|Year : 2015 | Volume
| Issue : 3 | Page : 209-211
Malignant round cell tumor of temporal bone masquerading as chronic suppurative otitis media
Madhuri Dadwal, Dev Raj Sharma, RS Minhas, Shallu, Payal
Department of ENT, Indira Gandhi Medical College, Shimla, Himachal Pradesh, India
|Date of Web Publication||17-Jul-2015|
Department of ENT, C/o Sh. O.P Dadwal, Flat 48, Block-3, Lane V, Sector-I, New Shimla - 171 009, Himachal Pradesh
Source of Support: None, Conflict of Interest: None
Chronic suppurative otitis media is a commonly encountered disease entity in pediatric population in India.Malignant small round cell tumors (MSRCT),are common malignant tumour seen frequently in pediatric age group, that are characterised by small, round, relatively undifferentiated cells. It is difficult to make a differential diagnosis due to their undifferentiated or primitive character. Immunohistochemical staining and chromosomal study is useful to categorize these tumors. Differential diagnosis of small round cell tumors is particularly difficult due to their undifferentiated or primitive character.Since these are undifferentiated tumors, treatment protocols are not well established. While combined treatments (surgery, chemotherapy, radiotherapy) is currently being applied, the tumor still has a poor prognosis. Since both CSOM and MSCRT are common in pediatric age group and have similar clinical presentations, possibility of MSCRT must be kept in mind while dealing with cases of CSOM in pediatric population. We present to you a rare case of malignant small round cell tumour of temporal bone in three years old child who presented with unsafe CSOM right ear for which Modified Radical mastoidectomy was done. But patient presented again after one month with fifth, sixth and seventh cranial nerve involvement presented with facial palsy and gradenigo syndrome of right side and nasal bleed from same side.
Keywords: Facial nerve palsy, Gradenigo syndrome, Malignant small round cell tumor, Temporal bone
|How to cite this article:|
Dadwal M, Sharma DR, Minhas R S, Shallu, Payal. Malignant round cell tumor of temporal bone masquerading as chronic suppurative otitis media. Indian J Otol 2015;21:209-11
|How to cite this URL:|
Dadwal M, Sharma DR, Minhas R S, Shallu, Payal. Malignant round cell tumor of temporal bone masquerading as chronic suppurative otitis media. Indian J Otol [serial online] 2015 [cited 2019 Nov 15];21:209-11. Available from: http://www.indianjotol.org/text.asp?2015/21/3/209/161039
| Introduction|| |
Malignant small round cell tumors (MSRCTs) is a broad term used for a group of neoplasms characterized by small, round, relatively undifferentiated cells. Definitive diagnosis is difficult because of undifferentiation of the cells.
| Case Report|| |
A 3-year-old male child presented with a history of blood stained purulent discharge from the right ear for 3 weeks duration. On examination, the child was irritable febrile, there was blood stained ear discharge. On the basis of history, clinical examination and contrast-enhanced computed tomography (CE-CT), the child was diagnosed with unsafe chronic suppurative otitis media (CSOM) right ear with petrous osteomyelitis. The patient underwent modified radical mastoidectomy (MRM) right ear under general anesthesia. Peroperatively, there was an intact lateral cortical wall, whole mastoid antrum filled with granulation tissue and pus under tension extending up to the mastoid tip, perifacial cells, aditus, and attic. Ossicles were intact, and the tympanic membrane was thickened. The patient was put on intravenous antibiotics postoperatively and was discharged on the 10 th postoperative day.
After 1-month, the child presented again with polypoidal mass protruding out of the right external auditory canal, facial asymmetry, inability to completely close the right eye, and epistaxis right side for the duration of 1-week. On examination, the child was irritable and afebrile. On ear examination, a fleshy polypoidal mass was seen in the right ear canal that could be probed all around, sensitive, and bled on touch. There were lower motor neuron type of facial nerve palsy with house brackmann grade 4 and sixth nerve palsy on the right side [Figure 1],[Figure 2] and [Figure 3].
