Critical Care: Neoplastic Disorders |

Primary Pulmonary Paraganglioma Presenting as a Solitary Lung Nodule FREE TO VIEW

Jonathan Chan, MD; Rahul Sangani, MD; Olusola Oduntan, MD; John Parker, MD
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West Virginia University, Morgantown, WV

Copyright 2016, American College of Chest Physicians. All Rights Reserved.

Chest. 2016;150(4_S):342A. doi:10.1016/j.chest.2016.08.355
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SESSION TITLE: Neoplastic Disorders

SESSION TYPE: Student/Resident Case Report Slide

PRESENTED ON: Monday, October 24, 2016 at 03:15 PM - 04:15 PM

INTRODUCTION: Paragangliomas are rare neuroendocrine tumors arising from extra-adrenal chromaffin cells. Primary pulmonary parangliomas (PPP) are especially scarce with fewer than 30 reported cases since 1958. We report an interesting case of asymptomatic PPP and its histopathology.

CASE PRESENTATION: A 52-year-old female smoker presents for evaluation of an incidental, 12x9 mm, well-circumscribed, solid right middle lobe lung nodule. Her baseline symptoms include a chronic smoker’s cough without hemoptysis or constitutional symptoms. Given intermediate pretest probability of malignancy, a positron emission tomography (PET) was performed and showed a mildly hypermetabolic nodule with no other abnormal uptake. Location of the nodule precluded bronchoscopy or CT-guided needle biopsy. The patient opted for serial radiographic follow-up instead of surgical lung biopsy. Repeat imaging demonstrated a 20% increase in size to 13x10 mm at 176 days with calculated doubling time of 666 days (Fig. 1). The patient agreed to thoracic surgery evaluation and underwent a video-assisted thoracoscopic right middle lobectomy with right hilar, interlobar, and subcarinal lymph node dissection. Pathology confirmed the diagnosis of paraganglioma (Fig. 2) with benign lymph nodes. Functional analysis for neuropeptides was unremarkable. The patient remained stable without new symptoms or recurrence on subsequent imaging.

DISCUSSION: Paragangliomas arise from neuroectodermal-derived tissue scattered throughout the body. PPPs are slow-growing neoplasms that typically occur in the interstitium. Most patients are asymptomatic with incidental finding of nodules, though symptoms occasionally develop from hypersecretion of specific neuropeptides. Extra-pulmonary paraganglioma must be ruled out to exclude pulmonary metastasis. Pathology demonstrates a Zellballen pattern (nests of ovoid-to-polygonal chief cells surrounded by sustentacular cells) and round-to-ovoid nuclei containing granular chromatin. Histochemically, the chief cells express neuroendocrine markers such as synaptophysin and chromogranin A, and the sustentacular cells express S-100 protein; cytokeratin is typically absent. Malignancy is uncommon, and PPP has been treated with local excision or lobectomy in most reported cases with no known recurrence.

CONCLUSIONS: This case was noteworthy because of the exceeding rarity of PPPs. Diagnosis requires histopathologic analysis given non-specific clinical presentation and imaging findings. Prognosis is generally favorable.

Reference #1: Zhang J-J, Liu T, Peng F. Primary paraganglioma of the lung: a case report and literature review. J Int Med Res. 2012;40(4):1617-26.

Reference #2: Aubertine CL, Flieder DB. Primary paraganglioma of the lung. Ann Diagn Pathol. 2004;8(4):237-41.

Reference #3: Ki NK, Lee KN, Mee SR, Pil JC, Doo KY. Pulmonary paraganglioma manifesting as an endobronchial mass. Korean J Radiol. 2008;9(1):87-90.

DISCLOSURE: The following authors have nothing to disclose: Jonathan Chan, Rahul Sangani, Olusola Oduntan, John Parker

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