Giant Cell Research Articles

MTOR signalling controls the formation of smooth muscle cell-derived luminal myofibroblasts during vasculitis

The accumulation of myofibroblasts within the intimal layer of inflamed blood vessels is a potentially catastrophic complication of vasculitis, which can lead to arterial stenosis and ischaemia. In this study, we have investigated how these luminal myofibroblasts develop during Kawasaki disease (KD), a paediatric vasculitis typically involving the coronary arteries. By performing lineage tracing studies in a murine model of KD, we reveal that luminal myofibroblasts develop independently of adventitial fibroblasts and endothelial cells, and instead derive from smooth muscle cells (SMCs). Notably, the emergence of SMC-derived luminal myofibroblasts—in both mice and patients with KD, Takayasu’s arteritis and Giant Cell arteritis—coincided with activation of the mechanistic target of rapamycin (mTOR) signalling pathway. Moreover, SMC-specific deletion of mTOR signalling, or pharmacological inhibition, abrogated the emergence of luminal myofibroblasts. Thus, mTOR is an intrinsic and essential regulator of luminal myofibroblast formation that is activated in vasculitis patients and therapeutically tractable. These findings provide molecular insight into the pathogenesis of coronary artery stenosis and identify mTOR as a therapeutic target in vasculitis.

Arteritic Ischemic Optic Neuropathy

Ischemic optic neuropathy is an important cause of blindness. There are two types of ischemic optic neuropathy: posterior and anterior ischemic optic neuropathy (AION). The arteritic type of AION (AAION) is usually due to giant cell arteritis. Giant cell arteritis can present with visual loss alone in up to 25% of cases. AAION is considered a neuro-ophthalmologic emergency. Early recognition and treatment of AAION is crucial to prevent severe visual loss. The gold standard of treatment is corticosteroids. There is no consensus in the literature on the optimal treatment approach in steroid-resistant or steroid-contraindicated cases. Biologic agents, especially tocilizumab, show promise in resistant cases.

News on the imaging of large vessel vasculitis

Large vessel vasculitis, including giant cell arteritis (GCA) and Takayasu arteritis (TAK), are autoimmune diseases primarily affecting the aorta and its branches. GCA is the most common primary vasculitis. Inflammatory changes in the vessel walls can cause serious complications such as amaurosis, stroke, and aortic dissection and rupture. Imaging techniques have become an integral part for the diagnosis and monitoring of large vessel vasculitis, allowing for effective disease monitoring. GCA and TAK exhibit similar patterns of vascular distribution. However, the temporal arteries are never involved in TAK, and axillary arteritis occurs more frequently in GCA. In most centers, ultrasound of the temporal and axillary arteries has replaced temporal artery biopsy as the primary diagnostic tool for GCA. In addition to ultrasound, magnetic resonance imaging (MRI), computed tomography (CT), and [18F]-FDG (fluorodeoxyglucose) positron emission tomography-computed tomography (PET) are important, particularly for visualizing the aorta. Moreover, PET-CT is now also capable of assessing the temporal arteries, although it is not yet widely available. In polymyalgia rheumatica (PMR), ultrasound of the shoulder and hip regions is part of the ACR/EULAR classification criteria. MRI allows detailed visualization of additional inflammatory extraarticular manifestations, showing characteristic inflammatory lesions in entheses, tendons, and ligaments. [18F]-FDG-PET-CT also enables the visualization of musculoskeletal inflammation, especially in the shoulder and hip regions, as well as paravertebral areas. Ultrasound can detect subclinical GCA in up to 23% of patients with PMR, which should be treated like GCA. Technological innovations such as new radiotracers and improved MRI imaging could further enhance the diagnosis and monitoring of large vessel vasculitis and PMR, thus playing acrucial role in improving the prognosis through faster initiation of therapy.

Antioxidant enzyme Prdx1 inhibits osteoclastogenesis via suppressing ROS and NFATc1 signaling pathways.

