Case Report Open Access
Copyright ©The Author(s) 2022. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Clin Cases. May 6, 2022; 10(13): 4196-4206
Published online May 6, 2022. doi: 10.12998/wjcc.v10.i13.4196
Primary pulmonary meningioma: A case report and review of the literature
Dan-Bin Zhang, Tao Chen, Department of Radiology, The First Affiliated Hospital, Zhejiang University School of Medicine, Hangzhou 310003, Zhejiang Province, China
ORCID number: Dan-Bin Zhang (0000-0002-4897-6358); Tao Chen (0000-0001-5013-323X).
Author contributions: Zhang DB was responsible for collecting the medical history of the patient and drafting the paper; Chen T reviewed the literature and revised the manuscript; all authors read and approved the final manuscript.
Informed consent statement: Written informed consent was obtained from the patient for the publication of this case report and accompanying images.
Conflict-of-interest statement: All authors declare no conflict of interest related to this study.
CARE Checklist (2016) statement: The authors have read the CARE Checklist (2016), and the manuscript was prepared and revised according to the CARE Checklist (2016).
Open-Access: This article is an open-access article that was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution NonCommercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: https://creativecommons.org/Licenses/by-nc/4.0/
Corresponding author: Tao Chen, Doctor, Department of Radiology, The First Affiliated Hospital, Zhejiang University School of Medicine, No. 79 Qingchun Road, Hangzhou 310003, Zhejiang Province, China. wmuchentao@zju.edu.cn
Received: September 14, 2021
Peer-review started: September 14, 2021
First decision: January 22, 2022
Revised: January 28, 2022
Accepted: March 14, 2022
Article in press: March 14, 2022
Published online: May 6, 2022

Abstract
BACKGROUND

Primary pulmonary meningioma (PPM) is a rare disease that is usually benign. The most common presentation of PPM is isolated pulmonary nodules or masses, so the disease can mimic any other lung tumor on imaging, especially lung cancer or metastasis.

CASE SUMMARY

A 47-year-old asymptomatic woman presented with a well-defined, lobulated pulmonary mass with calcification in the left lower lobe. The mass measured 69 mm × 57 mm × 61 mm and was found during a chest computed tomography (CT) performed for physical examination. Contrast-enhanced CT and positron emission tomography (PET)/CT revealed mild enhancement of the mass, with accumulation of 18-fluoro-2-deoxy-D-glucose (18F-FDG). Transbronchial biopsy suggested a provisional diagnosis of low-grade neuroendocrine tumor. Subsequent enhanced head magnetic resonance imaging revealed no positive lesions. An open cuff resection of the left lower lobe and wedge resection of the lingual segment were performed. Histopathological and immunohistochemical examination revealed that the mass was a PPM.

CONCLUSION

PPM should be considered in the differential diagnosis of isolated pulmonary masses found incidentally on CT and should be diagnosed based on a combination of radiological and histological features. Surgical resection is currently the main treatment strategy. No recurrence of benign PPMs has been reported after complete resection.

Key Words: Primary pulmonary meningioma, Contrast-enhanced computed tomography, Positron emission tomography, Case report

Core Tip: Primary pulmonary meningioma (PPM) is a rare tumour that usually presents as an asymptomatic solitary pulmonary mass. Limited knowledge of the disease can make diagnosis difficult. Here, we present the case of a 47-year-old woman with PPM.



INTRODUCTION

Primary ectopic meningiomas are rare tumors that occur in the head, neck, skin, peripheral nerves, bone, retroperitoneum, and lungs. They account for approximately 2% of meningiomas[1,2]. Primary pulmonary meningiomas (PPMs) are rare. Since the first case report in 1982 by Kemnitz et al[3], only 67 cases of PPMs have been reported domestically in the medical literature. Among these cases, only five were malignant meningiomas, and PPMs were more likely to be benign.

PPMs usually appear as isolated pulmonary nodules that are accidentally detected on chest radiographs or computed tomography (CT). Despite advancements in radiological examination such as enhanced CT and positron emission tomography (PET), it remains difficult to assess indeterminate isolated pulmonary nodules or masses, and many benign PPMs are misdiagnosed. The present paper reports a rare case of PPM. We also summarized the clinical imaging characteristics of PPMs in the literature to provide a reference for PPM diagnosis.

CASE PRESENTATION
Chief complaints

A 47-year-old woman had a pulmonary mass on physical examination 1 mo ago.

