- Email: [email protected]
Collision Tumor of the Mediastinum: A Rare Entity
330
CASE REPORT KAIDAR-PERSON ET AL SYNCHRONOUS TUMORS OF THE MEDIASTINUM
considered challenging or time-consuming [8]. On the contrary, it is established as the procedure of choice for aortic valve reoperations. Lateral chest roentgenography is useful to evaluate the sternal-aortic distance. This less invasive approach ensured reduced postoperative ventilation time and shortened the intensive care unit stay, allowing for low morbidity and excellent outcome. Exposure of the ascending aorta is excellent, and reentry problems are often avoided because adhesion dissection is limited to the structures directly involved in the procedure. We would like to express our gratitude to Professor Thomas M Egan, MD, MS, (University of North Carolina, Chapel Hill, NC) for editorial revision of the manuscript.
References
FEATURE ARTICLES
1. Ojo AO, Held PJ, Port FK, et al. Chronic renal failure after transplantation of a nonrenal organ. N Engl J Med 2003;349: 931– 40. 2. Lyu DM, Zamora MR. Medical complications of lung transplantation. Proc Am Thorac Soc 2009;6:101–7. 3. Mitruka SN, Griffith BP, Kormos RL, et al. Cardiac operations in solid-organ transplant recipients. Ann Thorac Surg 1997; 64:1270 – 8. 4. Musci M, Yankah CA, Klose H, et al. Heart valve operations in solid organ recipients: an 18-year single-center experience. Transplantation 2007;84:592–7. 5. Vistarini N, D’Armini AM, Pellegrini C, et al. Minimally invasive aortic valve replacement in a transplanted heart. Ann Thorac Surg 2010;90:1688 –90. 6. Christie JD, Edwards LB, Kucheryavaya AY, et al. The Registry of the International Society for Heart and Lung Transplantation: Twenty-eighth Adult Lung and Heart-Lung Transplant Report—2011. J Heart Lung Transplant 2011;30:1104 –22. 7. Manne MB, Shrestha NK, Lytle BW, et al. Outcomes after surgical treatment of native and prosthetic valve infective endocarditis. Ann Thorac Surg 2012;93:489 –93. 8. Totaro P, Carlini S, Pozzi M, et al. Minimally invasive approach for complex cardiac surgery procedures. Ann Thorac Surg 2009;88:462– 6.
Ann Thorac Surg 2013;95:330 –2
described mostly at the esophagogastric junction, where a squamous cell carcinoma of esophageal origin collides with a gastric adenocarcinoma. The present case is of 2 histologically distinct tumors with aggressive features occurring at the mediastinum. (Ann Thorac Surg 2013;95:330 –2) © 2013 by The Society of Thoracic Surgeons
T
he simultaneous occurrence of 2 distinct neoplasms derived from different cells of origin is a recognized rare entity called collision tumor. Diagnosis of a collision tumor requires that the 2 tumors be histologically distinct, which is often challenging in cases of poorly differentiated neoplasms. The case presented in this report is that of a collision tumor occurring in the mediastinum. A 56-year-old man presented with swelling of his right arm and right leg. Past medical history included diabetes mellitus, hypertension, and a cerebrovascular accident 4 years before presentation. Chest computed tomography indicated a mediastinal mass (Fig 1). Fine-needle aspiration was positive for spindle cell tumor, most probably sarcoma or sarcomatoid carcinoma. The patient underwent surgical resection of the mediastinal mass, and a description of the mass during the surgical procedure indicated gross involvement of the right lung, adhesion to the ascending aorta without invasion, and adhesion to the right phrenic nerve. The tumor extended to the left side of the chest and adhered to the pericardium without invasion but invaded the left upper lobe. The tumor was attached to the left chest wall and left mammary artery. Cranially, the tumor extended to the lower left side of the neck, where it involved the left phrenic nerve. Estimated size was 11 cm and it was composed of numerous nodules. Pathologic evaluation was performed at our institution and then sent for reevaluation by a highly regarded overseas center, which indicated that this was a collision
Collision Tumor of the Mediastinum: A Rare Entity Orit Kaidar-Person, MD, Inna Naroditsky, MD, Ludmila Guralnik, MD, Ran Kremer, MD, and Gil Bar-Sela, MD Division of Oncology, Pathology Institute, Department of Diagnostic Radiology, and Department of Thoracic Surgery, Rambam Health Care Campus & Technion-Israel Institute of Technology, Haifa, Israel
A collision tumor is a rare entity consisting of 2 different tumors that, because of proximity, merge together. The diagnosis of a collision tumor requires that the 2 tumors be histologically distinct. This phenomenon has been Accepted for publication June 4, 2012. Correspondence to Dr Bar-Sela, Division of Oncology, Rambam Health Care Campus, POB 9602, Haifa 31096 Israel; e-mail: g_barsela@ rambam.health.gov.il.