Bone is a dynamic organ which continuously undergoes remodeling throughout one's lifetime. Cellular production of reactive oxygen species (ROS) is essential for regulating bone homeostasis. Osteoclasts, multinucleated giant cells differentiated from macrophage lineage, are responsible for osteolytic bone conditions which are closely linked to ROS signaling pathways. In this study, an anti-ROS enzyme, peroxiredoxin 1 (Prdx1) was found to be expressed both in bone marrow macrophages and osteoclasts. Recombinant Prdx1 protein was found to dose-dependently inhibit ROS production and osteoclast differentiation. Mechanistically, Prdx1 protein also attenuated NFATc1 activation as well as the expression of C-Fos, V-ATPase-d2, Cathepsin K, and Integrin αV. Collectively, Prdx1 is a negative regulator on osteoclast formation via inhibiting RANKL-mediated ROS activity, thus suggesting its potential application for treating osteoclast related disorders.

Single-cell RNA-sequencing analysis of the effects of frozen-thawed embryo transfer on the transcriptome of trophoblasts

Objectives: Frozen-thawed embryo transfer (FET) is widely used in in vitro fertilization (IVF) clinics but is associated with an increased risk of several pregnancy complications, including large-for-gestational age and placenta-related diseases. However, the effects of FET on placentation remain unclear. Therefore, we used single-cell RNA-sequencing (scRNA-seq) technology to investigate the impact of FET on placental gene expression in different subtypes of trophoblasts. Methods: A mouse model of IVF and FET was constructed to collect placenta tissues. scRNA-seq was performed on placentas from two dams undergoing IVF-embryo transfer and two dams undergoing IVF-FET. Differentially expressed gene (DEG) analyses were performed in different subtypes of trophoblasts. Identified DEGs were PCR validated. Results: The fetal weights and placental efficiency were higher in the FET group than those on the IVF group at E18.5, with no significant difference in placental weights. Subsequently, 55,406 placental cells were captured and annotated. Upregulated DEGs in the FET group in syncytiotrophoblasts (SynTs) and sinusoidal trophoblast giant cells (S-TGCs) within the placental labyrinth were enriched in pathways related to vascular development and oxidative stress, respectively. The expression of the imprinted gene Igf2 in SynTs, S-TGCs, and spongiotrophoblasts was significantly increased. In the junctional zone, FET upregulated the expression of prolactin genes such as Prl3b1 in glycogen trophoblasts (GlyTs) while the downregulated expression of GlyT genes following FET was associated with mesenchyme development. Conclusion: This study first identifies DEGs and enriched pathways in different subtypes of trophoblasts following FET. These genes and pathways may contribute to the increased placental efficiency and fetal weights. Future studies are required to confirm these results and further explore the key mechanisms in placental pathologies.

Circulating Polyploid Giant Cancer Cells, a Potential Prognostic Marker in Patients with Carcinoma.

Polyploid Giant Cancer Cells (PGCCs) have been recognized as tumor cells that are resistant to anticancer therapies. However, it remains unclear whether their presence in the bloodstream can be consistently detected and utilized as a clinical marker to guide therapeutic anticancer regimens. To address these questions, we conducted a retrospective study involving 228 patients diagnosed with six different types of carcinomas (colon, gastric, NSCLC, breast, anal canal, kidney), with the majority of them (70%) being non-metastatic. Employing a highly sensitive liquid biopsy approach, ISET®, and cytopathological readout, we isolated and detected circulating PGCCs in the patients' blood samples. PGCCs were identified in 46 (20.18%) out of 228 patients, including in 14.47% of 152 non-metastatic and 29.85% of 67 metastatic cases. Patients were subsequently monitored for a mean follow up period of 44.74 months (95%CI: 33.39-55.79 months). Remarkably, the presence of circulating PGCCs emerged as a statistically significant indicator of poor overall survival. Our findings suggest that circulating PGCCs hold promise as a reliable prognostic indicator. They underscore the importance of further extensive investigations into the role of circulating PGCCs as a prognostic marker and the development of anti-PGCC therapeutic strategies to improve cancer management and patient survival.

