A single-center experience with giant cell tumors of sphenoid bone and clivus.

OBJECTIVE: To present pathologic, clinical, and treatment findings for giant cell tumors (GCTs) of sphenoid bone and clivus. METHODS: We describe the optimal treatment algorithm in patients with a histopathologic diagnosis of bone GCT by presenting the effects of denosumab treatment in both pediatric and adult patients with GCT undergoing endoscopic transnasal surgery. Clinicopathologic correlation is crucial for the differential diagnosis of GCT and the choice of treatment modality. CONCLUSION: GCT of bone is a local aggressive tumor that accounts for about 3%-7% of all bone tumors. GCTs located in the cranium are extremely uncommon neoplasms. There are no defined guidelines for the treatment of GCTs in skull base. Following surgical resection of the tumor, the addition of denosumab treatments to radiotherapy has a significant role in preventing the recurrence of GCT and in promoting regression of residual tumor size.

Is vitamin D deficiency behind the scenes for high incidence of Giant cell tumor amongst the Indian population? Unraveling the vitamin D - RANKL association.

INTRODUCTION: The major neoplastic and proliferative component of GCTB is the stromal tumor cells; that they have shown no evidence of bone destruction, instead the massive tissue destruction appears to be a result of tumor induced osteoclastogenesis. The discovery of receptor activator of nuclear factor kB (RANK) and RANK binding ligand (RANKL) uncovered the bone homeostasis and molecular mechanism by which multiple compounds (including vitamin D) regulated osteoclast differentiation; a function mediated by osteoblastic cells and osteoclast-precursor cells. HYPOTHESIS: In a country burdened by vitamin D deficiency, causal relation between hypovitaminosis D and GCTB was hypothesized based on the vitamin D mediated RANKL expression and osteoclastogenesis, as India is also a population with higher incidence of GCTB as compared to Western populations described in the literature. The possibility of vitamin D regulated osteoclastogenesis in GCTB is postulated on the evidence from molecular research linking it to the RANK/RANKL/OPG pathway. The aim of this study was to analyse the prevalence of Vitamin D deficiency in patients with primary GCTB and to elucidate any difference in serum Vitamin 25(OD)D3 levels amongst the matched control population data. MATERIALS AND RESULTS: 130 patients of primary GCTBs were matched to 310 controls from the general health check population and serum levels of 25(OH)D3 were analyzed. Statistical analysis performed on the non-parametric data and Mann Whitney U Test used to derive inference with significance set at p < 0.05. 56 females and 76 males with median Vitamin D level in the GCTB group was 15.9 ng/ml (Mean 19.41; Range 1.03 to 92) as compared to the control population with median level of 22.2 ng/ml (Mean 25.1; Range 2.6 to 87.9). The results were significant (p value < 0.05) as compared to the control population in all decades except the third decade (p value 0.0548). DISCUSSION: The differential expression of RANKL and OPG in response to levels of vitamin D has been established. The stromal cells of osteolytic GCTB express high levels of RANKL, which is a key signal regulator in development of this disease and bone destruction typical of GCTBs. This has resulted in research targeting this pathway for therapeutic approach in GCTBs. As vitamin D supplementation is simple and safe, increased awareness to assess and if necessary correct vitamin D status of patients is warranted, however the question as to whether patients with low vitamin D levels are more prone to develop GCTB and thus would profit from vitamin D supplementation remains unanswered. To conclude, it is essential to assess vitamin D levels in patients with GCTB as deficiency is pronounced. Future research on this hypothesis might lead to an association between Vitamin D deficiency and the onset/natural history of GCTB that may in the future help us cure or prevent GCTBs.

IMAGING DIAGNOSIS-THE COMPUTED TOMOGRAPHIC APPEARANCE OF A GIANT CELL TUMOR AFFECTING THE MANDIBLE IN A PYGMY GOAT.

A 3-year-old male neutered pygmy goat presented for evaluation of a progressive mandibular swelling and inappetence. A computed tomographic (CT) scan of the head and thorax was performed under general anesthesia. Computed tomography revealed an extensive multiloculated, markedly expansile lesion within the right hemimandible, which involved the articular surface of the temporomandibular joint. The goat was euthanased due to a poor prognosis and postmortem examination confirmed the diagnostic imaging findings. Histopathology was strongly suggestive of a multinucleated giant cell tumor, therefore this condition should be considered as a differential diagnosis in goats presenting with expansile mandibular mass lesions.

