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Minute pulmonary meningothelial-like nodules:

Minute pulmonary meningothelial-like nodules are incidental small (about 2 mm) subpleural nodules.

These are meningothelial arachnoid granulation tissue rather than a neoplasm. 

They share histologic, ultrastructural, and immunohistochemical features with meningiomas.

These nodules are generally asymptomatic and are usually found incidentally at autopsy or in surgical specimens resected for unrelated causes.

Histologically, the lesions were composed of small clusters of epithelioid cells with round to oval nuclei devoid of atypia and surrounded by abundant eosinophilic cytoplasm.

Immunohistochemical studies shows positivity of the tumor cells for epithelial membrane antigen and vimentin, and negative staining for cytokeratin, actin, S-100 protein, CD34, chromogranin, and synaptophysin.

Meningiomas :

Ectopic meningiomas arising in the lung are rare. These lesions are usually benign, grow slowly, and have an excellent prognosis.

Primary meningioma of lung  should be differentiated from metastatic meningioma .

Gross: Well circumscribed intraparanchymatous masses that measure upto 10cm in maximal dimension.

Microscopic features:  Both transitional and fibrous forms are noted.

Histopathologically the tumor is characterized by whorled nests of bland-appearing spindle cellscells accompanied by psammoma bodies.

Mitotic figures are rare and atypia is usually not present.

Immunohistochemistry:  Coexpression of EMA and vimentin in the same tumour cell. Keratin, S-100 protein and CD34 are focally positive.

Atypical meningioma:

Atypical meningioma with loss of architectural pattern, mild nuclear pleomorphism, increased mitotic counts (up to 15 mitotic figures per 10 high power fields), and focally prominent nucleoli.

Focally, cells with rhabdoid features were identified.

The tumour's immunohistochemical and ultrastructural profiles are consistent with a meningioma.

Rarely, primary pulmonary meningiomas may present as high-grade malignant lesions.

            

Diffuse pulmonary meningotheliomatosis. Am J Surg Pathol. 2007 Apr;31  (4):624-31

Minute pulmonary meningothelial nodules are rare lesions histologically composed of small nests of epithelioid cells located within the interstitium of the lung. These nodules are generally asymptomatic and are usually found incidentally at autopsy or in surgical specimens resected for unrelated causes. The lesions are most often single, although multiple lesions with unilateral involvement of one or even all lobes of the same lung have been described. To our knowledge, cases of meningothelial nodules with disseminated bilateral pulmonary involvement associated with clinical symptoms of restrictive pulmonary disease and radiologic evidence of diffuse reticulonodular pulmonary infiltrates have not been previously documented. We have studied 5 patients presenting with diffuse bilateral pulmonary involvement by numerous minute pulmonary meningothelial nodules. The patients were 4 women and a man aged 54 to 75 years who presented clinically with dyspnea and shortness of breath and the lesions were discovered on open lung biopsies performed for the evaluation of diffuse bilateral interstitial lung infiltrates found on chest x-rays and computed tomography scans. In 3 patients, there was a previous history of malignancy and the radiologic findings were suspected of representing diffuse metastatic disease. Histologically, the lesions were composed of small clusters of epithelioid cells with round to oval nuclei devoid of atypia and surrounded by abundant eosinophilic cytoplasm. Immunohistochemical studies showed positivity of the tumor cells for epithelial membrane antigen and vimentin, and negative staining for cytokeratin, actin, S-100 protein, CD34, chromogranin, and synaptophysin. Electron microscopic examination in 1 case confirmed the ultrastructural features of meningothelial cells, including complex cytoplasmic interdigitations joined by well-developed desmosomes and abundant intracytoplasmic intermediate filaments. The diffuse bilateral involvement of lung parenchyma in the present cases can lead to confusion on clinical and radiologic grounds with a variety of interstitial pulmonary processes, including idiopathic interstitial pneumonia and lymphangitis carcinomatosa. Diffuse pulmonary meningotheliomatosis should be considered in the clinical differential diagnosis of diffuse interstitial pulmonary infiltrates.

Pulmonary meningothelial-like nodules: a genotypic comparison with meningiomas. Am J Surg Pathol. 2004 Feb;28(2):207-14.

