Rhabdomyosarcama of the middle ear and mastoid By A. L. PAHOR (Birmingham) RHABDOMYOSARCOMA of the middle ear is a malignant tumour of childhood, usually associated with a very poor prognosis. Recent reports give a more hopeful outlook in terms of local control and possibly long term survival. It is a rare tumour; up to date 71 well-documented cases have been reported in the English literature (See References). In this paper a further case is reported.

Case History H.M., a white female child, date-of-birth 8.2.1963, attended the ENT Department of the Royal Victoria Hospital, Belfast, on 6.2.1967. Her complaint was of pain in the left ear for the previous three weeks for which she had received antibiotics and ear drops. On examination she was well nourished, quiet and rather apathetic; her weight was 31 pounds. The left external auditory meatus was full of smelly discharge and granulations arising from the anterior and posterior meatal walls. There was a hard indurated area below and behind the ear and the post-auricular sulcus was obliterated. A small lymph node was palpable below the ear. Throat and nose examinations were normal. There was definite weakness of the left side of the face. X-ray examination of the chest was normal as were films of the right mastoid. Radiograms of the left mastoid showed appearances suggestive of early mastoiditis; the base of the skull showed no evidence of bone erosion. A swab taken from the left ear showed coliforms; no acid fast bacilli were seen in stained films nor cultured on special media. Blood examination showed Hb to be 12-8 gm. per cent, W.B.C. count was io-8oo/cmm., with a differential count of: Neutrophils (mature) 56 per cent, Lymphocytes 42 per cent, Monocytes 2 per cent and the Platelets were plentiful in the film. E.U.A. of the left ear was carried out on 13.2.1967. Bare bone was seen in the depths of the meatus anteriorly and the tympanic membrane was found to be disorganized. A biopsy was taken and histological examination of the specimen showed a rhabdomyosarcoma. It was decided that treatment should be a combination of irradiation and chemotherapy using Actinomycin D. Treatment was started on 3.3.1967 in the Northern Ireland Radiotherapy Centre. Cobalt radiotherapy, with a tumour dose of 4,75or was given over 34 days. A dose of 75 micrograms per KBW of Actinomycin D was given intravenously half an hour before each treatment with radiotherapy. The patient was discharged on 6.4.1967 and no maintenance therapy was given. Her W.B.C. count was then 6,100. At review on 3.5.1967 her general condition was found to be reasonably good. The mass in the left ear had disappeared, but there was crusting in the external meatus. The patient was re-admitted on 2.6.1967. Her Hb was 13 -4 gm. per cent 585

A. L. Pahor and W.B.C. count was 8,100. She was discharged on 9.6.1967 and died on 25.6.1967, presumably as a direct result of the tumour or its complications. No post-mortem examination was obtained. The specimen submitted to pathology consisted of several small pieces of tissue. Histological examination showed mild hyperplasia of the overlying epithelium and revealed the presence of an undifferentiated sarcoma, with marked variation in the cellular appearance (Figs. 1 and 2). The predominant cells were spindle-shaped cells with oval centrally placed nuclei, and the cells

FIG. 1. Cellular Pleomorphism: spindle-shaped, round and tadpole cells. (H. & E.).

showed in areas a tendency to arrange themselves in groups. Round cells were seen which predominate in some fields. Tadpole cells and others with bipolar cytoplasmic processes were encountered, and giant cells were occasionally seen. A few cells showed transverse striations in sections stained by H. & E. (Fig. 2) and special staining with phosphotungstic acid-haematoxylin showed the same (Fig- 3)Discussion Horn and Enterline (1958) classified rhabdomyosarcomas on histological grounds into four types: Pleomorphic, Alveolar, Embryonal and Sarcoma botryoides. Most authors classify them into three clinopathologic varieties: Pleomorphic, Alveolar and Embryonal. However, Friedmann (1974) adopts the classification proposed by Ashton and Morgan (1965) which depends on the degree of differentiation of the tumour cells. The World Health Organization has divided rhabdomyosarcomas into four histological categories: Embryonal, Alveolar, Pleomorphic and mixed varieties 586

Clinical records

FIG. 2. Cellular Pleomorphism: round, spindle-shaped, tadpole and bipolar cells. Transverse striations are also seen (H. & E.).

FIG.

3.

Myoblasts with transverse striations (PTAH).

