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Primary Pulmonary Plasmacytoma: Unusual Presentation With Coexisting Amyloidosis and Bone Metaplasia FREE TO VIEW

Sravanthi Nandavaram, MBBS; Naveen Gnanabakthan, MD; Dinesh John, MD
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SUNY Upstate Medical University, Syracuse, NY

Chest. 2014;146(4_MeetingAbstracts):661A. doi:10.1378/chest.1989411
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SESSION TITLE: Cancer Student/Resident Case Report Posters II

SESSION TYPE: Medical Student/Resident Case Report

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

INTRODUCTION: Primary pulmonary plasmacytoma is a rare soft tissue extramedullary plasmacytoma which can present as a nodule or mass in the hilum. Here we present a case of plasmacytoma with co existing amyloidosis and metaplastic bone.

CASE PRESENTATION: A 48 year old male with chronic cough was referred to pulmonary clinic for a chest x ray with right hilar abnormality. CT Thorax revealed 2.1 X 3.0 cm mass in the right hilum and sub-carinal adenopathy. Subsequent bronchoscopy showed definite extrinsic mass or compression at sub carinal level and posterior aspect of RML bronchus. Transbronchial needle aspiraton of sub carinal and right hilar mass was pursued. However RML endobronchial biopsies could not be done due to excessive coughing despite using high doses of sedatives. Histopathology of sub carinal, right hilar mass and bronchial brushings revealed benign cellular elements. Follow up CT showed enlarged right hilar mass. In the light of positive PET scan, and enlarging mass, bronchoscopy was deemed to be non diagnostic and cervical mediastenoscopy and bronchoscopy was done, several R4 and sub carinal lymph nodes were identified and biopsied. Pathology revealed plasma cells with positive amyloid deposition and metaplastic bone. Immunohistochemical stains revealed kappa immunoreactivity and Congo red stain revealed apple green birefringence. Peripheral blood smear was normal. SPEP revealed small paraprotein (0.22%), identified as Ig G Kappa. Plasma cells constituted 1-2 % of the bone marrow aspirate. Patient was successfully treated with radiation therapy.

DISCUSSION: Soft tissue extramedullary plasmacytoma (SEP) constitute 3% of all plasma cell neoplasms. Most common site of being the mucous membranes of upper airways. Lung involvement is rare and can be asymptomatic or may present with cough or dyspnea. Diagnostic criteria includes: plasma infiltration on biopsy, bone marrow plasma cell infiltration not exceeding 5%, absence of osteolytic bone lesions, absence of hypercalcemia or renal failure. SEP can be associated with amyloid deposition and bone metaplasia and are related to presence of kappa light chain in both amyloid deposits and plasma cells . Metaplasia of fibrous tissue in the presence of amyloid can result in bone formation. Plasma cell tumors are radiosensitive and respond well to radiotherapy. Chemotherapy is indicated in refractory cases or with osteolytic bone lesions.

CONCLUSIONS: Given the excellent prognosis on treatment ,a thorough evaluation to exclude systemic involvement and a close follow-up is required as there is high risk of development of plasma cell dyscrasias.

Reference #1: Primary Pulmonary Plasmacytoma G Shaikh, R Sehgal, A Mehrishi,A Karnik

Reference #2: Primary Pulmonary Plasmacytoma Presenting as Multiple Lung Nodules S Kim, T Kim, J Sohn, H Yoon, D Shin, I Kim,S Park

Reference #3: Unusual Presentation Of Pulmonary Plasmacytoma And Amyloidoma In A Patient With Multiple Myeloma M. Tabassomi, E. Almasri, H. Abdulhaq

DISCLOSURE: The following authors have nothing to disclose: Sravanthi Nandavaram, Naveen Gnanabakthan, Dinesh John

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