Extrabronchial Primary Pulmonary Myxoid Sarcoma
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CASE REPORT
Extrabronchial Primary Pulmonary Myxoid Sarcoma Truong Giang Nguyen 1 & Van Nam Nguyen 2 & Duc Thang Vu 1 & The Kien Nguyen 2 & Ba Hanh Le 2 & Ngoc Trung Nguyen 2 Received: 17 April 2020 / Accepted: 26 August 2020 # Association of Surgeons of India 2020
Abstract Primary pulmonary myxoid sarcoma (PPMS) is a very rare condition of the lung that has been described recently. Here, we report the case of PPMS in an asymptomatic 30-year-old male patient. Chest CT showed a 45 × 53-mm mass located in the center of the left upper lobe, which was not detected during bronchoscopy. The tumor was safely and completely removed. Due to pathological studies of the tumor showing characteristics of a primary pulmonary myxoid sarcoma, neither adjuvant chemotherapy nor radiotherapy was prescribed postoperatively. The patient remained tumor-free during a follow-up of 4 years. Keywords Primary pulmonary myxoid sarcoma . Lung
Introduction
Case Report
Primary pulmonary myxoid sarcoma (PPMS) was first described by Nicholson A. G in 1999 [1]. This is a rare condition with only 21 PPMS cases reported in the literature so far. Most patients have tumors in their young to middle ages, and females are more affected than males (female/ male: 1.3/1). Primary pulmonary myxoid sarcoma appears on chest CT scan as a mildly and heterogeneously contrastenhanced mass. Therefore, it should be differentially diagnosed with other primary or secondary neoplasms of the lung. The diagnosis of PPMS could be confirmed by pathological studies showing multinodular lesions consisting of bland spindle and epithelioid cells arranged in reticular, corded, and diffuse patterns that are embedded in a prominently myxoid matrix [2]. Primary pulmonary myxoid sarcoma has a low level of malignancy, with only 14.3% of patients having metastases [3]. The prognosis of PPMS is better than other primary pulmonary sarcoma, due to early presentation and late metastasis [3].
A 30-year-old man with no notable medical history presented to our clinic with productive cough. Subsequent chest CT scan showed a hyper-dense mass (45 HU) measuring 45 × 53 mm, soft tissue in the central left upper lobe. No lymph node enlargement was detected. Metastasis screening with CT scan and bone scan showed negative findings. Bronchoscopy could not reveal any endobronchial involvement of the tumor. Further screening for metastasis was negative. Histopathological studies of samples taken from CT-guided percutaneous transthoracic needle biopsy of the tumor showed only necrotic and inflammatory changes. A decision was made to remove the tumor via left upper lobectomy. Intraoperative examination showed a soft tissue tumor in the center of the left upper lobe’s parenchyma. Complete removal of the left upper lobe was achieved, and subsequent immunohistochemistry analysis of the sample showed morphologic features of PPMS, including positive staining with vimentin, and negative staining with TTF-1, CK-7, CK-20, and MUC-1 markers. The postoperative course was uneventful, and the patient was disch
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