Ceruminoma
with
—
Ryoichi
Departments
Intracranial
Case
Invasion
Report—
SAITO,
Kohei
KODAKI,
Shigeo
TOYA
and
Ryuzo
SHIOBARA,
Jin KANZAKI*
of Neurosurgery and *Otorhinolaryngology, School of Medicine, Tokyo
Keio University
Abstract Ceruminous gland tumors (ceruminomas), which usually involve the external auditory canal, are rare. A case of ceruminoma invading the temporal bone and histologically proven to be papillary adenoma is presented. The tumor recurred and invaded intracranially after subtotal removal and was finally diagnosed as adenocarcinoma. The importance of early diagnosis and radical treatment is stressed. Key words:
adenoma,
adenocarcinoma,
ceruminoma,
Introduction
in February,
Ceruminous gland tumors of the external auditory canal (ceruminomas) are rare and are clinically treated by otorhinologists as a mass in the external auditory canal. '"2'4) Their intracranial extension is extremely rare .3,s-7.9-")Total removal of an intracra nial ceruminoma is difficult because of its high vascularity and invasiveness.',') We report a case of ceruminoma invading the posterior fossa, of which radical excision was impossible even though histo logical diagnosis was papillary adenoma. Case
Received
March present
28, 1990; address:
gland,
intracranial
invasion
1982.
On admission, examination of the left ear revealed a mass completely occluding the lumen of the exter nal auditory canal. Neurological examination reveal ed hypesthesia of the face, double vision on lateral gaze, impaired taste, and deafness, all on the left side. A coronal tomogram of the temporal bone showed extensive destruction of the left petrous portion (Fig. 1). Computed
tomographic
(CT) scans
Report
A 43-year-old housewife first noted vertigo and tin nitus in the left ear in September, 1971. She was diagnosed as having Meniere's disease and had been medicated for several years by an otologist. Eventual ly, she developed left hearing disturbance, bloody discharge from the left ear, double vision on left lateral gaze, and impaired taste. In March, 1981, a mass in her left external auditory canal was found and histologically diagnosed as adenoma by biopsy. In June, the tumor was totally removed by transmeatal approach. However, as the tumor recur red, she was referred to our department for surgery
Author's
ceruminous
Accepted K. Kodaki, Japan.
M.D.,
June
Fig. 1
Coronal tomogram of the temporal bone, demonstrating destruction of the left petrous portion (arrows).
5, 1990
Department
of Neurosurgery,
The
Second
Tokyo
National
Hospital,
Tokyo,
showed a markedly enhanced mass in the left cere bellopontine (CP) angle extending into the middle ear space with wide destruction of the left petrous bone (Fig. 2). Left carotid angiograms demon strated a highly vascular CP angle mass supplied by the occipital branch of the external carotid ar tery (Fig. 3). Left vertebral angiograms showed superior displacement of the peripheral branch of the anterior inferior cerebellar artery and faint tumor stain. These findings suggested a glomus jugulare tumor. A left subtemporal craniotomy was performed on May 4. The soft, highly vascular tumor was entirely located extradurally in the posterior fossa and destructively invaded the left middle ear space and the petrous bone. Only partial removal was possible because of massive bleeding. Histological examina tion revealed tumor cells arranged in a papillary pat
Fig. 3
Left carotid angiogram, demonstrating the vascular mass (arrow) fed by the occipital branch of the external carotid artery.
tern within prominent fibrous stroma, without atypical features and mitoses suggestive of malignan cy (Fig. 4). The histological diagnosis was thus papillary adenoma. Clinically, this tumor was con sidered to originate from the ceruminous gland. Postoperatively, she developed left facial paralysis and was discharged in June. Bloody discharge from the left ear developed, and partial removal of the tumor was again performed by transmastoidal approach in October, 1982. There after, because of the tumor extension into the mas toid cavity, partial removal of the tumor was per formed by the same approach in January, 1985 and again in March, 1986. All the tumors removed were histologically diagnosed as adenoma. In April, 1988, she was readmitted for resection of recurrent retroauricular tumor with bloody discharge. CT scans revealed a large tumor extending into the posterior and temporal fossae (Fig. 5). The retroauricular mass was resected in May, and its histological findings were largely similar to the
Fig. 2
Pre (upper) and postcontrast (lower) CT scans, showing a mass in the left CP angle with destruction of the left petrous bone.
previous one excepting for mitotic figures (Fig. 6). Considering the wide destruction of the temporal bone, the clinical course, and the histological find ings of the tumor, a final diagnosis of ceruminous adenocarcinoma was made. Mental disturbance and pneumonia then developed, and she died in Decem ber, 1988.
Fig.
