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Marc Freiman Wednesday Pulmonary Conference August 7, 2013 Long Case

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HPI 50 yo woman from the Dominican republic presenting to pulmonary clinic for cough 4-5 years Symptoms may have started after a cold ? Worse in the summer, no temporal relation to night/day Dry, non-productive DOE 2-3 city blocks, 2 flights of stairs ROS - Denies HA, sinus congestion, heartburn, reflux. Denies chest pain, palpitations, orthopnea, PND or edema

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PMHx/Soc Hx Vitiligo Denies childhood asthma From DR 3 years ago Worked in paper shredding factory for 1 yr Currently works in retail Never smoker No EtOH, illicits

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Physical exam Afeb P 95 130/84 96% RA; BMI 30 (150lbs, 5) General: comfortable Clear, no wheeze. ? Crackles at bases bilaterally Neck: supple, no masses, neck nodes not palpable CV: RRR No m/r/g No cervical LAD, neck supple No desaturation on exertion

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CXR

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Symptomatic treatment Benadryl Chlorpheniramine

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Return visit Benadryl lets her sleep through the night Still with continued cough She climbed 3 flights of stairs and became SOB but did not desaturate - minimal sats 96% HR 120

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PFT

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CT Scan

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CT read LUNGS: There are multiple nodules in both lungs measuring up to 5 mm. Mosaic attenuation is seen in both lungs most prominent in the lower lobes suggestive of small airways or small vessel disease.

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Labs CBC, Chem 7 wnl ANA, RF negative TTE unremarkable

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Chronic cough

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Just kidding

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VATS biopsy Had bronchoscopy w BAL VATS biopsy for right lung with RML and RLL biopsy Nodule palpated in RML

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Bronchoscopy and VATS results Middle lobe lavage cytology negative Aerobic, anaerobic, fungal and AFB cultures negative RIGHT LOWER LOBE BIOPSY: LUNG PARENCHYMA WITH CONGESTION, HEMORRHAGE AND HEMOSIDERIN LADEN MACROPHAGES. NO TUMOR IDENTIFIED.

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synaptophysin

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chromogranin

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RML biopsy IMMUNOHISTOCHEMICAL STUDIES PERFORMED ON PARAFFIN EMBEDDED TISSUE (BLOCK A2) SHOWS POSITIVE STAINING FOR CHROMOGRANIN, SYNAPTOPHYSINMULTIPLE FOCI OF NEUROENDOCRINE TUMOR, TUMORLETS/ SMALL CARCINOID TUMOR.

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DIPNECH Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia

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Overview of bronchopulmonary neuroendocrine tumors (BP-NET) 4 types Typical carcinoid Atypical Carcinoid Large cell neuroendocrine carcinoma Small cell neuroendocrine carcinoma

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Diffuse Idiopathic Pulmonary Endocrine Cell Hyperplasia (DIPNECH) Preneoplastic Pulmonary tumorlets (