Displasias

 y  Tumores  del  hueso  temporal  

 
Dr.  Alejandro  Paredes  Wrbka    
Transcribe:  Sebastian  Loch  
 
Generalidades:  
• Son  tumores  poco  frecuentes  
• Son   localmente   destructivos,   se   ubican   en   una   zona   pequeña:   daño  
proporcional  mayor.  
• Síntomas  inespecíficos.  
• Dificultad   en   el   diagnóstico   por   localización,   difícil   tomar   biopsias.   Ej:   Ápex  
petroso  
• Tratamientos  quirúrgicos  dejan  secuelas  como  gran  inconveniente  
 
Temas  en  esta  clase:  
1. Displasias  Oseas  
2. Tumores  del  oido  medio  
3. Lesiones  del  Apex  petroso  
 
DISPLASIAS  
 
OSTEITIS  DEFORMANTE  (Enf.  de  Paget)  
• Enfermedad   crónica   hereditaria   que   afecta   huesos   largos,   columna,   pelvis,  
columna  y  cráneo.  
• Prevalencia  3  a  11  %.  (aumenta  en  mayores  de  40  años.  
• Fisiopatología  
• Fase  osteolítica  
• Fase  osteoclástica-­‐osteoblástica  (remodelación  desorganizada,  hueso  de  
neoformación  de  mala  calidad)  
• Formas  de  presentación  
• Monostótica  15  %  de  los  casos  
N. L. DEEP ET AL.
• Poliostótica  85%  (65  a  70%  en  cráneo)  
 
69, and Nager in 1975, and TABLE 1. Clinical presentation of Paget’s disease of
 
her valuable contributions temporal bone (n ¼ 27)
 
histopathology of Paget’s No. (%)
 
ients with Paget’s   disease Hearing loss 23 (85%)
Headache 18 (67%)
ch rarely involves  the skull
Dizziness 14 (52%)
 
dentally in asymptomatic
Tinnitus 5 (19%)
the polyostotic  form of
Chronic otitis media 2 (7%)
the skull in 65 to   70% of Hemifacial spasm 1 (4%)
atients with skull  involve- Multiple cranial neuropathies 1 (4%)
in 30 to 50% of cases
  and Tumor development 1 (4%)
in 20 to 25% (7,8).  
ease of the temporal bone
Tabla   de    caracteristicas  clinicas:    
1975, during the precom-
-­‐ Hipoacusia  (SN,  mixta  o  de  Conduccion  de  tonos  graves)  
ere limited by the use of Clinical Presentation
-­‐ Cefalea,  vertigo   y   tinnitus  
The most common symptoms at presentation included
s including conventional
phy. Herein, we  report a hearing loss (n ¼ 23, 85%), headache (n ¼ 18, 67%),
ingle institution of a large dizziness (n ¼ 14, 52%), and tinnitus (n ¼ 5, 19%) (Table
aphic evidence of   PDTB. 1). Dizziness was patient-reported in 11 patients who 1  
ng otologic and neuroto- described a non-vertiginous generalized imbalance,
phic features, management while two patients endorsed true vertigo and had per-
 PAGET’S DISEASE OF THE TEMPORAL BONE 911

 
 
Imágenes:    
En   TAC   se   observa   tejido   de   neoformacion   osea   desorganizada,   reabsorcion   capsula  
otica,   según   la   fase   de   la   enfermedad   es   posible   observar   distintas   manifestasiones  
(esclerosis,  ocupacion  del  tejido  esponjoso  base  craneo).  
Grosor  del  hueso  va  aumentando,  pero  con  estructura  disgregada.  
 
Tratamiento:  Es  médico  y  va  en  función  de  la  sintomatologia  y  problemas  relacionados  
con  la  displasia.  
• Indicaciones  
• Dolor  
• Hipercalcemia  
• Preparación  para  cirugía  
FIG. 2. (Case #2) •Paget’s Déficit  
diseasenin eurológico  
a patient with bilateral hearing loss, right worse than left. A and B, Axial CT scan demonstrates
• Cardiopatía  
exuberant medullary expansion of the skull, temporal bones, and base of the skull. Several scattered sclerotic foci most prominent near
vertex and severe thinning of the outer cortex is noted (white arrow). Note demineralization resulting in expansion of the petrous apex
(asterisks) • andMedicamentos  
thinning of bone around the right otic capsule (black arrowhead), which is more pronounced on the right versus the left. Note
narrowing of the right internal auditory canal due to new bone deposition (black arrow). C, Coronal CT demonstrates diffusely thickened
calvarium (white arrow) • and Bifosfonatos  
upward slanting (right acción   inhibidora  
internal auditory canald e  osteoclastos)  
(black arrow), due to central skull base involvement and upward
tilting of the petrous apex. D, Axial T1 postcontrast fat-suppressed MRI demonstrates extensive expansion of the occiput and right otic
• Calcitonina  
capsule. The hypervascular nature of the disease process is demonstrated by the heterogeneous enhancement of the right temporal bone
• Malignización  
(white asterisks), which is significantly more prominent compared with the left. This area of enhancement corresponds to areas of bone
expansion on CT. E, Audiogram from this patient demonstrates asymmetric sensorineural hearing loss, significantly worse on the right,
• 1  a  10  %  (Sarcoma  osteogénicoà  Clínica:  aumento  progresivo  y  rápido  
consistent with the asymmetric otic capsule involvement seen on imaging. CT indicates computed tomography; MRI, magnetic resonance
imaging.
de  tamaño  del  hueso  +  dolor  +  elevación  de  la  fosfatasa  alcalina)  
• mas  habitual  en  las  formas  poliostoticas  
found a higher rate of hearing loss at 85%, likely reflect- patients to be greater than 2 dB per year (12). Two
ing   our selection of patients with confirmed radiographic patients (four ears) in the current series had long-term
DISPLASIA  
evidence OSTEOFIBROSA  
of temporal bone involvement   (Table 1). In audiometric follow-up and demonstrated a mean rate of
keeping with previous studies, we found the most com- PTA decline of 1.0 dB per year. These losses are greater
mon pattern • Consiste  en  reemplazo  de  médula  ósea  por  tejido  fibroso,  hay  estimulación  de  
of hearing loss to be a down-sloping, high than the 0.5 dB per year loss that is expected for pres-
actividad  ohearing
frequency sensorineural steoclástica.  loss, often   with a low bycusis alone (13). Furthermore, we can verify that the
frequency • Prevalencia  
conductive loss (1).1   a   2
Hearing  
typically bilateral, progressive, and occurs at a rate that is
p or   3 0.000.  
loss in PDTB is rate of progression is accelerated in ears affected by
Paget’s disease by analyzing the results from patients
greater •than 2,5   %  del  
observed intage-matched
otal  de  neoplasias   óseas  y  7%  
healthy controls. with de  unilateral
los  tumores  
temporalbenignos.  
bone involvement and compar-
One   study estimated the rate of hearing decline in Paget’s ing the involved side to the non-involved side, which
Formas  de  presentación   Otology & Neurotology, Vol. 38, No. 6, 2017
• Monostóticas  (70%  de  los  casos)  
• Poliostóticas  (27%  de  los  casos)  
• Síndrome   de   Mc   Cune   Albright   à   Manifestación   poliostoticas   +  
Copyright © 2017 Otology & Neurotology, Inc. Unauthorized reproduction of this article is prohibited.
manchas   café   con   leche   +   alteraciones   endocrinológicas   (pubertad  
precoz/hiperparatiroidismo/hipertiroidismo/Diabetes  
insípida/acromegalia)  

