Giant cell bone lesions in the craniofacial region: a diagnostic and therapeutic challenge

Abstract

Background: Giant cell tumors of bone (GCTs) are common in the long bones, but rare in the craniofacial region, with only 1% of cases occurring in the latter. Clinical, radiological, and anatomical diagnosis of this locally aggressive disease, which occurs in response to trauma or neoplastic transformation, poses a major challenge in clinical practice. Methods: The present study describes a series of 4 cases and highlights the main features of the differential diagnosis and treatment of these lesions: GCT, giant cell reparative granuloma (GCRG), and the brown tumor of hyperparathyroidism. Results: GCT presents as a benign neoplasm, most typically affecting the knees, and rarely in the temporal and sphenoid bones. It is radiologically indistinguishable from GCRG due to its lytic, poorly defined appearance. The distinction can only be made microscopically, as the presence of multinucleated giant cells scattered throughout the stroma and the absence of a history of trauma favor a diagnosis of GCT. The brown tumor of hyperparathyroidism occurs with rapid, localized osteoclast activity secondary to the effects of increased parathyroid hormone (PTH) levels; parathyroid examination is indispensable. Conclusion: The diagnosis and treatment of these lesions poses a major challenge due to their similar clinical presentation and radiological appearance. Accurate diagnosis is essential for definition of appropriate management, as complete resection is the goal in GCT and GCRG to avoid recurrence, whereas the brown tumor often yields to treatment of the underlying hyperparathyroidism.

Surgical management and long-term outcomes of intracranial giant cell tumors: a single-institution experience with a systematic review

“…At pathologic analysis, GCRG had a prominent fibroblastic stroma and small giant cells in granuloma-like arrangements around the foci of the hemorrhage. 4,26,31 Brown tumor associated with hyperparathyroidism was a nonneoplastic focal bone lesion that mostly involved the craniofacial bones, which could be differentiated because of metabolic abnormalities. 7,31 Pts = patients; RT = radiotherapy.…”

Giant Cell Tumor of the Temporal Bone With Skull Base and Middle Ear Extension

“…GCT derived from non-osteogenic stromal cells of bone marrow of endochondral bone [ 6 ]. It represents approximately 3-7% of all primary bone tumors and 20% of the benign bone neoplasms with a malignant phenotype in 5-10% of cases [ 1 , 7 ]. It is most commonly seen in the fourth or fifth decades of life with a slight female predominance with a ratio of 3:2 [ 3 , 8 ].…”

“…It is most commonly seen in the fourth or fifth decades of life with a slight female predominance with a ratio of 3:2 [ 3 , 8 ]. Usually it arises in the long bones, such as distal radius and femur and proximal tibia and fibula [ 7 ]. Skull is a rare location for GCT, with the sphenoid bone being the most common site followed by the temporal bone [ 2 , 5 ].…”

Rapid Skeletal Turnover in a Radiographic Mimic of Osteopetrosis

“…OCs accumulate in GCG (sometimes referred to as GC reparative granuloma), brown tumors from hyperparathyroidism, and GC tumor of bone (GCT). In the craniofacial region, these three lesions share clinical, radiological, and histopathologic features . Of interest, GCGs and GCTs complicate PBD with its one or more foci of rapidly remodeling bone .…”

“…In the craniofacial region, these three lesions share clinical, radiological, and histopathologic features. (40) Of interest, GCGs and GCTs complicate PBD with its one or more foci of rapidly remodeling bone. (41,42) Furthermore, we reported "extraskeletal osteoclastomas" in an African-American woman with PBD and neurofibromatosis type 1, (43) and in a Korean man with familial PBD.…”