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A rare case of extensive cranial Langerhans cell histiocytosis, synchronously presenting as otitis externa and giant cell arteritis

A rare case of extensive cranial Langerhans cell histiocytosis, synchronously presenting as... Langerhans cell histiocytosis (LCH) is a rare histiocytic disorder of unpredictable clinical course and varied modes of presen- tation. The spectrum of presentation is wide, ranging from isolated eosinophilic granulomas to multiple lesions and diffuse systemic involvement. We present the case of a 52-year-old man, who presented with an 8-week history of worsening otalgia and superficial temporal tenderness attributed to otitis externa within the community and subsequently giant cell arteritis. Computed tomography and magnetic resonance imaging were undertaken due to atypical features, which demon- strated bony destruction within the right greater wing of the sphenoid, squamous part of temporal and mastoid bone, with middle cranial fossa communication. Intra-orbital extension was noted with abutment of the lateral rectus muscle. Mastoid biopsies demonstrated a mixture of lymphocytes, eosinophils and monomorphic epithelial cells with pale cytoplasm and focal areas of granulation tissue/necrosis. The features were consistent with a diagnosis of LCH, and the patient was subsequently transferred to a tertiary centre for definitive treatment. CASE HISTORY medical team for assessment and management of a potential We present a rare case of a 52-year-old man, who presented to giant cell arteritis (GCA). A review was undertaken by the our otolaryngology emergency clinic, with an 8-week history of rheumatology team, who felt the nature of his presentation was worsening right-sided otalgia and superficial temporal tender- atypical of GCA but was nevertheless commenced on steroid ness attributed to otitis externa in the community. He had been therapy, and a further review by the otolaryngology team was commenced on several courses of oral antibiotics without any requested. On admission, with the exception of a mildly elevated alleviation in his symptoms, and concerns were raised regard- erythrocyte sedimentation rate (49) mm/hr, all biochemical and ing the possibility of necrotising otitis externa given a history haematological parameters were within normal range. On exam- of diabetes mellitus. On review, there was no overt evidence of ination, pain was localised superficially to the right temporal an infective pathology, and as a result, the patient was dis- artery distribution. Otoscopy was unremarkable, with no evi- charged accordingly. A fortnight later, the patient re-presented dence of granulation tissue, polypoidal masses or discharge within the community and was subsequently referred to the within the external auditory canal. On further questioning, the Received: April 11, 2016. Accepted: November 7, 2016 Published by Oxford University Press and JSCR Publishing Ltd. All rights reserved. © The Author 2016. This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/ licenses/by-nc/4.0/), which permits non-commercial re-use, distribution, and reproduction in any medium, provided the original work is properly cited. For commercial re-use, please contact journals.permissions@oup.com 1 2 A. Darr et al. patient did report intermittent nocturnal deep rooted otalgia, in demonstrated bony erosion of the lateral wall of the right orbit, the absence of tinnitus or vertigo. This was coupled with mild right superior rectus muscle abutment and minimal involvement intermittent right-sided retro-orbital pain within the preceding 4 of the antero-inferior surface of the temporal lobe. The appear- months, which prompted radiological evaluation. ancesweresuggestiveof fibrous dysplasia, chronic inflammatory/ A contrast enhanced computed tomography (CT) scan was infective pathology or less likely metastatic carcinoma. undertaken, which demonstrated diffuse pathology of the skull A right cortical mastoidectomy was undertaken shortly after base on the right, with mixed sclerotic and lytic destructive admission, and histopathological evaluation demonstrated areas pathology. There was a large segment of bony destruction with a mixture of lymphocytes, eosinophils and monomorphic within the right greater wing of the sphenoid bone, with ero- epithelial cells with pale cytoplasms. Focal areas of granulation sion of right foramina ovale. Further prominent defects were as well as necrosis were also noted. The epithelial cells stained found within the squamous part of temporal bone, as well as a with CD1a, S100 and CD68 indicating that they are epithelial cells. destructive lesion within the right mastoid (Fig. 1), communi- CD1a and CD68 show ghost cells in the necrotic areas, features cating with the middle cranial fossa. There was significant soft consistent with a diagnosis of Langerhans cell histiocytosis (LCH). tissue thickening within the infra-temporal fossa, with exten- The patient was subsequently referred to a tertiary centre for sion into middle cranial fossa region, with possible localised discussion at a regional skull base multidisciplinary meeting, and meningeal/dural thickening in the temporal fossa (Fig. 2). given the extensive nature of the disease process, surgical