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Radiculopathy as a manifestation of Langerhans' cell
histiocytosis.
Joint Bone Spine. 2007
Mar;74(2):190-3.
Langerhans'
cell histiocytosis (LCH) is a rare condition of children and young
adults in which Langerhans' cells proliferate. The clinical spectrum
ranges from solitary or few focal lesions to multisystem involvement
mimicking vasculitis or hematological malignancy. Focal bone
lesions, known as eosinophilic granulomas, are the most common
manifestations. Eosinophilic granuloma usually presents with a
variable combination of pain, swelling, fracture, and fever. Facial
bone involvement may manifest as an ear discharge, hearing loss, or
exophthalmos. Nerve root pain is rarely reported, even in patients
with lesions in the axial skeleton. We report four cases of nerve
root pain caused by LCH. Two male patients aged 25 and 34 years,
respectively, presented with truncated femoral neuralgia related to
acetabular granulomas. A 25-year-old woman with involvement of the
L5 vertebral body and a 41-year-old man with a sacral lesion
presented with sciatica.
Cochlear implantation for
treatment-induced ototoxic deafness in Langerhans cell histiocytosis.
A case report.Eur
Arch Otorhinolaryngol. 2005
Jun;262(6):496-500.
A case is
presented where the authors carried out a cochlear implantation on a
girl whose deafness was induced by cytostatic agents. The normally
hearing child was diagnosed with Langerhans cell histiocytosis at
the age of 3 years and received polychemotherapy for almost 2 years.
By her 5th year, she started to develop a bilateral, progressive
sensorineural hearing loss, necessitating a hearing aid in both
ears. While her histiocytosis was cured, her hearing ultimately
deteriorated to total deafness on the right and profound hearing
loss on the left ear. After 3 years, her hearing aids no longer
provided adequate hearing for the postlingually deafened girl. At
the age of 14, a cochlear implantation was performed on her right
ear with excellent results.
Mondini
dysplasia and recurrent bacterial meningitis in a girl with
relapsing Langerhans cell histiocytosis.
Pediatr Blood Cancer. 2004
Jul;43(1):85-7.
We report a
case of a girl with Langerhans cell histiocytosis (LCH) of
multifocal bone disease, who developed recurrent bacterial
meningitis and unilateral sensorineural hearing loss during the
relapsing course of the disease. Mondini dysplasia, a congenital
inner ear anomaly, was suspected by high resolution computed
tomographic scan and the dysplasia with cerebrospinal fluid leakage
was confirmed by surgery in the ipsilateral ear showing hearing
loss. Although rare, congenital inner ear anomalies such as Mondini
dysplasia should be kept in mind in pediatric patients with hearing
impairment and/or recurrent bacterial meningitis during chemotherapy
for various types of neoplasms including LCH.
Langerhans'
cell histiocytosis of the labyrinth in adults.Otol
Neurotol. 2004 Jan;25(1):27-32.
OBJECTIVE: To describe and analyze three unusual cases of Langerhans'
cell histiocytosis of the temporal bone in adults. STUDY DESIGN:
Retrospective case review. SETTING: A tertiary referral center.
PATIENTS: Three adult patients with progressive sensorineural or
mixed hearing loss, vertigo, and tinnitus as presenting symptoms of
Langerhans' cell histiocytosis of the temporal bone. INTERVENTION:
Patients were evaluated by means of computed tomography and magnetic
resonance imaging. All patients underwent complete surgical excision
of the lesion via a transmastoid approach, extended to a
translabyrinthine approach in one case. One patient with a
multifocal disease underwent excision of a mandibular lesion 1 year
later, followed-up by chemotherapy. RESULTS: The Langerhans' cell
histiocytosis was located adjacent to or within the area of the
endolymphatic sac region in all cases. The middle ear was spared.
Hearing function was preserved in the two patients who had
serviceable hearing preoperatively. No complication occurred.
CONCLUSION: Langerhans' cell histiocytosis of the temporal bone is
centered on or adjacent to the endolymphatic sac. The fact that the
endolymphatic sac plays an immunologic role in the inner ear
suggests that the infiltration of the Langerhans' cell may derive
from it.
Langerhans' cell histiocytosis with bilateral temporal bone
involvement.Am
J Otolaryngol. 2003
Jul-Aug;24(4):265-70.
BACKGROUND:
Langerhans' cell histiocytosis (LCH) is a rare disease with variable
clinical appearance. The etiology of LCH remains unclear to date. It
is currently believed that clonal accumulation and proliferation of
CD1a-positive Langerhans' cells are causative. METHODS: A 2-year-old
boy presented with hypacusis and disturbance of balance. Auditory
brainstem-evoked responses (ABRs) revealed severe bilateral
labyrinthine hearing loss. Magnetic resonance imaging (MRI) showed
inflammatory changes with bone erosion in both temporal bones
including labyrinthine systems and mastoid processes. RESULTS:
During bilateral surgical exploration, fragile slightly yellowish
tissue with necrotic areas was found that turned out to be LCH on
histology. Chemotherapy with vinblastine and prednisone was
subsequently initiated, leading to continuing complete remission.
