rare tumour first described by Enzinger in 1965. A case is reported which responded to bleomycin and vincristine. Such a response has not previously been.
1979, British Journal of Radiology, 52, 238-241 Case reports
Clear cell sarcoma of tendons and aponeuroses treated with bleomycin and vincristine By D. J. Radstone, M.B., B.S., P. A. Revell, M.R.C.Path., and B. S. Mantell, F.R.C.R. Department of Haematology, Institute of Pathology and Department of Radiotherapy and Oncology, The London Hospital, Whitechapel, London E1. {Received April, 1978 and in revised form August, 1978)
Clear cell sarcoma of tendons and aponeuroses is a rare tumour first described by Enzinger in 1965. A case is reported which responded to bleomycin and vincristine. Such a response has not previously been documented. CASE REPORT
The patient was a 30-year-old woman who first presented in June 1975 with painless lumps on her left leg. Seven years previously she had received a slight knock to her left shin and then noticed two firm lumps at the site. Two years before presentation the lumps had increased in size and there was a generalized swelling of the leg below the lumps.
Her appetite had diminished and she had begun to lose weight and experience pain in her left knee and ankle. For the last six months the area around the lumps had become red and hairless. Five weeks before presentation a new lump had appeared below the first two. On examination she was found to have a mass over the upper third of her left tibia (Fig. 1.) measuring 10x8 cm with an ulcerated centre 3.5x2 cm and below this two further masses 4 x 3 cm and 1.5x1.5 cm. The masses were firm and appeared attached to the underlying bone. The left leg distal to the masses was swollen and oedematous so that the circumference of the left leg was 9 cm greater than the right measured at a point 10 cm below the patella. The skin over the masses was hot, red, and atrophic and there was wasting of the left thigh muscles. She had no palpable lymph nodes at this time. An X-ray of the left leg showed a well circumscribed lobulated soft tissue mass with a periosteal reaction along the shaft of the tibia. The radiologist's opinion was that this was a primary soft-tissue tumour secondarily involving bone and probably a liposarcoma. An open biopsy was taken and a diagnosis of clear-cell sarcoma was made. A lymphangiogram showed no lymphatic involvement. Treatment and Progress From July 28 to August 26, 1975 she received a course of radiotherapy to her left leg (Table I). There was rapid regression of the tumour and at the end of the course she was left with an ulcer which in turn had healed by December 1975. She was well until August 1976 when she presented with a mass in the left femoral triangle. A drill biopsy revealed lymph node infiltration with sarcoma but a further lymphangiogram showed no other lymphatic involvement. This deposit proved to be the first in a series of further metastases in the right supraclavicular fossa, right thigh, abdomen, and right temple over the period from August 1976 to May 1977. These were treated as they arose with individual courses of radiotherapy (Table I). All the metastatic deposits regressed rapidly and completely by the end of each course of radiotherapy. On June 10, 1977 she was readmitted with a pathological fracture of her right femur at the upper third of the shaft. She was also found to have multiple deposits in her scalp, right humerus, right cheek, and right cervical nodes. Her blood count showed a haemoglobin of 7.9 g/dl, a white cell count of 4.4 X 109/litre and platelets of 976 X 109/ litre. The thrombocytopenia deepened to 44 X 10 /litre by June 15, 1977. Pending internal fixation of the fracture a Steinmann pin was inserted, the fracture was stabilized with traction, and she was transfused with blood. She continued to experience severe pain particularly at the fracture site and in the deposits in her neck with signs of peripheral nerve compression at the latter site. She required 20 mg of diamorphine orally every four hours for adequate analgesia. In view of her thrombocytopenia further radiotherapy was contraindicated but the tumour was obviously very radiosensitive and it appeared likely that it might also prove responsive to chemotherapy. It seemed necessary to select
FIG.1.
