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ORIGINAL ARTICLE
Year : 2016  |  Volume : 22  |  Issue : 1  |  Page : 40-43

Pediatric rhabdomyosarcoma of the ear and temporal bone: An analysis of seven cases in Sokoto, Nigeria


1 Department of ENT, Usmanu Danfodiyo University Teaching Hospital, Sokoto, Nigeria
2 Department of Histopathology, Usmanu Danfodiyo University Teaching Hospital, Sokoto, Nigeria

Date of Web Publication16-Feb-2016

Correspondence Address:
Mohammed Abdullahi
Department of ENT, Usmanu Danfodiyo University Teaching Hospital, Sokoto
Nigeria
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0971-7749.176506

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  Abstract 


Background: Rhabdomyosarcoma is a common childhood soft tissue sarcoma but rare in the ear and temporal bone region, often mimicking features of chronic suppurative otitis media. Objective: The objective of this analysis is to determine the clinical pattern, stage at presentation, traditional healer intervention, and outcome. Methodology: A retrospective study of patients under the age of 15 years with histological diagnosis of rhabdomyosarcoma of the ear and temporal bone, who presented at the Ear, Nose, and Throat Department of the Usmanu Danfodiyo University Teaching Hospital, Sokoto, Nigeria, from January 2000 to July 2015. Results: A total of seven patients were seen. Two (28.6%) were male and 5 (71.4%) were female. The age ranged between 3 and 9 years with mean age of 6.7 years. Clinical features mimic that of chronic suppurative otitis media. All were advanced embryonal rhabdomyosarcoma. Traditional healers performed incision in 3 (42.9%) patients with the aim of draining pus from the swelling. The incision was done with a knife and hot iron. Multimodality treatment was offered: mortality and discharge on parole to source for funds to undergo radiotherapy was recorded in 3 (42.9%) patients, but they never came back for follow-up. Conclusion: Advanced embryonal rhabdomyosarcoma of the ear and temporal bone were seen in all the patients with clinical presentation mimicking that of chronic suppurative otitis media. Outcome was not favorable because of the advanced tumor stage presentation, dangerous traditional healer intervention, and financial constraints. We recommend creation of patients' awareness and the improvement of the populace socioeconomic status.

Keywords: Ear, Rhabdomyosarcoma, Temporal bone, Traditional healers


How to cite this article:
Abdullahi M, Amutta S B, Iseh K R, Aliyu D, Yikawe S S, Abdullahi K. Pediatric rhabdomyosarcoma of the ear and temporal bone: An analysis of seven cases in Sokoto, Nigeria. Indian J Otol 2016;22:40-3

How to cite this URL:
Abdullahi M, Amutta S B, Iseh K R, Aliyu D, Yikawe S S, Abdullahi K. Pediatric rhabdomyosarcoma of the ear and temporal bone: An analysis of seven cases in Sokoto, Nigeria. Indian J Otol [serial online] 2016 [cited 2020 Feb 23];22:40-3. Available from: http://www.indianjotol.org/text.asp?2016/22/1/40/176506




  Introduction Top


Most soft tissue sarcomas in pediatrics are rhabdomyosarcoma (60%) accounting for about 13% of all pediatric malignancies.[1],[2] Rhabdomyosarcoma occurs commonly in the head and neck, seen mostly in the parameningeal region.[3],[4] The ear and temporal bone are uncommon sites of involvement.[5],[6],[7] Rhabdomyosarcoma of the parameningeal site have a poor prognostic outcome.[8],[9] However, with the new treatment modalities, prognosis has improved.[10]

Mimicking features of chronic suppurative otitis media is one of the common presentations of rhabdomyosarcoma of the ear and temporal bone.[5],[6] This may contribute to delayed diagnosis of rhabdomyosarcoma of the ear and temporal bone. The advanced disease will present with cranial nerve palsies and intracranial extension.

For head and neck cancer patients, many factors contribute to the delayed diagnosis [11],[12],[13] which often, is associated with unfavorable outcome. The role of traditional healers, especially in the developing countries, is increasingly being recognized [14] and their practices are often associated with life-threatening situations.[15]

Head and neck rhabdomyosarcoma have been reported in Nigeria [15],[16],[17],[18] with dearth of information on the ear and temporal bone as primary sites.

