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Maria Giulia Pirini
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EP1.09 - Pathology (ID 199)
- Event: WCLC 2019
- Type: E-Poster Viewing in the Exhibit Hall
- Track: Pathology
- Presentations: 1
- Now Available
- Moderators:
- Coordinates: 9/08/2019, 08:00 - 18:00, Exhibit Hall
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EP1.09-06 - An Unusual Diagnostic Case of Hypoglycemia: A Different Perspective of the Doege-Potter Syndrome (Now Available) (ID 2194)
08:00 - 18:00 | Author(s): Maria Giulia Pirini
- Abstract
Background
Solitary fibrous tumor (SFT) is an uncommon type of mesenchymal tumor that can occur anywhere in the body. A typical molecular feature is a NAB2-STAT6 fusion, that enhances cellular proliferation. Symptoms are often related to the mass effect exerted by the tumor, but paraneoplastic syndromes can occur. Among them the Doege-Potter syndrome, characterized by refractory hypoglycemia, occurs in <5 % of cases.
Method
A 60 years-old male patient was admitted to the hospital because of a recent severe recurrent hypoglycemia and an episode of syncope during his tennis play. No clinical history of co-morbidities were identified at the time of E.R. admittance. Blood tests were performed and endogen insulin, C-peptide and ACTH levels were below the lower limit, serum cortisol was low-normal. Ca125.5 and NSE were slightly elevated. Total body CT-scan revealed, in the right lung, a large mass (cranio-caudal extension 17 cm) dislocating the adjacent structures. While [18]FDG-PET was almost negative (SUVmax=2.6) the 68Ga-DOTATOC-PET showed faint focal uptake in patchy areas of the tumor (SUVmax=3.6). During diagnostic workup, hypoglycemia was treated with steroid administration.
Result
The patient underwent surgical resection, through a right-sided postero-lateral ribs "bellows" spreading thoracotomy, for a 17x16cm sessile lesion originating from the visceral pleura of the middle lobe of the lung. The tumor cells did not express neuroendocrine(chromogranin), nervous(PS100), but showed CD34+ and STAT6+ cells, with a Ki-67<10%, confirming the diagnosis of SFT. Hypoglycemia resolved after tumor removal, coherently with the Doege-Potter syndrome. At 12 months from surgery the patient is alive with no evidence of disease.
Hypoglycemia can be the single symptom of malignant tumors, frequently seen with insulinomas, but in our case, it was associated with an SFT, a tumor with uncertain biological behaviour. Surgical resection of the tumor is the mainstay of treatment.