Ann Thorac Surg 2006;82:e5-e7
© 2006 The Society of Thoracic Surgeons
Case report
Giant Pulmonary Hamartoma—A Rare Presentation of a Common Tumor
Joerg Hutter, MDa,*,
Silvia Reich-Weinberger, MDa,
Georg Hutarew, MDb,
Hubert J. Stein, MDa
a Department of Surgery, Paracelsus Private Medical University, Landeskliniken Salzburg, Austria
b Department of Pathology, Paracelsus Private Medical University, Landeskliniken Salzburg, Austria
Accepted for publication May 10, 2006.
* Address correspondence to Dr Hutter, Department of Surgery, Paracelsus Medical University, Landeskliniken Salzburg, Muellnerhauptstr 48, 5020 Salzburg, Austria (Email: j.hutter{at}salk.at).
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Abstract
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Hamartomata, the most common benign tumors of the lung, are rarely symptomatic and usually come to clinical attention as coin-shaped lesions on a routine plain chest roentgenogram. We report a case of a 63-year-old woman presenting with reduced endurance on exercise. The plain chest roentgenogram showed a tumor in the left chest. After clinical work-up, the patient underwent radical tumor resection through a lateral thoracotomy. The resected tumor, with two separate nodules, measured 26 x 25 cm and weighed 3.2 kg. The histopathology work-up showed pulmonary hamartoma.
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Introduction
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Hamartomata account for most of the benign tumors of the lung [1, 2]. They are found in about 0.25% of all patients at autopsy. These lesions represent an abnormal proliferation and mixing of the normal components of the lung tissue. Most hamartomata are asymptomatic. They become symptomatic only when located within a bronchus, which occurs in up to 14% of patients. The diagnosis is usually made during a routine plain chest roentgenogram. They are most often seen in patients between 20 and 60 years of age, with a male predominance of 2:1 to 3:1. A slow growth is normal for these tumors, with an observed increase of 3.2 ± 2.6 mm per year [3, 4].
Giant tumors of the thorax comprise pleural fibrous tumors [5] or malignant pleural and pulmonary tumors such as mesothelioma or secondary malignant disease. Giant pulmonary hamartomata are very rare.
A 64-year-old woman was admitted complaining of reduced endurance during the last 2 years, particularly when cross-country skiing. Her general practitioner had sent her for a plain chest roentgenogram, which demonstrated a tumor mass occupying two thirds of the left hemi thorax. Suspected invasion of the mediastinum and the contralateral pleural space was shown on the computed tomography (CT) (Fig 1).
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Fig 1. Preoperative computed tomography scan shows a large, left-sided tumor with suspected infiltration of the mediastinum and growth to the contralateral side.
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The clinical work-up was completed with upper gastrointestinal endoscopy, endoesophageal sonography, bronchoscopy and CT-guided transthoracic fine needle biopsy, and gynecologic and abdominal staging. Although the patient did not complain of dysphagia, the esophagus was markedly displaced. On endoesophageal sonography, no invasion of the esophageal wall could be identified. Bronchoscopy demonstrated a cranial deviation of the left-sided main and lobar bronchi. Lung function tests showed a reduced timed forced expiratory volume of 47% and forced vital capacity of 46%, with a normal blood gas analysis.
The histopathologic analysis of the biopsy specimen showed smooth muscle cells, little fat, fibrous tissue, and vascular structures, but no cartilage and no malignancy. The histopathologic diagnosis of the fine needle aspiration was benign, mesenchymal tumor.
The operation was performed through a left lateral thoracotomy with partial resection of the fifth rib. The tumor was found to originate from the basal segments of left lower lobe and was covered with visceral pleura. A complete resection of the tumor and the adjacent peripheral lung parenchyma was performed with linear staplers. There were only soft adhesions to the thoracic wall. There was no vascular pedicle to the mediastinum and no infiltration of mediastinal structures by the tumor.
The resected tumor with two separate nodules measured 26 x 25 cm and weighed 3.2 kg (Fig 2). Macroscopically, the cut surface revealed an encapsulated polycystic tumor with partially fibrotic, cartilaginous, and calcified connective tissue. The histology analysis (Fig 3) showed a partially cystic tumor covered by mesothelial cells, mesenchymal tissues with smooth muscle cells, fat, well-differentiated cartilage, bone, and foci of calcification within the sclerotic stroma. Entrapped respiratory-type, epithelium-formed clefts between the lobes of mesenchymal components in the tumor showed no signs of malignancy. Immunohistochemical staining was negative for CD-34, S-100, and CD-117 antigens and positive with smooth muscle cell antigen SMA. Ki-67 was positive in less than 2% of cells. The final pathology diagnosis was pulmonary hamartoma.
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Fig 3. Histology examination (hematoxylin and eosin staining) shows (A) composition of smooth muscle cells with clefts of respiratory-like epithelium (x 40 original magnification), (B) mature cartilage with bone (x 200 original magnification), and (C) smooth muscle cell, fat, and fibrovascular structures (x 15 original magnification).
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The patient had an uneventful postoperative course with rapid reexpansion of the lung and was discharged from the hospital on postoperative day 8. Two years after the operation, the patient is free of recurrence but has gastroesophageal reflux disease that is treated medically.
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Comment
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Hamartomas are the most frequent benign tumors in resection series for pulmonary nodules [3, 6]. They can be large, either solid or cystic, and even a case with high production of carbohydrate antigen 19-9 has been published [7, 8]. The histology of our patient's tumor showed the typical components of fibrous connective tissue, cartilage, fat, bone, smooth muscle cells, and clefts of respiratory-type epithelium. The diagnosis of a hamartoma is usually made by resection. Suspected primary or secondary malignant tumor or endobronchial location with poststenotic complications such as pneumonia are indications for the resection. Malignant sarcomatous transformation is rare, and only a few such cases have been reported [9].
Publications of giant symptomatic tumors describe large solitary fibrous pleural tumors [5] and malignant entities. Reports on giant hamartomas are very rare. In 1979, Petheram and Heard [10] reported a case of a recurrent large size cartilaginous hamartoma in a 32-year-old man not radically resected for bullous lung disease with an incidental hamartoma 16 years earlier. A similar growth of the tumor to the contralateral side was described in this patient, who made an uneventful postoperative course after a right-sided pneumonectomy.
Complete tumor resection is emphasized because of the risk of recurrence. Lack of tumor infiltration into mediastinal structures and lack of dense adhesions made our resection easy. The resection of the large tumor was technically easy in the presented case. This matches observations by others [11]. Although slow growth is the norm and large-size tumors, as in the presented patient, are very rare, lifelong follow-up has to be advocated in nonresection cases.
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References
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Abstract
Introduction
