Case Study
A case of huge solitary fibrous tumor with hypoglycemia
Asian Cardiovascular & Thoracic Annals 2015, Vol. 23(6) 719–721 ß The Author(s) 2014 Reprints and permissions: sagepub.co.uk/journalsPermissions.nav DOI: 10.1177/0218492314557182 aan.sagepub.com
Faouzi Alimi1, Chokri Kortas1, Nazih Chaouch1, Haythem Chemchik2 and Faouzi Limayem1
Abstract We report the case of a 64-year-old man with a huge solitary fibrous pleural tumor who presented with breathlessness and recurrent severe symptomatic hypoglycemia. The tumor was safely removed in toto via a median sternotomy. The patient had an uneventful postoperative recovery and no recurrent hypoglycemia.
Keywords Hypoglycemia, Solitary fibrous tumor, pleural
Introduction Solitary fibrous tumors (SFT) of the pleura are rare intrathoracic nonepithelial neoplasms. These tumors arise from the visceral pleura in 80% of cases, and most are asymptomatic and benign but complications or malignant transformation may occur. Doege-Potter syndrome, usually manifesting as recurrent and refractory hypoglycemia, has been reported since the 1930s as a common phenomenon in cases of SFT,1 possibly related to a malignant component or increasing size of the tumor. Surgery is the mainstay of treatment and offers spontaneous resolution of hypoglycemia. The surgery is often performed by a thoracotomy but an anterior thoracic approach may be helpful in cases of huge tumors and to prevent perioperative hemodynamic or vascular complications.
Case report A 64-year-old man was admitted with an opaque right hemithorax. He had no past history. He complained of breathlessness and recurrent severe symptomatic hypoglycemia. Computed tomography showed a large heterogeneous tumor with large necrotizing zones, occupying the entire right pleural space (Figure 1). The lung parenchyma was reduced to a thick volume. We performed a median sternotomy to safely remove a 35 15 15-cm multilobulated tumor (Figure 2). The tumor weighed 3.2 kg, and the resection was complete.
There were multiple pedicles arising from the mediastinal, parietal, and visceral pleura. The histopathological findings confirmed a solitary fibrous tumor composed predominantly of bland spindle cells with no nuclear atypia or high mitotic activity. On immunohistochemical analysis, the lesion was positive for CD34 and Blc2 and negative for PS100, smooth muscle actin, epithelial membrane antigen, and cytokeratin. The patient had an uneventful postoperative recovery and no recurrent hypoglycemia. There has been no tumor recurrence during 12 months of follow-up.
Discussion SFT of the pleura account for less than 5% of pleural tumors. Malignancy has been reported in this tumor localization, especially with huge masses, although most SFT are benign. They are observed frequently in the 6th and 7th decades of life but may appear at any age and equally in both sexes. SFT of the pleura 1 Department of Cardiothoracic Surgery, University Hospital Sahloul, Sousse, Tunisia 2 Department of Anesthesiology and Critical Care, University Hospital Sahloul, Sousse, Tunisia
Corresponding author: Faouzi Alimi, MD, Department of Cardiothoracic Surgery, Sahloul University Hospital, Sousse 4054, Tunisia. Email: [email protected]
Downloaded from aan.sagepub.com at Northeastern University on November 14, 2015
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Asian Cardiovascular & Thoracic Annals 23(6)
Figure 1. Computed tomography of the chest showing a large heterogeneous tumor occupying the whole right pleural cavity, adjacent to the mediastinum, with necrotizing zones.
Figure 2. The huge multilobulated 3.2-kg tumor removed from the right pleural cavity.
