TEMPORAL BONE PATHOLOGY , A REPORT OF THREE CASES

Temporal bone can be affected by different conditions. In our area, the most common disease conditions of the temporal bone are fibrous dysplasia, histiocytosis X and squamous cell carcinoma. This is a report of three females who have temporal bone disease conditions. They have poor clinical symptomatology. Since the incidence of malignancy is rare so differentiation between chronic inflammation and tumors is difficult and a biopsy is a must. Computed tomography (CT) was the diagnostic aid of choice which also was very helpful for follow-up to assess the disease progression, while Magnetic resonance imaging (MRI) was useful to identify intracranial extension. Treatment of these conditions included surgery, radiotherapy and chemotherapy. Introduction ibrous dysplasia is a tumour-like lesion of the bone. It is self-limiting, not encapsulated and is characterized by replacement of normal bone with cellular fibrous connective tissue and is of unknown etiology. The disease may develop at early age, and progress actively during childhood and stabilize in adulthood. This disease is much more frequent in white populations than black and the monostotic form is found more often in females than in males. In onethird of the cases, it is located in the maxilla or mandible and sometimes the frontal or sphenoid sinus are obliterated by the disease. Eosinophilic granuloma is an uncommon, benign disease described by uncontrolled expansion of Langerhans cells. The most successive destinations of inclusion are the skull, spine, ribs, femur, and pelvis. Lichtenstein assembled this infection range under the name histiocytosis X. The "X" because the etiology was obscure. It is a solitary condition, typically restricted to skeleton with great anticipation. Kids and youthful grownups are most ordinarily influenced. Male to female proportion is 2:1. In spite of the fact that the etiology is obscure, injury, genetic pattern, immune system mechanism, metabolic problems, and viral contaminations have all been suspected. Temporal bone contribution is moderately uncommon. One of the announced disease clinical presentation is osteolytic type. Normally present manifestations incorporate otorrhoea, otalgia, dizziness and conductive hearing misfortune, polyps and granulations of the middle ear. Once in a while, facial nerve and vestibulocochlear nerve paralyses might be seen in addition to sensorineural hearing misfortune. These symptoms may cover the fundamental illness and postpone diagnosis until disappointment of symptomatic treatment administrations. Squamous cell carcinoma of the middle ear and temporal bone is an uncommon threat yet is related with significant dismalness and mortality, both attributable to the disorder forms themselves and the treatment modalities. Squamous cell carcinoma represents 60% to 80% of all this bone malignancies then basal cell carcinoma. The disorder influence guys and females similarly, with an age scope of 34-85 years. It is F Bas J Surg, December, 23, 2017 81 Temporal bone pathology, a report of three cases Isam M AlShareda often contended that chronic otitis media is a chance factor for temporal bone threat, particularly squamous cell carcinoma. The early symptoms looks like those of chronic suppurative otitis media, including purulent putrid otorrhoea, extreme otalgia, bleeding, and pruritus. Since the rate of disease is uncommon, separating between chronically draining ear with related inflammation and a malignancy is typically troublesome. Other exhibiting clinical features of malignancy incorporate hearing misfortune, headache, tinnitus, vertigo, and aural stuffing. Cranial nerve paralyses may happen. Spread into the glenoid fossa can bring about trismus, dural involvement and deliver extreme torment and headache. Case one: An 8 years old female patient, accidentally discovered mass behind the right ear which causing narrowing of the external auditory canal. On examination, the mass was originated from post auricular area and spreading to squamous and mastoid parts of temporal bone with limited extension to petromastoid area. Few weeks later mastoidectomy was done and right mastoid bone mass was resected completely and sent for histopathology, the diagnosis was fibrous dysplasia of temporal bone. One year later the mass came back and another mastoidectomy was done. One year After a second mastoid resection the mass is again reoccurred but this time a watchful follow-up was chosen by a committee from otolaryngologists and neurosurgeons due to lacking of advanced facilities. A series of CT scans were taken for about 6 years. During this period the mass increases in size (figure 1) and extended to the occipital region. When the patient became 15 years old, she had repeated attacks of headache and craniotomy is decided (figure 2) . Figure 1: Extension of the disease after 2 mastoidectomies. This image was one of series imaging for follow-up which shows no intracranial invasion