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Pleomorphic Adenoma of the External Auditory Canal in Japan, with A Case Report
Case Report
Auris' Nasl/s' Larynx (Tokyo) 18, 271-279 (1991)
PLEOMORPHIC ADENOMA OF THE EXTERNAL AUDITORY CANAL IN JAPAN, WITH A CASE REPORT Kenji SUZUKI, M.D., Shunkichi BABA, M.D., and Naoya MIYAMOTO, M.D. Department of Otorhinolaryngology, Nagoya City University Medical School, Nagoya, Japan
A rare case of a 51-year-old female with a pleomorphic adenoma, measuring 0.9 X 0.7 X 0.6 (cm 3 ), originating from the right external auditory canal (EAC), was reported. The authors discussed the 7 reported cases of EAC pleomorphic adenoma in Japan, comparing them with pleomorphic adenomas occurring in the nasal cavity and the parotid gland. It is currently too early to conclude that pleomorphic adenoma in the EAC does not tend to recur or shows a marked tendency toward canceration. The best possible treatment for EAC pleomorphic adenoma at present seems to be, as in cases of tumors in other sites, surgical resection together with removal of a sufficient range of surrounding normal tissue, followed by careful long-term postoperative observation of the clinical course. Pleomorphic adenomas are also termed mixed tumors and have been reported to occur frequently in the major salivary glands (e.g., parotid and submandibular glands) as well as in minor salivary glands distributed in the oral palate (TOMMERUP and MOGENSEN, 1983). Rarer cases reportedly include onset of this type of tumor in the nasal cavity, laryngopharyngeal area, trachea, or lacrimal glands (NAIL, 1967). Whether malignant or benign in nature, these tumors seldom occur in the external auditory canal (EAC). Among them, pleomorphic adenomas are observed in but a few cases; only 6 cases are known to have been detected in Japan. The present article was intended to report a case of apocrine-glandderived pleomorphic adenoma. We also made statistical observations of EAC pleomorphic adenoma in Japan and comparative studies with other pleomorphic adenomas of the nasal cavity or parotid gland.
Received for publication November 1, 1990 271
272
K. SUZUKI ef al. CASE REPORT
Case: M. T., 51 years, female. Chief complaint: Bloody otorrhea in the right ear. Anamnesis: Hypertension. Familial history: Nothing remarkable. Present illness: As small amounts of blood were habitually found on the applicator when the patient cleaned her right EAC, an otolaryngologist discovered the tumor developing in her right EAC. The patient then was admitted to the present authors' hospital department. Present status: The patient's right EAC was occupied by an rigid-elastic tumor which extruded in the anterior bone-cartilage transitional zone of the canal. Although this swelling, accompanied by bleeding in several places, exhibited no adhesion to the auricular cartilage, it adhered to the dermal tissue of the canal (Fig. 1). The patient complained of right hearing loss without otalgia, otorrhea, or itching of the canal. No abnormalities were found radiologically in the middle and the inner ear. No abnormal findings were discovered in clinical laboratory tests or in the otolaryngological field. After diagnostic excision of the swelling, it was histopathologically diagnosed as a pleomorphic adenoma. Opel'ationa/.findings: An endaural operation was conducted under systemic anesthesia on June 17, 1985. The tumor was extracted together with accompanying sufficient normal tissue and EAC skin, and a piece collected from the temporal fascia was grafted onto this skin-lacking portion. The extracted specimen was a grayish-white, rigid-elastic piece of tumor, O.9xO.7xO.6 (cm S) in size, with a smooth surface accompanied by some normal
Fig. 1. Tumor; elastic-hard and bleeding in several places, arising from anterior bonecartilage transitional zone of right auditory canal.
PLEOMORPHIC ADENOMA OF EXTERNAL AUDITORY CANAL
273
Fig. 2. Extracted specimen; grayish white, smooth surface accompanied by some norm'll tissue, and strongly adhering to the skin.
Fig. 3. Histopathological finding; representative section of pleomorphic adenoma. of the glands have apocrine appearance. (Original magnification x 125.)
Some
healthy tissue, and was strongly adhered to the skin (Fig. 2). The cut surface was reddish brown in color and presented solid and rigid-elastic hemorrhagic lesions. According to histopathological observation under an optical microscope of weak magnification, the distinct formation of bilayered lumen, palisade pattern, and spindle-shaped cells was evident. The cells had hyaloid eosinophilic contents with sporadically distributed cartilagenous portions and myxomalike portions. The diagnosis was thus confirmed as pleomorphic adenoma. A distinct adenomatous structure containing cells arranged in a bilayer mode and including cubic cells was observed, which was a characteristic secretory morphological feature of the apocrine glands. Thus, this tumor was diagnosed as an