Radiological Appearance of Brown Tumors of the Maxilla Indicative of Primary Hyperparathyroidism: A Case Report

Brown tumors are a rare complication of hyperparathyroidism resulting from abnormalities in bone metabolism. Advances in biological exploration techniques enable early diagnosis before the onset of bone manifestations. While mandibular involvement is the most common in the maxillofacial region, maxillary involvement is extremely rare. We report a case of a patient with primary hyperparathyroidism revealed by a maxillary tumor. The pathological result concluded a benign giant cell tumor of the maxilla and fibrous dysplasia. The diagnosis of a brown tumor was considered based on the association of radiological data (osteolytic image and parathyroid adenoma), histological findings (giant cell tumor), and biological markers (hypercalcemia, hypophosphatemia, and elevated Parathyroid Hormone (PTH). This case highlights the difficulty in establishing a correct diagnosis in patients with an osteolytic maxillary process and the need to investigate hyperparathyroidism in the presence of a giant cell lesion.

Giant cell Fibroma of Lip: A case report

This case report describes a rare presentation of a giant cell fibroma (GCF) on the labial mucosa of a 24-year-old female. GCF is a benign fibrous connective tissue neoplasm, typically occurring on the gingiva and tongue. The patient presented with a small, pedunculated nodule on the lower lip. A clinical diagnosis of irritational fibroma was made, and excisional biopsy confirmed the presence of GCF based on histopathological examination, revealing characteristic stellate-shaped giant fibroblasts. The lesion was successfully excised with no recurrence at the one-year follow-up. This case highlights the importance of considering GCF in the differential diagnosis of benign fibrous lesions, even in atypical locations like the labial mucosa. Increased awareness and reporting of such cases can aid in better understanding the etiology and pathogenesis of GCF, ensuring accurate diagnosis and effective management.

Phenotypes, Genetics, and Estimated Prevalence of Focal Dermal Hypoplasia (Goltz Syndrome): A Single-Center Report.

Focal dermal hypoplasia (FDH), also known as Goltz syndrome, is a rare ectodermal dysplasia that primarily affects the skin, skeleton, and eyes. It is an X-linked dominant disorder, predominantly seen in females, caused by pathogenic variants in PORCN. We characterized a case series of four genetically confirmed FDH patients (three females, one male) at Aarhus University Hospital, Denmark. We estimated the FDH prevalence from our local cohort and nationwide registry data. Three patients had characteristic dermatological findings suspicious for FDH and confirmed by targeted PORCN analysis. One patient had an atypical presentation with several malformations but only subtle skin changes and was diagnosed following trio exome-sequencing analysis. Skin atrophy with fat herniations and telangiectasias were typical cutaneous findings. Limb malformations included oligodactyly (cleft foot), syndactyly, and polydactyly. Eye abnormalities included coloboma and microphthalmos. Facial dysmorphology was defined by asymmetry, thin upper lip, and malformed ears. One patient developed a giant cell bone tumor, which is a rare feature of FDH. Dental findings included enamel hypoplasia with vertical grooving and irregular crowns. Four PORCN variants were identified, including three not previously reported in the literature.We estimated a regional point prevalence in Western Denmark of 1.6 cases per million population (95% confidence intervals (CI): 0.7-3.7 per million) and a nationwide registry-based point prevalence of 1.2 cases per million population (95% CI: 0.6-2.4 per million). FDH is an extremely rare and complex multisystem disorder of variable presentation, which requires close multidisciplinary collaboration for diagnosis and patient care.

