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• W2765804359 abstract "SESSION TITLE: Metastatic and Other Primary Lung Tumors SESSION TYPE: Affiliate Case Report Poster PRESENTED ON: Tuesday, October 31, 2017 at 01:30 PM - 02:30 PM INTRODUCTION: Solitary fibrous tumor of the pleura (SFTP) is a rare malignancy complicated by hypoglycemia and hypertrophic osteoarthropathy, known under the eponyms, Doege-Potter syndrome and Pierre-Marie-Bamberg syndrome, respectively. CASE PRESENTATION: 73 year old woman presented with weight loss and syncope. FSBG = 20 mg/dL. Medical history was unremarkable. She never smoked. Decreased right sided breath sounds and digital clubbing were noted. CXR revealed opacification of the right lung base. Non-contrast CT chest showed a right pleural mass. Laboratory work-up obtained when FSBG = 37 mg/dL yielded low c-peptide, insulin, proinsulin, IGF-1, IGF-2 levels. The pleural tumor was excised, staining positive for CD34 and STAT-6, consistent with SFTP. The tumor was hypercellular with mitotic rate > 14 per 10 high power fields. Hypoglycemia resolved post-operatively. DISCUSSION: Hypoglycemia occurs in 20% of SFTP cases, while hypertrophic osteoarthropathy occurs in 55%. In one review complete surgical resection was achieved in nearly all cases resulting in long-term recurrence free survival and resolution of hypoglycemia and digital clubbing.[i] In a study of 110 cases with SFT from all sites, excluding meningeal tumors, 5- and 10-year metastasis-free rates were 74% and 55% respectively.[ii] Age ≥ 55, tumor size ≥ 15 cm, and mitotic rate ≥ 4 per 10 high power fields predict time to metastasis and disease-specific mortality. CONCLUSIONS: SFTP is a subset of mesenchymal neoplasia. 2/3 of SFTP occur in the visceral pleura, whereas 1/3 of cases occur in the parietal pleura. Rarely these tumors are associated with life threatening hypoglycemia. Hypoglycemia in SFT is mediated via high molecular weight IGF-2, which exerts biologic activity at the insulin receptor. Surgical resection resolves hypoglycemia, digital clubbing, and results in long-term recurrence free survival. Characteristics of malignant SFTP include mitotic rate > 4 per 10 high power fields, increased cellularity, nuclear atypia, and infiltrative growth. Unfortunately, this patient’s advanced age, large tumor size, and high mitotic rate predict both increased risks for metastatic spread and death. Reference #1: [i] Kalebi A. Surgically cured hypoglycemia secondary to pleural solitary fibrous tumour: case report and update review on the Doege-Potter syndrome. Journal of Cardiothoracic Surgery. 2009, 4:45. Reference #2: [ii] Demicco E. Solitary fibrous tumor: a clinicopathological study of 110 cases and proposed risk assessment model. Mod Pathol. 2012; 25:1298-1306. DISCLOSURE: The following authors have nothing to disclose: Michael Bender, Avani Sinha, Anthony Ogedegbe No Product/Research Disclosure Information" @default.
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• W2765804359 date "2017-10-01" @default.
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• W2765804359 title "Paraneoplastic Syndromes and Solitary Fibrous Tumor of the Pleura" @default.
• W2765804359 doi "https://doi.org/10.1016/j.chest.2017.08.699" @default.
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