274
K. SUZUKI et al.
apocrine-gland-derived pleomorphic adenoma (Fig. 3). Postoperative clinical course: The patient's course was satisfactory, and at 5 years after surgery no recurrence is seen. Her hearing ability has become normal, recovering from her former hearing level of 50 dB. INVESTIGATION OF REPORTED CASES OF PLEOMORPHIC ADENOMAS OCCURRING IN EAC IN JAPAN
Including the case reported here, a total of 7 cases of pleomorphic adenomas primarily occurring in the EAC reported in Japan are summarized in Table l. What follows is a brief summary of each case. Case No.1: The first case in Japan was reported by FUKUDA, KOMATSU, KUNIMOTO, and W AKAI (1953). The patient was a 44-year-old female, who had experienced right otorrhea for 3 years and 6 months. She was hospitalized with the complaint of right ear pain. At the time of the first examination, a tumor filled the right EAC, from where metastasis was observed to the lung and intracranial part, indicating a case of malignant mixed tumors. Radiological treatment was attempted (64.5 Gy), but death occurred 2 years and 6 months later. Case No.2: The patient was a 15-year-old female, reported by FUSHIMI, OKAMI, and OSHIMA (1965), who had been aware of a painless tumor for 5 years. She left it as it was, but the tumor was accompanied by an incurable ulcer. The first histopathological examination lead to the diagnosis of inflammatory granulation. However, since the second histological diagnosis was adenocarcinoma of low-grade malignancy, radiotherapy at a small dose (17.5 Gy) was started. Finally, the diagnosis of mixed tumor was given after the third histopathological examination, and the tumor was extirpated. Its size was 2.5 X 1.5 X 0.3 (cm 3 ). Case No.3: A male patient, 64 years of age, having otorrhea in the left ear for 2 years and 6 months and complaining of a tumor with otalgia, as reported by TANI and UCHIDA (1966). Histopathological examination led to a diagnosis of a mixed tumor. Since the patient also had heart disease, surgical intervention was considered risky. Radiotherapy at 65 Gy was performed. Recurrence was observed 1 year and 6 months later. The histological diagnosis was malignant mixed tumor. Metastasis to the right lung was seen. After overall evaluation, radical resection of the EAC malignant tumor was performed. Postoperative irradiation was conducted on the left ear and the metastatic lesions in the right lung. The size of the tumor was not reported. The patient was seen for one year after treatment, after which the clinical course is unknown. Case No.4: KOIDE (1970) reported a 76-year-old male patient with the chief complaint of a painless tumor in the right EAC. Although he had been aware of this for 5 to 6 years without seeing a physician, he finally had himself examined because of the onset of otorrhea and itching. Complete removal of the tumor, 2.9 X 2.4 X 2.0 (cm 3 ) in size, was performed, leading to recovery.
M F M F
64
76 31 52 51
TAN! et al. (1966)
(1970) TANAKA et al. (1982) TANAKA et al. (1984) Present case
3.
4. 5. 6. 7.
F
Painless tumor Painless tumor Ear obstruction Bloody otorrhea
Painful tumor
L
R L L R
Intractable ulcer
Painful tumor
Chief complaint
L
R
Site
Excision Excision Excision Excision Excision
1
Excision Radiotherapy (65 Gy)
1
Radiotherapy (64.5 Gy) Radiotherapy (17.5 Gy)
Treatment
Unknown 2.9x2.4 x 2 .0 1.5 x 1.2 x 1.2 One em in diameter 0.9 x O.7 x O. 6
2.5x1.5 x O.3
Unknown
Size (cm3 )
Pleomorphic adenomas of the external auditory canal in Japan.
F, female ; M, male; R, right side; L , left side; Gy, gray.
KOIDE
M
15
FUSHIMl ef al. (I 965)
2.
F
44
FUKUDA et al. (1953)
l.
Sex
Age
References
Case No.
Table l.
Malignant change; No recurrence, 1 year No recurrence, 1 year No recurrence, 1 year No information No recurrence, 5 years
No recurrence, 2 years
Malignancy; Death, 2.5 years
Sequelae
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Case No.5: TANAKA, INOUE, HIRADO, TSUBAKI, MIYAKOGAWA, and SAWADA (1982) reported a case of a tumor in the left EAC which was ignored for 2 years. The patient then began to experience itching, for which reason he had himself examined. The tumor was completely removed. It was a rigid, elastic swelling measuring 1.5 X 1.2 X 1.2 (cm 3). The operation lead to recovery. Case No.6: A 52-year-old male patient reported by TANAKA, SAITO, ISHIHARA, OHMICHI, and OGURA (1984) complained chiefly of a feeling of obstruction in his left ear. The tumor causing it measured 1 em in diameter and was surgically removed. Case No.7: The authors encountered a 51-year-old female with the chief complaint of hemorrhage in her right ear. The tumor removed was rigid-elastic, with a size of 0.9 X 0.7 X 0.6 (cm 3). No recurrence has been observed during the 5 years since the operation. To summarize the above cases, the patients' ages ranged from 15 to 76 years, with a mean of 47.6 years. There were 3 males and 4 females, and no significant sexual difference was seen. Three were on the right and four were on the left. There were 2 painless tumor cases (28.6 %; they were ignored for 2 and 6 years, respectively), 2 painful tumor cases (28.6 %; they experienced otorrhea for 2 years and 6 months and 3 years and 6 months, respectively), and one case each of incurable ulcer, obstructive feeling in the ear, and ear hemorrhage, respectively (14.3 %). Five (71.4 %) cases included complaints of otorrhea, while 2 (28.6 %) complained of ear itching. The size of the tumors ranged from the minimal 0.9 X 0.7 X 0.6 (cm 3) to the maximal 2.9 X 2.4 X 2.0 (cm 3). The size was not reported in 2 cases. One was malignant and one had inverted malignancy. DISCUSSION
Pleomorphic adenomas are frequently seen to occur in the major salivary glands, especially in the parotid gland. BERGMAN (1969) stated that the incidence in the major versus minor glands was about 6 : 1. Pleomorphic adenoma originating from the EAC is reportedly very rare, and NAIL (1967) indicated that no primary onset of the tumor was seen in the EAC among the 25 cases of mixed tumors treated at the Jefferson Davis-Ben Taub Hospital from 1953 to 1963. DEHNER and CHEN (1980), on the other hand, reported that only one out of 12 cases of ceruminous adenoma was diagnosed as pleomorphic adenoma. In Japan, only 7 cases of pleomorphic adenoma originating from the EAC had been reported by the end of 1989, including the one described here. Cases in the English literature are limited to the following 12 reports: MARK and ROTHBERG (1951), FINK (1953), NANDI and SHAW (1961), SMITH and DUARTE (1962), CANKER and CROWLEY (1964), NAIL (1967), PAHOR and O'HARA (1975), BAKER and DEBLANC (1977), GOLDENBERG and BLOOK (1980), DEHNER and CHEN (1980), HICKS (1983), and COLLINS and Yu (1989).
