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Rhabdomyosarcoma of the middle ear and mastoid: a case report and
review of the literature.Ear
Nose Throat J. 2005 Dec;84(12):780, 782, 784.
We report a
case of rhabdomyosarcoma of the middle ear and mastoid in a
3-year-old boy. The patient was treated according to Intergroup
Rhabdomyosarcoma Study IV protocol (chemo- and radiotherapy), and he
experienced a complete remission. However 7 months after the
completion of treatment, he experienced a recurrence at the primary
site that spread to the brain. Despite treatment, the patient died
of progressive metastasis to the lung 4 months later.
Paediatric
rhabdomyosarcoma of the ear and temporal bone.Clin
Otolaryngol Allied Sci. 2004
Feb;29(1):32-7.
The
objective of the present study was to review the presentation,
management, outcome and morbidity of paediatric patients presenting
to a single centre with rhabdomyosarcoma of the ear and temporal
region. All patients diagnosed with rhabdomyosarcoma of the ear and
temporal region between 1980 and 2000 were entered into this
retrospective study. Fourteen patients were identified. The median
age at presentation was 4.5 years with a mean time of onset of
symptoms to diagnosis of 21 weeks. In many patients, the
presentation mimicked that of chronic otitis media, delaying
diagnosis. Histological subtype was embryonal in 13 patients and
alveolar in 1. All patients underwent multimodality treatment. The
5-year disease-free survival rate was 81%. Regional post-treatment
morbidity included chronic aural discharge (6/14), facial palsy
(8/14), growth disturbance (4/14) and maxillo-facial deformity
occurring in four children. From the results, we conclude that these
patients should usually present to an ENT surgeon who should keep
the diagnosis in mind when dealing with children with chronic otitis
media as early diagnosis with referral to a specialist
multidisciplinary team will optimize the chance of survival.
Discharge, hearing loss and aural polyp, although commonly because
of chronic otitis media, should prompt urgent investigation and
biopsy, particularly if associated with facial palsy,
lymphadenopathy or an obvious mass.
Rhabdomyosarcoma of the ear and temporal bone.Laryngoscope.
1989 Nov;99(11):1188-92.
Rhabdomyosarcoma, the most common soft tissue sarcoma of childhood,
involves the temporal bone in approximately 7% of reported cases.
Until recently, the outcome of this disease was always fatal. The
recent Intergroup Rhabdomyosarcoma Study Group (IRS-I) reported on
the efficacy of multimodality therapy consisting of multiagent
chemotherapy, radiation, and surgical resection when indicated.
Twelve patients with rhabdomyosarcoma involving the temporal bone
were treated between 1966 and 1988. Three patients were treated
according to the IRS-I protocols and the remaining nine patients
received various combinations of treatment modalities. Ten patients
succumbed to their disease, most with distant metastases or
intracranial extension. Two patients are alive; one at 5 1/2 years
and one at 19 years. It is apparent that, although survival for
rhabdomyosarcoma in general has improved with the use of IRS-I
protocols, prognosis remains poor for disease involving the temporal
bone and other parameningeal sites.
Rhabdomyosarcoma of the middle ear and mastoid in children.J
Laryngol Otol. 1988 Jul;102(7):614-9.
Three cases
of embryonal rhabdomyosarcoma in the middle ear and mastoid in
children are presented. Diagnosis was confirmed by histopathology. A
multidisciplinary approach employing surgery, chemotherapy and
radiation therapy is the method of choice in the management of this
rare and highly lethal condition.
Embryonal
rhabdomyosarcoma of the ear: a review of the literature and case
history.J
Laryngol Otol. 1984 Dec;98(12):1261-6.
Approximately 50 per cent of all rhabdomyosarcomas in children occur
in the head and neck region with the orbit, nasopharynx and ear in
order of descending frequency. Embryonal rhabdomyosarcoma is the
commonest malignant tumour of the aural region in childhood and its
clinical course is usually rapidly fatal, with extensive local
disease and or distant metastases (Dehner and Chen, 1978). Other
malignant tumours that can occur in children include melanoma and
other mesenchymal tumours, including undifferentiated sarcoma,
fibrosarcoma, osteogenic sarcomas and Ewing sarcoma. Secondary
extension may occur from a meningioma. Osseous disorders of the
temporal bone, such as eosinophilic granuloma and Hand-Schüller-Christian
disease, should be included as a differential diagnosis (Lewis,
1979).
Rhabdomyosarcoma of the middle ear: a wolf in sheep's clothing.
Pediatrics.
1980 Jun;65(6):1131-3.
A 3-year-old
boy with inflammatory granulation tissue in the ear canal and
typanometric evidence of middle-ear effusion was examined.
Subsequently, rhabdomyosarcoma of the middle ear was diagnosed. The
insidious, deceptive presentation of this common pediatric tumor in
an uncommon location is discussed with indication that early
diagnosis and aggressive multimodal antitumor therapy seems to be
improving the hitherto gloomy prognosis for these highly lethal
middle-ear-neoplasms.
Temporal
bone findings in rhabdomyosarcoma with predominantly petrous
involvement.Arch
Otolaryngol. 1980 May;106(5):290-3.
Temporal bone rhabdomyosarcomas are rare tumors that occur almost
exclusively in children. They arise most commonly in the middle ear,
from which they extend through the mastoid and petrous portions of
the temporal bone, eventually invading the cranium. We report the
histopathologic findings of a rhabdomyosarcoma with anterior and
medial displacement, predominantly involving the petrous bone.
Recognition and separate study of this type of tumor is clinically
important. Predominantly petrosal rhabdomyosarcomas are less
prevalent than similar lesions with lateral expansion and tend to
develop significant ear symptoms late in the course of the disease.
When first diagnosed, these lesions usually exhibit signs of
intracranial extension, and consequently are less amenable to
treatment and are more rapidly fatal.
Rhabdomyosarcoma of the temporal bone. Is surgical resection
necessary?Arch
Otolaryngol. 1979 Jun;105(6):310-3.
Three patients had embryonal rhabdomyosarcoma of the temporal bone.
Their clinical appearances demonstrated the following
characteristics: (1) symptoms of an acute process in the middle ear
cleft and mastoid, (2) a rapidly growing polypoid mass that was
visible in the middle ear and external auditory canal, and (3)
seventh nerve involvement and destruction of bone. Definitive
treatment with the use of systemic chemotherapy and radiation
therapy to the invaded structures was followed by maintenance
chemotherapy for up to 24 months. Surgical treatment was sufficient
to obtain adequate biopsy material. All patients recovered variable
degrees of motor nerve functions. One patient experienced a
meningococcal meningitis years after treatment; this condition
resulted in total deafness. The results suggest that multiple-drug
chemotherapy and radiation therapy for all involved areas are the
mainstay of treatment for this disease entity.
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