|Year : 2021 | Volume
| Issue : 4 | Page : 155-157
Unusual presentation of embryonal rhabdomyosarcoma of the middle ear presenting as chronic otomastoiditis and its complication
Wejdan Qublan Almuqati1, Khalid Badr2, Saeed Alghamdi2, Ahmed Abouissa3, Amna Moulana1, Mohamed El Tahmody1
1 Maternity and Children Hospital, Makkah Al-Mukarramah, Saudi Arabia
2 King Abdullah Medical City, Makkah Al-Mukarramah, Saudi Arabia
3 Al Noor Specialist Hospital, Makkah Al-Mukarramah, Saudi Arabia
|Date of Submission||05-Mar-2021|
|Date of Decision||01-Apr-2021|
|Date of Acceptance||04-Apr-2021|
|Date of Web Publication||20-Oct-2021|
Wejdan Qublan Almuqati
Al Noor Specialist Hospital, Makkah Al-Mukarramah
Source of Support: None, Conflict of Interest: None
As one of the most frequently occurring soft-tissue sarcomas among the pediatric population, Rhabdomyosarcoma (RMS) is an extremely rare malignant neoplasm that originates from striated muscles. RMS is rarely known to occur in the mastoid and middle ear. RMS symptoms occurring in-ear are typically similar to that of chronic suppurative otitis media, which are unresponsive to conventional treatment. Consequently, this contributes to delayed diagnosis of RMS of the ear/temporal bone. This case pertains to an 8-year-old boy presenting with facial nerve palsy, chronic mastoiditis, and abducent nerve palsy. On biopsy, RMS was found in his middle ear.
Keywords: Abducent nerve palsy, chronic otomastoiditis, embryonal rhabdomyosarcoma, facial nerve palsy, middle ear tumor
|How to cite this article:|
Almuqati WQ, Badr K, Alghamdi S, Abouissa A, Moulana A, El Tahmody M. Unusual presentation of embryonal rhabdomyosarcoma of the middle ear presenting as chronic otomastoiditis and its complication. Saudi J Otorhinolaryngol Head Neck Surg 2021;23:155-7
|How to cite this URL:|
Almuqati WQ, Badr K, Alghamdi S, Abouissa A, Moulana A, El Tahmody M. Unusual presentation of embryonal rhabdomyosarcoma of the middle ear presenting as chronic otomastoiditis and its complication. Saudi J Otorhinolaryngol Head Neck Surg [serial online] 2021 [cited 2022 Jan 21];23:155-7. Available from: https://www.sjohns.org/text.asp?2021/23/4/155/328725
| Introduction|| |
As one of the most frequently occurring soft-tissue sarcomas among the pediatric population, Rhabdomyosarcoma (RMS) is an extremely rare malignant neoplasm that originates from striated muscles.,,,,,, Almost 30% of RMS affects the head and neck.,,, After neuroblastoma and nephroblastoma, RMS is the third-most common tumor in children,, although it rarely occurs in the middle ear and mastoid. Below, we report the case of an 8-year-old boy who presented with chronic mastoiditis, facial nerve palsy, and abducent nerve palsy. On biopsy, embryonal RMS was found in his middle ear.
| Case Report|| |
An 8-year-old boy patient presented to the out-patient-department with a history of recurrent left ear discharge for 1 month along with left facial nerve palsy for 2 months. The facial nerve palsy was sudden in onset. He was a twin for one boy and one girl, born through assisted reproductive therapies throw in-vitro-fertilization. His weight during the birth time was 800 g. On examination, his general condition was found to be excellent, and there was no fever as well. No postauricular swelling, erythema, auricular displacement, or mastoid region tenderness was observed. He had mild conductive hearing loss in the left side. In addition, his facial examination revealed a Grade III left-sided facial palsy of lower motor neuron type. Thereafter, he was seen initially by a neurologist who had advised a physiotherapy course when magnetic resonance imaging (MRI) was done. A week later, the MRI result came out [Figure 1]. Accordingly, the child was admitted to the hospital under general pediatric, which is why he was given IV antibiotics and prednisolone. After an urgent consultation with the otorhinolaryngologist, otoscopic examination showed the following: the left intact tympanic membrane was bulging and congested compared to an intact right tympanic membrane. Five days later, he had an abnormal gaze while looking at the left side associated with double vision and headache. A simple mastoidectomy revealed thick mucosa, even though the middle ear was filled with granulation tissue and friable unhealthy mastoid bones. At the same time, the mastoid cavity was scrapped and sent for histopathology [Figure 2]. Subsequently, MRI was done [Figure 3]. Two weeks later, the histopathology report revealed an Embryonal RMS of the middle ear [Figure 4]. After the diagnosis of the tumor, the radiology department re-evaluated the X-ray and found that he had an asymptomatic right lung lesion [Figure 5]. Despite multiple chemotherapeutic courses and radiotherapy, the condition of the child deteriorated quickly. Unfortunately, he passed away due to this disease within 11 months.
