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Leiomyomatous Hamartoma of the Posterior Mediastinum
304
CASE REPORT BIBAS ET AL MEDIASTINAL HAMARTOMA
Leiomyomatous Hamartoma of the Posterior Mediastinum Benoit Jacques Bibas, MD, Ricardo Mingarini Terra, MD, Angelo Fernandez, MD, Christina Shiang, MD, Paulo Manuel Pêgo-Fernandes, MD, and Fabio Biscegli Jatene, MD Division of Thoracic Surgery and Pathology, Hospital das Clínicas, University of São Paulo Medical School, São Paulo, Brazil
A posterior mediastinal mass was found in a 72-year-old man who complained of progressive shortness of breath. Computed tomography of the thorax showed a welldefined, heterogenous mass measuring 9.5 ⴛ 4.0 ⴛ 3.0 cm, located in the right costovertebral angle. Complete surgical resection was accomplished through a right posterolateral thoracotomy. The final diagnosis was a leiomyomatous hamartoma of the posterior mediastinum. This is an unusual location for this type of tumor, and it has rarely been reported in the literature. (Ann Thorac Surg 2010;89:304 –5) © 2010 by The Society of Thoracic Surgeons
H
FEATURE ARTICLES
amartomas are common pulmonary lesions, accounting for 8% of all pulmonary neoplasms [1]. The lesions are usually well circumscribed on radiographic images and are normally between 1 and 2 cm in diameter, although larger tumors are occasionally seen. Calcification is common and better visualized in computed tomography (CT) scans [1]. Most hamartomas are located in the periphery of the pulmonary parenchyma, but 1% to 20% may occur as endobronchial lesions and cause symptoms related to bronchial obstruction [1, 2]. We present a patient with a leiomyomatous hamartoma located in the posterior mediastinum. This location is atypical and rarely reported for this type of tumor.
Ann Thorac Surg 2010;89:304 –5
pleura. Based on that hypothesis, it was decided not to biopsy the lesion. The patient was operated on through a right posterolateral thoracotomy. A large, immobile, encapsulated, and lobulated mass was evident in the costovertebral region. It was fully covered with mediastinal pleura and had a rich vascular supply that had its origin in the mediastinum. The lesion compressed the right lower lobe, but with no evidence of local invasion. Complete en bloc resection of the tumor was achieved after careful dissection. The patient had an uneventful recovery and was discharged from the hospital on postoperative day 4. Gross examination revealed a circumscribed, tancolored mass measuring 9.5 ⫻ 4.0 ⫻ 3.0 cm and weighing 110 g. Histologically, it showed a proliferation of spindleshaped smooth muscle growing in between slit-like spaces lined by cytologically bland, flattened, cuboidal cells. These epithelial lining cells are type 2 pneumocytes that stain for thyroid transcription factor-1. Clefts of respiratory-type epithelium associated with mucinous gland hyperplasia were also seen focally (Fig 2). CD-34 stain was negative. The final diagnosis was a leiomyomatous hamartoma. It was classified as a hamartomatous lesion because of its varying proportions of mesenchymal tissue and respiratory-type epithelium. The mesenchymal component, represented by spindle cells, is actually smooth muscle tissue because of desmin and smooth muscle actin positivity. The patient is alive and without signs of recurrence 4 years after the operation.
Comment Mediastinal tumors are often asymptomatic and usually detected by chest roentgenogram. When symptoms are
A previously healthy 72-year-old man presented to his regular physician for a routine consultation in which he complained of progressive dyspnea in the last year. He had no history of pulmonary diseases and was a nonsmoker. Lung sounds were clear, except for decreased breath sounds and decreased fremitus at the right base, without egophony. Laboratory test results revealed no abnormalities. A chest roentgenogram showed a mass in the right hemithorax. CT depicted a large, heterogeneous, partially calcified, and well-defined mass located in the right costovertebral region (Fig 1). There was no sign of extension into the spinal canal or adjacent structures. Radiographic findings were compatible with a neurogenic tumor of the mediastinum or a fibrous tumor of the Accepted for publication June 9, 2009. Address correspondence to Dr Terra, Al Fernão Cardim 161, Ap. 61, São Paulo, 01403-020, Brazil; e-mail: [email protected].
