今日の臨床サポート

外耳・中耳腫瘍・グロームス腫瘍

著者: 角田篤信 順天堂大学 耳鼻咽喉・頭頸科

監修: 森山寛 東京慈恵会医科大学附属病院

著者校正/監修レビュー済:2016/07/21
患者向け説明資料

概要・推奨   

疾患のポイント:
  1. 外耳腫瘍とは、耳介または、外耳道の腫瘍のことである。
  1. 外耳、中耳腫瘍は外来で観察可能なものから頭蓋底手術を要する病変までさまざまである。
  1. 外耳道悪性腫瘍は百万人に数人のまれな腫瘍で扁平上皮癌が最も多い。中耳腫瘍は神経鞘腫、グロームス腫瘍などがあり、内頸静脈孔への進展がしばしばみられる。中耳悪性腫瘍はさらにまれであり、慢性中耳炎に合併する癌腫や横紋筋肉腫などがある。
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薬剤監修について:
オーダー内の薬剤用量は日本医科大学付属病院 薬剤部 部長 伊勢雄也 以下、林太祐、渡邉裕次、井ノ口岳洋、梅田将光による疑義照会のプロセスを実施、疑義照会の対象については著者の方による再確認を実施しております。
※薬剤中分類、用法、同効薬、診療報酬は、エルゼビアが独自に作成した薬剤情報であり、
著者により作成された情報ではありません。
尚、用法は添付文書より、同効薬は、薬剤師監修のもとで作成しております。
※薬剤情報の(適外/適内/⽤量内/⽤量外/㊜)等の表記は、エルゼビアジャパン編集部によって記載日時にレセプトチェックソフトなどで確認し作成しております。ただし、これらの記載は、実際の保険適用の査定において保険適用及び保険適用外と判断されることを保証するものではありません。また、検査薬、輸液、血液製剤、全身麻酔薬、抗癌剤等の薬剤は保険適用の記載の一部を割愛させていただいています。
(詳細はこちらを参照)
著者のCOI(Conflicts of Interest)開示:
角田篤信 : 特に申告事項無し[2021年]
監修:森山寛 : 特に申告事項無し[2021年]

まとめ

疾患情報(疫学・病態)  
  1. 外耳腫瘍には耳介腫瘍と外耳道腫瘍があり、頻度としては前者が多い。
  1. 外耳道腫瘍は骨腫、線維性骨異形成症など腫瘍類似疾患や疣贅、神経鞘腫などの良性疾患がみられる。
  1. 外耳道悪性腫瘍は百万人に数人のまれな腫瘍で扁平上皮癌が最も多い。
  1. 中耳腫瘍は神経鞘腫、グロームス腫瘍などがあり、内頸静脈孔への進展がしばしばみられる。
  1. 中耳悪性腫瘍はさらにまれであり、慢性中耳炎に合併する癌腫や横紋筋肉腫などがある。
問診・診察のポイント  
問診:
  1. 難聴・耳鳴・耳閉塞感の有無、特に拍動性耳鳴の有無と症状の変化・増悪

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文献 

著者: Paul W Gidley, Dianna B Roberts, Erich M Sturgis
雑誌名: Laryngoscope. 2010 Jun;120(6):1144-51. doi: 10.1002/lary.20937.
Abstract/Text OBJECTIVES/HYPOTHESIS: To study the survival outcomes of patients with squamous cell carcinoma (SCC) of the temporal bone. A secondary purpose was to evaluate the University of Pittsburgh staging system as a predictor of survival.
STUDY DESIGN: Retrospective review.
METHODS: We performed a single-institution retrospective review of the medical charts of patients diagnosed with SCC of the temporal bone between 1945 and 2005. We identified the patients' demographic characteristics, presenting symptoms, physical examination findings, tumor histology, disease extent, treatment course, and clinical outcomes. We used the Pittsburgh staging system (2000) to determine the patients' tumor classification and disease state. We then compared the overall and disease-free survival rates between patients with early-stage versus late-stage disease.
RESULTS: We identified 124 patients with SCC of the temporal bone. Of these, 71 had incident (untreated) SCC, 26 had recurrent SCC, and 27 had persistent SCC after treatment elsewhere. The 5-year overall survival rate for patients with incident SCC was 38%, and the disease-free survival rate was 60%. The overall survival rate for patients with incident SCC was similar to that for patients with persistent disease and was significantly better than that for patients with recurrent SCC (P = .008). Patients with early-stage tumors (T1 or T2) had longer overall survival than those with late-stage tumors (T3 or T4; P = .004, log-rank). The 5-year overall survival rate was 48% for patients with early-stage disease and 28% for patients with late-stage disease. Furthermore, patients with T1 tumors had significantly longer overall survival than patients with T2 tumors (P = .039) and patients with T3 and T4 tumors (P = .0008). Overall survival (OS) and disease-free interval (DFI) were improved for T2 tumors when radiotherapy was combined with surgery (OS, P = .011; DFI, P = .02). T1 tumors did not benefit in a statistically significant way with combined therapy. T3 and T4 tumors had relatively poor outcomes in spite of combined therapy. Twenty-two patients (31%) experienced a recurrence within 1 year of treatment, whereas only one patient developed recurrence after 1 year. Lymph node metastasis, facial paralysis, or involvement of the carotid artery, jugular foramen, or infratemporal fossa were not significantly associated with overall or disease-free survival.
CONCLUSIONS: Patients with recurrent SCC of the temporal bone had significantly shorter overall survival and disease-free interval than patients with incident SCC. In addition, patients with early-stage disease (T1 and T2) had significantly longer overall survival and disease-free survival than patients with late-stage tumors.