|Figure 1: Facial nerve palsy on the right side. Note the deviation of angle of mouth, absence|
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|Figure 2: Facial nerve palsy on the right side. Note the deviation of angle of mouth, absence of nasolabial furrow and absent wrinkling of forehead on right side|
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|Figure 3: Lateral rectus palsy right side on lateral gaze toward the right side. Note the normal medial position of left eyeball|
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The presence of polypoidal mass in the ear, seventh nerve palsy, and sixth nerve palsy within a month of MRM raised the suspicion of some malignancy. Urgent CE-CT of temporal bone was done, which revealed the presence of heterogenously enhancing soft tissue mass lesion in right temporal bone with erosion and destruction of right petrous apex with intracranial extension into right middle cranial fossa [Figure 4], perineural spread along right trigeminal nerve with involvement of right parapharyngeal space, and nasopharyngeal mucosa involvement [Figure 5].
|Figure 4: Destruction of petrous part of temporal bone with extension into middle cranial fossa|
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|Figure 5: Involvement of right nasopharyngeal mucosa and parapharyngeal space|
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| Discussion|| |
Temporal bone malignancy as such is uncommon, out of which most common type is squamous cell carcinoma. Temporal bone as the site of the malignant round cell tumor is extremely rare in adults. However, the incidence of the malignant round cell tumor is quite high in pediatric population.
Malignant small round cell tumors is a term used for tumors composed of malignant round cells that are slightly larger or double the size of red blood cells in air-dried smears [Figure 6]. 
|Figure 6: Histopathological view of biopsy showing malignant small round cells|
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This group of neoplasm is characterized by small, round, relatively undifferentiated cells. In general, they include Ewing's sarcoma, peripheral neuroectodermal tumor, rhabdomyosarcoma, synovial sarcoma, non-Hodgkin's lymphoma, retinoblastoma, neuroblastoma, hepatoblastoma, and nephroblastoma. 
Tumors that show good differentiation are generally easy to diagnose, but identification of the diagnostic, morphological features are difficult when a tumor is poorly differentiated, therefore, no definitive diagnosis may be possible.  For exact categorization of these tumors, immunohistochemistry and chromosomal study are useful.
Symptoms include ear discharge, polyp or granulations. Facial palsy occurs early Investigations include routine blood investigations and radiological imaging such as X-ray mastoid and computed tomography (CT) of the temporal bone. The role of CT imaging is very important to localize the disease and see for extension of disease. A biopsy is a mainstay for diagnosis, which leads to the histopathological diagnosis. Immunohistochemistry also has a major role in the exact configuration of MSRCT. Diagnosis of MSRCT in view of similar presentation as CSOM is a diagnostic challenge for otorhinolaryngologist.
The treatment protocol of MSRCT is not yet been established. Combined treatment (surgery, chemotherapy, and radiotherapy) is currently applied depending on the tumor size and location although the prognosis remains poor. Eralp et al.  reported with a 30.5% 3-years disease-free and overall survival rate.
| Conclusion|| |
Malignant small round cell tumor includes a group of neoplasm characterized by cells that are small, round, and relatively undifferentiated. MSRCT of the temporal bone is relatively uncommon. MSRCT presents with symptoms of ear discharge, polyp, and granulation tissue thus mimicking the CSOM. The possibility of malignancy should be kept in mind while dealing with unsafe CSOM, especially in young children with short symptomatic history and rapid progression of the disease.
Detailed clinical history, examination as well radiological imaging specially CT is essential in clinching the diagnosis is made by biopsy and immunohistochemistry studies. Treatment modalities include chemotherapy, radiotherapy or both are initiated as soon as the diagnosis is made keeping in view the poor progression and aggressive nature of MSRCT.
Hence, in view of the similar clinical presentation in the pediatric population, the possibility of MSRCTs should always be kept in the differential diagnosis of CSOM in children and should be ruled out.
| References|| |
Rajwanshi A, Srinivas R, Upasana G. Malignant small round cell tumors. J Cytol 2009;26:1-10.
Nikitakis NG, Salama AR, O′Malley BW Jr, Ord RA, Papadimitriou JC. Malignant peripheral primitive neuroectodermal tumor-peripheral neuroepithelioma of the head and neck: A clinicopathologic study of five cases and review of the literature. Head Neck 2003;25:488-98.
Boahene DO, Olsen KD, Driscoll C, Lewis JE, McDonald TJ. Facial nerve paralysis secondary to occult malignant neoplasms. Otolaryngol Head Neck Surg 2004;130:459-65.
Eralp Y, Bavbek S, Basaran M, Kaytan E, Yaman F, Bilgiç B, et al
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[Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6]