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A Rare Case of Pulmonary Lymphangitic Carcinomatosis as Primary Manifestation in a Patient With Metastatic Pancreatic Cancer FREE TO VIEW

Nabamita Bisen, MD; Vikram Bisen, MD; Subhraleena Das, MD; Robert Lenox, MD
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SUNY Upstate Medical University, Syracuse, NY

Chest. 2014;146(4_MeetingAbstracts):612A. doi:10.1378/chest.1995049
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SESSION TITLE: Cancer Case Report Posters I

SESSION TYPE: Affiliate Case Report Poster

PRESENTED ON: Tuesday, October 28, 2014 at 01:30 PM - 02:30 PM

INTRODUCTION: Pulmonary lymphangitic carcinomatosis (PLC) is a rare metastatic lung disease, characterized by infiltration of the pulmonary lymphatic system by tumor cells. We present a case where lymphangitic spread in lungs manifesting as dyspnea and cough, was the primary presentation in a patient with metastatic pancreatic cancer.

CASE PRESENTATION: A 61 y.o. man with atrial fibrillation, diabetes and 35 pack year history of smoking, presented with progressive dyspnea and dry cough for two months. CT scan showed multifocal opacities including ground glass and areas of nodularities and septal thickening. Right thoracoscopic lung biopsy was performed which showed well differentiated metastatic adenocarcinoma with lymphangitic spread. Immunohistochemical studies showed strong CK 7 and CK 19 expression by the tumor cells and weak to moderate CDX-2 expression. There was no expression of napsin, TTF-1, calretinin, WT-1, PAX-8, CK 20, or Ki-19. These were consistent with a pancreatic ductal or biliary tree primary adenocarcinoma. CT abdomen was then performed and showed a complex lesion within pancreatic body/tail concerning for primary neoplasm.

DISCUSSION: Intrathoracic metastasis occur in 30-40% patients with malignant disease, 6-8% have lymphangitic carcinomatosa. Common primary tumors associated with PLC include breast,stomach,lung, pancreas and prostate. Pathogenesis involves spread of tumor cells into the pulmonary lymphatic system or adjacent interstitium leading to thickening of bronchovascular bundles and septa. Desmoplastic reaction due to proliferation of neoplastic cells and lymphatic dilation by tumor secretions cause interstitial thickening. Spread to adjacent parenchyma leads to nodular pattern. Dyspnea and cough is the initial manifestation in most. PFT commonly shows a restrictive pattern and decreased diffusion capacity. Radiologic features include thickening of interlobular septa and peribronchovascular interstitium on CT scan. Biopsy is the gold standard for diagnosis. Prognosis is poor, around 50% patients die within first three months of symptom onset.

CONCLUSIONS: In summary, PLC is a rare but important cause of respiratory compromise, especially when present with characteristic radiologic features.

Reference #1: Bruce DM, Heys SD, Eremin O. Lymphangitis carcinomatosa: a literature review. J R Coll Surg Edinb. 1996 Feb;41(1):7-13

Reference #2: Thomas A, Lenox R. Pulmonary lymphangitic carcinomatosis as a primary manifestation of colon cancer in a young adult. CMAJ. 2008 Aug 12;179(4):338-40

DISCLOSURE: The following authors have nothing to disclose: Nabamita Bisen, Vikram Bisen, Subhraleena Das, Robert Lenox

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