A giant cell is a large, multinucleated cell formed by the fusion of multiple mononuclear cells, such as macrophages, monocytes, or epithelioid cells, typically measuring 40–120 µm in diameter and containing 15–30 nuclei arranged in characteristic patterns.[1] These cells arise primarily in response to persistent stimuli like chronic inflammation, infections, foreign bodies, or pathological conditions, serving as key components of the immune response.[2]Giant cells form through a process of cell adhesion and membrane fusion mediated by specific molecules, including DC-STAMP for fusion initiation, vitronectin for adhesion in certain types, and integrins like αvβ3 in bone-resorbing variants.[2] The stimuli triggering fusion vary: for instance, cytokines such as interleukin-4 (IL-4) induce foreign body giant cells at implant sites, while receptor activator of nuclear factor kappa-B ligand (RANKL) and macrophage colony-stimulating factor (M-CSF) drive osteoclast formation for bone remodeling.[2] In infectious contexts, high-virulence pathogens like Mycobacterium tuberculosis promote giant cell development within granulomas, often resulting in non-phagocytic cells with over 15 nuclei.[2]Several distinct types of giant cells exist, classified by their origin, nuclear arrangement, and pathological context. Macrophage-derived types include Langhans giant cells, with nuclei in a horseshoe pattern at the periphery, commonly seen in tuberculous granulomas; foreign body giant cells, featuring randomly scattered nuclei and forming at biomaterial interfaces; and Touton giant cells, characterized by a central ring of nuclei surrounding lipid droplets, as in xanthogranulomas.[1] Epidermal-derived variants, such as Tzanck cells with molded nuclei, appear in viral infections like herpes, while melanocyte-derived starburst giant cells indicate conditions like lentigo maligna melanoma.[1] Osteoclasts represent a specialized subtype involved in bone resorption, expressing high levels of tartrate-resistant acid phosphatase (TRAP).[2]Functionally, giant cells play diverse roles in immunity and tissue homeostasis, including phagocytosis of pathogens or debris, antigen presentation in granulomatous diseases like sarcoidosis, and degradation of substrates in foreign body reactions that can complicate medical implants.[2] In pathology, their presence aids diagnosis: for example, they are hallmark features in giant cell tumors of bone, which are benign but aggressive lesions near joints driven by RANKL overproduction. Despite their protective intent, giant cells can contribute to tissue damage, as in chronic inflammatory disorders, and their formation is influenced by the local microenvironment, leading to phenotypic variations across diseases.[2]
Definition and Formation
Morphological and Functional Characteristics
Giant cells are atypical, enlarged, multinucleated cells typically measuring 40–120 μm in diameter, formed primarily through the fusion of mononuclear precursor cells such as macrophages or monocytes, though some arise from failed mitosis in neoplastic contexts.[1][3] These cells represent a specialized response in physiological and pathological settings, distinguishing them from typical mononuclear cells by their increased size and nuclear multiplicity.Morphologically, giant cells exhibit varied shapes, including round, ovoid, irregular, or spindled forms, depending on the tissue context and stimuli. They possess abundant cytoplasm, often containing phagocytic inclusions or vacuoles, which supports their role in tissue interactions. Nuclei, numbering from 10 to 100 per cell, are arranged in patterns such as scattered distribution, clustering, or a characteristic horseshoe configuration along the periphery. Typical diameters range from 40 to 120 μm, enabling them to handle larger substrates than mononuclear counterparts. Specific subtypes, like osteoclasts, demonstrate tartrate-resistant acid phosphatase (TRAP) positivity, aiding in their identification via histochemical staining.[3][4][5]Functionally, giant cells primarily engage in phagocytic or resorptive activities to clear debris, pathogens, or extraneous materials from tissues, or to facilitate remodeling processes such as bone resorption. Their multinucleated structure enhances efficiency in engulfing large particles—up to 20-45 μm—or degrading extracellular matrices through lysosomal enzymes and reactive oxygen species, capabilities that surpass those of mononucleated macrophages. Unlike mononuclear macrophages, which focus on routine phagocytosis and antigen presentation, giant cells exhibit specialized, amplified responses tailored to persistent challenges, though their phagocytic capacity can vary by type and stimulus.[4][6]
Mechanisms of Formation
Giant cells primarily form through the homotypic fusion of monocytes or macrophages, a process that integrates multiple precursor cells into a single multinucleated entity capable of enhanced phagocytic or degradative functions. This fusion mechanism involves initial cell-cell recognition and adhesion, followed by membrane merger, and is distinct from alternative pathways like polyploidization. In the polyploidization route, particularly observed in osteoclast precursors, failed karyokinesis or incomplete cytokinesis during cell division leads to DNA replication without nuclear separation, resulting in enlarged, polyploid cells that may contribute to giant cell-like structures. However, fusion remains the dominant pathway for most inflammatory and physiological giant cells, driven by specific molecular cues that synchronize cytoskeletal rearrangements and membrane dynamics.