This study aimed to determine the clinical features, stage at presentation, traditional healer intervention, and outcome in a Nigerian tertiary health institution.


  Methodology Top


This was a retrospective study of patients under the age of 15 years with histological diagnosis of rhabdomyosarcoma of the ear and temporal bone, who presented at the Ear, Nose, and Throat Department of the Usmanu Danfodiyo University Teaching Hospital, Sokoto, Nigeria, from January 2000 to July 2015. Data retrieved from the case notes include age, sex, clinical presentation, traditional healer intervention, histological type, stage of the tumor based on the tumor-node-metastasis pretreatment staging classification for intergroup rhabdomyosarcoma study-IV,[19] radiological evaluation, treatment, and outcome.

Inclusion criteria

Children under the age of 15 years with rhabdomyosarcoma affecting only the ear and temporal bone and the exclusion criteria include rhabdomyosarcoma of other sites.

Data were analyzed with simple statistics and results were presented in tables and figures.


  Results Top


A total of seven patients were seen during the study period. There were 2 (28.6%) male and 5 (71.4%) female patients with male to female ratio of 1:2.5. The age ranged between 3 and 9 years with the mean age of 6.7 years. Hearing loss was seen in all patients with ear and temporal bone rhabdomyosarcoma and the other symptoms were ear discharge and facial nerve palsy 5 (71.4%), neck swelling and blurring of vision 4 (57.1%), aural polyps 3 (42.9%), and otalgia 2 (28.6%) as shown in [Figure 1]. Some of these symptoms are characteristic of chronic suppurative otitis media.
Figure 1: Symptomatology of patients with ear and temporal bone rhabdomyosarcoma

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Involvement of right and left ears was 5 (71.4%) and 2 (28.6%), respectively, and there was no bilateral ear involvement. In [Table 1], three (42.9%) patients sought the intervention of traditional healers who performed incision with the aim of draining pus from the swelling. The incision was done with a knife and hot iron. All the patients had advanced embryonal rhabdomyosarcoma [Figure 2] and [Figure 3] with intracranial extension seen in 4 (57.1%) patients [Figure 2].
Table 1: Summary of patients' data

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Figure 2: Axial computed tomography scan showing bone erosion involving the petromastoid, infratemporal fossa, and posterior cranial fossa (case 4)

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Figure 3: Rhabdomyosarcoma (case 4) showing tadpole cells (white arrow), strap cell (orange arrow), and myxoid stroma (green arrow) H and E, ×200

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Treatment and outcome

Primary surgery with incomplete resection was attempted in three patients (cases): 2 (7 years of age), 4, and 7, followed by postoperative chemotherapy. The remaining four patients had only chemotherapy. A patient (case 4) was referred for radiotherapy to another center after the completion of chemotherapy because of nonavailability of facility for radiotherapy in the index center before 2010. During the course of chemotherapy, cases 2 (7 years of age), 3, and 5 died due to progression of the disease and complications of the cytotoxic drugs.

Case 1 (9 years of age), 6, and 7 requested for discharge to sought for fund to undergo radiotherapy; however, they never came back for radiotherapy and follow-up.


  Discussion Top


Rhabdomyosarcoma accounts for up to 60% of childhood soft tissue sarcoma,[3] occurring mostly in the head and neck region but rarely in the ear and temporal areas.[20] This could explain why few patients were seen with rhabdomyosarcoma of the ear and temporal bone for this and other reports.[5],[6],[20],[21]

Presentation of rhabdomyosarcoma of the ear and temporal bone usually mimics that of the chronic suppurative otitis media that is unresponsive to the usual treatment.[5],[6] In this present study, the main presenting symptoms were consistent with these reports. More advance presentation with intracranial extension was noted in our study; probably, this may be due to the delayed presentation to qualified healthcare givers and diagnosis before referral.