PLEOMORPHIC ADENOMA OF EXTERNAL AUDITORY CANAL
277
While the present tumor has been termed a "mixed tumor" ever since Billroth's report (BILLROTH, 1859), the term was derived from "the mixed tumor theory" that this type of tumor is derived from mesenchymal cells. According to ENEROTH (1966), there are still 50 or more synonyms in use. WILLIS (1967) proposed the term "pleomorphic adenoma," which has since found widespread acceptance. WELSH and MEYER (1968) and QUINTARELLI and RUBINSON (1967) argued against the viewpoint that this tumor is derived purely from epithelial cells. A present popular view, however, is that the inerstitial tissue-resembling composition of the tumor is also derived from the epithelial structure (KROMPECHER, 1908), specifically, from muscular epithelial cells (ERLANDSON, CARDO, and HIGGINS, 1984). Comparison of the pleomorphic adenomas in the EAC, nasal cavity (SUZUKI, BABA, and MORIBE, 1990) and parotid gland (KITAMURA, KANEKO, TOGAWA, NOGUCHI, SHIMADA, ASANO, IIZUMI, CHIN, TSUKAMOTO, NAITO, and TORII, 1971) among patients in Japan has led to the following results (Table 2). In terms of sexual difference, the male: female ratios are 1 : 1.3 for the EAC, I : 1.8 for the nasal cavity, and 1 : 2.1 for the parotid gland, reflecting prevalence among females. The mean ages of patients at first examination were 47.6 years for lesions in the EAC, 44.6 for the nasal cavity, and 32.8 for the parotid gland; older people tended to have pleomorphic adenomas of the EAC and nasal cavity. Anatomically, lesions in the EAC and nasal cavity appeared comparatively early, and removal was easy because important constructive elements, such as facial nerves, did not interfere with the operational procedures. Due to these anatomical features, the rate of recurrence in the parotid gland is 8.8 % as against 2.4 and 0 % in the nasal cavity and EAC, respectively. While malignant cases of the parotid gland occurred at a rate of 4.9 %, the rate was 2.4 %, or only one case, for nasal cavity lesions. However, the malignancy rate was higher-one out of 7 cases (14.3 %) for EAC lesions. The figure is due to the lower frequency of manifestation in the EAC. DUTRA (1960) pointed out that mixed tumors occurring in the skin do not exhibit local recurrence or distant metastasis. STOUT and GURMAN (1959) reTable 2. Pleomorphic adenomas in Japan: cavity, and parotid gland. "-'----
Comparison of external auditory canal, nasal
- - - - - - -
Categories Sexual ratio (male: female) Mean age Recurrence rate after excision Malignancy rate
External auditory canal (7 cases) 1: 1. 3 47.6 0% 14.3%
Nasal cavity (SUZUKI et al., 1990) (41 cases) 1 : 1.8
Parotid gland (KITAMURA et al., 1971) (122 cases) 1 : 2.1
44.6 2.4%
32.8 8.8%
2.4%
4.9%
278
K. SUZUKI et al.
ported 39 cases of primary mixed tumors in the sweat glands, which did not include a single malignant case. On the other hand, ASH and RAUM (1956) noted a higher malignancy rate in ectopic sites than in major salivary glands. The present statistic also indicates a high frequency of 14.3 %. Radiotherapy has been ineffective for pleomorphic adenomas. Irradiation may in fact induce canceration, as observed in a case in this article and mentioned by SAKSERA, TARKKANEN, and KOHONEN (1970). The only sure method for treatment is surgical removal of the tumor. In growing, the tumor pushes out from a weak portion of its capsule, which is often very thin or practically absent in some places. The simple renucleation of the tumor may thus leave a microscopic tumor tissue islet, or the operation may allow a tumor seeding so as to lead to recurrence at a high rate. Hence, as proposed by CANKER and CROWLEY (1964), NAIL (1967), and HICKS (1983), careful resection including removal of the surrounding normal healthy tissue must be performed. CONCLUSION
Since only a few cases of EAC pleomorphic adenoma have been encountered thus far in Japan, it is too risky to derive the conclusion that EAC pleomorphic adenoma does not tend to recur or shows a marked tendency toward canceration. Sufficient surgical removal and careful long-term postoperative observation of the patient's clinical course are necessary. REFERENCES ASH, J. E., and RAUM, M.: An atlas of otolaryngic pathology, Armed Forces Institute of Pathology, Washington, D.C., 1956. BAKER, B. B., and DEBLANC, G. B.: Pleomorphic adenoma of the external auditory canal. ENT J. 56: 59-61, 1977. BERGMAN, F.: Tumors of minor salivary glands: Report of 46 cases. Cancer 23: 534-543, 1969. BILLROTH, T.: Beobachtungen tiber Geschwtilste der Speicheldrllsen. Virchow Arch. Palhol. Anatol1l. 17: 357-375, 1859. CANKER, Y., and GROWLEY, H.: Tumors of ceruminous glands. A clinicopathological study of 7 cases. Cancer 17: 67-75, 1964. COLLINS, R. J., and Yu, H. C.: Pleomorphic adenoma of the external auditory canal. An immunohistochemical and ultrastructural study. Cal/cer 64: 870-875, 1989. DEHNER, L. P., and CHEN, K. T. K.: Primary tumors of the external and middle ear. Benign and malignant glandular neoplasmas. Arch. OtolarYl/gol. 106: 13-19, 1980. DUTRA, F. R.: Mixed tumors, salivary gland type, of deep facial resion of thigh. Arch. Pathol. 70: 562-564, 1960. ENEROTH, C. M.: Classification of parotid tumor. Proc. R. Soc. Med. 59: 429--431, 1966. ERLANDSON, R. A., CARDO, C. c., and HIGGINS, P. J.: Histogenesis of benign pleomorphic adenoma (mixed tumor) of the major salivary glands. An ultrastructural and immunohistochemical study. Am. J. Surg. Pathol. 8: 803-820, 1984. FINK, H.: External auditory canal: Mixed tumor of salivary gland type. Brooklyn Hosp. J. 11: 104-105, 1953.