History of present illness

The patient was hospitalized due to chest CT findings of a pulmonary mass in the left lower lobe of the lung upon physical examination 1 mo prior.

History of past illness

The patient had a free previous medical history.

Personal and family history

The patient had no personal and family history.

Physical examination

Physical examination revealed no obvious positive signs.

Laboratory examinations

All tumor marker results were within the normal range.

Imaging examinations

Contrast-enhanced chest CT revealed a 6.9 cm diameter mass with a well-circumscribed margin in the left lower lobe of the lung. The adjacent left lower lobar bronchus and lingual segment of the left upper lobar bronchus were compressed by the mass. The lesion was confined to the lung parenchyma and showed striated calcification. After contrast enhancement, the mass showed mild homogeneous enhancement, from a pre-contrast attenuation of 40 HU to a postcontrast attenuation of 60 HU (Figure 1).

Figure 1
Figure 1 Contrast-enhanced chest computed tomography images of (A, B) unenhanced and (C) enhanced scan. A 6.9-cm diameter well-circumscribed mass in the left lower lobe of the lung shows mild homogeneous enhancement.

On 18-fluoro-2-deoxy-D-glucose (FDG) PET imaging, the standardized uptake value (SUV) of the mass increased unevenly, with a maximum value of 4.4, which suggested malignant lesion (Figure 2). No other lesions were detected on PET/CT. Moreover, enhanced magnetic resonance imaging (MRI) of the brain showed no evidence of intracranial tumors or metastases. Bronchoscopy revealed partial obstruction of the lower left lobe by the mass and narrowing of the lingual opening in the upper left lobe. A subsequent transbronchial biopsy result suggested a low-grade neuroendocrine tumor (Figure 3).

Figure 2
Figure 2 Positive uptake by the mass on 18F-fluorodeoxyglucose-positron emission tomography suggesting malignancy.
Figure 3
Figure 3 The transbronchial biopsy result: Hematoxylin and eosin staining showed that a few nested epithelioid cells and abnormal cells were observed in the tissue (200×).
FINAL DIAGNOSIS

The final diagnosis of the presented case was PPM.

TREATMENT

Considering the CT and PET features of the mass and the results of transbronchial biopsy, an open cuff resection of the left lower lobe and wedge resection of the lingual segment were performed. Gross examination revealed a 6.5 cm, off-white, tenacious texture mass. Microscopic examination revealed a tumor with focal bronchial cartilage involvement, no pleural involvement, and fusiform nests of cells arranged in fascicles or whorls. Immunohistochemistry showed positivity for epithelial membrane antigen (EMA), progesterone receptor (PR), somatostatin receptor 2 (SSTR2), D2-40, and CD34, and negativity for S-100, cytokeratin (CK), glial fibrillary acidic protein, CgA, SOX10, and SMA; the Ki-67 index was about 5%-10% positive (Figure 4). These morphological and immunohistochemical features were suggestive of a PPM. Preoperative contrast-enhanced chest CT, contrast-enhanced brain MRI, and PET-CT did not reveal evidence of intracranial or spinal meningioma.

Figure 4
Figure 4 Histological features of primary pulmonary meningioma. A-D: Macroscopically, primary pulmonary meningioma (PPM) showed as spindle or oval cells organized in bundles and whorls on hematoxylin-eosin staining (25×; 50×; 100×; 200×); E-H: Immunohistochemically (200×), PPM showed negativity for E: Cytokeratin, positive for F: Epithelial membrane antigen; G: Progesterone receptor; H: Somatostatin Receptor 2 (SSTR2).
OUTCOME AND FOLLOW-UP

The patient was disease-free after 3 mo of follow-up.

DISCUSSION

A total of 68 patients diagnosed with PPM were reported in the English literature from 1982 to 2021. All of these patients received histological assessment confirming PPM. Eighteen cases were excluded because (1) They underwent no radiological examination; or (2) They received no radiological evaluation of the CNS negative for meningioma. Ultimately, 50 patients (including the case reported above) were included in the analysis.

Patient characteristics

The study group comprised 50 patients: 19 men and 31 women. The age range was 18–108 years (median age: 58.0 years). Thirty-five patients were asymptomatic and only occasionally showed pulmonary nodules or masses on chest CT or X-ray. Thirteen patients had respiratory symptoms, including chest pain, chest tightness, hemoptysis, cough, and sputum). In addition, two patients had non-specific symptoms[3,4]. There were nine patients with a history of malignancy: two had suffered lung adenocarcinoma[5,6], two colorectal cancer[7,8], two breast cancer[9,10], one buccal cancer[11], one papillary thyroid carcinoma[12], and one thymoma and kidney cancer[13] (Table 1)[14-47].

