Chordoma of the Oropharynx

Abstract

Background:

Chordoma is a rare malignant neoplasm that predominantly arises from the axial skeleton, but can also develop in unusual locations. However, there are also rare cases of „NOS“ chordoma involving the oropharyx and epithelial-myoepithelial carcinoma of the parotid gland in the same patient. According to contemporary research, chordoma is a rare malignant neoplasm that arises from the embryonic remnants of the notochord. and typically involves the clivus, sacrococcygeal bones or vertebrae. Studies have shown that the incidence of chordoma has been estimated to be one per one million people per year. Chordoma can occur at any age, but most commonly it is diagnosed in the 40–60 year old age group with the male predominance.

Objective:

The aim of this article was to review the case of a 74-year-old female patient with epithelial-myoepithelial carcinoma of the parotid gland and a case of „NOS“ chordoma involving the oropharyx.

Methods:

Diagnostic methods were used to examine a female patient with two primary malignant tumors: CT neck scan, CT of paranasal sinuses, ultrasound examination, scintigraphy and operative finding.

Case presentzation:

Due to the anatomy complexity, complete resection of the tumor through a transoral-transpharyngeal approach was not possible. Intraoperative palpation of the mass revealed well defined submucosal lesion 20x43x46mm beginning at the level of the oro- and hypopharynx and extending superiorly to the nasopharynx, and posteriorly into the spinal canal and intervertebral foramen causing near complete occlusion of the oro and hypopharynx. The patient also underwent extracapsular dissection of the parotid tumor. Postoperative palliative radiotherapy was performed.

Conclusion:

Surgical treatment remains the mainstay of treatment for EMC and radiation is imperative for patients who refuse surgery and for those with advanced or inoperable diseases.

1. BACKGROUND

Chordoma is a rare malignant neoplasm that arises from the embryonic remnants of the notochord (1)., and typically involves the clivus, sacrococcygeal bones or vertebrae.

The incidence of chordoma has been estimated to be one per one million people per year (1). Chordoma can occur at any age, but most commonly is diagnosed in the 40–60 year old age group with the male predominance 2:1 (2). According to Qiong Qiong Chen et al. tumors along extra-axial locations in the head and neck are rare (3). Chordomas can develop in unusual locations, such as oropharyngeal chordomas (4). Oropharyngeal chordomas are extremly rare and only few cases are reported in the literature. On the other hand, EMC is a rare low-grade malignant tumor, accounting for <1% of all salivary gland neoplasms. EMC has predilection for the major salivary glands, especially for the parotid gland (5).

2. OBJECTIVE

We examine the case of a 74-year-old female patient with epithelial-myoepithelial carcinoma of the parotid gland acommpanied with a case of „NOS“ chordoma involving the oropharyx

3. PATIENT AND METHODS

Diagnostic methods were used to examine a female patient with two primary malignant tumors: CT neck scan, CT of paranasal sinuses, ultrasound examination, scintigraphy and operative finding.

4. CASE PRESENTATION

A 74 year old female patient was presented to our institution after a tracheotomy that was performed in the local hospital, with a history of a dysphagia, dyspnea and 15-kg weight loss during the last six months. She denied tobacco or alcohol use. In the family history her brother had laryngeal cancer. Physical examination revealed a 3 cm mass in the right parotid gland with normal overlying skin.Intraorally there was an 5 cm mass (submucosal lesion) filling the pharynx that appeared to originate from the posterior pharyngeal wall. A neck CT scan revealed a oro- and hypopharyngeal prevertebral mass 20x43x46mm that was extending into the spinal canal and intervertebral foramen causing near complete occlusion of the oro and hypopharynx.

CT scan showed also a mass 22x28x22mm in the right parotid gland. CT angiography revealed atherosclerotic plaques and stenosis in the internal carotid arteries. The patient had also had a normal CT scan of paranasal sinuses.

Due to the anatomy complexity, complete resection of the tumor through a transoral-transpharyngeal approach was not possible. Intraoperative palpation of the mass revealed well defined submucosal lesion 20x43x46mm beginning at the level of the oro- and hypopharynx and extending superiorly to the nasopharynx, and posteriorly into the spinal canal and intervertebral foramen causing near complete occlusion of the oro and hypopharynx. The patient also underwent extracapsular dissection of the parotid tumor. Postoperative palliative radiotherapy was performed.

Figure 1. CT scan revealed oro- and hypopharyngeal prevertebral mass and a mass in the right parotid gland.

