Cholesteatoma secondary to temporal bone involvement by Langerhans cell histiocytosis: a complication amenable to curative surgery.
OBJECTIVE
To describe secondary acquired cholesteatoma in patients previously treated for Langerhans cell histiocytosis (LCH). To focus on misleading symptoms suggesting LCH relapse.


METHODS
This study involved 3 patients aged 12, 20, and 58 months whose conditions were diagnosed with LCH. All 3 had involvement of the mastoid and of the skin of the external ear canal (EAC) at diagnosis. They were treated with steroids and vinblastine.


METHODS
Serial computed tomographic (CT) scans and clinical follow-up.


METHODS
Exploratory surgery of the mastoid.


RESULTS
Otorrhea recurred in all 3 patients at 24, 17, and 26 months, respectively, with difficulties to clinically identify a hole in the posterior part of the EAC. The otorrhea was considered a sign of a new occurrence of LCH, leading to systemic chemotherapy in 2 cases. A CT scan showed a defect in the posterior wall of the EAC and suggested cholesteatomatous invasion of the mastoid (2 true cholesteatomas and 1 precholesteatomatous case).Surgery (canal wall up mastoidectomies) successfully removed the cholesteatoma (bilateral in 2 cases) and reconstructed the bony defect using cartilage. Biopsies ruled out LCH recurrence.


CONCLUSIONS
Secondary acquired cholesteatoma (through a bony defect of the EAC) may occur in patients previously treated for LCH. Recurrence of symptoms and bone destruction on CT may wrongly suggest LCH recurrence. Surgery allows removal of the cholesteatoma as well as EAC reconstruction and control biopsy.
Roger
Gilles
G
ENT Department, Armand Trousseau Children's Hospital, University Paris VI, AP-HP, Paris, France. gilles.roger@trs.aphp.fr
Dupré
Marie
M
Leboulanger
Nicolas
N
Godey
Benoit
B
Thomas
Caroline
C
Gandemer
Virginie
V
Bordure
Philippe
P
Garabedian
Erea-Noel
EN
Donadieu
Jean
J
D000972:Antineoplastic Agents, Phytogenic / Q000627:therapeutic use; D002558:Cerebrospinal Fluid Otorrhea / Q000209:etiology; D002675:Child, Preschool; D018424:Cholesteatoma, Middle Ear / Q000209:etiology* / Q000601:surgery*; D004424:Ear Canal / Q000473:pathology; D005260:Female; D006646:Histiocytosis, Langerhans-Cell / Q000150:complications* / Q000188:drug therapy / Q000473:pathology*; D006801:Humans; D007223:Infant; D008297:Male; D008416:Mastoid / Q000473:pathology; D013506:Otologic Surgical Procedures; D013256:Steroids / Q000627:therapeutic use; D013701:Temporal Bone / Q000473:pathology*; D014057:Tomography, X-Ray Computed; D016896:Treatment Outcome; D014747:Vinblastine / Q000627:therapeutic use
D000972:Antineoplastic Agents, Phytogenic; D013256:Steroids; D014747:Vinblastine