Traditional healers are a major source of healthcare givers in the developing countries with about 80% of the population (Malaysia) consulted traditional healers and their positive role is being recognized;[22] however, their unsafe and unusual practices may lead to life-threatening conditions.[14] Dangerous intervention by the traditional healers was noted in this study, which involved the use of probably unclean knife and hot iron rod for incision for the purpose of draining of pus. These practices not only worsen the prognosis especially those with head and neck cancers, but also contribute to the delay in patients' presentation for appropriate treatment.

Histologically, rhabdomyosarcoma resembles normal skeletal muscle before innervation, and it is classified into embryonal (with botryoid and spindle cell variants) and alveolar types, of which the embryonal type has a better prognosis.[19],[23] In this study, all the series were embryonal rhabdomyosarcoma which agrees with other reports,[6] though in their report, they recorded a case of alveolar type out of six patients.

Prognosis of parameningeal rhabdomyosarcoma is poor, especially those <1 year of age, intracranial extension with bony erosion, and involvement of infratemporal fossa and paranasal sinus.[24] The outcome of pediatric patients with rhabdomyosarcoma has improved with multiple drug chemotherapy in addition to radiotherapy and or surgery. In this study, tumors were all advanced at the time of presentation with 57.1% of these patients having intracranial extensions, coupled with financial constraint to undergo radiotherapy and poor follow-up contributed to the poor outcome of our patients with rhabdomyosarcoma of the ear and temporal bone. Improved outcome can be improved when these shortcomings are addressed through patients and professional colleagues (on the need for early referral) education and improving the socioeconomic status of the general populace through good policies from the government concerned.


  Conclusion Top


Only seven cases, all of which were advanced embryonal rhabdomyosarcoma of the ear and temporal bone and 57.1% of these patients had intracranial extension. These seven cases were seen in the 15-year period with clinical presentation mimicking that of chronic suppurative otitis media. Outcome was not favorable because of the late tumor stage presentation, traditional healer intervention, financial constraint to commence the necessary treatment, and poor follow-up. These shortcomings can be improved through creation of public awareness and good government policies to improve the socioeconomic status of the populace.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

 
  References Top

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Adigun IA, Rahman GA, Buhari MO, Ogundipe KO, Omotayo JA. Pattern of rhabdomyosarcoma in Nigerian children. J Natl Med Assoc 2008;100:906-9.  Back to cited text no. 16
    
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Brown BJ, Oluwasola AO. Childhood rhabdomyosarcoma in Ibadan, Nigeria: 1984-2003. Ann Trop Paediatr 2006;26:349-55.  Back to cited text no. 17
    
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Fajemisin AA. Rhabdomyosarcoma of the middle ear and mastoid. J Laryngol Otol 1974;88:809-16.  Back to cited text no. 18
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19.
MacArthur JC, Smith HJ. Pediatric head and neck malignancies. In: Paul WF, Bruce HH, Valerie JL, John KN, Mark AR, Robin KT, editors. Cummings Otolaryngology Head and Neck Surgery. 5th ed. Philadelphia: Mosby, Elsevier; 2010. p. 2835-49.  Back to cited text no. 19
    
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Daya H, Chan HS, Sirkin W, Forte V. Pediatric rhabdomyosarcoma of the head and neck: Is there a place for surgical management? Arch Otolaryngol Head Neck Surg 2000;126:468-72.  Back to cited text no. 21
    
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Diaconescu S, Burlea M, Miron I, Aprodu SG, Mihaila D, Olaru C, et al. Childhood rhabdomyosarcoma. Anatomo-clinical and therapeutic study on 25 cases. Surgical implications. Rom J Morphol Embryol 2013;54:531-7.  Back to cited text no. 23
    
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Merks JH, De Salvo GL, Bergeron C, Bisogno G, De Paoli A, Ferrari A, et al. Parameningeal rhabdomyosarcoma in pediatric age: Results of a pooled analysis from North American and European cooperative groups. Ann Oncol 2014;25:231-6.  Back to cited text no. 24
    


    Figures

  [Figure 1], [Figure 2], [Figure 3]
 
 
    Tables

  [Table 1]


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