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Simultaneous Mantle Cell Lymphoma and Undifferentiated Carcinoma Manifested by Pleural Studding and Effusion FREE TO VIEW

Andrey Zinchuk, MD; Jussi Saukkonen, MD; Valia Boosalis, MD; Parbati Brahma, MD
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Yale University, New Haven, CT

Chest. 2013;144(4_MeetingAbstracts):597A. doi:10.1378/chest.1702019
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SESSION TITLE: Cancer Case Report Posters I

SESSION TYPE: Affiliate Case Report Poster

PRESENTED ON: Tuesday, October 29, 2013 at 01:30 PM - 02:30 PM

INTRODUCTION: Synchronous malignancies affecting the pleural space are rare. We encourage reassessment in cases that do not follow the anticipated course.

CASE PRESENTATION: 71 year old man with extensive tobacco abuse, but no history of asbestos exposure, presented with dyspnea on exertion and right scapular pain. Signs of effusion over the majority of the right lung were the only physical finding. An isolated peripheral blood polymorphonuclear predominant leukocytosis (14,000) was found. Chest CT revealed a large right pleural effusion with atelectasis, thickened, nodular pleura as well as few < 4mm left lung nodules, but no lymphadenopathy. PET-CT showed FDG uptake isolated to the entire right pleura. Pleural fluid on thoracentesis was bloody and exudative. Pleural fluid cytology demonstrated CD5+, CD10- and CD20+ B-cell clonopathy, while flow cytometry showed kappa restricted, CD5+, CD23-, CD10- cells consistent with mantle cell lymphoma (MCL). CT of the abdomen and pelvis and a bone marrow biopsy showed no evidence of lymphoma. Flow cytometry of peripheral blood and bone marrow were consistent with MCL. Bronchoscopy and repeat pleural fluid cytology were unremarkable. Initiation of rituximab, cyclophosphamide, doxorubicin, vincristine and prednisone (R-CHOP) did not elicit an expected response and the patient deteriorated. Fine needle aspiration of the pleura revealed CK 7 positive and LCA, Calretinin, Cytokeratin HMW, p63, CK20, CEA, BerEp and TTF-1 negative cells, consistent with undifferentiated carcinoma, likely of lung origin. Patient elected comfort-based palliative treatment and died within several days.

DISCUSSION: Extranodal MCL with isolated effusion involvement is uncommon (case reports). As in our patient, MCL is often diagnosed at the advanced stage (III-IV). It is moderately aggressive (median survival 3-4 years) and generally responsive to R-CHOP. Although, extranodal MCL presenting synchronously with another malignancy is exceedingly rare (most often associated with gastrointestinal adenocarcinoma) lack of expected response to treatment warrants further diagnostic exploration. It is plausible that the undifferentiated, probable lung carcinoma-related pleural disease rather than the MCL was responsible for our patient’s clinical course.

CONCLUSIONS: Synchronous extranodal MCL and an undifferentiated carcinoma can rarely present with isolated pleural involvement. Clinical suspicion, judicious biopsies and careful cytological evaluation at clinical junctures are essential for diagnosis.

Reference #1: Hatzibougias et al., A rare tumoral combination, synchronous lung adenocarcinoma and mantle cell lymphoma of the pleura. World J Surg Oncol. 2008 Dec 29;6:137.

Reference #2: Pérez-Galán et al. Mantle cell lymphoma: biology, pathogenesis, and the molecular basis of treatment in the genomic era. Blood. 2011;117(1):26-38.

DISCLOSURE: The following authors have nothing to disclose: Andrey Zinchuk, Jussi Saukkonen, Valia Boosalis, Parbati Brahma

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