|Figure 1: Magnetic resonance imaging Coronal T2 (a) and axial postcontrast (b) show infiltration of the left middle ear and mastoid with fluid and soft-tissue lesion. There is ill-definition of bony boundaries and subtle meningeal enhancement|
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|Figure 2: (a and b) Mastoid cavity after cortical mastoidectomy fill with granulation tissue|
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|Figure 3: (a and b) Follow-up magnetic resonance imaging after simple mastoidectomy shows progressive course with intra-and extra-cranial soft-tissue components (thick and double thin arrows)|
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|Figure 4: (a) Infiltrating sheets of small round blue cells separated by fibrous bundles (H and E × 40). (b) Higher power view exhibiting variable skeletal muscle differentiation ranging from immature cells with round blue color to ganglion cell-like rhabdomyoblasts with prominent nucleoli (H and E × 200). (c) Infiltrating cells are positive for Desmin (immunoperoxidase × 200). (d) Infiltrating cells are positive for Myo D1 (immunoperoxidase × 100)|
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|Figure 5: Chest X-ray shows rounded nodule in right lower lung zone (arrow) suggesting a metastatic deposit and raised the concern of malignant process|
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| Discussion|| |
RMS s occur predominantly in the head-and-neck regions, orbits, skull base, nasal cavity, and nasopharynx. In children, nearly 30% occur of all cases are known to take place around the head/neck, while the ear and the temporal bone denote an uncommon site.,, Symptoms of RMSs that occur in-ear and temporal bone are typically similar to that of chronic suppurative otitis media, which are unresponsive to conventional treatments.,,,, This, in turn, delayed the diagnosis of RMS of the ear and temporal bone.,,, The advanced disease presents with cranial nerve palsies and intracranial extension.,,, Hence, the suspicion for a middle ear tumor should be raised if any presumed otologic infections do not respond to medical therapy on the average of 2 weeks in a row, and facial nerve palsy and auricular polyp are found. In the course of his disease, the involvement of the facial nerve was seen at an early stage. In this case, RMS began from the boy's middle ear and encompassed the mastoid, subsequently also involving the sixth cranial nerve. Histopathologically, these tumors are classified into five categories: botryoid, spindle cell, alveolar, undifferentiated, and embryonal.,,, Most RMSs occurring in the neck and head are embryonal types which have been discussed in this case.,,,, According to the previous study, being born as a twin or a multiple could exacerbate the risk of having RMS as well; this finding, however, was not statistically significant. A study conducted over a 17-year-period indicated that the birth after assisted conception could increase the risks of hepatoblastoma and RMS. RMS spread locally and metastasize into remote areas both by hematogenous and to lymphatic area.,, Distant metastasis is mostly found to occur in the lung.,, However, direct meningeal extension from the middle ear/mastoid RMS occurs frequently. Such cases often entail a poor prognosis, even though the involvement of intensive treatment. Before obtaining a biopsy, imaging should be performed. For the initial evaluation, it is acceptable to perform a computed tomography scan of the temporal bone with contrast. Nevertheless, MRI with contrast is most specific for diagnosing soft-tissue tumors and assessing meningeal invasion. RMS of the middle ear and mastoid have a poorer prognosis as compared to those in the head and neck.,,The rate of surviving is not very encouraging. Some studies revealed that the survival rate is as low as zero in the presence of metastatic disease.
| Conclusion|| |
Not only did this case represent an unusual presentation of RMS of the middle ear but it also highlighted the difficulty in the diagnosis of middle ear soft-tissue tumors in children. Early diagnosis and treatment considerably improve clinical outcomes. All children with persistent suppurative otitis media, which is unresponsive to medical management should be considered for an initial examination and biopsy of the middle ear cavity under anesthesia.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]