Answer for BIR CoW 01 July 2018
CHORDOMA
Findings
T1 and T2 heterointense lesion with cystic areas noted involving clival region with extension into nasopharynx and pituitary region. Pituitary gland could not be separately visualised. The lesion causes compression of lateral and third ventricles with dilatation of frontal horn of lateral ventricle The lesion infiltrates the optic tract with partial suppressibility in FLAIR.The lesion extends into body of sphenoid and the lesion causes compression effect on midbrain and pons.
Discussion
⦁ Chordomas are malignant tumours of the axial skeleton ⦁ 1% of intracranial tumours and 4% of all primary bone tumours. ⦁ originate from embryonic remnants of the primitive notochord ⦁ extradural ⦁ local bone destruction ⦁ locally aggressive ⦁ uncommonly metastasise. ⦁ AGE DISTRIBUTION AND LOCATION ⦁ Any age ⦁ usually seen in adults (30-70 years). ⦁ spheno-occipital region in patients 20-40 years of age ⦁ sacrococcygeal chordomas in a slightly older age group ⦁ They are slow growing tumours ⦁ mass effect on adjacent structures (brainstem, cranial nerves, nasopharynx, spinal cord), or palpable mass (e.g. sacrococcygeal chordoma) PATHOLOGY ⦁ Macroscopically, present as firm masses. ⦁ Fluid and gelatinous mucoid substance (associated with recent and old haemorrhage) and necrotic areas are found within these tumours. ⦁ calcification and sequestered bone fragments In some patientS ⦁ Microscopically- characterised by physaliphorous cells. ⦁ often poorly marginated and microscopic distal extension of tumour cells likely explains the frequency of recurrences. ⦁ Three subtypes are recognized : ⦁ conventional chordoma (most common) ⦁ chondroid chordoma (best prognosis) ⦁ dedifferentiated chordoma (least common, worst prognosis) ⦁ LOCATION ⦁ Chordomas are found along the axial skeleton and a relatively evenly distributed among three locations: ⦁ sacrococcygeal: 30-50% ⦁ spheno-occipital: 30-35% ⦁ vertebral body: 15-30% ⦁ RADIOGRAPHIC FEATURES: ⦁ CT evaluation is needed to assess the degree of bone involvement and to detect patterns of calcification within the lesion. ⦁ MRI provides excellent anatomical delineation of adjacent structures and is able to characterise the signal of the lesion usually allowing for a confident preoperative diagnosis ⦁ CT ⦁ centrally located ⦁ well-circumscribed ⦁ destructive lytic lesion, sometimes with marginal sclerosis ⦁ expansile soft-tissue mass ⦁ usually hyper-attenuating relative to adjacent brain; however, inhomogenous areas may be seen due to necrosis or haemorrhage ⦁ soft-tissue mass is often disproportionately large relative to the bony destruction ⦁ irregular intratumoral calcifications (thought to represent sequestra of normal bone rather than dystrophic calcifications) ⦁ moderate to marked enhancement ⦁ MRI ⦁ T1 ⦁ intermediate to low-signal intensity ⦁ small foci of hyperintensity (intratumoral haemorrhage or a mucus pool) ⦁ T2: most exhibit very high signal ⦁ T1 C+ (Gd) ⦁ heterogeneous enhancement with a honeycomb appearance corresponding to low T1 signal areas within the tumour ⦁ greater enhancement has been associated with poorer prognosis . ⦁ SWI/GE: variable intralesional haemorrhage, suggested by the presence of blooming artefact ⦁ DWI/ADC ⦁ conventional chordoma: 1474 ± 117 x 10-6 mm2/s ⦁ dedifferentiated chordoma: 875 ± 100 x 10-6 mm2/s DIFFERENTIAL DIAGNOSIS ⦁ For clival/spheno-occipital lesions : ⦁ chondrosarcoma ⦁ plasmacytoma ⦁ meningioma ⦁ pituitary macroadenoma ⦁ benign notochordal cell tumour ⦁ For vertebral lesions: ⦁ Chondrosarcoma ⦁ Giant cell tumour ⦁ Spinal metastasis ⦁ Plasmacytoma ⦁ Spinal lymphoma ⦁ TREATMENT ⦁ surgical resection - first line of treatment ⦁ Radiotherapy – recurrence ⦁ Percutaneous radiofrequency ablation - trialled as an adjunct therapy ⦁ PROGNOSIS: ⦁ Metastatic spread of chordomas in 7-14% of patients- includes nodal, pulmonary, bone, cerebral or abdominal visceral involvement. ⦁ Prognosis is poor due to the locally aggressive nature of these tumours ⦁ overall 10-year survival of approximately 40%. ⦁ Histologically, ⦁ chondroid chordoma -best prognosis ⦁ dedifferentiated chordoma -worst prognosis; ⦁ conventional chordoma -intermediate prognosis
Findings
T1 and T2 heterointense lesion with cystic areas noted involving clival region with extension into nasopharynx and pituitary region. Pituitary gland could not be separately visualised. The lesion causes compression of lateral and third ventricles with dilatation of frontal horn of lateral ventricle The lesion infiltrates the optic tract with partial suppressibility in FLAIR.The lesion extends into body of sphenoid and the lesion causes compression effect on midbrain and pons.