inter- Furthermore, there was mild intra-orbital extension with abut- vention was deemed futile, and an onward referral was made to ment of the right lateral rectus muscle. haematology. A diagnosis of cranial diabetes insipidus, a finding Duetothe extensivenatureofthe findings, a magnetic in up to 30% of patients [1], was coincidentally made, and the resonance imaging (MRI) scan of the head was undertaken and patient was initially commenced on azathioprine (a single system regime). At a 2-month follow-up, a repeat MRI scan demonstrated drastic regression in the disease process, particularly within the temporal and suborbital components. Although the extensive involvement of >1 facial/cranial bones would categorise this pro- cess as multi-system LCH (thus warranting treatment with cytar- abine and etoposide), adequate control of symptoms, coupled with radiological recovery, has dictated an observational approach with no further change to his current treatment regime. DISCUSSION LCH (formerly referred to as Histiocytosis X), includes the disor- ders eosinophilic granuloma, Letterer–Siwe disease and Hand– Schüller–Christian disease. Although the aetiology remains uncertain, it is postulated to be a reactive disease secondary to abnormal immune regulation and demonstrates a predilection for the paediatric population [2–4]. Extensive skull base involve- ment at presentation is extremely rare [5] and usually confined to the orbit and cranial base [2, 6] Otologic manifestations, although rare, may include aural Figure 1: Axial CT images demonstrating bony involvement of the right mastoid discharge, postauricular swelling, vertigo and subjective hear- and greater wing of the sphenoid. ing loss [2, 3, 6], which explains why in its early stages, the disease is attributed to an underlying acute or chronic inflam- matory process [6]. Clinical findings included otitis media, otitis externa with or without granulation tissue, soft tissue masses and osteolytic lesions of the temporal bone [3]. Contrast enhanced CT proves useful in delineating osseous as well as temporal soft tissue involvement while simultan- eously demonstrating destruction of petrous apex. MRI further serves to support the aforementioned information while further clarifying the extent of soft tissue involvement. Confirmation of diagnosis is by means of a tissue biopsy, with immunohisto- chemistry demonstrating Langerhans’ cells in association with inflammatory infiltrate consisting of lymphocytes, disproportion- ate eosinophils, giant cells and plasma cells. Our rare case of extensive multifocal skull base disease illustrates the rapid onset, uncommon nature and variation in presentation of LCH. It is more routinely associated in the paediatric population and with systemic involvement in approximately two-thirds of cases [2, 3]. Our report consoli- dates further on the limited literature available on LCH-related skull vault lesions within adults while emphasising the crucial role of an otolaryngologist in the early evaluation, staging and Figure 2: Axial MRI image demonstrating ocular involvement, with abutment of right lateral rectus muscle, as well as enhancement of temporal lobe. subsequent diagnosis of LCH. This is particularly crucial given Extensive LCH presenting as otitis externa and GCA 3 the age at diagnosis, and initial response correlates closely to in adult patients with Langerhans cell histiocytosis: clinical, prognosis and recurrence. Persistent ear symptoms and atyp- endocrinological and radiological features and response to ical localised deep rooted and superficial vault pain should treatment. J Clin Endocrinol Metab 2000;85:1370–6. prompt otolaryngologists to incorporate LCH when formulating 2. Cunningham MJ, Curtin HD, Jaffe R, Stool SE. Otologic mani- differential diagnoses. Long-term follow-up in such patients is festations of Langerhans’ cell histiocytosis. Arch Otolaryngol critical, with relapses likely to occur years beyond initial treat- Head Neck Surg 1989;115:807–13. ment [5], which may incorporate a conservative medical 3. McCaffrey TV, McDonald TJ. Histiocytosis X of the ear and (chemotherapy, local steroid therapy or bisphosphonates) or a temporal bone: review of 22 cases. Laryngoscope 1979;89: surgical (curette or excision) approach. Radiotherapy is gener- 1735–42. ally reserved for cases of neurological deficits. 4. Irving RM, Broadbent V, Jones NS. Langerhans’ cell histiocy- tosis in childhood: management of head and neck manifes- tations. Laryngoscope 1994;104:64–70. CONFLICT OF INTEREST STATEMENT 5. Quraishi MS, Blayney AW, Walker D, Breatnach FB, Bradley None declared. PJ. Langerhans’ cell histiocytosis: head and neck manifesta- tions in children. Head Neck 1995;17:226–31. 6. Modest MC, Garcia JJ, Arndt CS, Carlson ML. Langerhans REFERENCES cell histiocytosis of the temporal bone: a review of 29 1. Katlas GA, Powles TB, Evanson J, Plowman PN, Drinkwater cases at a single center. Laryngoscope 2015; Epub. PMID: JE, Jenkins PJ, et al. Hypoathalamo-pituitary abnormalities 26535795. http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Journal of Surgical Case Reports Oxford University Press

A rare case of extensive cranial Langerhans cell histiocytosis, synchronously presenting as otitis externa and giant cell arteritis

