|Year : 2015 | Volume
| Issue : 3 | Page : 645
Metastatic carcinoma of breast or a chordoma? A case report and clinical perspectives
Sachin Trivedi1, Karel Odrazka2
1 Department of Oncology, Charles University Teaching Hospital, Hradec Kralove, Czech; Royal Marsden Hospital, Sutton, Surrey, UK
2 Department of Oncology and Radiotherapy, Charles University Teaching Hospital, Hradec Kralove, Czech Republic
|Date of Web Publication||9-Oct-2015|
Department of Oncology, Charles University Teaching Hospital, Hradec Kralove, Czech; Royal Marsden Hospital, Sutton, Surrey, UK
Source of Support: None, Conflict of Interest: None
We present a case of chordoma in a patient who had been previously treated for ductal carcinoma of the breast. The initial clinical findings and radiological studies suggested a possibility of metastases. However, the findings also adhered to the classical presentations and findings of the chordoma of the base of skull. It was only after the surgical resection and immunohistochemical confirmation that the diagnosis of chordoma could be established. Here, we discuss chordoma with the analysis of our clinical intrigue.
Keywords: Chordoma, central nervous system metastasis, ductal carcinoma breast
|How to cite this article:|
Trivedi S, Odrazka K. Metastatic carcinoma of breast or a chordoma? A case report and clinical perspectives. J Can Res Ther 2015;11:645
| > Introduction|| |
Chordoma is a rare tumor of the remnant of notochord. It occurs at the base of skull in about half of the all cases. These tumors can present with various neurological symptoms including those associated with the cranial nerves. A surgical resection and histopathological confirmation is required to establish the diagnosis. Immunohistochemistry of chordoma shows positive cytokeratin, epithelial membrane antigen and S-100 protein.
| > Case report|| |
A 71-year-old female was diagnosed to have T1N0M0 ductal carcinoma of the upper outer quadrant of the right breast. Histochemically, it was found to be Oestrogen receptor positive and HER-2 negative tumor. Subsequently the tumor was treated with partial mastectomy, adjuvant radiotherapy of 48.6 Gy in 27 fractions and tamoxifen. Following the treatment, patient showed no signs of residual disease and recovered well.
About 30 months later, she presented with pain in her left ear, over the jawbone, pain over the left side of face and over the left angle of mandible and worsening dysarthria. These symptoms worsened over a period of 3 weeks. She did not have any history of trauma or of neurological pathology. The personal history and the history of other illness were insignificant.
On examination, the vital functions appeared to be normal. There were no signs of herpes, inflammation or of any other neurological deficit. The higher functions were well-preserved. There were no signs of any other systemic disease. The reports of otoscopy, larnygoscopy, nasal endoscopy and oral examination didn't reveal any pathology. The hematocrit, liver functions tests, calcium levels, urea and electrolytes were all within normal ranges.
After a thorough examination, she was referred to a Neurologist who confirmed the somatosensory problems along the branches of V, VII and the XII nerve. The Neurologist ruled out the infectious pathology and a computed tomography (CT) scan of the head was requested.
The CT showed an osteoclastic lesion at the base of skull along the anterior margin of foramen magnum. There was a mass of about 35 mm in size growing toward the nasopharynx. The lateral compartment showed asymmetry and the lesion indicated the possibility of metastases from the previous malignancy of the breast. A subsequent magnetic resonance imaging (MRI) showed osteolytic lesion (50 mm) of the clivus with an extraosseous portion (25 mm) in the anterior epidural space [Figure 1] and [Figure 2]. Bone scan did not reveal a lesion in other parts of skeleton. She was urgently referred to the neurosurgeons.