Figure 2: Pre-craniotomy when the patient was 15 years old Bas J Surg, December, 23, 2017 82 Temporal bone pathology, a report of three cases Isam M AlShareda She had craniotomy and cranioplasty with bone cement in Tehran, Iran(fig.3). There was no full intraoperative details about findings, but she developed partial facial nerve palsy which lasts few months until functions returns normal. Few months later she developed postaural discharging sinus which is healed and reoccurred multiple times after treatment. Three years later the sinus remains discharging and the patient had some feeling of instability in her operated side of skull. Figure 3: Postoperative, craniotomy and cranioplasty with bone cement When the patient became 18 years old, another craniotomy was done in India, in which inferior part of cranioplasty was found infected with granulation tissue, a fistula was found leading up to external auditory canal (EAC) with normal tympanic membrane (fig.4). Cranioplasty implant was removed. There was a removal of the epithelium of EAC, tympanic membrane and remnant of malleus which was the only ossicle found. The Eustachian orifice was blocked with mucosa and fat. Figure 4. Preoperative revision craniotomy shows instability of the previous bone cement fragments. After 9 months a second cranioplasty was done using Midpore implant with clearing of middle ear cleft. Till now, after 6 years from the last operation, the patient condition is stable with no mass and live a healthy life and she have a beautiful two years old daughter. Case two : The condition started as bilateral ear discharge when a female patient was 2 years old. The discharge was foul white to green in color and was associated with right sided aural polyps. Treatment lasts for about 8 months with no complete response. CT scan was done when the patient was 2 years old which shows bilateral temporal lesion in hematogenous soft tissue masses, on the right the dimension was (44mm x32 mm) and on the left side was (33mm x 23 mm) with Bas J Surg, December, 23, 2017 83 Temporal bone pathology, a report of three cases Isam M AlShareda heterogenous enhancement after contrast injection. There was bony destruction of right mandibular ramus and both squamous and petrous parts of temporal bones. Also there was soft bony defect with irregular margins of upper petrous part of parietal bones with no intracranial extension or SOL as shown in figure 5. Figure 5: Bilateral soft tissue density with surrounding tissue destructions at time of diagnosis when the patient was 2 years old. Biopsy was taken from external auditory meatus as multiple pieces of soft tissue and the report showed features of Langerhans cells histiocytosis X or eosinophil granuloma involving external auditory meatus. Patient received chemotherapy as vinblastine, pridnisolon, 6-mercapto purine and co-trimoxazole for two years. Few years later, the patient still had repeated right ear infection and mastoiditis so modified radical mastoidectomy was done with canal wall down and meatoplasty. Cholesteatoma present when the patient was 10 years old which when sent to histopathology study shows granulation tissue with no Langerhance cell histeocytosis. Multiple imaging studies was done since then as CT (native and contrast) and brain MRI which shows complete resolution of the lesion, no mass but persistence mastoiditis at the right side (figure 6). Figure 6: Recent MRI from serial follow-up imaging studies when the patient is 12 years old with no recurrence of the disease. Now the patient is 12 years old, and still have mastoiditis with moderate CHL that affect her school performance, a hearing aid was prescribed to her. Case three: A 52 year old female presented with recent history of intense discharge from the right ear. She had history of intermittent recurrent right ear discharge for over 20 years as on & off attacks especially after exposures to water or wet climate. The discharge is foul, yellowish to green in color comes one to two times per month. This was followed gradually by loss of hearing and the patient described it as dead. The case was diagnosed as chronic suppurative otitis media and treated as such for many years but with no response. She had severe headache which was gradually increased by time, it started 6 months before consultation with vertigo and severe otalgia. Her past history was inconclusive, apart from pulmonary TB Bas J Surg, December, 23, 2017 84 Temporal bone pathology, a report of three cases Isam M AlShareda 10 years ago which has been treated by anti TB therapy. Otoscopic examination shows: Scanty discharge, sever retraction of tympanic membrane with invagination pocket at superior and inferior parts of TM with prominent middle folds, the attic cannot be visualized. Bilateral Rinne test was positive, Weber test is central. PTA: Right moderate mixed hearing loss. Left mild SNHL. Computerized tomography (CT) three months before surgery showed: Signs of obliteration of mastoid air cells and d


