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

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Histiocytic Sarcoma in a 3-Year-Old Male: a Case Report Histiocytic Sarcoma in a 3-Year-Old Male: A Case Report Samuel Buonocore, MD*; Alfredo L. Valente, MD‡; Daniel Nightingale, MD‡; Jeffrey Bogart, MD§; and Abdul-Kader Souid, MD, PhD* ABSTRACT. We describe a pediatric patient with his- CASE REPORT tiocytic sarcoma involving the T6 and L4 vertebral bodies This previously healthy 3-year-old boy experienced intermit- and the lungs. His tumor progressed during chemother- tent low back pain radiating to the right inguinal region for ϳ2 apy designed for Langerhans’ cell histiocytosis and sar- months. His symptoms initially responded to ibuprofen. The pain coma. High-dose radiation, on the other hand, was intensity increased over a 2-week period, and he refused to walk. effective. Pediatrics 2005;116:e322–e325. URL: www. Review of systems was significant for pain with urination. With the exception of being unable to stand, his physical examination pediatrics.org/cgi/doi/10.1542/peds.2005-0026; sarcoma, was unremarkable. The laboratory tests showed normal blood histiocytes, Langerhans’ cell histiocytosis, histiocytic sar- counts and normal liver and renal function. An MRI showed coma. 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 nor- ABBREVIATIONS. LCH, Langerhans’ cell histiocytosis; CT, com- mal. Bone marrow aspiration revealed no malignant infiltration. A puted tomography; 2CdA, 2-chlorodeoxyadenosine. 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 istiocytic and dendritic neoplasms are rare, sheets and clusters of large ovoid cells with abundant eosinophilic cytoplasm (Fig 2A). The nuclei were round/oval, eccentric, and especially in children. These tumors arise pleomorphic with occasional grooving. Distinct, small nucleoli Hfrom antigen-processing phagocytes (histio- were present. Multinucleated giant cells were also present (Fig cytes) and antigen-presenting dendritic cells. The cell 2B). Immunohistochemistry showed expression of CD68 (macro- types are derived from hematopoietic or mesenchy- phage/histiocytic marker; Fig 2C) and focal expression of S100 1 protein and CD15 (granulocyte marker). The following markers mal stem cells. Currently, the World Health Orga- were negative: CD1a (Langerhans’ and immature T-cell marker; nization includes the following 6 entities under this Fig 2D), CD3 (T-cell marker), CD20/CD79a (B-cell markers), CD23 designation: Langerhans’ cell histiocytosis (LCH), (B-cell chronic lymphocytic leukemia/lymphoproliferative dis- ease marker), CD21 (mature B-cell and follicular dendritic cell Langerhans’ cell sarcoma, follicular dendritic cell marker), CD35 (follicular dendritic cell marker), CD30 (anaplastic sarcoma, interdigitating dendritic cell sarcoma, den- large-cell lymphoma and Reed-Sternberg cell marker), ALK (ana- dritic cell sarcoma not otherwise specified, and his- plastic large-cell lymphoma marker), desmin, smooth muscle ac- tiocytic sarcoma.2 The latter malignancy is a prolif- tin, and myeloperoxidase (myeloid cell marker). Electron micros- copy revealed numerous cytoplasmic lysosomes; however, eration of histiocytes (tissue macrophages), which Birbeck granules (typical of Langerhans’ cells), interdigitating cell are characterized by positive expression of the mac- junctions, cytoplasmic projections, and desmosomes were not rophage-associated antigen CD68, negative expres- identified. These morphologic and immunohistochemical features sion of the T-cell–associated antigen CD1a, negative are typical of histiocytic sarcoma (Table 1). The patient was started on LCH-based therapy (prednisone, expression of the dendritic cell–associated antigens 6-merpactopurine, methotrexate, vinblastine, and etoposide). CD21/CD35, and lack of Birbeck granules on elec- However, within ϳ2 weeks, his condition deteriorated and he tron microscopy.3 developed neurogenic bladder. He then received radiation (4500 cGy in 180-cGy fractions) to the L4 vertebral body and associated The rarity of histiocytic sarcoma continues to make paraspinal mass. He gradually improved. At the end of the radi- its management challenging, and we are unaware of ation treatment, he was walking without limitation and had full any recommended therapy for young children. We bladder control. A follow-up MRI showed significant diminution describe a child with histiocytic sarcoma to highlight of the paraspinal mass (Fig 1 C and D); the T6 vertebra remained unchanged. his poor response to LCH- and sarcoma-based che- The radiation was followed by 3 monthly cycles of cyclophos- motherapy. By contrast, high-dose radiation proved phamide (2.2 g/m2) and actinomycin D (0.045 mg/kg). At