Discussion
⦁ Chordomas are malignant tumours of the axial skeleton ⦁ 1% of intracranial tumours and 4% of all primary bone tumours. ⦁ originate from embryonic remnants of the primitive notochord ⦁ extradural ⦁ local bone destruction ⦁ locally aggressive ⦁ uncommonly metastasise. ⦁ AGE DISTRIBUTION AND LOCATION ⦁ Any age ⦁ usually seen in adults (30-70 years). ⦁ spheno-occipital region in patients 20-40 years of age ⦁ sacrococcygeal chordomas in a slightly older age group ⦁ They are slow growing tumours ⦁ mass effect on adjacent structures (brainstem, cranial nerves, nasopharynx, spinal cord), or palpable mass (e.g. sacrococcygeal chordoma) PATHOLOGY ⦁ Macroscopically, present as firm masses. ⦁ Fluid and gelatinous mucoid substance (associated with recent and old haemorrhage) and necrotic areas are found within these tumours. ⦁ calcification and sequestered bone fragments In some patientS ⦁ Microscopically- characterised by physaliphorous cells. ⦁ often poorly marginated and microscopic distal extension of tumour cells likely explains the frequency of recurrences. ⦁ Three subtypes are recognized : ⦁ conventional chordoma (most common) ⦁ chondroid chordoma (best prognosis) ⦁ dedifferentiated chordoma (least common, worst prognosis) ⦁ LOCATION ⦁ Chordomas are found along the axial skeleton and a relatively evenly distributed among three locations: ⦁ sacrococcygeal: 30-50% ⦁ spheno-occipital: 30-35% ⦁ vertebral body: 15-30% ⦁ RADIOGRAPHIC FEATURES: ⦁ CT evaluation is needed to assess the degree of bone involvement and to detect patterns of calcification within the lesion. ⦁ MRI provides excellent anatomical delineation of adjacent structures and is able to characterise the signal of the lesion usually allowing for a confident preoperative diagnosis ⦁ CT ⦁ centrally located ⦁ well-circumscribed ⦁ destructive lytic lesion, sometimes with marginal sclerosis ⦁ expansile soft-tissue mass ⦁ usually hyper-attenuating relative to adjacent brain; however, inhomogenous areas may be seen due to necrosis or haemorrhage ⦁ soft-tissue mass is often disproportionately large relative to the bony destruction ⦁ irregular intratumoral calcifications (thought to represent sequestra of normal bone rather than dystrophic calcifications) ⦁ moderate to marked enhancement ⦁ MRI ⦁ T1 ⦁ intermediate to low-signal intensity ⦁ small foci of hyperintensity (intratumoral haemorrhage or a mucus pool) ⦁ T2: most exhibit very high signal ⦁ T1 C+ (Gd) ⦁ heterogeneous enhancement with a honeycomb appearance corresponding to low T1 signal areas within the tumour ⦁ greater enhancement has been associated with poorer prognosis . ⦁ SWI/GE: variable intralesional haemorrhage, suggested by the presence of blooming artefact ⦁ DWI/ADC ⦁ conventional chordoma: 1474 ± 117 x 10-6 mm2/s ⦁ dedifferentiated chordoma: 875 ± 100 x 10-6 mm2/s DIFFERENTIAL DIAGNOSIS ⦁ For clival/spheno-occipital lesions : ⦁ chondrosarcoma ⦁ plasmacytoma ⦁ meningioma ⦁ pituitary macroadenoma ⦁ benign notochordal cell tumour ⦁ For vertebral lesions: ⦁ Chondrosarcoma ⦁ Giant cell tumour ⦁ Spinal metastasis ⦁ Plasmacytoma ⦁ Spinal lymphoma ⦁ TREATMENT ⦁ surgical resection - first line of treatment ⦁ Radiotherapy – recurrence ⦁ Percutaneous radiofrequency ablation - trialled as an adjunct therapy ⦁ PROGNOSIS: ⦁ Metastatic spread of chordomas in 7-14% of patients- includes nodal, pulmonary, bone, cerebral or abdominal visceral involvement. ⦁ Prognosis is poor due to the locally aggressive nature of these tumours ⦁ overall 10-year survival of approximately 40%. ⦁ Histologically, ⦁ chondroid chordoma -best prognosis ⦁ dedifferentiated chordoma -worst prognosis; ⦁ conventional chordoma -intermediate prognosis
Note:
We do not discourage differential diagnosis. But all the differentials must satisfy the findings noted in the case.
If you feel you have answered rightly but cannot find your name in the above list, please call 09551942599.
Did you Know?
The order in which the names appear in this winner's list is based on the time of submission. The first person to send the correct answer gets his/her name on top of the list!
We do not discourage differential diagnosis. But all the differentials must satisfy the findings noted in the case.
If you feel you have answered rightly but cannot find your name in the above list, please call 09551942599.
Did you Know?
The order in which the names appear in this winner's list is based on the time of submission. The first person to send the correct answer gets his/her name on top of the list!