|Year : 2018 | Volume
| Issue : 3 | Page : 130-133
Botryoid rhabdomyosarcoma in mastoid and middle ear in a 4-year-old boy: A rare case report
Abdelgalil Ali Ragab, Mouhannad Mahmoud Abdulber Fakoury, Jamal Kassouma, Kamal Moustafa, Fateh Al Mohamad Al Salem
Department of ENT, Dubai Hospital, DHA, Dubai, United Arab Emirates
|Date of Web Publication||24-Sep-2018|
Abdelgalil Ali Ragab
Department of ENT, Dubai Hospital, DHA, Po Box 7272, Dubai
United Arab Emirates
Source of Support: None, Conflict of Interest: None
A 4-year-old boy presented with the left ear discharge and polyp of the external canal. The patient was treated as a case of chronic ear infection with mastoidectomy done twice and polypectomy done three times and histopathology came as non-specific inflammation, then as botryoid rhabdomyosarcoma. The patient underwent bone marrow biopsy and confirmation of the diagnosis done. The patient received chemotherapy and doing well. The case discussed because it is rare and because rhabdomyosarcoma rarely presented as external canal polyp and to highlight the presentation of serious malignant problems may mimic the benign diseases.
Keywords: Botryoid rhabdomyosarcoma, mastoid and middle ear, middle ear rare tumour
|How to cite this article:|
Ragab AA, Abdulber Fakoury MM, Kassouma J, Moustafa K, Al Salem FA. Botryoid rhabdomyosarcoma in mastoid and middle ear in a 4-year-old boy: A rare case report. Hamdan Med J 2018;11:130-3
|How to cite this URL:|
Ragab AA, Abdulber Fakoury MM, Kassouma J, Moustafa K, Al Salem FA. Botryoid rhabdomyosarcoma in mastoid and middle ear in a 4-year-old boy: A rare case report. Hamdan Med J [serial online] 2018 [cited 2020 May 28];11:130-3. Available from: http://www.hamdanjournal.org/text.asp?2018/11/3/130/238567
| Introduction|| |
Weber first described rhabdomyosarcoma (RMS) in 1854 and stout described morphology in 1946 describing histology. The most common sarcoma of childhood is RMS. RMS is the third most common neoplasm in childhood after neuroblastoma and nephroblastoma. The name is derived from Greek words rhabdo, which means rod shape and myo, which means muscle. It is considered malignant neoplasia derived from striated muscle arising in any part of the body; however, it is more common in regions of the head and neck, genitourinary tract, retroperitoneum and extremities.
The most common site that involved by RMS is orbit (about one-third of the cases). After that, in decreasing order, RMS affects oral cavity and pharynx (29%), the face and neck region (24%), involvement of the ear and temporal bone with RMS is uncommon. Pathologic subtypes of RMS include as follows: embryonal, botryoid, alveolar, pleomorphic, spindle cell and anaplastic variants. The embryonal RMS includes about 60%—70% of RMS cases. RMS of the middle ear and mastoid is the most common malignant aural neoplasm in the paediatric population and commonly with advanced presentation. Aggressive bone destruction with obliteration of the normal landmarks occurs in the majority. The standard of treatment for RMS includes excisional surgery, multidrug chemotherapy and external beam radiation therapy. External beam radiation therapy has many side effects due to the collateral damage of surrounding anatomical structures. In the head-and-neck region, external beam radiation therapy can result in hearing loss, facial hypoplasia, developmental delay, xerostomia, dental problems, problems with the pituitary/hypothalamic hormones and second malignancies., Brachytherapy is comparatively less damaging because of the localised mechanism of action, which should spare the surrounding tissue.
The AMORE protocol consists of Ablative Surgery, MOld technique with after loading brachytherapy and immediate surgical reconstruction after chemotherapy. The patients with head-and-neck RMS treated with external beam radiation therapy were found to have higher incidences of hearing loss compared to survivors who had received AMORE-based therapy.
| Case Report|| |
A 4-year-old boy who was completely healthy with no active issues or complains, started to have the left ear discharge from July 2017 and was seen in private hospital and diagnosed as polyp of the external auditory canal CT scan of temporal bone done and showed soft-tissue opacity of middle ear, mastoid and external auditory canal [Figure 1] and [Figure 2] and then, he underwent polypectomy under general anaesthesia on 5th September, 2017. Histopathological examination of the polyp showing the fragments of granulation tissue with heavy inflammatory cell infiltrates with the prominent lymphohistiocytic component. Three weeks later, the patient had recurrence of ear discharge and the polyp. The child was taken to another hospital where imaging study was done and showed that the polyp is extensive and not merely confined to the external ear; he was posted for mastoidectomy and resection of the polyp in October 2017 [Figure 3]. Histopathology report showed Langerhans cell histiocytosis.
|Figure 1: Pre-operative coronal computed tomography scan showing the obliteration of the mastoid with soft-tissue opacity|
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|Figure 2: Pre-operative axial computed tomography scan of the temporal bone showing the soft-tissue opacity obliterating the middle ear and external canal|
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|Figure 3: Three weeks post-operative axial computed tomography scan showing the recurrence of the mass|
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The patient then referred to another hospital where immunohistochemical study (Langerin, CD1a, S100, CD68, CD20, CD30, CD15 and CD3) was done to rule out Langerhans cell histiocytosis or lymphoproliferative disorders and the possibility of both was ruled out.