Introduction
ibrous dysplasia is a tumour-like lesion of the bone 1 .It is self-limiting, not encapsulated and is characterized by replacement of normal bone with cellular fibrous connective tissue and is of unknown etiology 2 .The disease may develop at early age, and progress actively during childhood and stabilize in adulthood 3 .This disease is much more frequent in white populations than black 1 and the monostotic form is found more often in females than in males.In onethird of the cases, it is located in the maxilla or mandible and sometimes the frontal or sphenoid sinus are obliterated by the disease 4 .
Eosinophilic granuloma is an uncommon, benign disease described by uncontrolled expansion of Langerhans cells.The most successive destinations of inclusion are the skull, spine, ribs, femur, and pelvis [5][6][7] .Lichtenstein assembled this infection range under the name histiocytosis X 8 .The "X" because the etiology was obscure.It is a solitary condition, typically restricted to skeleton with great anticipation.Kids and youthful grownups are most ordinarily influenced.Male to female proportion is 2:1 9 .In spite of the fact that the etiology is obscure, injury, genetic pattern, immune system mechanism, metabolic problems, and viral contaminations have all been suspected.Temporal bone contribution is moderately uncommon 6,[10][11][12] .One of the announced disease clinical presentation is osteolytic type.Normally present manifestations incorporate otorrhoea, otalgia, dizziness and conductive hearing misfortune, polyps and granulations of the middle ear.Once in a while, facial nerve and vestibulocochlear nerve paralyses might be seen in addition to sensorineural hearing misfortune.These symptoms may cover the fundamental illness and postpone diagnosis until disappointment of symptomatic treatment administrations [13][14][15] .Squamous cell carcinoma of the middle ear and temporal bone is an uncommon threat yet is related with significant dismalness and mortality, both attributable to the disorder forms themselves and the treatment modalities.Squamous cell carcinoma represents 60% to 80% of all this bone malignancies then basal cell carcinoma 16 .The disorder influence guys and females similarly, with an age scope of 34-85 years 17 .It is F Bas J Surg, December, 23, 2017  often contended that chronic otitis media is a chance factor for temporal bone threat, particularly squamous cell carcinoma 16 .The early symptoms looks like those of chronic suppurative otitis media, including purulent putrid otorrhoea, extreme otalgia, bleeding, and pruritus.Since the rate of disease is uncommon, separating between chronically draining ear with related inflammation and a malignancy is typically troublesome.Other exhibiting clinical features of malignancy incorporate hearing misfortune, headache, tinnitus, vertigo, and aural stuffing.Cranial nerve paralyses may happen.Spread into the glenoid fossa can bring about trismus, dural involvement and deliver extreme torment and headache 16 .