© 2013 by The Society of Thoracic Surgeons Published by Elsevier Inc
Fig 1. Chest computed tomography indicated a large mediastinal mass. A large lobulated inhomogeneous soft tissue mass in the left thymic lobe is seen. The right thymic lobe is enlarged as well by a lobulated, mostly homogeneous soft tissue mass. The masses are separate from each other. 0003-4975/$36.00 http://dx.doi.org/10.1016/j.athoracsur.2012.06.030
tumor. One part of the tumor consisted of lymphoepithelial thymoma (World Health Organization type B2) (Fig 2). Immunostaining for cytokeratin AE1/AE3 and p63 highlighted numerous scattered epithelial cells admixed with the lymphoid infiltrate, supporting this diagnosis. There was already evidence of microinvasion of the adjacent mediastinal fat. Since there was obvious gross involvement of the lung and overt invasion at the time of operation, this tumor corresponded to a Masaoka stage III thymoma. Other parts of the mass had completely different features and corresponded to a malignant spindle cell tumor with areas of necrosis and high mitotic count (Fig 3). The differential diagnosis of that part of the specimen included spindle cell thymic carcinoma, synovial sarcoma, or other high-grade malignant spindle cell sarcoma. Given the presence of a thymoma adjacent to this mass, the first possibility that needed to be eliminated was that of a spindle cell thymic carcinoma arising from the thymoma [1]. Spindle epithelial tumor with thymuslike differentiation is a rare and distinctive low-grade neoplasm of thymic differentiation. The tumor usually presents in the thyroid or lateral neck of children and adolescents and could mimic spindle cell carcinoma [1]. A battery of immunohistochemical stains for cytokeratin AE1/AE3, CK19, and p63 was used, and results were all negative, essentially ruling out a thymic carcinoma. There was no thymic tissue within the tumor. The AE1/ AE3 stain did reveal a few isolated scattered cells showing cytoplasmic staining, but the pattern of distribution of these cells was more suggestive of entrapped residual epithelial cells than being part of the neoplastic proliferation. There were also numerous dilatated glandular spaces scattered throughout the lesion, which probably corresponded to entrapped pulmonary epithelium. Stains for bcl-2 and CD99 showed focal cytoplasmic positivity, which was stronger in the latter. Staining for CD34 was negative in the spindle cells and only labeled entrapped blood vessels. A Ki-67 proliferation marker was positive in about 15% of the tumor cell nuclei. The 2
CASE REPORT KAIDAR-PERSON ET AL SYNCHRONOUS TUMORS OF THE MEDIASTINUM
331
Fig 3. Malignant spindle cell tumor with nuclear atypia and mitotic activity was diagnosed as a malignant solitary fibrous tumor. (Hematoxylin and eosin; ⫻200.)
