Embryonal rhabdomyosarcoma of the middle ear

Abstract

Embryonal rhabdomyosarcoma of the middle ear, as previously reported in the literature, is an almost uniformly lethal entity. An unusual case presenting with bone destruction and later demonstrating a metastatic locus has achieved a relatively long‐term NED survival status. Adequate radiotherapy, when combined with both concomitant and then maintenance chemotherapy, may not only control the local tumor but delay, prevent, or treat peripheral macroscopic or microscopic disease.

Rhabdomyosarcama of the middle ear and mastoid

“…McNeer et al (1968), in an extensive review of 653 cases of soft tissue sarcomas of all types, showed that radiotherapy may markedly improve survival periods of patients with such tumouis. Edland (1965) and Nelson (1968) regarded embryonal rhabdomyosarcoma as a tumour of moderate radiosensitivity. Conte and Sagerman (1971) recommended aggressive irradition once the tumour is diagnosed.…”

Soft tissue sarcomas of the extremities: Their natural history, treatment, and radiation sensitivity

“…However, useful dose-time fraction data is scarce. Low dose control (less than 6,000 rads) of minimal disease has been documented by many authors (8,10,13,15,20,21,25,27,30). Table I11 demonstrates that for all soft tissue sarcomas, the better cure rates are for microscopic disease and doses above 6-7,000 rads (10, 12, 15-17, 23,25,29-31,34).…”

“…planes and muscular compartments often recur with local excision alone. Tumors arising in the buttocks and shoulder girdle often recur even with hemipelvectomy or shoulder disarticulation (16,25). sarcomas often eventually require amputation (1,11,13,18,23).…”

“…Although Suit et al (1 5 ) claim to have many patients with functional extremities, the inadvisability of delivering 6-7,000 rads in consecutive fractions to large volumes is well known (12, 15, 17, 19, 3 1-33). Hesitance concerning delivering intensive irradiations to hands, feet, achilles tendon, joints, and patella have been voiced by many investigators (12,15,23,25,32). Certainly, if one must limit his radiations to less than the full circumference and length of the involved extremity (avoiding the joints and including the scar) the number of patients after wide excision that fulfill these criteria is select.…”

Rhabdomyosarcoma

“…1965'de Edland ilk kez tam cerrahi rezeksiyon yapılamayan bir olguda yüksek doz RT kullanmıştır (4) . Sonrasında adjuvan kemoterapinin (KT) tedaviye eklenmesi ile RMS'da çok-lu tedavi yaklaşımı oluşturulmuş ve RMS ile ilgili geniş verilere ulaşılmıştır.…”