PLEOMORPHIC ADENOMA OF EXTERNAL AUDITORY CANAL
279
FUKUDA, E., KOMATSU, H., KUNIMOTO, S., and WAKAI, K.: A case of mixed parotid tumor in the external auditory canal. Jibi Inkoka (in Japanese) 25: 336-339, 1953. FUSHIMI, N., OKAMI, H., and OSHIMA, A.: A mixed salivary gland tumor originating from the external ear canal. Jibi Inkoka (in Japanese) 37: 849-852, 1965. GOLDENBERG, R. A., and BLOCK, B. L.: Pleomorphic adenoma manifesting as aural polyp. Arch. Otolaryngol. 106: 440-441, 1980. HICKS, G. W.: Tumors arising from the glandular structures of the external auditory canal. Laryngoscope 93: 326-340, 1983. KITAMURA, T., KANEKO, T., TOGAWA, K., NOGUCHI, U., SHIMADA, F., ASANO, H., IIZUMI, 0., CHIN, Z., TSUKAMOTO, K., NAITO, J., and TORII, M.: Clinical study on parotid gland tumors. Fract. Otol. (Kyoto) (in Japanese) 64: 1286-1301, 1971. KOIDE, Y.: A mixed tumor in the skin of the external auditory canal. J. OtolarYl/gol. Jpl/. (in Japanese) 73: 1780-1783, 1970. KROMPECHER, E.: Zur Histogenese und Morphologie der Mischgeschwiilste del' Haut sowie der Speichel- und Schleimdriisen. Beitr. Path. AI/at. M. Z. Allg. Path. 44: 51-87, 1908. MARK, 1., and ROTHBERG, M.: Mixed tumor of skin of external auditory canal. Arch. Otolaryl/gol. 53: 556-559, 1951. NAIL, B. M., Jr.: Mixed tumors in ectopic sites. Tex. Med. 63: 82-85, 1967. NANDI, S. P., and SHAW, H. J.: Hidradenoma of the external auditory meatus associated with facial palsy and chronic otitis media. J. LarYl/gol. 75: 992-996, 1961. PAHOR, A. L., and O'HARA, M. D.: Hidradenomata of the external auditory meatus (Review of the literature and report of a pleomorphic adenoma). J. Laryngol. 0101. 89: 707-720, 1975. QUINTARELLI, G., and RUBISON, L.: The glycosaminoglycans of salivary gland tumors. A histochemical characterization and a critical evaluation. Alii. J. PatllOl. 51: 19-37, 1967. SAKSERA, E., TARKKANEN, J., and KOHONEN, A. : The malignancy of mixed tumors of the parotid gland. A clinicopathological analysis of 70 cases. Acta Otolaryngol. 70: 62-70, 1970. SMITH, H. W., and DUARTE, 1.: Mixed tumors of the external auditory canal. Arch. 010laryngol. 75: 108-113, 1962. STOUT, A. P., and GURMAN, J. G.: Mixed tumors of the skin of the salivary gland type. Cancel' 12: 537-543, 1959. SUZUKI, K., BABA, S., and MORIBE, K.: A case of pleomorphic adenoma of lateral wall in nasal cavity -With special reference of statistical observation of pleomorphic adenoma in Japan- (in Japanese). J. Ololaryngol. Jpn. 93: 740-745, 1990. TANAKA, E., INOUE, T., HIRADO, H., TSUBAKI, Y., MIYAKOGAWA, N., and SAWADA, M.: A mixed tumor of the external auditory canal (in Japanese). Clin. Otol. Jpn. 9: 104-105, 1982. TANAKA, T., SAITO, R., ISHIHARA, M., OHMICHI, T., and OGURA, Y.: Glandular tumors of the external auditory canal. Hiroshima J. Med. Sci. 33: 17-22, 1984. TANI, I., and UCHIDA, K.: A mixed parotid tumor of the external aural canal (in Japanese). Jibi Inkoka 38: 1065-1067, 1966. TOMMERUP, B., and MOGENSEN, c.: Pleomorphic adenoma of the nose. Rhinology 21: 79-82, 1983. WELSH, R. A., and MEYER, A. T.: Mixed tumors of human salivary gland. Arch. PalllOl. 85: 433-447,1968. WILLIS, R. A.: Pathology of Tumours, 3rd ed., CV Mosby, Co., St Louis, 1967.
