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Malignant Fibrous Histiocytoma of the Lung In a Patient With SLE: A Common Soft-Tissue Sarcoma in an Uncommon Site FREE TO VIEW

Jacob Mathew, MD; Raminderjit Sekhon, MD; Zeron Ghazarian, MD; M Khan, MD
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St. Joseph's Regional Medical Center / Seton Hall University, Paterson, NJ

Chest. 2015;148(4_MeetingAbstracts):526A. doi:10.1378/chest.2280001
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SESSION TITLE: Lung Cancer Case Report Posters II

SESSION TYPE: Affiliate Case Report Poster

PRESENTED ON: Tuesday, October 27, 2015 at 01:30 PM - 02:30 PM

INTRODUCTION: Malignant fibrous histiocytoma (MFH) is one of the most common soft-tissue sarcomas in adults. Metastases are known to occur,with the lungs being a common site. Primary MFH of the lung,however, is extremely rare.

CASE PRESENTATION: We present a 71 y/o African American male with past medical history of SLE, history of renal cell carcinoma s/p partial nephrectomy,COPD,iron deficiency anemia,history of alcohol/polysubstance abuse,and CAD/PVD who presented with shortness of breath. CT chest identified a right sided pleural effusion and a large mediastinal mass in the right paratracheal/subcarinal region measuring 10.9 x 4.2 cm causing severe compression of the SVC and narrowing of the bronchus intermedius. Thoracentesis revealed bloody pleural fluid,but no evidence of malignant cells. A chest tube was placed and pleurodesis with doxycycline performed, with subsequent resolution. Patient also underwent bronchoscopy,which was non-diagnostic,as well as CT guided biopsy which identified the specimen as poorly differentiated malignant neoplasm;later identified as MFH via microRNA analysis. Patient was subsequently arranged for radiotherapy/chemotherapy.

DISCUSSION: MFH has been classically described as a tumor rich in histiocytes with a storiform growth pattern. Histologically, there is a broad range with four subtypes: storiform-pleomorphic,myxoid,giant cell,and inflammatory. Associations with other disease states exist. SLE has been associated with sarcomas due to its paraneoplastic nature. Despite the above,the origin remains unknown. Favorable prognostic factors include: age <60 years,tumor size <5 cm,superficial location,low grade,the absence of metastatic disease,and a myxoid subtype. Invasion of the mediastinum/chest wall at initial diagnosis,and recurrence or metastases are poor signs. Surgical resection,when possible, remains the cornerstone,followed by radiation to kill microscopic disease and prevent local recurrence. The utility of chemotherapy is uncertain,but is often given due to the reported improvement in disease-free survival.

CONCLUSIONS: Primary MFH of the lung is a very rare entity. Despite identified associations and significant research since being first identified in the 1960’s,the origin of this tumor remains unknown. As such,prompt identification and subsequent evaluation for degree of invasion and metastases are critical in initiating best known treatment strategies.

Reference #1: Rossi G, Beluffi G, Ravelli A. Uncommon Syndromes and Treatment Manifestations of Malignancy. JCO 2003; 21(1): 166-167.

DISCLOSURE: The following authors have nothing to disclose: Jacob Mathew, Raminderjit Sekhon, Zeron Ghazarian, M Khan

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