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Oxford University Press
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Published by Oxford University Press and JSCR Publishing Ltd. All rights reserved. © The Author 2016.
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2042-8812
DOI
10.1093/jscr/rjw094
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Abstract

Langerhans cell histiocytosis (LCH) is a rare histiocytic disorder of unpredictable clinical course and varied modes of presen- tation. The spectrum of presentation is wide, ranging from isolated eosinophilic granulomas to multiple lesions and diffuse systemic involvement. We present the case of a 52-year-old man, who presented with an 8-week history of worsening otalgia and superficial temporal tenderness attributed to otitis externa within the community and subsequently giant cell arteritis. Computed tomography and magnetic resonance imaging were undertaken due to atypical features, which demon- strated bony destruction within the right greater wing of the sphenoid, squamous part of temporal and mastoid bone, with middle cranial fossa communication. Intra-orbital extension was noted with abutment of the lateral rectus muscle. Mastoid biopsies demonstrated a mixture of lymphocytes, eosinophils and monomorphic epithelial cells with pale cytoplasm and focal areas of granulation tissue/necrosis. The features were consistent with a diagnosis of LCH, and the patient was subsequently transferred to a tertiary centre for definitive treatment. CASE HISTORY medical team for assessment and management of a potential We present a rare case of a 52-year-old man, who presented to giant cell arteritis (GCA). A review was undertaken by the our otolaryngology emergency clinic, with an 8-week history of rheumatology team, who felt the nature of his presentation was worsening right-sided otalgia and superficial temporal tender- atypical of GCA but was nevertheless commenced on steroid ness attributed to otitis externa in the community. He had been therapy, and a further review by the otolaryngology team was commenced on several courses of oral antibiotics without any requested. On admission, with the exception of a mildly elevated alleviation in his symptoms, and concerns were raised regard- erythrocyte sedimentation rate (49) mm/hr, all biochemical and ing the possibility of necrotising otitis externa given a history haematological parameters were within normal range. On exam- of diabetes mellitus. On review, there was no overt evidence of ination, pain was localised superficially to the right temporal an infective pathology, and as a result, the patient was dis- artery distribution. Otoscopy was unremarkable, with no evi- charged accordingly. A fortnight later, the patient re-presented dence of granulation tissue, polypoidal masses or discharge within the community and was subsequently referred to the within the external auditory canal. On further questioning, the Received: April 11, 2016. Accepted: November 7, 2016 Published by Oxford University Press and JSCR Publishing Ltd. All rights reserved. © The Author 2016. This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/ licenses/by-nc/4.0/), which permits non-commercial re-use, distribution, and reproduction in any medium, provided the original work is properly cited. For commercial re-use, please contact journals.permissions@oup.com 1 2 A. Darr et al. patient did report intermittent nocturnal deep rooted otalgia, in demonstrated bony erosion of the lateral wall of the right orbit, the absence of tinnitus or vertigo. This was coupled with mild right superior rectus muscle abutment and minimal involvement intermittent right-sided retro-orbital pain within the preceding 4 of the antero-inferior surface of the temporal lobe. The appear- months, which prompted radiological evaluation. ancesweresuggestiveof fibrous dysplasia, chronic inflammatory/ A contrast enhanced computed tomography (CT) scan was infective pathology or less likely metastatic carcinoma. undertaken, which demonstrated diffuse pathology of the skull A right cortical mastoidectomy was undertaken shortly after base on the right, with mixed sclerotic and lytic destructive admission, and histopathological evaluation demonstrated areas pathology. There was a large segment of bony destruction with a mixture of lymphocytes, eosinophils and monomorphic within the right greater wing of the sphenoid bone, with ero- epithelial cells with pale cytoplasms. Focal areas of granulation sion of right foramina ovale. Further prominent defects were as well as necrosis were also noted. The epithelial cells stained found within the squamous part of temporal bone, as well as a with CD1a, S100 and CD68 indicating that they are epithelial cells. destructive lesion within the right mastoid (Fig. 1), communi- CD1a and CD68 show ghost cells in the necrotic areas, features cating with the middle cranial fossa. There was significant soft consistent with a diagnosis of Langerhans cell histiocytosis (LCH). tissue thickening within the infra-temporal fossa, with exten- The patient was subsequently referred to a tertiary centre for sion into middle cranial fossa region, with possible localised discussion at a regional skull base multidisciplinary meeting, and meningeal/dural thickening in the