Figure 2. „NOS“ chordoma involving the oropharyx and epithelial-myoepithelial carcinoma of the parotid gland.

5. DISCUSSION

Chordomas are rare, but aggresive bone tumors representing 1% of all malignant bone tumors (6) and affect rather male patients than a female patients,while children and adolescents are rarely affected (<5% of cases) (7).

First described chordoma was in 1857 by German physician and pathologist Rudolf Virchow who noted on autopsy tumor on the surface of the clivus (dorsum sellae) (7).

Chordoma is categorized into three types: conventional chordoma, chondroid chordoma and dedifferentiated or sarcomatoid chordoma ( which confers the worst prognosis).

Histology of chordoma was first described by Rudolf Virchow as a physaliphorous (i.e., vacuolated or bubble-bearing) mass.

Reviewing a literature we found 15 reported cases of chordoma from the period 1959-2021 detailed in Table 1.

Table 1. Cases of chordoma from the period 1959-2021.

Study	Sex	Age	Location
Essammaa E 1959	M	35	Oropharynx
Burge AJS 1975	M	68	Posterior pharyngeal wall of the orpharynx
D Hazarika et al 1995	N/A	N/A	Nasopharyngeal mass extending into the oropharynx
Gladstone HB et al 1998	M	81	Parapharyngeal space
UB Makhmudov et al 1998	F	58	Oropharynx
Succo G et al 2001	F	66	From the posterior oropharyngeal wall to the hypopharynx
WangYP et al 2004	F	50	Right posterior wall of the oropharynx
Koybasioglu F et al 2005	M	50	Left posterior aspect of the oropharynx
Coppens JR et al 2009	F	32	Nasopharynx
Coppens JR et al 2009	F	8	Nasopharynx
Coppens JR et al 2009	F	5	Nasopharynx
Vasudevan V et al 2015	F	73	Left parapharyngeal area
Khurram SA et al 2016	M	63	Right tonsillar region
Xiang Li et al 2017	M	32	Left oropharyngeal mass
Chi-Yuan Yeh 2021	F	63	Nasopharynx with extension to the oropharynx

Female being more affected 8 patients or (57,14 %) than male 6 patients or (42, 85 %) and there is no data available for one patient. Avarage age was 45,6.

Children and adolescents are rarely affected and we found only two cases in the pediatric age with the localisation in the nasopharynx. Surgical excision is the mainstay of treatment for chordomas., however complete resection is very difficult because of the anatomical complexity (6). Chordomas are radioresistant, but for incomplete excision radiation is valuable (1). Very high doses of radioation are required to control these tumors. Also with new radiation modalities it is possible to deliver high doses of radioation while avoiding surrounding tissue (1). Chemotherapy is ineffective in chordoma, but might be an option for the patients with advenced or inoperable disease.

We believe that surgery remains the treatment mainstay of chordoma, and postoperative radiotherapy is valuable, especially those with subtotal and partial resection. However, because of rarity of chordoma, more studies should be performed to entirely explain the nature of the chordoma (4). EMC is also a rare tumor, with a recurrence of 40% after excision according to Poonam Rani (8). It is interesting to note that in the period between 1993-2010 there were only 246 cases of salivary gland epithelial-myoepithelial carcinomas reported in the study Vazquez et al. (5). Because of aggressive behavior of EMC metastasis can occur to lymph nodes, lung, liver, but distant metastases have been rarely reported.

A study by Gore MR in 2018 highlighted that the age at diagnosis, T,N,M stage and treatment type affected survival (9).

However, no case of double primary cancer, oropharyngeal chordoma and epithelial-myoepithelial carcinoma of the parotid gland was reported in the literature (10).

6. CONCLUSION

Overall, we conclude that it is still a great challenge to find adequate anticancer therapy that covers both cancer types, without negative impact on the final outcome .

Surgical treatment is the mainstay of treatment for EMC and radiation is valuable for patients who refuse surgery and for those with advences or inoperable disease.

Further research in the area of chordoma is definitely needed, especially when referring to the areas of the treatment of patients with multiple primary cancers.

Patient Consent Form:

All participants were informed about subject of the study.

Author’s contribution:

A.R. and M.K. gave substantial contributions to the conception or design of the work in acquisition, analysis, or interpretation of data for the work. M.M, L.T., and E.S. had a part in article preparing for drafting or revising it critically for important intellectual content. A.R, M.K., and S.U. gave final approval of the version to be published and agreed to be accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved.

Conflicts of interest:

There are no conflicts of interest.

Financial support and sponsorship:

None.