Tuberc Respir Dis.  2015 Apr;78(2):120-124. 10.4046/trd.2015.78.2.120.

A Case of Solitary Fibrous Pleura Tumor Associated with Severe Hypoglycemia: Doege-Potter Syndrome

• Jang JG
• Chung JH
• Hong KS
• Ahn JH
• Lee JY
• Jo JH
• Lee DW
• Shin KC
• Lee KH
• Kim MJ
• Lee JC
• Lee JH
• Lee JK

• KMID: 2320604
• DOI: http://doi.org/10.4046/trd.2015.78.2.120

Abstract

Solitary fibrous tumor of the pleura (SFTP) is a rare primary intrathoracic tumor that arises from mesenchymal tissue underlying the mesothelial layer of the pleura. It usually has an indolent clinical course. The hypoglycemia that accompanies SFTP was first described by Doege and Potter independently in 1930, hence the eponym Doege-Potter syndrome (DPS). The incidence of DPS is reported to be ~4%. In this report, we present a typical case of DPS that was cured through complete surgical resection.

Keyword

Solitary Fibrous Tumors; Pleura; Hypoglycemia

MeSH Terms

Eponyms
Hypoglycemia*
Incidence
Pleura*
Solitary Fibrous Tumor, Pleural
Solitary Fibrous Tumors

Figure

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  Figure 1 Plain chest radiography showed a large opacity in his right hemithorax.

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  Figure 2 Contrast-enhanced chest computed tomography (CT) revealed a lobulated 15×10×19.5-cm-sized mass in his right hemithorax that showed heterogenous enhancement (A). Compared with the chest CT images taken 5 years earlier (B), the size of the mass had increased.

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  Figure 3 Histopathological findings. (A) The tumor showed a short intersecting fascicular pattern, composed of spindle cells with mild atypia, hypercellularity, and high mitotic figures (average 10-12/10 HPF) (H&E stain, ×200). (B) There is coexistence of hypo- and hypercellular areas. The spindle cells show moderate atypism with ropy collagenous stroma. Mitotic figures were less than 4/10 HPF (H&E stain, ×200).

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  Figure 4 The tumor cells were immunohistochemically strongly reactive for Bcl-2 (A), CD34 (B), and STAT6 (C) a specific marker for solitary fibrous tumors (A-C, ×200).

Reference

1. Lee CE, Zanariah H, Masni M, Pau KK. Solitary fibrous tumour of the pleura presenting with refractory non-insulin mediated hypoglycaemia (the Doege-Potter syndrome). Med J Malaysia. 2010; 65:72–74.

2. Campbell NA, Antippa PN. Solitary fibrous tumour of the pleura. Heart Lung Circ. 2006; 15:400–401.

3. de Perrot M, Fischer S, Brundler MA, Sekine Y, Keshavjee S. Solitary fibrous tumors of the pleura. Ann Thorac Surg. 2002; 74:285–293.

4. Robinson LA. Solitary fibrous tumor of the pleura. Cancer Control. 2006; 13:264–269.

5. Doege KW. Fibro-sarcoma of the mediastinum. Ann Surg. 1930; 92:955–960.

6. Leng XF, Xian L, Qin JJ, Lei BF. Malignant solitary fibrous tumor of pleura accompanied with first symptoms of chest pain and hemoptysis: a case report. Ann Thorac Cardiovasc Surg. 2012; 18:251–255.

7. Briselli M, Mark EJ, Dickersin GR. Solitary fibrous tumors of the pleura: eight new cases and review of 360 cases in the literature. Cancer. 1981; 47:2678–2689.

8. Klemperer P, Rabin CB. Primary neoplasm of the pleura: a report of five cases. Arch Pathol. 1931; 11:385–412.

9. Zhu Y, Du K, Ye X, Song D, Long D. Solitary fibrous tumors of pleura and lung: report of twelve cases. J Thorac Dis. 2013; 5:310–313.

10. Magdeleinat P, Alifano M, Petino A, Le Rochais JP, Dulmet E, Galateau F, et al. Solitary fibrous tumors of the pleura: clinical characteristics, surgical treatment and outcome. Eur J Cardiothorac Surg. 2002; 21:1087–1093.

11. England DM, Hochholzer L, McCarthy MJ. Localized benign and malignant fibrous tumors of the pleura: a clinicopathologic review of 223 cases. Am J Surg Pathol. 1989; 13:640–658.

12. Doyle LA, Vivero M, Fletcher CD, Mertens F, Hornick JL. Nuclear expression of STAT6 distinguishes solitary fibrous tumor from histologic mimics. Mod Pathol. 2014; 27:390–395.

13. Chilosi M, Facchettti F, Dei Tos AP, Lestani M, Morassi ML, Martignoni G, et al. bcl-2 expression in pleural and extrapleural solitary fibrous tumours. J Pathol. 1997; 181:362–367.

14. Masson EA, MacFarlane IA, Graham D, Foy P. Spontaneous hypoglycaemia due to a pleural fibroma: role of insulin like growth factors. Thorax. 1991; 46:930–931.

15. Schutt RC, Gordon TA, Bhabhra R, Cathro HP, Cook SL, Mc-Cartney CR, et al. Doege-Potter syndrome presenting with hypoinsulinemic hypoglycemia in a patient with a malignant extrapleural solitary fibrous tumor: a case report. J Med Case Rep. 2013; 7:11.