|Year : 2011 | Volume
| Issue : 4 | Page : 173-175
Myxoma of the temporal bone: A rare neoplasm
Kapil Sikka1, Rajeev Kumar1, Rakesh Kumar1, Prem Sagar1, Lavleen Singh2
1 Department of ENT, All India Institute of Medical Sciences, New Delhi, India
2 Department of Pathology, All India Institute of Medical Sciences, New Delhi, India
|Date of Web Publication||29-Mar-2012|
Room No. 4057, ENT Office, 4th Floor, Teaching Block, AIIMS, Ansari Nagar, New Delhi - 110 029
Source of Support: None, Conflict of Interest: None
Myxomas of the temporal bone are rare mesenchymal tumors. We present a case of a 16-year-old girl who presented with complaint of mass lesion protruding from the right ear canal and from behind the ear for last 2 years. Biopsy of the mass was suggestive of benign mesenchymal lesion with prominent myxoid changes. Radiology of the temporal bone showed soft tissue density involving right side external auditory canal, middle ear, mastoid antrum and both lobes of the parotid gland. Tumour excision along with total parotidectomy was performed. Postoperative histopatholgy was consistent with myxoma. The patient is on regular follow-up for last 5months with no recurrence till date. Temporal bone myxomas are rare neoplasms which are surgically treatable but have a strong tendency to recur, necessiating long-term follow-up.
Keywords: Benign temporal bone tumour, Myxoid tumors, Temporal bone
|How to cite this article:|
Sikka K, Kumar R, Kumar R, Sagar P, Singh L. Myxoma of the temporal bone: A rare neoplasm. Indian J Otol 2011;17:173-5
| Introduction|| |
Myxoid tumors are a rare cause of mass lesion of the temporal bone. These benign tumors are generally slow growing, with a tendency to infiltrate the surrounding structures. The clinical presentation may mimic a case of chronic suppurative otitis media (CSOM); although, on contrast-enhanced computed tomography (CECT), these tumors are enhancing in nature. Radical surgery with wide margins is the treatment of choice, although these tumors have a tendency to recur. We discuss about a young patient who was diagnosed to have myxoma of the temporal bone and the management issues.
| Case Report|| |
A 16-year-old girl presented to the outpatient department with complaint of mass lesion in the right ear canal for 2 years, which was insidious in onset and progressive. It was associated with ear pain, purulent ear discharge, and hearing loss; although there was no history of ear bleed or facial weakness. For last 1 year, there was another slow-growing mass in the postauricular region. On local examination, there was a pinkish, mucosa-covered mass protruding from right external auditory canal (EAC) [Figure 1]a. Another lesion of approximately 5 cm × 4 cm was present in the postauricular region. It was mucosa covered and had a bosselated surface [Figure 1]b. Alongside, an infraauricular ulcerated (2 cm × 2 cm) lesion was present. There was no lymphadenopathy. The facial nerve was normal on both sides. A biopsy of the canal mass was performed under local anesthesia for histopathology, which showed benign mesenchymal lesion with prominent myxoid changes. Radiology was performed to know the disease extent and to plan the surgical approach. CECT of the temporal bone revealed enhancing mass in the retroauricular region which was involving both lobes of the parotid gland. The bony EAC was expanded as well as partially eroded by the mass lesion. The middle ear and mastoid antrum was filled with the mass. The Fallopian canal More Details and cochlea were uninvolved. There was thinning of the inferior portion of the temporomandibular joint.
The patient was taken up for tumor excision by the lateral skull base approach. The tumor was removed from the mastoid, middle ear, and EAC. Total parotidectomy was done along with sacrifice of the upper facial nerve branches, as they were involved by the tumor. Multiple lymph nodes were present in level IIa/ IIb, which were removed and sent for histopathology.
Postoperative histopathology showed tumor with spindle-to-stellate cells in abundant myxoid stroma, forming vague lobules separated by fibrovascular spaces [Figure 2]a. Tumor lobules showed presence of thin walled vascular channel. There was mixed inflammatory infiltrate comprising of neutrophils, plasma cells, and lymphocytes. Tumor cells were immunopositive for CD34 but immunonegative for SMA, S100, desmin, and cytokeratin [Figure 2]b. Overall, the histomorphological and immunohistochemical features were in favor of superficial angiomyxoma.
|Figure 2: (a) Low magnification (40X) shows a vaguely multilobulated tumor with abundant myxoid stroma and prominent arborizing vasculature. (b) Tumor cells are immunopositive for CD34 while immunonegative for pancytokeratin, S100 and SMA|
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Postoperatively, the patient is on rgular follow-up for last 5months, with no disease recurrence till date.
| Discussion|| |
Myxomas are rare benign tumors arising from the skin, subcutaneous tissues, skeletal muscles, heart, and genitourinary system. These tumors are mesenchymal in origin. They are generally classified into five types: cutaneous (superficial angiomyxoma), intramuscular, juxta-articular, nerve sheath, and aggressive angiomyxoma. , The most common site of occurrence is within the myocardium.  Myxomas of the head and neck region are rare, occurring more commonly in bones than in soft tissues.  Literature has reported less than 170 cases in the head and neck region until 2004.  However, myxomas arising in the temporal bone are quite rare, and to the best of our knowledge, only 12 cases have been reported so far. 
The etiology of myxomas occurring in the head and neck region remains unclear, although it has been suggested that head and neck myxomas arise from the tooth germ cells, because of their common presence in the maxillary and mandibular region.  However, Kleinsasser suggested that the primitive mesenchyme filling the middle ear space in the embryo and in the newborn may give rise to temporal bone myxomas. 
The presentation of temporal bone myxomas is variable, but clinically, it mimics CSOM. Patients may present with recurrent otitis media, facial nerve paralysis, mass in the external auditory meatus, or retroauricular mass. These benign tumors are, however, locally aggressive and can cause significant destruction of the adjacent structures. These tumors are generally slow growing but infiltrative in nature. Radiology will generally show enhancing mass lesion in the temporal bone with infiltration into surrounding structures like parotid gland, temporomandibular joint, and soft tissues of the neck. These tumors are difficult to diagnose histopathologically and need immunohistochemistry for proper evaluation. The differential diagnosis of mass lesion in the temporal bone includes fibrous dysplasia, chondromyxoid fibroma, fibrosarcoma, chondrosarcoma, schwannoma, giant cell granuloma, chondroma, and hemangioma of the bone. However, as per Stout's definition of a true myxoma, it is a true mesenchymal neoplasm consisting of undifferentiated stellate cells in a loose myxoid stroma, and its diagnosis excludes the presence of lipoblast, myoblast, chondroblast, or other elements. 
The management of these rare tumors is mainly surgical excision with wide healthy soft tissue margins. Myxomas has a strong tendency to recur, especially in the bones.  Therefore, in temporal bone myxomas, where margins cannot be achieved, drilling needs to be adequate. The need for radical surgery must be evaluated with regard to facial nerve and inner ear function, especially in young children. It has been advocated by Windfuhr and Schwerdtfeger  that in temporal bone myxomas, en bloc excision with wide margins is not possible in view of ill-defined margins of tumor and complex anatomy of temporal bone. Therefore, it is wise to operate thoroughly but without sacrificing vital structures. Regular follow-up is required, both clinically and radiologically, to detect recurrence early.
| Conclusion|| |
Myxomas of the temporal bone are rare mesenchymal tumors, which are surgically treatable but have strong tendency to recur. These tumors need to be kept in the differential diagnosis of mass lesion presenting in the temporal bone.
| References|| |
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[Figure 1], [Figure 2]
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