[2]The formation process unfolds in sequential stages: monocyterecruitment to the site of inflammation or stimulus via chemotactic signals, such as CCR2-mediated mobilization from bone marrow; activation of these precursors by environmental cues or cytokines, which upregulate fusogenic proteins; and subsequent homotypic fusion, where activated cells extend protrusions like lamellipodia to contact and merge with neighbors. Key cytokines orchestrate differentiation and fusion: RANKL promotes osteoclast precursor activation and fusion alongside M-CSF, while IL-4 and IL-13 induce foreign body giant cell formation through STAT6 signaling, enhancing E-cadherin expression for stable cell-cell adhesion; IFN-γ similarly drives fusion in granulomatous contexts by modulating STAT pathways. Central molecular players include DC-STAMP, a seven-transmembrane protein essential for initiating cell-cell fusion in both osteoclasts and foreign body giant cells by facilitating membrane apposition, and OC-STAMP, which cooperates with DC-STAMP to modulate fusion efficiency and podosome belt organization in these cells. Additional fusogens like syncytin-B, derived from endogenous retroviral envelopes, contribute to early fusion stages in macrophage and osteoclast lineages by promoting membranefusion without altering overall cell size or resorption capacity. Environmental triggers, such as persistent foreign materials that frustrate phagocytosis, or hypoxic conditions that polarize macrophages toward an IL-4-responsive M2 phenotype, further potentiate recruitment and activation, amplifying fusion propensity.[7][8][9][10][11]Experimental evidence from in vitro models underscores these mechanisms, demonstrating that fusion rates are highly sensitive to microenvironmental factors. For instance, culturing macrophages in 3D collagen matrices reveals that increased stiffness (e.g., 7.5% gel concentration) elevates fusion indices up to 34% with multinucleated cells containing ≥6 nuclei, by modulating E-cadherin and integrin-mediated adhesions that enhance cell-cell contacts. These models, often using IL-4 stimulation or biomaterial substrates, confirm that blocking DC-STAMP or E-cadherin abolishes fusion, yielding mononuclear cells incapable of giant cell maturation.[12]
Physiological Roles
Osteoclasts in Bone Remodeling
Osteoclasts are multinucleated giant cells derived from hematopoietic monocyte-macrophage lineage precursors, essential for bone resorption during physiological remodeling.[13] These cells originate from circulating precursors that fuse to form mature osteoclasts, a process primarily driven by the receptor activator of nuclear factor kappa-B ligand (RANKL) pathway. Osteoblasts and stromal cells express RANKL, which binds to RANK receptors on osteoclast precursors, triggering differentiation and fusion in the presence of macrophage colony-stimulating factor (M-CSF). This interaction is tightly regulated by osteoprotegerin (OPG), a soluble decoy receptor produced by osteoblasts that inhibits RANKL binding, thereby preventing excessive osteoclast formation.[14][13]In bone remodeling, osteoclasts attach to the bone surface via integrin-mediated sealing zones, forming a resorption compartment where they actively degrade mineralized matrix. They generate an acidic microenvironment through vacuolar H+-ATPase (V-ATPase) proton pumps on their ruffled border, which demineralizes hydroxyapatite by lowering the pH to approximately 4.5.[15] Subsequently, lysosomal enzymes such as cathepsin K, a cysteine protease, and matrix metalloproteinases (MMPs, notably MMP-9) are secreted to hydrolyze organic components like type I collagen, enabling efficient bone matrix breakdown.[16] This coordinated resorption creates Howship's lacunae, shallow pits on the bone surface, allowing for the removal of old or damaged bone tissue.[15]Osteoclast activity is regulated by systemic hormones and local factors to maintain bone homeostasis, with osteoblasts playing a central role in coupling resorption to formation. Parathyroid hormone (PTH) indirectly stimulates osteoclastogenesis by enhancing RANKL expression and reducing OPG in osteoblasts, promoting bone resorption during calcium mobilization.[17] Similarly, 1,25-dihydroxyvitamin D (calcitriol) upregulates RANKL in osteoblasts while suppressing OPG, amplifying osteoclast differentiation and function to support mineral homeostasis.[18] This osteoblast-osteoclast coupling ensures that resorption releases growth factors from the matrix, such as TGF-β, which recruit and activate osteoblasts for subsequent bone formation, balancing turnover in a tightly coordinated manner.[19]In healthy adults, osteoclasts contribute to physiological bone remodeling, replacing approximately 10% of the skeleton annually to repair microdamage and adapt to mechanical stress.[20] Individual osteoclasts have a short lifespan of about 2 weeks, after which they undergo apoptosis, limiting prolonged resorption and supporting steady-state bone maintenance.[21] This turnover rate varies by skeletal site, with trabecular bone exhibiting higher remodeling (up to 25% per year) compared to cortical bone (around 3%), reflecting the dynamic equilibrium essential for skeletal integrity.[22]
Other Normal Physiological Contexts
In the context of human reproduction, syncytiotrophoblasts represent a prominent example of multinucleated giant cells essential for placental function. These cells form through the continuous fusion of underlying cytotrophoblast progenitor cells, creating a vast, multinucleated syncytial layer that lines the placental villi. This structure serves as the primary interface for maternal-fetal exchange, facilitating the transport of nutrients, oxygen, and waste products while acting as a barrier to pathogens.[23][24] Additionally, syncytiotrophoblasts are major producers of pregnancy-sustaining hormones, including human chorionic gonadotropin (hCG), which maintains the corpus luteum and supports early embryonic development.[25][26]These fusion-derived giant cells, such as syncytiotrophoblasts and osteoclasts, exemplify the primary physiological roles of giant cells in humans, consistent with their formation by cell fusion in response to specific developmental and homeostatic needs.The formation and maintenance of these giant cells are tightly regulated by tissue-specific signaling pathways that coordinate fusion, polyploidization, and differentiation. For instance, in placental development, epidermal growth factor (EGF) and its receptor signaling promote cytotrophoblast fusion into syncytiotrophoblasts by activating downstream cascades like ERK/MAPK, which enhance cell-cell adhesion and membrane remodeling.[27][28]
