Primary leiomyosarcoma of the lung in a girl

Abstract

Leiomyosarcoma of the lung is a type of tumour rarely found in childhood. It arises from smooth muscle either of bronchial or arterial walls, has a variable pattern of local growth, blood-borne metastatic spread with lymph nodes sparing and a clinical course characterized by fever, cough and worsening dyspnea. We report a case which occurred in a 14-year-old girl, reviewing all the other cases found in the literature.Primary leiomyosarcoma of the lung is a malignant tumour occurring rarely both in children [2-5, 6, 8, 9, 11] and adults [1, 2, 7, 10].The tumour arises from smooth muscle either of the bronchial or arterial walls and is characterised by a variable pattern of local growth and blood-borne metastatic spread, while lymphatics and lymph nodes are usually spared [2,7,10].

Response to chemotherapy in a child with primary bronchopulmonary leiomyosarcoma

“…In two cases, response to radiation was not evaluable due to the death of the patients, in one there was no significant disease control [9]. Chemotherapy was given to 2 children: 1, without measurable disease, was the child who died of pulmonary hemorrhage [11]; no data on tumor mass reduction was given in the other, but the patient died of disease 16 months after diagnosis [10].…”

“…Six of the seven patients with complete resection were alive in first complete remission, having been treated with surgery alone [2][3][4][6][7][8]12]. Seven patients died: 1 shortly after birth [8], 3 of disease progression (with the onset of distant metastases in 2 cases) [9,10,12], 3 of treatmentrelated causes (1 intraoperative death due to cardiac arrest [5], 1 respiratory failure due to pulmonary hemorrhage secondary to thrombocytopenia and viral pneumonia [11], and 1 due to anemia [3]). …”

“…Tumor excision was defined as complete in seven patients and incomplete in one patient with pericardial invasiveness [11]. Two patients only underwent biopsy because the tumor was unresectable [3,10], while the diagnosis was made at autopsy in two others: a newborn dying shortly after birth [8] and an intraoperative death due to cardiac arrest [5].…”

Leiomyosarcoma of the main bronchus in a girl: A long‐time survivor with multiple lung metastases

“…Since 1950, only 15 pediatric cases of leiomyosarcoma of the respiratory tract have been reported in the English-language literature ( Table 1). [1][2][3][4][5][6][7][8][9][10][11][12][13] These reports documented the disease in 9 males and 6 females, with ages ranging from newborn to 14 years. Clinical symptoms are characterized by cough, fever, wheezing, chest pain, and increasing respiratory difficulty leading to dyspnea.…”

Primary Endobronchial Leiomyosarcoma

“…Fewer than 100 cases have been reported [4, 5, 6]. Among the primary pulmonary leiomyosarcoma, the tumor that presented as a single mass in the endobronchial lumen is very rare.…”