  2  
 normal fibrodysplastic bone growth causing displacement (canaloplasty)
and compression of adjacent structures resulting in loss of tions, such as
function. In the temporal bone, the organs of balance and group of patie
hearing are in intimate relation, and a space-occupying volved after a
lesion within the middle ear may ultimately compromise such patients p
• Malignización  0,5  a  1%  (osteosarcoma,  condrosarcoma,  fibrosarcoma,  tumor  de  
the audiovestibular system. The most appropriate man-
células  gigantes)     teatoma (15%
agement• option for rápido.  
Crecimiento   preserving hearing and managing (16,22,24), an
bothersome • Dolor.   disease symptoms in patients with FDTB vention in pa
may be •either observation
Elevación   or aotologic
de  fosfatasas   lcálinas   surgery. and/or canal
• Fases   clínicas  
The (evolucion)  
reported clinical presentation of patients with further compl
• Latente  
FDTB is variable, and the natural history of the disease Middle ear
• Sintomática  
is often difficult to predict, with involvement of the
• Complicaciones  
may be neede
  the middle ea
  cations such a
TABLE 4. Otologic manifestations of FDTB
  of canal wall
Presenting symptoms n = cases Clinical findings n = cases was 56% in c
Conductive hearing loss 121 EAC stenosis 91
using a canal
Sensorineural hearing loss 9 Cholesteatoma 30 be interpreted
Otorrhea 17 Middle ear mass 20 patients, surg
Dizziness 15 Deformity/swelling 14 surgical appro
Otalgia 14 Cutaneous fistula 2 of the diseas
Tinnitus 12 Mastoid abscess 1
Facial palsy 9 SCC dehiscence 1 garding the po
Ear fullness 3 Visual loss 1 Anecdotally
Preauricular mass 2 Labyrinthine fistula 1 with middle
Facial tingling 1 Preauricular mass 2 stapes and th
Trismus 1
without exter
Clínica   FDTB indicates fibrous dysplasia of the temporal bone. other than a c
-­‐ Estenosis   del   CAE   singno   caracteriztico.   Asociacion   a   colesteatoma   en   hasta  
40%  de  
Otology &pacientes  
Neurotology, con  dVol.
isplasia  
35, oNo.
steofibrosa  
10, 2014del  hueso  temporal.  
-­‐ Hipoacusia  de  Conduccion,  mixta  o  SN,  depende  de  ubicación  de  la  lesion  (si  
genera  fistula,  compresion  nervio  auditivo,  compromiso  oido  medio)  
 
Imagenologia    
-­‐ Patrones:  Fases  pagetoide,  esclerotica  y  quistica.  
Copyright
-­‐ Distinta   densidad   © 2014
del   tejido,   Otology
imagen   & Neurotology,
en   vidrio   esmerilado  Inc. Unauthorized
(zonas   densas,     reproduction
hipodensas  y  esclerosis)  
-­‐ Pueden   producir  
erosiones   y   lesiones   a   nivel   de   la   capsula  
otica.  
 
*   TAC   flecha   blanca:   Canal   semicircular  
erosionado,   dentro   de   un   quiste,   que  
corresponde  a  la  displasia  osteofibrosa.  
tejido   denso   heterogéneo,   que   da   el  
aspecto  de  Vidrio  Esmerilado.  
 
RM  à  imágenes  no  especificas.  
Cintigrafia   Oseaà   poca   evidencia,   pero  
sirve   para   descartar   un   probable  
segundo  foco  displasico  concomitante.    
  3  
Tratamiento:  
1. Observacion:   cuando   manifestaciones   clinicas   son   minimas   o   ausentes.   Sin  
deformidad  estetica  que  altere  calidad  de  vida.  
2. Cirugia:   Clinica   o   estetica   (+).   Se   realiza   despues   de   la   pubertad.   Extirpacion  
completa   de   la   displasia.   Si   queda   tejido   displasico   post   a   la   cirugia,   presenta  
alta  tasa  de  recidiva  10-­‐25%.  
3. Farmacologico:  
a. Bifosfonatos  (inhibe  actividad  de  osteoclastos)  
i. RAM:   cuadro   simil   a   influenza,   gastrointestinal,   osteonecrosis  
mandibular.  
b. Anticuerpos  Monoclonales  (inhibe  activacion  de  ligando  nuclear  Kappa-­‐
B)  
 
 
OSTEOGÉNESIS  IMPERFECTA  
• Enfermedad   genética,   herencia   autosomica   con   penetrancia   variable,   que  
afecta   la   producción   de   colágeno   tipo   1:   genes   de   cadenas   alfa   1   y   2   (genes  
COL1A1  y  COL1A2  de  los  cromosomas  7  y  17)  
• Prevalencia  1/10.000  a  20.000  RN  
• Caracteristicas  Clínicas:    
a. Ostopenia  con  fragilidad  osea  
b. Escleras  Azules  
c. Hipoacusia  
d. Hiperlaxitud  ligamentosa  
e. Denticion  defectuosa  
 
Clasificación:    
se  pueden  describir  hasta  7  tipos,  las  más  frecuentes  son:  
•   Tipo   I:   la   mas   leve,   herencia   autosómica   dominante,   fragilidad   ósea   leve,  
fracturas  tardías,  escleras  azules,  hipoacusia  en  50%.  
•  Tipo  II:  la  forma  más  severa,  asociada  a  mortalidad,  que  se  presenta  desde  muy  
temprana  edad.  Herencia  autosómica  recesiva,  fragilidad  ósea  extrema,  fracturas  
en  período  perinatal,  escleras  azules.  
•   Tipo   III:   forma   severa,   herencia   autosómica   recesiva   o   dominante,   fracturas  
óseas  severas,  deformidad  ósea,  escleras  normales,  poca  asociación  a  hipoacusia.  
•   Tipo  IV:   herencia   autosómica   dominante,   estatura   baja,   huesos   frágiles,   escleras  
normales,  a  veces  hipoacusia.  
• Tipo   V:    dominante,  fragilidad  moderada  a  severa,  calcificación  de  membrana  
interosea,  callo  óseo  hipertrófico,  escleras  normales.  
 
Clínica  
•   Triada   clásica:   Escleras   azules,   Fracturas   espontáneas   (osteopenia),   Hipoacusia   (a  
pesar  de  que  la  penetrancia  de  cada  una  es  muy  variable)  
•  Hipoacusia  en  26-­‐78%  
•  Otopatología:  
-­‐  Fenómenos  de  Otoesclerosis  como  forma  de  reparación  ósea  
-­‐  Fractura  y/o  atrofia  del  Estribo  
-­‐  Degeneración  neural  primaria  (causa  vascular)  
 
  4  
Imagenologia:  
• En  TAC  imagen  caracteristica  es  la  de  doble  anillo,    zona  hipodensa,  asociado  a  
una  zona  de  realce  externo  .  Se  produce  a  nivel  de  Cóclea,  Vestíbulo  y  CSC,  o  
sea  
Figure 3. rodeando  
Axial todo  
CT section. Stapes el  Othickening
footplate ído  interno.  
Figure 11. Axial CT section. Obliteration of round window (arrow).
(arrow).
Figure 12. Axial CT section. Dilated vestibular aqueducts (arrows).
Figure 4. CT, coronal reconstruction. Focus in the round window (arrow).