Pathological Types in Inflammation
Langhans Giant Cells
Langhans giant cells are a subtype of multinucleated giant cells characterized by their distinctive peripheral arrangement of nuclei in a horseshoe or ring-like pattern, typically containing 10 to 30 nuclei clustered along the cell periphery within abundant eosinophilic cytoplasm. These cells measure approximately 40 to 120 micrometers in diameter and are derived from the fusion of epithelioid macrophages during chronic granulomatous inflammation. Unlike foreign-body giant cells, their organized nuclear configuration reflects an adaptive immune response rather than a random fusion.[29][1][30]The formation of Langhans giant cells is triggered by persistent antigens in the context of type IV (delayed-type) hypersensitivity, where T-cell activation leads to the release of interferon-gamma (IFN-γ), promoting macrophage fusion through pathways involving CD40-CD40L interactions, toll-like receptor 2, and adhesion molecules like β1 integrin. This process occurs in response to intracellular pathogens or non-infectious stimuli that evade clearance, resulting in granuloma formation as a containment mechanism. Histologically, these cells are identified in tissue biopsies using hematoxylin and eosin (H&E) staining, where they appear as prominent components of well-formed granulomas surrounded by lymphocytes.[29][1][30]Langhans giant cells are primarily associated with granulomatous diseases driven by cell-mediated immunity, including infections such as tuberculosis caused by Mycobacterium tuberculosis, where they rim areas of caseous necrosis, and secondary syphilis, in which they contribute to granuloma formation in affected tissues. They also appear in non-infectious conditions like sarcoidosis, featuring non-necrotizing granulomas with minimal surrounding inflammation.[29][1][30]Diagnostically, the presence of Langhans giant cells serves as a marker of effective cell-mediated immune responses, indicating organized granulomatous inflammation rather than acute processes. Although they exhibit limited phagocytic activity compared to mononuclear macrophages, these cells often contain cellular debris and antigen remnants, aiding in the histological diagnosis when combined with special stains or molecular tests to identify underlying etiologies.[29][30][1]
Foreign-Body Giant Cells
Foreign-body giant cells (FBGCs) are multinucleated macrophages that form through the fusion of multiple mononucleated macrophages in response to persistent, non-immunogenic foreign materials. Morphologically, they are characterized by an irregular shape, large size (up to 1 mm in diameter), and numerous nuclei (often hundreds) that are scattered heterogeneously throughout the cytoplasm or occasionally clustered centrally, as observed in histological sections of implant sites. This nuclear arrangement arises from the fusion process, which is predominantly induced by Th2 cytokines such as interleukin-4 (IL-4) and interleukin-13 (IL-13); these cytokines activate STAT6 signaling to upregulate key fusogenic proteins like DC-STAMP (dendritic cell-specific transmembrane protein) and E-cadherin, facilitating cell-cell adhesion, migration, and membrane merger over several days.[31][4][32]FBGC formation is triggered by inert, biocompatible materials that cannot be readily phagocytosed by individual macrophages, including silica particles, tattoo pigments, surgical sutures, and implanted medical devices such as orthopedic prostheses, vascular grafts, or polymer-based scaffolds like expanded polytetrafluoroethylene or poly-lactic acid. These triggers elicit a non-antigenic, mechanical response independent of T-cell involvement, distinguishing FBGCs from immune-driven giant cells; instead, the reaction depends on biomaterial surface properties, such as adsorbed proteins (e.g., fibrinogen or vitronectin), which promote macrophage recruitment and activation at the material-tissue interface.[31][33][32]Functionally, FBGCs attempt to engulf and degrade large foreign particles exceeding 10 μm in diameter—beyond the phagocytic capacity of single macrophages—through a process termed "frustrated phagocytosis," where partial enclosure leads to persistent adhesion rather than internalization. They exhibit a distinct cytokine profile, secreting high levels of pro-inflammatory mediators like tumor necrosis factor-alpha (TNF-α) and reactive oxygen species to erode the material, alongside anti-inflammatory factors such as transforming growth factor-beta (TGF-β) that modulate the response toward resolution. However, FBGCs display limited phagocytic efficiency compared to mononuclear macrophages and cannot resorb structured tissues like bone, focusing instead on surface degradation of biomaterials.[4][31][33]In histological examinations, FBGCs appear prominently at the biomaterial-host interface, often embedded in a dense layer of activated macrophages and surrounded by progressive fibrosis that forms a collagenous capsule to encapsulate and isolate the foreign material. This fibrotic encapsulation, driven by FBGC-derived TGF-β and other pro-fibrogenic signals, is a key feature of the chronic foreign body reaction and can impair implant integration or functionality, as seen in biomaterial responses to silicone-based devices or wear debris from joint replacements.[32][33][4]
Giant Cells in Vascular and Autoimmune Diseases
Giant Cell Arteritis