commonly presents as a small intrathoracic tumor and is usually resected through a conventional thoracotomy. In the case of a benign SFT of the pleura, the clinical course is often silent, but symptoms are present in malignant types and in larger ones. The latter is at high risk of developing malignant transformation and inducing refractory, severe, and recurrent hypoglycemia. It is recognized that spontaneous hypoglycemia may occur in large mesenchymal tumors including SFT. We observed severe and refractory hypoglycemia before surgery in this case. Non-islet cell tumor-induced hypoglycemia (NICTH), or paraneoplastic hypoglycemia, is mediated by the incompletely processed precursors of immunoglobulin F2 (bigIGF2), and the diagnosis can be established by the association of low serum insulin concentrations.2,3 This has been reported in several cases since 1930,1 whether malignant or
benign SFT of the pleura, and highlighted when the histological diagnosis was confirmed preoperatively.2–4 In our case, hypoglycemia was recognized preoperatively but not explored, and the computed tomography findings were nonspecific for SFT preoperatively but it was highly suspected from its shape, topography, and relationship. The hypoglycemia disappeared spontaneously postoperatively with no recurrence. In benign SFT of the pleura, this syndrome has been described and especially reported when the SFT are huge and have a high mitotic index. In our case, the large size of the tumor may explain NICTH; the histopathological findings ruled out NICTH-related malignant component etiology. Complete resection provides instant resolution of hypoglycemia. NICTH or paraneoplastic hypoglycemia is a very rare condition. It is a hypoinsulinemic hypoglycemia that should be considered in patients with SFT, and surgical management should be performed promptly to avoid wasting time in exploring the hypoglycemia. The classic thoracic approach is a posterolateral thoracotomy; lateral positioning of the patient with a large-sized tumor is not possible.5,6 Through a lateral approach in the case of a huge tumor, the relationship of the tumor to the mediastinal structures cannot be assessed. Furthermore, the pressure of the mass on the heart, especially on the right side, induces hemodynamic instability that could increase the mortality risk. In our patient, the lateral decubitus position of a conventional thoracotomy induced rapid hemodynamic deterioration, and fortunately, we acted quickly to avoid cardiac and hemodynamic complications due to decreased venous return. The anterior thoracic approaches (median sternotomy or clamshell thoracotomy) provide safe exposure for tumor resection and avoid perioperative complications.5,6 A median sternotomy allowed us to better expose the hilar and mediastinal vasculature, and removal of the large SFT was complete and secure. Huge SFT of the pleura are better resected through an anterior thoracic approach which offers better exposure of the surgical field, safe resection, and easy management in the event of vascular injury. Doege-Potter syndrome, inherent to large SFT due to insulin-like growth factor, manifests as severe and refractory hypoglycemia. Complete resection of SFT, whether benign or malignant, remains the only way cure it.7 Funding This research received no specific grant from any funding agency in the public, commerical, or not-for-profit sectors.
Conflict of interest statement None declared.
Downloaded from aan.sagepub.com at Northeastern University on November 14, 2015
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References 1. Doege KW. Fibro-sarcoma of the mediastinum. Ann Surg 1930; 92: 955–960. 2. Moat NE, Teale JD, Lea RE and Matthews AW. Spontaneous hypoglycemia and pleural fibroma: role of insulin like growth factors. Thorax 1991; 46: 932–933. 3. Mohammadi K, Abi Khalil C, Olivier S, Benabad I, Roussel R and Marre M. Paraneoplastic hypoglycemia in a patient with malignant solitary fibrous tumor. Endocrinol Diabetes Metab Case Rep 2014; 2014: 140026. doi: 10.1530/EDM-14-0026. 4. Zafar H, Takimoto CH and Weiss G. Doege-Potter syndrome: hypoglycemia associated with malignant solitary fibrous tumor. Med Oncol 2003; 20: 403–408.
5. Khan JH, Rahman SB, Clary-Macy C, et al. Giant solitary fibrous tumor of the pleura. Ann Thorac Surg 1998; 65: 1461–1464. 6. Kanlioglu Kuman N, Sen S, Cokpinar S, Ceylan E, Tataroglu C and Boga M. A hemiclamshell incision for a giant solitary fibrous tumor of the right hemithorax. Case Rep Surg 2012; 2012: 826454. doi: 10.1155/2012/826454. 7. Kalebi AY, Hale MJ, Wong ML, Hoffman T and Murray J. Surgically cured hypoglycemia secondary to pleural solitary fibrous tumor: case report and update review on the Doege-Potter syndrome. J Cardiothorac Surg 2009; 4: 45.
