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An unusual case of oropharyngeal chordoma: A case report and literature review.

Abstract Chordomas are rare malignant neoplasms derived from incomplete regression of notochordal tissue along the cranio-coccygeal axis. Chordomas that develop in an atypical position are called ectopic chordomas, such as oropharyngeal chordomas (OCs). OCs are exceedingly rare; only 11 cases have been reported to date. Preoperative diagnosis is challenging, and an accurate diagnosis thus is based on postoperative pathologic examination findings and immunohistochemistry. Although surgical therapy and radiotherapy is performed in some patients, the 5-year survival rate is low. Increasingly more studies of chordomas have been based on molecular biology to increase the survival rate, and targeted therapy could be a new therapy in the future.
PMID
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Chordoma.

Authors

Mayor MeshTerms
Keywords
Journal Title medicine
Publication Year Start




PMID- 29310398
OWN - NLM
STAT- MEDLINE
DCOM- 20180116
LR  - 20180116
IS  - 1536-5964 (Electronic)
IS  - 0025-7974 (Linking)
VI  - 96
IP  - 48
DP  - 2017 Dec
TI  - An unusual case of oropharyngeal chordoma: A case report and literature review.
PG  - e8963
LID - 10.1097/MD.0000000000008963 [doi]
AB  - RATIONALE: Chordomas are rare malignant neoplasms derived from incomplete
      regression of notochordal tissue along the cranio-coccygeal axis. Chordomas that 
      develop in an atypical position are called ectopic chordomas, such as
      oropharyngeal chordomas (OCs). OCs are exceedingly rare; only 11 cases have been 
      reported to date. Preoperative diagnosis is challenging, and an accurate
      diagnosis thus is based on postoperative pathologic examination findings and
      immunohistochemistry. Although surgical therapy and radiotherapy is performed in 
      some patients, the 5-year survival rate is low. Increasingly more studies of
      chordomas have been based on molecular biology to increase the survival rate, and
      targeted therapy could be a new therapy in the future. PATIENT CONCERNS: The
      patient presented with a left oropharyngeal mass that had begun slowly enlarging 
      1 year previously. He reported a foreign body sensation and dysphonia during this
      time period. DIAGNOSES: The patient was initially diagnosed with a neurogenic
      tumor. Routine postoperative pathology showed that the mass was consistent with a
      chordoma. INTERVENTION: Mass resection was performed. OUTCOME: One year after the
      initial surgery, magnetic resonance imaging revealed block signal images at the
      left retropharyngeal space and clivus. The patient developed recurrence of the
      OC. LESSONS: Surgical resection is the mainstay of treatment for OC, and
      postoperative adjuvant radiotherapy is also important. An understanding of the
      unusual case described in this report may be helpful in diagnosing OC, and
      development of targeted therapy may help clinicians to provide novel treatment
      for patients with OC.
FAU - Li, Xiang
AU  - Li X
AD  - Department of Oral and Maxillofacial Surgery, Peking University Shenzhen
      Hospital, Shenzhen, Guangdong Province.
AD  - Graduate Department, Anhui Medical University, Hefei, Anhui Province, P.R. China.
FAU - Wang, Yufan
AU  - Wang Y
AD  - Department of Oral and Maxillofacial Surgery, Peking University Shenzhen
      Hospital, Shenzhen, Guangdong Province.
FAU - Wang, Feng
AU  - Wang F
AD  - Department of Oral and Maxillofacial Surgery, Peking University Shenzhen
      Hospital, Shenzhen, Guangdong Province.
FAU - Li, Bowen
AU  - Li B
AD  - Department of Oral and Maxillofacial Surgery, Peking University Shenzhen
      Hospital, Shenzhen, Guangdong Province.
AD  - Graduate Department, Anhui Medical University, Hefei, Anhui Province, P.R. China.
FAU - Sun, Shuai
AU  - Sun S
AD  - Department of Oral and Maxillofacial Surgery, Peking University Shenzhen
      Hospital, Shenzhen, Guangdong Province.
AD  - Graduate Department, Anhui Medical University, Hefei, Anhui Province, P.R. China.
FAU - Yang, Hongyu
AU  - Yang H
AD  - Department of Oral and Maxillofacial Surgery, Peking University Shenzhen
      Hospital, Shenzhen, Guangdong Province.
LA  - eng
PT  - Case Reports
PT  - Journal Article
PT  - Review
PL  - United States
TA  - Medicine (Baltimore)
JT  - Medicine
JID - 2985248R
SB  - AIM
SB  - IM
MH  - Adult
MH  - Chordoma/*diagnosis/pathology/*surgery
MH  - Humans
MH  - Male
MH  - Oropharyngeal Neoplasms/*diagnosis/pathology/*surgery
MH  - Oropharynx/diagnostic imaging/pathology/surgery
MH  - Recurrence
PMC - PMC5728799
EDAT- 2018/01/10 06:00
MHDA- 2018/01/18 06:00
CRDT- 2018/01/10 06:00
PHST- 2018/01/10 06:00 [entrez]
PHST- 2018/01/10 06:00 [pubmed]
PHST- 2018/01/18 06:00 [medline]
AID - 10.1097/MD.0000000000008963 [doi]
AID - 00005792-201712010-00078 [pii]
PST - ppublish
SO  - Medicine (Baltimore). 2017 Dec;96(48):e8963. doi: 10.1097/MD.0000000000008963.