most likely diagnostic possibilities were probably synovial sarcoma and malignant solitary fibrous tumor. Although synovial sarcoma is positive for cytokeratin (in a spotty pattern, as in this tumor), bcl-2, and CD99, the histologic features in this case are unusual for synovial sarcoma, particularly the areas of linear stromal hyalinization demonstrated here. Additionally, fluorescence in situ hybridization for t(X;18) was negative. Conversely, the histologic features would seem to fit better a solitary fibrous tumor (ie, variegation of patterns, linear stromal hyalinization, short storiform pattern, and so on). Given that solitary fibrous tumor can be negative for CD34 in up to 35% of cases and is also positive for CD99 and bcl-2, it was concluded that this tumor was a malignant solitary fibrous tumor [2]. The presence of areas of necrosis automatically placed this in the high-grade sarcoma category. Spindle cells seen in fine-needle aspiration biopsy of mediastinal lesions can be a component of a wide variety of benign and malignant conditions [3]. Accurate diagnosis is usually achieved after surgical resection [3]. Extensive synchronous thoracic tumors are rare but have been reported, with grave prognoses [4 – 6]. Solitary fibrous tumors are rare and are mostly described as arising from the pleura [7, 8]. The current malignancy showed histologic features such as necrosis and high mitotic counts. These were reported to be associated with aggressive behavior, recurrences, and distant metastases [7]. Other malignant features included a tendency to be larger and demonstration of positive uptake on positron emission tomography [8].
Comment
Fig 2. Lymphoepithelial thymoma (World Health Organization type B2). Numerous scattered epithelial cells admixed with lymphoid infiltrate are seen. (Hematoxylin and eosin; ⫻200.)
A retrospective analysis of data collected from the population-based Surveillance, Epidemiology, and End Results of malignant solitary fibrous tumors of the lung, pleura, or mediastinum indicated that surgical resection has the most implication on survival [7]. Although it is a relatively rare malignancy and was mostly reported as arising from the
FEATURE ARTICLES
Ann Thorac Surg 2013;95:330 –2
332
CASE REPORT MARTENS ET AL MULTIPLE ANEURYSMS IN PATIENT WITH AOS
pleura, it seems that free surgical margins are of the utmost importance [7–9]. Taking that into account, along with the positive surgical margins in the case presented, it was decided that adjuvant radiotherapy be delivered to the mediastinum, to a total dose of 64 Gy (2 Gy per fraction). The malignant solitary fibrous tumor described in this patient did not arise from the pleura. It presented in proximity to another rare mediastinal tumor. We were unable to find histologic or genetic features linking these tumors together, indicating that this is a case of collision mediastinal tumor.
Ann Thorac Surg 2013;95:332–5
The aneurysms-osteoarthritis syndrome (AOS) was recently described and encompasses multiple aneurysms and tortuosity of the great arteries. Most patients have early-onset osteoarthritis. We report the diagnosis, treatment, and follow-up of a patient presenting with bilateral aneurysms of the common iliac arteries and an ascending aortic aneurysm. After proper surgical treatment, genetic studies revealed a causal mutation in the SMAD3 gene. (Ann Thorac Surg 2013;95:332–5) © 2013 by The Society of Thoracic Surgeons
I The authors thank Professor Saul Suster, Head of the Pathology Department at the Medical College of Wisconsin, Milwaukee, Wisconsin, for having reviewed the case in consultation.
