- Email: [email protected]
Benign but Not Harmless: Solitary Fibrous Tumor
Accepted Manuscript Benign but not harmless: solitary fibrous tumor Jason Chertoff, MD, MPH, Azka Ali, MD, Brandon Dyer, MD, James Wynne, MD PII:
S0002-9343(16)31219-0
DOI:
10.1016/j.amjmed.2016.11.011
Reference:
AJM 13805
To appear in:
The American Journal of Medicine
Received Date: 19 September 2016 Revised Date:
4 November 2016
Accepted Date: 4 November 2016
Please cite this article as: Chertoff J, Ali A, Dyer B, Wynne J, Benign but not harmless: solitary fibrous tumor, The American Journal of Medicine (2016), doi: 10.1016/j.amjmed.2016.11.011. This is a PDF file of an unedited manuscript that has been accepted for publication. As a service to our customers we are providing this early version of the manuscript. The manuscript will undergo copyediting, typesetting, and review of the resulting proof before it is published in its final form. Please note that during the production process errors may be discovered which could affect the content, and all legal disclaimers that apply to the journal pertain.
ACCEPTED MANUSCRIPT 16-1633ChertoffCHS, Page 1 of 5
By
RI PT
Benign but not harmless: solitary fibrous tumor
Jason Chertoff MD, MPH (Corresponding Author) * Azka Ali, MD **
James Wynne, MD *
SC
Brandon Dyer, MD **
M AN U
* University of Florida College of Medicine - Department of Internal Medicine - Division of Pulmonary and Critical Care Medicine - North Florida/South Georgia Veterans Administration Hospital Pulmonary Division
** University of Florida College of Medicine - Department of Internal Medicine
Conception and design: JC, AA, BD, JW; Drafting the manuscript for important intellectual
Funding Source: None Conflicts of Interest: None
TE D
content: JC, AA, BD, JW
Running Head: A Gigantic Tumor of the Pleura
AC C
EP
** None of the images or any of the submitted material has been previously published.
Contact: Corresponding Author Jason Chertoff MD, MPH 917-232-0297 [email protected] University of Florida College of Medicine 1600 SW Archer Road; Gainesville, FL; 32608
ACCEPTED MANUSCRIPT 16-1633ChertoffCHS, Page 2 of 5
Images in Radiology
Robert G. Stern, MD, Images in Radiology Editor
RI PT
Benign but not harmless: solitary fibrous tumor
a
SC
Jason Chertoff,a,c MD, MPH, Azka Ali, MD,b Brandon Dyer, MD,b James Wynne, MDa,c
Division of Pulmonary, Critical Care, and Sleep Medicine, bDepartment of Medicine, University
Veterans Administration System
M AN U
of Florida College of Medicine and the cDivision of Pulmonology, North Florida/South Georgia
Requests for reprints should be addressed to Jason Chertoff, MD, MPH, University of Florida College of Medicine, 1600 SW Archer Road, Gainesville, FL, 32608.
PRESENTATION
TE D
Email: [email protected]
EP
For one unfortunate patient, benign histopathology did not lead to a good outcome. A 74-yearold woman presented with dyspnea on exertion and altered mental status. She had no history of
AC C
lung disease and denied other cardiopulmonary, abdominal, or constitutional symptoms. Her past medical history, besides many years of tobacco abuse, was unremarkable.
ASSESSMENT
While the patient did not appear to be in respiratory distress, a physical examination revealed hypoxemia on room air with an initial oxygen saturation of 86%. Percussion of the right lower and middle fields produced stony dull sounds, and breath sounds were absent in these areas. Mild wheezing accompanied by prolonged expiration was audible in all lung fields on the left side. Arterial blood gas demonstrated chronic respiratory acidosis and hypoxemia.
ACCEPTED MANUSCRIPT 16-1633ChertoffCHS, Page 3 of 5 A radiograph of the chest showed a large opacity occupying the right mid- to lower thorax (Figure 1). Computerized tomography (CT) (Figure 2) identified a giant 18 cm x 13 cm x 15 cm mass in the right hemithorax. The growth appeared to compress the right middle lobe and restrict expansion of the right upper and lower lobes. No infiltrates, effusion, or evidence of
process (Figure 3). No complications occurred.
