Portuguese Version

Year:  1974  Vol. 40   Ed. 2 - (21º)

Artigos Originais

Pages: 198 to 199

Rhabdomyosarcoma of the Middle Ear

Author(s): Silvia Bolliger e
George Bernarde

Rhabdomyosarcoma in childhood is an uncommon tumor which rarely originates in the temporal bone but could initially presents as a suppurative painless middle otitis. In regard of diagnosis we should consider the lack of symptoms and the abundance of signs as painless ear discharge, granulation tissue in the external canal, peripheral facial paralysis, cervical adenopathy and extensive destructive bone lesions on X-ray films not proporcional to the short course of the disease Symptoms would be restricted to mild local or regional pain, if present, because loss of hearing is never referred by the child patient. Rhabdomyosarcoma recapitulares the normal process of skeletal muscle development from the primitive round cell to the multinucleated muscle cell with characteristic striations only as a disorganized process. Many classifications were suggested but the most accepted one was proposed by Horn and Enterline based on the histopathological features:

1. PLEOMORPHIC TYPE: incidence in adults; basically a tumor of spindle cells, differenciated from the fibrosarcoma by its cellularity and pleomorphism; cells present themselves with longitudinal striations or myofibrils.

2. ALVEOLAR TYPE: appears in adolescents and young adults; similar to an adenocarcinoma, characterized by connective tissue trabeculae among cells either giant multinucleated cells or oval cells; presence of several free-floating cells.

3. EMBRYONIC TYPE: strap-like cells usually with one central nucleus, these cells are wide in one extremity similarly to a tadpole; cross striations are present.

4. BOTRYOID TYPE: as the anterior type, occurs in children; its characteristic feature is given by a pavimentouos, i.e., a multilayer ed strip of indifferenciated cells localized parallel to the tumor surface.

CASE REPORT: M.A.O., a five-year-old girl was admitted in July 1973, with a history of right facial paralysis of three months' duration associated with mild pain and foul-smelling discharge of the right ear. Twenty days prior to admission, a tender swelling appeared posterior to the right ear and also a hard swelling in the parotid region. The child had been treated with antibiotics and a previous drainage of the posterior swelling was tried by the family physician. Mastoid X-ray films showed destruction of the whole mastoid portion of the temporal bone. Biopsy of the ulcerative mass posterior to the ear was reported as rhabdomyosarcoma of the middle ear embryonic type. After radiotherapy in massive dosage no improvement was obtained.

One month later, an X-ray skeletal survey showed bilateral destructive lesions localized in the femural and fibular metaphises. The patient ran a rapid downhill course and expired two months following admission.

The therapy of this tumor should Se installed as soon as possible. A radical excision of the temporal bone is advisable when still feasible associated with facial and neck ganglionarremoval followed by radiotherapy and chemotherapy. Some authors obtained survivals ranging from 34 to 54 months in 5 out of 73 cases reported in the world literature. As we see, precocious diagnosis is essentially necessary to reach a longer term survival with the therapeutical tools actually available.

Summary

The authors present one case of rhabdomyosarcoma of the middle ear in an attempt to aware the otolaryngologists of conditions such as' a discharging ear with absence of pain, a peripheral facial paralysis, presence of polyps in the external canal, destruction of the bony trabeculae or cloudiness of the mastoid associated with mild shortstanding symptoms when this type of tumor should be suspected. Although therapy is usually unsuccessful an early diagnosis and an appropriated management will achieve a longer term survival.

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