References
FEATURE ARTICLES
1. Su L, Beals T, Bernacki EG, Giordano TJ. Spindle epithelial tumor with thymus-like differentiation: a case report with cytologic, histologic, immunohistologic, and ultrastructural findings. Mod Pathol 1997;10:510 – 4. 2. Ali SZ, Hoon V, Hoda S, Heelan R, Zakowski MF. Solitary fibrous tumor. A cytologic-histologic study with clinical, radiologic, and immunohistochemical correlations. Cancer 1997;25:81:116 –21. 3. Slagel DD, Powers CN, Melaragno MJ, Geisinger KR, Frable WJ, Silverman JF. Spindle-cell lesions of the mediastinum: diagnosis by fine-needle aspiration biopsy. Diagn Cytopathol 1997;17:167–76. 4. Nishimura M, Kodama T, Nishiyama H, Nishiwaki Y, Yokose T, Shimosato Y. A case of sarcomatoid carcinoma of the thymus. Pathol Int 1997;47:260 –3. 5. Okumori M, Mabuchi M, Nakagawa M. Malignant thymoma associated with liposarcoma of the mediastinum—a case report. Jpn J Surg 1983;13:512– 8. 6. Cushing B, Bhanot PK, Watts FB, Jr, Hertzler JH, Brough AJ. Rhabdomyosarcoma and benign teratoma. Pediatr Pathol 1983;1:345– 8. 7. Milano MT, Singh DP, Zhang H. Thoracic malignant solitary fibrous tumors: a population-based study of survival. J Thorac Dis 2011;3:99 –104. 8. Magdeleinat P, Alifano M, Petino A, et al. Solitary fibrous tumors of the pleura: clinical characteristics, surgical treatment and outcome. Eur J Cardiothorac Surg 2002;21:1087–93. 9. Kohler M, Clarenbach CF, Kestenholz P, et al. Diagnosis, treatment and long-term outcome of solitary fibrous tumours of the pleura. Eur J Cardiothorac Surg 2007;32:403– 8.
Multiple Aneurysms in a Patient With Aneurysms-Osteoarthritis Syndrome Thomas Martens, MD, Isabelle Van Herzeele, MD, PhD, Frederic De Ryck, MD, PhD, Marjolijn Renard, MD, PhD, Anne De Paepe, MD, PhD, Katrien François, MD, PhD, Frank Vermassen, MD, PhD, and Julie De Backer, MD, PhD Department of Thoracic and Vascular Surgery, Centre for Medical Genetics, Department of Cardiac Surgery, and Department of Cardiology, University Hospital Gent, Gent, Belgium © 2013 by The Society of Thoracic Surgeons Published by Elsevier Inc
n the spectrum of inheritable connective tissue disorders with arterial involvement, a new genetic syndrome caused by mutations in the SMAD3 gene was recently delineated and named aneurysms-osteoarthritis syndrome (AOS) [1, 2]. Since AOS is a recently discovered clinical entity, no data regarding its prevalence are available yet, and the clinical suspicion for the disease is still low. With this case presentation, we want to raise the awareness for AOS among clinicians and emphasize the need for extended clinical investigation, especially in young patients.
A 32-year-old male patient was referred to the vascular surgeon because of unspecified pulsatile sensations in the lower abdomen for 4 weeks. He had a medical history of lower back pain and lumbar hernia. Clinical examination revealed a thrill over the right fossa with tenderness on palpation. Bilateral femoral pulses were equal in strength, with a full complement of pulses in both lower limbs. A computed tomographic scan of the abdomen showed a saccular aneurysm (maximal diameter of 69 mm) of the right common iliac artery and a partially thrombosed left common iliac artery aneurysm (Fig 1). An aortobifurcated graft was implanted in an end-to-end configuration to both internal iliac arteries; both external iliac arteries were reimplanted in an end-to-side fashion (Gelsoft prosthesis 16 mm ⫽ proximal diameter, 8 mm ⫽ distal diameter, Vascutek, Renfrewshire, UK). No postoperative complications occurred. Diagnostic workup including transthoracic echocardiography revealed a dilatated aortic root of 49 mm at the level of the sinuses of Valsalva. Magnetic resonance angiography from head to pelvis confirmed the aortic root dilatation (Fig 2) and showed moderate tortuosity of the thoracic and abdominal aorta and iliac arteries. The vertebral and internal carotid arteries also showed greater tortuosity than expected for the age of the patient (Fig 3). Additional clinical features included a narrow face with discrete proptosis of the eyes. The uvula showed a raphe and was slightly deviated. There was no joint hyperlaxity and the skin was normal. Roentgenograms of Accepted for publication May 11, 2012. Address correspondence to Dr Martens, Department of Thoracic and Vascular Surgery, University Hospital Gent, De Pintelaan 185, 9000 Gent, Belgium; e-mail: [email protected].