On surgery, the tumor was found to be of about 4 cm in size and it extended behind clivus. The tumor mass had a slimy and slippery texture that resembled a chordoma than a metastatic lesion. The immunohistochemical study showed it was positive for epithelial membrane antigen, cytokeratin and S-100 protein. The histopathology corroborated the conclusions of gross pathology and the final diagnosis of chordoma was established.
|Figure 1: Magnetic resonance imaging (MRI) brain 1. Horizontal section of brain of patient with a history of carcinoma of the breast. Following a subsequent MRI showed osteolytic lesion (50 mm) of the clivus with an extraosseous portion (25 mm) in the anterior epidural space. Initially, it was suspected to be a metastasis. The immunohistochemical study of the lesion showed it was positive for epithelial membrane antigen, cytokeratin and S-100 protein. The diagnosis of chordoma was established|
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|Figure 2: Magnetic resonance imaging (MRI) brain 2. Sagittal section of the brain of a patient with history of Carcinoma of the breast. Following a subsequent MRI showed osteolytic lesion (50 mm) of the clivus with an extraosseous portion (25 mm) in the anterior epidural space. Initially, it was suspected to be a metastasis. The immunohistochemical study of the lesion showed it was positive for epithelial membrane antigen, cytokeratin and S-100 protein. The diagnosis of chordoma was established|
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After the surgery, patient had to undergo radiotherapy for the possible residual disease. At the time of writing this report, patient was free of her neurological symptoms and was recuperating in the wards.
| > Discussion|| |
Chordoma is a rare tumor that arises from the remnant of primitive notochord. The incidence of this tumor among the intracranial primary tumor is 0.5%.  The male to female incidence is 2:1.  This tumor does not seem to have preponderance in any fixed age group. These occur along the midline with about 50% occurring around the sacrococcygeal area and about 30-40% at the basisphenoid area.
The clinical presentation of chordoma of the base of skull is complex. Symptoms can vary from a mass in the neck, tinnitus, hearing loss and other cranial nerves associated symptoms to pain. The CT reveals an area on destruction with clear confines. 
Metastases and ecchordosis physaliphora are important differential diagnoses of chordoma. Ecchordosis physaliphora is an aggressive non-neoplastic lesion and a possible heterogenic remnant of the notochord without intrinsic growth potential. , However, in our patient, the progressive character of symptoms indicated an active pathology. The other differential diagnoses are myxopapillary ependymoma and chondrosarcoma and have to be ruled out histologically and by immunohistochemical studies.  There have been case reports of dural metastasis and associated subdural hematoma  and a similar condition could have given rise to progression of symptoms in our patient, but this ruled out by radio diagnosis.
The diagnosis of chordoma is established by the histopathology and immunohistochemical studies. Macroscopically, it appears as a mass that has slimy and slippery texture. It is soft in consistency and is variable in size. Histopathology shows the presence of typical physaliferous cells in mucin-rich stroma.  Immunohistochemical characteristics show typical co-expression of epithelial markers (cytokeratins, MNF 116, Cam 5,2), Epithelial Membrane Antigen, S-100 protein and neuron-specific enolase. 
The effective treatment of chordoma involves a multidisciplinary team effort. Early clinical suspicion and investigation are paramount. Surgery seems to be an effective and first mode of treatment of chordoma. Radiotherapy is delivered after the surgical removal of the tumor. The medical management using the inhibition of the epidermal growth factor pathway by cetuximab and gefitinib seems to be effective, but needs further studies. ,
The brain is a common site of metastases of breast cancer and has been a difficult problem for clinical management  and isolated brain metastasis can be the only manifestation of relapse of carcinoma breast.  Besides the brain, spinal cord compression is the second most common neurological complication of carcinoma of the breast.  In our patient, the chances of brain stem metastasis and the metastases into the cerbellopontine angle were possibilities that had to be ruled out.
The previous history of a breast cancer, the higher chances of relapse of the breast cancer with central nervous system (CNS) metastases, the misleading osteoclastic lesion, the rare occurrence of chordomas, the reports of association of some CNS tumors with carcinoma of the breast and the progressive symptoms requiring an urgent decision made this an intriguing case. In cases with isolated mass in these typical locations along with the CT and MRI findings, the chordoma should be added to the list of differential diagnoses.
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[Figure 1], [Figure 2]