4
Photomicrographs tumor
cells
There cy.
are
of
arranged no findings
HE stain,
upper:
the in
tumor,
a papillary
suggestive
showing pattern.
of malignan
x 100, lower:
x 200.
Fig. 6
Photomicrographs showing
similar
of
4 excepting
for a mitotic
per:
lower:
x200,
the
histological
recurrent findings
figure.
tumor, as in Fig.
HE stain,
up
x400.
by Berlin in 19493) followed by nine such cases in the literature,'-',9-l') as far as we are aware. Those 10 cases showed no sex difference in incidence with the ages of patients ranging from 7 to 77 years. In most patients, the tumor invaded volved the facial nerve. The was long, often over several
Fig.
5
Postcontrast invading
CT
scans,
the temporal
revealing and posterior
a large
mass
fossae.
Discussion Ceruminous glands are modified apocrine glands, which lie deep in the dermis of the cartilaginous por tion of the external auditory canal. 1,2,41Tumors of ceruminous first report
glands rarely of intracranial
extend intracranially. ceruminous adenoma
The was
the CP angle and in duration of symptoms years. Cerebral angio
grams demonstrated highly vascular masses mainly supplied by the external carotid artery, especially by its occipital and meningeal branches.',',"' in most cases, the preoperative clinical diagnosis was chemodectoma.5'6"") In our case, CT scans revealed a markedly enhanced isodense mass; these findings are the same as those reported by Cilluffo et al.') In 1972, Wetli et al. 12) simply classified the ceruminous gland tumors into four types: adenoma, adenocarcinoma, adenoid cystic carcinoma, and pleomorphic adenoma. Histologically, tumors ex tending intracranially have been either adeno mas 3.6,9,'0>or adenocarcinomas.s''"") Although adeno carcinoma histologically resembles adenoma, the crucial feature of adenocarcinoma is invasion.") In our case, the initial histological diagnosis was ade
noma, but the tumor recurred invasively and was finally diagnosed as adenocarcinoma. The fact that adenocarcinoma often recurs after surgery shows the difficulty of radical treatment.'," Hicks" recom mended wide local excision only for benign tumors and aggressive wide en bloc resection followed by irradiation for malignant tumors. However, total removal of a tumor with intracranial extension is difficult because of its invasiveness and hypervas cularity. For such tumors, Cilluffo et a!.,5) consider ing their slow natural growth, recommended sub total excision followed by radiation therapy. In adequate initial treatment or delayed diagnosis for ceruminoma, as in our case, results in intracranial invasion, which makes total removal of the tumor difficult.,') Histological differentiation of adenoma from adenocarcinoma is impossible,'") and for this reason, early radical excision is important even if the biopsy shows a histologically benign form. References 1) 2)
3) 4)
Althaus SR, Ross JAT: Cerumen gland neoplasia. Arch Otolaryngol 92: 40-42, 1970 Batsakis JG, Hardy GC, Hishiyama RH: Cerumi nous gland tumors. Arch Otolaryng (Chicago) 86: 66-69, 1967 Berlin L: Intracranial ceruminous adenoma. J Neurosurg 6: 415-418, 1949 Canker V, Crowley H: Tumors of ceruminous
glands: Cancer
A clinicopathological 17: 67-75, 1964
study
of seven
cases.
5)
Cilluffo JM, Harrier SG, Miller ceruminous gland adenocarcinoma. 952-965, 1981
6)
Dilenge D, Nutik S, Poliquin J, Masse S: Ceruminous tumor of the jugular foramen. J Can Assoc Radiol 28: 287-290, 1977
7)
Hageman MEJ, Becker AE: Intracranial invasion of a ceruminous gland tumor. Arch Otolaryngol 100: 395-397, 1974 Hicks GW: Tumors arising from the glandular struc tures of the external auditory canal. Laryngoscope 93: 326-340, 1983 Kleinsasser O, Scharfetter G: Zeruminaldrusenade noma mit Einbruch in Dura and Kleinhirn. Zbl Neurochir 17: 4-12, 1957 Norman D, Newton TH: Ceruminous tumors of the cerebellopontine angle. Neuroradiology 10: 1-4, 1975 Rossato RG, Timperly WR: Posterior fossa ceruminoma. Acta Neurochir (Wien) 28: 315-322, 1973 Wetli CV, Pardo V, Millard M, Frcpi MB, Path FRC, Gerston K: Tumors of ceruminous glands. Cancer 29: 1169-1178, 1972
8)
9)
10) 11)
12)
RH: Intracranial J Neurosurg 55:
Address reprint requests to: S. Toya, M.D., Department of Neurosurgery, Keio University School of Medicine, 35 Shinano-machi, Shinjuku-ku, Tokyo 160, Japan.
