Massive low-grade fibromyxoid sarcoma presenting as acute respiratory distress in a 12-year-old girl

Abstract

Low-grade fibromyxoid sarcoma (LGFMS) is a rare soft-tissue sarcoma that usually presents in young adults as a painless, slow-growing mass. Evans first described LGFMS in 1987 as a spindle-cell sarcoma with bland histological features and paradoxically aggressive behavior. Although young adults are most frequently affected, recent reports describe pediatric cases being increasingly more common. Males and females are affected approximately equally and common locations include the deep soft tissue of the lower extremity, particularly the thigh and trunk. Primary occurrence within the chest cavity is exceedingly rare. Local recurrence and metastasis are not uncommon and present the clinician and radiologist with challenges regarding follow-up recommendations. Review of the literature reveals many cases of slowly progressive symptoms related to a mass effect. We present a healthy 12-year-old African-American girl who interestingly developed acute symptoms of shortness of breath and chest pain while playing with her brother.

Imaging findings of thoracic low-grade fibromyxoid sarcoma: report of three cases

“…[5][6][7][8][9][10] For our fi rst two cases, in which CT studies alone were performed, strongly enhancing areas correlated with the hypercellular zone, and weakly enhancing areas correlated with the myxoid zone with less cellularity, or hyalinization. These correlations appear to contradict those of previous reports, but the well-enhancing hypercellular zone in our case may be more abundant in free water within the fi brous stroma, or poorly enhancing myxoid areas may be more abundant in myxoid stroma, and the cellular or vascular densities were sparse.…”

An Intrathoracic Low-Grade Fibromyxoid Sarcoma Arising from the Chest Wall with Massive Pleural Effusion

“…3,4) Steiner, et al have reported a case of a 12-year-old girl who developed a huge LGFMS that occupied the entire hemithorax with acute respiratory distress. 4) Following surgery in that case, the mass was revealed to be attached to the posterior lateral chest wall, but a combined chest wall resection was not performed and the tumor margin was positive. In contrast, Higuchi, et al reported a case of an intrathoracic LGFMS which was successfully treated using a combined chest wall resection.…”

Low grade fibromyxoid sarcoma: a case report and review of the literature

“…LGFMS usually presents as a painless soft-tissue mass with a pre-biopsy duration of over 5 years in 15% of patients [2]. In rare occasions acute presentations of the disease may occur, such as acute respiratory distress and chest pain in case of chest wall LGFMS or seizure activity in a patient with intracranial LGFMS [3,18]. …”

“…These tumours usually occur in the proximal extremities and trunk [2]. Sporadically they may be found in unusual locations, such as the retroperitoneum, head or the chest wall [2,3]. The majority of LGFMS occur in a subfascial location, but in rare occasions the subcutis or dermis may be affected [4].…”

“…The majority of LGFMS occur in a subfascial location, but in rare occasions the subcutis or dermis may be affected [4]. LGFMS typically involves young or middle-aged adults, but a large number of paediatric cases has also been described [3,5-9]. …”