Request reprints to:
Dr. K. Suzuki, Department of Otorhinolaryngology, Nagoya City University Medical School, 1 Kawasumi, Mizuho-cho, Mizuho-ku, Nagoya 467, Japan
Auris' Nasl/s' Larynx (Tokyo) 18, 271-279 (1991)
PLEOMORPHIC ADENOMA OF THE EXTERNAL AUDITORY CANAL IN JAPAN, WITH A CASE REPORT Kenji SUZUKI, M.D., Shunkichi BABA, M.D., and Naoya MIYAMOTO, M.D. Department of Otorhinolaryngology, Nagoya City University Medical School, Nagoya, Japan
A rare case of a 51-year-old female with a pleomorphic adenoma, measuring 0.9 X 0.7 X 0.6 (cm 3 ), originating from the right external auditory canal (EAC), was reported. The authors discussed the 7 reported cases of EAC pleomorphic adenoma in Japan, comparing them with pleomorphic adenomas occurring in the nasal cavity and the parotid gland. It is currently too early to conclude that pleomorphic adenoma in the EAC does not tend to recur or shows a marked tendency toward canceration. The best possible treatment for EAC pleomorphic adenoma at present seems to be, as in cases of tumors in other sites, surgical resection together with removal of a sufficient range of surrounding normal tissue, followed by careful long-term postoperative observation of the clinical course. Pleomorphic adenomas are also termed mixed tumors and have been reported to occur frequently in the major salivary glands (e.g., parotid and submandibular glands) as well as in minor salivary glands distributed in the oral palate (TOMMERUP and MOGENSEN, 1983). Rarer cases reportedly include onset of this type of tumor in the nasal cavity, laryngopharyngeal area, trachea, or lacrimal glands (NAIL, 1967). Whether malignant or benign in nature, these tumors seldom occur in the external auditory canal (EAC). Among them, pleomorphic adenomas are observed in but a few cases; only 6 cases are known to have been detected in Japan. The present article was intended to report a case of apocrine-glandderived pleomorphic adenoma. We also made statistical observations of EAC pleomorphic adenoma in Japan and comparative studies with other pleomorphic adenomas of the nasal cavity or parotid gland.
Received for publication November 1, 1990 271
272
K. SUZUKI ef al. CASE REPORT
Case: M. T., 51 years, female. Chief complaint: Bloody otorrhea in the right ear. Anamnesis: Hypertension. Familial history: Nothing remarkable. Present illness: As small amounts of blood were habitually found on the applicator when the patient cleaned her right EAC, an otolaryngologist discovered the tumor developing in her right EAC. The patient then was admitted to the present authors' hospital department. Present status: The patient's right EAC was occupied by an rigid-elastic tumor which extruded in the anterior bone-cartilage transitional zone of the canal. Although this swelling, accompanied by bleeding in several places, exhibited no adhesion to the auricular cartilage, it adhered to the dermal tissue of the canal (Fig. 1). The patient complained of right hearing loss without otalgia, otorrhea, or itching of the canal. No abnormalities were found radiologically in the middle and the inner ear. No abnormal findings were discovered in clinical laboratory tests or in the otolaryngological field. After diagnostic excision of the swelling, it was histopathologically diagnosed as a pleomorphic adenoma. Opel'ationa/.findings: An endaural operation was conducted under systemic anesthesia on June 17, 1985. The tumor was extracted together with accompanying sufficient normal tissue and EAC skin, and a piece collected from the temporal fascia was grafted onto this skin-lacking portion. The extracted specimen was a grayish-white, rigid-elastic piece of tumor, O.9xO.7xO.6 (cm S) in size, with a smooth surface accompanied by some normal
Fig. 1. Tumor; elastic-hard and bleeding in several places, arising from anterior bonecartilage transitional zone of right auditory canal.
PLEOMORPHIC ADENOMA OF EXTERNAL AUDITORY CANAL
273
Fig. 2. Extracted specimen; grayish white, smooth surface accompanied by some norm'll tissue, and strongly adhering to the skin.
Fig. 3. Histopathological finding; representative section of pleomorphic adenoma. of the glands have apocrine appearance. (Original magnification x 125.)
Some
healthy tissue, and was strongly adhered to the skin (Fig. 2). The cut surface was reddish brown in color and presented solid and rigid-elastic hemorrhagic lesions. According to histopathological observation under an optical microscope of weak magnification, the distinct formation of bilayered lumen, palisade pattern, and spindle-shaped cells was evident. The cells had hyaloid eosinophilic contents with sporadically distributed cartilagenous portions and myxomalike portions. The diagnosis was thus confirmed as pleomorphic adenoma. A distinct adenomatous structure containing cells arranged in a bilayer mode and including cubic cells was observed, which was a characteristic secretory morphological feature of the apocrine glands. Thus, this tumor was diagnosed as an
