Key result
Surgical segmentectomy and adjuvant chemotherapy resulted in a 9-year-old female with primary pulmonary rhabdomyosarcoma remaining disease-free after 6 years of surveillance.
Case Report (n=1)
This case report highlights the successful management of a rare primary pulmonary rhabdomyosarcoma in a child using segmentectomy and adjuvant chemotherapy, resulting in long-term disease-free survival.
Supports segmentectomy plus adjuvant chemotherapy as feasible in rare pediatric pulmonary rhabdomyosarcoma; hypothesis-generating pending larger cohorts.
A 9-year-old female presented with fever, cough, and hemoptysis for a week. The chest skiagram and contrast-enhanced computerized tomography delineated a well-defined solid lesion localized to the superior segment of the right lower lobe with features of a congenital pulmonary airway malformation. The lesion was surgically managed with a segmentectomy and histopathology confirmed a contained pulmonary rhabdomyosarcoma (RMS). No other primary site of origin was evident, and a final diagnosis of "primary" pulmonary RMS was made. She received adjuvant chemotherapy and was disease free after 6 years of surveillance. The unique clinicoradiological features of the case are discussed and the sparse literature is reviewed.
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Das et al. (2016) conducted a case report in Primary pulmonary rhabdomyosarcoma (n=1). Segmentectomy and adjuvant chemotherapy was evaluated on Disease-free survival. Surgical segmentectomy and adjuvant chemotherapy resulted in a 9-year-old female with primary pulmonary rhabdomyosarcoma remaining disease-free after 6 years of surveillance.
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