with
—
Ryoichi
Departments
Intracranial
Case
Invasion
Report—
SAITO,
Kohei
KODAKI,
Shigeo
TOYA
and
Ryuzo
SHIOBARA,
Jin KANZAKI*
of Neurosurgery and *Otorhinolaryngology, School of Medicine, Tokyo
Keio University
Abstract Ceruminous gland tumors (ceruminomas), which usually involve the external auditory canal, are rare. A case of ceruminoma invading the temporal bone and histologically proven to be papillary adenoma is presented. The tumor recurred and invaded intracranially after subtotal removal and was finally diagnosed as adenocarcinoma. The importance of early diagnosis and radical treatment is stressed. Key words:
adenoma,
adenocarcinoma,
ceruminoma,
Introduction
in February,
Ceruminous gland tumors of the external auditory canal (ceruminomas) are rare and are clinically treated by otorhinologists as a mass in the external auditory canal. '"2'4) Their intracranial extension is extremely rare .3,s-7.9-")Total removal of an intracra nial ceruminoma is difficult because of its high vascularity and invasiveness.',') We report a case of ceruminoma invading the posterior fossa, of which radical excision was impossible even though histo logical diagnosis was papillary adenoma. Case
Received
March present
28, 1990; address:
gland,
intracranial
invasion
1982.
On admission, examination of the left ear revealed a mass completely occluding the lumen of the exter nal auditory canal. Neurological examination reveal ed hypesthesia of the face, double vision on lateral gaze, impaired taste, and deafness, all on the left side. A coronal tomogram of the temporal bone showed extensive destruction of the left petrous portion (Fig. 1). Computed
tomographic
(CT) scans
Report
A 43-year-old housewife first noted vertigo and tin nitus in the left ear in September, 1971. She was diagnosed as having Meniere's disease and had been medicated for several years by an otologist. Eventual ly, she developed left hearing disturbance, bloody discharge from the left ear, double vision on left lateral gaze, and impaired taste. In March, 1981, a mass in her left external auditory canal was found and histologically diagnosed as adenoma by biopsy. In June, the tumor was totally removed by transmeatal approach. However, as the tumor recur red, she was referred to our department for surgery
Author's
ceruminous
Accepted K. Kodaki, Japan.
M.D.,
June
Fig. 1
Coronal tomogram of the temporal bone, demonstrating destruction of the left petrous portion (arrows).
5, 1990
Department
of Neurosurgery,
The
Second
Tokyo
National
Hospital,
Tokyo,
showed a markedly enhanced mass in the left cere bellopontine (CP) angle extending into the middle ear space with wide destruction of the left petrous bone (Fig. 2). Left carotid angiograms demon strated a highly vascular CP angle mass supplied by the occipital branch of the external carotid ar tery (Fig. 3). Left vertebral angiograms showed superior displacement of the peripheral branch of the anterior inferior cerebellar artery and faint tumor stain. These findings suggested a glomus jugulare tumor. A left subtemporal craniotomy was performed on May 4. The soft, highly vascular tumor was entirely located extradurally in the posterior fossa and destructively invaded the left middle ear space and the petrous bone. Only partial removal was possible because of massive bleeding. Histological examina tion revealed tumor cells arranged in a papillary pat
Fig. 3
Left carotid angiogram, demonstrating the vascular mass (arrow) fed by the occipital branch of the external carotid artery.
tern within prominent fibrous stroma, without atypical features and mitoses suggestive of malignan cy (Fig. 4). The histological diagnosis was thus papillary adenoma. Clinically, this tumor was con sidered to originate from the ceruminous gland. Postoperatively, she developed left facial paralysis and was discharged in June. Bloody discharge from the left ear developed, and partial removal of the tumor was again performed by transmastoidal approach in October, 1982. There after, because of the tumor extension into the mas toid cavity, partial removal of the tumor was per formed by the same approach in January, 1985 and again in March, 1986. All the tumors removed were histologically diagnosed as adenoma. In April, 1988, she was readmitted for resection of recurrent retroauricular tumor with bloody discharge. CT scans revealed a large tumor extending into the posterior and temporal fossae (Fig. 5). The retroauricular mass was resected in May, and its histological findings were largely similar to the
Fig. 2
Pre (upper) and postcontrast (lower) CT scans, showing a mass in the left CP angle with destruction of the left petrous bone.
previous one excepting for mitotic figures (Fig. 6). Considering the wide destruction of the temporal bone, the clinical course, and the histological find ings of the tumor, a final diagnosis of ceruminous adenocarcinoma was made. Mental disturbance and pneumonia then developed, and she died in Decem ber, 1988.
Fig.
