Abstract
Benign Notochordal Cell Tumour of vertebra: The navigation of diagnostic challenges through radiological and histological pathways
D. Grabovska1, A. Balodis1, I. Štrumfa2, I. Liepniece-Karele2, K. Bičevska1, E. Birkenfelds1
1. Pauls Stradins Clinical University Hospital, Institute of Diagnostic Radiology, Riga, Latvia, 2. Pauls Stradins Clinical University Hospital, Department of Pathology, Riga, Latvia
Introduction
Benign notochordal cell tumour (BNCT) is a rare bone tumour that arises from notochordal remnants [1]. BNCT commonly occurs in the sacrum and clivus, but it can also occur in the cervical and lumbar spine [2,3]. These tumors are often incidental findings on unrelated imaging studies. BNCT’s significance and management are still under investigation, with the rise in cases linked to increased use of MRI [4]. Although typically benign, their histological characteristics can resemble more aggressive tumours such aschordomas or chondrosarcomas, complicating diagnosis and treatment [5]. BNCT and chordoma often occur in similar locations, and occasionally co-exist. Some suggest that BNCT could be a precursor to chordoma, but this is debated due to limited evidence [6,7,8,9,10].
Case report
A 16-year-old girl reported pain in the lumbar-sacral region. To find the cause, a CT scan of the lumbar spine was performed, showing heterogeneous sclerotic changes in the S1 vertebra (Fig.1). Subsequent contrast-enhanced MRI showed centrally located sclerotic lesion in the S1 vertebra with a hyperintense signal on T2, hypointense on T1 (Fig.2). Imaging features indicated fibrous dysplasia, although the differential diagnosis included osteoid osteoma or another benign lesion. The patient had no neurological deficits, external abnormalities, or relevant medical history of secondary illnesses or cancer. A biopsy was inconclusive, prompting a repeat open biopsy for further examination. A static skeletal scintigraphy did not show evidence of osteoblastic changes in the bones (Fig.3). A follow-up MRI showed a persistent high-signal lesion in the S1 vertebra without contrast enhancement (Fig.4,5) ruling out malignant bone tumours. A repeat biopsy revealed calcified fragments and bone trabeculae. An open biopsy with L1 laminoplasty confirmed BNCT diagnosis after a two-year evaluation (Fig.6,7,8). The follow-up CT scan showed no significant changes (Fig.9).
Discussion
Differentiating BNCT from aggressive tumors requires comprehensive assessment. Histologically, BNCT presents vacuolated cells with bland, round nuclei resembling adipocytes or brown fat cells, often requiring immunohistochemistry for definitive diagnosis [5,12,13]. Unlike chordomas, BNCT lacks specific features like extracellular myxoid matrix and osteolysis. Radiological differentiation can be challenging. CT scans may show sclerotic changes, but MRI is preferred for its soft tissue contrast. BNCT generally has low to intermediate signal on T1-weighted images and high signal on T2-weighted images. The lack of contrast enhancement helps distinguish BNCT from malignant tumours [11,14].
Conclusion
This case underscores the diagnostic complexities of BNCT, highlighting the importance of histological and radiological examinations with crucial multidisciplinary care. Collaborative efforts and documentation of similar cases will further refine treatment approaches for this rare bone tumour.
D. Grabovska1, A. Balodis1, I. Štrumfa2, I. Liepniece-Karele2, K. Bičevska1, E. Birkenfelds1
1. Pauls Stradins Clinical University Hospital, Institute of Diagnostic Radiology, Riga, Latvia, 2. Pauls Stradins Clinical University Hospital, Department of Pathology, Riga, Latvia
Introduction
Benign notochordal cell tumour (BNCT) is a rare bone tumour that arises from notochordal remnants [1]. BNCT commonly occurs in the sacrum and clivus, but it can also occur in the cervical and lumbar spine [2,3]. These tumors are often incidental findings on unrelated imaging studies. BNCT’s significance and management are still under investigation, with the rise in cases linked to increased use of MRI [4]. Although typically benign, their histological characteristics can resemble more aggressive tumours such aschordomas or chondrosarcomas, complicating diagnosis and treatment [5]. BNCT and chordoma often occur in similar locations, and occasionally co-exist. Some suggest that BNCT could be a precursor to chordoma, but this is debated due to limited evidence [6,7,8,9,10].
Case report
A 16-year-old girl reported pain in the lumbar-sacral region. To find the cause, a CT scan of the lumbar spine was performed, showing heterogeneous sclerotic changes in the S1 vertebra (Fig.1). Subsequent contrast-enhanced MRI showed centrally located sclerotic lesion in the S1 vertebra with a hyperintense signal on T2, hypointense on T1 (Fig.2). Imaging features indicated fibrous dysplasia, although the differential diagnosis included osteoid osteoma or another benign lesion. The patient had no neurological deficits, external abnormalities, or relevant medical history of secondary illnesses or cancer. A biopsy was inconclusive, prompting a repeat open biopsy for further examination. A static skeletal scintigraphy did not show evidence of osteoblastic changes in the bones (Fig.3). A follow-up MRI showed a persistent high-signal lesion in the S1 vertebra without contrast enhancement (Fig.4,5) ruling out malignant bone tumours. A repeat biopsy revealed calcified fragments and bone trabeculae. An open biopsy with L1 laminoplasty confirmed BNCT diagnosis after a two-year evaluation (Fig.6,7,8). The follow-up CT scan showed no significant changes (Fig.9).
Discussion
Differentiating BNCT from aggressive tumors requires comprehensive assessment. Histologically, BNCT presents vacuolated cells with bland, round nuclei resembling adipocytes or brown fat cells, often requiring immunohistochemistry for definitive diagnosis [5,12,13]. Unlike chordomas, BNCT lacks specific features like extracellular myxoid matrix and osteolysis. Radiological differentiation can be challenging. CT scans may show sclerotic changes, but MRI is preferred for its soft tissue contrast. BNCT generally has low to intermediate signal on T1-weighted images and high signal on T2-weighted images. The lack of contrast enhancement helps distinguish BNCT from malignant tumours [11,14].
Conclusion
This case underscores the diagnostic complexities of BNCT, highlighting the importance of histological and radiological examinations with crucial multidisciplinary care. Collaborative efforts and documentation of similar cases will further refine treatment approaches for this rare bone tumour.
Original language | English |
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Pages | 1 |
Number of pages | 1 |
Publication status | Published - 8 Jun 2024 |
Event | European Musculosceletal Radiology congress 2024 - Lugano, Switzerland Duration: 6 Jun 2024 → 8 Jun 2024 https://www.essr.org/congress/ |
Congress
Congress | European Musculosceletal Radiology congress 2024 |
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Abbreviated title | ESSR |
Country/Territory | Switzerland |
City | Lugano |
Period | 6/06/24 → 8/06/24 |
Internet address |
Keywords*
- musculosceletal Radiology
- Notochordal cell tumor
- Histological pathways
Field of Science*
- 3.2 Clinical medicine
Publication Type*
- 3.4. Other publications in conference proceedings (including local)