DIAGNOSIS
RI PT
metastases were seen. A CT-guided biopsy was performed to investigate the suspected neoplastic
SC
The pathology study classified the mass as a solitary fibrous tumor, a diagnosis confirmed by CD34-positive staining (Figures 4). These rare primary tumors of the pleura originate from the
M AN U
mesenchymal layer.1-5 Overall, primary tumors of the pleura can be diffuse or localized.5 Diffuse pleural neoplasms arise from mesothelial cells, are associated with asbestos exposure, and have a highly malignant potential, while localized neoplasms, termed solitary fibrous tumors, arise from the submesothelial layer and tend to follow a more benign course; 10-20% are malignant.1-3 More than 50% of benign solitary fibrous tumors are asymptomatic, but others can manifest with intrathoracic or constitutional symptoms.2 Our patient’s case appears to be the first reported
TE D
solitary fibrous tumor diagnosed within the Veterans Health Administration. It reminds us to consider benign pleural tumors in the differential diagnosis of lung masses.
MANAGEMENT
EP
Wide surgical resection without adjuvant chemotherapy is, if feasible, the optimal treatment strategy for patients with a solitary fibrous tumor.2,5 The likelihood of recurrence and mortality
AC C
mainly depends on whether the tumor is benign or malignant and whether the tumor location and patient’s health status permit resection. Benign pedunculated tumors that are excised with clear margins have a recurrence rate of 2%.1 In contrast, sessile malignant tumors can recur up to 63% of the time.
Our patient’s tumor proved benign, but its large size caused marked compressive
atelectasis and associated severe symptoms. As solitary fibrous tumors continue to grow, they inevitably squeeze the bronchi further, producing a mass effect on the mediastinum.4 Cardiothoracic surgeons were consulted on the potential for resection of our patient’s tumor. A clamshell thoracotomy was the only surgical option, and given her greatly diminished
ACCEPTED MANUSCRIPT 16-1633ChertoffCHS, Page 4 of 5 respiratory reserve, she was deemed to be at high risk for intraoperative mortality and thus, a poor candidate. She was discharged home with her family.
References
RI PT
1. Cardillo G, Facciolo F, Cavazzana AO, Capece G, Gasparri R, Martelli M. Localized (solitary) fibrous tumors of the pleura: an analysis of 55 patients. Ann Thorac Surg. 2000;70:1808-1812.
Opin Pulm Med. 2012;18:339-346.
SC
2. Cardillo G, Lococo F, Carleo F, Martelli M. Solitary fibrous tumors of the pleura. Curr
3. Ali SZ, Hoon V, Hoda S, Heelan R, Zakowski MF. Solitary fibrous tumor. A cytologic-
M AN U
histologic study with clinical, radiologic, and immunohistochemical correlations. Cancer. 1997;81:116-121.
4. de Perrot M, Kurt AM, Robert JH, Borisch B, Spiliopoulos A. Clinical behavior of solitary fibrous tumors of the pleura. Ann Thorac Surg. 1999;67:1456-1459. 5. Khan JH, Rahman SB, Clary-Macy C, et al. Giant solitary fibrous tumor of the pleura.
EP
Figure Legends
TE D
Ann Thorac Surg. 1998;65:1461-1464.
Figure 1. A chest x-ray revealed a large mass. A, The tumor abutted the right diaphragm in this
AC C
posterior-anterior view. B, A lateral view shows the same.
Figure 2. Computed tomography (CT) of the chest disclosed the sizable tumor and its extensive occupation of the right hemithorax. A, This is the axial view. B, A coronal view is provided. C, The sagittal view is also impressive.
Figure 3. A, CT shows the mass in the axial view. B, Placement of the biopsy needle within the tumor is illustrated.
ACCEPTED MANUSCRIPT 16-1633ChertoffCHS, Page 5 of 5 Figure 4. A, The pathology slides indicated that the mass was a solitary fibrous tumor (low magnification). B, A high-magnification view can be seen here. C, CD34 staining confirmed that the patient had a solitary fibrous tumor (low magnification). D, This is a high-magnification
AC C
EP
TE D
M AN U
SC
RI PT
image of the stained specimen.
