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Aggressive giant cell tumour (GCT) of the distal humerus in a young pregnant woman
  1. Raju Vaishya1,
  2. Abhishek Vaish1,
  3. Nishant Pawar1 and
  4. Pankaj Baweja2
  1. 1Orthopaedic & Joint Replacement Surgery, Indraprastha Apollo Hospital, New Delhi, Delhi, India
  2. 2Pathology, Indraprastha Apollo Hospital, New Delhi, Delhi, India
  1. Correspondence to Dr Abhishek Vaish; drabhishekvaish{at}gmail.com

Abstract

A 25-year-old pregnant woman reported suffering from an aggressive giant cell tumour of the distal humerus extending into the elbow joint. The successful management required a careful evaluation of the various treatment options due to her pregnancy. Surgical excision of the mass was performed. The residual bone defect was reconstructed with an elbow mega-prosthesis. The patient made a pain-free recovery with a good range of motion and delivered the baby at term with no complications.

  • Orthopaedics
  • Cancer - see Oncology
  • Obstetrics and gynaecology
  • Pathology

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Background

Giant cell tumours (GCT) of the bone are reported during pregnancy, and the presence of such a tumour in these cases poses several challenges in their management. A rapid progression of such lesions during pregnancy is documented, though not common.1 Due to the probable adverse effects of drugs such as denosumab and bisphosphonates, these agents are not used for treatment during pregnancy.2 3 Hence, the aggressive GCT may require surgical management in such cases, as it is a relatively safer option. We report a rare case of a 25-year-old woman with an aggressive GCT of the distal humerus presenting in the third trimester of pregnancy, which was treated successfully by surgery.

Case presentation

A 25-year-old woman presented to us at 31 weeks of pregnancy with pain and swelling of the left elbow for 5 months. The pain was increased significantly in the last month due to which she had difficulty in moving the left elbow and upper limb and required support for this limb. The size of the swelling had also increased substantially in the past few weeks. There was a diffuse, firm to boggy swelling around the left elbow joint and tenderness, at the local examination. The patient did not allow movements of the limb because of severe pain. There was no neurovascular deficit.

Investigations

Plain radiographs revealed a large expansile, lytic lesion around the left elbow. There was associated soft-tissue involvement, anteriorly and posteriorly (figure 1A,B). MRI revealed a large expansile osteolytic lesion in the left distal humerus, extending up to the articular surface. An extensive cortical breach with extraosseous soft-tissue swelling (7×6.4×5.6 cm) was present on the medial and lateral aspect of the elbow joint. An altered intensity signal was found around the left distal humerus that extended approximately 6 cm distal to the radial head (figure 2A–D). These findings suggested an aggressive GCT of the left distal humerus, with a possible extension into the elbow joint. A high-resolution CT (HRCT) of the lungs did not show any evidence of metastasis. However, the patient did not agree to take another HRCT after the pregnancy in the postoperative period. All biochemical parameters were normal, including the parathyroid hormone.

Figure 1

Antero-–posterior and lateral radiographs of the elbow showing a large lytic lesion with cortical breach in the distal humerus.

Figure 2

MRI of the elbow showing the extent of the lesion, soft-tissue involvement.

Because the tumour was soft and there was a risk of bursting and spillage of the tumour cells, a core biopsy (due to its thicker needle) was not preferred as it might have ruptured the tumour. Instead, a fine-needle aspiration cytology was performed. It showed cellular smears consisting of clusters of round to oval spindle-shaped stromal cells with oval to spindle nuclei and a moderate amount of cytoplasm. Frequent mitotic figures and sheets of giant cells rimming the clusters of stromal cells were found. These findings were suggestive of an aggressive GCT of bone (GCTB).

Differential diagnosis

An aggressive GCT in a young adult should be differentiated from a chondroblastoma, telangiectatic osteosarcoma, giant-cell-rich osteosarcoma, clear cell chondrosarcoma and brown tumour of hyperparathyroidism.4

Treatment

An en block resection of the lesion and management of the defect with an elbow mega-prosthesis was planned. This decision was taken due to the aggressiveness of the lesion and the inability to use the medical treatment (bisphosphonates and denosumab) to contain the disease. The patient was counselled about the requirement of multiple revision surgeries in the future, as an inherent complication of the mega prosthesis in young and active individuals. Informed consent was obtained. Two doses of 12 mg intramuscular betamethasone were administered preoperatively to minimise the risk of fetal respiratory distress, and a fetal cardiotocography was performed to supervise the fetal heart rate.