0003-4975/$36.00 http://dx.doi.org/10.1016/j.athoracsur.2012.05.085
CASE REPORT KAIDAR-PERSON ET AL SYNCHRONOUS TUMORS OF THE MEDIASTINUM
considered challenging or time-consuming [8]. On the contrary, it is established as the procedure of choice for aortic valve reoperations. Lateral chest roentgenography is useful to evaluate the sternal-aortic distance. This less invasive approach ensured reduced postoperative ventilation time and shortened the intensive care unit stay, allowing for low morbidity and excellent outcome. Exposure of the ascending aorta is excellent, and reentry problems are often avoided because adhesion dissection is limited to the structures directly involved in the procedure. We would like to express our gratitude to Professor Thomas M Egan, MD, MS, (University of North Carolina, Chapel Hill, NC) for editorial revision of the manuscript.
References
FEATURE ARTICLES
1. Ojo AO, Held PJ, Port FK, et al. Chronic renal failure after transplantation of a nonrenal organ. N Engl J Med 2003;349: 931– 40. 2. Lyu DM, Zamora MR. Medical complications of lung transplantation. Proc Am Thorac Soc 2009;6:101–7. 3. Mitruka SN, Griffith BP, Kormos RL, et al. Cardiac operations in solid-organ transplant recipients. Ann Thorac Surg 1997; 64:1270 – 8. 4. Musci M, Yankah CA, Klose H, et al. Heart valve operations in solid organ recipients: an 18-year single-center experience. Transplantation 2007;84:592–7. 5. Vistarini N, D’Armini AM, Pellegrini C, et al. Minimally invasive aortic valve replacement in a transplanted heart. Ann Thorac Surg 2010;90:1688 –90. 6. Christie JD, Edwards LB, Kucheryavaya AY, et al. The Registry of the International Society for Heart and Lung Transplantation: Twenty-eighth Adult Lung and Heart-Lung Transplant Report—2011. J Heart Lung Transplant 2011;30:1104 –22. 7. Manne MB, Shrestha NK, Lytle BW, et al. Outcomes after surgical treatment of native and prosthetic valve infective endocarditis. Ann Thorac Surg 2012;93:489 –93. 8. Totaro P, Carlini S, Pozzi M, et al. Minimally invasive approach for complex cardiac surgery procedures. Ann Thorac Surg 2009;88:462– 6.
Ann Thorac Surg 2013;95:330 –2
described mostly at the esophagogastric junction, where a squamous cell carcinoma of esophageal origin collides with a gastric adenocarcinoma. The present case is of 2 histologically distinct tumors with aggressive features occurring at the mediastinum. (Ann Thorac Surg 2013;95:330 –2) © 2013 by The Society of Thoracic Surgeons
T
he simultaneous occurrence of 2 distinct neoplasms derived from different cells of origin is a recognized rare entity called collision tumor. Diagnosis of a collision tumor requires that the 2 tumors be histologically distinct, which is often challenging in cases of poorly differentiated neoplasms. The case presented in this report is that of a collision tumor occurring in the mediastinum. A 56-year-old man presented with swelling of his right arm and right leg. Past medical history included diabetes mellitus, hypertension, and a cerebrovascular accident 4 years before presentation. Chest computed tomography indicated a mediastinal mass (Fig 1). Fine-needle aspiration was positive for spindle cell tumor, most probably sarcoma or sarcomatoid carcinoma. The patient underwent surgical resection of the mediastinal mass, and a description of the mass during the surgical procedure indicated gross involvement of the right lung, adhesion to the ascending aorta without invasion, and adhesion to the right phrenic nerve. The tumor extended to the left side of the chest and adhered to the pericardium without invasion but invaded the left upper lobe. The tumor was attached to the left chest wall and left mammary artery. Cranially, the tumor extended to the lower left side of the neck, where it involved the left phrenic nerve. Estimated size was 11 cm and it was composed of numerous nodules. Pathologic evaluation was performed at our institution and then sent for reevaluation by a highly regarded overseas center, which indicated that this was a collision
Collision Tumor of the Mediastinum: A Rare Entity Orit Kaidar-Person, MD, Inna Naroditsky, MD, Ludmila Guralnik, MD, Ran Kremer, MD, and Gil Bar-Sela, MD Division of Oncology, Pathology Institute, Department of Diagnostic Radiology, and Department of Thoracic Surgery, Rambam Health Care Campus & Technion-Israel Institute of Technology, Haifa, Israel
A collision tumor is a rare entity consisting of 2 different tumors that, because of proximity, merge together. The diagnosis of a collision tumor requires that the 2 tumors be histologically distinct. This phenomenon has been Accepted for publication June 4, 2012. Correspondence to Dr Bar-Sela, Division of Oncology, Rambam Health Care Campus, POB 9602, Haifa 31096 Israel; e-mail: g_barsela@ rambam.health.gov.il.