PMID 20513031  Laryngoscope. 2010 Jun;120(6):1144-51. doi: 10.1002/lar・・・
著者: David A Moffat, Sherryl A Wagstaff, David G Hardy
雑誌名: Laryngoscope. 2005 Feb;115(2):341-7. doi: 10.1097/01.mlg.0000154744.71184.c7.
Abstract/Text OBJECTIVES/HYPOTHESIS: The objective was to analyze the clinical data and outcome of all the patients treated surgically for squamous carcinoma of the temporal bone in a tertiary referral department of skull base surgery over a 20-year period.
STUDY DESIGN: Retrospective.
METHODS: Thirty-nine patients with squamous carcinoma of the temporal bone were analyzed. The patients were staged according to the University of Pittsburgh system. The surgical technique, reconstruction of the surgical defect, postoperative radiotherapeutic treatment, follow-up regimen, and results were all described in detail.
RESULTS: Disease-free survival for T2N0M0 tumors or stage II disease was 100%. The survival for stage III disease was also 100%. Overall, T3 tumors resulted in 50% survival. Overall, survival for stage IV tumors 34.3%, and for T4 tumors, 38%.
CONCLUSION: The overall disease-free survival for the entire series was 43.2%. Node-positive disease, poorly differentiated squamous cell histological findings, brain involvement, and salvage surgery were associated with a poorer outcome. The improved survival (66%) of patients treated de novo in the study series compared with those treated with salvage surgery (33%) suggested that early referral and aggressive primary surgical treatment with postoperative radiotherapy offer the greatest chance of cure.

PMID 15689763  Laryngoscope. 2005 Feb;115(2):341-7. doi: 10.1097/01.ml・・・
著者: Takashi Nakagawa, Yoshihiko Kumamoto, Yoshihiro Natori, Hideki Shiratsuchi, Satoshi Toh, Yasuhiro Kakazu, Shumei Shibata, Torahiko Nakashima, Shizuo Komune
雑誌名: Otol Neurotol. 2006 Feb;27(2):242-8; discussion 249. doi: 10.1097/01.mao.0000190463.88873.3d.
Abstract/Text OBJECTIVE: Treatment outcomes for squamous cell carcinoma of the temporal bone were evaluated regarding stage, therapeutic strategy, and prognostic factors.
STUDY DESIGN: Retrospective case review.
SETTING: University hospital and outpatient clinic.
PATIENTS: Twenty-five patients with primary squamous cell carcinoma of the external auditory canal and middle ear.
INTERVENTION: Preoperative chemoradiotherapy and radiotherapy were used in 7 of 12 patients. Lateral temporal bone resection was performed for the lesions not beyond the tympanic membrane. Subtotal temporal bone resection was chosen for lesions extending to the middle ear cavity when there was no invasion to the pyramidal apex, carotid canal, or dura or metastasis. Others were conservatively treated by chemoradiotherapy. When the performance status was poor or an agreement regarding the operation could not be reached, the treatment was modified.
MAIN OUTCOME MEASURE: Estimated survival rates.
RESULTS: The 3-year estimated survival for T1 and T2 lesions was 100%. The 5-year estimated survival for T3 and T4 lesions was 80% and 35%, respectively. The 5-year estimated survival improved up to 75% for T4 tumors with operation and 16% for those without operation after 47 months. The tumor-free surgical margin is significantly related to patient survival in T3 and T4 lesions. Multivariate analysis predicted that concomitant chronic otitis media and positive lymph nodes were significantly associated with poorer survival.
CONCLUSION: The tumor-free surgical margin was important to survival. When T4 lesions did not involve the pyramidal apex, carotid canal, dura, or any lymph nodes, the surgical intervention improved the estimated survival rate to a level as good as T3 lesions.