Giant cell tumor of distal phalanx in an adolescent with Goltz-Gorlin syndrome.

We report on a unique case of a young female patient with the Goltz-Gorlin syndrome who developed a giant cell tumor of bone in the distal phalanx of the thumb. This case is noteworthy because of the combination of some unusual features. Firstly, it is only the fifth case report on the association of giant cell tumor of bone and the Goltz-Gorlin syndrome. Also the localization of the lesion in the bones of the hand and the presentation at adolescent age is rarely seen.

Giant cell tumors of the tendon sheaths in the hand: review of 96 patients with an average follow-up of 12 years.

INTRODUCTION: Giant cell tumors (GCT) of the hand are relatively common and have a good prognosis, but the risk of recurrence is high. The goal of this study was to evaluate the long-term clinical results of a consecutive series of patients and to determine the risk factors for recurrence. MATERIAL AND METHODS: This was a retrospective study of 96 patients (57 women, 39 men) operated between February 1982 and October 2005 for GCT of the tendon sheaths in the hand. The average age at the time of the procedure was 47.7±14.5 years (range 13-75). All the patients were reviewed by an independent surgeon. The following were recorded: clinical result (QuickDASH, satisfaction), recurrence, histological appearance of tumor, location of tumor, excision margins and extension into the neighboring anatomical structures (tendon, joint). The tumor was located in the index finger in 29 cases, middle finger in 23 cases, thumb in 21 cases, ring finger in 11 cases, little finger in 11 cases, hypothenar area in two cases and thenar area in one case. In all cases, the lesion was isolated. The swelling was palmar in 27 cases, dorsal in 20 cases and medial or lateral in 59 cases. The most common joint location was the DIP joint (35% of cases). The swollen area was sensitive in 12 cases. The time from the appearance of the tumor to physician consultation ranged between 1 month and 7 years. Before the surgery, standard X-rays were taken in all patients; ultrasonography was also performed in eight patients and MRI in one patient. The tumor had an average diameter of 15.8±2.6mm (range 5-30). Histological analysis revealed a multilobed lesion with multinucleated giant cells, with or without encapsulation. RESULTS: The average follow-up at the time of review was 12.1±3.8 years (range 5-29). There were eight recurrences in seven patients (8.3%). The average time to recurrence was 2.75±2 years (range 1-6.5). In every case of recurrence, there had been intra-articular tumor development and/or tendon destruction (P<0.01). There was one functional complication: one DIP joint fusion secondary to one of the recurrences. The average QuickDASH was 2.3/100 (range 0-31). CONCLUSION: Giant cell tumors of the synovial sheaths in the hand are benign lesions where recurrence is the primary risk. The recurrence typically occurred within 36 months of the excision. Intra-articular tumor development, marginal resection and tendon involvement seem to contribute to recurrence. There was no correlation found between the histological type of tumor (encapsulated or not) and recurrence. LEVEL OF PROOF: IV.

Tumor marrom bilateral do hiperparatiroidismo primário em mandíbula: relato de caso / Bilateral brown tumor of primary hyperparathyroidism in the mandible: case report

OBJETIVOS: Apresentar um caso de envolvimento mandibular bilateral por tumor marrom do hiperparatireoidismo primário e discutir a diferenciação do tumor marrom do hiperparatireoidismo com a lesão central de células gigantes. DISCUSSÃO E CONCLUSÃO: A diferenciação é estabelecida pela confirmação do distúrbio endócrino, fator determinante no tratamento do tumor. O tratamento foi a resolução da endocrinopatia pela exérese de adenoma nas glândulas paratireoide.

OBJECTIVES: To presente a cased of bilateral mandibular involvement by a brown tumor of hyperparathyreoidism,in which the treatment consisted in resolution of the endocrinopathy by neoplasm exeresis from the parathyroid glands. DISCUSSION AND CONCLUSION: The correct differentiation of the hyperparathyroidism brown tumor from the central giant cell lesion is an essential factor to avoid diagnostic errors and unnecessary treatments.This differentiation is established by the confirmation of endocrinal disturbance, a determinant factor in the treatment of the tumor.