 
Tratamiento:  
Figure 13. CT,   coronal reconstruction. Distance from the incus to the stapes Figure 14. CT, axial, oblique reconstruction. Imperfect osteogenesis.

1.Figure
Farmacologico  
footplate.
5. Axial CT section. Pericochlear focus (arrows), bilaterally. Figure 6. Axial CT section. Pericochlear focus with endosteal involvement (ar-
It isa. Bifosfonatos   row).
important to emphasize that the pre- tion. Even being useful in such a context, 2. Vicente AO, Penido NO, Yamashita HK, et al.
Tomografia computadorizada no diagnóstico da
b. Anticuerpos   monoclonales  
viously mentioned tomographic findings currently the main role of MRI is the inves-
otosclerose retrofenestral. Rev Bras Otorrinolarin-
are not
Radiol exclusive
Bras. 2013 of otosclerosis, and
Set/Out;46(5):307–312 that tigation of active focus, intracanalicular gol. 2004;70:74–82. 309
2. other
Audiológico:  
diseases may generate     similar alter- extension of the focus and mainly the de- 3. Miranda GG, Orellana PP, Matus CL, et al. Otos-
clerosis: análisis imagenológico con tomografia
ations, such as otosyphilis, osteogenesis tection of postoperative complications.
a. (Figure
imperfecta Audífonos   (6)
14) and Paget’s disease . Thus the knowledge on the temporal bone
computada multicorte. Rev Hosp Clin Univ
Chile. 2006;17:356–9.
b. Estapedostomía   anatomy and on the peculiarities of otoscle-
rosis at MDCT is a skill expected of the
4. Menif E, Bejjar S, Miladi S, et al. Diagnostic de
l’otodpongiose et classification tomodensitomé-

c. Implante  coclear   general radiologist, since such knowledge

CONCLUSION trique pronostique. Société Française de Radio-
logie. Posters électroniques. [acessado em 10 de
Multidetector computed tomography, is useful for an accurate diagnosis and ap-
 
abril de 2012]. Disponível em: http://pe.sfrnet.
with its different planes, has been increas- propriate management of such disease. org/ModuleConsultationPoster/consultation
Poster.aspx?intIdPoster=4238.
OSTEOPETROSIS    
ingly utilized as it allows for the confirma-
tion and appropriate characterization of REFERENCES
5. Poirrier V, Escude B, Granier I, et al. Imagerie de
l’otospongiose. Société Française de Radiologie.
Grupo  dase  well
• otosclerosis, enfermedades  
as the identification of caracterizadas  
1. Mori N, Toyama Y, Kimura p or  
N, et eal. sclerosis  
Detection of
small fenestral otosclerotic lesions by high-reso-
d el  esqueleto.  
Posters électroniques. [acessado em 10 de abril
de 2012]. Disponível em: http://pe.sfrnet.org/
other causes related to deafness, either
mimickingo orAlteracion  
associated with such en  deaf-
la   regulacion  
lution computed de   la   using
tomography actividad  
multiplanar
reconstruction. Auris Nasus Larynx. 2013;40:36–
del   osteoclasto,   no   existe  
ModuleConsultationPoster/posterDetail.aspx?
tIdPoster=1195.
in-

remodelacion  
ness, and preoperative anatomic evalua-osea.  T40.ejido  oseo  esponjoso  no  s6.e   desarrolla  y  se  produce  
Lee TC, Aviv RI, Chen JM, et al. CT grading of

engrosamiento  de  la  cortical  osea,  de  modo  desorganizado  y  por  ende  
Radiol Bras. 2013 Set/Out;46(5):307–312 311
fragil.  
• Herencia  autosomica  con  penetrancia  variable.  
• Enfermedad  muy  poco  frecuente,  prevalencia  1:20.000  a  1:200.000  habitantes.  
 
Fisiopatología:  
– Ausencia  o  reducción  de  la  actividad  de  osteoclastos  
– Problemas   del   ambiente   que   rodea   al   Osteoclasto   no   permitiendo   su  
funcionamiento  
– Déficit   enzimáticos   que   interfieren   con   el   transporte   de   H+   y   Cl-­‐   a   nivel   de  
Osteoclastos  
 
Caracteristicas  Clinicas:  
• Déficit  neurológicos  (estrechez  canales  PC)  
• Alteraciones   hematológicas   (medula   ósea   va   desapareciendo   por   ocupación  
tejido  óseo)  
• Hepatoesplenomegalia  (secundaria  a  anemia)  
• Acidosis   tubular   renal   (falta   actividad   anhidrasa   carbónica,   relacionada   en  
actividad  osteoclastica)  

  5  
  
 
 
 
 
 
 
Clasificación:  
• Severa  
o Herencia  autosómica  recesiva.  
o Curso  agresivo  desde  el  nacimiento.    
o Huesos  densos  frágiles.  
o Falla  médula  ósea,  Hepatoesplenomegalia  compensatoria.  
o Síntomas   neurológicos   e   hidrocefalia   por   compresión   del   cráneo   sobre  
el  encéfalo  y  sistema  de  drenaje  del  LCR  producto  del  crecimiento  óseo.  
o Hiperparatiroidismo  secundario  por  alteración  metabólica  del  Calcio.  
o Problemas  dentales  
 
• Intermedia  
o Herencia  autosómica  dominante  o  recesiva  
o Afecta  a  menores  de  10  años.  
o Presencia  de  calcificaciones  cerebrales,  asociado  a  deterioro  cognitivo.  
o Acidosis  tubular  renal  (falla  en  Anhidrasa  carbónica)  
o Alteraciones  óseas  moderadas,  fracturas  y  baja  estatura  
• Tardía  
o Herencia  autosómica  dominante  
o Es  la  forma  más  benigna,  se  presenta  en  adultos.  
o Puede  ser  asintomática.  
o Tipo   I:   esclerosis   difusa,   sin   alteraciones   del   metabolismo   óseo   ni  
recuento  de  células  sanguíneas  
o Tipo   II:   presentación   heterogénea:   fracturas,   osteomielitis,   dolor   óseo  
alteraciones  neurológicas  y  hematológicas.    
 
Estudio    
• Imágenes     à   TAC   hueso   se   observa   como   una   tabla   gruesa,   sin   trabéculas  
neumatizadas,   solamente   hueso   denso,   que   se   describe   como   hueso  
marmolado.  
• Estudio  de  genetica  molecularà  ya  que  el  tratamiento  se  enfoca  en  relacion  a  
la  alteracion  genetica  que  tiene  el  paciente.  
• Biopsia  de  médula  osea.  
 
 
 
 
 
 
 
 

  6  
*   En  estos  pacientes  se  produce  hipoacusia  por  estenosis  del  CAE,  CAI  o  por  lesion  a  
nivel  de  cadena  de  huesecillos.  
 
Complicaciones  
 
 
 
 
 
 
 
 
Tratamiento  
• Suplemento  de  calcio  y  vitamina  D.  
• Calcitrion.  
• Transfusión  de  glóbulos  rojos.  
• Interferón  γ1b.  
• Trasplante  de  Stem  Cell  hematopoiética.  
• Cirugía  (descompresion)  
• Otros  ORL  (audifono,  un  caso  descrito  de  implante  coclear)  
 
Diagnóstico  Diferencial  
• Fibroma  osificante  y  no  osificante.  
• Osteocondroma.  
• Exostosis.  
• Osteoma.  
• Quiste  óseo  aneurismático.  
• Quiste  óseo  unicameral.  
• Granuloma  central  de  células  gigantes.  
• Sarcoma.  
• Tumor  café  asociado  a  hiperparatiroidismo.  
 