Giant cell arteritis (GCA), also known as temporal arteritis, is a chronic systemic vasculitis that primarily affects medium- and large-sized arteries, particularly the branches of the external carotid artery such as the temporal arteries. It is characterized by granulomatous inflammation featuring multinucleated giant cells infiltrating the arterial media, leading to vessel wall thickening, stenosis, and ischemia. This condition is noninfectious and autoimmune in nature, with dendritic cells playing a key role in recruiting and activating T-cells, which drive cytokine production (e.g., IL-6 and IFN-γ) and perpetuate arterial inflammation.[34][35]Epidemiologically, GCA predominantly occurs in individuals older than 50 years, with peak incidence between 70 and 80 years, and shows a strong female predominance (2-3:1 ratio). It is more common among those of Northern European or Scandinavian descent, with an annual incidence of approximately 20 per 100,000 in high-risk populations such as in Olmsted County, USA, though rates are lower in African American (3.1 per 100,000), Asian (1.47 per 100,000 in Japan), and Middle Eastern groups. Up to 40-60% of patients also experience polymyalgia rheumatica concurrently. The lifetime risk is about 1% for women and 0.5% for men in the United States.[34][35]Clinically, GCA presents with a range of symptoms, including new-onset headache (affecting two-thirds of patients), scalp tenderness, and jaw claudication (in about 50%), which results from ischemic pain during mastication. Ocular involvement occurs in 20-30% of cases, often manifesting as sudden vision loss due to anterior ischemic optic neuropathy, with an 8.2% incidence of permanent blindness if untreated. Systemic symptoms such as fever, fatigue, weight loss, and malaise are reported in 50% of patients, while rarer features include tonguepain or stroke (1.5-7.5% risk within 4 weeks of onset).[34][35]Diagnosis relies on a combination of clinical evaluation, laboratory tests, imaging, and histopathology. Elevated erythrocyte sedimentation rate (ESR >50 mm/h in most cases) and C-reactive protein (CRP) are common but nonspecific; ESR may be normal in 25% of patients, making CRP a more reliable monitoring tool. Temporal artery biopsy remains the gold standard, revealing skip lesions with multinucleated giant cells, granulomatous inflammation, and intimal hyperplasia in 24-94% of positive cases (sensitivity improves with biopsy lengths >1 cm). Noninvasive imaging includes color Doppler ultrasound showing the "halo sign" (sensitivity 74-77%, specificity 81-96%), MRI/MRA (sensitivity 73%, specificity 88%), and PET/CT for detecting large-vessel involvement.[34][35]Treatment is initiated promptly upon suspicion to prevent vision loss, starting with high-dose glucocorticoids such as oral prednisone (40-60 mg/day) or intravenous methylprednisolone (500-1000 mg/day for 3 days in cases of ocular involvement). This leads to rapid symptom resolution and reduced ischemic risk. For refractory or relapsing disease, biologic agents like tocilizumab (162 mg subcutaneous weekly), an IL-6 inhibitor, are FDA-approved and reduce steroid dependence while lowering relapse rates. Long-term management includes corticosteroid tapering with monitoring for complications such as aortic aneurysm (increased risk due to large-vessel inflammation) and steroid side effects like osteoporosis or diabetes. Low-dose aspirin may be added for patients with critical arterial involvement to mitigate thrombotic events.[34][35]
Other Vasculitides Involving Giant Cells
Takayasu arteritis, also known as Takayasu disease, is a chronic granulomatous large-vessel vasculitis predominantly affecting the aorta and its major branches, often featuring multinucleated giant cells in the inflammatory infiltrates during active disease phases.[36] It typically manifests in young women under 40 years of age, with clinical presentations including pulse deficits in affected limbs, vascular bruits, and constitutional symptoms such as fatigue and weight loss.[37] Histopathologically, early lesions show panarteritis with giant cells and lymphocytes disrupting the elastic lamina, progressing to fibrosis and stenosis in chronic stages, distinguishing it from elderly-onset giant cell arteritis by demographic and vessel distribution patterns.[37] Diagnosis relies on angiography demonstrating arterial narrowing, occlusion, or aneurysms, supported by elevated inflammatory markers like erythrocyte sedimentation rate.[36]Giant cell-rich variants occur in certain ANCA-associated vasculitides, particularly granulomatosis with polyangiitis (GPA, formerly Wegener's granulomatosis), where granulomatous inflammation incorporates multinucleated giant cells alongside necrotizing vasculitis of small to medium vessels.[38] In GPA, these giant cells are evident in biopsies of affected respiratory tract tissues, forming poorly organized granulomas with central necrosis, often accompanied by palisading histiocytes.[38] This granulomatous subtype contrasts with pauci-immune nongranulomatous forms like microscopic polyangiitis, and is associated with anti-proteinase 3 ANCA positivity in over 80% of cases, leading to upper and lower respiratory involvement with renal glomerulonephritis.[39]Rare vasculitides involving giant cells include extracranial giant cell arteritis, a subtype affecting large extracranial arteries such as the subclavian or femoral without cranial symptoms, showing identical granulomatous histology with giant cell infiltration and elastic lamina fragmentation as in cranial forms.[40] Another uncommon entity is hypocomplementemic urticarial vasculitis syndrome, a small-vessel vasculitis presenting with recurrent urticarial lesions that may exhibit leukocytoclastic changes with occasional multinucleated giant cells in dermal infiltrates, often linked to systemic features like arthritis or angioedema and low complement levels.[41] These rare presentations highlight histopathological overlaps but differ in vessel size and clinical distribution from primary giant cell arteritis.Management of these vasculitides centers on immunosuppression to control inflammation and prevent vascular complications like stenosis or aneurysms. Glucocorticoids form the cornerstone, often combined with steroid-sparing agents such as methotrexate (typically 15-25 mg weekly) for Takayasu arteritis to achieve remission and reduce relapse rates.[42] In GPA variants, rituximab or cyclophosphamide is preferred alongside steroids for induction, with methotrexate used for maintenance to monitor for disease progression via serial imaging and inflammatory markers.[38] Long-term surveillance includes vascular imaging to detect stenosis, with biologic agents like tocilizumab considered for refractory cases in large-vessel forms.[43]