EP94 - Occurrence of Clavicle Tumors and its Surgical Outcome

Abstract Clavicle tumors have an occurrence of around 1% of all bone tumors. They have a very limited literature regarding their features and outcome which makes the treating physician face a big diagnostic obstacle. This study aims to provide a systematic review on the clinicopathological characteristics and the management outcome. Twenty seven cases of primary clavicle tumors were studied and analyzed during 2021-2023. All cases underwent the initial investigations and were confirmed on histopathology. The Age, sex, location, type and surgical outcome was analyzed and compared. Malignant tumors were found in 55.6%of the cases, 44.4% were benign. The histopathological types in this group were osteosarcoma(9 cases),giant cell tumour(6 cases),Ewing sarcoma(5 cases), Aneurysmal Bone Cyst(4 cases), Simple bone cyst(2 cases),Mixed fibroma(1 case). Majority were located in the sternal end and the middle area was the least. Complete or Partial Claviculectomy was done in all our cases. Resection of the medial end/partial claviculectomy was done in 66.6% of the total 18 cases and 33.33% of the cases were giant cell carcinoma (6 cases), no complications found post operatively. The patients were examined for functioning and adequacy of the involved limb and the end result of all were satisfactory. No patients had any limitations in their daily activities and all patients had a full range of motion. No neurovascular deficit was found. Reconstruction of any manner was not required following claviculectomy which is supported by the above assessed report. Our study also highlights that the dispersal of tumors in the clavicle is quite different as compared to long bone tumors.

ThP8.12 - To do or not to do-Temporal artery Biopsy in Giant Cell arteritis

Abstract Aims Temporal artery biopsy (TAB) remains the gold-standard diagnostic test for giant cell arteritis (GCA). Our aim is to evaluate the correlation between initiation of corticosteroid therapy, timing of biopsy, and histological diagnosis of GCA through TAB. Moreover, analyse the usefulness of this practice. Methods Operative notes of TABs performed by a Vascular Surgery department at a teaching hospital between August 2021 and January 2023 were reviewed. We looked into the relationship between histological findings and the timing of corticosteroid treatment initiation prior to TAB. Results A total of eight patients had a TAB, with a male-to-female ratio of 2:6. The median age of the patients was 68-years-old (range 53-84). TABs were performed within 16 days of the referral (range 2-16). All were performed on elective theatre lists and none on emergency theatre lists (CEPOD). All patients had started corticosteroids by the time they had TAB, with a median duration of 11.5 days before TAB (range 2-16). The histopathology report was positive for active GCA in 1 case (12.5%) where the TAB was performed on day 3. In another performed on day 16, histopathology showed signs of healed inflammation (12.5%). In all other cases the report was negative (75%). All patients completed a full weaning course of steroids regardless of histological findings. Conclusion GCA is a systemic inflammatory vasculitis. Prompt initiation of high-dose corticosteroids is the mainstay management to reduce risks of ischaemic-related complications. Our work suggests the timing of biopsy is a key determinant of histological diagnosis of GCA.

Giant Cell Arteritis and Takayasu Arteritis: Are They Similar or Different?

Vasculitis is a group of heterogeneous conditions characterised by inflammation of blood vessels. Based on the predominant size of the affected vessel, vasculitis is classified into small, medium, and large vessel vasculitis (LVV). Giant cell arteritis (GCA) and Takayasu’s arteritis (TAK) are the two major types of LVV. GCA was thought to affect only the external carotid artery and its branches, whereas, TAK was thought to affect the aorta and its major branches. Advances in imaging techniques have led to a better understanding of these conditions. It has become increasingly clear that GCA can involve the aorta and its branches. GCA and TAK have overlapping features like propensity to affect the aorta and its branches, similar histopathology, and similar clinical features. However, there are differences in age, geographic location, genetics, and predominant vascular territory involvement. There are subtle differences in clinical features. Are these conditions different ends of the same spectrum or different conditions with similar features? This review compares and contrasts these two conditions based on epidemiology, genetics, pathology, predominant vascular territory involvement, and any differential response to treatment. Based on current evidence, GCA and TAK seem to be distinct conditions and not different ends of the same spectrum.