CONCLUSIONS: The initial presentation of LCH with bilateral temporal
bone involvement is a very rare condition. The signs and symptoms of
otologic histiocytosis can mimic those of acute and chronic
infectious ear disease. Only a surgically obtained biopsy leads to
definitive diagnosis and appropriate therapy.
Eosinophilic
granuloma of the temporal bone.Auris
Nasus Larynx. 2001 Jan;28(1):99-102.
Histiocytosis X or Langerhans cell histiocytosis (LCH) is a disease
that possesses three less distinctive and overlapping states called
eosinophilic granuloma (EG), Hand-Schuller-Christian (HSC) disease
and Letterer-Siwe (LS) disease. EG is the least severe and localized
form of all LCHs and possesses the best prognostic result. A high
index of suspicion is required to diagnose the EG, especially when
an ear disease is refractory to medical treatment. Early detection
is important to manage the EG properly and to minimize the
complications or sequels of treatment. Definitive diagnosis of
histiocytosis is made by histopathological means and
immunohistochemical detection of S-100 and CD1 antigens in the
tissue samples. And differential diagnosis of the subgroups is made
according to the clinical manifestations such as visceral organ or
bone involvement. Surgical excision, radiotherapy and chemotherapy,
either alone or in combination, are the main treatment options.
Ear
involvement in childhood Langerhans' cell histiocytosis.Head
Neck. 2000;22(1):42-7.
BACKGROUND:
Ear involvement (EI) in Langerhans' cell histiocytosis (LCH) occurs
quite often. We reviewed the Italian pediatric population of 251
children with LCH diagnosed between 1982 and 1995, focusing on EI,
to highlight the prevalence, the clinical presentation, the outcome
during follow-up, and the prognostic impact of otologic LCH.
METHODS: EI was defined by chronic otorrhea and/or mastoid
infiltration, external auditory meatus lesions, and middle/internal
EI. The age at diagnosis, sex, system involved, organ dysfunction,
treatment, disease control, and outcome were recorded. RESULTS: EI
was noted at presentation in 34 children (13. 5%). They had a
younger age at diagnosis (p=.0013) and near totality of multisystem
disease (93.8% of patients with EI). Among patients with multisystem
disease, children with EI seemed to have a higher risk of poor
response and a higher percentage of second line treatment (p=.003).
CONCLUSIONS: EI seems to identify patients with a particular disease
behavior, which requires a more accurate evaluation at diagnosis,
staging and treatment, and a strict follow-up, considering the
possibility of an unfavorable outcome.
Langerhans'
cell histiocytosis--a rare cause of sudden onset unilateral
sensorineural hearing loss.J
Laryngol Otol. 1999
Dec;113(12):1098-100.
Langerhans'
cell histiocytosis is a rare disorder of unknown aetiology in which
pathological Langerhans' cells accumulate and destroy local tissue.
We report a 38-year-old female who presented with a sudden onset of
left sensorineural hearing loss. Magnetic resonance imaging (MRI)
revealed a contrast-enhancing lesion in the left mastoid and a
second lesion in the hypothalamus. Following left mastoid
exploration and biopsy a definitive diagnosis of Langerhans' cell
histiocytosis was made and the patient was treated with external
beam radiotherapy. Subsequent right femur and right mastoid
involvement were successfully treated with steroids and cytotoxic
chemotherapy. At one year follow-up the patient had residual
left-sided sensorineural hearing loss with normal hearing in the
right ear. To our knowledge, Langerhans' cell histiocytosis has not
been previously reported as a cause of unilateral sudden onset
sensorineural hearing loss. It should be considered in the
differential diagnosis of this condition.
A case of
bilateral eosinophilic granuloma in the temporal bone.Auris
Nasus Larynx. 1999 Jul;26(3):343-8.
We present a
case of bilateral eosinophilic granuloma in the temporal bone in a
47-year-old woman, who visited our hospital with a headache and a
feeling of occlusion in her left ear. Her left tympanic membrane was
slightly turbid and pure tone audiometry revealed mild left
sensorineural deafness. CT disclosed a shadow of soft tissue in the
left mastoid antrum and mastoid cells, which was indicative of
marked destruction of the bone. Because MRI findings led us to
suspect otitis media cholesteatoma, a mastoidectomy was performed.
The mastoid antrum and mastoid cells were filled with easily
bleeding granulation, and there was a wide range of bone deficit in
the posterior cranial fossa. Histopathologically, the granulation
tissue was an eosinophilic granuloma. Her postoperative clinical
progress was good and she was discharged. However 2 months after
discharge, she had a feeling of occlusion in the right ear and CT
revealed a shadow in the right mastoid antrum and cells. Therefore,
right tympanoplasty was performed and the same findings as in the
left ear were obtained. A histopathological diagnosis of
eosinophilic granuloma was made again. To date, there has been no
recurrence.