A right lateral position was used for the surgery. The surgery was carried out under general anaesthesia, using the posterior approach to the distal humerus and elbow joint. First, the ulnar nerve was identified and preserved. The distal humerus was excised 3 cm above the proximal extent of the tumour using an oscillating saw. Further dissection of the peritumourous tissue was carried out in a proximal to distal direction. The tumour did not involve the proximal radius and ulna, but it had extended into the elbow joint. Adjoining muscles and neurovascular structures were not involved. An encapsulated mass with boggy consistency was removed and sent for histopathological and cytological examination. The resultant defect after excision of the mass was reconstructed (figure 3A,B), using a cemented tumour mega prosthesis (Restor TM, Smith and Nephew). No intraoperative complications related to the mother or the fetus were encountered.

Figure 3

Intraoperative photographs of the elbow showing the prosthetic elbow joint.

Gross pathological examination of the excised tumour showed an expansile lesion of the distal humerus 4.8 cm from the proximal resection margin. The cut surface was soft with focal areas of haemorrhage. Extension of the tumour, up to 1 mm, into the surrounding soft-tissue and muscle was noted (figure 4).

Figure 4

Gross appearance of the resected distal humerus, showing an expansile lesion of the distal humerus.

The microscopic examination revealed a well-circumscribed lobulated encapsulated tumour, with a variable portion of mononuclear cells and osteoclast-like giant cells. The stroma was well vascularised, with focal areas of haemorrhage. In addition, there was hyalinisation along with the presence of haemosiderin and xanthomatous histiocytes. There was some infiltration into the surrounding adipose tissue and skeletal muscles, but no evidence of lymphovascular invasion, significant mitosis, necrosis or pleomorphism (figure 5). The immunohistochemistry was negative for both the oestrogen (ER) and progesterone receptors (PR). Due to the non-availability of the GCTB specific markers, such as Histone H3.3 Glycine 34 mutation marker, these tests could not be undertaken.

Figure 5

Microscopic examination of the resected tumour, showing variable portion of mononuclear cells and osteoclast-like giant cells, and the vascularised stroma.

Outcome and follow-up

The postoperative radiographs showed a well-aligned elbow prosthesis. The wound healed with primary intention and the elbow pain resolved within 2 weeks. The elbow was put in a splint for 3 weeks, followed by a sling for another 3 weeks. Gradual mobilisation of the elbow under the supervision of a physiotherapist was started after 3 weeks of the surgery. Weight lifting was prevented for 6 weeks and was then incrementally increased. The patient delivered the baby at term without any complications. A follow-up examination at 1 year revealed a pain-free elbow with a good range of motion (figure 6A,B) and no evidence of prosthetic loosening (figure 7A,B). Till now, there was no evidence of local recurrence.

Figure 6

Follow-up clinical Photographs showing a pain-free and good range of motion of the operated elbow.

Figure 7

Follow-up radiographs (antero–posterior and lateral views), at 1 year, showing a well-aligned megaprosthesis of the elbow, with no evidence of loosening. intellectual property rights assignment or licence statement.

Discussion

GCTs are osteolytic lesions of bone, with 50% of the tumours occurring around the distal femur or proximal tibia and are rare around the elbow joint.5 The distal humerus is not a common site for bone tumours, and the reported incidence of primary bone tumours in the distal humerus is about 1%. About 4% of GCT occurs in the humerus, but these almost exclusively involve the proximal humerus.6 We could not find a similar case of GCT in distal humerus in a pregnant woman in the literature.

The most common location of a GCT in a pregnant woman is the sacrum. It is also reported that these tumours behave more aggressively during pregnancy as these lesions may be hormonally regulated. The GCT may express ER or PR, and elevated levels of these hormones could potentially influence the tumour cells.7 8 It is proposed that the aggressive nature of such lesions could be due to the resemblance of the oncofetal antigens of tumour cells with the fetal antigens.9 However, in our case, the immunohistochemistry was negative for hormonal receptors.

