|Year : 2016 | Volume
| Issue : 4 | Page : 284-287
Giant cell tumor of the spine masquerading as spinal tuberculosis: An uncommon entity
Karuna Jha1, Ishita Pant1, Gurbachan Singh2, Monali Raval3, Sujata Chaturvedi1
1 Department of Pathology, Institute of Human Behaviour and Allied Sciences, New Delhi, India
2 Department of Neurosurgery, Guru Teg Bahadur Hospital, New Delhi, India
3 Department of Radiology, Institute of Human Behaviour and Allied Sciences, New Delhi, India
|Date of Web Publication||15-Nov-2016|
Dr. Karuna Jha
Department of Pathology, Institute of Human Behaviour and Allied Sciences, Dilshad Garden, New Delhi - 110 095
Source of Support: None, Conflict of Interest: None
The spine is an infrequent location for giant cell tumor (GCT). The incidence of GCT in the spine, above sacrum, is only 1%-1.5%. We report a case of recurrent GCT of the lumbar spine which was clinically diagnosed as spinal tuberculosis. A 24-year-old female presented with complaints of pain in the right leg during the last trimester of her pregnancy. One month later, she complained of retention of urine along with weakness in both the lower limbs. X-ray showed destruction and sclerosis of L3 vertebra with involvement of the adjacent discs. The magnetic resonance imaging showed severe compression and collapse of L3 vertebra with diffusely altered marrow signals and an associated expansible lobulated soft tissue component. With a provisional clinical diagnosis of spinal tuberculosis, the mass was decompressed and sent for histopathological examination, where it was diagnosed as GCT of the spine. Follow-up details were not available. However, the patient presented with similar complaints 6 months later. A near total resection of the tumor was done with fixation of the spine. Histopathology confirmed it as GCT. Although rare, still GCT needs to be considered in the differential diagnosis of spinal lesions so that specific treatment modality may be provided to the patient.
Keywords: Giant cell tumor, recurrent giant cell tumor, spine
|How to cite this article:|
Jha K, Pant I, Singh G, Raval M, Chaturvedi S. Giant cell tumor of the spine masquerading as spinal tuberculosis: An uncommon entity. Int J Health Allied Sci 2016;5:284-7
|How to cite this URL:|
Jha K, Pant I, Singh G, Raval M, Chaturvedi S. Giant cell tumor of the spine masquerading as spinal tuberculosis: An uncommon entity. Int J Health Allied Sci [serial online] 2016 [cited 2020 Feb 23];5:284-7. Available from: http://www.ijhas.in/text.asp?2016/5/4/284/194136
| Introduction|| |
Giant cell tumor (GCT) of the spine is an uncommon entity. The largest of the series was published by Dahlin in the year 1977. The incidence of GCTs is 5% of bone tumors and 21% of all benign bone tumors. Involvement of the spine, above sacrum, has been documented only in 1%-1.5% of cases. However, few such cases have been reported in the past. The tumor does not figure in the differential diagnoses of a paravertebral mass with vertebral collapse and bears a challenge for the treating surgeons and orthopedicians. A young patient presenting with clinical signs of vertebral collapse and subacute spinal cord compression, the most common provisional clinical diagnosis considered in tropical countries is Pott's spine. We report here a case of recurrent GCT of lumbar vertebrae and presenting with signs of spinal cord compression and a provisional clinical diagnosis of spinal tuberculosis.
| Case Report|| |
A 24-year-old female presented with pain in the right leg during the last trimester of her gestation which did not subside even after delivery. One month later, the patient suffered from retention of urine for which she was catheterized. X-ray showed destruction and sclerosis of L3 vertebra with involvement of the disc at L2-L3 and L3-L4. Magnetic resonance imaging (MRI) showed severe compression and collapse of L3 vertebral body with diffusely altered iso-hyperintense marrow signals and soft tissue component in the right pre- and para-vertebral space extending through bilateral neural foramina into ventral epidural space causing severe compression of thecal sac and spinal canal stenosis [Figure 1]a and b. Gross total excision of the tumor was done and sent for histopathological examination. Histologically, it turned out to be a hypercellular tumor showing fair number of multinucleated osteoclastic giant cells distributed uniformly among the stromal cells [Figure 2]a and b. Histopathologically, it was diagnosed as GCT of the spine. The patient was lost to follow-up. However, she came to the surgeon with similar symptoms 6 months later. MRI showed similar picture at the L3 level with increase in the tumor bulk and complete effacement of the spinal canal [Figure 3]a and b. Chest X-ray and high-resolution computed tomography lungs were done to rule out metastasis. This time too an en bloc resection of the tumor was done with fixation of the spine. Histologically, it was confirmed as GCT again. However, this time, the stromal cells showed moderate pleomorphism at places [Figure 2]c. Mitotic figures seen were 5-6/10 high-power field [Figure 2]a - inset. Immunohistochemistry was done for p53 and it showed strong upregulation in the tumor cells [Figure 2]d. Radiotherapy was planned, but the patient did not turn up for further treatment.