BACKGROUND: Minute pulmonary meningothelial-like nodules (MPMNs) are incidental interstitial pulmonary nodules. They share histologic, ultrastructural, and immunohistochemical features with meningiomas (MGs). DESIGN: Sixteen cases yielding 33 separate MPMNs and 10 cases of benign MG were studied. Immunohistochemical studies and mutational analyses were performed on microdissected tissue using 20 polymorphic microsatellite markers targeting 11 genomic regions in an effort to identify genetic similarities of MPMN and MG. RESULTS: A total of 96.6% of MPMNs stained positive for vimentin, 33.3% for epithelial membrane antigen, 3% for S-100, and all were negative for cytokeratin and synaptophysin. Loss of heterozygosity (LOH) was identified in 25% of single MPMN affecting 3 genomic loci. No solitary MPMN had more than 1 LOH event. Multiple LOHs were seen only in MPMN-omatosis syndrome, where 33.3% of MPMNs showed LOH affecting 7 genomic loci. MG showed the highest frequency of LOH with major events seen at 22q (60%), 14q (42.8%), and 1p (44.4%) that were not shared by MPMN. CONCLUSION: Isolated MPMN lacks mutational damage, consistent with a reactive origin. MPMN-omatosis syndrome might represent the transition between a reactive and neoplastic proliferation. MPMNs are different from MG based on the major molecular genetic events seen in their formation and progression.

Minute pulmonary meningothelial-like nodules: a case report.Nihon Kokyuki Gakkai Zasshi. 2002 Jun;40(6):499-502.

The presence of multiple small nodular shadows on a routine chest radiograph was noticed in a 56-year-old woman who had undergone a left mastectomy on the diagnosis of breast cancer 8 years before. Chest CT films revealed small nodules scattered beneath the pleura mainly in both lower lobes. A biopsy was performed during video-assisted thoracoscopy to rule out metastasis from breast cancer. Biopsy specimens showed spindle--or oval-shaped cells arranged in nests associated with a dedicated network of capillaries. These findings were compatible with minute meningothelium-like nodules (MN). The pathogenesis of MN is still unknown. It is common in elderly women, and the prognosis is excellent without any treatment. MN is an important disease in the differential diagnosis of multiple nodular shadows found on chest CT.

Progesterone receptor immunoreactivity in minute meningothelioid nodules of the lung.Virchows Arch. 2002 May;440(5):543-6.  

Meningothelioid nodules (MNs) are not uncommon lesions of the pulmonary interstitium composed of monomorphic round to spindle cells, likely reactive in nature. Their origin is unsettled, though a derivation from arachnoid-like cells in conditions of perturbed perfusion of the pulmonary tissue has been proposed. Here we confirm the consistent occurrence of intense progesterone-receptor immunoreactivity in MNs fortuitously detected in surgical specimens of lung carcinomas. This finding corroborates the view that these proliferations exhibit arachnoid cell-like differentiation and suggests a role for sex-steroid hormones in the control of their growth.

Minute pulmonary meningothelial-like nodules: thin-section CT appearance.J Comput Assist Tomogr. 2001 Mar-Apr;25(2):311-3

Minute pulmonary meningothelial-like nodules are often incidentally discovered during pathologic evaluation of pulmonary parenchymal specimens. These lesions were once thought to represent pulmonary chemodectomas, but pathological studies have shown that they are not of neuroendocrine origin. Minute pulmonary meningothelial-like nodules are benign, perhaps reactive in nature, but are occasionally found in association with lung carcinoma. They may appear as randomly distributed well-defined micronodules on thin-section chest CT, and thus may simulate metastatic disease when associated with lung carcinoma.

Immunohistochemical and clonal analysis of minute pulmonary meningothelial-like nodules. Hum Pathol. 1999 Apr;30(4):425-9.