AC C
EP
TE D
M AN U
SC
RI PT
ACCEPTED MANUSCRIPT
AC C
EP
TE D
M AN U
SC
RI PT
ACCEPTED MANUSCRIPT
AC C
EP
TE D
M AN U
SC
RI PT
ACCEPTED MANUSCRIPT
AC C
EP
TE D
M AN U
SC
RI PT
ACCEPTED MANUSCRIPT
AC C
EP
TE D
M AN U
SC
RI PT
ACCEPTED MANUSCRIPT
S0002-9343(16)31219-0
DOI:
10.1016/j.amjmed.2016.11.011
Reference:
AJM 13805
To appear in:
The American Journal of Medicine
Received Date: 19 September 2016 Revised Date:
4 November 2016
Accepted Date: 4 November 2016
Please cite this article as: Chertoff J, Ali A, Dyer B, Wynne J, Benign but not harmless: solitary fibrous tumor, The American Journal of Medicine (2016), doi: 10.1016/j.amjmed.2016.11.011. This is a PDF file of an unedited manuscript that has been accepted for publication. As a service to our customers we are providing this early version of the manuscript. The manuscript will undergo copyediting, typesetting, and review of the resulting proof before it is published in its final form. Please note that during the production process errors may be discovered which could affect the content, and all legal disclaimers that apply to the journal pertain.
ACCEPTED MANUSCRIPT 16-1633ChertoffCHS, Page 1 of 5
By
RI PT
Benign but not harmless: solitary fibrous tumor
Jason Chertoff MD, MPH (Corresponding Author) * Azka Ali, MD **
James Wynne, MD *
SC
Brandon Dyer, MD **
M AN U
* University of Florida College of Medicine - Department of Internal Medicine - Division of Pulmonary and Critical Care Medicine - North Florida/South Georgia Veterans Administration Hospital Pulmonary Division
** University of Florida College of Medicine - Department of Internal Medicine
Conception and design: JC, AA, BD, JW; Drafting the manuscript for important intellectual
Funding Source: None Conflicts of Interest: None
TE D
content: JC, AA, BD, JW
Running Head: A Gigantic Tumor of the Pleura
AC C
EP
** None of the images or any of the submitted material has been previously published.
Contact: Corresponding Author Jason Chertoff MD, MPH 917-232-0297 [email protected] University of Florida College of Medicine 1600 SW Archer Road; Gainesville, FL; 32608
ACCEPTED MANUSCRIPT 16-1633ChertoffCHS, Page 2 of 5
Images in Radiology
Robert G. Stern, MD, Images in Radiology Editor
RI PT
Benign but not harmless: solitary fibrous tumor
a
SC
Jason Chertoff,a,c MD, MPH, Azka Ali, MD,b Brandon Dyer, MD,b James Wynne, MDa,c
Division of Pulmonary, Critical Care, and Sleep Medicine, bDepartment of Medicine, University
Veterans Administration System
M AN U
of Florida College of Medicine and the cDivision of Pulmonology, North Florida/South Georgia
Requests for reprints should be addressed to Jason Chertoff, MD, MPH, University of Florida College of Medicine, 1600 SW Archer Road, Gainesville, FL, 32608.
PRESENTATION
TE D
Email: [email protected]
EP
For one unfortunate patient, benign histopathology did not lead to a good outcome. A 74-yearold woman presented with dyspnea on exertion and altered mental status. She had no history of
AC C
lung disease and denied other cardiopulmonary, abdominal, or constitutional symptoms. Her past medical history, besides many years of tobacco abuse, was unremarkable.
ASSESSMENT
While the patient did not appear to be in respiratory distress, a physical examination revealed hypoxemia on room air with an initial oxygen saturation of 86%. Percussion of the right lower and middle fields produced stony dull sounds, and breath sounds were absent in these areas. Mild wheezing accompanied by prolonged expiration was audible in all lung fields on the left side. Arterial blood gas demonstrated chronic respiratory acidosis and hypoxemia.
ACCEPTED MANUSCRIPT 16-1633ChertoffCHS, Page 3 of 5 A radiograph of the chest showed a large opacity occupying the right mid- to lower thorax (Figure 1). Computerized tomography (CT) (Figure 2) identified a giant 18 cm x 13 cm x 15 cm mass in the right hemithorax. The growth appeared to compress the right middle lobe and restrict expansion of the right upper and lower lobes. No infiltrates, effusion, or evidence of
process (Figure 3). No complications occurred.
