|Year : 2019 | Volume
| Issue : 4 | Page : 382-385
Nasopharyngeal carcinoma presenting as muscular atrophy – A rare case report and role of oral physician
Ravinder Singh1, Amit Aggarwal1, Deepak Gupta1, Samita Singal2
1 Department of Oral Medicine and Radiology, Maharishi Markandeshwar College of Dental Sciences and Research, Mullana, Ambala, Haryana, India
2 Radiodiagnosis, Consultant, Punjab, India
|Date of Submission||09-Mar-2019|
|Date of Acceptance||16-Aug-2019|
|Date of Web Publication||03-Mar-2020|
Dr. Amit Aggarwal
Department of Oral Medicine and Radiology, Maharishi Markandeshwar College of Dental Sciences and Research, Mullana, Ambala, Haryana
Source of Support: None, Conflict of Interest: None
| Abstract|| |
The presenting features of nasopharyngeal carcinoma (NPC) are varied and very few cases present with symptoms unless advanced stages of NPC are present. The disease is one of the most baffling, difficult to diagnose, and abjectly understood entities because of the location of the involved area. The role of oral physician is difficult to specify in this disease except that sometimes patients report to them for facial symptoms. We are presenting a rare case in which the facial muscles were atrophied on one side with mild facial pain which on advanced radiological imaging and histopathology turned out to be nasopharyngeal carcinoma. The role of oral physician becomes critical in diagnosing cases of nasopharyngeal carcinoma which present with no symptoms or few symptoms related to the disease.
Keywords: Imaging, muscular atrophy, nasopharyngeal carcinoma, oral physician
|How to cite this article:|
Singh R, Aggarwal A, Gupta D, Singal S. Nasopharyngeal carcinoma presenting as muscular atrophy – A rare case report and role of oral physician. J Indian Acad Oral Med Radiol 2019;31:382-5
|How to cite this URL:|
Singh R, Aggarwal A, Gupta D, Singal S. Nasopharyngeal carcinoma presenting as muscular atrophy – A rare case report and role of oral physician. J Indian Acad Oral Med Radiol [serial online] 2019 [cited 2020 Jun 4];31:382-5. Available from: http://www.jiaomr.in/text.asp?2019/31/4/382/279864
| Introduction|| |
Nasopharyngeal carcinoma (NPC) is a unique malignancy that arises from the epithelium of the nasopharynx and has a restricted prevalence in certain regions of the world. NPC shows the highest incidence rate in the Southeast China, Southeast Asia, North Africa, Middle East, and the Arctic regions, but it is rare in most other parts of the world with less than 1:100000 incidence., NPC is uncommon in the Indian subcontinent except in the North-eastern part of the country. It has been reported that the NPC has bimodal incidence that emerged across low-risk populations, irrespective of geographic location. There is a continual increase in NPC risk by age up to a first peak in late adolescence or early adulthood, ages 15–24 years, followed by a second peak later in life, age 65–79 years.
Nasopharyngeal Carcinoma seems to occur due to a multi-factorial process as well as investable corollary in which it involved the contributions of Epstein Barr Virus (EBV), ethnic background, and environmental carcinogens. Other known factors that contribute to the variation in epidemiological distribution have been dietary preferences rich in nitrosamines, such as preserved foods and salted fish.
The clinical presentation of NPC depends on tumor size and extent as well as cervical nodal metastasis. Small lesions may be asymptomatic. Adenopathy is most often the initial complaint because of cervical metastatic spread. The early presenting features of NPC are given as: A lump in neck caused by a swollen lymph node, blood in saliva, nasal congestion, epistaxis, dysphagia, hearing loss, frequent ear infections, trismus, hoarseness, proptosis, pain, and headaches. Analyzing the changing clinical trends of presentation of patients with NPC would be very useful for early diagnosis and would be the most hopeful means of reducing the high morbidity and mortality of NPC.,
An oral physician is identified by the medical community as a dentist who is adequately trained in the field of Medicine, Radiology and Applied Oral pathology in order to manage variety of stomatological diseases that fall outside the scope of routine dentistry but oral physicians are usually not the first healthcare workers that the patients having symptoms of NPC report to making it even more essential for dentists and oral physicians to understand the signs and symptoms of this disease.