No definite guidelines exist for the treatment of GCT occurring during pregnancy. Since these tumours are slow-growing and locally aggressive, prompt management is required. Medical management of GCT during pregnancy cannot be done since bisphosphonates cannot be administered as they cross the placenta, while denosumab is contraindicated during pregnancy. It is believed that the bisphosphonate administration during pregnancy may lead to low birth weight, transient hypocalcaemia and fetal mal-development. Similarly, the use of denosumab may be associated with fetal morbidity, stillbirths and decreased growth. Animal studies suggest that its use is also associated with lactation and lymph node abnormalities.10 11 Hence, surgery is a safer alternative option. But, it requires careful monitoring of the maternal and fetal status. Therefore, such cases are managed best by multidisciplinary teamwork. Following a wide surgical excision, the various options for limb management include arthrodesis or reconstruction using allograft or a custom prosthesis. Allograft reconstructions are associated with a high failure rate, and the reconstruction using elbow prosthesis is associated with good function and fewer complications. However, the use of a prosthesis is associated with the risk of aseptic loosening, infection, dislocation and periprosthetic fractures. Hence, these cases are likely to need multiple revision surgeries in the future, especially in young and active individuals like our cases.12 13

We encountered several challenges in the surgical management of this case. Due to the risk of premature birth, steroids are given to ensure lung maturation 24–48 hours before the surgery. It may impair the maternal immune system, and there is a risk of postoperative infection. Positioning of the patient was another concern since the prone position could not be used due to pregnancy, and the right lateral position was chosen. The latter is associated with its drawbacks due to compression of the inferior vena cava, leading to reduced venous return and the risk of thromboembolism.14 The use of general anaesthesia reduces the risk of preterm labour and hence was preferred in this case. Regional anaesthesia is an alternative technique used due to reduced risk of exposure of the fetus to anaesthetic drugs, no need for intubation and postoperative pain management.15 Patient and fetal monitoring is carefully required for maternal oxygenation, perfusion and haemostasis. Systemic arterial blood pressure monitoring is recommended during the surgery and was done in this case. Although the teratogenic effect of methylmethacrylate cannot be ruled out, there exists no compelling scientific evidence on the pregnancy-related complications of bone cement.15 Postoperatively, a meticulous observation is needed to prevent infection. The safety profile of antibiotics should be evaluated before administration. Denosumab can be safely administered after delivery and during lactation, and we would consider its use if the disease shows any signs of recurrence.16

Patient’s perspective

I had severe pain and swelling in my left elbow for 5 months, and it was a massive shock to find out that it was because of a tumour. Finding out the diagnosis was hard because I was worried about my baby and also because the tumour was aggressive. The doctors reassured me and explained everything in detail, after which I agreed to undergo surgery. I am very pleased with the elbow movements after a period of regular physiotherapy. I was able to deliver the baby at term without any complications.

Learning points

  • Giant cell tumour (GCT) of the bone is rare in the distal humerus.

  • This rare case of an aggressive GCT of the distal humerus coexisted in a pregnant woman.

  • Surgical management was required to avoid the adverse effects of denosumab and bisphosphonates.

  • Surgical excision and prosthetic replacement offer a good range of motion and functional outcomes. However, this may lead to morbidity in younger, active patients, owing to high functional demands requiring multiple revision surgeries in the future.

  • Effective teamwork between the surgeon, anaesthetist and obstetrician is crucial for maternal and fetal well-being.

Ethics statements

Patient consent for publication

References

Footnotes

  • Contributors RV: concept, editing the manuscript. AV: concept, research, editing and submitting. NP: research, writing of manuscript, editing. PB: pathology workup, provided slides, research and methods.

  • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

  • Case reports provide a valuable learning resource for the scientific community and can indicate areas of interest for future research. They should not be used in isolation to guide treatment choices or public health policy.

  • Competing interests None declared.

  • Provenance and peer review Not commissioned; externally peer reviewed.