TUMORES  DEL  OIDO  MEDIO  
 
PARAGANGLIOMA  
• Tumor   vascularizado   del   sistema   paragangliónico   simpático   y   parasimpático,  
proviene  del  neuroectodermo  (cresta  neural)  
• Incidencia  1  en  300.000  
• Puede   presentarse   en   forma   espontánea   (80%)   o   heredada   (autosómica  
dominante)  
• Los   más   frecuentes   son   de   abdomen   (feocromocitoma   à   liberan   péptidos  
vasoactivos).  Los  que  se  presentan  en  cabeza  y  cuello,  inhabitual  que  secreten  
péptidos  vasoactivos.  
 
Caracteristicas  
• Crecimiento  lento  (doblan  el  tamaño  en  10  años).  
• Forma   heredada   es   multicéntrico,   presenta   inicio   precoz   de   síntomas   y   baja  
incidencia  de  malignización.  

  7  
 anterior mesotympanum, and protympanum. Degloving the panum. The tumor was then c
tympanic membrane off the malleus handle and umbo also
provides additional exposure of the anterior superior mesotym-
cup forceps in a piecemeal fa
panum and the Eustachian tube, but increases the risk of bleeding was controlled by t
perforating the tympanic membrane (12). middle ear with hemostatic age
The mesotympanum is entered taking care to separate the flap was then replaced and th
tympanic membrane from the surface of the tumor. The tumor is Postoperatively, she had imm
initially dissected from the ossicular chain to reduce the risk of pulsatile tinnitus. An audiogram
• Malignización   se   determina   iatrogenic hearing por   metástasis  
loss from excessive a   ossicular
tejido  manipulation.
no   neuroendocrino  
demonstrated improved hearing
A diode, KTP or CO2 laser, was used to cauterize blood vessels of her ABG (right PTA 46 dB, 4
(ganglios  linfáticos,  hueso,  
supplying pulmón,  
the tumor hígado).  
in cases where the feeding vessels were other surgical complications an
o Vagal  17  %  y  Cuerpo  
identified. carotideo   6,4  %
Cauterization of  (the
malignización  
blood supply m canas   frecuente)  
greatly recurrence at last follow-up.
improve hemostasis before tumor removal. Additional hemo-
Fisiopatología   stasis is obtained by temporarily packing the middle ear with
Case B
Se   pierde   el   control  hemostatic
de   Succinato   deshidrogenasa,  
agents like oxidized cellulose polymer or el  absorbable
cual   suprime  
A la  
63-year-old woman present
gel foam powder mixed with topical thrombin or the use of
sobreexpresión   de   factor   1α   (inducible  
removable cottonoids por   hipoxia)  
soaked in que  
1:1000estimula  
epinephrine.la  
Theproliferación  
oto- de  
sided pulsatile tinnitus. She had
tejido  paraganglionar.     logic drill or curettes can be used to remove significant canal loss (right PTA 36 dB, 11 dB ABG
wall overhangs to provide additional exposure when necessary. Otologic examination revealed
  The laser was used in some cases to vaporize the tumor bed after
Localización  cabeza  y  cuello  (en  
gross o rden   de  frecuencia)  
total removal of the lesion.
Transitioning to a transcanal microscope approach may become
1. Cuerpo  carotídeo,  cerca  de  división  de  carótida  interna  y  externa,  donde  están  
necessary when adequate hemostasis cannot be obtained using a
los  receptores  de  pH,  Oone-handed
2  ywithout
or
 CO2.   mastoidectomy
endoscopic technique. A postauricular approach with
is performed in cases where disease
2. Nervio  vago   extends into the mastoid or when adequate visualization cannot be
obtained with a transcanal endoscopic or microscopic approach.
3. Plexo  timpánico  
4. Pared  del  bulbo  yugular   CASE VIGNETTES
5. Nervio  facial   The following clinical vignettes illustrate the common
  presentation and endoscopic management of middle
ear paragangliomas.
Clínica  (principalmente  yugulotimpanicos)  
• Masa  cervical.   Case A
• Tinnitus  pulsátil.   An 82-year-old woman presented with a history of
pulsatile tinnitus for several months. She had a moderate
• Hipoacusia.     mixed hearing loss (right PTA 51 dB, 15 dB ABG) and
• Vértigo.   normal facial function. Otologic examination demonstrated
• Otalgia.   a red retrotympanic mass filling the posterior mesotympa-
num with extension to the hypotympanum (Fig. 1C). CT of
• Parálisis  pares  craneanos.  
the  temporal bone demonstrated a 5 mm middle ear mass
• Masa  vascular  roja.   originating from the cochlear promontory (Fig. 1A and B). FIG. 1. Corresponds to Case A (
The patient was offered observation or surgical resection
• Otorragía.   and chose to have the lesion removed.
contrasted computed tomography
reveals tumor extension into the hy
  A tympanomeatal flap was raised with a 0-degree the temporal bone demonstrates a
from the promontory. Bone overlying
Estudio   endoscope. The annulus was elevated from a 2 to 10 C, Right-sided preoperative otos
o’clock position to allow access to the entire mesotym- retrotympanic mass. D, Intraoperat
1. Bioquímica:   medir   para   evaluar   funcionalidad   del  
panum and hypotympanum. The vascular middle ear tumor   (pcte   sintomatico,  
paraganglioma after elevation of the
taquicardia,  HTA)  
Otology & Neurotology, Vol. 38, No. 3, 2017
•Catecolaminas  séricas,  metanefrina  urinaria,  ácido  vainillilmandélico.  
 
2. Imágenes  
Copyright ©– 2017TAC   de   &corte  
Otology fino:  Inc.
Neurotology, se  Unauthorized
observa   reproduction
hueso   of this article is pr
apolillado  
– Resonancia  Magnética:  T1  imagen  en  sal  
y  pimienta  (focos  de  vasos  rodeados  de  fibrosis),  
T2  hiperintenso  
– PET   con   Fluorina   o   Desoxiglucosa:   busca  
sitios  de  tumores  simultáneos  
– Angioresonancia   con   técnica   de  
sustracción:  evalua  compromiso  de  carotida.  
– Angiografía   con   prueba   de   balón:  
consiste   en   un   bloqueo   que   se   le   hace   vía  
cateterismo  a  las  carótidas  para  ver  la  tolerancia  
que  tiene  el  paciente  frente  a  la  eventualidad  de  
tener  que  ligar  la  carótida  interna.  
 

  8  
 • TAC:   a   nivel   mesotimpánico   se   observa   erosión   del   hueso   producida   por   un  
paraganglioma  yúgulo-­‐timpánico.  
 
 
 

*Angiografía:  típica  imagen  “en  ovillo”,  que  desaparece  después  de  la  embolización  
*RM:  Imagen  en  “sal  y  pimienta”  (sal:  fibrotico,  pimienta:  vascular).  
 
Pricipales   arterias   que   irrigan   paraganglioma:   Arteria   faringea   ascendete,  
estilomastoidea  y  arteria  auricular  posterior.  
 
Clasificación  de  Fish  
Enfocada   a   la   localización   del   tumor   y   al   compromiso   que   pueda   producir   por   la  
extensión  vertical  y  horizontal:  principalmente  en  relación  a  carótida  y  al  crecimiento  
hacia  endocráneo,  ya  sea  extradural  o  intradural.  
• A:  a  nivel  timpanico  
• B:  Timpanico  con  extension  a  mesotimpano  y  mastoides.  
• C:  Salen  de  area  timpanica  con  extension  a  golfo  yugular  
• D:  Extensión  Intracraneal.  
 