We present a rare case of nasopharyngeal carcinoma which presented with no clinical symptoms typically associated with nasopharyngeal carcinoma except for mild facial pain on one side of the face and muscular atrophy.
| Case Report|| |
A 40-year-old male patient reported to the department of Oral Medicine and Radiology with a chief complaint of progressive sunken appearance of right side of the face especially in the temple and cheek region since 4 months. History also revealed mild pain with difficulty on chewing from the right side of the face and intra-oral mild persistent pain which was non-radiating in nature since 4 months. No contributing medical history or habit history was elicited.
On clinical examination [Figure 1], the right temporalis muscle appeared to be atrophied with outlines of the underlying bone clearly visible. The right masseter muscle did not appear to be grossly atrophied but presented with sunken appearance. On palpation the area was non-tender. All other visualized areas were normal with no secondary changes. The lymph nodes were non-palpable.
|Figure 1: Atrophy of the right temporalis muscle with outlines of the underlying bone clearly visible|
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Intra-orally, grossly carious 18, 28, 36, and 46 were seen which were non-tender on percussion. The patient was subjected to a panoramic radiographic view [Figure 2] which revealed grossly carious 18, 28, 36, and 46 with periapical radiolucency with 36 and 46. Horizontal impactions were present with 38 and 48. Slightly enlarged mandibular canal was visible bilaterally. Maxillary sinus on left and right side appeared normal with mid facial structures unclear. Bilaterally, condyles appeared normal with right articular space mildly increased. The alveolar bone appeared normal with no erosion. A provisional diagnosis of muscular atrophy with a differential diagnosis of localized scleroderma was given with multiple carious and impacted teeth as visualized.
|Figure 2: Panoramic X-ray view showing impacted mandibular molars and carious teeth|
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The patient was sent to general radiology department for further investigations after taking written informed consent for advanced investigations in which NCCT (Non-Contrast Computed Tomography) face was done which revealed a large soft tissue mass in right nasopharyngeal region. CECT (Contrast Enhanced Computed Tomography) face was done which revealed a [Figure 3] and [Figure 4] large hypodense lesion in the right nasopharyngeal region obliterating the fossa of rosenmuller extending inferiorly involving the anterior tonsilar pillar. Superiorly, it was invading the base of skull with involvement of various skull foramina and extending further superiorly up to the right cavernous sinus and middle cranial fossa through right foramen ovale causing its widening suggestive of perineural extension. Laterally the mass was invading the right parapharyngeal space and medially bulging into the nasopharyngeal airway. Posteriorly, it was invading in the retropharyngeal space. There was evidence of denervation atrophy of right sided muscles of mastication including masseter, temporalis, medial, and lateral pterygoid muscles. There was evidence of heterogeneous enhancement of the soft tissue mass and presence of enlarged lymph nodes level I and level II.
|Figure 3: CECT (coronal View) reveals large hypodense lesion in the right nasopharyngeal region obliterating the fossa of rosenmuller extending inferiorly involving the anterior tonsilar pillar|
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|Figure 4: CECT (axial view) reveals involvement of the right cavernous sinus and middle cranial fossa through right foramen ovale causing its widening suggestive of perineural extension|
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The patient was subjected to biopsy through endoscopy and the histopathological investigation revealed the presence of squamous epithelial cells arranged in the form of small islands within the connective tissue [Figure 5] and [Figure 6]. The lesional epithelial cell showed close resemblance to the cell of origin along with numerous keratin pearl formations, the section also revealed varying degrees of cellular and nuclear pleomorphism, individual cell keratinization, mitotic figures, increase in the number of nucleoli, and keratin pearl formation. A final diagnosis of Nasopharyngeal carcinoma was made on radiological and histopathological basis.
|Figure 5: Histopathology (10X)—revealed the presence of squamous epithelial cells arranged in the form of small islands within the connective tissue|
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|Figure 6: Histopathology (40X) —reveals varying degrees of cellular and nuclear pleomorphism, individual cell keratinization, mitotic figures, increase in the number of nucleoli and keratin pearl formation|
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| Discussion|| |
The presenting stage is one of the most important prognostic factors affecting the survival for patients with NPC. As the above case revealed, NPC can present as a completely silent disease except for very few facial symptoms in which muscular atrophy is a rare presentation. The nasopharynx is a relatively clinically silent area, and the most common presentation may be with cervical nodal or distant metastasis.