Giant Cells in Neoplastic Conditions
Reed-Sternberg Cells
Reed-Sternberg cells are the pathognomonic neoplastic giant cells of classical Hodgkin lymphoma, characterized by their large size (typically 15–50 μm in diameter) and distinctive binucleated or multinucleated morphology with bilobed nuclei featuring prominent eosinophilic nucleoli often described as having an "owl-eye" appearance.[44] These cells possess abundant, slightly basophilic cytoplasm and a perinuclear halo, with variants including mononuclear Hodgkin cells and lacunar cells seen in the nodular sclerosis subtype.[44] Immunohistochemically, classic Reed-Sternberg cells typically express CD15 and CD30 while lacking CD45, aiding in their identification and distinction from reactive cells.[45]These cells originate from germinal center B-lymphocytes, as evidenced by clonal immunoglobulin gene rearrangements detected in isolated Reed-Sternberg cells, confirming their B-cell lineage despite loss of typical B-cell markers.[46] In approximately 40–50% of cases, particularly the mixed cellularity subtype, Epstein-Barr virus (EBV) is associated with Reed-Sternberg cells, where viral genomes are clonally integrated, suggesting a role in pathogenesis in EBV-positive Hodgkin lymphomas.[47]Subtypes include classic Reed-Sternberg cells found in the four variants of classical Hodgkin lymphoma (nodular sclerosis, mixed cellularity, lymphocyte-rich, and lymphocyte-depleted), which share the aforementioned immunophenotype and are diagnostic when present in an appropriate lymph node background of mixed inflammatory cells.[48] In contrast, nodular lymphocyte-predominant Hodgkin lymphoma features lymphocyte-predominant cells, often termed "popcorn cells" due to their multilobated nuclei, which express CD20 and CD45 but lack CD15 and CD30.[48]Diagnosis requires histopathological examination of lymph node biopsies confirming these cells amid a reactive milieu, with immunophenotyping essential for subtype classification.[44]In the disease context, Reed-Sternberg cells, though comprising less than 1% of the tumor mass, orchestrate the inflammatory microenvironment through secretion of cytokines such as interleukin-6 (IL-6), tumor necrosis factor-alpha (TNF-α), and granulocyte-macrophage colony-stimulating factor (GM-CSF), which recruit eosinophils, lymphocytes, and other immune cells, thereby promoting lymphomagenesis and immune evasion.[44] This cytokine-driven recruitment fosters a permissive niche that sustains tumor growth.[49]
Touton Giant Cells and Tumor-Associated Variants
Touton giant cells are multinucleated histiocytic cells distinguished by a characteristic wreath-like arrangement of nuclei surrounding a central zone of homogeneous eosinophiliccytoplasm, with peripheral foamy lipid-laden cytoplasm.[50] This morphology arises from the accumulation of lipid vacuoles within the cytoplasm, often imparting a ringed or haloed appearance under light microscopy. They are most classically observed in juvenile xanthogranuloma (JXG), a benign non-Langerhans cell histiocytosis primarily affecting infants and young children, where they represent a mature histologic feature amid sheets of foamy histiocytes and Touton cells.[51]In non-neoplastic contexts, Touton giant cells emerge as part of the macrophage response to lipid accumulation, particularly in hyperlipidemia-associated xanthomas. These cells derive from foam cells—lipid-engorged macrophages that phagocytose excess cholesterol and triglycerides in conditions like familial hypercholesterolemia or severe hypertriglyceridemia—leading to cellular fusion and multinucleation.[52] They also appear in various histiocytoses, such as Erdheim-Chester disease, where foamy histiocytes intermixed with Touton giant cells contribute to fibrotic tissue responses.[53]Tumor-associated variants of Touton giant cells occur in certain soft tissue neoplasms, including localized tenosynovial giant cell tumor (formerly giant cell tumor of the tendon sheath) and dermatofibroma, where older lesions may exhibit Touton-like cells amid foamy histiocytes and spindle cells.[54] In more aggressive contexts, such as undifferentiated pleomorphic sarcoma or atypical fibroxanthoma, Touton-like giant cells can appear as reactive components within the histiocytic infiltrate. Differential diagnosis relies on immunohistochemistry: these cells typically express CD68 (a macrophage marker) diffusely while lacking S100 protein expression, helping distinguish them from melanocytic or dendritic cell lesions like melanoma or Langerhans cell histiocytosis.[55]The pathogenesis of Touton giant cells involves macrophage recruitment and lipid uptake via scavenger receptors, followed by cell-cell fusion mediated by cytokines such as interleukin-6 and interferon-gamma, resulting in the lipid-rich multinucleated form. In neoplastic settings, their presence often reflects a reactive histiocytic response rather than neoplastic transformation, with limited direct prognostic impact; however, in JXG-like tumor variants, they correlate with indolent behavior and favorable outcomes post-excision.30004-X/fulltext)
