Journal of Postgraduate Medicine
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Year : 2013  |  Volume : 59  |  Issue : 1  |  Page : 64-66  

Malignant solitary fibrous tumor with hypoglycemia (Doege-Potter syndrome)

CY Yang1, CW Chou1, LJ Hao2,  
1 Department of Internal Medicine, Division of Endocrinology and Metabolism, Chi-Mei Medical Center, Tainan, Taiwan, Republic of China
2 Department of Internal Medicine, Kaohsiung Veteran General Hospital Tainan Branch; Department of Optometry, Chung Hwa University of Medical and Technology, Tainan, Taiwan, Republic of China

Correspondence Address:
L J Hao
Department of Internal Medicine, Kaohsiung Veteran General Hospital Tainan Branch; Department of Optometry, Chung Hwa University of Medical and Technology, Tainan, Taiwan
Republic of China




How to cite this article:
Yang C Y, Chou C W, Hao L J. Malignant solitary fibrous tumor with hypoglycemia (Doege-Potter syndrome).J Postgrad Med 2013;59:64-66


How to cite this URL:
Yang C Y, Chou C W, Hao L J. Malignant solitary fibrous tumor with hypoglycemia (Doege-Potter syndrome). J Postgrad Med [serial online] 2013 [cited 2023 Jan 30 ];59:64-66
Available from: https://www.jpgmonline.com/text.asp?2013/59/1/64/109503


Full Text

The occurrence of hypoglycemia with an intrathoracic tumor was first reported by Doege and Potter independently in 1930 hence the eponym Doege-Potter syndrome (DPS). [1] In this report we present a previously unpublished but very typical example of the rare occurrence of hypoglycemia with pleural solitary fibrous tumor (SFTP) in an adult Taiwanese male.

A 77-year-old man presented with spontaneous hypoglycemia due to a primary malignant fibrous tumor of the lung. The initial evaluation revealed severe hypoglycemia (blood sugar, 27 mg/dl). There was no diabetes mellitus history, no antidiabetic agents or other medications history. Patient had normal heart, thyroid, liver, renal and adrenal function. But a right lower lung (RLL) nodular lesion had been found 4.5 years ago [Figure 1]. The lesion had enlarged progressively and became a 15-cm huge mass seen on chest computed tomography (CT) [Figure 2] recently. Laboratory data showed normal Insulin (11.4 mIU/l; normal range 3.0-25.0) and C-peptide (2.59 ng/ml; normal range 0.81-3.85) levels and normal Insulin antibody (6.26%; normal range <10.0) in combination with low levels of growth hormone (0.012 ng/ml; normal range 0.003-0.971) and insulin-like growth factor type 1 (IGF-I) (33.1 ng/ml; normal range 59-177). The elevated IGF-II level (620 ng/ml; normal range 265-596) and decreased IGF-binding protein 3 (1.7 mg/l; normal range 2.5-5.1) indicated a high free IGF-II activity. After surgery, blood glucose (148 mg/dl) returned to normal with normal insulin (10.2 mIU/L) and C-peptide (2.14 ng/ml). Serum growth hormone (0.276 ng/ml), IGF-I (74.0 ng/ml) and the IGF-I/IGF-II ratio (0.05 preoperative vs. 0.12 postoperative; normal range >0.20) increased. Pathology revealed malignant solitary fibrous tumor [Figure 3]. Immunohistochemically, these spindled cells express vimentin and CD34 but not AE1/AE3. The proliferation index determined by Ki-67 is focally increased (up to 10%). There was no further recurrence of hypoglycemia after complete excision of the mass [Figure 4].{Figure 1}{Figure 2}{Figure 3}{Figure 4}