© 2010 by The Society of Thoracic Surgeons Published by Elsevier Inc
Fig 1. Computed tomography of the thorax shows a well-defined mass measuring 9.5 ⫻ 4.0 ⫻ 3.0 cm located in the right costovertebral region. The vascular supply (arrow) to the mass has its origin in the mediastinum. 0003-4975/10/$36.00 doi:10.1016/j.athoracsur.2009.06.046
Ann Thorac Surg 2010;89:304 –5
CASE REPORT BIBAS ET AL MEDIASTINAL HAMARTOMA
305
present, the most common are chest pain, cough, dyspnea, and superior vena cava syndrome [3]. Approximately 25% of the mediastinal neoplasms occur in the posterior compartment, and 75% of these tumors are of neurogenic origin [4]. Other less common masses of the posterior mediastinum include tumors of mesenchymal origin and gastroenteric cysts [3, 4]. Hamartomas may be defined as a disorganized focus or an overgrowth of mature tissues found normally at the location of the lesion. With low capacity of growth and benign behavior, the implicated tissues proliferate in a disorganized manner, and its descriptive name depends on the predominant tissue [5]. Therefore, hamartomas may be designated as rhabdomyomatous, neurovascular, lymphatic, fatty, and so forth [5]. The histology of the pulmonary lesions reveals a predominant chondroid differentiation in 80% of the cases, with fibroblastic (12%), fatty (5%), and osseous (3%) differentiation comprising the rest [6]. Leiomyomatous hamartomas are particularly rare, and the presence of smooth muscle predominates over other possible components [5]. The location of the hamartoma in this patient is unusual. Although extremely rare, a hamartoma of chondromatous differentiation in the posterior mediastinum was reported by Gholoum and colleagues [7]. In the resected specimen, a small amount of compressed lung parenchyma bordered by pleural tissue was evident around the periphery of the tumor and confirmed by immunohistochemical analysis. The authors postulated that this layer of pulmonary parenchyma surrounding the lesion could indicate that an intraparenchymal hamartoma had migrated across the visceral pleura into the mediastinum. Such migration within the lung is uncommon but is well described with foreign bodies and calcified necrotic material within peribronchial lymph nodes (resulting in
broncholithiasis), and is probably related to the cyclic motion of respiration [7]. Indeed, penetration of pulmonary hamartoma through the visceral pleura with adhesion to the mediastinal pleura has been reported [8]. Whether mediastinal hamartomas may be described as primary mediastinal tumors or lesions that have migrated through the pulmonary parenchyma is a question to be answered and open to speculation. The case reported here favors the hypothesis of a primary mediastinal mass because the tumor was fully covered with mediastinal pleura and its vascular supply clearly originated from the mediastinum. To our knowledge, this is only the second report of a posterior mediastinal hamartoma.
References 1. Shrager JB, Kaiser LR. Benign lung tumors. In: Patterson GA, Cooper JD, Deslauriers J, Lerut A, Luketich JD, Rice TW (eds). Pearson’s thoracic & esophageal surgery. 3rd ed. Philadelphia: Churchill Livingstone 2008:691– 8. 2. Whyte RI, Donington JS. Hamartomas of the lung. Semin Thorac Cardiovasc Surg 2003;15:301– 4. 3. Macchiarini P, Ostertag H. Uncommon primary mediastinal tumours. Lancet Oncol 2004;5:107–18. 4. Strollo DC, Rosado-de-Christenson ML, Jett JR. Primary mediastinal tumors: part II. Tumors of the middle and posterior mediastinum. Chest 1997;112:1344 –57. 5. Nava-Villalba M, Ocampo-Acosta F, Seamanduras-Pacheco A, Aldape-Barrios BC. Leiomyomatous hamartoma: report of two cases and review of the literature. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2008;105:e39 – 45. 6. Ganti S, Milton R, Davidson L, Anikin V. Giant pulmonary hamartoma. J Cardiothorac Surg 2006;1:19. 7. Gholoum S, Fraser R, Ferri LE. Posterior mediastinal chondromatous hamartoma. Ann Thorac Surg 2007;83:1528 –30. 8. Tomiyasu M, Yoshino I, Suemitsu R, Shoji F, Sugimachi K. An intrapulmonary chondromatous hamartoma penetrating the visceral pleura: report of a case. Ann Thorac Cardiovasc Surg 2002;8:42– 4.
FEATURE ARTICLES
Fig 2. (A) Histology specimens show smooth muscle proliferation around slit-like spaces lined by pneumocytes. Intraalveolar respiratory macrophages are also seen (hematoxylineosin stain, original magnification ⫻200). (B) Clefts are lined by respiratory-type epithelium (hematoxylin-eosin stain, original magnification ⫻200). (C) Immunohistochemistry shows thyroid transcription factor-1 positivity in the alveolar-like space lining (original magnification ⫻200). (D) Strong positivity for desmin in the spindle cell proliferation (original magnification ⫻100).