© 2013 by The Society of Thoracic Surgeons Published by Elsevier Inc
Fig 1. Chest computed tomography indicated a large mediastinal mass. A large lobulated inhomogeneous soft tissue mass in the left thymic lobe is seen. The right thymic lobe is enlarged as well by a lobulated, mostly homogeneous soft tissue mass. The masses are separate from each other. 0003-4975/$36.00 http://dx.doi.org/10.1016/j.athoracsur.2012.06.030
tumor. One part of the tumor consisted of lymphoepithelial thymoma (World Health Organization type B2) (Fig 2). Immunostaining for cytokeratin AE1/AE3 and p63 highlighted numerous scattered epithelial cells admixed with the lymphoid infiltrate, supporting this diagnosis. There was already evidence of microinvasion of the adjacent mediastinal fat. Since there was obvious gross involvement of the lung and overt invasion at the time of operation, this tumor corresponded to a Masaoka stage III thymoma. Other parts of the mass had completely different features and corresponded to a malignant spindle cell tumor with areas of necrosis and high mitotic count (Fig 3). The differential diagnosis of that part of the specimen included spindle cell thymic carcinoma, synovial sarcoma, or other high-grade malignant spindle cell sarcoma. Given the presence of a thymoma adjacent to this mass, the first possibility that needed to be eliminated was that of a spindle cell thymic carcinoma arising from the thymoma [1]. Spindle epithelial tumor with thymuslike differentiation is a rare and distinctive low-grade neoplasm of thymic differentiation. The tumor usually presents in the thyroid or lateral neck of children and adolescents and could mimic spindle cell carcinoma [1]. A battery of immunohistochemical stains for cytokeratin AE1/AE3, CK19, and p63 was used, and results were all negative, essentially ruling out a thymic carcinoma. There was no thymic tissue within the tumor. The AE1/ AE3 stain did reveal a few isolated scattered cells showing cytoplasmic staining, but the pattern of distribution of these cells was more suggestive of entrapped residual epithelial cells than being part of the neoplastic proliferation. There were also numerous dilatated glandular spaces scattered throughout the lesion, which probably corresponded to entrapped pulmonary epithelium. Stains for bcl-2 and CD99 showed focal cytoplasmic positivity, which was stronger in the latter. Staining for CD34 was negative in the spindle cells and only labeled entrapped blood vessels. A Ki-67 proliferation marker was positive in about 15% of the tumor cell nuclei. The 2
CASE REPORT KAIDAR-PERSON ET AL SYNCHRONOUS TUMORS OF THE MEDIASTINUM
331
Fig 3. Malignant spindle cell tumor with nuclear atypia and mitotic activity was diagnosed as a malignant solitary fibrous tumor. (Hematoxylin and eosin; ⫻200.)