Labyrinthine
involvement in Langerhans' cell histiocytosis.:
Int J Pediatr Otorhinolaryngol. 1998 Nov 15;46(1-2):109-15.
BACKGROUND:
Langerhans' cell histiocytosis, a rare condition caused by the
proliferation of abnormal Langerhans' cells ('LCH cells') and an
accompanying granulomatous infiltrate, can affect several organs
including the ear. External and middle ear involvement are common
with a reported incidence as high as 61%. The bony labyrinth is
resistant to erosion by the granulation tissue, thereby protecting
the cochlea and vestibular structures. Probably for this reason,
involvement of the inner ear is rare, with few case reports in the
literature. PATIENTS: We report two girls, one with bilateral and
the other with unilateral mastoid involvement, in whom there was
invasion of the labyrinth. The first girl had 'single system' LCH
affecting only bone and developed an acute hearing loss due to
invasion of the cochlea, while the second had both bone and skin
involvement and labyrinthine involvement was diagnosed on imaging
prior to the onset of labyrinthine symptoms. CONCLUSION: Inner ear
involvement can lead to permanent deafness, which may be prevented
by early institution of treatment. Threatened inner ear involvement
requires urgent systemic medical therapy with steroids, possibly
combined with chemotherapy.
Aural
symptoms as primary presentation of Langerhan's cell histiocytosis.
Clin Otolaryngol Allied Sci.
1993 Aug;18(4):317-23.
Langerhan's
cell histiocytosis is an uncommon granulomatous disease,
characterized by the idiopathic proliferation of Langerhan's cells
or their marrow precursors. It encompasses the diseases previously
associated with histiocytosis X-eosinophilic granuloma, Hand-Schuller-Christian
syndrome and Letterer-Siwe syndrome. A series of 54 patients were
diagnosed with this condition in Dublin over a 33-year-period
(1959-1992). Twenty-seven patients had aural symptoms, of whom 15
had no other lesions at the time of presentation. Otorrhoea was the
most frequent otological symptom, followed by lesions in the
temporal bone. LCH may mimic common aural conditions such as otitis
externa, otitis media or acute mastoiditis and a high index of
suspicion is required to recognize it on clinical presentation. The
clinical presentation of multi-focal disease may be so dramatic that
the otologic findings may be initially overlooked. The mortality
rate was 14.8%. Therapeutic regimes included no treatment,
curettage, chemotherapy, radiotherapy or multi-modality treatment.
Unifocal
Langerhans' cell histiocytosis (eosinophilic granuloma) of the
petrous apex.
Arch Otolaryngol Head Neck Surg. 1993
Jan;119(1):113-6.
Eosinophilic
granuloma is a localized form of histiocytosis X, or Langerhans'
cell histiocytosis, a benign lesion of proliferating Langerhans'
cells. It is the least severe of the histiocytosis syndromes, and is
characterized by lytic lesions of one or more bones. Temporal bone
lesions usually occur in association with multifocal disease;
however, isolated lesions may occur in either the mastoid bone
alone, or in the entire temporal bone, without disease elsewhere in
the body. We present the first case (to our knowledge) of
eosinophilic granuloma, or unifocal Langerhans' cell histiocytosis,
limited to the petrous apex. The patient, an 8-year-old girl,
presented with facial nerve paralysis. Because of delay in
definitive diagnosis, the disease extended beyond its original
boundaries and resulted in complete destruction of the temporal
bone. We will also review Langerhans' cell histiocytosis, with
attention to its involvement of the temporal bone.
Eosinophilic
granuloma of the ear. General review apropos of 2 case reports.
Ann Otolaryngol Chir Cervicofac. 1979
Apr-May;96(4-5):251-60.
The
eosinophilic granuloma is part of the histiocytosis X
classification, having a common histological substratum, the
granuloma, which is characterized by a proliferation of histiocytes
(reticulo-endothelial cells) and a cytoplasmic inclusion body (the X
body) whose étiopathogenic significance is not yet established.
These histiocytes are accompanied by other cells and in particular
granulocytes, the majority of which are eosinophils. Histiocyte
proliferation leads to a destruction of bone which is the organ
preferentially affected. At first there is osteolysis (this explains
the lacunar aspects seen radiologically) followed by sclerosis. The
clinical classification established by Lichtenstein in 1953 outlines
the tissue form (eosinophil granuloma), the acute disseminated form
(Letterersive) and the chronic disseminated form (Hand-Schüller-Christian).
This classification is still accepted today but it needs to be
refined to include the transitionary forms, and solitary bone
granulomas whose long terme evolutive nature it is impossible to
measure clinically, biologically, or histologically. From our
observation we note that therapy (surgery, radiotherapy,
chemotherapy) is effective in controlling the acute growth spurts in
a majority of the cases but does not seem to effect the potential
long term evolution.
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