The histogenesis of meningothelial-like nodule or so-called minute pulmonary chemodectoma remains unclear, with various immunohistochemical analyses giving inconsistent results. We performed an immunohistochemical and clonal analysis of minute pulmonary meningothelial-like nodules. Thirty-one histologically defined meningothelial-like nodules in 14 cases were stained immunohistochemically. One case had multiple lesions with brown pigment granules, which were positively stained with Berlin blue method, indicating the presence of hemosiderin. All meningothelial-like nodules were positive for vimentin and epithelial membrane antigen (EMA), but not for S-100 protein, chromogranin A, or synaptophysin. Five of 13 cases (13 of 28 lesions) were positive for CD68 by KP-1. Ten cases (24 lesions) stained for CD68 by PG-M1 were weakly positive. All lesions were negative for lysozyme, myosin, actin, keratin, and melanoma-associated antigen. Alveolar macrophages were intensely positive for CD68 and lysozyme in all examined cases. We analyzed the clonality of 11 minute pulmonary meningothelial-like nodule lesions in two female cases based on an X-chromosome-linked polymorphic marker, the human androgen receptor gene (HUMARA). The HUMARA was found to be amplified with or without prior digestion by the methylation-sensitive restriction endonuclease HpaII. Six of 11 lesions showed monoclonal expansion. Five lesions in a multiple case showed different patterns of monoclonality. Our findings showed that minute pulmonary meningothelial-like nodules have meningothelial-like and phagocytic characteristics but no muscular phenotype. Furthermore, some minute pulmonary meningothelial-like nodules may show monoclonal expansion, whereas others are polyclonal. Our data indicate that minute pulmonary meningothelial-like nodules are reactive rather than neoplastic.

Primary pulmonary malignant meningioma. Ann Thorac Surg. 2005Oct;80 (4):1523-5.

Primary pulmonary meningiomas are relatively rare and mostly benign. To exclude pulmonary metastasis of an intracranial meningioma, imaging studies of the brain should be performed. We believe that only one primary pulmonary malignant meningioma in which a metastasis from the brain was excluded has been reported. In this report we describe a second case with malignant features.

Primary pulmonary meningioma; report of a case.Kyobu Geka. 2005 Jun;58(6):512-5.

Primary pulmonary meningiomas are quite rare, and their occurrence has been reported only sporadically. A 49-year-old, asymptomatic female was hospitalized for the evaluation of a coin lesion in the left lung radiography. She has no history of previous neoplasm or symptom referable to the central nervous system. Chest computed tomography (CT) demonstrated a 9 x 14 mm, round, noncalcified, well-demarcated lesion in the left upper lobe of the lung (S(1+2)). For diagnostic purposes, enucleation of the tumor was performed. The resected specimen revealed histologically classical typical meningioma. Because postoperative magnetic resonance imaging (MRI) of the brain did not show any intracranial mass, this case was and diagnosed as a primary pulmonary meningioma. The patient was discharged with no complication, and alive without recurrence of disease 14 months after surgery.

Primary pulmonary meningioma first suspected of being a lung metastasis. Ann Thorac Surg. 2005 Apr;79(4):1407-9.

Primary extracranial and extraspinal meningiomas are rare tumors. We describe a primary pulmonary meningioma first suspected of being a metastasis because it presented as a solitary subpleural pulmonary nodule in a patient with breast cancer. The absence of radiographic change after 6 months of chemotherapy led to resection of the breast and lung lesions. A complete central nervous system evaluation eliminated other locations of meningioma, allowing the diagnosis of primary pulmonary meningioma.

Primary pulmonary meningioma manifesting as a solitary pulmonary nodule with a false-positive PET scan.J Thorac Imaging. 2006;21 (3):225-7. 

Primary pulmonary meningioma is very rare, with about 30 cases reported in the English literature. These lesions are usually benign, grow slowly, and have an excellent prognosis. However, they can mimic any other pulmonary tumor, as the most common presentation is as a solitary pulmonary nodule. We report a case of a primary pulmonary meningioma manifesting as a solitary lung nodule with a very high metabolic activity on the positron emission tomography, mimicking a primary lung cancer.

Primary pulmonary meningioma: report of a case and review of the literature.Tumori. 2003 Jan-Feb;89(1):102-5.

Primary pulmonary meningiomas are rare and their occurrence has been reported only sporadically. The diagnosis of such tumors should be accepted only after the presence of an intracranial or intraspinal meningioma has been excluded. The morphological and immunohistochemical features are similar to those of intracranial meningiomas. A case of primary pulmonary meningioma along with a review of the literature and discussion of other lesions in the differential diagnosis of this rare neoplasm are here presented.

Fine needle aspiration cytology of primary pulmonary meningioma associated with minute meningotheliallike nodules. Report of a case with histologic, immunohistochemical and ultrastructural studies. Acta Cytol. 2002 Sep-Oct;46(5):899-903.

