Med Pediatr Oncol 2002;39:55–57 BRIEF REPORT Response to Chemotherapy in a Child With Primary Bronchopulmonary Leiomyosarcoma Andrea Ferrari, MD, 1 * Paola Collini, MD, 2 Michela Casanova, MD, 1 Cristina Meazza, MD, 1 Marta Podda, MD, 1 and Elena A. Mazza, MD 1 Primary bronchopulmonary leiomyosarcoma (PBPL) is one of the rarest primary lung tumors in childhood [1,2]. This tumor is believed to arise from pluripotent mesenchymal cells surrounding large bronchi, and could represent a different clinicopathological entity from leio- myosarcomas at other sites. Scanty data are available on its clinical management. Complete surgical excision seems the mainstay of treatment and the role of chemo- therapy is not known. Our experience with PBPL in a child who received chemotherapy on relapse and achieved a complete remission is therefore of interest. He was a 4-year-old boy, who in March 1998 pre- sented with a 3-week history of cough and fever. Chest X-ray films, computed tomography (CT), and a nuclear magnetic resonance (NMR) revealed a sharply-defined, round, dense mass in the hilar region of the upper lobe of the right lung, with a maximum diameter of 2.5 cm and contrast enhancement. After partial surgical resection of the right paramediastinal mass elsewhere, the child was referred to our institution and a histopathological dia- gnosis of PBPL of childhood was made. Microscopically, the neoplasm was composed of a proliferation of spindle cells with bland, slender nuclei with fine chromatin, and small nucleoli; only sparse mitoses were evident and necrosis was absent (Fig. 1). The neoplasm was immu- noreactive for smooth-muscle-alpha-actin (1A4), desmin, and calponin, whereas S100 protein was negative. The proliferation index, as assessed by MIB1 immunocyto- chemistry, was low. The tumor extended to the surgical margins; re-operation confirmed residual neoplastic disease, but the surgical margins were not evaluable because the tumor fragmented during excision. Three excised paratracheal lymph nodes were unremarkable. The boy remained disease-free until February 2000, when he suffered a local relapse with a pericaval contrast- enhancing lesion of 20 mm  15 mm adhering to the vena cava. Chemotherapy was administered with the VAIA regimen used by the Italian Soft Tissue Sarcoma Cooperative Group (RMS’96 protocol), i.e., vincristine 1.5 mg/m 2 intravenously (i.v.), weeks 1–7; dactinomycin 1.5 mg/m 2 i.v., weeks 1 and 7; ifosfamide 2 g/m 2 i.v. for 2 days, weeks 1, 4, 7; doxorubicin 40 mg/m 2 i.v. for 2 days, week 4; for a total of 27 weeks. After three cycles of chemotherapy, NMR showed only an enhanced linear signal in T1-weighted scans, consistent with minimal residual disease or fibrous scarring. In June 2000, the boy underwent re-sternotomy: no lesion was identifiable and biopsies of the vena cava wall and of paratracheal lymph nodes were taken. Histologic evaluation showed no evidence of disease. Nine cycles of chemotherapy were concluded and thereafter radiotherapy was delivered to the right hilar and paramediastinal regions at a dose of 44.8 Gy with hyperfractionation of 160 cGy twice daily. No relevant acute treatment toxicity was observed. At latest follow-up in June 2001, the patient was alive and disease-free, 34 months after diagnosis and 16 months after relapse. DISCUSSION PBPL is a very unusual cancer in childhood, with only 13 cases younger than 16 years reported in the English- speaking literature, since it was first descripted in 1950 by Holinger et al. [2–12]. Childhood PBPL probably ought to be distinguished from adult bronchopulmonary leiomyosarcoma and from childhood leiomyosarcomas of other sites, that is characterized by local aggressiveness, a propensity for Key words: primary bronchopulmonary leiomyosarcoma; chemotherapy; pediatric soft tissue sarcoma —————— 1 Pediatric Oncology Units, Istituto Nazionale Tumori, Milano, Italy 2 Pediatric Pathology Units, Istituto Nazionale Tumori, Milano, Italy *Correspondence to: Andrea Ferrari, MD, Pediatric Oncology Unit, Istituto Nazionale Tumori, via G. Venezian 1-20133, MI, Italy. E-mail: ferrari@istitutotumori.mi.it Received 16 August 2001; Accepted 1 October 2001 ß 2002 Wiley-Liss, Inc. DOI 10.1002/mpo.10041