DIAGNOSIS
RI PT
metastases were seen. A CT-guided biopsy was performed to investigate the suspected neoplastic
SC
The pathology study classified the mass as a solitary fibrous tumor, a diagnosis confirmed by CD34-positive staining (Figures 4). These rare primary tumors of the pleura originate from the
M AN U
mesenchymal layer.1-5 Overall, primary tumors of the pleura can be diffuse or localized.5 Diffuse pleural neoplasms arise from mesothelial cells, are associated with asbestos exposure, and have a highly malignant potential, while localized neoplasms, termed solitary fibrous tumors, arise from the submesothelial layer and tend to follow a more benign course; 10-20% are malignant.1-3 More than 50% of benign solitary fibrous tumors are asymptomatic, but others can manifest with intrathoracic or constitutional symptoms.2 Our patient’s case appears to be the first reported
TE D
solitary fibrous tumor diagnosed within the Veterans Health Administration. It reminds us to consider benign pleural tumors in the differential diagnosis of lung masses.
MANAGEMENT
EP
Wide surgical resection without adjuvant chemotherapy is, if feasible, the optimal treatment strategy for patients with a solitary fibrous tumor.2,5 The likelihood of recurrence and mortality
AC C
mainly depends on whether the tumor is benign or malignant and whether the tumor location and patient’s health status permit resection. Benign pedunculated tumors that are excised with clear margins have a recurrence rate of 2%.1 In contrast, sessile malignant tumors can recur up to 63% of the time.
Our patient’s tumor proved benign, but its large size caused marked compressive
atelectasis and associated severe symptoms. As solitary fibrous tumors continue to grow, they inevitably squeeze the bronchi further, producing a mass effect on the mediastinum.4 Cardiothoracic surgeons were consulted on the potential for resection of our patient’s tumor. A clamshell thoracotomy was the only surgical option, and given her greatly diminished
ACCEPTED MANUSCRIPT 16-1633ChertoffCHS, Page 4 of 5 respiratory reserve, she was deemed to be at high risk for intraoperative mortality and thus, a poor candidate. She was discharged home with her family.
References
RI PT
1. Cardillo G, Facciolo F, Cavazzana AO, Capece G, Gasparri R, Martelli M. Localized (solitary) fibrous tumors of the pleura: an analysis of 55 patients. Ann Thorac Surg. 2000;70:1808-1812.
Opin Pulm Med. 2012;18:339-346.
SC
2. Cardillo G, Lococo F, Carleo F, Martelli M. Solitary fibrous tumors of the pleura. Curr
3. Ali SZ, Hoon V, Hoda S, Heelan R, Zakowski MF. Solitary fibrous tumor. A cytologic-
M AN U
histologic study with clinical, radiologic, and immunohistochemical correlations. Cancer. 1997;81:116-121.
4. de Perrot M, Kurt AM, Robert JH, Borisch B, Spiliopoulos A. Clinical behavior of solitary fibrous tumors of the pleura. Ann Thorac Surg. 1999;67:1456-1459. 5. Khan JH, Rahman SB, Clary-Macy C, et al. Giant solitary fibrous tumor of the pleura.
EP
Figure Legends
TE D
Ann Thorac Surg. 1998;65:1461-1464.
Figure 1. A chest x-ray revealed a large mass. A, The tumor abutted the right diaphragm in this
AC C
posterior-anterior view. B, A lateral view shows the same.
Figure 2. Computed tomography (CT) of the chest disclosed the sizable tumor and its extensive occupation of the right hemithorax. A, This is the axial view. B, A coronal view is provided. C, The sagittal view is also impressive.
Figure 3. A, CT shows the mass in the axial view. B, Placement of the biopsy needle within the tumor is illustrated.
ACCEPTED MANUSCRIPT 16-1633ChertoffCHS, Page 5 of 5 Figure 4. A, The pathology slides indicated that the mass was a solitary fibrous tumor (low magnification). B, A high-magnification view can be seen here. C, CD34 staining confirmed that the patient had a solitary fibrous tumor (low magnification). D, This is a high-magnification
AC C
EP
TE D
M AN U
SC
RI PT
image of the stained specimen.
AC C
EP
TE D
M AN U
SC
RI PT
ACCEPTED MANUSCRIPT
AC C
EP
TE D
M AN U
SC
RI PT
ACCEPTED MANUSCRIPT
AC C
EP
TE D
M AN U
SC
RI PT
ACCEPTED MANUSCRIPT
AC C
EP
TE D
M AN U
SC
RI PT
ACCEPTED MANUSCRIPT
AC C
EP
TE D
M AN U
SC
RI PT
ACCEPTED MANUSCRIPT