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Management of benign middle ear tumors: A series of 7 cases.

Abstract

Benign middle ear tumors represent a rare group of neoplasms that vary widely in their pathology, anatomy, and clinical findings. These factors have made it difficult to establish guidelines for the resection of such tumors. Here we present 7 unique cases of these rare and diverse tumors and draw from our experience to recommend optimal surgical management. Based on our experience, a postauricular incision is necessary in nearly all cases. Mastoidectomy is required for tumors that extend into the mastoid cavity. Whenever exposure or hemostasis is believed to be inadequate with simple mastoidectomy, canal-wall-down mastoidectomy should be performed. Finally, disarticulation of the ossicular chain greatly facilitates tumor excision and should be performed early in the procedure.

Introduction

Middle ear tumors constitute a diverse group of rare neoplasms that exhibit great variation in pathology, anatomy, and clinical manifestation. They may be broadly classified into two groups: (1) primary tumors of the middle ear and (2) tumors of adjacent structures that involve the middle ear space. The most common primary tumors are paragangliomas (glomus tumors), followed by middle ear adenomas in adults and by hemangiomas in children. Primary tumors may extend from the middle ear space intracranially or into other portions of the temporal bone or skull base. (1) Benign tumors of adjacent structures may invade the middle ear space; these tumors include vestibular and facial nerve schwannomas, temporal meningiomas, and parotid gland tumors.

Computed tomography (CT) and magnetic resonance imaging (MRI) are useful in defining the extent and nature of these lesions, but histologic analysis is often necessary to reach a definitive diagnosis. (2) Surgical excision is usually the recommended treatment.

In this article, we describe a case series of these rare, diverse, and anatomically complex tumors, and we discuss their optimal surgical management.

Case series

We retrospectively selected 7 representative cases of middle ear tumors that were treated at the Columbia University Medical Center in 2013 and 2014. They were chosen for representing various clinical manifestations, pathologies, and extents of regional involvement. Histopathologic descriptions for each case were provided by a pathologist upon reexamination of fixed sections from archival files for the purpose of this report.

Approval for this review was obtained from the Institutional Review Board of Columbia University.

The management of our 7 cases is summarized in the table.

Patient 1: Neuroendocrine adenoma. A 22-yearold man underwent a brain MRI during a seizure workup. Imaging incidentally revealed the presence of enhancing soft tissue in the right middle ear and mastoid cavity.

Surgery was performed via a transmastoid/transcanal approach. Intraoperatively, a tumor was found to occupy the entire epitympanum and mesotympanum with extension into the hypotympanum. The mass had encased the entire ossicular chain. The incus and malleus were removed to facilitate tumor excision. A laser was used to dissect the tumor off the stapes, and a near-total resection was achieved, leaving behind only a small fragment within the stapedial arch. The ossicular chain was reconstructed in a primary fashion.

Microscopic analysis identified the lesion as a neuroendocrine adenoma (figure 1).

Patient 2: Jugulotympanic paraganglioma. A 64-year-old woman presented with worsening left-sided hearing loss and persistent pulsatile tinnitus. The patient had previously undergone resection of a left jugulotympanic paraganglioma (glomus tympanicum). At this most recent presentation, she underwent cerebral angiography for a presumed recurrence of her paraganglioma, and the suspicion was confirmed. The tumor featured a dual blood supply.

Preoperatively, the left middle meningeal artery was embolized, but the transosseous branch of the occipital artery was not because the patient developed transient facial nerve palsy upon vasoconstriction. The tumor resection began via a transmastoid/ transcanal approach, but it was converted to a canal-wall-down mastoidectomy in view of the size of the remaining tumor and the difficulty in achieving hemostasis. In this way, the tumor, which had occupied the entire middle ear space and protruded into the eustachian tube, was completely removed along with the malleus and a previously placed prosthesis that it had encased. The ossicular chain reconstruction and tympanoplasty were performed in a primary fashion.

Microscopic analysis confirmed the lesion as a jugulotympanic paraganglioma (figure 2).

Patient 3: Hemangioma. A 16-year-old boypresented with left-sided hearing loss. On otoscopic examination, the tympanic membrane appeared quite red, and a vascular mass was visible behind it. CT and MRI identified an avidly enhancing mass that occupied the entire middle ear space and mastoid cavity and extended into the external auditory canal.

The initial surgery consisted of a mastoidectomy and biopsy. The tumor bled significantly, so the decision was made to attempt arterial embolization before further resection. However, vasoconstriction of the dominant feeder resulted in complete facial nerve palsy, so embolization was not performed. During the resection, the canal wall was taken down so that hemostasis could be better achieved.

The tumor was adherent to the tympanic membrane, which was removed along with the incus and malleus. A small fragment of tumor that adhered to the stapes superstructure and the second genu of the facial nerve was left in place. The ossicular chain was reconstructed in a primary fashion. Estimated blood loss from the procedure was 100 ml.

Microscopic analysis showed a hemangioma with numerous small slit-like vascular spaces lined with endothelial cells (figure 3).