ThP8.7 - Giant Cell Arteries: Diagnostics

Abstract Aim Giant cell arteritis (GCA) poses a risk of severe ischaemic complications such as irreversible blindness which mandates early recognition of condition. We aimed to evaluate effectiveness of ultrasound doppler (USD) in diagnosing GCA compared to temporal artery biopsy (TAB) and American College of Rheumatology (ACR) score. Method This retrospective study included patients with suspected GCA who had consecutive TAB and USD examinations performed over a course of three years (2017-2022) which was correlated with ACR score. The data was also analysed to assess further management. Results There were 64 patients over this period who had both studies performed (22 were excluded as they did not have TAB) with 64% having an ACR score ≥3. Twenty males and forty-four females with a median age of 77 years (IQR 84-67). Overall, 81% patients had a negative USD result. Comparing USD results with TAB gold standard, there was sensitivity of 63% and specificity of 84%. Comparing USD results with ACR as gold standard, there was sensitivity of 20% and specificity of 83%. Forty percent patients with negative doppler result were continued on treatment mainly due to improvement of symptoms in response to steroids. Conclusion US Doppler has high diagnostic specificity and low sensitivity which makes it time-efficient, cost-effective and allows for the implementation of fast-track management pathway for majority patients. In certain circumstances, as cases of low clinical suspicion and positive doppler result, an additional test (TAB) would be required to confirm the diagnosis and cases where clinical suspicion is high with negative doppler, TAB should be considered.

Amitotic Cell Division, Malignancy, and Resistance to Anticancer Agents: A Tribute to Drs. Walen and Rajaraman.

Cell division is crucial for the survival of living organisms. Human cells undergo three types of cell division: mitosis, meiosis, and amitosis. The former two types occur in somatic cells and germ cells, respectively. Amitosis involves nuclear budding and occurs in cells that exhibit abnormal nuclear morphology (e.g., polyploidy) with increased cell size. In the early 2000s, Kirsten Walen and Rengaswami Rajaraman and his associates independently reported that polyploid human cells are capable of producing progeny via amitotic cell division, and that a subset of emerging daughter cells proliferate rapidly, exhibit stem cell-like properties, and can contribute to tumorigenesis. Polyploid cells that arise in solid tumors/tumor-derived cell lines are referred to as polyploid giant cancer cells (PGCCs) and are known to contribute to therapy resistance and disease recurrence following anticancer treatment. This commentary provides an update on some of these intriguing discoveries as a tribute to Drs. Walen and Rajaraman.

The impact of histopathological criteria for definite vasculitis in giant cell arteritis: retrospective analysis of temporal artery biopsies.

Histopathological findings associated with definite vasculitis in temporal artery biopsy (TAB) defined in 2022 ACR/EULAR classification criteria for Giant Cell Arteritis (GCA) was published in 2022. We aimed to evaluate the TAB of our GCA patients for histopathological findings associated with definite vasculitis. Patients who were diagnosed with GCA by clinicians and underwent TAB between January 2012 and May 2022 were included. Hospital electronic records and patients' files were reviewed retrospectively. A total of 90 patients' pathology reports were evaluated by a pathologist and a rheumatologist. In cases where microscopic findings were not specified in the pathology reports, histopathologic specimens were re-evaluated (n = 36). A standard checklist was used for histopathological findings of definite vasculitis. Patients were divided into two groups; (i) definite vasculitis-GCA and (ii) non-definite-GCA group, and the clinical and demographic characteristics for all patients were compared. The mean age of patients was 69.8 (± 8.5) years and 52.2% were female. In the first evaluation, 66 (73.3%) patients had a diagnosis of vasculitis according to pathology reports. In the re-evaluation of biopsy specimens, at least one definite finding of vasculitis was observed in TAB of 10/24 (41.6%) patients whose microscopic findings were not specified in the pathology reports. The ROC analysis showed that biopsy length had diagnostic value in predicting the diagnosis of definite vasculitis (AUC: 0.778, 95% CI: 0.65-0.89, p < 0.001). In those with a biopsy length of ≥ 1cm, sensitivity was 76.5%, specificity was 64.3%, and PPV value was 92. In multivariate analysis, the most significant factor associated with definite vasculitis was biopsy length (OR: 1.18 (1.06-1.31), p = 0.002). Microscopic findings were reported in over 70% of patients. Reinterpretation of results according to a standard check-list improved the impact of TAB in the diagnosis of GCA. A biopsy length ≥ 1cm was found to contribute towards a definitive histopathological vasculitis diagnosis.