CASE REPORT BIBAS ET AL MEDIASTINAL HAMARTOMA
Leiomyomatous Hamartoma of the Posterior Mediastinum Benoit Jacques Bibas, MD, Ricardo Mingarini Terra, MD, Angelo Fernandez, MD, Christina Shiang, MD, Paulo Manuel Pêgo-Fernandes, MD, and Fabio Biscegli Jatene, MD Division of Thoracic Surgery and Pathology, Hospital das Clínicas, University of São Paulo Medical School, São Paulo, Brazil
A posterior mediastinal mass was found in a 72-year-old man who complained of progressive shortness of breath. Computed tomography of the thorax showed a welldefined, heterogenous mass measuring 9.5 ⴛ 4.0 ⴛ 3.0 cm, located in the right costovertebral angle. Complete surgical resection was accomplished through a right posterolateral thoracotomy. The final diagnosis was a leiomyomatous hamartoma of the posterior mediastinum. This is an unusual location for this type of tumor, and it has rarely been reported in the literature. (Ann Thorac Surg 2010;89:304 –5) © 2010 by The Society of Thoracic Surgeons
H
FEATURE ARTICLES
amartomas are common pulmonary lesions, accounting for 8% of all pulmonary neoplasms [1]. The lesions are usually well circumscribed on radiographic images and are normally between 1 and 2 cm in diameter, although larger tumors are occasionally seen. Calcification is common and better visualized in computed tomography (CT) scans [1]. Most hamartomas are located in the periphery of the pulmonary parenchyma, but 1% to 20% may occur as endobronchial lesions and cause symptoms related to bronchial obstruction [1, 2]. We present a patient with a leiomyomatous hamartoma located in the posterior mediastinum. This location is atypical and rarely reported for this type of tumor.
Ann Thorac Surg 2010;89:304 –5
pleura. Based on that hypothesis, it was decided not to biopsy the lesion. The patient was operated on through a right posterolateral thoracotomy. A large, immobile, encapsulated, and lobulated mass was evident in the costovertebral region. It was fully covered with mediastinal pleura and had a rich vascular supply that had its origin in the mediastinum. The lesion compressed the right lower lobe, but with no evidence of local invasion. Complete en bloc resection of the tumor was achieved after careful dissection. The patient had an uneventful recovery and was discharged from the hospital on postoperative day 4. Gross examination revealed a circumscribed, tancolored mass measuring 9.5 ⫻ 4.0 ⫻ 3.0 cm and weighing 110 g. Histologically, it showed a proliferation of spindleshaped smooth muscle growing in between slit-like spaces lined by cytologically bland, flattened, cuboidal cells. These epithelial lining cells are type 2 pneumocytes that stain for thyroid transcription factor-1. Clefts of respiratory-type epithelium associated with mucinous gland hyperplasia were also seen focally (Fig 2). CD-34 stain was negative. The final diagnosis was a leiomyomatous hamartoma. It was classified as a hamartomatous lesion because of its varying proportions of mesenchymal tissue and respiratory-type epithelium. The mesenchymal component, represented by spindle cells, is actually smooth muscle tissue because of desmin and smooth muscle actin positivity. The patient is alive and without signs of recurrence 4 years after the operation.
Comment Mediastinal tumors are often asymptomatic and usually detected by chest roentgenogram. When symptoms are
A previously healthy 72-year-old man presented to his regular physician for a routine consultation in which he complained of progressive dyspnea in the last year. He had no history of pulmonary diseases and was a nonsmoker. Lung sounds were clear, except for decreased breath sounds and decreased fremitus at the right base, without egophony. Laboratory test results revealed no abnormalities. A chest roentgenogram showed a mass in the right hemithorax. CT depicted a large, heterogeneous, partially calcified, and well-defined mass located in the right costovertebral region (Fig 1). There was no sign of extension into the spinal canal or adjacent structures. Radiographic findings were compatible with a neurogenic tumor of the mediastinum or a fibrous tumor of the Accepted for publication June 9, 2009. Address correspondence to Dr Terra, Al Fernão Cardim 161, Ap. 61, São Paulo, 01403-020, Brazil; e-mail: [email protected].
