Metastatic disease from chordoma

Vergara, Gloria; Belinchón, Belén; Valcárcel, Francisco; Veiras, M.; Zapata, I.; Torre, Alejandro de la

doi:10.1007/s12094-008-0243-4

Cited by 29 publications

(34 citation statements)

References 28 publications

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“…Treatment is challenging because of their unique location, close vicinity to key structures, contiguous spread and relapse. The reported incidence of metastases in the literature vary widely from 3 to 48% with the lungs, liver, bone and lymph nodes being the commonly involved sites [1][2][3][4]. We describe two cases of metastatic sacral chordoma, stable on molecular targeted therapy.…”

Section: Introductionmentioning

confidence: 93%

“…Metastases are known to occur more commonly when the primary is located in the sacrococcygeal region, compared to the clivus [1]. Metastatic disease has been reported in almost every organ: lung, liver, bone, lymph node, brain, solid abdominal organs, peritoneum and even in the breast [1][2][3][4]18]. Occurrence range of the metastases has been reported to be as early as 2-3 months [19,20] to as late as 9-10 years after initial treatment [19].…”

Section: A B E F C Dmentioning

confidence: 99%

“…Prognosis of chordomas is variable. Tumour size, inadequate surgical margins, local aggressiveness, local recurrence, presence of tumour necrosis, Ki67 labelling index and invasive diagnostic procedure outside a tumour centre, are associated with a poorer prognosis [1,21].…”

Section: A B E F C Dmentioning

confidence: 99%

“…Chordomas are slow growing, locally aggressive malignant neoplasms with a predilection for the sacrococcygeal region (50%), clivus (35%) and vertebral bodies (15%) [1]. Treatment is challenging because of their unique location, close vicinity to key structures, contiguous spread and relapse.…”

Section: Introductionmentioning

confidence: 99%

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Metastatic Chordoma: Report of the Two Cases and Review of the Literature

et al. 2015

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Chordomas are rare malignant bone tumours with a predilection for the axial skeleton, especially the sacrum and skull base. Median survival in patients with metastatic disease is usually dismal. Treatment is challenging due to the propensity for local recurrence, metastatic disease as well as lack of clear consensus regarding the optimal management. Our case report highlights two cases of sacral chordoma with locally recurrent and widespread metastatic disease, stable on molecular targeted therapy. Keywords: Chordoma, metastasis, imatinib, positron emission tomography ÖzetKordomalar özellikle sakrum ve kafa tabanı ile eksenel iskelet yerleş-me eğiliminde olan, nadir görülen malign kemik tümörleridir. Metastatik hastalığı olan hastalarda sağkalım ortanca değeri genellikle umutsuzdur. Tedavi, lokal nüks, metastatik hastalık yanı sıra optimum tedaviye ilişkin net konsensus eksikliği nedeniyle zordur. Bizim olgu sunumumuzda moleküler hedefli tedavinin istikrarlı yerel tekrarlayan ve yaygın metastatik hastalığı olan sakral kordomayla ilgili iki olgu bulunmaktadır. Anahtar Kelimeler: Kordoma, metastaz, imatinib, pozitron emisyon tomografiCorrespondence to: Saurabh Rohatgi,

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Section: Introductionmentioning

confidence: 93%

Section: A B E F C Dmentioning

confidence: 99%

Section: A B E F C Dmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Metastatic Chordoma: Report of the Two Cases and Review of the Literature

et al. 2015

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“…A total of 49% arise from sacrococcygeal, 36% from cranial, nasopharyngeal and spheno-occipital and 15% from vertebral regions 1. They can be locally destructive and tend to recur but have low potential of metastasis.…”

Section: Descriptionmentioning

confidence: 99%

A fast growing sternal mass

Demetriou

Marcou²,

Demetriou³

et al. 2013

BMJ Case Reports

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DESCRIPTIONAn 80-year-old man, presented with local relapse, bony and lung metastasis 1 year following a resection of a sacral chordoma. He required chemotherapy and several procedures for improving his neurological symptoms in the cervical, thoracic and sacral vertebrae. Chordoma was diagnosed based on the morphological and immunohistochemistry results, which showed positive CK-8, CK-19, S-100, cadherin and negative CK-7 and CK-20.At the same time an anterior 9×11×15 cm sternal mass was noticed (figures 1 and 2). The CT of the chest (figure 3) showed that it was compressing the right atrium and had multiple smaller lesions in the lungs and thoracic vertebrae. A CT of the abdomen and pelvis was normal apart from a cyst in the right kidney, which was the same size as from a previous scan. The tumour was not operated owing to the extensive bony and soft tissue destruction of the anterior chest wall and the involvement of right atrium and also because of the extent of the metastatic disease. It was therefore not biopsied.Chordoma are malignant tumours arising from the primitive notochord remnants. A total of 49% arise from sacrococcygeal, 36% from cranial, nasopharyngeal and spheno-occipital and 15% from vertebral regions.1 They can be locally destructive and tend to recur but have low potential of metastasis. However, disseminated metastatic disease has been reported in the literature and mostly occurs from primaries in sacrococcygeal region.1 The chest wall metastasis was reported only twice.2 In our case the history and image findings suggest that even though it had never been proven by histology, the possibility of sternal chordoma is most likely the case. Learning points▸ Chordomas are locally aggressive and tend to relapse. A radical resection is paramount to prevent relapse and metastasis. ▸ Despite the low metastatic potential, metastasis has still been reported, but anterior chest wall is an uncommon site.Competing interests None. Patient consent Obtained.Provenance and peer review Not commissioned; externally peer reviewed.

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Fine‐needle aspiration cytology with histological correlation of chordoma metastatic to the lung: A diagnostic dilemma

Kanthan

Senger

2010

Diagnostic Cytopathology

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Chordoma is an uncommon tumor initially believed to be benign due to the rarity of its metastasis. Cytological, morphological, and immunohistochemical features of chordoma, relating to its origin from notochordal remnants, allows for its accurate diagnosis. A 75-year-old man with a known history of tuberculosis (TB) presented with shortness of breath and a dry cough. A chest X-ray demonstrated a diffuse, infiltrative miliary pattern in both lungs. Bronchial washings submitted for culture and cytological examination did not identify any tuberculous bacilli. Fine needle aspiration cytology (FNAC) showed focal areas of myxoid regions with small, round, uniform mononuclear cells. There was a documented past history of chordoma arising from the L2 vertebrae. Because of the rarity of this lesion in the lung and the limited diagnostic material available with a clinical history of TB and lumbar chordoma, the pathological report rendered on the FNAC was "atypical cells suspicious for metastatic chordoma." A wedge biopsy of the lung confirmed the presence of metastatic pulmonary chordoma. Despite palliative treatment, he died within a year. Although the metastatic potential of chordomas has been recognized and documented, to the best of our knowledge, metastasis of chordoma to the lung diagnosed by FNAC with cytohistological correlation has not been previously reported in the English literature.

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Metastatic disease from chordoma

Cited by 29 publications

References 28 publications

Metastatic Chordoma: Report of the Two Cases and Review of the Literature

Metastatic Chordoma: Report of the Two Cases and Review of the Literature

A fast growing sternal mass

Fine‐needle aspiration cytology with histological correlation of chordoma metastatic to the lung: A diagnostic dilemma

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