Downloaded from aan.sagepub.com at Northeastern University on November 14, 2015
A case of huge solitary fibrous tumor with hypoglycemia
Asian Cardiovascular & Thoracic Annals 2015, Vol. 23(6) 719–721 ß The Author(s) 2014 Reprints and permissions: sagepub.co.uk/journalsPermissions.nav DOI: 10.1177/0218492314557182 aan.sagepub.com
Faouzi Alimi1, Chokri Kortas1, Nazih Chaouch1, Haythem Chemchik2 and Faouzi Limayem1
Abstract We report the case of a 64-year-old man with a huge solitary fibrous pleural tumor who presented with breathlessness and recurrent severe symptomatic hypoglycemia. The tumor was safely removed in toto via a median sternotomy. The patient had an uneventful postoperative recovery and no recurrent hypoglycemia.
Keywords Hypoglycemia, Solitary fibrous tumor, pleural
Introduction Solitary fibrous tumors (SFT) of the pleura are rare intrathoracic nonepithelial neoplasms. These tumors arise from the visceral pleura in 80% of cases, and most are asymptomatic and benign but complications or malignant transformation may occur. Doege-Potter syndrome, usually manifesting as recurrent and refractory hypoglycemia, has been reported since the 1930s as a common phenomenon in cases of SFT,1 possibly related to a malignant component or increasing size of the tumor. Surgery is the mainstay of treatment and offers spontaneous resolution of hypoglycemia. The surgery is often performed by a thoracotomy but an anterior thoracic approach may be helpful in cases of huge tumors and to prevent perioperative hemodynamic or vascular complications.
Case report A 64-year-old man was admitted with an opaque right hemithorax. He had no past history. He complained of breathlessness and recurrent severe symptomatic hypoglycemia. Computed tomography showed a large heterogeneous tumor with large necrotizing zones, occupying the entire right pleural space (Figure 1). The lung parenchyma was reduced to a thick volume. We performed a median sternotomy to safely remove a 35 15 15-cm multilobulated tumor (Figure 2). The tumor weighed 3.2 kg, and the resection was complete.
There were multiple pedicles arising from the mediastinal, parietal, and visceral pleura. The histopathological findings confirmed a solitary fibrous tumor composed predominantly of bland spindle cells with no nuclear atypia or high mitotic activity. On immunohistochemical analysis, the lesion was positive for CD34 and Blc2 and negative for PS100, smooth muscle actin, epithelial membrane antigen, and cytokeratin. The patient had an uneventful postoperative recovery and no recurrent hypoglycemia. There has been no tumor recurrence during 12 months of follow-up.
Discussion SFT of the pleura account for less than 5% of pleural tumors. Malignancy has been reported in this tumor localization, especially with huge masses, although most SFT are benign. They are observed frequently in the 6th and 7th decades of life but may appear at any age and equally in both sexes. SFT of the pleura 1 Department of Cardiothoracic Surgery, University Hospital Sahloul, Sousse, Tunisia 2 Department of Anesthesiology and Critical Care, University Hospital Sahloul, Sousse, Tunisia
Corresponding author: Faouzi Alimi, MD, Department of Cardiothoracic Surgery, Sahloul University Hospital, Sousse 4054, Tunisia. Email: [email protected]
Downloaded from aan.sagepub.com at Northeastern University on November 14, 2015
720
Asian Cardiovascular & Thoracic Annals 23(6)
Figure 1. Computed tomography of the chest showing a large heterogeneous tumor occupying the whole right pleural cavity, adjacent to the mediastinum, with necrotizing zones.
Figure 2. The huge multilobulated 3.2-kg tumor removed from the right pleural cavity.
