Asian Cardiovasc Thorac Ann 1998;6:323-324
© 1998 Asia Publishing EXchange Pte Ltd
Primary Pulmonary Paraganglioma
Erdem Silistreli, MD,
Unal Acikel, MD,
Oztekin Oto, MD,
Hudai Catalyurek , MD ,
Nejat Sariosmanoglu , MD,
Cuneyt Narin , MD ,
Ozgul Sagol , MD1,
Aydanur Kargi , MD1,
Eyup Sabri Ucan , MD2
Department of Thoracic & Cardiovascular Surgery
1 Department of Pathology
2 Department of Pulmonary Medicine Dokuz Eylul Medical Faculty  zmir, Turkey
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For reprint information contact: Erdem Silistreli, MD Department of Thoracic & Cardiovascular Surgery Dokuz Eylul Medical Faculty Inciralti, zmir 35340, Turkey Tel:90 232 277 7777 Fax:90 232 277 2165 Email:silistre{at}cs.med.deu.edu.tr
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ABSTRACT
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A 2-cm nodular mass was detected by thoracic computed tomography in the peripheral region of the right middle lobe of the lung in a 60-year-old female with a 5-year history of cough and respiratory distress. The resected tumor was diagnosed as a primary paraganglioma on pathologic examination.
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INTRODUCTION
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Reports of extra-adrenal paraganglioma as a primary lesion of the lung are extremely uncommon. This case of pulmonary paraganglioma is described because of its rarity and the need for differentiation of this tumor.
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CASE REPORT
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A 60-year-old female with no history of smoking was investigated for cough and respiratory distress of 5 years duration. There was no pathologic finding at the physical examination. A nodular micro-calcified lesion was seen at the right paracardiac region in a posteroanterior chest radiograph. By thoracic computed tomography, a 2-cm nodular mass was detected in the peripheral region of the right middle lobe. Flexible bronchoscopic examination was normal and no metastatic lesion was found on total body bone scintigraphy, cranial and abdominal computed tomography, or abdominal ultrasonography. A right middle lobectomy was performed through a standard right posterolateral thoracotomy. The patient had an uneventful postoperative period and was discharged after 7 days. At the last follow-up 8 months after the operation, there was no local recurrence or metastatic finding.
In the pathological macroscopic examination, a well-circumscribed greyish-white tumor, 2.5 x 1.5 x 1 cm in size, was observed in the right middle lobe. On microscopic examination, the lesion was well-delineated and capsulated and showed a highly vascular uniform proliferation of round cells with clear or eosinophilic cytoplasm arranged in nests and separated by thin fibrous septa (Figure 1
). The tumor cells showed immunoreactivity with neuron-specific enolase and chromatography. A small number of cells showed immunoreactivity with S100 protein. These findings indicated a primary pulmonary paraganglioma with an absence of peripheral and lymph node invasion.
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Figure 1. Tumor cell nests with uniform nuclei and clear or eosinophilic cytoplasm (hematoxylin and eosin stain, original magnification x 200).
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DISCUSSION
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Extra-adrenal paragangliomas constitute about 10% of such tumors and are usually located in the abdomen. Less than 2% of them are thoracic and they are almost always located in the posterior mediastinum along the sympathetic chain.1 Two forms of primary pulmonary paragangliomas have been described. Multiple miliary tumors, often grossly visible, occur frequently in association with chronic lung disorders and may be found incidentally in approximately 3% of autopsies. These tumors may represent preexisting nests of paraganglionic cells that have been stimulated by ischemia, and an association with pulmonary thromboembolism has been shown.2 The second and rarer form, primary pulmonary paraganglioma, consists of a large solid tumor. The patients are usually female and of middle age, most of whom are free of symptoms and normotensive.3 The tumors are often discovered incidentally on routine chest radiography. Rarely, malignancy or endocrinologic activity of these tumors has been reported.4 We found 22 cases described in the literature, including 1 in which the tumor was locally invasive, 2 malignant cases with hilar metastasis, and 2 other cases of secondary metastatic masses in the lung.2,4–6 The only parameters found to correlate with aggressive behavior was the extent of circumscription and local infiltration of the tumor at the initial resection.6
Standard therapy for primary pulmonary paraganglioma ranges from enucleation, wedge resection, and segmentectomy for localized lesions to lobectomy for larger tumors. We consider that the collective experience does not justify extensive radical resection.
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Figure 2. Low-power magnification of the tumor (hematoxylin and eosin stain, original magnification x 20).
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REFERENCES
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Kitahara M, Mori I, Seki H, Washizawa K, Amano Y, Nakahata I, et al. Malignant paraganglioma presenting as Cushing syndrome with virilism in childhood. Production of cortisol, androgens, and adrenocorticotrophic hormone by the tumor. Cancer
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ABSTRACT
INTRODUCTION