Case one:
An 8 years old female patient, accidentally discovered mass behind the right ear which causing narrowing of the external auditory canal.On examination, the mass was originated from post auricular area and spreading to squamous and mastoid parts of temporal bone with limited extension to petromastoid area.Few weeks later mastoidectomy was done and right mastoid bone mass was resected completely and sent for histopathology, the diagnosis was fibrous dysplasia of temporal bone.One year later the mass came back and another mastoidectomy was done.One year After a second mastoid resection the mass is again reoccurred but this time a watchful follow-up was chosen by a committee from otolaryngologists and neurosurgeons due to lacking of advanced facilities.A series of CT scans were taken for about 6 years.During this period the mass increases in size (figure 1) and extended to the occipital region.When the patient became 15 years old, she had repeated attacks of headache and craniotomy is decided (figure 2) .She had and cranioplasty with bone cement in Tehran, Iran(fig.3).There was no full intraoperative details about findings, but she developed partial facial nerve palsy which lasts few months until functions returns normal.Few months later she developed postaural discharging sinus which is healed and reoccurred multiple times after treatment.Three years later the sinus remains discharging and the patient had some feeling of instability in her operated side of skull.

Figure 3: Postoperative, craniotomy and cranioplasty with bone cement
When the patient became 18 years old, another craniotomy was done in India, in which inferior part of cranioplasty was found infected with granulation tissue, a fistula was found leading up to external auditory canal (EAC) with normal tympanic membrane (fig.4).Cranioplasty implant was removed.There was a removal of the epithelium of EAC, tympanic membrane and remnant of malleus which was the only ossicle found.The Eustachian orifice was blocked with mucosa and fat.

Figure 4. Preoperative revision craniotomy shows instability of the previous bone cement fragments.
After 9 months a second cranioplasty was done using Midpore implant with clearing of middle ear cleft.Till now, after 6 years from the last operation, the patient condition is stable with no mass and live a healthy life and she have a beautiful two years old daughter.

Case two :
The condition started as bilateral ear discharge when a female patient was 2 years old.The discharge was foul white to green in color and was associated with right sided aural polyps.Treatment lasts for about 8 months with no complete response.CT scan was done when the patient was 2 years old which shows bilateral temporal lesion in hematogenous soft tissue masses, on the right the dimension was (44mm x32 mm) and on the left side was (33mm x 23 mm) with heterogenous enhancement contrast injection.There was bony destruction of right mandibular ramus and both squamous and petrous parts of temporal bones.Also there was soft bony defect with irregular margins of upper petrous part of parietal bones with no intracranial extension or SOL as shown in figure 5.  Now the patient is 12 years old, and still have mastoiditis with moderate CHL that affect her school performance, a hearing aid was prescribed to her.

Case three:
A 52 year old female presented with recent history of intense discharge from the right ear.She had history of intermittent recurrent right ear discharge for over 20 years as on & off attacks especially after exposures to water or wet climate.The discharge is foul, yellowish to green in color comes one to two times per month.This was followed gradually by loss of hearing and the patient described it as dead.The case was diagnosed as chronic suppurative otitis media and treated as such for many years but with no response.She had severe headache which was gradually increased by time, it started 6 months before consultation with vertigo and severe otalgia.
Her past history was inconclusive, apart from pulmonary TB 10 years ago which has been treated by anti TB therapy.Otoscopic examination shows: Scanty discharge, sever retraction of tympanic membrane with invagination pocket at superior and inferior parts of TM with prominent middle folds, the attic cannot be visualized.Bilateral Rinne test was positive, Weber test is central.PTA: Right moderate mixed hearing loss.Left mild SNHL.Computerized tomography (CT) three months before surgery showed: Signs of obliteration of mastoid air cells and destruction of its wall with destruction of ossicles and blunting of scutum but intact tegmen tympani and normal inner ear as shown in figure 7.

F
The patient had exploration of the right astoidectomy showed patient said it is ther had been referred to ear and radical m large mass eroded temporal bone, sigmoid sinus and dura.Biopsy was taken which revealed invasive well to moderately differentiated squamous cell carcinoma of mastoid cavity grade 1 and 2.She had uneventful post-operative period with no complications.Postoperative breast lump was noted but the advised fine needle aspiration but she refused.One month post-operative brain MRI showed: 25x27x33 mm heterogeneous enhancing tissue in right petrous bone.The internal auditory canal, cochlea and semicircular canals appear intact, with no evident brain metastasis as shown in figure 9. Squamous cell carcinoma represents above all temporal bone se in between.Now the patient condition is stable but there is some tinnitus and 30 months after mastoidectomy (fig.10) and now she is on follow-up.