Giant cell tumor at tibial screw site after anterior cruciate ligament reconstruction.

We report a case of giant cell tumor that occurred in the proximal tibia of a 52-year-old man 13 years after bone-patella-bone anterior cruciate ligament reconstruction. The tumor was at the site of the metal interference screw. We discuss the differential diagnosis of proximal tibia lesions that occur after anterior cruciate ligament reconstruction and the importance of recognizing potentially life-threatening sports tumors.

Extraventricular subependymal giant cell tumor in a child with tuberous sclerosis complex.

Subependymal giant cell tumors (SGCTs) are observed in 5-20% of patients with tuberous sclerosis complex (TSC) but account for approximately 25% of neurological morbidity. The authors report the case of a 7-year-old girl with TSC and multiple cortical tubers who presented with worsening seizures in the context of the rapid growth of a cystic, calcified, extraventricular SGCT in the right frontal lobe, initially thought to represent a cortical tuber. The tumor and surrounding tubers were excised, and clinical seizures resolved. This is the first report of an extraventricular SGCT in a child with TSC outside the neonatal period.

Evidence for increased clinical severity of familial and sporadic Paget's disease of bone in Campania, southern Italy.

UNLABELLED: The analysis of 236 Italian patients with Paget's bone disease showed higher clinical severity and greater frequency of neoplastic degeneration among patients who live or descend from individuals living in the Campania region (southern Italy). A prevalent involvement of the spine and the skull, the sites preferentially involved in giant cell tumors complicating Paget's disease, was also shown in familial cases from this geographical region. INTRODUCTION: The Campania region in southern Italy has been recently indicated as a high prevalence area for Paget's disease of bone (PDB), and most pagetic families with multiple occurrence of neoplasms in affected members were from this geographical region. MATERIALS AND METHODS: We evaluated the PDB epidemiological characteristics in 125 patients from Campania in comparison with 111 patients from other Italian regions. Twenty-three patients from Campania and 26 patients from other Italian areas had at least one first-degree relative affected by PDB (familial cases). The remaining patients made up the sporadic cases. RESULTS: Among subjects from Campania, the patients in the familial group tended to come from larger families and showed at diagnosis higher serum total alkaline phosphatase, larger extension of disease, and earlier mean age with respect to patients with PDB of the sporadic group. The skull, spine, and humerus were the sites preferentially involved in the familial cases. In contrast, no such differences were observed between familial and sporadic PDB cases among patients from the other geographical areas, except for a lower age at diagnosis. An increased PDB clinical severity was finally observed in the PDB cohort from Campania in comparison with patients from other Italian regions. Neoplastic degeneration of pagetic bones (osteosarcoma and giant cell tumor) was exclusively observed in patients with polyostotic PDB from Campania. CONCLUSIONS: We showed a higher clinical severity of PDB with occurrence of neoplastic degeneration in the high prevalence area of Campania, with its maximum expression in cases with familial disease. This peculiar pattern might be traced to genetic predisposition and/or to the abnormal impact of a still undefined environmental trigger.

[Giant-cell tumors of the tendon sheath involving the hand or the wrist: an analysis of 141 patients]. / El ve el bileginde tendon kilifinin dev hücreli tümörü: 141 hastanin degerlendirilmesi.

OBJECTIVES: Giant-cell tumors of the tendon sheath localized in the hand or wrist were retrospectively reviewed with respect to diagnosis, surgical treatment, and postoperative complications. METHODS: During a 21-year period, a total of 141 patients (83 females, 58 males; mean age 37.5 years; range 6 to 77 years) underwent surgery for 146 lesions that developed in the hand (n=134) or the wrist (n=12). Involvement was on the right side in 77 patients, and on the left side in 64 patients. The mean follow-up period was 3.5 years (range 6 months to 11 years). RESULTS: On presentation, the most common symptom was the presence of a painless soft tissue mass. The most frequent localization was the volar part (76%) of the second (27%) and the third (24) fingers, or the proximal phalanx (57%). Forty per cent of tumors were encountered at ages between 30 and 50 years. The duration of symptoms ranged from one month to five years and the highest number of presentations fell within the first six months. In eighteen patients, radiologic studies showed osseous involvement, being cortical sclerosis in 12 patients, and erosion in eight patients. Postoperative complications included digital nerve injuries in four patients, superficial infection in three patients, and joint stiffness in 12 patients. Twenty-three patients (16%) developed recurrences within a mean of 3.7 years (range 2 months to 7 years). CONCLUSION: Taking high rates of recurrences into consideration, surgery for giant-cell tumors of the tendon sheath requires wide surgical exposure, attentive skills, and the use of magnification.