Giant Cells in Infectious and Other Diseases
Role in Granulomatous Infections
Giant cells play a central role in the host immune response during granulomatous infections, where persistent microbial antigens trigger the formation of organized granulomas to contain and isolate pathogens that evade initial macrophage clearance. In these chronic infections, multinucleated giant cells, often derived from fused epithelioid macrophages, enclose infectious agents such as fungi, parasites, and bacteria, forming a protective barrier that limits dissemination but may also harbor viable organisms.[30][56] This process is particularly prominent in infections like tuberculosis, where Langhans giant cells contribute to granuloma architecture, though similar structures appear across diverse pathogens.[29]Fungal infections such as histoplasmosis and cryptococcosis frequently feature granulomas with giant cells that surround yeast forms, aiding in their containment within pulmonary or disseminated sites. In histoplasmosis, caused by Histoplasma capsulatum, granulomas in the lungs and gastrointestinal tract contain small intracellular yeasts within giant cells, reflecting a Th1-mediated response to antigen persistence.[57] Similarly, cryptococcosis due to Cryptococcus neoformans elicits granulomatous inflammation with multinucleated giant cells in the lungs or central nervous system.[58] Parasitic infections like leishmaniasis involve giant cells in cutaneous or visceral granulomas that enclose amastigotes of Leishmania species, particularly in the skin lesions of the New World form. Bacterial infections, notably leprosy (Mycobacterium leprae), showcase tuberculoid variants with non-necrotizing granulomas rich in epithelioid cells and giant cells that surround acid-fast bacilli, primarily affecting peripheral nerves and skin.[59][60] In all these cases, giant cells actively phagocytose and sequester pathogens, preventing systemic spread.[61]The formation of giant cells in these granulomas arises from the fusion of macrophages driven by persistent antigens that elicit a Th1 immune response, involving cytokines like IFN-γ to promote epithelioid differentiation and cell-to-cell fusion via adhesion molecules such as CD301.[62][63] This mechanism enhances antigen processing and presentation but can lead to incomplete pathogen eradication if the granuloma wall becomes impermeable. Diagnostic confirmation often relies on special stains; for instance, Ziehl-Neelsen staining highlights acid-fast mycobacteria within giant cells in leprosy and tuberculosis granulomas, revealing bacilli clustered in foamy macrophages.[64] Clinically, these infections manifest organ-specifically: pulmonary involvement predominates in histoplasmosis and cryptococcosis with cough and fever, while skin nodules and hypopigmented patches characterize leprosy and cutaneous leishmaniasis. In tuberculosis, granulomas may progress to caseation necrosis, where central cheesy degeneration within giant cell-rich cores signifies tissue destruction and higher bacterial load, often in the lungs.[65][66]Treatment of granulomatous infections targets the walled-off pathogens within giant cell-containing structures, necessitating prolonged antimicrobial regimens to penetrate the granuloma barrier. For tuberculosis and leprosy, multi-drug therapies like rifampin-isoniazid-pyrazinamide-ethambutol combinations disrupt mycobacterial replication inside granulomas, reducing caseation and promoting resolution.[67] In fungal cases such as histoplasmosis, itraconazole effectively clears yeasts from giant cells in immunocompetent hosts, while amphotericin B is used for severe cryptococcosis to address encapsulated organisms.[57] Parasitic leishmaniasis responds to antimonials or miltefosine, which target amastigotes sequestered by giant cells, though incomplete granuloma penetration can lead to relapse. Emerging insights from post-2020 research emphasize adjunctive immunomodulation to enhance drug delivery into these walled compartments, improving outcomes in chronic infections.[68]
Multinucleated Giant Cells in Viral Infections
Multinucleated giant cells, also known as syncytia, form in viral infections through mechanisms driven by viral glycoproteins that mediate cell-cell fusion. In human immunodeficiency virus (HIV) infection, the envelope glycoprotein gp120 binds to CD4 and co-receptors on adjacent cells, triggering fusion via gp41, leading to syncytial formation particularly in macrophages and microglia in the brain and lungs.[69] Similarly, the severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) spike protein facilitates fusion by engaging angiotensin-converting enzyme 2 (ACE2) receptors on neighboring cells, resulting in multinucleated pneumocytes and other epithelial syncytia.[70] This process enhances viral spread by allowing direct intercellular transmission without extracellular virion release, amplifying infection efficiency in tissues like the respiratory tract.[71]Several viruses prominently feature syncytial giant cells in histological examinations of infected tissues. Respiratory syncytial virus (RSV) induces fusion in bronchial epithelial cells, forming multinucleated giant cells with intracytoplasmic inclusions visible in autopsy or biopsy samples, contributing to airway obstruction in severe pediatric cases.[72]Measles virus, through its fusion (F) protein, promotes syncytia in respiratory epithelium and other sites, where these giant cells exhibit characteristic nuclear inclusions and are a hallmark of the infection's cytopathic effect in lung tissues.[73] In HIV, syncytia in the central nervous system, often termed multinucleated giant cells, are observed in autopsy brains of patients with AIDS, harboring viral proteins and driving neuroinflammation.