|Figure 1: (a and b) Sagittal and axial T2-weigted images showing severe compression and collapse of L3 vertebral body with diffusely altered iso-hyperintense marrow signals and soft tissue component in the right pre- and para-vertebral space|
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|Figure 2: (a) Photomicrograph showing uniform spatial arrangement of multinucleated giant cells among the mononuclear stromal cells (H and E, ×100), inset shows mitosis (black arrow). (b) Photomicrograph showing nuclear similarity between giant cells and stromal cells (H and E, ×400). (c) Photomicrograph showing nuclear pleomorphism in stromal cells (H and E, ×400). (d) Photomicrograph showing p53 expression in the stromal cells and the giant cells (H and E, ×200)|
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|Figure 3: (a and b) Sagittal and axial T2-weighted magnetic resonance imaging (MRI) revealing recurrence of tumor at same level with increase in tumor bulk|
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| Discussion|| |
In developing countries such as India, the thoracic and lumbar vertebrae are a common site of spinal tuberculosis. Extraosseous involvement is often seen leading to symptoms of pain and neurologic deficits. The classic location of GCT, on the other hand, is the epiphyseal ends of long bones. When it occurs in vertebrae at sites other than sacrum, it warrants an alternate diagnosis. The patient in this case report presented with signs of radiculopathy and bladder involvement. Based on the clinical picture and considering the young age of the patient, clinically a provisional diagnosis of spinal tuberculosis was made.
The radiographic features of GCT at sites other than the long bones are nonspecific. "Soap bubble appearance" which is a classic X-ray finding of GCT was not seen in our case. Review of literature reveals that GCT of the spine is an expansile lytic lesion and almost always begins in the vertebral body. It may lead to vertebral collapse or extend into the surrounding soft tissues. On MRI, irrespective of the pulse sequence used, the tumor shows heterogeneous signal intensities.
There are few case reports which have reported the drastic growth of GCTs during pregnancy. Estrogen has been found to have both proapoptotic and antiosteolytic effect through differential action on different cell types. However, no association has yet been clarified between pregnancy and tumor growth.
Histologically, GCT is composed of the neoplastic stromal cells and the multinucleated osteoclastic giant cells. Other tumors with giant cells are aneurysmal bone cyst, chondroblastoma, benign fibrous histiocytoma, chondromyxoid fibroma, brown tumor of hyperparathyroidism, and giant cell-rich osteosarcoma. The distinctive spatial distribution of giant cells and the bland uniform stromal cells with striking nuclear resemblance to giant cells differentiates GCT from others. A higher potential for recurrence and metastasis has been suggested in tumors overexpressing TP53. Strong immunopositivity for mutated p53 protein was seen in our case too.
The true histogenesis of these tumors remains unclear as there is no consensus among the majority of authors. However, evidence favors a mesenchymal origin and it has been hypothesized that mononuclear cells are the progenitors of the giant cells that give name to the tumor.
Microscopic grading of GCTs is not found to be of great value except for the obviously sarcomatous (Grade III) lesions. Our case did not qualify for the worrisome histological grade due to only a few small areas showing pleomorphism with the absence of atypical mitoses. According to Enneking's criteria for staging, our case fell into the Stage III category as there was a large soft tissue extension and breach of the normal anatomic barriers. Review of literature mentions a similar grading system developed by Campanacci. The Grade I tumor presents well-defined margins and a thin "halo" of mature bone. The Grade II tumor is well delimited, but does not present a sclerotic halo. The Grade III lesion has ill-defined borders, suggesting aggressiveness. Our case according to this grading, too, fell into the Grade III category.
The most significant factor determining the risk of recurrence is the type of initial surgical intervention. Furthermore, increased rate of recurrences have been mentioned in Grade III lesions, where the tumor breach the normal anatomic boundaries and extend into the soft tissue. Recurrences in GCT occur within months to years, and hence, close and long-term surveillance is recommended. In our patient, it recurred within 6 months.
The incidence and potential risks of metastasis of GCT of spine are less well studied and are 13.7% according to Donthineni et al. The most common site for metastasis is lungs. Other possible sites are lymph nodes, bone, skin, and breast. The treatment of spinal GCT is not well defined as it is for the long bones. Surgery is not curative as en bloc excision with allograft replacement or curettage with bone grafting would levy grave neurologic deficits on the patient. Subtotal excision leaves the possibility of recurrence and reemergence of symptoms. Radiotherapy is an issue under debate as postradiation sarcoma is a common occurrence after the treatment.
| Conclusion|| |
Although this tumor is categorized as benign, it behaves very aggressively, has high incidence for recurrences and can metastasize. A large mass in the spinal lesion in a young female patient is usually and more frequently diagnosed as tubercular lesion in tropical countries. A differential diagnosis of GCT should also be kept in mind. An accurate pre- or intra-operative diagnosis with precise treatment is required as this tumor has been known to implant into the surrounding soft tissues during surgeries as well as cases of metastasis have been reported after a surgical intervention to the primary tumor and malignant transformation (sarcoma) following radiation therapy.
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[Figure 1], [Figure 2], [Figure 3]