587

A. L. Pahor (Enzinger, Lattes and Torloni, 1969). Sarcoma botryoides is regarded as a variant of the embryonal type, showing oedematous multiple polypoid grape-like mucinous structures, with a concommitant change in the microscopic pattern. The different variants of rhabdomyosarcomas mimic the stages in the development of striated muscle (Cappel and Montgomery, 1937; Patton and Horn, 1962). The embryonal and alveolar types represent early embryonal growth of striated muscle and usually do not show cross striations. The former type is mostly found in children and is located in the head and neck; the latter type is encountered most commonly in the extremities, and to a lesser extent in the head and neck of children and young adults. The more mature pleomorphic variety represents a de-differentiation of adult muscle cells and usually exhibits striations and it occurs mostly in the extremities of middle-aged adults. Most ihabdomyosarcomas reported in the middle ear are of the embryonal and botryoides types. The tissue of origin of rhabdomyosarcomas is derived either from totipotential mesenchymal cells (Evans, 1966), or from immature myoblastic tissue (Willis, 1967), or from the muscles of the middle ear (Russell and Rubenstein, 1959). The characteristic histologic featuie is the presence of embryonal myoblasts which may display striations. These striations are actomyosin myofibrils that can be seen under the light microscope, but in some cases are seen only on electron microscopy (Williams, Martinson and Alii, 1968). Clinically the tumour presents initially as chronic suppurative otitis media, mainly with blood stained auial discharge and a polypus or granulations in the external auditory meatus. Cranial nerve involvement is common, particularly the Vllth cranial nerve. Intracranial extension is also a common finding. Cervical lymph node metastases may be present. The lungs and bones are the commonest sites for distant metastases. Energetic therapy with radical surgery, regional irradiation and systemic chemotherapy may materially increase the prospects of survival for rhabdomyosarcomas in general (Grosfeld, Clatworthy and Newton, 1969; Donaldson et al., 1973). When these principles are applied to cases arising in the middle ear, ladical excision beyond the boundaries of the tumour is impractical as the tumour has a great tendency for destruction of bone and extension into the neck, infratemporal fossa, paraphaiyngeal space and intracranially. This led some authors to recommend irradiation and chemotherapy alone (Jaffe et al., 1971). 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. The potentiation of the X-ray effects by Actinomycin D was demonstrated and such a combination was used in the treatment of a variety of neoplasms, including rhabdomyosarcomas, with encouraging results (Farber, 1958; D'Angio, Farber and Maddock, 1959). Pinkel and Pickren (1961) reported a case of rhabdomyosarcoma of the middle ear extending to the nasopharynx treated by irradiation and Actinomycin D; the patient survived for six months. 588

Clinical records In reviewing recent papers, there is a trend for treatment with aggressive irradiation (6,ooor or more tumour dose) combined with systemic chemotherapy; the drugs generally used are Actinomycin D, Vincristine, Cyclophosphamide and Dactinomycin. Usually two or three of these chemotherapeutic agents are used in combination with a maintenance therapy over a period of six to thirty-six months (Ragab et al., 1972; Cunningham and Kung, 1972; Fish, Koch and Canales, 1972; Donaldson et al., 1973; Webb and McFarland, 1973). The combination of irradiation and chemotherapy seems also to control the boney involvement by the tumour (Donaldson et al., 1973). On the other hand, the role of surgery in achieving long term survival is not clearly shown. Control of the tumour is reported in cases which had preradiation surgery (Ragab et al., 1972; Cunningham and Kung, 1972; Webb and McFarland, 1973; Sessions et al., 1973), whereas other cases which did not have surgery did equally well (Edland, 1972; Fish et al., 1972). Sessions et al. (1973) recommended pre-radiation wide local resection whenever possible. The tumour was considered lethal. Jaffe et al. (1971) showed that the mean survival period was 7 • 4 months from the time of diagnosis. The recent trends in treatment led to a more hopeful prognosis and longer survival periods have been reported. The longest survival so far is 12 years in a case reported by Barnes and Maxwell (1972). The case presented is of the embryonal variety and displays such histological features as described by Stobbe and Dargeon (1950). The patient was treated by radiotherapy and Actinomycin D. This treatment induced regression of the tumour as shown by the disappearance of the mass in the external auditory meatus after treatment. Neveitheless, the tumour rapidly regrew and eventually led to the death of the patient 4 • 5 months after presentation. Summary

A case of embryonal rhabdomyosarcoma of the middle ear and mastoid is presented. The patient was treated by irradiation and Actinomycin D, and survived 4 • 5 months. Acknowledgement

The author wishes to thank Mr. R. S. McCrea, Consultant ENT Surgeon, Royal Victoria Hospital, Belfast and Dr. A. R. Lyons, Consultant Radiotherapist, Northern Ireland Radiotherapy Centre, Belfast, for their permission to publish this case, and Mr. Roy Creighton for the photomicrographs. REFERENCES ARNOLD, R., and BAYLIN, G. (1959) Laryngoscope, 69, 766.

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Rhabdomyosarcoma of the middle ear and mastoid.

Rhabdomyosarcama of the middle ear and mastoid By A. L. PAHOR (Birmingham) RHABDOMYOSARCOMA of the middle ear is a malignant tumour of childhood, usua...
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