commonly presents as a small intrathoracic tumor and is usually resected through a conventional thoracotomy. In the case of a benign SFT of the pleura, the clinical course is often silent, but symptoms are present in malignant types and in larger ones. The latter is at high risk of developing malignant transformation and inducing refractory, severe, and recurrent hypoglycemia. It is recognized that spontaneous hypoglycemia may occur in large mesenchymal tumors including SFT. We observed severe and refractory hypoglycemia before surgery in this case. Non-islet cell tumor-induced hypoglycemia (NICTH), or paraneoplastic hypoglycemia, is mediated by the incompletely processed precursors of immunoglobulin F2 (bigIGF2), and the diagnosis can be established by the association of low serum insulin concentrations.2,3 This has been reported in several cases since 1930,1 whether malignant or
benign SFT of the pleura, and highlighted when the histological diagnosis was confirmed preoperatively.2–4 In our case, hypoglycemia was recognized preoperatively but not explored, and the computed tomography findings were nonspecific for SFT preoperatively but it was highly suspected from its shape, topography, and relationship. The hypoglycemia disappeared spontaneously postoperatively with no recurrence. In benign SFT of the pleura, this syndrome has been described and especially reported when the SFT are huge and have a high mitotic index. In our case, the large size of the tumor may explain NICTH; the histopathological findings ruled out NICTH-related malignant component etiology. Complete resection provides instant resolution of hypoglycemia. NICTH or paraneoplastic hypoglycemia is a very rare condition. It is a hypoinsulinemic hypoglycemia that should be considered in patients with SFT, and surgical management should be performed promptly to avoid wasting time in exploring the hypoglycemia. The classic thoracic approach is a posterolateral thoracotomy; lateral positioning of the patient with a large-sized tumor is not possible.5,6 Through a lateral approach in the case of a huge tumor, the relationship of the tumor to the mediastinal structures cannot be assessed. Furthermore, the pressure of the mass on the heart, especially on the right side, induces hemodynamic instability that could increase the mortality risk. In our patient, the lateral decubitus position of a conventional thoracotomy induced rapid hemodynamic deterioration, and fortunately, we acted quickly to avoid cardiac and hemodynamic complications due to decreased venous return. The anterior thoracic approaches (median sternotomy or clamshell thoracotomy) provide safe exposure for tumor resection and avoid perioperative complications.5,6 A median sternotomy allowed us to better expose the hilar and mediastinal vasculature, and removal of the large SFT was complete and secure. Huge SFT of the pleura are better resected through an anterior thoracic approach which offers better exposure of the surgical field, safe resection, and easy management in the event of vascular injury. Doege-Potter syndrome, inherent to large SFT due to insulin-like growth factor, manifests as severe and refractory hypoglycemia. Complete resection of SFT, whether benign or malignant, remains the only way cure it.7 Funding This research received no specific grant from any funding agency in the public, commerical, or not-for-profit sectors.
Conflict of interest statement None declared.
Downloaded from aan.sagepub.com at Northeastern University on November 14, 2015
Alimi et al.
721
References 1. Doege KW. Fibro-sarcoma of the mediastinum. Ann Surg 1930; 92: 955–960. 2. Moat NE, Teale JD, Lea RE and Matthews AW. Spontaneous hypoglycemia and pleural fibroma: role of insulin like growth factors. Thorax 1991; 46: 932–933. 3. Mohammadi K, Abi Khalil C, Olivier S, Benabad I, Roussel R and Marre M. Paraneoplastic hypoglycemia in a patient with malignant solitary fibrous tumor. Endocrinol Diabetes Metab Case Rep 2014; 2014: 140026. doi: 10.1530/EDM-14-0026. 4. Zafar H, Takimoto CH and Weiss G. Doege-Potter syndrome: hypoglycemia associated with malignant solitary fibrous tumor. Med Oncol 2003; 20: 403–408.
5. Khan JH, Rahman SB, Clary-Macy C, et al. Giant solitary fibrous tumor of the pleura. Ann Thorac Surg 1998; 65: 1461–1464. 6. Kanlioglu Kuman N, Sen S, Cokpinar S, Ceylan E, Tataroglu C and Boga M. A hemiclamshell incision for a giant solitary fibrous tumor of the right hemithorax. Case Rep Surg 2012; 2012: 826454. doi: 10.1155/2012/826454. 7. Kalebi AY, Hale MJ, Wong ML, Hoffman T and Murray J. Surgically cured hypoglycemia secondary to pleural solitary fibrous tumor: case report and update review on the Doege-Potter syndrome. J Cardiothorac Surg 2009; 4: 45.
Downloaded from aan.sagepub.com at Northeastern University on November 14, 2015