 
IAM:  internal  auditory  meatus;  CPA:  cerebello  pontine  angle  

  9  
 Tratamiento    
• Observación:  Crecimiento  lento,  pcte  edad  avanzada.  
• Cirugía:  localizada  o  no  
o Tumores  A  y  B  cirugía  otológica  
o Tumores  C  y  D  abordaje  combinado  vía  fosa  infratemporal  
o Antes  de  intervenir  el  pcte  debe  ser  embolizado  
• Radioterapia  estereotáxica,  gamma  knife,  radioterapia  externa.  (problemas  en  
el  seguimiento  por  irradiacion)  
• Screening  genético  molecular  del  gen  de  succinato  deshidrogenasa.  
 
HISTIOCITOSIS  DE  CÉLULAS  DE  LANGERHANS  
• Proliferación   monoclonal   de   células   dendríticas   inmaduras   por   mutación   del  
gen  BRAF  V600E.  (células  presentadoras  de  linfocitos  T  a  nivel  de  piel)  
•  Prevalencia  4,6  /  1.000.000  menores  de  15  años.  
• Compromiso  de  hueso  temporal  de  4  a  8  %  de  los  casos  con  HCL  (45%  bilateral)  
• Mediana  de  presentación  32  años  (1  a  88  años)  45%  menores  de  10  años.  
• 60  %  hombres,  45%  tiene  síntomas  sistémicos  
 
some reports of intralesional steroid injections
7
beneficial. RadiationClínica  
is now primarily used as
TABLE I.
Disease Presentation.
 
ant therapy or for recurrent disease. Patients who
No. (%)
nt with limited disease– 1,8 Sintomas  
confined to ithe
nespecificos.  
skeletal sys-
arry the best prognosis.
  Symptom, n 5 20
Given the rarity of the studied condition, the Otorrhea 11 (55)
nt of clinical data is   limited for patients with oto- Postauricular swelling 5 (25)
 
involvement. Our institution previously reported a
Subjective hearing loss 10 (50)
s of 22 cases of LCH  involving the ear and temporal
Otalgia 7 (35)
that were evaluated at the Mayo Clinic between
 
and 1978. Herein we present a subsequent series Soft tissue swelling 3 (15)
 
cases that were evaluated between 1978 and 2014, Exam finding, n 5 20
eview modern disease   presentation, clinical course, Otitis media 4 (20)
gement strategy, and Otitis externa 1 (5)
  outcomes for these patients.
Conductive hearing loss 3 (20)
  Osteolytic lesion of temporal bone 13 (65)
ERIALS AND METHODS
 
Following institutional review board approval (#14- Granulation tissue 9 (45)
  review was performed at a terti-
1), a retrospective chart EAC stenosis 4(20)
 
eurotologic referral center, and all patients with histopa-
EAC 5 external auditory canal.
ically confirmed LCH of the temporal bone diagnosed
 
en 1978 and 2014 were identified. Data collection included
Comparación  
t demographics, clinical presentation, cimaging
línica  dstudies,
e  las  HCL   diagnosis. One patient who suffered a relapse with a
athology, management strategy, recurrence, and complica- large destructive lesion of his cerebellum subsequently
of disease or treatment. Defining cure in patients with developed ataxia and dysphagia (case 5). All patients
s difficult due to the potential for multisite involvement
with a preexisting diagnosis of multisystem LCH had no
ariable clinical course. As defined by the Histiocyte Soci-
CH can be divided into nonactive disease where the delay in diagnosis of their temporal bone LCH, whereas
t has resolution of all signs and symptoms and active dis- there was a median 4-month delay (range 0–14months)
Active disease can be further divided into regressive dis- in patients presenting with unifocal temporal bone dis-
stable disease, and progressive disease.9 Continuous ease. These patients were often initially treated with
es were calculated using the unpaired t test and summar- conventional methods for otitis media before a correct
using medians and ranges, whereas categorical features diagnosis was made following biopsy.
calculated using Fischer exact test and presented using
ncy counts and percentages.
Diagnostic Workup: Imaging and
Histopathology  
ULTS *desde  mas  severo  (Letterer  Siwe)  a  mAll
enos   (granuloma   eosinofilico).  
patients underwent advanced imaging as part of
ent Demographics   and Disease Presentation their diagnostic workup, with imaging findings supporting
A total of 29 cases (20
  patients) met inclusion crite- the diagnosis of LCH. Because the current study spans a
The median age at time of presentation was 32.5 period of nearly 4 decades, a variety of imaging modalities
 
(range 1.25–88 years), 45% (9 of 20) were diag- were utilized depending on the era. CT and plain x-ray
before age 10 years, and 60% (12 of 20) were skeletal survey were the most commonly employed imag-
Forty percent (8 of   20) of patients presented with ing modalities (13 of 20; 65%). Magnetic resonance imag-
10  
mic symptoms, 45% (9 of 20) had bilateral simulta- ing (MRI) was used in 40% of patients. Images depicting
s or sequential temporal bone disease, and 35% (7 the characteristic MRI and CT findings of LCH involving
were found to have intracranial involvement.
 Clasificación  
Nombre  tradicional     Nombre  actual  
Enfermedad  de  Letterer-­‐Siwe     HCL  aguda  diseminada  
Enfermedad  de  Hand-­‐Schüller-­‐Christian     HCL  crónica  multifocal  
Granuloma  eosinofílico     HCL  crónica  focal  
La  de  mejor  pronóstico  es  la  HCL  crónica  foca  
 
Head and Neck Pathol (2016) 10:209–212

 
Axial CT image in the
ue window, a shows a
ue mass centered in the

Imágenes  
poral bone (arrow). The
CT image in the bone
, b shows that the lesion
l-defined margins with
-edges (arrowheads) En  el  TAC  lesion  osteolitica  en  hueso  temporal.  
 
 
 
 
 
 
 
Axial fat-suppressed T2-
d (a) and coronal Diagnóstico  (se  hace  por  histología)  
A)   Diagnóstico   presuntivo:   características   morfológicas   ópticas,   identificables   con   la  
uppressed post-contrast
ghted MR images show a
ointense avidly
ng mass centered in the
ral skull base with
and dermal extension
tinción  por  Hematoxilina-­‐eosina  
B)  Diagnóstico  propuesto:  características  morfológicas  ópticas  más  2  o  más  resultados  
(+)   complementarios   de   tinciones:   de   adenosina   trifosfatasa,   proteína   s100,   alfa   D  
manosidasa  y  lectina  de  maní  
C)   Diagnóstico   definitivo:   Características   morfológicas   ópticas   más   Gránulos   de   Birbeck  
en  la  célula  de  la  lesión  (por  microscopía  electrónica)  o  resultados  (+)  de  la  tinción  para  
antígeno  CD1  en  la  célula  de  la  lesión  
 
Tratamiento  
• Medico:  Corticoide  +  Vinblastina  
• Cirugía  
§ Curetaje  y  aseo  en  lesiones  simples  
3 § Excisión,  hasta  extirpación  del  hueso  si  es  necesario  
• Radioterapia  en  casos  difusos,  no  muy  buenos  resultados.  
 
