Giant primary low‐grade fibromyxoid sarcoma arising from the left pulmonary parenchyma: A case report and literature review

Abstract

Background: Low-grade fibromyxoid sarcoma is a rare painless neoplasm that primarily grows in young adults' proximal extremities and trunks. The lungs are infrequent sites for this type of sarcoma.Case Presentation: We reported a 26-year-old female that presented with a chief complaint of chest pain from a few months ago to Kasra hospital, Tehran, Iran, in August 2021. Chest computed tomography (CT) showed a hypodense mass with a well-defined margin measuring 9.3 cm in the left upper lobe and multiple hypodense lesions with a lobulated appearance with a total diameter of 15.5 Â 13.5 cm in the left lower lobe of the lung. Conclusion:This is the largest case of primary pulmonary low-grade fibromyxoid sarcoma (30 Â 28 Â 7 cm), which seemed unresectable at first evaluation. Due to the extent of the tumor, left pneumonectomy was performed, leading to attenuation of symptoms and no recurrence at a six-month follow-up.

Thoracoscopic Wedge Resection for Low-Grade Fibromyxoid Sarcoma (Evans Tumor) with Massive Calcification and Originating from the Lung: A Rare Case in an Unexpected Location

“… 2) The previous case reports did not observe a recurrence of LGFMS originating from the lung, although the follow-up periods were unknown or short. 4 – 8) In our case, there were no signs of recurrence on CT during the six-month follow-up. Notably, we need to pay particular attention to the lung dissection line because we performed a wedge resection.…”

“…The treatment for all the previously reported cases of LGFMS originating from the lung was anatomical resection, including lobectomy and pneumonectomy. 4 – 8) In our case, although the tumor had a diameter of 8.0 cm, it was pedunculated and had no evidence of invasion into the surrounding organs. Therefore, we were able to remove the tumor with enough margins by wedge resection.…”

Case report: Primary pleural low-grade fibromyxoid sarcoma in a 4-year-old boy with molecular confirmation

“…Furthermore, primary intrathoracic LGFMS is also very rare, and only 27 cases have been reported before (including the current case) ( Table 1 ) ( 7 – 9 , 22 – 42 ). The historical intrathoracic cases were composed of 13 male and 14 female patients, aged 4 to 77 years old (median 35 years).…”

“…The morphologic results showed that 9 (9/26, 34.6%) cases were classic LGFMS and 13 (13/26, 50.0%) cases were LGFMS with a collagen rosette structure. In addition, 4 (4/26, 15.4%) cases had unusual features, consisting of 1 case with collagen rosettes and epithelioid component, 1 case with collagen rosettes and SEF-like areas, 1 lesion with round and epithelioid cell areas, and 1 case with classic morphology in the primary tumor and pleomorphic areas in the recurrent tumor ( 22 , 29 , 37 , 42 ). The frequency of these unusual features in the intrathoracic LGFMS (15.4%) was slightly higher than that of the overall LGFMS cases (<10%) ( 1 ).…”

“…Surgical resection is the main therapy for LGFMS, and our case showed no evidence of the disease after 11 months of the surgery. In 27 previously reported intrathoracic LGFMS cases, follow-up information was available in 16 cases who received surgical resection (including the current case), with a median follow-up of 23 months (range 6–110 months) ( 7 , 22 , 26 , 30 , 32 – 34 , 38 , 40 – 42 ). Three patients (3/16, 18.8%) developed recurrence, and none of the patients developed metastasis.…”