Table 1 Patient characteristics.
No.
Ref.
Age (Gender)
Symptom
Size (cm)
Histology
Follow-up
1Kemnitz et al[3]59 (F)Weakness, loss ofappetite, weight loss4.0B30
2Chumas et al[14]58 (F)None4.0B12
3Zhang et al[15]58 (F)None2.5B18
4Kodama et al[16]53 (M)None2.6B84
5Drlicek et al[17]41 (M)None2.5B72
662 (F)None6.0B72
7Flynn et al[18]63 (F)Coughing3.0B44
874 (F)None1.7B37
9Maiorana et al[19]68 (M)None1.8B24
10Kaleem et al[20]45 (F)None1.2B10
11Lockett et al[21]65 (M)None0.8B5
12Ueno et al[22]61 (F)None0.4-1.5B36
13de Perrot et al[5]57 (F)None0.9B30
14Prayson et al[23]51 (M)None6.5M10
15Spinelli et al[24]71 (F)Bronchitis1.5B96
16Falleni et al[7]59 (M)None2.5B30
17Cesario et al[25]56 (M)None2.0B72
18CURA et al[26]58 (F)None2.0BN
19Comin et al[27]33 (M)Hemoptysis and thoracic pain2.0B47
20Rowsell et al[28]51 (M)None4.0B8
21Picquet et al[9]54 (F)None1.4B6
22Kaneda et al[29]59 (F)None1.4B14
23van der Meij et al[30]40 (F)Dyspnea, coughing dysphagia5.0M40
24Meirelles et al[31]48 (M)None1.5BN
25Incarbone et al[32]24 (M)Hemoptysis2.4B42
26Izumi et al[33]18 (F)Hemoptysis on exertion3.3B15
27Weber et al[4]108 (F)Asthenia, lack of appetite, loss of weight and anxiety15.0MN
28Lepanto et al[10]60 (F)None1.6B12
29Kim et al[34]61 (F)Chest pain2.5B84
30Jiang et al[35]63 (F)None3.5BN
31Juan et al[11]55 (M)None4.5B6
32Oide et al[36]44 (M)None2.0BN
33Huang et al[37]44 (F)Chest pain2.5B6
34Žulpaitė et al[38]43 (F)None4.5M24
35Hong et al[39]54 (M)Cough and sputum1.6B24
36Luo et al[40]65 (F)Cough3.5BN
37Xu et al[41]65 (F)Chest pain and tightness0.7BN
38Ohashi et al[42]60 (F)None2.0B36
39Bae et al[43]43 (F)None1.9B26
40Cimini et al[13]80 (M)None1.4BN
4180 (M)None1.2MN
42Wang et al[44]64 (F)None3.4 (cystic nodules 0.8-2)BN
43Fujikawa et al[12]62 (F)None0.8B20
45Han et al[6]64 (F)None0.6B28
4475 (F)None0.6B2
46Gürçay et al[45]55 (F)Cough 2.0BN
47Jiang et al[8]70 (M)None1.5BN
48Bas et al[46]57 (M)Cough1.0BN
49Oh et al[47]54 (M)None0.5-1.3B24
50Present report46 (F)None6.9B3
Radiological characteristics

Most PPMs were benign, and only five cases were malignant[4,13,23,30,38]. Benign PPMs were generally well-circumscribed on radiological studies, with diameters ranging from 0.4 to 6 cm (median: 2 cm). The five malignant PPMs ranged in diameter from 1.5 to 15 cm (median: 6.4 cm). On chest CT scan, benign PPMs usually appear as isolated, rounded, solid, well-defined nodules or masses, with or without lobulation. Five cases were lobulated[24,31,32,37,40], two manifested as ground glass density[11,45], and two showed burrs on the edges[6]. In addition, one recent study reported that the PPM showed multiple cystic lesions with a solid component[44]. The CT features of the lesions were not described in the remaining eight cases (Table 2)[14-47].