temporal fossa (Fig. 2). given the extensive nature of the disease process, surgical inter- Furthermore, there was mild intra-orbital extension with abut- vention was deemed futile, and an onward referral was made to ment of the right lateral rectus muscle. haematology. A diagnosis of cranial diabetes insipidus, a finding Duetothe extensivenatureofthe findings, a magnetic in up to 30% of patients [1], was coincidentally made, and the resonance imaging (MRI) scan of the head was undertaken and patient was initially commenced on azathioprine (a single system regime). At a 2-month follow-up, a repeat MRI scan demonstrated drastic regression in the disease process, particularly within the temporal and suborbital components. Although the extensive involvement of >1 facial/cranial bones would categorise this pro- cess as multi-system LCH (thus warranting treatment with cytar- abine and etoposide), adequate control of symptoms, coupled with radiological recovery, has dictated an observational approach with no further change to his current treatment regime. DISCUSSION LCH (formerly referred to as Histiocytosis X), includes the disor- ders eosinophilic granuloma, Letterer–Siwe disease and Hand– Schüller–Christian disease. Although the aetiology remains uncertain, it is postulated to be a reactive disease secondary to abnormal immune regulation and demonstrates a predilection for the paediatric population [2–4]. Extensive skull base involve- ment at presentation is extremely rare [5] and usually confined to the orbit and cranial base [2, 6] Otologic manifestations, although rare, may include aural Figure 1: Axial CT images demonstrating bony involvement of the right mastoid discharge, postauricular swelling, vertigo and subjective hear- and greater wing of the sphenoid. ing loss [2, 3, 6], which explains why in its early stages, the disease is attributed to an underlying acute or chronic inflam- matory process [6]. Clinical findings included otitis media, otitis externa with or without granulation tissue, soft tissue masses and osteolytic lesions of the temporal bone [3]. Contrast enhanced CT proves useful in delineating osseous as well as temporal soft tissue involvement while simultan- eously demonstrating destruction of petrous apex. MRI further serves to support the aforementioned information while further clarifying the extent of soft tissue involvement. Confirmation of diagnosis is by means of a tissue biopsy, with immunohisto- chemistry demonstrating Langerhans’ cells in association with inflammatory infiltrate consisting of lymphocytes, disproportion- ate eosinophils, giant cells and plasma cells. Our rare case of extensive multifocal skull base disease illustrates the rapid onset, uncommon nature and variation in presentation of LCH. It is more routinely associated in the paediatric population and with systemic involvement in approximately two-thirds of cases [2, 3]. Our report consoli- dates further on the limited literature available on LCH-related skull vault lesions within adults while emphasising the crucial role of an otolaryngologist in the early evaluation, staging and Figure 2: Axial MRI image demonstrating ocular involvement, with abutment of right lateral rectus muscle, as well as enhancement of temporal lobe. subsequent diagnosis of LCH. This is particularly crucial given Extensive LCH presenting as otitis externa and GCA 3 the age at diagnosis, and initial response correlates closely to in adult patients with Langerhans cell histiocytosis: clinical, prognosis and recurrence. Persistent ear symptoms and atyp- endocrinological and radiological features and response to ical localised deep rooted and superficial vault pain should treatment. J Clin Endocrinol Metab 2000;85:1370–6. prompt otolaryngologists to incorporate LCH when formulating 2. Cunningham MJ, Curtin HD, Jaffe R, Stool SE. Otologic mani- differential diagnoses. Long-term follow-up in such patients is festations of Langerhans’ cell histiocytosis. Arch Otolaryngol critical, with relapses likely to occur years beyond initial treat- Head Neck Surg 1989;115:807–13. ment [5], which may incorporate a conservative medical 3. McCaffrey TV, McDonald TJ. Histiocytosis X of the ear and (chemotherapy, local steroid therapy or bisphosphonates) or a temporal bone: review of 22 cases. Laryngoscope 1979;89: surgical (curette or excision) approach. Radiotherapy is gener- 1735–42. ally reserved for cases of neurological deficits. 4. Irving RM, Broadbent V, Jones NS. Langerhans’ cell histiocy- tosis in childhood: management of head and neck manifes- tations. Laryngoscope 1994;104:64–70. CONFLICT OF INTEREST STATEMENT 5. Quraishi MS, Blayney AW, Walker D, Breatnach FB, Bradley None declared. PJ. Langerhans’ cell histiocytosis: head and neck manifesta- tions in children. Head Neck 1995;17:226–31. 6. Modest MC, Garcia JJ, Arndt CS, Carlson ML. Langerhans REFERENCES cell histiocytosis of the temporal bone: a review of 29 1. Katlas GA, Powles TB, Evanson J, Plowman PN, Drinkwater cases at a single center. Laryngoscope 2015; Epub. PMID: JE, Jenkins PJ, et al. Hypoathalamo-pituitary abnormalities 26535795.

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Journal of Surgical Case Reports – Oxford University Press

Published: Nov 11, 2016

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