DPS is a paraneoplastic syndrome in which hypoglycemia is associated with the presence of one or more non-islet fibrous tumors in the pleural cavity. [2],[3],[4] These are rare and may be discovered incidentally, during non-specific respiratory symptoms or during hypoglycemia. [4] The prevalence is 2.8 per 100,000 cases, and <800 cases have been reported in the literature. There is no gender predominance, and it has been reported in patients aged 5 to 87 years, most often presenting in the sixth to seventh decade of life. Asbestos, tobacco, and environmental exposures are not considered to be risk factors. [5],[6] Significant hypoglycemia is related to SFTP in approximately 4% of cases. Hypoglycemia is more common in right-sided tumors and three times more common in women than in men. [6]

The hypoglycemia is the result of the tumors producing IGF-II. [6],[7],[8] Paraneoplastic hypoglycemia results from secretion of an unprocessed or incomplete high-molecular-weight (HMW) form of IGF-II. This HMW IGF-II is capable of activating insulin receptor, thereby inhibiting hepatic gluconeogenesis and increasing peripheral glucose uptake, which results in hypoglycemia. [9] The HMW IGF-II is also capable of binding to IGF-I receptors, leading to the suppression of the growth hormone by the pituitary, as well as a reduction of insulin, IGF-I, and IGF-binding protein-3 by the pancreas. [10] The detection of HMW IGF-II requires immunoblot analysis to distinguish it from normal IGF-II; unfortunately, this technology was not available to us. Nevertheless, all the features in this case fit the diagnosis of the Doege-Potter syndrome, including severe intractable hypoglycemia with suppressed insulin secretion and immediate resolution of the hypoglycemia following excision of the tumor. [9]

The chest CT scan is the key examination; it more clearly shows the size and location of the tumor and aids in surgical planning. CT scan imaging studies of SFTPs typically show a smooth, well-circumscribed, and homogenous mass, typically located peripherally and with the same density as muscle. [5] Ipsilateral pleural effusion was observed in 17% of cases. [6] Contrast enhancement on CT scans can be seen, depending on the vascularity of the tumor. However, CT scans cannot differentiate benign from malignant SFTP and difficulties in differentiating these tumors from others originating from the mediastinum or chest wall are possible. Furthermore, if the lesion is not homogeneous, the differential diagnosis of a bronchogenic carcinoma may also be more difficult. [6] Magnetic resonance imaging (MRI) can be a useful alternative, but its use is limited to establishing fibrous tissue characteristics. A benign characteristic can be predicted by low-intensity signals on T2-weighted imaging, in contrast to the high-intensity signals found in images of pulmonary carcinoma. The role of positron emission tomography (PET) scanning in the imaging of pleural disease is currently being debated and more experience is needed before conclusions can be reached. [11]

The definitive diagnosis was obtained by histopathology after resection. Identification of malignancy was established on the basis of criteria suggested by England et al., [12] which are widely accepted and used in most recent series. Moreover, immunohistochemical analysis played a key role in differentiating SFTPs from mesotheliomas and other similar neoplasms. SFTPs were positive for vimentin, but they lacked cytokeratin expression. In addition, most SFTPs were positive for CD34, which was a reliable marker for distinguishing SFTP from mesothelioma. Bcl-2 was also helpful in the diagnosis of SFTP, especially in the CD34-negative examples. Malignant SFTP was more frequently positive for Ki-67 than was benign SFTP. [13]

The differential diagnosis of pleural-based tumors includes lipoma, leiomyosarcoma, rhabdomyosarcoma, malignant fibrous histiocytoma, angiosarcoma, epithelioid hemangioma, and chondrosarcoma. Some may also manifest with hypoglycemia. However, paraneoplastic hypoglycemia in carcinomas is attributed to IGF-I rather than IGF-II. [14]

Complete surgical excision of the tumor remains the best treatment and most important predictor of clinical outcome. Removal of the tumor will normally resolve the hypoglycemia symptoms. The serum glucose level of our case returned to normal after the operation. Five months after the operation, this patient had no further hypoglycemia attack. Long-term follow-up is recommended due to varyingly high recurrence rates, particularly for tumors that were difficult to completely excise and had histological features for aggressive or malignant behavior.

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