274
K. SUZUKI et al.
apocrine-gland-derived pleomorphic adenoma (Fig. 3). Postoperative clinical course: The patient's course was satisfactory, and at 5 years after surgery no recurrence is seen. Her hearing ability has become normal, recovering from her former hearing level of 50 dB. INVESTIGATION OF REPORTED CASES OF PLEOMORPHIC ADENOMAS OCCURRING IN EAC IN JAPAN
Including the case reported here, a total of 7 cases of pleomorphic adenomas primarily occurring in the EAC reported in Japan are summarized in Table l. What follows is a brief summary of each case. Case No.1: The first case in Japan was reported by FUKUDA, KOMATSU, KUNIMOTO, and W AKAI (1953). The patient was a 44-year-old female, who had experienced right otorrhea for 3 years and 6 months. She was hospitalized with the complaint of right ear pain. At the time of the first examination, a tumor filled the right EAC, from where metastasis was observed to the lung and intracranial part, indicating a case of malignant mixed tumors. Radiological treatment was attempted (64.5 Gy), but death occurred 2 years and 6 months later. Case No.2: The patient was a 15-year-old female, reported by FUSHIMI, OKAMI, and OSHIMA (1965), who had been aware of a painless tumor for 5 years. She left it as it was, but the tumor was accompanied by an incurable ulcer. The first histopathological examination lead to the diagnosis of inflammatory granulation. However, since the second histological diagnosis was adenocarcinoma of low-grade malignancy, radiotherapy at a small dose (17.5 Gy) was started. Finally, the diagnosis of mixed tumor was given after the third histopathological examination, and the tumor was extirpated. Its size was 2.5 X 1.5 X 0.3 (cm 3 ). Case No.3: A male patient, 64 years of age, having otorrhea in the left ear for 2 years and 6 months and complaining of a tumor with otalgia, as reported by TANI and UCHIDA (1966). Histopathological examination led to a diagnosis of a mixed tumor. Since the patient also had heart disease, surgical intervention was considered risky. Radiotherapy at 65 Gy was performed. Recurrence was observed 1 year and 6 months later. The histological diagnosis was malignant mixed tumor. Metastasis to the right lung was seen. After overall evaluation, radical resection of the EAC malignant tumor was performed. Postoperative irradiation was conducted on the left ear and the metastatic lesions in the right lung. The size of the tumor was not reported. The patient was seen for one year after treatment, after which the clinical course is unknown. Case No.4: KOIDE (1970) reported a 76-year-old male patient with the chief complaint of a painless tumor in the right EAC. Although he had been aware of this for 5 to 6 years without seeing a physician, he finally had himself examined because of the onset of otorrhea and itching. Complete removal of the tumor, 2.9 X 2.4 X 2.0 (cm 3 ) in size, was performed, leading to recovery.
M F M F
64
76 31 52 51
TAN! et al. (1966)
(1970) TANAKA et al. (1982) TANAKA et al. (1984) Present case
3.
4. 5. 6. 7.
F
Painless tumor Painless tumor Ear obstruction Bloody otorrhea
Painful tumor
L
R L L R
Intractable ulcer
Painful tumor
Chief complaint
L
R
Site
Excision Excision Excision Excision Excision
1
Excision Radiotherapy (65 Gy)
1
Radiotherapy (64.5 Gy) Radiotherapy (17.5 Gy)
Treatment
Unknown 2.9x2.4 x 2 .0 1.5 x 1.2 x 1.2 One em in diameter 0.9 x O.7 x O. 6
2.5x1.5 x O.3
Unknown
Size (cm3 )
Pleomorphic adenomas of the external auditory canal in Japan.
F, female ; M, male; R, right side; L , left side; Gy, gray.
KOIDE
M
15
FUSHIMl ef al. (I 965)
2.
F
44
FUKUDA et al. (1953)
l.
Sex
Age
References
Case No.
Table l.
Malignant change; No recurrence, 1 year No recurrence, 1 year No recurrence, 1 year No information No recurrence, 5 years
No recurrence, 2 years
Malignancy; Death, 2.5 years
Sequelae
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Case No.5: TANAKA, INOUE, HIRADO, TSUBAKI, MIYAKOGAWA, and SAWADA (1982) reported a case of a tumor in the left EAC which was ignored for 2 years. The patient then began to experience itching, for which reason he had himself examined. The tumor was completely removed. It was a rigid, elastic swelling measuring 1.5 X 1.2 X 1.2 (cm 3). The operation lead to recovery. Case No.6: A 52-year-old male patient reported by TANAKA, SAITO, ISHIHARA, OHMICHI, and OGURA (1984) complained chiefly of a feeling of obstruction in his left ear. The tumor causing it measured 1 em in diameter and was surgically removed. Case No.7: The authors encountered a 51-year-old female with the chief complaint of hemorrhage in her right ear. The tumor removed was rigid-elastic, with a size of 0.9 X 0.7 X 0.6 (cm 3). No recurrence has been observed during the 5 years since the operation. To summarize the above cases, the patients' ages ranged from 15 to 76 years, with a mean of 47.6 years. There were 3 males and 4 females, and no significant sexual difference was seen. Three were on the right and four were on the left. There were 2 painless tumor cases (28.6 %; they were ignored for 2 and 6 years, respectively), 2 painful tumor cases (28.6 %; they experienced otorrhea for 2 years and 6 months and 3 years and 6 months, respectively), and one case each of incurable ulcer, obstructive feeling in the ear, and ear hemorrhage, respectively (14.3 %). Five (71.4 %) cases included complaints of otorrhea, while 2 (28.6 %) complained of ear itching. The size of the tumors ranged from the minimal 0.9 X 0.7 X 0.6 (cm 3) to the maximal 2.9 X 2.4 X 2.0 (cm 3). The size was not reported in 2 cases. One was malignant and one had inverted malignancy. DISCUSSION
Pleomorphic adenomas are frequently seen to occur in the major salivary glands, especially in the parotid gland. BERGMAN (1969) stated that the incidence in the major versus minor glands was about 6 : 1. Pleomorphic adenoma originating from the EAC is reportedly very rare, and NAIL (1967) indicated that no primary onset of the tumor was seen in the EAC among the 25 cases of mixed tumors treated at the Jefferson Davis-Ben Taub Hospital from 1953 to 1963. DEHNER and CHEN (1980), on the other hand, reported that only one out of 12 cases of ceruminous adenoma was diagnosed as pleomorphic adenoma. In Japan, only 7 cases of pleomorphic adenoma originating from the EAC had been reported by the end of 1989, including the one described here. Cases in the English literature are limited to the following 12 reports: MARK and ROTHBERG (1951), FINK (1953), NANDI and SHAW (1961), SMITH and DUARTE (1962), CANKER and CROWLEY (1964), NAIL (1967), PAHOR and O'HARA (1975), BAKER and DEBLANC (1977), GOLDENBERG and BLOOK (1980), DEHNER and CHEN (1980), HICKS (1983), and COLLINS and Yu (1989).
