Histiocytic Sarcoma in a 3-Year-Old Male: A Case Report

Abstract

ABSTRACT. We describe a pediatric patient with histiocytic sarcoma involving the T6 and L4 vertebral bodies and the lungs. His tumor progressed during chemotherapy designed for Langerhans' cell histiocytosis and sarcoma. High-dose radiation, on the other hand, was effective. Pediatrics 2005;116:e322-e325. URL: www. pediatrics.org/cgi/doi/10.1542/peds.2005-0026; sarcoma, histiocytes, Langerhans' cell histiocytosis, histiocytic sarcoma.ABBREVIATIONS. LCH, Langerhans' cell histiocytosis; CT, computed tomography; 2CdA, 2-chlorodeoxyadenosine.H istiocytic and dendritic neoplasms are rare, especially in children. These tumors arise from antigen-processing phagocytes (histiocytes) and antigen-presenting dendritic cells. The cell types are derived from hematopoietic or mesenchymal stem cells. 1 Currently, the World Health Organization includes the following 6 entities under this designation: Langerhans' cell histiocytosis (LCH), Langerhans' cell sarcoma, follicular dendritic cell sarcoma, interdigitating dendritic cell sarcoma, dendritic cell sarcoma not otherwise specified, and histiocytic sarcoma. 2 The latter malignancy is a proliferation of histiocytes (tissue macrophages), which are characterized by positive expression of the macrophage-associated antigen CD68, negative expression of the T-cell-associated antigen CD1a, negative expression of the dendritic cell-associated antigens CD21/CD35, and lack of Birbeck granules on electron microscopy. 3 The rarity of histiocytic sarcoma continues to make its management challenging, and we are unaware of any recommended therapy for young children. We describe a child with histiocytic sarcoma to highlight his poor response to LCH-and sarcoma-based chemotherapy. By contrast, high-dose radiation proved to be effective. CASE REPORTThis previously healthy 3-year-old boy experienced intermittent low back pain radiating to the right inguinal region for ϳ2 months. His symptoms initially responded to ibuprofen. The pain intensity increased over a 2-week period, and he refused to walk. Review of systems was significant for pain with urination. With the exception of being unable to stand, his physical examination was unremarkable. The laboratory tests showed normal blood counts and normal liver and renal function. An MRI showed collapse of the T6 and L4 vertebral bodies and a soft tissue mass in the anterior epidural space at the level of L4 (Fig 1 A and B). The chest and abdominal computed tomography (CT) scans were normal. Bone marrow aspiration revealed no malignant infiltration. A technetium bone scan showed increased uptake limited to the T6 and L4 regions. CT-scan-guided needle biopsy of the L4 mass revealed infiltrative proliferation of the bone and soft tissue by sheets and clusters of large ovoid cells with abundant eosinophilic cytoplasm (Fig 2A). The nuclei were round/oval, eccentric, and pleomorphic with occasional grooving. Distinct, small nucleoli were present. Multinucleated giant cells were also present ( Fig 2B). Immunohistochemistry showed expression of CD68 (macroph...

Pulmonary histiocytic sarcoma mimicking pulmonary Langerhans cell histiocytosis in a young adult presenting with spontaneous pneumothorax: a potential diagnostic pitfall

“…In case of a multiple involvement of different sites, this systemic presentation is referred to as "malignant histiocytosis" [3]. HS arising in the lung has rarely been reported; indeed, only few well-documented reports of cases in humans exist [4,5]. According to the WHO, HS should be regarded as an aggressive haematopoetic neoplasm with a poor response to therapy being reflected by the study of Vos et al [6] who reported five cases of HS.…”

Sarcoma histiocítico de intestino delgado: Reporte de un caso y revisión de la literatura

“…Se ha ubicado en diferentes sitios extraganglionares, que incluyen piel, pulmón, cavidad nasal, paladar, hueso, tracto gastrointestinal, bazo, hígado, tejidos blandos y sistema nervioso central [6][7][8][9][10][11][12] . El presente caso se suma a los previamente descritos en el tracto gastrointestinal, que incluyen localizaciones en el esófago 13 , estóma-go 14 , intestino delgado [6][7][8][15][16][17] , colon 8,9 , recto y ano 6 .…”

Histiocytic Sarcoma Originating in the Lung in a 16-Year-Old Male

“…Only 3 cases of HS originating in the lung have previously been reported. 2,5,6 We encountered a young patient with HS of the lung containing spindle cells, mainly, and foam cells.…”