Leiomyosarcoma of soft tissue in children: clinicopathologic analysis of 20 cases.

Abstract

Leiomyosarcoma in the pediatric age group is uncommon and incompletely characterized. A series of 20 primary leiomyosarcomas of soft tissue occurring in children younger than 16 years is presented. No significant gender predilection was observed (11 girls and 9 boys). Patient age ranged from 4 to 15 years (median, 12 years). Tumor size ranged from 0.5 to 13 cm (median, 2.5 cm); subcutaneous and deep locations were equally represented. Tumors were evenly distributed among the trunk (30%), head and neck (25%), lower limbs (25%), and upper limbs (20%). All lesions showed at least focally typical features of smooth muscle differentiation, principally in the form of fascicles of eosinophilic spindle cells with cigar-shaped nuclei. An unusual whorled growth pattern was seen in two cases. Morphologic variants including inflammatory leiomyosarcoma (one case), granular cell leiomyosarcoma (two cases), giant-cell rich leiomyosarcoma (two cases), and epithelioid leiomyosarcoma (one case) were seen. Dystrophic calcifications were present in two cases. Most lesions (85%) were low grade. Immunohistochemical staining showed positivity for alpha-smooth muscle actin in 89% of the cases, HHF-35 in 87%, and desmin in 61%. Positivity for cytokeratins, observed in 6 (43%) of 14 cases tested, was usually strong and was diffuse in two cases. Follow-up data, available in 15 (75%) patients (median duration, 49 months), showed late local recurrence in only two cases, one with progression to a higher grade lesion, and no metastasis. These results show that, although extremely rare, soft-tissue leiomyosarcomas do occur in children, in whom they usually present as small morphologically low-grade lesions that seem to behave in a relatively indolent fashion, although longer follow-up data are needed. Differential diagnosis in this setting includes infantile myofibromatosis, leiomyoma, monophasic synovial sarcoma, and spindle cell rhabdomyosarcoma.