F Discussion
The simple presence of fibrous does not justify if followed by significant clinical symptoms 18 .The indication for surgery of temporal bone fibrous dysplasia includes bone invasion of external auditory canal enough to produce conductive hearing loss, recurrent infections and secondary cholesteatoma.Surgical procedure aims at achieving 3 objectives: restoration of the function, prevention of complications, and case condition improvement.Radiotherapy should be avoided owing to high incidence of malignant transformation 19 .Prognosis is good in most cases as in case one but it depends on the disease severity.There are no particular signs or symptoms for Langerhans Cell His including the skull, and the most widely recognized introduction is an agonizing and stable scalp mass which might be obvious in certain cases 20,21 .In the present investigation, the patient experienced bilateral ear discharge mainly from the right ear with no reaction to treatment.In this way, imaging examinations were fundamental which was situated in both temporal bones, and a wide hard defect was distinguished.As the greater part of LCH cases have skull contribution 21 , it was suitable to think that the lesion begun from the skull.Lately, positron emanation recognizable proof of LCH lesions and in the assessment of patient reactions to therapy 22,23 .Once the finding of LCH has been affirmed, it is vital to evaluate the association of other organs, as LCH is normally a systemic condition.In the present case, many general tests were performed, which affirm that no other organs were included.Due to the potential for improvement of systemic treatment is suggested for the treatment of patients with lesions including the skull base, parietal and temporal bones 24 .Thus, chemotherapy treatment was performed first in the present case which was therapeutic to her.There is as yet a debate [25][26][27] with utilization of radiotherapy to treat LCH patients, this case got chemotherapy alone since radiotherapy was not accessible in the country at that time.Temporal bone and middle ear squamous cell carcinoma is uncommon, however, is related with impressive morbidity and mortality, both due to process itself and the treatment modalities..Mastoidectomy, resection of the lateral temporal bone and its subtotal resection are currently more appropriate 28 .In a review investigation, radical radiotherapy was proposed a treatment of choice for patients with beginningof squamous cell carcinoma of the outer auditory canal and middle ear 29 .Additionally, surgery, with negative surgical edges if conceivable, and radiotherapy was prescribed in this investigation as the standard care to cases of advanced stage.
The disparity in the studies itself shows that it is di about treatment results for mastoid squamous carcinoma from the literature.As a rule, the treatment of temporal bone carcinoma potentially followed by radiation.The disease prognosis remains terribly bad owing to the late presentation is far more worse in cases with facial nerve paralysis, positive tumor edges, dural inclusion, and local lymph nodes inclusion 16 .

Figure 2 :
Figure 2: Pre-craniotomy when the patient was 15 years old

Figure 5 :
Figure 5: Bilateral soft tissue density with surrounding tissue destructions at time of diagnosis when the patient was 2 years old.Biopsy was taken from external auditory meatus as multiple pieces of soft tissue and the report showed features of Langerhans cells histiocytosis X or eosinophil granuloma involving external auditory meatus.Patient received chemotherapy as vinblastine, pridnisolon, 6-mercapto purine and co-trimoxazole for two years.Few years later, the patient still had repeated right ear infection and mastoiditis so modified radical

Figure 6 :
Figure 6: Recent MRI from serial follow-up imaging studies when the patient is 12 years old with no recurrence of the disease.

Figure 9 :
Figure 9: MRI R The disease can be treated by, surgery or radiotherapy or a joined approach is still dubious as a conclusive study isn't yet done because the disease is rare.Surgeries classically incorporate sleeve resection, lateral temporal bone resection, subtotal or total resection of temporal bone.While a few investigators advocate total en bloc removal of the tumor, the others contend for piecemeal expulsion of gross tumor with protection of indispensable neurovascular structures took after by radiation therapy