Table 2 Radiological characteristics.
No.
Ref.
Location
CT feature
Enhancement feature
PET/CT
1Kemnitz et al[3]RL-PWell-circumscribedNN
2Chumas et al[14]RL-PWell-circumscribedNN
3Zhang et al[15]LU-PWell-circumscribedNN
4Kodama et al[16]LU-PNNN
5Drlicek et al[17]LL-NWell-circumscribedNN
6LL-NNNN
7Flynn et al[18]LU-CWell-circumscribedNN
8LL-PWell-circumscribedNN
9Maiorana et al[19]N-PWell-circumscribedNN
10Kaleem et al[20]LL-PWell-circumscribedNN
11Lockett et al[21]LL-PWell-circumscribedNN
12Ueno et al[22]Bil-NNNN
13de Perrot et al[5]RL-PWell-circumscribedNN
14Prayson et al[23]RU-PSmooth margins and focal necrosisNN
15Spinelli et al[24]N-PLobulated marginsNN
16Falleni et al[7]LU-PWell-circumscribedNN
17Cesario et al[25]LU-PWell-circumscribedNN
18CURA et al[26]RU-CWell-circumscribedEnhancementHigh uptake (no value)
19Comin et al[27]LU-PNNN
20Rowsell et al[28]RL-CNNN
21Picquet et al[9]LL-PWell-circumscribedNN
22Kaneda et al[29]N-PWell-circumscribedNN
23van der Meij et al[30]RH-CNNN
24Meirelles et al[31]RL-CLobulated marginsNHigh uptake (12.9)
25Incarbone et al[32]RU-PLobulated marginsNHigh uptake (10.14)
26Izumi et al[33]LU-CWell-circumscribedNN
27Weber et al[4]RL-CNNN
28Lepanto et al[10]LL-PNNLow uptake (1.2)
29Kim et al[34]RU-PWell-circumscribedHomogeneous enhancement enhancementN
30Jiang et al[35]LU-PWell-circumscribedNN
31Juan et al[11]LU-PGround-glass opacityNN
32Oide et al[36]LU-PWell-circumscribedNN
33Huang et al[37]RL-PCalcifications, mild peripheral lobulationMild enhancementN
34Žulpaitė et al[38]LU-PNHomogeneous enhancementN
35Hong et al[39]LU-PWell-circumscribedHeterogeneous enhancementN
36Luo et al[40]RL-PHeterogeneous lobulatedNN
37Xu et al[41]RL-PWell-circumscribedNN
38Ohashi et al[42]RL-PNNN
39Bae et al[43]RL-COval-shapedWell-enhancementMildly high uptake (2.48)
40Cimini et al[13]RU-PNNo significant enhancementHigh uptake (4.63)
41LU-NNEnhancementMildly high uptake (2.46)
42Wang et al[44]RL-NMultiple thin-, smooth-walled cysts or cystic nodules with solid componentMild enhancementN
43Fujikawa et al[12]LL-PWell-circumscribedNN
45Han et al[6]RL-PBurrs on the edgesNN
44RL-PBurrs on the edgesNN
46Gürçay et al[45]RU-PPeripheral ground-glassNLow uptake (1.89)
47Jiang et al[8]RL-PWell-circumscribedMild centripetal enhancementLow uptake (0.6)
48Bas et al[46]LL-PWell-circumscribedNLow uptake (no value)
49Oh et al[47]scatteredWell-circumscribedNMildly high uptake (3.1)
50Present reportLL-CWell-circumscribedMild homogeneous enhancement High uptake (4.4)

The CT enhancement patterns were described in 11 patients: six cases showed homogeneous enhancement, one showed heterogeneous enhancement[39], two showed mild enhancement[37,44], one showed mild concentric enhancement[8], and one showed no significant enhancement[13].

18F-fluorodeoxyglucose-PET was performed in 12 patients, including our reported case. The PET scans of four patients showed no accumulation of 18F-FDG in lung lesions[8,10,45,46]. Seven patients showed metabolically active lesions suspicious for malignancy, with a reported SUV range from 2.46 to 12.9 in seven cases. No other extra-pulmonary sites with increased FDG uptake were detected in any of the patients.

The prognosis of benign PPM resection is good, with almost no recurrence or metastasis. Follow-up was reported in 35 benign cases, ranging from 2 to 96 mo (median: 24 mo). However, two malignant PPMs relapsed[23,30]. The above summary is presented in Table 3.