PMID 16436996  Otol Neurotol. 2006 Feb;27(2):242-8; discussion 249. do・・・
著者: Min Yin, Kazuo Ishikawa, Kouhei Honda, Takuya Arakawa, Yasuaki Harabuchi, Tatsumi Nagabashi, Satoshi Fukuda, Atsushi Taira, Tetsuo Himi, Narihiro Nakamura, Katuhiko Tanaka, Manabu Ichinohe, Hideichi Shinkawa, Yoshihiko Nakada, Hiroaki Sato, Kiyoto Shiga, Toshimitsu Kobayashi, Tomoo Watanabe, Masaru Aoyagi, Hiroshi Ogawa, Koichi Omori
雑誌名: Auris Nasus Larynx. 2006 Sep;33(3):251-7. doi: 10.1016/j.anl.2005.11.012. Epub 2006 Jan 20.
Abstract/Text OBJECTIVE: To analyze the clinical characteristics, 5-year survival, and prognostic factors of squamous cell carcinoma (SCC) of the external and middle ear.
METHODS: A multi institutional study. Ninety five cases of patients from 10 institutions were reviewed on their age and sex distribution, initial complaints, stages, tumor locations, treatments, and outcomes. Prognostic factors were discussed based on the Pittsburgh staging system.
RESULTS: This disease seems to appear in the elderly with a peak age of 50-69 years. Males appear to be more predisposed than females with an odd ratio of 1.7. The initial complaints were not typical, while 12.6% of patients presented a history of recurrent otitis externa or chronic otitis media. Regional metastasis was recognized in 13.7% of patients, while no distant metastasis was confirmed. SCC located in the external ear could be detected in an earlier stage than that in the middle ear. The overall 5-year survival was 66.8% in total, and decreased significantly with stage. SCC in stages I and II was susceptible to each therapeutic strategy with a 5-year survival of 100%. Operation combined with radiotherapy and/or chemotherapy was the major treatment for stages III and IV SCC, while radiotherapy and chemotherapy were applied mainly for those who had been considered inappropriate for operation. The overall survival was 67.2% for stage III and 29.5% for stage IV, and operation with pathologically tumor free margin could improve the survival to 72.7% when combined with radio- and chemotherapy. Stage, completeness of operation with tumor free margin, recurrence, and metastasis have significant influence on survival.
CONCLUSION: Local infiltration seems to be the main behavior of SCC in the external and middle ear. Early diagnosis and treatment were important because SCC in the earlier stage is susceptible to be cured. For tumors of advanced stage, operation should be performed with pathologically tumor free margin, and operation combined with radiotherapy and chemotherapy could improve the survival. Tumor stage adds more influence on survival than its location. Recurrence and metastasis mainly occur in advanced stages and result in a poor survival.

PMID 16431060  Auris Nasus Larynx. 2006 Sep;33(3):251-7. doi: 10.1016/・・・
著者: Seiji Hosokawa, Kunihiro Mizuta, Goro Takahashi, Jun Okamura, Yoshinori Takizawa, Kumiko Hosokawa, Takashi Yamatodani, Hiroyuki Mineta
雑誌名: Otol Neurotol. 2012 Apr;33(3):450-4. doi: 10.1097/MAO.0b013e318245ccbf.
Abstract/Text OBJECTIVE: Treatment outcomes for carcinomas of the external auditory canal (EAC) were evaluated regarding radiologic and pathologic factors.
STUDY DESIGN: Retrospective case review.
SETTING: University hospital.
PATIENTS: Fifteen patients histologically diagnosed with carcinomas of the EAC.
INTERVENTION: A radiologic and pathologic analysis was performed on these patients histologically diagnosed with carcinomas of the EAC and treated surgically at our institution. We evaluated the size of focal defects in the anteroinferior (AI) canal wall of the tympanic bone with preoperative computed tomographic (CT) scans. Histopathologic slides for the same patients were evaluated according to the same criteria as the CT scans.
MAIN OUTCOME MEASURE: Pathologic features and estimated survival rate.
RESULTS: Preoperative CT scans of 15 temporal bones demonstrated an AI canal wall defect ranging from less than 1 mm to full-thickness destruction. Six of 15 patients had an AI canal wall defect greater than 2 mm on preoperative CT scan. Pathologic findings in these 6 cases showed extension of the tumor through the AI defect into the anterior soft tissues. Information on patients' survival status was obtained after a median follow-up period of 78.3 months (range, 18-151 mo).
CONCLUSION: Preoperative CT can be used to accurately determine the pathologic extent of tumor invasion in carcinomas of the EAC. This diagnostic method facilitates exchange of accurate clinical data in a comparable form and can be used to evaluate the efficacy of existing and proposed treatments for EAC tumors.