Pronostico  Favorable  cuando:  
• Ausencia  de  compromiso  multisistemico.  
• Respuesta  a  tratamiento  entre  6  y  12  semanas.  
• Edad  mayor  a  2  años.  
• Presencia  de  nódulos.  
 
 
 
 
 
 
 
 
  11  
 Otology
LESIONES   DEL  
2' s 6OLUME  .UMBERAPEX  PETROSO   !BDEL 2AZEK AND (UANG 
 
Classification of Petrous Apex Lesions on the Basis of Origin and Imaging Characteristics Cystic Lesions o
Lesions Imaging Characteristics
Developmental lesions
Cholesterol granuloma Increased signal intensity on both T1WI and T2WI
Cholesteatoma Restricted diffusion at DWI Anatomy of petrous apex (PA)
Mucocele
Cephalocele
...
PA: truncated pyramid, medial part of
CSF signal intensity with all sequences
Inflammatory lesions Four surfaces: superior, posterior, infe
Petrous apicitis ...
Osteomyelitis (bacterial, fungal, or tuberculous) ... Superior: part of middle cranial fo
Inflammatory pseudotumor ...
Wegener granulomatosis ... lacerum anterior; superior petro
Benign tumors
Meningioma ... eminence (SSCC), ascending porti
Schwannoma ...
Paraganglioma
ganglion fossa (Meckle’s cave)
Salt-and-pepper appearance at MR imaging, perme-
ative bone changes
Chondroma Chondroid matrix calcification Posterior: anterior lateral wall of p
Chondroblastoma Chondroid matrix calcification
Myxoma ...
petro-occipital suture inferior, i
Osteoblastoma ...
VI, vestibular aqueduct (external a
Giant cell tumor ...
Malignant tumors Inferior: part of skull base, bounde
Chondrosarcoma Chondroid matrix calcification
Chordoma Honeycomb enhancement foramen for ICA entry, cochlear aq
Endolymphatic sac tumor Hemorrhage and bone destruction
Metastasis ... IAC divided PA into anterior petrous a
Plasmacytoma ...
Lymphoma ... Pneumatic (mucosa-lined air cells), sc
Nasopharyngeal carcinoma ...
Rhabdomyosarcoma ...
Pneumatization of temporal bone: 5 re
LCH ... petrous apex, acessroy)
Vascular lesions
Petrous carotid aneurysm ... Perilabyrinthine: supralabyrinthine,
Intraosseous dural arteriovenous fistula Multiple intraosseous flow voids
Osseous dysplasias communication between mastoi
Fibrous dysplasia Ground-glass matrix calcification
Paget disease ... Petrous apex: peritubal, apical
Pseudolesions
Asymmetric marrow Fat signal intensity with all sequences
Effusion
Pseudofracture
...
Classification (lesions type): cystic, sol
Runs through the superior semicircular canal
Infantile pseudolesion ... Vascular cystic lesions: internal carotid an
Note.—CSF = cerebrospinal fluid, DWI = diffusion-weighted imaging, LCH = Langerhans cell histiocy-
tosis, T1WI = T1-weighted images, T2WI = T2-weighted images. Nonvascular cystic   lesions: petrous apiciti
  cyst), retension mucus, mucocele, choleste
Clínica:  
prefer to categorize petrous apex abnormalities vascular lesions, or osseous dysplasias (Table). In
pneumatization
on the basis of their specific cause or origin into the remainder of this article, we discuss some of
• Cefalea.  
developmental lesions, inflammatory or infectious these lesions in detail.
lesions, Hipoacusia.  
• neoplastic (benign or malignant) lesions,

• Tinitus  
• Vértigo.  
• Dolor   trigeminal.   (ganglio  
gasser)  
• Diplopía.  
• Espasmo  facial.  
• Disfonía.  
• Disfagia.  
 
Prevalencia  

  12  
 • Lesiones  mas  frecuentes  àQuisticas  
o Granuloma  colesterol  (mas  frecuente)  
 
 
GRANULOMA  DE  COLESTEROL  
• Reacción  inflamatoria  contra  productos  de  degradación  de  la  hemoglobina.  
• Se  ubican  en  mesotimpano,  mastoides  y  apex  petroso.  
• Patogenésis  (2  teorias)  
• Obstrucción-­‐vacío.   (extravasación   de   sangre   que   se   deposita   en   mucosa   y  
genera  reacción  inflamatoria  granulomatosa)  
• Exposición   de   médula   ósea.   (celdas   de   hueso   temporal   dejen   áreas  
expuestas   de   medula   ósea,   en   que   se   produce   microsangrado   con   igual  
reacción  inflamatoria)  
• Prevalencia  0.6:  1.000.000  habitantes  
• Crecen  en  forma  silente.    
• Síntomas  inespecíficos:  hipoacusia,  vértigo  y  cefalea  
• Aspecto  de  quiste  amarillentos,  con  contenido  seroso  amarillo-­‐café.  
Imagen:  
• TAC   tumor   bien   delimitado,   sin   refuerzo,   isodenso   con   el   cerebro,  
remodelación  ósea,  sin  erosión  ósea.  
• RM  hipertenso  en  T1  y  T2,  no  se  refuerza  con  medio  de  contraste.  
Tratamiento:  
• Observación  
• Cirugía  
• Endoscópica  base  de  cráneo  
• Abordaje  por  hueso  temporal:  infralaberintico,  transcloclear,  etc  
 

*TAC:  remodelación  osea  

*RM:  T1  hiperintenso  
 
COLESTEATOMA  CONGÉNITO  
• Presencia   de   epitelio   escamoso   estratificado   a   medial   de   la   membrana  
timpánica.  
• Se   ubican   en   mesotímpano,   ganglio   geniculado,   apex   petroso   y   ángulo  
pontocerebeloso.  
• Patogenésis  
  13  
 • Restos  epidérmicos  ectópicos.  
• Inclusión  de  piel  meatal.  
• Metaplasia.  
• Reflujo  de  líquido  amniótico.  
 
• Aspecto  de  nódulos  redondos  blancos,  lisos.  
• TAC   corte   fino   ocupación   por   tejido   blando   que   no   se   realza,   erosión   ósea  
variable.  
• Resonancia   T1   señal   intermedia   o   baja,   T2   señal   hiperintensa,   no   se   refuerza  
con   medio   de   contraste.   Para   diferenciar   de   quiste   aracnoidal   en   secuencia  
FLAIR  y  difusión  se  observa  hiperintenso.  
• Tratamiento  
• Cirugía  
 
 
 
 
 
 
 
 
 
 
 
 
 
MUCOCELE  
• Causado   por   obstrucción   postinflamación   de   una   celda   neumatizada,   crece  
produciendo  remodelación  y  erosión  ósea.  
• TAC   corte   fino   ocupación   por   tejido   blando   con   remodelación   y   erosión   ósea  
variable,  isointenso  con  líquido  cefalorraquideo..  
• Resonancia   T1   señal   isointensa,   T2   señal   hiperintensa,   no   se   refuerza   con  
medio  de  contraste.    
• Tratamiento  
• Cirugía  
 
MENINGIOMA  
• Originado  en  las  células    de  la  membrana  aracnoidea.    
• Localización   dentro   del   hueso   temporal   son   extracraneales,   los   intracraneales  
se  originan  en  sutura  petroclival  y  ángulo  pontocerebeloso.  
• Poco  frecuentes  (1  a  2  %)  
• Más  frecuente  en  mujeres  2:1.  
• Crecimiento  lento  0,8  mm/año.  
Imagen  
• TAC:  masa  de  la  duramadre  levemente  más  intensa  que  el  cerebro.  
• RM  T1  iso  a  hipointensa,  T2  iso  a  hiperintensa.  
Tratamiento    
• Cirugía  
• Radioterapia  
  14  
 – Casos  complejos.  
– Resección  parcial.  
• Recurrencia  
4 Hunter et al
• 6  %  a  5  años  y  20  %  a  15  años  
• Sobrevida  a  5  años  85  %.  
 
