MEDIASTINAL SHIFT USING 2D ECHO PROBE

 

ABSTRACT

Giant Cell Tumor (GCT) is a rare, locally aggressive bone tumor that commonly affects young adults. The treatment of GCT often involves a multimodal approach, including surgical resection, adjuvant therapies, and, in some cases, targeted therapy. This case report describes the therapeutic modalities utilized in the management of a 26-year-old female with recurrent GCT. The patient underwent multiple surgeries, received denosumab treatment, and explored alternative Ayurvedic medications. Here, we present a case of recurrent GCT of the left distal femur in a young female and discuss the diagnostic challenges, treatment options, and the patient's response to unconventional therapy.

INTRODUCTION

Giant cell tumor (GCT) is one of the most common benign bone tumors, which occurs in young adults 20-40 years old with a high recurrence rate and a potential for aggressive behavior.[1] It is most commonly located at the metaphyseal or epiphyseal portion of the tibia or femur. While overall GCT has a benign characteristic, the disease behavior spectrum is extremely unpredictable. Local aggressiveness can range from focal symptoms due to bony or cortical destruction and surrounding soft tissue expansion and metastasis. An occurrence within the axial skeleton can lead to severe local complications and is usually unresectable.[2]

The biopsied tissue has multinucleated giant cells under the microscope. They consist of three different cell types:

1. Giant cell tumor stromal cells of osteoblastic origin
2. Mononuclear histiocytic cells
3. The multinucleated giant cell of an osteoclast-monocyte lineage.[3] 

Although benign, these tumors exhibit locally aggressive behavior and own the potential to metastasize. Around 1 to 5% of cases show metastasis and there is a positive correlation between the occurrence of metastases and local aggressiveness and recurrence.[8] Metastases most frequently occur in the lungs.[9] Varying degrees of local aggressiveness, like a simple cortical breakthrough, extension into surrounding soft tissues, and articular structures can cause severe and debilitating local complications. The risk of recurrence is approximately 35%.[10]

CASE PRESENTATION 

A 26-year-old female, born in India in 1996, achieved normal developmental milestones during childhood and had an overall pleasant upbringing. However, her medical history took a turn in 2014 when she started experiencing left knee pain towards the end of May. Initially, she attributed the pain to physical training classes and tried using topical analgesics, but found no relief. Instead, the pain worsened over time, limiting her mobility. Seeking medical attention, she underwent an X-ray that revealed a hypodense lesion, prompting further evaluation with an MRI. This MRI confirmed the presence of an abnormal growth, leading to her admission to a hospital in China.

In June, her orthopedician recommended an open cut biopsy, which revealed the presence of giant cells, indicating a diagnosis of Giant Cell Tumor. A week later, she underwent surgery, during which the affected bone was resected and replaced with cement and nails. However, postoperatively, she experienced severe and excruciating pain even with minimal movement. Upon examination, the doctors discovered that the nails had been placed close to an artery. Consequently, on the second postoperative day, she underwent a minor surgery to remove the nails, while leaving the cement in place.

Following the initial surgery, the patient continued to experience persistent discomfort in her left femur, which persisted even at rest. Due to a lack of awareness, she believed this discomfort to be a normal part of the post-surgical recovery process. However, in March 2015, the discomfort gradually worsened, prompting a follow-up visit with her orthopedician. At this appointment, the doctor suspected a recurrence of the Giant Cell Tumor but advised waiting for three months before conducting an X-ray to confirm the recurrence. Three months later, the X-ray indeed showed an increase in the hypodense lesion, confirming the recurrence. A CT scan was performed for further evaluation, including ruling out pulmonary metastases associated with Giant Cell Tumors.

Concerned about the recurrence and seeking further evaluation and management she visited a renowned Orthopedician and underwent a surgical procedure involving the removal of the cement spacer, extended curettage, cryotherapy, contra-lateral proximal fibula bone grafting, and allografting under general anesthesia. Two weeks later, the sutures were removed, and she remained under follow-up care since then.

 However, during regular follow-up visits in July 2017, X-ray changes were observed, leading the doctor to suggest trying subcutaneous injections of Denosumab every 28 days. After reviewing relevant articles and consulting long-distance doctors and patients who had undergone Denosumab treatment for Giant Cell Tumor, the patient and her doctor decided to proceed with this treatment option.

In simple terms, the hypothesis behind using Denosumab in 2016 was that it would shrink the tumor and render it inactive, making surgical resection simpler and more effective. Alternatively, to put it in more layman's terms, Denosumab would "put the aggressive lion (tumor) to sleep" and make it easier to manage.

In July 2020, the patient presented with complaints of knee pain and limited mobility. Investigations revealed a recurrence of the condition. As a result, the patient was started on Denosumab and received 12 doses until June 2021, after which the treatment was discontinued.

In August 2021, the patient sought a second opinion due to similar complaints. During this consultation, she encountered a doctor who proposed a different hypothesis regarding Denosumab. According to this hypothesis, Denosumab can conceal cells within the cortex, making them undetectable in scans and resulting in a normal histopathology report. Consequently, the recurrence rate with Denosumab is higher.

Based on the recommendations from doctors, the patient was advised to consider a prosthesis as a potential treatment option. However, before resorting to this final intervention, the patient followed her mother's advice and began trying herbal medications from Ayurveda in November 2021. As a result, her symptoms gradually subsided, and she regained complete mobility in her leg.

The patient continued using Ayurvedic treatments for one year, and follow-up X-rays indicated a gradual decrease in the size of the lesion. Eventually, in December 2022, the patient decided to gradually discontinue Ayurvedic medications.

However, in February 2023, the patient began experiencing slight discomfort, which subsequently progressed. By April, she had developed restricted motion and underwent an MRI examination to further investigate her condition.The MRI revealed a large and heterogeneous expansile osseous lesion in the distal femur, displaying various cystic areas and areas of hemorrhage within it. The lesion was located approximately 1.0 cm proximal to the articular surface of the distal femur, with cortical thinning and a suspected breach on its anterior aspect. Adjacent soft tissue components and mild joint space extension were also noted, along with synovitis and partial disruption of the medial flexor retinaculum.

On May 15th, 2023, the patient underwent surgery involving curettage, placement of bone cement, and plating, and the procedure went smoothly.

PROBLEM LIST

1) What is the etiology of GCT bone?

2) cause of recurrence

3) Prognosis of GCT bone and rate of pulmonary metastasis. which GCT patients are more vulnerable for metastatsis

4)explored management options and lack of clear cut efficacy data adding to therapeutic uncertainity
5) complications associated with gct and post operative complications

ETIOLOGY

The tumors occur spontaneously. They are not known to be caused by trauma, environmental factors, or diet. Giant cell tumors of bone are not inherited.

The exact etiology of GCT is not fully understood yet. It remains uncertain whether it is a true neoplasm or just a reactive condition. A 20q11 amplification is seen in 54% of GCTs, over-expression of p53 in 20% of them. Centrosome amplification, and boosted telomerase activity with the prevention of telomeres shortening support a neoplastic etiology.[4][5][6]

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