Clinicopathologic features of four rare types of chordomas, confirmed by brachyury immunostaining

Rekhi, Bharat; Banerjee, Devmalya; Ramadwar, Mukta; Bajpai, Jaya; Jambhekar, Nirmala A.

doi:10.4103/ijpm.ijpm_409_16

Cited by 10 publications

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“…Exact pathohistological diagnosis requires strict IHC analysis, taking into account the constellation for notochordal tumor chordoma. By IHC, the tumor cells are positive for CK, AE1/AE3, S-100 protein, and INI1/SMARCB1 [17]. The best immunohistochemical markers useful for the evaluation of tumors with chordoid morphology are D2-40, EMA, cytokeratin, and GFAP.…”

Section: Discussionmentioning

confidence: 99%

Clinical observations in three clinical cases with locally advanced chordomas. What is needed for early diagnosis with improved survival?

Marinova¹,

Georgiev²,

Evgeniev³

et al. 2020

J Clin Images Med Case Rep

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Chordoma is a rare malignant neoplasm. It is usually diagnosed at an advanced stage, which contributes for the poor prognosis and high mortality. In this article, we will consider three clinical cases with locally advanced chordomas in order to emphasize the importance of early diagnosis by MRI and the need for strict pathohistological and immunohistochemical analysis. Our observations reported late diagnosis, slow tumor growth, and numerous partial tumor resections, enhancing the mitotic activity of chordoma tumor cells. After numerous operations, rapid local tumor progression with aggressive infiltration of adjacent healthy tissues was observed. Hematogenous dissemination is observed after the third year of primary diagnosis and repeated surgical interventions of locally advanced chordoma. The prognosis in locally advanced chordomas is extremely unfavorable. Early diagnosis requires MRI and biopsy with exact pathomorphological and immunohistochemical analysis. To improve the treatment results, early diagnosis and complex treatment, including radical surgery and assessment of the need for high-tech postoperative radiotherapy are required.

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Section: Discussionmentioning

confidence: 99%

Clinical observations in three clinical cases with locally advanced chordomas. What is needed for early diagnosis with improved survival?

Marinova¹,

Georgiev²,

Evgeniev³

et al. 2020

J Clin Images Med Case Rep

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“…[8][9][10][11][12] Almost all cases of poorly differentiated chordomas showing brachyury immunopositivity and loss of INI1 have been reported in pediatric patients, except a single case in an adult patient, showing retained immunoexpression of INI1. 4,[8][9][10][11][12][13] Only 3 cases have been reported at extracranial sites. 8,11 The present study describes 2 rare cases of poorly differentiated chordomas, occurring in the cervical location of a middle-aged lady and a male child.…”

Section: Discussionmentioning

confidence: 99%

“…Among various IHC markers, brachyury constitutes the most specific and sensitive diagnostic marker for differentiating a chordoma from its mimics, namely, myoepithelial tumors, chondrosarcomas, and metastatic carcinomas. [1][2][3][4] Brachyury has also been useful in identification of chordomas at extra-axial sites, including some cases reported in the appendicular skeleton. [4][5][6][7] Poorly differentiated chordoma has been recently proposed as yet another aggressive subtype of chordoma, with reported cases showing distinct clinicopathologic features, including young age and loss of IHC expression of INI1/SMARCB1.…”

Section: Introductionmentioning

confidence: 99%

“…[1][2][3][4] Brachyury has also been useful in identification of chordomas at extra-axial sites, including some cases reported in the appendicular skeleton. [4][5][6][7] Poorly differentiated chordoma has been recently proposed as yet another aggressive subtype of chordoma, with reported cases showing distinct clinicopathologic features, including young age and loss of IHC expression of INI1/SMARCB1. [8][9][10][11][12] In this article, we present 2 unusual cases of poorly differentiated chordomas displaying classic histopathological and IHC features, except the occurrence of one of the tumors in an adult patient.…”

Section: Introductionmentioning

confidence: 99%

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Poorly Differentiated Chordomas Showing Loss of INI1/SMARCB1: A Report of 2 Rare Cases With Diagnostic Implications

et al. 2018

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Poorly differentiated chordomas are rare musculoskeletal tumors. Case 1. A 42-year-old lady presented with quadriparesis of 2 months' duration. Radiologic imaging disclosed a soft tissue mass in her left prevertebral- and paravertebral cervical region. Case 2. A 4-year-old male child presented with neck pain and restricted head movements of 1-year duration. Radiologic imaging revealed a contrast enhancing, paraspinal soft tissue mass in his cervical region. Microscopic examination in both the cases revealed a cellular malignant tumor composed of moderate to markedly pleomorphic cells with interspersed mitotic figures, along with focal myxoid change and necrosis. By immunohistochemistry, tumor cells in both cases were diffusely positive for pan cytokeratin (AE1/AE3) and brachyury, whereas these were negative for INI1/SMARCB1. Tumor cells in the second case were also positive for glypican3. The first case developed pulmonary metastasis, while the second case developed recurrence. Poorly differentiated chordomas are uncommon tumors, invariably characterized by loss of INI1. These tumors can be rarely seen in adults and need to be differentiated from their diagnostic mimics, in view of treatment implications and their relatively aggressive clinical outcomes.

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“…En sık görülen tipler klasik ve kondroid alt tiplerdir. Nadiren dediferansiye tip kordomalar bildirilmiştir (7,8). Yavaş seyir gösteren ve lokal invazyon yapan bu tümörlerin tedavisi cerrahidir.…”

Section: Introductionunclassified

Sacral Chordoma

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2020

Turk J Bioch

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Kordomalar, primitif notokord hücre artıklarından köken alan nadir görülen primer kemik tümörleridir. Tüm malign kemik tümörleri içinde görülme insidansları %1-4'tür. Kordomalar 40-70 yaş arasında daha sık görülse de her yaş ve cinsiyette oluşabilir. Erkeklerde nispeten daha sık görülür. Aksial aks boyunca en sık sakrokoksigeal (%50) bölgede yerleşim gösterirler. Burada 50 yaş erkek hastada sakral bölge lokalizasyonlu kordoma olgusu sunulmaktadır. Kordomalar nadir görülmeleri, sıklıkla lokal nüks göstermeleri ve hem radyolojik hem de morfolojik açıdan diğer benign ve malign neoplazmlar ile karışmaları nedeniyle önemli bir yere sahiptir.

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Clinicopathologic features of four rare types of chordomas, confirmed by brachyury immunostaining

Cited by 10 publications

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Clinical observations in three clinical cases with locally advanced chordomas. What is needed for early diagnosis with improved survival?

Clinical observations in three clinical cases with locally advanced chordomas. What is needed for early diagnosis with improved survival?

Poorly Differentiated Chordomas Showing Loss of INI1/SMARCB1: A Report of 2 Rare Cases With Diagnostic Implications

Sacral Chordoma

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