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Setting
This case is from Masanga hospital, a rural training hospital located in the jungle of central Sierra Leone. The hospital has four physicians Global Health and Tropical Medicine, six community health officers (of which two are surgically trained), two midwives, sixty nurses and around ten clinical health officers who follow the local surgical or paediatric training programme. The hospital has 120 beds and provides healthcare for about 12,500 patients every year (inpatient and outpatient department). It is one of the few hospitals that provides trauma care on a large scale in the rural area and therefore receives a lot of referrals from other hospitals. The local infrastructure is very poor, while around 440,000 people in the widespread area are dependent on this hospital for healthcare.
Specialist advice
The specialists of the Consult Online panel thought of a giant cell carcinoma. Other potential diagnoses could be a bone cyst or fibrous dysplasia. It should be noted that the differential diagnosis, in a case like this with little information and without additional diagnostic tests, can be broad. The panel agreed that the clinical findings, course of the disease and X-ray were not suspect for an osteosarcoma (in which faster growth and a sunburst aspect and spiculae on the X-ray would be expected). The process on the X-ray was described as benign (at most low grade malign) and the panel advised a biopsy with a sharp spoon and histological analysis. The therapy advice for a benign tumor like this would be to scrape out the bone and fill it with autologous spongiosa bone (for instance from the pelvis), and then place an external fixator to prevent fractures. The panel advised this operation to be performed by an experienced surgeon to spare the ulnar and median nerves and thereby the function of the hand.
Figure 1 The patient at presentation with a large swelling at the ulnar side of the right wrist
Figure 2 The X-ray of the right wrist
| Case A 23-year-old man presents at the hospital with a swelling of the right wrist (Figure 1). It originated a few months earlier and slowly increased in size. It was not preceded by any trauma. On clinical examination a round, hard swelling with varying surface is palpable on the ulnar side of the right wrist. No enlarged lymph nodes can be palpated. An X-ray of the wrist shows an expansive lytic lesion of the distal ulnar bone of the right wrist (Figure 2). A chest X-ray was performed and showed no abnormalities. A biopsy was not performed right away because of lack of the patient’s financial means and because there is only one pathologist in the whole country. Based on the clinical findings and x-ray the medical team made a differential diagnosis of an osteosarcoma or benign bone cyst and consulted the Consult Online panel for help. On clinical examination a round, hard swelling with varying surface is palpable on the ulnar side of the right wrist. The swelling does not feel warm, and the function of the hand is normal. |
Giant cell tumor of the bone
A giant cell tumor of the bone (GCTB) is a rare, often benign, osteolytic skeletal neoplasm characterized by a myriad of giant osteoclast-type cells. Although it is non-cancerous it can be locally aggressive and lead to destruction of surrounding tissue.[1,2]
GCTB represents 3-5% of primary bone tumors and 15-20% of benign tumors in the ‘western’ world.[1,3] Studies estimate an incidence rate of 1.03-1.7 per million persons per year.[2,3] The frequency of occurrence of GCTB seems to be higher in the Chinese population, where GCTB represents roughly 20% of all primary bone tumors.[1]
GCTB usually occurs after the closing of the epiphyseal plates and mostly affects young adults between the age of 20-40.[1,3] Described by the radiologist of the consult online panel as: An expansive bone lesion of (unknown) cm in the distal ulna, with a predominantly osteolytic matrix, some ground glass areas and no obvious calcifications. Some thin septae and cortical thinning is seen. Females are slightly more often affected than males.[1,2,3] An occurrence of the disease before the age of 20 is uncommon. The disease most often presents solitary, although multicentric GCTB has been seen in younger individuals.[1] Risk factors of the disease and the exact pathogenesis is not yet fully understood.[1]
GCTB is a relatively slow growing tumor that most commonly affects the meta-epiphysis of the long bones. It is often seen around the knee (proximal tibia, distal femur) and wrist (distal radius) but can present in almost all bony parts of the body.[1,3,4] Patients frequently present with pain, swelling and reduced joint movement leading to mechanical difficulty.[3,4] Destruction and thereby thinning and weakening of bone can often lead to pathologic fractures.[1,4] Distant metastases of GCTB can occur and are seen in less than 5% of cases and most frequent to the lungs.[1,3] The clinical behavior of these metastases is different from metastases derived from malignant tumors and does not often lead to death of the patients.[1] A primary malignant form and malignant transformation, although very rare, exist and have poor prognosis.[1,3]
The diagnosis is based on clinical findings in combination with medical imaging (X-ray, CT or MRI) to assess the extent of the disease and a biopsy for histopathologic evaluation.[1,2] The Campanacci grading system (table 1) can be used to categorize the extent of the lesion based on radiographic imaging, and could be helpful in determining definitive treatment.[1,4]
Table 1: The Campanacci grading system for giant cell tumor of the bone. [1]
| GRADE I | Intraosseous lesions with well-marginated borders and an intact cortex. |
| GRADE II | More extensive intraosseous lesions having a thin cortex without loss of cortical continuity. |
| IIa: without pathologic fracture | |
| IIb: with pathologic fracture | |
| GRADE III | Extraosseous lesions that break through the cortex and extend into soft tissue. |
Surgery is the treatment of choice.[1,4] Surgical treatment options depend on localization and extent (size of the tumor, extent to surrounding tissue and the existence of a pathologic fracture) and range from local intralesional (extended) curettage to en-bloc resection with or without reconstruction.[1] Risk of recurrence in benign GCTB varies in the literature (6-42%) en is more often seen with simple curettage.[1,2] This risk, in combination with morbidity and postoperative functional outcomes, should be considered before surgical treatment.[1] For patients with a contraindication to surgery, non-surgical treatment options include radiotherapy, arterial embolization or systemic therapy with denosumab, but have shown limited success.[1,3] The latter is often not possible in low-resource settings.
Follow up
A short while after the first consultation, the patient returned to the hospital with a pathology report. It turned out he had been to a hospital in the capital Freetown a few months earlier, where a biopsy of the swelling was performed and sent to India for analysis. The report showed a biopsy result of a giant cell tumor. A conservative approach was agreed upon and the patient was sent home. When an experienced trauma- orthopaedic- or plastic surgeon will visit the hospital the plan for an operation as advised by the panel will be further discussed. It must be noted that an operation with autologous spongiosa transplantation can be very challenging in a low-resource setting like this. After this last consultation the patient was not yet seen again.
Described by the radiologist of the consult online panel as: An expansive bone lesion of (unknown) cm in the distal ulna, with a predominantly osteolytic matrix, some ground glass areas and no obvious calcifications. Some thin septae and cortical thinning is seen.
References
- Thomas DM, Desai J, Damron TA. Giant cell tumor of bone [Internet]. UpToDate 2022 Available from: https://www.uptodate.com/contents/giant-cell-tumor-of-bone#H974489649
- Verschoor AJ, Bovée JVMG, Mastboom MJL, et al. Incidence and demographics of giant cell tumor of bone in The Netherlands: First nationwide Pathology Registry Study. Acta Orthop. 2018 Oct;89(5):570-574.
- Amelio JM, Rockberg J, Hernandez RK, et al. Population-based study of giant cell tumor of bone in Sweden (1983-2011). Cancer Epidemiol. 2016 Jun;42:82-9. doi: 10.1016/j.canep.2016.03.014. Epub 2016 Apr 6. PMID: 27060625.
- Raskin KA, Schwab JH, Mankin HJ, et al. Giant cell tumor of bone. J Am Acad Orthop Surg. 2013 Feb;21(2):118-26. doi: 10.5435/JAAOS-21-02-118. PMID: 23378375.
