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Soft Tissue Pathology  

Pathology of Chordoma                 

Dr Sampurna Roy MD    

 

                                                                                                                      

 

Chordoma is a slow growing malignant bone tumour arising from the notochord remnants.

The tumour causes local destruction of the bone and may extend into the surrounding  soft tissue.

Site and presentation:

1) 50% arise from sacrococcygeal area 

2) 35% in the spheno-occipital area (base of skull).

3) 15% cervico- thoraco-lumbar (mobile) spine.

Symptoms depend on the location and are usually present for a long time.

Sacrococcygeal chordoma may present as a anterior mass and rarely as a posterior mass.

A portion of the sacrum may be destroyed. 

Retroperitoneal space is involved by direct extension of the tumour.

The enlarged tumour may compress the spinal cord, and may displace or surround the urinary bladder and large bowel.

The tumour may directly invade into the skin.

The patient always complains of pain and often present with constipation and urinary incontinence.

Spheno-occipital chordoma may present as nasal, paranasal or nasopharyngeal mass and the cranial nerves may also be involved.

Pituitary gland may be destroyed by the tumour.

Rarely, the tumour may appear as a tumour of cerebellopontine angle and acute pontocerebellar hemorrhage may occur.

Age:

The tumour usually occurs between 55 and 65 years.

Spheno-occipital tumours may occur in children. Over 60% cases occur in men.

Chondroid chordomas are more common in women.
 

Gross:

Soft, blue gray, lobulated tumour with gelationous appearance.

Focal areas of hemorrhage and areas of cystic degeneration may be present.

Tumour in the soft tissue appear to be encapsulated.

X-ray findings:

Chordoma appears as midline mass with areas of bony destruction.

An accompanying soft tissue lesion is also identified.

There are focal areas of calcification.

Chordoma at the base of the skull shows destructive, sclerotic lesion of the clivus.  

FNAB:

FNAB is a simple, rapid  and cost effective method.

Chordoma has characteristic cytological features and a correct  preoperative diagnosis is possible.

The aspirate shows large cells with pale stained vacuolated cytoplasm and ovoid nuclei. Some smaller uniform tumour cells are also present.

The cells are present in a myxoid backround.

Microscopic features: 

The tumour is composed of lobules separated by fibrous tissue.

The tumour cells are arranged in cords, columns or trabeculae.

The tumour cells are set in a basophilic to metachromatic mucinous or myxoid stroma.

There are two types of cell - small, uniform, non vacuolated cells with ovoid nuclei and larger cells with mutivacuolated or bubble like cytoplasm and vesicular nuclei.

These large cells are known as  "Physaliphorous cells" (Greek word for bubble or drop) and are diagnostic of chordomas.

Occasional mitotic figures may be identified. Some cellular pleomorphism may be present which is of no prognostic significance.

Special stains:

The physaliphorous cells are PAS positive.

The matrix is alcian blue and mucicarmine positive.

Immunohistochemistry:

The tumour cells are positive for cytokeratins, epithelial membrane antigen (EMA), S100 protein and are rarely positive for CEA .

Microscopic variants of chordoma:

Chondroid chordoma-  Shows prominent chondroid differentiation. Has a better prognosis.

Commonly located in the spheno-occipital region. Some authors consider these tumours  to be chondrosarcoma.  EMA and CEA positivity is less common in this variant.

 
Further reading:

Chordoma: an update on the pathophysiology and molecular mechanisms.

Intracranial chordoma presenting as acute hemorrhage in a child: Case report and literature review

[Chondroid chordoma--a variant of chordoma. A morphologic and immunohistochemical study.

Immunohistochemical distinction of classic and chondroid chordomas.

Chondroid chordoma. A hyalinized chordoma without cartilaginous differentiation.

Intraoperative squash and touch cytology of chondroid chordoma of the skull base.  Report of a case with immunocytochemical and immunohistochemical studies.

 

Dedifferentiated chordoma- Highly malignant biphasic tumour composed of areas of high grade sarcoma and areas of typical chordoma. Indicates poor prognosis.

Differential diagnosis: Myxoid Tumours of Soft Tissue

Chondrosarcoma: Epithelial markers are negative. There are no fibrous bands separating lobules.  Chondrosarcoma of the base of the skull: a clinicopathologic study of 200 cases with emphasis on its distinction from chordoma.Am J Surg Pathol. 1999 Nov;23(11): 1370-8.

Myxopapillary ependymoma:

Epithelial markers are negative.

Metastatic carcinoma (Example- Signet ring carcinoma, renal cell carcinoma): Lobulated growth pattern is absent.

Epithelial markers are positive like chordoma. S100 protein is negative in metastatic carcinoma.

Giant notochordal rest - These are benign remnants of notochord tissue and there is no bony destruction.

Chordoma has a high rate of recurrence. The patient may have a long disease free interval (almost 10 years) following radical resection of the tumour with excision margins free of tumour. 

Rarely, distant metastasis may occur (lungs, liver, bone , lymph node). Metastatic tumour in the skin may simulate a sweat gland tumour.

Parachordoma:
Located in the soft tissue of the lower extremities and  trunk. The tumour consists of epithelioid cells, glomoid cells and spindle cells and are cytokeratin 8/18 , S100 protein and sometimes EMA positive. CEA is negative. Parachordoma is not distinguishable from axial chordoma using immunohistochemistry.Pathol Int. 2004 May;54(5):364-70.

 

Further reading:

Clinical and pathological study of chordoma in the skull base.

Chordomas of the craniocervical junction: follow-up review and prognostic factors.

Prognostic factors in chordoma of the sacrum and mobile spine: a study of 39 patients.

Sacral chordoma: retrospective review of 11 surgically treated cases.

Cytogenetic investigation of chordomas of the skull.Cancer Genet Cytogenet. 

Cytokeratin subtyping in chordomas and the fetal notochord: an immuno histochemical analysis of aberrant expression.

A clinicopathologic review of 25 cases of chordoma (a pleomorphic and metastasizing neoplasm).

Intralesional fibrous septum in chordoma: a clinicopathologic and immuno histochemical study of 122 lesions.

Epithelioid cellular chordoma of the sacrum: a potential diagnostic problem.

Chordomas: a histological and immunohistochemical study of cases with and without recurrent tumors.

Chordomas: a histological and immunohistochemical study of cases with and without recurrent tumors.

 

 

 

Dr Sampurna Roy  MD

Consultant  Histopathologist (Kolkata - India)


 

 

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