[69] These formations contrast with slower granulomatous responses in bacterial infections by enabling rapid viral dissemination.In COVID-19, multinucleated pneumocytes were a frequent finding in lung autopsies from 2020 to 2023, particularly in severe cases, where syncytia correlated with diffuse alveolar damage and poorer outcomes due to enhanced viral propagation and tissue injury.[74] Early variants like Delta promoted robust syncytia formation, associating with high pathogenicity, but Omicron and subsequent subvariants from 2022 onward exhibited reduced fusogenic activity owing to mutations in the spike protein, diminishing syncytia prevalence and overall lung severity by 2025.[75] Emerging 2024-2025 data link persistent SARS-CoV-2 effects to long COVID, where foam cell accumulation may contribute to pulmonary fibrosis and chronic inflammation in affected lungs.[76]Diagnosis of these multinucleated giant cells in viral infections often relies on electron microscopy (EM) to visualize viral inclusions and fusion structures. TransmissionEM reveals characteristic inclusions, such as nucleocapsids in measles or coronavirus particles in SARS-CoV-2 syncytia, confirming viral etiology in tissue samples where light microscopy shows only giant cells.[77] Post-pandemic virology updates emphasize EM's role in distinguishing variant-specific syncytia, though its use has declined with molecular diagnostics; however, it remains essential for histological confirmation in atypical or persistent cases.[78]
Broader Pathogenic Roles
Endogenous Causative Agents
In autoimmune conditions such as rheumatoid arthritis, self-antigens like citrullinated proteins in the synovium are recognized by autoantibodies, including anti-citrullinated protein antibodies (ACPAs), leading to the formation of immune complexes that deposit in joint tissues.[79] These immune complexes activate the complement system via the classical pathway, generating anaphylatoxins such as C5a that amplify inflammation and recruit macrophages to the synovium.[80] The resulting chronic inflammatory milieu promotes the fusion of synovial macrophages into multinucleated giant cells, which contribute to tissue destruction and synovitis severity.[81]Metabolic disturbances, particularly hypercholesterolemia, drive giant cell formation in atherosclerosis by promoting the uptake of oxidized low-density lipoprotein (oxLDL) by macrophages in arterial walls, resulting in lipid-laden foam cells.[7] In advanced plaques, these foam cells can undergo further fusion into multinucleated giant cells, exacerbating plaque instability and inflammation through enhanced lipid processing and cytokine release.[82]Genetic factors influence giant cell formation by altering fusion propensity; for instance, dysregulation in the NF-κB pathway, often through activating mutations or enhanced signaling, upregulates genes like DC-STAMP essential for macrophage-osteoclast fusion.[83][84] Animal models such as op/op mice, which carry a mutation in the Csf1 gene leading to deficient macrophage colony-stimulating factor (CSF-1) and absence of osteoclasts, illustrate that CSF-1 signaling is essential for osteoclast formation but not for foreign body giant cell formation in response to inflammatory stimuli like implants.[85]Emerging evidence points to oxidative stress from endogenous reactive oxygen species (ROS), generated via NADPH oxidase in macrophages, as a trigger for giant cell formation by facilitating membrane alterations and fusion protein expression.[86]
Contribution to Tumor Formation
Giant cells, particularly osteoclast-like multinucleated variants, play a pivotal role in tumor progression by secreting pro-angiogenic factors such as vascular endothelial growth factor (VEGF) and platelet-derived growth factor (PDGF), which support stromal remodeling and vascularization within the tumor microenvironment. In giant cell tumor of bone (GCTB), these giant cells express VEGF, contributing to local angiogenesis and facilitating tumor expansion through enhanced blood supply and extracellular matrix support. Similarly, PDGF secretion by osteoclast-like giant cells promotes fibroblast recruitment and stromal fibrosis, creating a permissive niche for neoplastic growth. This paracrine signaling not only sustains the tumor's structural integrity but also enables invasion into surrounding tissues by degrading bone and soft tissue barriers.Beyond angiogenesis, giant cells exert immunosuppressive effects that shield tumors from immune surveillance, primarily through the release of transforming growth factor-beta (TGF-β). Multinucleated giant cells derived from tumor-associated macrophages produce TGF-β, which inhibits T-cell activation and proliferation while promoting regulatory T-cell expansion, thereby dampening anti-tumor immunity. In GCTB, elevated TGF-β levels from giant cells correlate with reduced cytotoxic T-lymphocyte infiltration, allowing neoplastic stromal cells to evade immune-mediated clearance. This mechanism underscores the dual role of giant cells in both structural and immunological support of tumorigenesis.In specific tumor types, these contributions are evident. Osteoclast-like giant cells in GCTB, recruited via RANKL signaling from neoplastic stromal cells, drive osteolysis that accommodates tumor growth and local invasion, distinguishing this entity from other bone neoplasms. In glioblastoma multiforme (GBM), particularly the giant cell variant, neoplastic multinucleated cells form syncytia through cell fusion, enhancing collective migration and resistance to apoptosis, which accelerates tumor dissemination along neural tracts.The presence of giant cells often portends adverse outcomes. In sarcomas such as angiosarcoma, bizarre multinucleated giant cells confer chemoresistance and are associated with reduced overall survival, independent of tumor stage. Therapeutic strategies targeting giant cell function, such as denosumab—a monoclonal antibody against RANKL—have shown efficacy in GCTB by depleting osteoclast-like giant cells, halting bone resorption, and inducing tumor regression in unresectable cases. This approach not only controls local disease but also improves progression-free survival.Emerging research from 2023 to 2025 highlights gaps in understanding giant cell-derived extracellular vesicles, particularly exosomes, in metastasis. Tumor-derived exosomes laden with miRNAs such as miR-574-5p promote bone metastasis by enhancing osteoclast differentiation in preclinical models of solid tumors like liver cancer.[87] Moreover, recent studies indicate immunotherapy implications; for instance, anti-PD-L1 blockade has elicited durable responses in recurrent GCTB with high PD-L1 expression on giant cells, as reported in a 2025 case of relapsed malignant GCTB treated with pembrolizumab, potentially synergizing with denosumab to restore anti-tumor immunity.[88]
