Lung Cancer |

An Extremely Rare Case of a Locally Invasive Primary Pleomorphic Sarcoma of the Lung FREE TO VIEW

Ameer Rasheed, MD; Viswanath Vasudevan, MD; Qammar Abbas, MD
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The Brooklyn Hospital Center, Brooklyn, NY

Chest. 2014;146(4_MeetingAbstracts):628A. doi:10.1378/chest.1994072
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SESSION TYPE: Affiliate Case Report Slide

PRESENTED ON: Wednesday, October 29, 2014 at 11:00 AM - 12:15 PM

INTRODUCTION: Malignant fibrous histiocytoma (MFH) is a common soft tissue sarcoma in adult, arising frequently in the extremity, retroperitoneum and trunk. Metastasis to the lung as secondary MFH is not uncommon but primary MFH of the lung is an extreme rarity. It usually presents as a small intrabronchial lesion or a peripheral nodule and tends to have early lymphatic spread. In our case it presented as a large lung mass. This is unique presentation of an extremity rare disease.

CASE PRESENTATION: An 86 years old male with a 42-pack-year smoking history presented with right sided chest pain, shortness of breath and weight loss. Physical examination revealed decreased aeration to right lower lung zone. Chest X-ray showed RLL mass. CT chest revealed a large mass (10x8cm) in RLL impinging on RML bronchus. PET/CT did not reveal any evidence of metastasis. CT guided needle biopsy demonstrated tumor cell that contained large vesicular to pleomorphic nuclei and moderate amount of eosinophilic cytoplasm consistent with undifferentiated pleomorphic carcinoma. Immunostaining was consistent with pleopmorphic sarcoma. A 92-gene expression molecular analysis confirned MFH subtype. He underwent RLL and RML lobectomy, had tumor free margins and is currently tumor free.

DISCUSSION: Primary Sarcoma of the lung is very rare. The microscopic features of lung sarcoma are similar to other soft tissue sarcoma. Immunohistochemical stain is required for the precise diagnosis. Gene analysis can be done for subtype analysis including MFH, osteosarcoma, synovial sarcoma, liposarcoma, leiomyosarcoma, and primitive neuroactodermal. Our patient had findings consistent with MFH. Early lymphatic spread and systemic metastases at presentation are common. MFH is highly aggressive and recur locally after excision or may recur distantly through lymphatic spread. Surgical resection is the most important therapy. Adjuvant chemoradiation is controversial. Metastases may appear years after resection of the primary lesion.

CONCLUSIONS: MFH of the lung is a substype of primary lung sarcoma. It lacks detailed guidance about treatment and prognosis. It is highly malignant sarcoma and has guarded prognosis. The main effective treatment is surgical resection, yet the role of postoperative adjuvant chemotherapy in the whole course of treatment is undefined.

Reference #1: da Matta Abreu M1, Marcondes Kozlowski B, de Biasi Cordeiro P, de Sousa A, F Nascimento M, Zamboni M. Primary pleomorphic sarcoma of the lung. Rev Port Pneumol. 2008 Jul-Aug;14(4):535-9.

DISCLOSURE: The following authors have nothing to disclose: Ameer Rasheed, Viswanath Vasudevan, Qammar Abbas

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