Diffuse pulmonary meningotheliomatosis with pan-TRK expression by immunohistochemistry: a novel finding and potential pitfall

Abstract

Background: Pulmonary meningothelial-like nodules (PMNs) are benign proliferations of unclear clinical significance. They are mainly asymptomatic lesions that are usually discovered during the pathologic evaluation of resected pulmonary specimens or following post-mortem examination. Diffuse pulmonary meningotheliomatosis (DPM), which presents as bilateral multiple PMNs throughout the lungs, has been described less frequently. DPMs are benign lesions associated with both neoplastic and non-neoplastic pulmonary conditions.

Case presentation: We report the case of a 59-year-old female patient who presented with a history of cough. Computerized tomography (CT) imaging revealed multiple subcentimeter bilateral pulmonary nodules. transbronchial biopsies were obtained which revealed foci of nodular interstitial proliferations composed of epithelioid to spindled cells in a vague whorled pattern. Immunohistochemical stains were diffusely positive for EMA and progesterone receptor. Furthermore, pan-TRK exhibited strong and diffuse membranous expression in the lesional cells. INSM1 was negative for expression. RNA-based next-generation sequencing for the detection of NTRK fusions was performed and was negative for gene rearrangements involving NTRK1, NTRK2, and NTRK3.

Conclusion: Here, we report a rare case of DPM and report pan-TRK expression in PMNs which has not been described. We provide a brief review of the literature and provide insight into the potential physiologic nature of PMNs. Lastly, we emphasize the recognition of pan-TRK immunoexpression in PMNs to avoid potential diagnostic errors.

Keywords: Diffuse pulmonary meningotheliomatosis; Immunohistochemistry; Lung; Meningothelial-like nodule; NTRK; Pan-TRK.

Fig. 1

Imaging findings. Selected axial non-contrast chest CT image shows bilateral multiple small nodules randomly distributed through both lungs

Fig. 2

Histological and immunohistological findings. A-B H&E sections showed meningothelial-like nodules which are distinct from the neighboring normal lung architecture. C Higher magnification shows an interstitial proliferation of epithelioid and spindled cells with a vague whirling and streaming pattern. D The cells comprising the nodules showed diffuse cytoplasmic staining with EMA (E), strong nuclear PR expression (E) and diffuse membranous expression with pan-TRK (F). Original magnification: x100 (A-B), x200 (C-F).