SURVIVAL IN PATIENTS WITH HISTOLOGIC VARIANTS OF UROTHELIAL TYPE BLADDER CANCER

Objective: This study aims to determine the life expectancy of patients with histologic variants in urothelial type bladder cancer and the highest or lowest mortality rate in Hasan Sadikin Academic Medical Centre. Material &amp; Methods: A cross-sectional study with total sampling. The samples in this study were patients with histologic variants of urothelial cell carcinoma bladder who received treatment at Hasan Sadikin Academic Medical Centre during the period of 2011 to 2022. Results: A total of 470 patients with a diagnosis of urothelial bladder cancer, 62 patients (13.19%) with histologic variants of urothelial bladder cancer treated with radical cystectomy or TURBT + intravesical chemotherapy at Hasan Sadikin Academic Medical Centre. The highest survival rate was in patients diagnosed with giant cell (66.7%) followed by small cell (50%) and glandular differentiation (40.0%). The 5-yearr survival rate of patients treated with radical cystectomy alone had a higher survival rate of 75.3% followed by radical cystectomy and adjuvant therapy, which was 75.2%. Conclusion: Histologic variants of bladder carcinoma that have the highest mortality rate are tropoblastic differentiation, nested type, micropappilary, and sarcomatoid with the lowest survival rate of 0%. Also, histologic variant of bladder carcinoma that has the lowest mortality rate is giant cell with a survival rate of 66.7%. Keywords: Bladder carcinoma, chemotherapy, trophoblastic differentiation.

Perinatal HIV infection and opportunistic infectious pathology: morphological features of the placenta

Opportunistic infections account for more than 90% of all deaths associated with immunosuppression resulting from exposure to the human immunodeficiency virus (HIV). Fatal opportunistic infections include Pneumocystis pneumonia, cryptococcosis, cytomegalovirus infection, and viral hepatitis B and/or C. HIV-infected pregnant women have a high incidence of cytomegalovirus infection, which increases the risk of transplacental transmission of HIV from mother to fetus. In addition, an important factor in perinatal transmission of HIV is a genital infection caused by herpes simplex virus type 2 detected during pregnancy in HIV-infected women. Also, at present, there is no doubt about the possibility of damage to placental cells by the SARS-CoV-2 virus and its transplacental transmission.The aim of this study was to study the morphological features of the placenta in the presence of opportunistic infections caused by viruses of the herpes family (herpes simplex viruses types 1/2, cytomegalovirus, Epstein-Barr virus), as well as SARS-CoV2 in HIV-infected pregnant women.Materials and methods. A study was conducted of 21 placentas with various pregnancy outcomes in HIV-infected women, including 12 placentas obtained as a result of term birth, 1 placenta from premature birth at 29 weeks, and 8 observations of failed miscarriages (non-developing pregnancy).Results and discussion. Viral lesions were represented by the action of HIV with giant cell metamorphosis of trophoblast cells and placental macrophages, as well as infiltration by immunocompetent cells and fibrosis of the villous stroma. In addition, groups of immature villi were identified, the edematous stroma of which contained an increased number of large cells with light nuclei. In HIV-infected pregnant women with immunosuppression, the outcome of pregnancy in 8 cases was a miscarriage with a morphologically detected and immunohistochemically confirmed infection caused by herpes simplex virus types 1/2 (3 observations), cytomegalovirus (2 observations), and SARS-CoV-2 (3 observations), in 1 case the outcome of pregnancy was premature birth with morphologically identified and immunohistochemically confirmed infection caused by the Epstein-Barr virus.Conclusion. The placentas of HIV-infected pregnant women are characterized by impaired villous maturation with stromal fibrosis, which is the morphological substrate of chronic placental insufficiency with varying degrees of compensation. If HIVinfected pregnant women have opportunistic infections caused by viruses of the herpes family (herpes simplex viruses types 1/2, cytomegalovirus, Epstein-Barr virus), as well as SARS-CoV-2, pronounced involutive-dystrophic changes are observed in the placentas — perivillous deposition fibrinoid, petrification, which increases the likelihood of an unfavorable pregnancy outcome in the form of miscarriage or premature birth.