Historical Development
Early Discoveries
The earliest documented observations of giant cells in pathology emerged in the mid-19th century, particularly in the context of inflammation and cellular pathology. Rudolf Virchow's foundational work in cellular pathology, including his 1858 lectures later compiled as Cellular Pathology, integrated multinucleated giant cells into a broader framework of inflammation, emphasizing that abnormal cellular proliferations arose from localized irritative processes rather than systemic imbalances, linking them directly to pathological inflammation in tissues like those affected by tuberculosis. Virchow's paradigm shift underscored giant cells as key players in host responses, influencing subsequent classifications and paving the way for distinguishing inflammatory variants from neoplastic ones.[89]Concurrent with these inflammation-related findings, giant cells began to be observed in bonetissue dynamics, though early microscopy imposed significant limitations. Pathologists of the era, constrained by rudimentary optical tools that often distorted cellular details, frequently misinterpreted these structures as degenerative remnants of normal tissue or even parasitic invaders, reflecting the prevailing humoral and vitalistic views of disease. Such misconceptions delayed precise classification, as giant cells appeared anomalous in size and nuclear arrangement compared to typical somatic cells.[61]A pivotal advancement came in 1868 when German pathologist Theodor Langhans provided the first detailed microscopic description of multinucleated giant cells within tuberculosis granulomas. In his seminal paper published in Virchows Archiv, Langhans characterized these cells' distinctive "horseshoe" or "horse-collar" nuclear configuration along the cell periphery, distinguishing them from other inflammatory elements and associating them specifically with tuberculoid tissue responses. This observation not only highlighted giant cells' prevalence in chronic infections but also spurred further investigation into their formation in granulomatous diseases.[90]In 1873, Swiss anatomist Albert von Kölliker described large, multinucleated cells actively participating in bone resorption during physiological and pathological remodeling processes. These cells, termed osteoclasts, were noted for their role in breaking down bonematrix, marking an important recognition of giant cells as functional elements in skeletal pathology.[91]
Key Advances in Understanding
In the mid-20th century, the advent of electron microscopy provided the first direct evidence that multinucleated giant cells form through the fusion of monocyte-derived macrophages, revealing ultrastructural details such as plasma membrane apposition and cytoplasmic continuity during the process.[92] This technological breakthrough, emerging in the 1950s, shifted understanding from earlier light microscopy observations to confirming cell fusion as the primary mechanism of giant cell genesis in granulomatous conditions.[61]The discovery of receptor activator of nuclear factor kappa-B ligand (RANKL) in 1998 marked a pivotal molecular advance in osteoclast biology, identifying it as the essential cytokine produced by osteoblasts that induces fusion and differentiation of monocyte precursors into multinucleated osteoclasts, thereby regulating bone resorption.[93] During the immunology era of the 1980s, research elucidated the role of cytokines like interleukin-4 (IL-4) in promoting macrophage fusion; specifically, IL-4 was shown to induce cultured monocytes to form giant multinucleated cells in vitro, highlighting its fusogenic properties in alternative macrophage activation. Concurrently, the identification of multinucleated giant cells as a hallmark of HIV-induced cytopathology in the 1980s demonstrated virus-mediated cell fusion in infected macrophages and T cells, contributing to tissue damage in AIDS.[94]In the 21st century, gene knockout studies have pinpointed key regulators of fusion; for instance, disruption of dendritic cell-specific transmembrane protein (DC-STAMP) in 2005 completely abrogated cell-cell fusion in osteoclasts and foreign body giant cells, establishing DC-STAMP as an indispensable seven-transmembrane receptor for multinucleation without affecting precursor differentiation. Recent advances include single-cell RNA sequencing analyses in 2022, which unveiled transcriptional heterogeneity among osteoclast-lineage cells, revealing distinct subpopulations with varying fusion potential and gene expression profiles during differentiation.[95] Therapeutically, bisphosphonates, introduced clinically in the 1990s, inhibit osteoclast activity by disrupting mevalonate pathways essential for giant cell function, while denosumab, approved in 2010, neutralizes RANKL to prevent fusion and bone destruction in conditions like giant cell tumor of bone.[96] Emerging structural biology in 2024 has utilized cryo-electron microscopy to resolve high-resolution structures of fusion-related proteins in macrophage lineages, addressing longstanding gaps in understanding membrane dynamics during giant cell formation.[97]