Sze Kiat Tan1, Lee Onn Chieng1, Karthik Madhavan1, Andrew Rosenberg2, Ian Cote3. 1. Department of Neurological Surgery and the Miami Project to Cure Paralysis, University of Miami Miller School of Medicine, Miami, Florida, USA. 2. Department of Pathology, University of Miami Miller School of Medicine, Miami, Florida, USA. 3. Department of Neurological Surgery and the Miami Project to Cure Paralysis, University of Miami Miller School of Medicine, Miami, Florida, USA. Electronic address: icote@med.miami.edu.
Abstract
BACKGROUND: Indeterminate dendritic cell tumor (IDCT) is an extremely rare hematologic disorder with poorly understood pathogenesis. Occasionally encountered by hematologists, unusual presentations of IDCT have not been reported in the spine literature. METHODS: We report a 51-year-old man who presented with a 3-month history of progressively worsening axial thoracic back pain radiating to his sides. Magnetic resonance imaging revealed a 3-cm enhancing mass at the T9 vertebral body with an exophytic component causing significant canal stenosis. Initial percutaneous biopsy revealed histiocytic sarcoma. RESULTS: The patient underwent exploratory thoracotomy and en bloc resection of the lesion with T8-10 fusion. Final pathology results revealed IDCT with fibrosis. IDCT immunostaining was partially positive for Langerhans cell marker (positive for S100 and CD1a, but lacked Birbeck granules and Langerin stain) and partially positive for blastic plasmacytoid dendritic cell neoplasm. Additionally, it was positive for CD45, CD68, and CD163. Lymphadenopathy was absent in this patient. CONCLUSIONS: Although first reported in the 1980s, IDCT has been omitted from most classifications owing to its rarity. Hematologists have debated the cell of origin; it is believed to comprise pre-Langerhans cells, as Birbeck granules are acquired after migration to the epidermis. IDCT remains of indeterminate origin. We report the first case of spinal IDCT. Familiarity with the histologic features is warranted to ensure accurate diagnosis and appropriate treatment.
BACKGROUND: Indeterminate dendritic cell tumor (IDCT) is an extremely rare hematologic disorder with poorly understood pathogenesis. Occasionally encountered by hematologists, unusual presentations of IDCT have not been reported in the spine literature. METHODS: We report a 51-year-old man who presented with a 3-month history of progressively worsening axial thoracic back pain radiating to his sides. Magnetic resonance imaging revealed a 3-cm enhancing mass at the T9 vertebral body with an exophytic component causing significant canal stenosis. Initial percutaneous biopsy revealed histiocytic sarcoma. RESULTS: The patient underwent exploratory thoracotomy and en bloc resection of the lesion with T8-10 fusion. Final pathology results revealed IDCT with fibrosis. IDCT immunostaining was partially positive for Langerhans cell marker (positive for S100 and CD1a, but lacked Birbeck granules and Langerin stain) and partially positive for blastic plasmacytoid dendritic cell neoplasm. Additionally, it was positive for CD45, CD68, and CD163. Lymphadenopathy was absent in this patient. CONCLUSIONS: Although first reported in the 1980s, IDCT has been omitted from most classifications owing to its rarity. Hematologists have debated the cell of origin; it is believed to comprise pre-Langerhans cells, as Birbeck granules are acquired after migration to the epidermis. IDCT remains of indeterminate origin. We report the first case of spinal IDCT. Familiarity with the histologic features is warranted to ensure accurate diagnosis and appropriate treatment.
Authors: Rosita Sortino; Michael Schmid; Yassir El Baz; Antonia Loosen; Ignazio Tarantino; Thomas Steffen; Bruno M Schmied; Fariba Abbassi Journal: J Surg Case Rep Date: 2020-07-16