Table 3 Clinical and imaging characteristics of primary pulmonary meningioma patients.
Variables

Number
Ratio (%)
Gender (n = 50)Female3162.0
Male1938.0
Age (n = 50)≤ 40 yr48.0
40–60 yr2652.0
≥ 60 yr2040.0
Symptoms (n = 50)No3570.0
Yes1530.0
Size (n = 50)≤ 3 cm3774.0
> 3 cm1326.0
Histology (n = 50)Benign4590.0
Malignant510.0
Site (n = 47)RL1531.9
RU612.8
LL1021.3
LU1327.7
Other36.4
Location (n = 45)Peripheral3680.0
Centrilobar920.0
Main CT features (n = 38)Well-circumscribed2771.1
Lobulated513.2
Burrs25.3
Ground-glass density25.3
Calcification25.3
PET/CT (n = 12)High uptake866.7
Low uptake433.3

Primary ectopic pulmonary meningiomas are very rare, and only 67 cases (including our report) of PPM have been reported in the English language medical literature. The present study reported a case with very complete clinical procedure and imaging data, including preoperative enhanced CT examination, PET-CT examination, bronchoscopy biopsy, and postoperative pathological results. There were rare signs of calcification on CT, false positives on PET-CT and errors in our biopsy results. This suggests that we need to be cautious when excluding PPM only through auxiliary examination or even needle biopsy in clinical work.

The pathogenesis of PPMs remains unclear. One hypothesis is that the tumors develop from multipotent mesenchymal cells. Another states that PPMs originate from minute pulmonary meningothelial nodules that are occasionally found in approximately 1% of autopsies and excised lung specimens[48]. However, the incidence of meningiomas is much lower than that of meningeal epithelial nodules. Moreover, previous genotypic comparisons have failed to demonstrate pulmonary meningeal epithelial nodules or intracranial meningiomas, further supporting the hypothesis[49].

To date, approximately 90% of PPMs reported in the literature have been benign, while five have been malignant[4,13,23,30,38]. Most patients with PPM have no obvious symptoms, while some have respiratory or non-specific symptoms. Clinical symptoms may be related to the lesion location. As previously reported, benign PPMs are usually located in the peripheral pulmonary region, with no involvement of the bronchi, blood vessels, or pleura. Some PPM patients have a known history of malignancy[8], so a comprehensive and careful evaluation of pulmonary lesions must be carried out to avoid the misdiagnosis of metastasis.

Radiologically, PPMs usually appear as isolated, solid, and well-defined parenchymal coin-like lesions, ranging in size from 0.4 to 6.5 cm. Approximately 74.0% of PPMs are less than 3 cm in diameter. The lesions may present with burrs, lobulation, ground-glass density, or calcification, but these features are uncommon. Furthermore, one study reported a PPM presenting as multiple cystic lesions[44]. PPMs have diverse enhancement CT manifestations. They usually show different degrees of enhancement, or even no significant enhancement. Hence, the pattern of lesion enhancement may not help to determine whether the lesion is benign or malignant. On 18F-FDG PET, most PPMs exhibit high or mildly high metabolic activity, as in in our reported case. Only four PPMs showed low uptake of 18F-FDG[8,10,45,46]. However, one recent study reported a patient with both benign and malignant PPMs, both characterized by increased glucose uptake[13]. This suggests that the malignancy of PPMs may not be related to 18F-FDG uptake.

Pathological identification is necessary to allow PPM diagnosis; however, diagnosis can sometimes be difficult using needle biopsy alone[32]. False positives are sometimes reported, in addition to negative reports. For instance, in the case reported by Žulpaitė et al[38], a false positive diagnosis of paraganglioma was given based on preoperative transthoracic needle biopsy. The present patient was misdiagnosed as having low-grade neuroendocrine tumor based on preoperative bronchoscopic biopsy.

CONCLUSION

In conclusion, the accurate diagnosis of PPM is challenging because the tumors are rare and show variable radiological manifestations. A single 18F FDG PET or contrast-enhanced CT examination may not be sufficient to evaluate patients with PPM. Surgical resection is the main treatment strategy, and no relapse has been reported in benign cases after complete resection. In clinical practice, attention should be paid to common isolated pulmonary nodule or mass, especially in asymptomatic patients. PPM should be considered in the differential diagnosis of lung diseases.

Footnotes

Provenance and peer review: Unsolicited article; Externally peer reviewed.

Peer-review model: Single blind

Specialty type: Radiology, nuclear medicine and medical imaging

Country/Territory of origin: China

Peer-review report’s scientific quality classification

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Grade B (Very good): B

Grade C (Good): C

Grade D (Fair): 0

Grade E (Poor): 0

P-Reviewer: Soriano-Ursúa MA, Mexico; Sultana N, Bangladesh S-Editor: Ma YJ L-Editor: A P-Editor: Ma YJ

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