most likely diagnostic possibilities were probably synovial sarcoma and malignant solitary fibrous tumor. Although synovial sarcoma is positive for cytokeratin (in a spotty pattern, as in this tumor), bcl-2, and CD99, the histologic features in this case are unusual for synovial sarcoma, particularly the areas of linear stromal hyalinization demonstrated here. Additionally, fluorescence in situ hybridization for t(X;18) was negative. Conversely, the histologic features would seem to fit better a solitary fibrous tumor (ie, variegation of patterns, linear stromal hyalinization, short storiform pattern, and so on). Given that solitary fibrous tumor can be negative for CD34 in up to 35% of cases and is also positive for CD99 and bcl-2, it was concluded that this tumor was a malignant solitary fibrous tumor [2]. The presence of areas of necrosis automatically placed this in the high-grade sarcoma category. Spindle cells seen in fine-needle aspiration biopsy of mediastinal lesions can be a component of a wide variety of benign and malignant conditions [3]. Accurate diagnosis is usually achieved after surgical resection [3]. Extensive synchronous thoracic tumors are rare but have been reported, with grave prognoses [4 – 6]. Solitary fibrous tumors are rare and are mostly described as arising from the pleura [7, 8]. The current malignancy showed histologic features such as necrosis and high mitotic counts. These were reported to be associated with aggressive behavior, recurrences, and distant metastases [7]. Other malignant features included a tendency to be larger and demonstration of positive uptake on positron emission tomography [8].
Comment
Fig 2. Lymphoepithelial thymoma (World Health Organization type B2). Numerous scattered epithelial cells admixed with lymphoid infiltrate are seen. (Hematoxylin and eosin; ⫻200.)
A retrospective analysis of data collected from the population-based Surveillance, Epidemiology, and End Results of malignant solitary fibrous tumors of the lung, pleura, or mediastinum indicated that surgical resection has the most implication on survival [7]. Although it is a relatively rare malignancy and was mostly reported as arising from the
FEATURE ARTICLES
Ann Thorac Surg 2013;95:330 –2
332
CASE REPORT MARTENS ET AL MULTIPLE ANEURYSMS IN PATIENT WITH AOS
pleura, it seems that free surgical margins are of the utmost importance [7–9]. Taking that into account, along with the positive surgical margins in the case presented, it was decided that adjuvant radiotherapy be delivered to the mediastinum, to a total dose of 64 Gy (2 Gy per fraction). The malignant solitary fibrous tumor described in this patient did not arise from the pleura. It presented in proximity to another rare mediastinal tumor. We were unable to find histologic or genetic features linking these tumors together, indicating that this is a case of collision mediastinal tumor.
Ann Thorac Surg 2013;95:332–5
The aneurysms-osteoarthritis syndrome (AOS) was recently described and encompasses multiple aneurysms and tortuosity of the great arteries. Most patients have early-onset osteoarthritis. We report the diagnosis, treatment, and follow-up of a patient presenting with bilateral aneurysms of the common iliac arteries and an ascending aortic aneurysm. After proper surgical treatment, genetic studies revealed a causal mutation in the SMAD3 gene. (Ann Thorac Surg 2013;95:332–5) © 2013 by The Society of Thoracic Surgeons
I The authors thank Professor Saul Suster, Head of the Pathology Department at the Medical College of Wisconsin, Milwaukee, Wisconsin, for having reviewed the case in consultation.
