Eur J Cardiothorac Surg 2002;21:553-555
© 2002 Elsevier Science NL
Unsuspected primary pulmonary meningioma
Alfredo Cesario*,
Domenico Galetta,
Stefano Margaritora,
Pierluigi Granone
Division of General Thoracic Surgery, Catholic University of Rome, Largo A. Gemelli 8, 00168 Rome, Italy
Received 30 October 2001;
received in revised form 19 December 2001;
accepted 19 December 2001.
* Corresponding author. Tel.: +39-335-836-6161; fax: +39-6-305-1162
e-mail: alfcesario{at}yahoo.com
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Abstract
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Primary pulmonary meningioma is an uncommon, usually benign, soft tissue tumour which has rarely been reported. We report an additional case of primary pulmonary meningioma occurring in an asymptomatic 56-year-old man whose diagnosis was only established after resection. The features of this lesion together with a review of the previous literature are described.
Key Words: Primary pulmonary meningioma • Surgery
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1. Introduction
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Primary ectopic meningiomas are rare tumours. They occur in the region of the head, neck, skin, and peripheral nerves. Their incidence has been estimated to range from 0.9 to 20%. Primary intra-pulmonary localization has been sporadically reported in the literature. Most of these ‘primary pulmonary meningiomas’ (PPM) are benign neoplasms. Only two cases with malignant features have been described [1,2]. We describe herein an additional case of PPM in a man and briefly review the literature.
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2. Case report
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A 56-year-old man with a history of smoking 20 cigarettes per day for 20 years was referred to our department for investigation of an abnormal shadow located in the left middle lung field by a routine chest X-ray. The patient was asymptomatic from a pulmonary standpoint; he had a past history of mild hypertension, but was otherwise in good general health.
The chest radiograph showed a 2-cm solitary well-circumscribed nodular mass in the middle pulmonary field which was confirmed to be in the lingula by a computer tomography (CT) scan of the thorax. No hilar or mediastinal enlarged lymph node were identified. The lesion, smaller in its maximum diameter, was not present in a previous chest X-ray carried out 3 years before the date of diagnosis. Flexible bronchoscopy was negative for intra-bronchial anomalies; cytology on bronchial washings and material obtained by fine-needle aspiration biopsy (FNAB) did not show any tumour specificity and was negative for malignant cells (blood, macrophages and inflammatory material). Systemic survey, accomplished by CT scan of the brain, abdomen and pelvis, liver ultrasound scan, colonic barium enema, oesophagogastroduodenoscopy and whole-body radionuclide scan failed to demonstrate a primary tumour. Moreover, blood serum levels of TPA, CEA, CA 19-9, CYPHRA 21-1 and NSE were within the normal range. Thus, the lesion was considered to be primitive pulmonary and surgical removal was recommended.
A wedge resection was performed through a vertical axillary thoracotomy. The rationale for the open approach was that in absence of a clear pre-operative diagnosis we decided to have the chance to palpate manually the whole lung parenchyma. The tumour was a subpleural, 2 cm in maximum diameter nodule, well demarcated from the adjacent pulmonary parenchyma, with a pale yellow-tan cut surface. No other lesions were detected intra-operatively. Frozen section of the lesion suggested a malignant neoplasm, due to possible high number of mitoses in nests of small cells, so we performed a left upper lobectomy. Hilar and mediastinal lymph node dissections were performed also. Pathology did not confirm the suspected malignancy and demonstrated a benign tumour composed of bland spindle and cuboid cells with a whorled pattern containing solid nests of meningothelial cells surrounded by a thin collagen tissue layer (Fig. 1)
. Neither a high number of mitoses, nor pleomorphism was evident. Immunohistochemically, stains revealed a strong positivity for vimentin and epithelial membrane antigen (EMA); stains for cytokeratins AE1/AE3, desmin, and S-100 protein were negative. The histological and immunohistochemical features strongly suggested the diagnosis of benign meningioma. The pathological examination of the resected lymph nodes did not show any malignancy.
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Fig. 1. Photomicrograph showing proliferation of spindle-ovoid shaped cells, with helicoidal pattern and fascicle formation. The central area demonstrates solid nests of meningothelial cells with uniform nuclei surrounded by collagen tissue (Hematoxylin–eosin, x200).
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The patient's post-operative course was uneventful, and he was discharged on the seventh post-operative day. He is well and without any sign of local and distant recurrence at 7-year follow-up evaluation.
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3. Discussion
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Primary pulmonary meningioma (PPM) is a rare lung neoplasm. First described in 1981 by Erlandson [1], only 27 cases have been described in the English literature [2–10] to date. With this limited number of patients only a few generalizations can be drawn (Table 1). The neoplasm appears to have a preponderance in older women with a female/male ratio of 16:11; onset age range is from 24 to 74 years (average 55.4 years). Almost all of the described cases had had their diagnosis made on the basis of a pulmonary solitary and asymptomatic coin lesion, incidentally found in chest X-rays. In two patients a symptomatic cough has been reported [6,7]. Tumours ranged in size from 4 mm to 12 cm and were located prevalently in the left lung. PPM usually appeared singular and benign: only two cases with clearly malignant features have been reported, the first in a 41-year-old woman [1] and the second in a 51-year-old man, the latter with local and distant recurrence within 5 months [2]. To date only one case of multiple PPM has been reported [3]. Recently, Spinelli described a case where multiple meningothelium-like nodules were present, reporting for the first time a preoperative diagnosis of PPM made on the basis of cytological examination of material obtained by FNAB [8].
Histologically, benign PPM appeared well circumscribed and often localized in the lung parenchyma, without any bronchial or pleural involvement. Pleural malignant effusion associated with pleural metastatic implants were seen only in the first of the two reported malignant PPM cases. Microscopically, these lesions usually presented with spindle-, polygonal- or ovoid-shaped cells, usually arranged in lobules and whorls, more often without mitoses; a high number of mitoses were prominent in malignant cases [1,2]. Psammoma bodies have often been reported.
Immunohistochemical findings often showed a strong positivity for vimentin and EMA, and more often a variable degree of expression for cytokeratin, desmin and S-100 protein [3–6,9].
The origin of these tumours in the thorax is debated and different theories have been previously advocated considering the neoplasms arising from intra-thoracic differentiation of meningocytes or arachnoid cells, or from ectopic proliferation of arachnoid cells [8] or from direct/indirect extension of primary intra-cranial meningioma [10].
Prognostically, PPM behaves as a benign tumour in the majority of the observed patients. Treatment consisted of complete resection in all patients except one [4], who remained untreated and died 6 years after the diagnosis, the cause of death being pulmonary embolism. In addition, the two malignant PPMs received adjuvant radiotherapy with subsequent chemotherapy in one [5]. PPM appears to have a rather indolent course, with the majority of the patients being disease-free at up to 24 years of follow-up.
In conclusion, primary pulmonary meningioma is a rare entity. The diagnosis is clinically and radiologically difficult and cytology on aspiration biopsies might not provide a definitive diagnosis. Surgical resection provides the material for final pathology and an excellent long-term prognosis, especially in those cases that proved to be benign.
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References
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Abstract
1. Introduction
