NIV and noted patient had superior vena cava syndrome (SVCS) and she was started on IV Dexametasone high dose. SVCS and refractory hypoglycemia improved remarkably with dexamethasone. Trucut biopsy of right lung mass done under ultrasound guided.
Result
Microscopically, the biopsy show spindle to oval shaped tumor cells with hypocellular and hypercellular area within a ropey collagenous stroma. Pericytic vascular pattern are seen. The tumor cells are positive for CD34, BCL2, CD99 and STAT 6 with patchy CK AE1/AE3 positivity, thus the impression of solitary fibrous tumor (SFT) given.
Conclusion
Our case highlights the importance of considering Doege-Potter syndrome in a patient with SFT and hypoglycemia. Non islet cell tumor hypoglycaemia (NICTH) is the main clinical characteristic of Doege–Potter syndrome. It occurs in < 5 percent of cases and is primarily seen in large peritoneal/ primary tumor and caused by tumor secretion of large insulin like growth factor II (IGF2) and responded well with high dose steroid. |
|---|