the end to be effective. of the third cycle, his chest CT scan revealed 2 nodules in the right and left upper lobes. A positron emission tomography scan showed abnormal uptake limited to the lung nodules. The meta- static lesions were confirmed by a CT-guided biopsy. He received 2 From the Departments of *Pediatrics, ‡Pathology, and §Radiation Oncol- 2 monthly cycles of idarubicin (10 mg/m ) and 2-chlorodeoxya- 2 ogy, State University of New York, Upstate Medical University, Syracuse, denosine (2CdA [or cladribine] at 8 mg/m ). A follow-up chest CT New York. showed diminution of the lung lesions. Localized radiation (4500 Accepted for publication Feb 28, 2005. cGy at 180 cGy per fraction) was delivered to the metastatic lung doi:10.1542/peds.2005-0026 nodules. This treatment was followed by 6 monthly cycles of 2 No conflict of interest declared. 2CdA (8 mg/m ). Address correspondence to Abdul-Kader Souid, MD, PhD, Department of Pediatrics, State University of New York, Upstate Medical University, 750 E DISCUSSION Adams St, Syracuse, NY 13210. E-mail: [email protected] PEDIATRICS (ISSN 0031 4005). Copyright © 2005 by the American Acad- Histiocytes (tissue macrophages) are derived from emy of Pediatrics. hematopoietic monoblasts. These phagocytes contain e322 PEDIATRICS Vol.Downloaded 116 No. 2 from August www.aappublications.org/news 2005 www.pediatrics.org/cgi/doi/10.1542/peds.2005-0026 by guest on September 27, 2021 Fig 1. MRIs showing collapse of the T6 and L4 vertebral bodies (white arrows) (A) and associated soft tissue mass in the anterior epidural space at the L4 level (black double arrows) with cord compression (single black arrow) (A and B) and follow-up MRI (ϳ2 months postradiation to the L4 region) show- ing diminution of the soft tissue mass (C) and absence of cord compression (D). organelles (vacuoles and lysosomes) and enzymes diffuse/anaplastic large B-cell lymphoma, peripheral (lysozyme, ␣-1-antitrypsin, acid phosphatase, and T-cell lymphoma associated with hemophagocytic nonspecific esterases) capable of processing antigens syndrome, or lymphoma with associated reactive for T lymphocytes. The cells also lack Birbeck gran- macrophages (eg, histiocyte recruitments by mac- ules (typical of Langerhans’ cells), complex cell junc- rophage-activating factors).4,5 tions (typical of interdigitating dendritic cells), and Morphologically, tumor cells are characterized by desmosomes (typical of follicular dendritic cells). abundant eosinophilic cytoplasm (Fig 2A). The nu- CD68 (a lysosome-associated protein) is the most clei are eccentric and round/oval, with atypia vary- important antigen that detects macrophages.2 ing from mild to pleomorphic. The nucleoli are small Histiocytic sarcoma is an exceedingly rare ma- and distinct. Binucleated giant cells are common (Fig lignancy that demonstrates morphologic and immu- 2B). The ultrastructural features show numerous nophenotypic features of macrophage/histiocytic vacuoles and lysosomes. Interdigitating cell junc- differentiation. Before the development of immuno- tions and Birbeck granules are essentially absent. The histochemistry, this diagnosis was more common. It latter features distinguish this entity from Langer- is now recognized that most cases of “histiocytic hans’ and dendritic cell tumors, especially when sarcoma” described in the past actually represented S100 protein is positive (Table 1). The immunohisto- Downloaded from www.aappublications.org/newswww.pediatrics.org/cgi/doi/10.1542/peds.2005-0026 by guest on September 27, 2021 e323 Fig 2. A, Neoplastic cells showing abundant eosinophilic cytoplasm, round to oval vesicular nuclei, and dis- tinct nucleoli. B, Binucleated cells ad- mixed with stromal inflammatory in- filtrates. C, Strongly positive granular cytoplasmic staining for the lysosomal marker CD68 with the monoclonal an- tibody KP1. D, Negative CD1a stain. TABLE 1. Antigen-Detectable Profiles of Macrophage/Histiocytic and Dendritic Neoplasms CD1a CD21/CD35 CD68 Lysozyme S100 LCH/sarcoma ϩϪϩϮ* ϩ Interdigitating dendritic cell tumor/sarcoma ϪϪϮ* Ϯ* ϩ Follicular dendritic cell tumor/sarcoma ϪϩϮ* ϪϪ Dendritic cell sarcoma, not otherwise specified ϮϮϮϮϮ Histiocytic sarcoma ϪϪϩϩϮ* Case patient ϪϪϩϩϩ† * Expressed in ϳ25% to 50% of cases. † Focally positive.1 chemical profile shows expression of the lysosome- shared the rearrangements involving immunoglobu- associated (macrophage/histiocytic) markers CD68 lin heavy chain and T-cell receptor ␥-chain genes, (in 100% of cases) and lysozyme (in 94% of cases).1 suggesting lineage infidelity for the recurrent dis- The Langerhans’ cell, follicular dendritic cell, my- ease.7 Two other similar cases have also been de- eloid,
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