PMID 22314922  Otol Neurotol. 2012 Apr;33(3):450-4. doi: 10.1097/MAO.0・・・
著者: Kiyoto Shiga, Takenori Ogawa, Atsuko Maki, Masanori Amano, Toshimitsu Kobayashi
雑誌名: Skull Base. 2011 May;21(3):153-8. doi: 10.1055/s-0031-1275244.
Abstract/Text We sought to characterize the effectiveness of concomitant chemoradiotherapy (CCRT) for patients with squamous cell carcinoma of the temporal bone. We performed a retrospective chart review of 14 patients with cancer of the temporal bone who were provided initial treatment in our hospital from December 2001 to November 2008. Four patients with stage I tumors were treated by radiation therapy alone or with oral administration of S1. One patient with a stage II tumor was treated by radiation therapy concomitant with low dose docetaxel. Nine patients with stage IV tumors were treated by CCRT using the TPF regimen (docetaxel, cisplatin, and 5-fluorouracil). As an initial treatment, all patients but one were treated by radiation therapy with or without chemotherapy. Grade 4 adverse events of patients who received CCRT using the TPF regimen involved the leukopenia in one patient and the neutropenia in two patients. Local recurrences were observed in three patients including two patients with T4 tumors. Five-year disease-specific survival rates for all patients and for patients with T4 tumors were 78% and 67%, respectively. CCRT using the TPF regimen is safe and effective as the first treatment for patients with cancer of the temporal bone.

PMID 22451818  Skull Base. 2011 May;21(3):153-8. doi: 10.1055/s-0031-1・・・
著者: M K Schwaber, M E Glasscock, A J Nissen, C G Jackson, P G Smith
雑誌名: Laryngoscope. 1984 Aug;94(8):1008-15.
Abstract/Text The clinical and diagnostic features of catecholamine secreting glomus tumors are reviewed. Three cases are reported, including the first documented case of a dopamine secreting glomus jugulare tumor. Based on this experience, the authors have outlined the indications for selective venous catheterterization studies and for pharmacologic blockage in the management of these patients. In addition to routine urinary screening, a high index of clinical suspicion is needed to avoid the complications associated with catecholamine secreting tumors.

PMID 6087052  Laryngoscope. 1984 Aug;94(8):1008-15.
著者: Tahl Y Colen, Frederick G Mihm, Theodore P Mason, Joseph B Roberson
雑誌名: Skull Base. 2009 Nov;19(6):377-85. doi: 10.1055/s-0029-1224771.
Abstract/Text Catecholamine-secreting paragangliomas (CSPs) present challenges for the managing team of surgeons and anesthesiologists. Without proper preoperative management and planning, the patient is at high risk for complications and significant morbidity. A review of the literature looking at all aspects of the care of patients with CSP was performed to provide a consensus on the comprehensive care of these difficult patients. A case study is also provided to illustrate the management algorithm. Specific recommendations are made with regards to preoperative workup, including serum and urine testing, tumor localization, angiography, and embolization. Preoperative and intraoperative management techniques by the surgical and anesthesiology teams are discussed, including pharmaceutical interventions and fluid management. Aspects of postoperative care are also discussed. Management of patients with CSP requires significant attention to detail by a multidisciplinary team of surgeons and anesthesiologists. By following the recommendations included within this article, the morbidity associated with removal of these tumors can be significantly reduced or eliminated.

PMID 20436839  Skull Base. 2009 Nov;19(6):377-85. doi: 10.1055/s-0029-・・・

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