Tumor marrón del hiperparatiroidismo. A propósito de un caso / Maroon tumor of hyperparathyroidism. Apropos of a case

El tumor marrón del hiperparatiroidismo en los maxilares es poco frecuente. Se reporta 1 caso de una paciente tratada quirúrgicamente por lesión osteolítica maxilar. Se describen los antecedentes, la clínica y diferentes exámenes de valor diagnóstico. Se realizó diagnóstico diferencial con el tumor central de células gigantes. Se revisó el tema y se brindaron algunas consideraciones reportadas en la literatura(AU)

The maroon tumor of hyperparathyroidism is a rare tumor. The case of a female patient surgically treated due a maxillary osteolytic lesion was reported. The history, clinic and different examinations of diagnostic value are described.. A differential diagnosis was made with the central tumor of giant cells. The topic was reviewed and some considerations reported in literature were made(AU)

Malignant giant-cell tumor of the parietal bone: case report and review of the literature.

OBJECTIVE AND IMPORTANCE: Giant-cell tumors (GCTs) are primary bone tumors that involve long bones in 75 to 90% of patients. They seldom develop in the cranium and are very rare in patients older than 60 years of age. A GCT rarely occurs with Paget's disease; when it does, however, it is most commonly associated with the polyostotic form and tends to involve the craniofacial bones. Pagetic GCTs are less aggressive than GCTs that are not associated with Paget's disease. CLINICAL PRESENTATION: We report the case of an 81-year-old woman with a painless left parietal mass and asymptomatic monostotic parietal Paget's disease. INTERVENTION: Surgical resection was performed, and histological examination of the lesion demonstrated Paget's disease with a malignant GCT. An incidental, low-grade, small-cell lymphocytic lymphoma also was noted. The patient experienced local recurrence of the malignant GCT and eventually died after developing pulmonary metastases of the malignant GCT. CONCLUSION: This case is the first reported example of a patient with a malignant GCT of the cranium associated with monostotic Paget's disease. It provides evidence that not all pagetic GCTs in the cranium are benign, as has been reported.

Tumor de células gigantes / Giant cell tumor

O tumor de células gigantes do osso é uma neoplasia de malignidade indeterminada que com frequência cursa com recidiva e, em raras ocasiões, pode evoluir com metástases ou para forma poliostótica...O índice de proliferação celular não identificou outras formas evolutivas do tumor de células gigantes. Os resultados do presente estudo nos permitiu concluir que a avaliação do índice de proliferação celular através do antígeno nuclear de proliferação celular é um método limitado na definição do prognóstico do tumor de células gigantes. O tipo de evolução do tumor de células gigantes parece estar diretamente relacionado com o tipo de tratamento empregado.

Malignant giant cell tumor of the tendon sheaths and joints (malignant pigmented villonodular synovitis).

Malignant pigmented villonodular synovitis (PVNS) is a rare lesion whose existence may be debatable. We studied eight cases that we consider to be examples of malignant PVNS. The three male and five female patients were aged 12 to 79 years. The knee was involved in three cases; the ankle in two; and the cheek, dorsum of the foot, and thigh in one each. Four patients had swelling for 6 months to 17 years before presentation. Three cases of malignant PVNS were secondary, arising in patients in whom PVNS had been documented previously, and five cases were primary, with histologic features similar to those of the secondary ones. Important histologic features of malignancy were (a) a nodular, solid infiltrative pattern of the lesion; (b) large, plump, round or oval cells with deep eosino philic cytoplasm and indistinct borders; (c) large nuclei with prominent nucleoli; and (d) necrotic areas. Atypical mitoses were occasionally seen. Four patients died with pulmonary metastasis (two also had metastasis to inguinal lymph nodes). Four patients are alive from 3 1/2 to 5 years after the last surgical treatment. The malignant nature of this lesion, the histologic architecture similar to that of PVNS, and the fibrohistiocytic appearance of the cells suggest that malignant PVNS is an entity.