Enlargement and extension of the tumor in the nasopharynx may result in symptoms of nasal obstruction, changes in hearing, and cranial nerve palsies. The most common physical finding is a neck mass consisting of painless firm lymph node enlargement (80%), which was missing from the present case. Ocular disturbances have been reported in the literature with the involvement of multiple cranial nerves. Middle ear effusion is also one of the common presentations of NPC patients, which was also silent in the present case. A poor prognosis has been associated with the following: cranial nerve palsies, intracranial extension of tumor, cervical lymph node metastasis, level V (supraclavicular) cervical node involvement, nodes greater than 8 centimeters (cm), WHO type I histology (well differentiated squamous cell carcinoma), older age, and distant metastasis.,,
NPC can extend superiorly into the cavernous sinus with extra dural involvement while direct invasion of the brain is rare at diagnosis, which was seen in the CECT in the present case. After clinical assessment, bony involvement was suspected and it was subjected to NCCT. On looking at the changes, CECT was done in accordance with metastatic evaluation of the lesions to assess the lymph node status and extent of lesion.
In general, NPC spreads along well-defined routes. Nerves are resistant to tumor, and perineural tumor spread is an insidious and often asymptomatic process by which NPC can invade upward and backward through the skull base to the cavernous sinus and middle cranial fossa.
This case presented with a category T4 NPC. It involved the fossa of rosenmuller extending inferiorly into the anterior tonsilar pillar. Superiorly, it invaded the base of skull with involvement of various skull foramina and extending further superiorly upto the right cavernous sinus and middle cranial fossa through right foramen ovale causing its widening suggestive of perineural extension with skull base erosion and destruction. Other authors have reported infiltration of middle cranial fossa can be through the inferior orbital fissure, optic canal and superior orbital fissure, also involving the cavernous sinus. It can also invade the anterior cranial fossa through the ethmoid sinus and cribrifrom plate anteriorly.
Laterally the mass was invading the right parapharyngeal space and medially bulging into the nasopharyngeal airway and can be recognized by the partial or complete effacement of the fat-filled parapharyngeal space. Further lateral spread takes the tumor into the masticator space. The masticator spaces are paired suprahyoid cervical spaces on each side of the face. Each space is enveloped by the superficial (investing) layer of the deep cervical fascia. The masticator space contains the mastication muscles, ramus of the mandible, and mandibular nerve. Because clinical assessment of lesions in this space may be difficult, advanced imaging is important for the characterization and mapping of the pathology.
When the medial or lateral pterygoid muscles are infiltrated, the patient may complain of trismus but as revealed in the present case atrophy of the muscles of the mastication can also occur through the perineural extension. Posteriorly it was invading in the retropharyngeal space. Posteriorly, it can also spread to the prevertebral muscle, including the longus colli muscle. Inferiorly, it can invade to the oropharynx. It most commonly spreads via the foramen lacerum, otherwise, it also commonly invades the posterior cranial fossa via the jugular foramen. Infiltration of the medial and lateral pterygoid muscles, infratemporal fat, and temporalis muscle is found when tumors extend laterally from the parapharyngeal space, pterygoid base, or the pterygomaxillary fissure, as was true for the present case.
The presenting features of NPC are generally varied and diagnostically a challenge. While the common features involve lymph node enlargement, ocular symptoms, headaches, etc., NPC causing atrophy of muscles of mastication with negligible symptoms is rare. The drawback of reporting present case is lack of MR imaging as the sensitivity and specificity rates of enhanced CT for the diagnosis of cervical lymph adenopathy are approximately 14–60% and 78–92% respectively, compared to 29–80% and 82–92% respectively for MRI and some authors believe that MRI should be the standard imaging modality for diagnosing and staging of NPC.
Nonetheless, this case highlights the importance of considering NPC as one of the causes of muscular atrophy with mild pain. The clinician should always be alert to the possibility of infiltrative tumor and the role of an oral physician and maxillofacial radiologist in diagnosing this rare disease is emphasized through this report.
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Conflicts of interest
There are no conflicts of interest.
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