© 2010 by The Society of Thoracic Surgeons Published by Elsevier Inc
Fig 1. Computed tomography of the thorax shows a well-defined mass measuring 9.5 ⫻ 4.0 ⫻ 3.0 cm located in the right costovertebral region. The vascular supply (arrow) to the mass has its origin in the mediastinum. 0003-4975/10/$36.00 doi:10.1016/j.athoracsur.2009.06.046
Ann Thorac Surg 2010;89:304 –5
CASE REPORT BIBAS ET AL MEDIASTINAL HAMARTOMA
305
present, the most common are chest pain, cough, dyspnea, and superior vena cava syndrome [3]. Approximately 25% of the mediastinal neoplasms occur in the posterior compartment, and 75% of these tumors are of neurogenic origin [4]. Other less common masses of the posterior mediastinum include tumors of mesenchymal origin and gastroenteric cysts [3, 4]. Hamartomas may be defined as a disorganized focus or an overgrowth of mature tissues found normally at the location of the lesion. With low capacity of growth and benign behavior, the implicated tissues proliferate in a disorganized manner, and its descriptive name depends on the predominant tissue [5]. Therefore, hamartomas may be designated as rhabdomyomatous, neurovascular, lymphatic, fatty, and so forth [5]. The histology of the pulmonary lesions reveals a predominant chondroid differentiation in 80% of the cases, with fibroblastic (12%), fatty (5%), and osseous (3%) differentiation comprising the rest [6]. Leiomyomatous hamartomas are particularly rare, and the presence of smooth muscle predominates over other possible components [5]. The location of the hamartoma in this patient is unusual. Although extremely rare, a hamartoma of chondromatous differentiation in the posterior mediastinum was reported by Gholoum and colleagues [7]. In the resected specimen, a small amount of compressed lung parenchyma bordered by pleural tissue was evident around the periphery of the tumor and confirmed by immunohistochemical analysis. The authors postulated that this layer of pulmonary parenchyma surrounding the lesion could indicate that an intraparenchymal hamartoma had migrated across the visceral pleura into the mediastinum. Such migration within the lung is uncommon but is well described with foreign bodies and calcified necrotic material within peribronchial lymph nodes (resulting in
broncholithiasis), and is probably related to the cyclic motion of respiration [7]. Indeed, penetration of pulmonary hamartoma through the visceral pleura with adhesion to the mediastinal pleura has been reported [8]. Whether mediastinal hamartomas may be described as primary mediastinal tumors or lesions that have migrated through the pulmonary parenchyma is a question to be answered and open to speculation. The case reported here favors the hypothesis of a primary mediastinal mass because the tumor was fully covered with mediastinal pleura and its vascular supply clearly originated from the mediastinum. To our knowledge, this is only the second report of a posterior mediastinal hamartoma.
References 1. Shrager JB, Kaiser LR. Benign lung tumors. In: Patterson GA, Cooper JD, Deslauriers J, Lerut A, Luketich JD, Rice TW (eds). Pearson’s thoracic & esophageal surgery. 3rd ed. Philadelphia: Churchill Livingstone 2008:691– 8. 2. Whyte RI, Donington JS. Hamartomas of the lung. Semin Thorac Cardiovasc Surg 2003;15:301– 4. 3. Macchiarini P, Ostertag H. Uncommon primary mediastinal tumours. Lancet Oncol 2004;5:107–18. 4. Strollo DC, Rosado-de-Christenson ML, Jett JR. Primary mediastinal tumors: part II. Tumors of the middle and posterior mediastinum. Chest 1997;112:1344 –57. 5. Nava-Villalba M, Ocampo-Acosta F, Seamanduras-Pacheco A, Aldape-Barrios BC. Leiomyomatous hamartoma: report of two cases and review of the literature. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2008;105:e39 – 45. 6. Ganti S, Milton R, Davidson L, Anikin V. Giant pulmonary hamartoma. J Cardiothorac Surg 2006;1:19. 7. Gholoum S, Fraser R, Ferri LE. Posterior mediastinal chondromatous hamartoma. Ann Thorac Surg 2007;83:1528 –30. 8. Tomiyasu M, Yoshino I, Suemitsu R, Shoji F, Sugimachi K. An intrapulmonary chondromatous hamartoma penetrating the visceral pleura: report of a case. Ann Thorac Cardiovasc Surg 2002;8:42– 4.
FEATURE ARTICLES
Fig 2. (A) Histology specimens show smooth muscle proliferation around slit-like spaces lined by pneumocytes. Intraalveolar respiratory macrophages are also seen (hematoxylineosin stain, original magnification ⫻200). (B) Clefts are lined by respiratory-type epithelium (hematoxylin-eosin stain, original magnification ⫻200). (C) Immunohistochemistry shows thyroid transcription factor-1 positivity in the alveolar-like space lining (original magnification ⫻200). (D) Strong positivity for desmin in the spindle cell proliferation (original magnification ⫻100).