 
 
 
 
 
 
 
 
 
 
 
*RM  T1(A)  y  T2  (b)  
  Fig. 2. A 35-year-old woman with right-sided petroclival meningioma. Note the difference
CONDROSARCOMA  
in intensity between images. (A) T1-weighted postcontrast axial image. (B) T2-weighted
Originado  
• axial image. en  degeneración  de  remanente  embrionario  de  cártilago  encondral  a  

nivel  de  sincondrosis.  

• Presentación  entre  2da  y  3era  decada.  
of an arachnoid plane on T1-weighted or T2-weighted imaging indicating pial invasion,
findings which would suggest a more difficult resection (Fig. 4).1,9,17
• 0,5   a  6  %  dtoe  preoperative
In addition lesiones  del  
CTapex   petroso.  
and MRI, many groups still use preoperative 4-vessel
Tiene   angiography
• cerebral 5   subtipos  tohistológicos   definidos,  vasculature.
assess the intracranial lo   mas   importante  
In one of the es  largest
el   grado   de  
diferenciación.  Determina  pronostico.  
studies to date, Natarajan and colleagues 9
obtained preoperative cerebral angiog-
• raphy on all patients, embolizing 61%. However, most groups will only obtain angiog-
Requiere   diferenciar   por   inmunohistoquímica   del   cordoma   condroide  
raphy if the cavernous sinus and the carotid or basiliar arteries are involved, and thus,
(citoqueratina  
assess if preoperative negativo).  
embolization may facilitate surgical resection (Fig. 5).3,18
Imagen  
TAC:  mHISTORY
• NATURAL asa  del  apex  petroso  con  arcos  y  anillos  de  calcificación.  Destructiva.  
• TheRM   T1   benefits,
risks, señal   de  and baja   a   intermedia,  
alternatives T2   petroclival
of treating señal   hiperintensa  
meningiomas al  must
comparar  
be con  
cerebro.  
weighed against their natural history. Van Havenbergh and colleagues 13
retrospec-
tively reviewed 21 patients with petroclival meningiomas who underwent no treatment
Tratamiento    
for a minimum of 4 years, and an average of almost 7 years. They found that 76% of
Cirugía.  
• tumors demonstrated growth on imaging, with 50% of the original asymptomatic pa-
Radioterapia.  
• tients exhibiting at least one CN deficit, and 20% of those patients with at least one CN
Sobrevida   según   grado  dCN
e  dinvolvement.
iferenciación  Furthermore,
histológica  dby e  9using
0  a  4either
3%.   CT or
13
• deficit developing additional
MRI to determine the tumor-equivalent diameter and tumor volume, they found that
 
the overall tumor diameter and tumor volume growth rates were 0.81 mm/y and
0.81 cm3/y, respectively.13 These growth rates pale in comparison with a previous
study reviewing the postoperative courses of 38 patients who underwent subtotal
resection of petroclival meningiomas without further treatment, reporting tumor diam-
eter and volume growth rates of 3.7 mm/y and 4.94 cm3/y, respectively.19 Despite the
different growth rates, without intervention, petroclival meningiomas are expected to
grow.

TREATMENT

With the propensity for patients with petroclival meningiomas to present with signifi-
cant complaints and the tendency for the tumors to grow, there are many studies

  15  
ER  !BDEL 2AZEK AND (UANG   .UMBER 
2' s 6OLUME

508 ISAACSON et al
LESIONS OF THE PETROUS APEX 507

normal patients. They are midline lesions that occur anywhere from the
clivus to the sacrum, with approximately one third involving the clivus
[107]. Chordomas are rare, with an incidence of 0.08 cases per 100,000
[108]. Chordomas are more common in male patients and rarely present
in patients older than age 40. Female patients and younger patients are
more likely to present with a chordoma located in the skull base [108].
Because chordomas are slow growing and have an insidious course, most
tumors are large at the time diagnosis. In a large series by Tzortzidis and
colleagues [109], 81% of tumors were larger than 2 cm at presentation,  
with 37.8% larger than 4 cm. The most common presenting symptoms are
*destruccion   apex  petroso   y  anillos  y  aSkull
rcos  de  calcificacion.   RM  t2  hiperintensa.  
Figure 12. Chondrosarcoma. (a) Axial T2-weighted MR image s
a. (a)diplopia, headache,
Axial T2-weighted and lower cranial
MR image showsnerve a mass deficits
with[110,111].
heterogeneousbase but
 
chordomas are typically more aggressive in the pediatric
ensity centered in the region of the petroclival fissure. (b) Axial CT image
population, with predominantly high signal intensity centered in the region of the pe
a wider range of   presentation, atypical morphology, and greater incidence shows arc- and ringlike matrix calcification in the lesion, a finding su
calcification in the
of metastasis lesion, a finding suggestive of a chondroid tumor.
[112].
Radiographic CORDOMA  
evaluation is essential not only for diagnosis but also for
treatment planning. CT Tumor  originado  
• demonstrates a locallyedestructive
n  remanente   lesionecentered
mbrionario  de  la  notocorda.  
Más  frecuente  en  hombres,  rara  vez  se  presenta  sobre  los  40  años  (En  niños:  
at the clivus (Fig. 12).• Bony trabeculae may be seen and true calcifications
may occur in the chondroid chordoma variant. There is moderate to marked
enhancement with contrast, más  
andalow
gresivo,   comportamiento  
signalFig.
areas may be present and errático,  
repre- puede  producir  MTT).  
13. Chordoma. Axial T2-weighted MRI demonstrates a hyperintense lesion centered in the
sent areas of gelatinous TAC   MRI
• material. lesión  
allows localmente  
determination
right petrous apex and clivus. of destructiva  
the extent of con   calcificaciones.   (similar   a  
disease and reveals the presence of intracranial or cavernous sinus involve-
condrosarcoma)  
ment. With T1-weighted MRI, chordomas are hypointense
gadolinium, chordomas andhave contrast
variable enhancement and may have a charac-
• RM  fat
well against the hyperintense T1  inseñal   hipointensa,  
the teristic
clival bone marrow.
‘‘honeycomb’’
T2   Chordomas
señal   hiperintensa,   refuerzo   variable   (patrón   en  
pattern (Fig. 14) [113].
panal  de  aMRI
appear hyperintense on T2-weighted beja)  
and contrast well against adja-
Grossly, chordomas are grayish, semitransparent, and multiloculated. His-
cent neural structures (Fig. 13). With T1-weighted MRI enhanced with
• Sobrevida  59%   a  5reveals
tology  años   y  4characteristic
the 4%   a  10  años.  
physaliphorous cell, which has a vacuolated
508 et al
 