PLEOMORPHIC ADENOMA OF EXTERNAL AUDITORY CANAL
277
While the present tumor has been termed a "mixed tumor" ever since Billroth's report (BILLROTH, 1859), the term was derived from "the mixed tumor theory" that this type of tumor is derived from mesenchymal cells. According to ENEROTH (1966), there are still 50 or more synonyms in use. WILLIS (1967) proposed the term "pleomorphic adenoma," which has since found widespread acceptance. WELSH and MEYER (1968) and QUINTARELLI and RUBINSON (1967) argued against the viewpoint that this tumor is derived purely from epithelial cells. A present popular view, however, is that the inerstitial tissue-resembling composition of the tumor is also derived from the epithelial structure (KROMPECHER, 1908), specifically, from muscular epithelial cells (ERLANDSON, CARDO, and HIGGINS, 1984). Comparison of the pleomorphic adenomas in the EAC, nasal cavity (SUZUKI, BABA, and MORIBE, 1990) and parotid gland (KITAMURA, KANEKO, TOGAWA, NOGUCHI, SHIMADA, ASANO, IIZUMI, CHIN, TSUKAMOTO, NAITO, and TORII, 1971) among patients in Japan has led to the following results (Table 2). In terms of sexual difference, the male: female ratios are 1 : 1.3 for the EAC, I : 1.8 for the nasal cavity, and 1 : 2.1 for the parotid gland, reflecting prevalence among females. The mean ages of patients at first examination were 47.6 years for lesions in the EAC, 44.6 for the nasal cavity, and 32.8 for the parotid gland; older people tended to have pleomorphic adenomas of the EAC and nasal cavity. Anatomically, lesions in the EAC and nasal cavity appeared comparatively early, and removal was easy because important constructive elements, such as facial nerves, did not interfere with the operational procedures. Due to these anatomical features, the rate of recurrence in the parotid gland is 8.8 % as against 2.4 and 0 % in the nasal cavity and EAC, respectively. While malignant cases of the parotid gland occurred at a rate of 4.9 %, the rate was 2.4 %, or only one case, for nasal cavity lesions. However, the malignancy rate was higher-one out of 7 cases (14.3 %) for EAC lesions. The figure is due to the lower frequency of manifestation in the EAC. DUTRA (1960) pointed out that mixed tumors occurring in the skin do not exhibit local recurrence or distant metastasis. STOUT and GURMAN (1959) reTable 2. Pleomorphic adenomas in Japan: cavity, and parotid gland. "-'----
Comparison of external auditory canal, nasal
- - - - - - -
Categories Sexual ratio (male: female) Mean age Recurrence rate after excision Malignancy rate
External auditory canal (7 cases) 1: 1. 3 47.6 0% 14.3%
Nasal cavity (SUZUKI et al., 1990) (41 cases) 1 : 1.8
Parotid gland (KITAMURA et al., 1971) (122 cases) 1 : 2.1
44.6 2.4%
32.8 8.8%
2.4%
4.9%
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K. SUZUKI et al.
ported 39 cases of primary mixed tumors in the sweat glands, which did not include a single malignant case. On the other hand, ASH and RAUM (1956) noted a higher malignancy rate in ectopic sites than in major salivary glands. The present statistic also indicates a high frequency of 14.3 %. Radiotherapy has been ineffective for pleomorphic adenomas. Irradiation may in fact induce canceration, as observed in a case in this article and mentioned by SAKSERA, TARKKANEN, and KOHONEN (1970). The only sure method for treatment is surgical removal of the tumor. In growing, the tumor pushes out from a weak portion of its capsule, which is often very thin or practically absent in some places. The simple renucleation of the tumor may thus leave a microscopic tumor tissue islet, or the operation may allow a tumor seeding so as to lead to recurrence at a high rate. Hence, as proposed by CANKER and CROWLEY (1964), NAIL (1967), and HICKS (1983), careful resection including removal of the surrounding normal healthy tissue must be performed. CONCLUSION
Since only a few cases of EAC pleomorphic adenoma have been encountered thus far in Japan, it is too risky to derive the conclusion that EAC pleomorphic adenoma does not tend to recur or shows a marked tendency toward canceration. Sufficient surgical removal and careful long-term postoperative observation of the patient's clinical course are necessary. REFERENCES ASH, J. E., and RAUM, M.: An atlas of otolaryngic pathology, Armed Forces Institute of Pathology, Washington, D.C., 1956. BAKER, B. B., and DEBLANC, G. B.: Pleomorphic adenoma of the external auditory canal. ENT J. 56: 59-61, 1977. BERGMAN, F.: Tumors of minor salivary glands: Report of 46 cases. Cancer 23: 534-543, 1969. BILLROTH, T.: Beobachtungen tiber Geschwtilste der Speicheldrllsen. Virchow Arch. Palhol. Anatol1l. 17: 357-375, 1859. CANKER, Y., and GROWLEY, H.: Tumors of ceruminous glands. A clinicopathological study of 7 cases. Cancer 17: 67-75, 1964. COLLINS, R. J., and Yu, H. C.: Pleomorphic adenoma of the external auditory canal. An immunohistochemical and ultrastructural study. Cal/cer 64: 870-875, 1989. DEHNER, L. P., and CHEN, K. T. K.: Primary tumors of the external and middle ear. Benign and malignant glandular neoplasmas. Arch. OtolarYl/gol. 106: 13-19, 1980. DUTRA, F. R.: Mixed tumors, salivary gland type, of deep facial resion of thigh. Arch. Pathol. 70: 562-564, 1960. ENEROTH, C. M.: Classification of parotid tumor. Proc. R. Soc. Med. 59: 429--431, 1966. ERLANDSON, R. A., CARDO, C. c., and HIGGINS, P. J.: Histogenesis of benign pleomorphic adenoma (mixed tumor) of the major salivary glands. An ultrastructural and immunohistochemical study. Am. J. Surg. Pathol. 8: 803-820, 1984. FINK, H.: External auditory canal: Mixed tumor of salivary gland type. Brooklyn Hosp. J. 11: 104-105, 1953.
