Anaplastic lymphoma kinase (ALK)-positive histiocytosis is a rare newly recognized subtype of histiocytic neoplasms. The disease can involve multiple systems or single system. We present FDG PET/CT findings in a pediatric patient with ALK-positive histiocytosis isolated to the left nasal cavity. The nasal cavity tumor invaded the nasal septum, left nasal bone, and left ethmoid sinus and showed intense FDG activity with an SUVmax of 9.9.
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Langerhans Cell Histiocytosis and Other Histiocytic Lesions.
Head Neck Pathol
February 2025
Molecular Diagnostics Laboratories, The Joint Pathology Center, Bethesda, MD, USA.
Background: Histiocytoses, including Langerhans cell histiocytosis (LCH), comprise a diverse group of histiocytic disorders characterized by the abnormal accumulation and proliferation of histiocytes in various tissues or organs throughout the body, ranging from benign, self-limited conditions to aggressive malignancies and systemic inflammatory syndromes. These lesions present unique diagnostic challenges due to their broad spectrum of clinical presentations, overlapping histopathological and immunophenotypical features, and genetic complexity.
Methods: This review analyzes major histiocytic lesions, focusing on their epidemiology, clinical presentations, histologic and immunophenotypic features, and genetic characteristics to facilitate accurate diagnosis and differentiation among these histiocytoses.
FDG PET/CT in ALK-positive Histiocytosis of the Nasal Cavity in a Pediatric Patient.
Clin Nucl Med
February 2025
Department of Nuclear Medicine, Ganzhou People's Hospital, Ganzhou, China.
Anaplastic lymphoma kinase (ALK)-positive histiocytosis is a rare newly recognized subtype of histiocytic neoplasms. The disease can involve multiple systems or single system. We present FDG PET/CT findings in a pediatric patient with ALK-positive histiocytosis isolated to the left nasal cavity.
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Department of Neurosurgery, St. Vincent's Hospital, College of Medicine, The Catholic University of Korea, Seoul, Korea.
This paper discusses a rare case of a 49-year-old female diagnosed with a central nervous system-isolated neoplasm with histiocytic features, classified as histiocytic neoplasm, not otherwise specified (NOS). Despite comprehensive histopathological and genetic studies, accurately diagnosing the case proved challenging. Initial surgical findings and frozen biopsy suggested a low-grade glioma; however, subsequent immunohistochemistry and genetic analysis excluded known histiocytic subtypes such as ALK-positive histiocytosis and Erdheim-Chester disease.
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Department of Pathology, Shanxi Province Cancer Hospital/Shanxi Hospital Affiliated to Cancer Hosipital, Chinese Academy of Medical Sciences/Cancer Hospital Affiliated to Shanxi Medical University, Taiyuan, China.
In this article, we report the first case of a 61-year-old woman who was diagnosed with both nodules and cystic lesions in her lungs. The lung nodules were diagnosed as ALK-positive histiocytosis (APH) carrying an gene fusion, which microscopically displayed a mixed morphology of foamy cells, spindle cells, and Touton's giant cells. Immunohistochemistry showed expression of CD163, CD68, and ALK, while fluorescence hybridization (FISH) with second-generation sequencing (NGS) showed the ALK gene fusion with the FLCN gene variant.
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