After 6—8 weeks of second surgery, the polyp could be visualised again by the parents, hence, taken again to the hospital where the parents have been informed that further evaluation and management is necessary and have been referred to ENT Department of Dubai Hospital.
In Dubai Hospital, the boy underwent exploration of the left ear on 28th December, 2017 where microscopic examination of the left ear revealed the polyp in external meatus and granulation tissues with fragile bone filling the mastoid cavity. The middle ear exposure revealed that the granulation tissues filling the whole middle ear cavity, the ossicles except the stapes were removed with the granulation. The jugular bulb and the dura were exposed and surrounded by granulation. The eustachian tube was cleaned from granulation tissues.
Granulation tissue was sent to histopathological examinations. Immunohistochemistry study (Desmin, Myogenin and Ki67) showed that the presence of other types of small round-cell tumour. The three markers showed patchy strong positivity in the small cell infiltrate. These results are consistent with the diagnosis of embryonal RMS (botryoid type). The patient was referred to Paediatric Haematology-Oncology for further evaluation and management. The X-ray skeletal survey was done and revealed normal bony alignment, normal joint spaces, no lytic or sclerotic bony lesions, no bony fractures and no gross soft-tissue abnormalities [Figure 4] and [Figure 5].
The patient underwent bone marrow aspirate and trephine, and the histopathological study revealed no evidence of non-haematological infiltration. Cerebrospinal fluid (CSF) examination came as negative for malignant cells that indicate neither metastasis to bone nor central nervous system. Positron emission tomography—computed tomography (PET CT) scan done and showed moderate hypermetabolic activity in the left external auditory canal and operated mastoid region [Figure 6] and [Figure 7], most probably due to post-operative changes; however, at this stage due to the short gap between operation and scan residual tumour cannot be ruled out. A close follow-up scan in 3 months was recommended. No evidence of increased uptake elsewhere [Figure 8]. After confirming that the disease is confined to the mastoid area, the patient was admitted for Port-A-Cath insertion and was started on chemotherapy as per Paediatric EpSSG-RMS-2005 Protocol, Standard Risk Group, Subgroup-D (vincristine, dactinomycin and ifosfamide).
|Figure 6: Positron emission tomography—computed tomography scan of the head showing hypermetabolic activity in the region of the left mastoid and external auditory canal|
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|Figure 7: Positron emission tomography scan showing hypermetabolic activity in the region of left mastoid and external auditory canal|
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|Figure 8: Positron emission tomography—computed tomography scan of the whole body showing evidence of increased uptake elsewhere|
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| Discussion|| |
RMS is a malignant tumour arising from sites with absent striated muscle i.e. nasal cavity, middle ear and vagina. In this case, it was rising from the middle ear and mastoid. Embryonal is the most common histologic subtype of RMSs. It is responsible for 70%—80% of the presentations and its origin is supposed to be triggered by a disorder in mesenchymal primitive differentiation of the musculoskeletal cells in their first or earliest stages of embryogenesis. In paediatric cases, about 30%—40% occur in the head-and-neck regions; however, the ear and the temporal bone are uncommon sites of involvement., Middle ear RMSs were reported in the literature with a range of symptoms that clinically simulates chronic otitis media. The most commons are facial nerve palsy, headache, hearing impairment and bleeding. In our case, it presented with ear pain, discharge and aural polyp. Enlarged lymph nodes are more likely associated with distant metastasis. Metastasis can be present in up to 30% of the cases. The most common affected sites are the lungs, liver, bones and extremities., In this case, there was no metastasis to bone and perineural as indicated by negative CSF and bone marrow aspirates.
The diagnosis of the middle ear and temporal bone RMS is difficult when associated inflammatory infectious diseases such as exudative otitis media that may mask the base disease and delay the definite diagnosis. In these cases, early correlation with imaging screenings is essential. In this case, the diagnoses were delayed and the histopathology report came first as non-specific inflammation due to current infection. RMSs comprise three distinct histologic subtypes: embryonal, alveolar and pleomorphic. Of these, the embryonal type is the most common one found in the head and neck comprising 50%—70% of cases. Alveolar occurs in 20%—30% of cases and 5% of occurrences are pleomorphic. This case come under the embryonal type.
RMS of the head-and-neck region falls into three categories, 51% of cases occur in the parameningeal region (nose, paranasal sinuses, middle ear and mastoid), 24.5% of cases present in the non-parameningeal region (Parotid gland, oral cavity and larynx) and 24.5% of cases are in the orbit. RMS of the ear is rare, with only about 3% of patients presenting with the middle ear disease. In this case, it arises from the middle ear and mastoid.
| Conclusion|| |
This case report demonstrates an uncommon manifestation of RMS at an unusual location, which can be misdiagnosed as a benign lesion. Delay in diagnosis can result in further growth of the tumour and worse. Maintaining a wide differential for recurrent non-remitting otitis media is important for this reason. Cases like this need a multidisciplinary team to reach the ideal management.
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], [Figure 6], [Figure 7], [Figure 8]