Diffuse pulmonary meningotheliomatosis
Diffuse pulmonary meningotheliomatosis is a rare condition manifested by multiple minute pulmonary meningothelial nodules (MPMNs) scattered throughout the lungs.
There may be an increased female predilection and they tend to peak around the 5th to 7th decades of life at the time of detection.
These nodules are generally asymptomatic and are usually found incidentally at autopsy or in surgical specimens resected for unrelated causes. Some patients may have clinical symptoms such as dyspnea and shortness of breath 3.
They are minute pulmonary meningothelial nodules histologically composed of small nests of epithelioid cells located within the lung interstitium.
Some MPMNs show monoclonal expansion, whereas others can be polyclonal, which generally suggest that MPMNs are reactive rather than being neoplastic.
Can show positivity for:
- epithelial membrane antigen
Negative staining for:
- S-100 protein
They may be seen as diffuse micronodules (with the sizes ranging of around 100 µm and 11 mm 1) similar to a miliary pattern seen in miliary tuberculosis or miliary metastases but can also present as small ground glass nodular opacities. Cavitation is rarely reported 1.
Treatment and prognosis
At the initial time of initial writing (early 2018), they are generally considered benign. Some authors have shown isolated MPMN lacking mutational damage, consistent with a reactive origin with some suggestion of MPMN-omatosis syndrome representing the transition between a reactive and neoplastic proliferation 8.
History and etymology
Minute pulmonary meningothelial‐like nodules (MPMNs) were first described by Korn et al. in 1960 7.
If a miliary pattern is seen in the lungs, refer to miliary opacities.
- 1. Jayaschandran V, Gjorgova-Gjeorgjievski S, Siddique H. An uncommon cause of miliary pattern of pulmonary nodules-diffuse pulmonary meningotheliomatosis. (2017) Respirology case reports. 5 (4): e00238. doi:10.1002/rcr2.238 - Pubmed
- 2. Gaffey MJ, Mills SE, Askin FB. Minute pulmonary meningothelial-like nodules. A clinicopathologic study of so-called minute pulmonary chemodectoma. (1988) The American journal of surgical pathology. 12 (3): 167-75. Pubmed
- 3. Suster S, Moran CA. Diffuse pulmonary meningotheliomatosis. (2007) The American journal of surgical pathology. 31 (4): 624-31. doi:10.1097/01.pas.0000213385.25042.cf - Pubmed
- 4. Morresi-Hauf AT, Reu S, Heiß-Neumann M, Gesierich W, Richter T, Wagner PK. [Diffuse pulmonary meningotheliomatosis]. (2015) Pneumologie (Stuttgart, Germany). 69 (9): 553-9. doi:10.1055/s-0034-1392437 - Pubmed
- 5. Huang EC, Zhang Y, Bishop JW, Gandour-Edwards RF, Afify AM. Diffuse pulmonary meningotheliomatosis: A diagnostically challenging entity on fine-needle aspiration cytology. (2015) Diagnostic cytopathology. 43 (9): 727-30. doi:10.1002/dc.23276 - Pubmed
- 6. Mukhopadhyay S, El-Zammar OA, Katzenstein AL. Pulmonary meningothelial-like nodules: new insights into a common but poorly understood entity. (2009) The American journal of surgical pathology. 33 (4): 487-95. doi:10.1097/PAS.0b013e31818b1de7 - Pubmed
- 7. Korn D, Bensch K, Liebow AA, Castleman B. Multiple minute pulmonary tumors resembling chemodectomas. (1960) The American journal of pathology. 37: 641-72. Pubmed
- 8. Ionescu DN, Sasatomi E, Aldeeb D, Omalu BI, Finkelstein SD, Swalsky PA, Yousem SA. Pulmonary meningothelial-like nodules: a genotypic comparison with meningiomas. (2004) The American journal of surgical pathology. 28 (2): 207-14. Pubmed