BACKGROUND: Pulmonary meningioma is an unusual tumor located in the lung. Minute pulmonary meningotheliallike nodules are also uncommon tumor-like lesions found at the pleuropulmonary level as solitary or multiple nodules. The association of both entities is described in a recent report. CASE: A 58-year-old, male smoker without pathology underwent radiologic study before a surgical procedure. A peripheral nodule was found in the left lower lobe of the lung with benign characteristics. Fine needle aspiration cytology was performed under computed tomography guidance. Cytologic examination showed features of transitional meningioma and other lesions near the tumor. Minute pulmonary meningotheliallike nodules were also present. CONCLUSION: A diagnosis of meningioma should be considered when there is a combination of scanty material, whorls composed of concentrically arranged cells and isolated cells with intranuclear inclusions. The simultaneous presence of meningioma and meningothelial-like nodules corroborates the unifying hypothesis of their common origin and the nature of these rare pulmonary lesions.

Cytology of primary pulmonary meningioma. Report of the first multiple case. Acta Cytol. 1998 Nov-Dec;42(6):1424-30.

BACKGROUND: Ectopic meningiomas arising in the lung are rare. We report here the first multiple primary case diagnosed by intraoperative imprint cytology. CASE: Asymptomatic pulmonary nodules, two in the left and three in the right lung, were found in a 61-year-old woman, and video-assisted thoracoscopic surgery was undertaken. Because the largest tumor was diagnosed as a meningioma by intraoperative imprint cytology using an excised biopsy specimen, further resection was not performed immediately. Histopathologically the tumor was characterized by whorled nests of cells accompanied by psammoma bodies intermingled with a fibrous pattern. The diagnosis was a transitional meningioma, positive for vimentin and epithelial membrane antigen and negative for keratin immunohistochemically. All the nodules were subsequently surgically resected and showed a similar cytohistologic appearance. Ultrastructurally the tumor cells demonstrated interdigitation of adjacent plasma membranes with numerous desmosomes and hemidesmosomes, typical of meningiomas. We failed to detect another primary tumor in the nervous system, and at this writing the patient was healthy three years after the operation. CONCLUSION: Because of the characteristic cytomorphologic features of primary pulmonary meningioma, the cytologic approach provides useful information for therapy.

Unsuspected primary pulmonary meningioma.Eur J Cardiothorac Surg. 2002 Mar;21(3):553-5.

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.

Primary pulmonary meningioma may arise from meningothelial-like nodules. Adv Clin Path. 2000 Jan;4(1):35-9.

A rare case of primary pulmonary meningioma, associated with meningothelial-like nodules in the same lung resection, with preoperative fine needle aspiration cytological findings and ultrastructural features, is described. The simultaneous presence of meningioma and meningothelial-like nodules, never before described, corroborates the unifying hypothesis of their common origin and nature.

Primary pulmonary malignant meningioma.Am J Surg Pathol. 1999 Jun;23(6):722-6.

Fewer than 20 cases of primary pulmonary meningioma have been reported. Most of these cases have been histologically and clinically benign. We report an unusual case of primary pulmonary malignant meningioma with atypical histologic features and malignant behavior. A computed tomography scan of the head did not show evidence of tumor. The right upper lobe mass was resected and showed features of an atypical meningioma with loss of architectural pattern, mild nuclear pleomorphism, increased mitotic counts (up to 15 mitotic figures per 10 high power fields), and focally prominent nucleoli. Focally, cells with rhabdoid features were identified. The tumor's immunohistochemical and ultrastructural profiles were consistent with a meningioma. The tumor stained negative for estrogen and focally positive for progesterone receptors and had a MIB-1 labeling index (marker of cell proliferation) of 9.2%. Approximately 5 months after the initial resection, the patient experienced a tumor recurrence with multiple lymph node metastases, spread to the middle and lower lobes of the right lung, and metastasis to the diaphragm. Rarely, primary pulmonary meningiomas may present as high-grade malignant lesions.

Primary pulmonary meningioma presenting as lung metastasis.
Scand Cardiovasc J. 1999;33(2):121-3.

A benign primary pulmonary meningioma, an extremely rare tumour, was incidentally detected in a 57-year-old woman in association with a contralateral pulmonary adenocarcinoma. The meningioma was initially suspected to be a metastasis. Both tumours were excised, with excellent outcome. Anatomic features of primary pulmonary meningioma and differential diagnosis are discussed.

 
November  2009

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