Patient 4: Pleomorphic adenoma. A 46-year-old man presented with progressive left-sided hearing loss. CT of the left ear revealed a soft-tissue mass within the middle ear that extended into the external auditory canal. Opacification of the mastoid air cells was observed.

The tumor was exposed via a postauricular transcanal approach. Tumor occupied the entire canal and pushed the tympanic membrane onto the promontory. The membrane itself was atelectatic. The tumor was completely resected.

On microscopy, the lesion was identified as a pleomorphic adenoma (figure 4).

Patient 5: Facial nerve schwannoma. A 60-year-old woman was referred from the internal medicine service after she had been admitted for a syncopal episode that was preceded by acute vertigo. She also had a several-month history of left-sided hearing loss and otalgia. MRI of the brain revealed the presence of an enhancing mastoid mass that extended into the external auditory and facial canals.

Surgical exploration identified a large tumor that involved the mastoid air cells from the mastoid antrum all the way up to the stylomastoid foramen. The entire tumor was decompressed within the mastoid cavity up to the mastoid tip. The facial nerve exhibited normal function as evidenced by stimulation; thresholds were maintained throughout the entire decompression. The decision was made to observe the patient rather than excise the tumor because excision would have led to complete facial paralysis.

Microscopic analysis identified the tumor as facial nerve schwannoma (figure 5).

Patient 6. Meningioma. A 41-year-old woman presented with a history of progressive left-sided hearing loss and vertigo. Brain MRI detected an enhancing mass on the inferior aspect of the left temporal lobe that extended into the middle ear. These findings were suggestive of a meningioma.

A two-stage surgery began with a neurosurgeon performing an intercranial resection through a middle fossa craniotomy. Next, the middle ear resection was begun by extending the incision postauricularly to facilitate a transmastoid/transcanal approach. The tumor encased the incus and malleus, which were removed along with the tumor. Six months later, the ossicular chain was reconstructed in a secondary fashion.

Microscopy confirmed the lesion as a meningioma (figure 6).

Patient 7. Non-neoplastic refractile middle ear tumor. An 8-year-old boy with a chromosomal anomaly (2q13 and 3p24 deletions) presented with bloody otor-rhea. A dedicated left temporal bone CT demonstrated soft tissue in the mesotympanum.

The patient underwent surgery for a presumed cholesteatoma. A postauricular incision was made to facilitate a transcanal resection. Intraoperatively, the middle ear tumor was whitish, firm, malleable, and rubbery. It appeared to arise from a central area of the tympanic membrane, and it extended underneath the malleus into the anterior mesotympanum. The mass was completely excised with a laser, with preservation of the chorda tympani nerve and ossicular chain.

Histology identified refractile material of unknown significance (figure 7).

Follow-up. At 2 years postoperatively, no patient exhibited any sign of recurrence.

Discussion

Middle ear tumors are rare and are best evaluated with imaging studies. (3) Surgical excision with histologic analysis is usually required for definitive diagnosis and treatment. To effect a complete resection, optimization of exposure to define the circumferential tumor margins is critical before proceeding with a piecemeal resection. (4,5)

Traditional transcanal approaches should be reserved for small, discrete masses in the mesotympanum. A postauricular incision is more appropriate for excising small, discrete lesions of the anterior mesotympanum or when mastoidectomy is anticipated for tumors that extend to the epitympanum, hypotympanum, or outside the middle ear space.

For tumors that involve the mastoid cavity, canal-wall-up mastoidectomy is preferred in uncomplicated resections. However, there should be little hesitation to convert it to a canal-wall-down procedure to achieve better exposure and hemostasis. In cases of recurrence, the canal-wall-down procedure has the added advantage of making invasion into deeper structures less likely. (6)

Finally, a complete and safe resection of a middle ear tumor often requires disarticulating the ossicular chain, and surgeons should have a low threshold for doing so. Ossicular chain reconstruction can be performed in the same procedure following tumor resection.

References

(1.) Razek AA, Huang BY. Lesions of the petrous apex: Classification and findings at CT and MR imaging. Radiographics 2012;32(1): 151-73.

(2.) Isenring D, Pezier TF, Vrugt B, Huber AM. Middle ear adenoma: Case report and discussion. Case Rep Otolaryngol 2014;2014: 342125.

(3.) Zan E, Limb CJ, Koehler fF, Yousem DM. Middle ear adenoma: A challenging diagnosis. AJNR Am J Neuroradiol 2009;30(8):1602-3.

(4.) Marsh M, Jenkins H. Temporal bone neoplasms and lateral cranial base surgery. In: Flint PW, Haughey BH, Lund VJ, et al, eds. Cummings Otolaryngology Head & Neck Surgery. 5th ed. Philadelphia: Mosby Elsevier; 2010.

(5.) Sanna M, Fois P, Pasanisi E, et al. Middle ear and mastoid glomus tumors (glomus tympanicum): An algorithm for the surgical management. Auris Nasus Larynx 2010;37(6):661-8.

