Malignant Mesenchymoma Associated with a Congenital Lung Cyst in a Child: Case Report and Review of the Literature

Abstract

Primary lung tumors are uncommon in children, and malignant mesenchymal tumors form only a small proportion of these. Leiomyosarcomas occur more commonly than rhabdomyosarcomas, whereas malignant mesenchymomas are exceedingly rare. Of the total number of primary pulmonary rhabdomyosarcomas and malignant mesenchymomas of lung reported in children, 50% have occurred in association with congenital lung cysts. The relationship between abnormal morphogenesis and neoplasia is well documented in the kidney. A similar relationship may exist in the lung between cystic parenchymal maldevelopment and embryonal mesenchymal tumors. We report a 4-year-old boy with a malignant mesenchymoma of lung arising within a congenital lung cyst; one similar case has previously been reported to our knowledge.

Perinatal management of congenital cystic lung lesions in the age of minimally invasive surgery

“…Of note, all patients in their series underwent an open procedure. More recent reports of malignant transformation (the youngest, a 22-month-old child [9]) have further prompted discussion regarding preemptive excision of asymptomatic lesions [9][10][11][12]. Although it is generally accepted that the risk of malignant transformation exists only with CCAM (albeit still a very uncommon event), it has become increasingly clear that neither prenatal nor postnatal imaging can accurately determine the exact nature of the lesion (Fig.…”

Postnatal spontaneous resolution of congenital cystic adenomatoid malformations

“…With respect to infants with persistence of CCAMs at birth, operation is generally planned depending on symptoms. Removal of a CCAM in the asymptomatic child is indicated because of the risk for infection and malignancy in persistent lesions [5][6][7][8][9][10]. Symptomatic children have earlier intervention, with those who are asymptomatic often coming to operation between ages of 6 and 9 months.…”

Pleuropulmonary blastoma

“…Intrathoracic malignant mesenchymoma (IMM) of childhood is a polyphenotypic tumor which closely simulates PPB. A comparison of the 2 entities from published reports suggests that IMM and PPB probably represent the same entity [4]. Both are pleomorphic tumors with a complex multidirectional growth pattern with the same spectrum of clinical presentation and biological behavior.…”