Cholesterol granuloma of the maxillary sinus: a case report

BackgroundCholesterol granuloma is not a common entity in the paranasal sinuses. It is a foreign body reaction to the cholesterol crystal deposition. Mostly associated with chronic middle ear diseases.Case presentationThis article reports a case of cholesterol granuloma in the maxillary sinus. A 23-year-old Asian man presented with cough, nasal obstruction, and postnasal discharge. On endoscopy, there was a mass protruding from the right maxillary ostium. On computed tomograpy imaging, there was a polypoidal mass in the right maxillary sinus. Endoscopic sinus surgery was performed, the cholesterol granuloma was removed from the right maxillary sinus, and the tissue was submitted for histopathological examination, which showed foreign body giant cell reaction to cholesterol crystals.ConclusionCholesterol granuloma of maxillary sinus is not common and often missed by clinicians. It is necessary to consider the cholesterol granuloma of maxillary sinus in the differential diagnosis sinonasal lesions. Histopathological analysis is required for confirmation and should be removed surgically. This case may help as a reference for clinician to approach these kinds of cases.

Revision arthrodesis in a failed knee arthrodesis for a giant cell tumor excision of distal femur: a case report

Giant cell tumor is a benign tumor with locally aggressive behaviour. It is common in 20–40 years of age group. Common location of the tumor includes distal end of femur, proximal end of tibia. It is slightly more common in females than males. We present a case of forty-four years-old lady with a persistent swelling over her left knee and was diagnosed with a distal femur giant cell tumor. After a wide excision, dual free fibula grafting and arthrodesis of the knee with a long nail was performed. However, she experienced pain and progressive deformity three years after surgery. X-rays revealed the failure of union of the free fibula graft to the residual femur and the breakage of the nail at that site. A revision arthrodesis was performed with removal of implant and stabilizing the non-united ends with dual plating with bone grafting from lateral tibial condyle and bone graft substitutes. She was discharged with left side non weight bearing mobilization with walker support and was started on full weight-bearing mobilization after one year with satisfactory union on X-rays.

Surgical management of giant cell arteritis of the proximal aorta

ObjectiveGiant cell arteritis (GCA) may present as proximal aortic pathology requiring surgical intervention. We present our experience with surgical management of GCA in patients presenting with proximal aortic disease. MethodsFrom January 1993 to May 2020, 184 adult patients were diagnosed with GCA on histopathology after undergoing cardiac surgery. Survival was estimated with Kaplan-Meier method. Reoperation rates were estimated with cumulative incidence accounting for competing risks of death. ResultsThe most common indication for surgery was ascending aortic aneurysm (n = 179, 97.3%). Stroke occurred in 6 (3.3%), pneumonia in 8 (4.4%), and dialysis in 3 (1.6%) patients. Multivariable analysis found advanced age (hazard ratio [HR], 1.054; 95% confidence interval [CI], 1.026-1.082, P < .001), recent heart failure (HR, 1.890; 95% CI, 1.016-3.516, P = .04), peripheral vascular disease (HR, 2.229; 95% CI, 1.458-3.624, P < .001), and cerebrovascular disease (HR, 1.762; 95% CI, 1.035-3.000, P = .03) as predictors of late mortality. Median follow-up was 13.7 years, and 30-day mortality was 1.5%. Nineteen patients underwent 24 aortic reinterventions including aortic arch reconstruction (n = 4), descending thoracic aorta aneurysm repair (n = 8), thoracoabdominal aortic aneurysm repair (n = 11), and pseudoaneurysm repair (n = 1). Rate of reintervention on the aorta was 3.9% (95% CI, 1.9%-8.1%), 7.1% (95% CI, 4.1%-12.3%), 12.8% (95% CI, 8.3%-19.6%), and 12.8% (95% CI, 8.3%-19.6%) at 1, 5, 10, and 15 years, respectively. ConclusionsSurgery in patients with GCA can be performed with acceptable early and late outcomes. Advancing age, heart failure, peripheral vascular disease, and cerebrovascular disease are risk factors for worse survival. Postoperative surveillance is important as need for aortic reintervention is not uncommon.