ISAACSON

&IGURE  Chordoma. (a) Axial CT image shows a destructive m

Axial CT image shows a destructive mass involving the clivus, nasopharynx,left masticator space, and left petrous apex. Small foci of calcificatio
petrous apex. Small foci of calcification in the lesion may represent residualbone. (b) Gadolinium-enhanced fat-suppressed T1-weighted MR
ced fat-suppressed T1-weighted MR image shows avid enhancement of themass. Some areas have a honeycomb pattern of enhancement (arrow
ycomb pattern of enhancement (arrow).
onstrate variable degrees of enhancement after A
enhancement after At CT, skull base chordomas appear as lo- contrast material administration (3,43). cally
theinc
ationFig. 12. Chordoma. Axial temporal boneyCT
(3,43). *TAC,   R M   T 1  
cally  Tdestructive
2.  
scan demonstrates soft-tissue masses
a lytic lesion centered centered Fig.
in the in 13. Chordoma. Axial T2-weighted MRI demonstrates a hyperintense lesion centered
  into the right
clivus. This lesion extends thepetrous
clivus.
Fig. 14. Chordoma. Axial T1-weighted MRI
apex Calcifications
posterior to the verticalare often
petrous Chordoma
evident
carotid
with fat saturation
and
and
right petrous gadolinium
apex and clivus. demonstrates repre
an enhancing lesion centered in the right petrous apex and clivus.
artery.
represent residual bone trabeculae; true tumor Chordomas gadolinium, chordomas have variable enhancement and may have acalci
are rare tumors that originate from
TUMOR  DE   SACO  ENDOLINFÁTICO   chara

rs that originate from • Se  ocalcifications may occur in the chondroid embryologic

vari-Grossly, chordomas are grayish, semitransparent, and multiloculated. Ho
remnants
teristic ‘‘honeycomb’’ of the
pattern notochord
(Fig. 14) [113]. and ant
rigina  del  epitelio   del  saco   y  conducto   canendolinfático.  
occur anywhere from the skull base tocell, thewhich has a vacuolat
occa
he notochord and ant of chordoma.
• Crecimiento   Low-attenuation
lento,  localmente   agresivo,   areas on  are
csacrum.
tology
bajo  Skull
reveals the characteristic
grado   de  chordomas
physaliphorous
malignidad.  are typically mid-
base tumo
he skull base to the occasionally seen and represent portions of the
• Aparece   en   promedio   a   los   50   años,  line puede  
lesionsser  arising
espontaneo  
in the clivuso   asociado  
but may a   extend imag
mas are typically mid- tumor containing gelatinous material. At MR
livus but may extend
Síndrome   de  Von  Hippel-­‐Lindau.  
imaging, chordomas are typically hypointense
laterally to involve the petrous apex. Chordomas on T
Imágenes   are most frequently seen in males younger than weig
ous apex. Chordomas on T1-weighted images and hyperintense on T2-
40 years but can occur at any age. In the pediat- istra
n males younger than • TAC   tumor  de  
weighted partes  After
images. blandas   con  calcificaciones,  
contrast material admin- produce    erosión  ósea.  
ric population, skull base chordomas are typically men
any age. In the pediat- • RM  istration, T1  y  T2  cthey
on  señal   heterogénea,  
demonstrate variable captación  
enhance- irregular  de  gadolinio.  
more aggressive and show a wider range of clini- enha
hordomas are typically• Angiografía.   ment and(may lesiones  
havemauy   vascularizadas)  
characteristic honeycomb
cal features, atypical morphologies, and a greater
a wider range of clini-Tratamiento   enhancement pattern (44) (Fig 13). prevalence of metastases (1,3).
hologies, and a greater
  16  
1,3).

Fig. 14. Chordoma. Axial T1-weighted MRI with fat saturation and gadolinium demonstra
 • Cirugía  
• Radioterapia   radiographics.rsna.org164 January-February 2012
 

 
 
mor. (a) Axial unenhanced   T1-weighted MR image shows a heterogeneous mass Figure 14. Endolymphatic sac tumor. (a) Axial unenhanced T1-weighted MR i
he posterior wall of the petrous apex. Note the high-signal-intensity areas in the in the temporal bone that destroys the posterior wall of the petrous apex. Note th
  breakdown products from intratumoral hemorrhage. posterior portions of the mass, which likely represent hemoglobin breakdown produ
h likely represent hemoglobin
ained in another patient METÁSTASIS  
shows a lesion that destroys the posterior wall of the left (b) Axial unenhanced CT image obtained in another patient shows a lesion that de
• 22%  tumores  de  hueso  temporal   petrous bone (arrow).
• La  mayoria  sobre  los  50  años  
Metastasis Endolymphatic Sac Tumor
• Ubicación  mas  frecuente  à  apex  petroso   Metastasis
ocally aggressive Metastases to the petrous apex are most com- Endolymphatic sac tumors are locally aggressive Metastases to the
l rugose por-   monly seen in patients aged 50–70 years. The tumors arising from the proximal rugose por- monly seen in pat
hich is situated petrous apex is the most common site for metas- tion of the endolymphatic sac, which is situated petrous apex is th
gular foramen. tases in the temporal bone (83% of cases) and halfway between the IAC and jugular foramen. tases in the tempo
racteristically is the sole site of temporal bone involvement in Therefore, these tumors are characteristically is the sole site of t
of the temporal 31% of cases. The most common tumor to me- located along the posterior wall of the temporal 31% of cases. The
astoid or the tastasize to the petrous apex is breast cancer, fol- bone and may extend into the mastoid or the tastasize to the pe
pex. They are lowed by lung, prostate, and renal cell carcinomasanterior portion of the petrous apex. They are lowed by lung, pro
dolymphatic sac (4). Metastatic tumor involvement of the petrous usually sporadic, but bilateral endolymphatic sac (4). Metastatic tu
Hippel–Lindau apex may be due to hematogenous spread from tumors are associated with von Hippel–Lindau apex may be due
distant tumors, direct extension of an extra- or syndrome (45). distant tumors, di
mors appear as intracranial tumor, or leptomeningeal extension At CT, endolymphatic sac tumors appear as intracranial tumor
t intratumoral of a distant or intracranial tumor. Susceptibility soft-tissue masses with prominent intratumoral of a distant or intr
e bone erosion of the petrous apex to hematogenous metastases calcification that cause permeative bone erosion of the petrous ape
petrous bone. is thought to be due to slow blood flow through along the posterior surface of the petrous bone. is thought to be d
ogeneous signal the petrous apex marrow, which allows filtering At MR imaging, they have heterogeneous signal the petrous apex m
ghted images. and deposition of tumor cells (1,40). intensity on both T1- and T2-weighted images. and deposition of
strate areas of The imaging characteristics of petrous apex Up to 88% of the lesions demonstrate areas of The imaging c
ted images due metastases are nonspecific. Frequently, they high signal intensity on T1-weighted images due metastases are no
hemosiderin, and demonstrate significant bone destruction and to deposition of methemoglobin, hemosiderin, and demonstrate sign
d intratumoral marked enhancement. CT usually shows an cholesterol crystals from repeated intratumoral marked enhancem
surgical resection aggressive lytic lesion destroying the skull base. hemorrhage (Fig 14). Complete surgical resection aggressive lytic le
of choice (45–47). MR imaging depicts the soft-tissue extent of of these tumors is the treatment of choice (45–47). MR imaging depi
metastases, which usually have low to intermedi- metastases, which
ate signal intensity on T1-weighted images and  
ate signal intensit
  variable signal intensity (usually intermediate to variable signal int
Los   cánceres   que   metastizan   son   de   mamas,   tumores   de   cabeza   y   cuello,   y   menos  
frecuente  tumores  de  pulmón  y  próstata  
 
 

  17