title: Pulmonary extraskeletal myxoid chondrosarcoma: A case report and literature review.
abstract: BACKGROUND
  Extraskeletal myxoid chondrosarcoma (EMC) accounts for the 3% of all soft tissue sarcomas and it's categorized as a tumour of uncertain differentiation. This entity has shown to have the recurrent balanced chromosomal translocation t(9;22) (q22;q12.2), which leads to the oncogenic fusion gene EWSR1-NR4A3. This sarcoma usually presents as a slow growing, palpable mass in the extremities. EMC arising from the lung is extremely infrequent. We report one case of pulmonary extraskeletal mixoid chondrosarcoma and a review of the world literature.
  
  
  METHODS
  A 69-year-old male patient presented with intermittent hemoptysis for the last 6 months. A PET/CT scan showed a hypermetabolic solid mass with lobulated borders of approximately 29×26mm in the inferior right lobe. We performed a right thoracotomy with inferior lobectomy and lymphadenectomy of levels VII, VIII, X, and XI levels. The neoplasm was constituted by cords of small cells with small round nucleus and scarce cytoplasm immerse in an abundant myxoid matrix. The immunophenotype was positive for MUM-1, CDK4, MDM2, and showed focal expression for S-100 protein and CD56. The final pathology report revealed a pulmonary extraskeletal mixoid chondrosarcoma. No further surgical interventions or adjuvant therapies were needed.
  
  
  CONCLUSIONS
  EMC is an intermediate-grade neoplasm, characterized by a long clinical course with high potential for local recurrence and distant metastasis. Treatment for EMC is surgical and non-surgical treatment is reserved for recurrence or metastatic disease. Pulmonary extraskeletal myxoid chondrosarcoma is a rare neoplasm with only isolated case reports found in the literature.
authors: General Surgery Resident, Hospital Ángeles Lomas, Av. Vialidad de la Barranca s/n, Col. Valle de las Palmas Huixquilucan, Estado de México, C.P. 52763, Mexico.
authors: Ricardo
authors: R
authors: Balanzá
authors: Department of Surgery, Hospital Ángeles Lomas, Av. Vialidad de la Barranca s/n, Col. Valle de las Palmas Huixquilucan, Estado de México, C.P. 52763, Mexico; Department of Surgery, Centro Médico ABC, Av. Carlos Graef Fernández 154, Col. Tlaxala, Del. Cuajimalpa de Morelos, México Distrito Federal, C.P. 05300, Mexico. Electronic address: rodrigo.arrangoiz@gmail.com.
authors: Rodrigo
authors: R
authors: Arrangoiz
authors: Department of Surgery, Hospital Ángeles Lomas, Av. Vialidad de la Barranca s/n, Col. Valle de las Palmas Huixquilucan, Estado de México, C.P. 52763, Mexico; Department of Surgery, Centro Médico ABC, Av. Carlos Graef Fernández 154, Col. Tlaxala, Del. Cuajimalpa de Morelos, México Distrito Federal, C.P. 05300, Mexico.
authors: Fernando
authors: F
authors: Cordera
authors: Department of Surgery, Centro Médico ABC, Av. Carlos Graef Fernández 154, Col. Tlaxala, Del. Cuajimalpa de Morelos, México Distrito Federal, C.P. 05300, Mexico.
authors: Manuel
authors: M
authors: Muñoz
authors: Department of Surgery, Centro Médico ABC, Av. Carlos Graef Fernández 154, Col. Tlaxala, Del. Cuajimalpa de Morelos, México Distrito Federal, C.P. 05300, Mexico.
authors: Enrique
authors: E
authors: Luque-de-León
authors: Department of Surgery, Centro Médico ABC, Av. Carlos Graef Fernández 154, Col. Tlaxala, Del. Cuajimalpa de Morelos, México Distrito Federal, C.P. 05300, Mexico.
authors: Eduardo
authors: E
authors: Moreno
authors: Department of Pathology, Hospital Ángeles Lomas, Av. Vialidad de la Barranca s/n, Col. Valle de las Palmas Huixquilucan, Estado de México, C.P. 52763, Mexico.
authors: Lourdes
authors: L
authors: Molinar
authors: Universidad Anáhuac Norte, Av. Universidad Anáhuac 46, Col. Lomas Anáhuac, Huixquilucan, Estado de México, C.P. 52786, Mexico.
authors: Nicole
authors: N
authors: Somerville
keywords: Extraskeletal myxoid chondrosarcoma; Fusion gene EWSR1-NR4A3; Lung cancer; Lung neoplasm; Pulmonary lobectomy