References
FEATURE ARTICLES
1. Su L, Beals T, Bernacki EG, Giordano TJ. Spindle epithelial tumor with thymus-like differentiation: a case report with cytologic, histologic, immunohistologic, and ultrastructural findings. Mod Pathol 1997;10:510 – 4. 2. Ali SZ, Hoon V, Hoda S, Heelan R, Zakowski MF. Solitary fibrous tumor. A cytologic-histologic study with clinical, radiologic, and immunohistochemical correlations. Cancer 1997;25:81:116 –21. 3. Slagel DD, Powers CN, Melaragno MJ, Geisinger KR, Frable WJ, Silverman JF. Spindle-cell lesions of the mediastinum: diagnosis by fine-needle aspiration biopsy. Diagn Cytopathol 1997;17:167–76. 4. Nishimura M, Kodama T, Nishiyama H, Nishiwaki Y, Yokose T, Shimosato Y. A case of sarcomatoid carcinoma of the thymus. Pathol Int 1997;47:260 –3. 5. Okumori M, Mabuchi M, Nakagawa M. Malignant thymoma associated with liposarcoma of the mediastinum—a case report. Jpn J Surg 1983;13:512– 8. 6. Cushing B, Bhanot PK, Watts FB, Jr, Hertzler JH, Brough AJ. Rhabdomyosarcoma and benign teratoma. Pediatr Pathol 1983;1:345– 8. 7. Milano MT, Singh DP, Zhang H. Thoracic malignant solitary fibrous tumors: a population-based study of survival. J Thorac Dis 2011;3:99 –104. 8. Magdeleinat P, Alifano M, Petino A, et al. Solitary fibrous tumors of the pleura: clinical characteristics, surgical treatment and outcome. Eur J Cardiothorac Surg 2002;21:1087–93. 9. Kohler M, Clarenbach CF, Kestenholz P, et al. Diagnosis, treatment and long-term outcome of solitary fibrous tumours of the pleura. Eur J Cardiothorac Surg 2007;32:403– 8.
Multiple Aneurysms in a Patient With Aneurysms-Osteoarthritis Syndrome Thomas Martens, MD, Isabelle Van Herzeele, MD, PhD, Frederic De Ryck, MD, PhD, Marjolijn Renard, MD, PhD, Anne De Paepe, MD, PhD, Katrien François, MD, PhD, Frank Vermassen, MD, PhD, and Julie De Backer, MD, PhD Department of Thoracic and Vascular Surgery, Centre for Medical Genetics, Department of Cardiac Surgery, and Department of Cardiology, University Hospital Gent, Gent, Belgium © 2013 by The Society of Thoracic Surgeons Published by Elsevier Inc
n the spectrum of inheritable connective tissue disorders with arterial involvement, a new genetic syndrome caused by mutations in the SMAD3 gene was recently delineated and named aneurysms-osteoarthritis syndrome (AOS) [1, 2]. Since AOS is a recently discovered clinical entity, no data regarding its prevalence are available yet, and the clinical suspicion for the disease is still low. With this case presentation, we want to raise the awareness for AOS among clinicians and emphasize the need for extended clinical investigation, especially in young patients.
A 32-year-old male patient was referred to the vascular surgeon because of unspecified pulsatile sensations in the lower abdomen for 4 weeks. He had a medical history of lower back pain and lumbar hernia. Clinical examination revealed a thrill over the right fossa with tenderness on palpation. Bilateral femoral pulses were equal in strength, with a full complement of pulses in both lower limbs. A computed tomographic scan of the abdomen showed a saccular aneurysm (maximal diameter of 69 mm) of the right common iliac artery and a partially thrombosed left common iliac artery aneurysm (Fig 1). An aortobifurcated graft was implanted in an end-to-end configuration to both internal iliac arteries; both external iliac arteries were reimplanted in an end-to-side fashion (Gelsoft prosthesis 16 mm ⫽ proximal diameter, 8 mm ⫽ distal diameter, Vascutek, Renfrewshire, UK). No postoperative complications occurred. Diagnostic workup including transthoracic echocardiography revealed a dilatated aortic root of 49 mm at the level of the sinuses of Valsalva. Magnetic resonance angiography from head to pelvis confirmed the aortic root dilatation (Fig 2) and showed moderate tortuosity of the thoracic and abdominal aorta and iliac arteries. The vertebral and internal carotid arteries also showed greater tortuosity than expected for the age of the patient (Fig 3). Additional clinical features included a narrow face with discrete proptosis of the eyes. The uvula showed a raphe and was slightly deviated. There was no joint hyperlaxity and the skin was normal. Roentgenograms of Accepted for publication May 11, 2012. Address correspondence to Dr Martens, Department of Thoracic and Vascular Surgery, University Hospital Gent, De Pintelaan 185, 9000 Gent, Belgium; e-mail: [email protected].
0003-4975/$36.00 http://dx.doi.org/10.1016/j.athoracsur.2012.05.085