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FUKUDA, E., KOMATSU, H., KUNIMOTO, S., and WAKAI, K.: A case of mixed parotid tumor in the external auditory canal. Jibi Inkoka (in Japanese) 25: 336-339, 1953. FUSHIMI, N., OKAMI, H., and OSHIMA, A.: A mixed salivary gland tumor originating from the external ear canal. Jibi Inkoka (in Japanese) 37: 849-852, 1965. GOLDENBERG, R. A., and BLOCK, B. L.: Pleomorphic adenoma manifesting as aural polyp. Arch. Otolaryngol. 106: 440-441, 1980. HICKS, G. W.: Tumors arising from the glandular structures of the external auditory canal. Laryngoscope 93: 326-340, 1983. KITAMURA, T., KANEKO, T., TOGAWA, K., NOGUCHI, U., SHIMADA, F., ASANO, H., IIZUMI, 0., CHIN, Z., TSUKAMOTO, K., NAITO, J., and TORII, M.: Clinical study on parotid gland tumors. Fract. Otol. (Kyoto) (in Japanese) 64: 1286-1301, 1971. KOIDE, Y.: A mixed tumor in the skin of the external auditory canal. J. OtolarYl/gol. Jpl/. (in Japanese) 73: 1780-1783, 1970. KROMPECHER, E.: Zur Histogenese und Morphologie der Mischgeschwiilste del' Haut sowie der Speichel- und Schleimdriisen. Beitr. Path. AI/at. M. Z. Allg. Path. 44: 51-87, 1908. MARK, 1., and ROTHBERG, M.: Mixed tumor of skin of external auditory canal. Arch. Otolaryl/gol. 53: 556-559, 1951. NAIL, B. M., Jr.: Mixed tumors in ectopic sites. Tex. Med. 63: 82-85, 1967. NANDI, S. P., and SHAW, H. J.: Hidradenoma of the external auditory meatus associated with facial palsy and chronic otitis media. J. LarYl/gol. 75: 992-996, 1961. PAHOR, A. L., and O'HARA, M. D.: Hidradenomata of the external auditory meatus (Review of the literature and report of a pleomorphic adenoma). J. Laryngol. 0101. 89: 707-720, 1975. QUINTARELLI, G., and RUBISON, L.: The glycosaminoglycans of salivary gland tumors. A histochemical characterization and a critical evaluation. Alii. J. PatllOl. 51: 19-37, 1967. SAKSERA, E., TARKKANEN, J., and KOHONEN, A. : The malignancy of mixed tumors of the parotid gland. A clinicopathological analysis of 70 cases. Acta Otolaryngol. 70: 62-70, 1970. SMITH, H. W., and DUARTE, 1.: Mixed tumors of the external auditory canal. Arch. 010laryngol. 75: 108-113, 1962. STOUT, A. P., and GURMAN, J. G.: Mixed tumors of the skin of the salivary gland type. Cancel' 12: 537-543, 1959. SUZUKI, K., BABA, S., and MORIBE, K.: A case of pleomorphic adenoma of lateral wall in nasal cavity -With special reference of statistical observation of pleomorphic adenoma in Japan- (in Japanese). J. Ololaryngol. Jpn. 93: 740-745, 1990. TANAKA, E., INOUE, T., HIRADO, H., TSUBAKI, Y., MIYAKOGAWA, N., and SAWADA, M.: A mixed tumor of the external auditory canal (in Japanese). Clin. Otol. Jpn. 9: 104-105, 1982. TANAKA, T., SAITO, R., ISHIHARA, M., OHMICHI, T., and OGURA, Y.: Glandular tumors of the external auditory canal. Hiroshima J. Med. Sci. 33: 17-22, 1984. TANI, I., and UCHIDA, K.: A mixed parotid tumor of the external aural canal (in Japanese). Jibi Inkoka 38: 1065-1067, 1966. TOMMERUP, B., and MOGENSEN, c.: Pleomorphic adenoma of the nose. Rhinology 21: 79-82, 1983. WELSH, R. A., and MEYER, A. T.: Mixed tumors of human salivary gland. Arch. PalllOl. 85: 433-447,1968. WILLIS, R. A.: Pathology of Tumours, 3rd ed., CV Mosby, Co., St Louis, 1967.
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Dr. K. Suzuki, Department of Otorhinolaryngology, Nagoya City University Medical School, 1 Kawasumi, Mizuho-cho, Mizuho-ku, Nagoya 467, Japan