4
Photomicrographs tumor
cells
There cy.
are
of
arranged no findings
HE stain,
upper:
the in
tumor,
a papillary
suggestive
showing pattern.
of malignan
x 100, lower:
x 200.
Fig. 6
Photomicrographs showing
similar
of
4 excepting
for a mitotic
per:
lower:
x200,
the
histological
recurrent findings
figure.
tumor, as in Fig.
HE stain,
up
x400.
by Berlin in 19493) followed by nine such cases in the literature,'-',9-l') as far as we are aware. Those 10 cases showed no sex difference in incidence with the ages of patients ranging from 7 to 77 years. In most patients, the tumor invaded volved the facial nerve. The was long, often over several
Fig.
5
Postcontrast invading
CT
scans,
the temporal
revealing and posterior
a large
mass
fossae.
Discussion Ceruminous glands are modified apocrine glands, which lie deep in the dermis of the cartilaginous por tion of the external auditory canal. 1,2,41Tumors of ceruminous first report
glands rarely of intracranial
extend intracranially. ceruminous adenoma
The was
the CP angle and in duration of symptoms years. Cerebral angio
grams demonstrated highly vascular masses mainly supplied by the external carotid artery, especially by its occipital and meningeal branches.',',"' in most cases, the preoperative clinical diagnosis was chemodectoma.5'6"") In our case, CT scans revealed a markedly enhanced isodense mass; these findings are the same as those reported by Cilluffo et al.') In 1972, Wetli et al. 12) simply classified the ceruminous gland tumors into four types: adenoma, adenocarcinoma, adenoid cystic carcinoma, and pleomorphic adenoma. Histologically, tumors ex tending intracranially have been either adeno mas 3.6,9,'0>or adenocarcinomas.s''"") Although adeno carcinoma histologically resembles adenoma, the crucial feature of adenocarcinoma is invasion.") In our case, the initial histological diagnosis was ade
noma, but the tumor recurred invasively and was finally diagnosed as adenocarcinoma. The fact that adenocarcinoma often recurs after surgery shows the difficulty of radical treatment.'," Hicks" recom mended wide local excision only for benign tumors and aggressive wide en bloc resection followed by irradiation for malignant tumors. However, total removal of a tumor with intracranial extension is difficult because of its invasiveness and hypervas cularity. For such tumors, Cilluffo et a!.,5) consider ing their slow natural growth, recommended sub total excision followed by radiation therapy. In adequate initial treatment or delayed diagnosis for ceruminoma, as in our case, results in intracranial invasion, which makes total removal of the tumor difficult.,') Histological differentiation of adenoma from adenocarcinoma is impossible,'") and for this reason, early radical excision is important even if the biopsy shows a histologically benign form. References 1) 2)
3) 4)
Althaus SR, Ross JAT: Cerumen gland neoplasia. Arch Otolaryngol 92: 40-42, 1970 Batsakis JG, Hardy GC, Hishiyama RH: Cerumi nous gland tumors. Arch Otolaryng (Chicago) 86: 66-69, 1967 Berlin L: Intracranial ceruminous adenoma. J Neurosurg 6: 415-418, 1949 Canker V, Crowley H: Tumors of ceruminous
glands: Cancer
A clinicopathological 17: 67-75, 1964
study
of seven
cases.
5)
Cilluffo JM, Harrier SG, Miller ceruminous gland adenocarcinoma. 952-965, 1981
6)
Dilenge D, Nutik S, Poliquin J, Masse S: Ceruminous tumor of the jugular foramen. J Can Assoc Radiol 28: 287-290, 1977
7)
Hageman MEJ, Becker AE: Intracranial invasion of a ceruminous gland tumor. Arch Otolaryngol 100: 395-397, 1974 Hicks GW: Tumors arising from the glandular struc tures of the external auditory canal. Laryngoscope 93: 326-340, 1983 Kleinsasser O, Scharfetter G: Zeruminaldrusenade noma mit Einbruch in Dura and Kleinhirn. Zbl Neurochir 17: 4-12, 1957 Norman D, Newton TH: Ceruminous tumors of the cerebellopontine angle. Neuroradiology 10: 1-4, 1975 Rossato RG, Timperly WR: Posterior fossa ceruminoma. Acta Neurochir (Wien) 28: 315-322, 1973 Wetli CV, Pardo V, Millard M, Frcpi MB, Path FRC, Gerston K: Tumors of ceruminous glands. Cancer 29: 1169-1178, 1972
8)
9)
10) 11)
12)
RH: Intracranial J Neurosurg 55:
Address reprint requests to: S. Toya, M.D., Department of Neurosurgery, Keio University School of Medicine, 35 Shinano-machi, Shinjuku-ku, Tokyo 160, Japan.