(6.) Walker PC, Mowry SE, Hansen MR, Gantz BJ. Long-term results of canal wall reconstruction tympanomastoidectomy. Otol Neurotol 2014;35(6):954-60. Z. Jason Qian, MD; Amy M. Coffey, MD; Kathleen M. O'Toole, MD; Anil K. Lalwani, MD

From the Department of Otolaryngology-Head and Neck Surgery, Stanford University School of Medicine, Stanford, Calif. (Dr. Qian); and the Department of Pathology (Dr. Coffey and Dr. O'Toole) and the Division of Otology, Neurotology, and Skull Base Surgery, Department of Otolaryngology-Head and Neck Surgery (Dr. Lalwani), Columbia University College of Physicians and Surgeons, New York City. The cases described in this article occurred at the Columbia University Medical Center.

Corresponding author: Anil K. Lalwani, MD, Division of Otology, Neurotology, and Skull Base Surgery, Department of Otolaryngology-Head and Neck Surgery, Columbia University College of Physicians and Surgeons, 180 Fort Washington Ave., Harkness Pavilion HP818, New York, NY 10032. Email: anil.lalwani@columbia.edu

Caption: Figure 1. Patient 1. Histopathology shows a cellular neoplasm with a varied growth pattern consisting of organoid, glandular, trabecular, and ribbon-like areas arranged in a collagenous stroma. The cells are uniformly round to oval with stippled chromatin and relatively abundant eosinophilic cytoplasm, consistent with a neuroendocrine adenoma (hematoxylin and eosin, original magnification x20).

Caption: Figure 2. Patient 2. In the jugulotympanic paraganglioma, nests and trabeculae are composed of small to medium-sized cells with ovoid nuclei and abundant pink cytoplasm within a rich vascular network, characteristic of the Zellballen pattern (hematoxylin and eosin, original magnification x20).

Caption: Figure 3. Patient 3. The hemangioma is composed of numerous small, slit-like vascular spaces lined with endothelial cells (CD34 immunohistochemistry, original magnification x4).

Caption: Figure 4. Patient 4. A: On hematoxylin and eosin staining the pleomorphic adenoma contains islands of hyaline to myxoid cartilage admixed with tubules, glands, and cords composed of uniform epithelial cells (original magnification x4). B: On p63 immunohistochemistry, the myoepithelial cell nuclei stain brown (x20).

Caption: Figure 5. Patient 5. The schwannoma is composed of bland spindle cells and is diffusely positive for S-100 protein (hematoxylin and eosin, original magnification x10).

Caption: Figure 3. Patient 3. The hemangioma is composed of numerous small, slit-like vascular spaces lined with endothelial cells (CD34 immunohistochemistry, original magnification x4).

Caption: Figure 4. Patient 4. A: On hematoxylin and eosin staining the pleomorphic adenoma contains islands of hyaline to myxoid cartilage admixed with tubules, glands, and cords composed of uniform epithelial cells (original magnification x4). B: On p63 immunohistochemistry, the myoepithelial cell nuclei stain brown (x20).

Caption: Figure 5. Patient 5. The schwannoma is composed of bland spindle cells and is diffusely positive for S-100 protein (hematoxylin and eosin, original magnification x10).

Caption: Figure 6. Patient 6. The meningioma is composed of whorled clusters of spindled epithelioid cells (hematoxylin and eosin, original magnification x20).

Caption: Figure 7. Patient 7. Collections of refractile crystals are observed under polarized light. There is no evidence of neoplasia (hematoxylin and eosin, original magnification x20).
Table. Summary of the 7 cases

                                   Time of
Pt.   Age/sex   Diagnosis          diagnosis

1      22/M     Neuroendocrine     Intraoperatively
                adenoma

2      64/F     Jugulotympanic     Preoperatively
                paraganglioma

3      16/M     Flemangioma        Preoperatively

4      46/M     Pleomorphic        Intraoperatively
                adenoma

5      60/F     Facial nerve       Intraoperatively
                schwannoma

6      41/F     Meningioma         Preoperatively

7       8/M     Non-neoplastic     Intraoperatively
                foreign material

                       Canal   Structures
Pt.   IHC              wall    removed           OC reconstruction

1     Synaptophysin,    Up     Incus, malleus    Primary
      CD56, pancyt-
      okeratin, CK7
      CK5

2     Synaptophysin,   Down    Malleus,          Primary
      S-100 protein,           previously
      chromogranin             placed
                               prosthesis

3     CD31, CD34       Down    Incus, malleus,   Primary
                               tympanic
                               membrane

4     p63, SMA          N/A    None              N/A

5     S-100 protein     Up     None              N/A

6     EMA, PR           Up     Incus, malleus    Secondary

7     N/A               Up     None              N/A

Key: IHC = immunohistochemistry; OC = ossicular chain;
SMA = smooth-muscle actin; EMA = epithelial membrane
antigen; PR = progesterone receptor; N/A = not applicable.
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Article Details
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Title Annotation:ORIGINAL ARTICLE
Author:Qian, Z. Jason; Coffey, Amy M.; O'Toole, Kathleen M.; Lalwani, Anil K.
Publication:Ear, Nose and Throat Journal
Article Type:Report
Geographic Code:1USA
Date:Oct 1, 2017
Words:2291
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