Photograph of left leg at presentation. 238
MARCH 1979
Case reports TABLE I SCHEDULE OF RADIOTHERAPY
Date 1975 28/7 7Q7/» / y iO
20/9 15/12 1977 6/4 3/5 13/5 18/5 28/10 3/11
Length of RT (days)
Radiation
29
8MVx
30 34
8MVx 8MVx
26 13 5 5
8MVx 8MVx 8MVx 10MeV electrons 60 Coy 300 kV x
13 11
Dose (rad)
Number of fractions
Left leg
4000
20
Left groin Mediastinum, bilateral axillae, supraclavicular fossae, and cervical nodes
4000 4000
20 20
Right hip Abdomen Left side of neck Right temple
3200 900 2000 2000
16 9 4 4
Skull Lumbar spine
3000 1600
10 8
Site
agents of low myelosuppressive activity. High dose bleomycin and vinca alkaloids as described by Samuel et al., (1976) were chosen as a suitable basis for treatment. It was proposed to give her 2 mg of vincristine as a bolus intravenously followed by 90 mg of bleomycin as an intravenous infusion over 72 hours. She started the first course on June 17, 1977 but there was a severe reaction to the bleomycin which was discontinued after only 20 mg had been given. Nevertheless a dramatic response was achieved in that all the tumour masses had become impalpable within fourteen days. Her general condition also improved considerably and her analgesic requirement fell so that she could be weaned off the diamorphine entirely. She had two further courses of chemotherapy which included bleomycin to full doses (the first under steroid cover) and had improved sufficiently by August 1977 to have an operative internal fixture of the fracture of her femur. She was discharged home in October 1977 by which time she had received a total cumulative dose of bleomycin of 200 mg. On October 21, 1977 she was readmitted complaining of headaches, diplopia, and difficulty in walking. On examination she had a partial third nerve palsy on the left side, saddle anaesthesia and absent ankle jerks. Apart from the neurological involvement she also had two new deposits in her scalp. Two days after admission she developed signs of meningeal irritation with neck stiffness, vomiting and photophobia. There were no localizing signs, no papilloedema and no neoplastic cells or evidence of infection in the CSF. A diagnosis of intracranial and cauda equina deposits was made. She was given dexamethasone and commenced a course of radiotherapy (Table I.). The meningism and headaches disappeared and her third cranial nerve palsy improved by the end of the course. She died at home in December 1977 and no post mortem was obtained. PATHOLOGY
Biopsy material from the left leg comprised several fragments of soft uniformly grey tissue, the largest measuring 1.5x1.5x1.3 cm. On microscopy the tumour was made up of oval or epithelioid uniform cells with vesicular nuclei containing prominent nucleoli and pale cytoplasm (Fig. 2). The matrix
between these cells was collagenous with areas of vacuolated myxoid material. Occasional abnormal mitoses were present among the clear cells and there was a small round cell infiltrate of varying density throughout. There were diastase-sensitive, periodic acid Schiff (PAS) positive granules in the cytoplasm of the tumour cells. A reticulin stain showed an alveolar-like pattern around nests of clear cells (Fig. 3). The Schmorl and Masson-Fontana reactions were negative. A diagnosis of clear cell sarcoma of tendons and aponeuroses was made. A drill biopsy from a mass in the left femoral triangle showed lymphoid tissue containing tumour having appearances similar to that described above apart from the fact that the alveolar pattern in the reticulin stain was not marked. A further drill biopsy of a mass in the supraclavicular fossa showed fragments of tumour containing clear cells and with an alveolar arrangement in the reticulin stain. This was diagnosed as recurrent clear cell sarcoma. DISCUSSION
Clear cell sarcoma of tendons and aponeuroses is a rare condition. Enzinger (1965) reported 21 cases, two of which were from Bennett's series (Bennett 1947), and only 17 cases have been reported since— Kubo (1969), Dutra (1970), MacKenzie (1974), Bearman et al., (1975) and Tsuneyoshi (1975). It has been suggested that clear-cell sarcoma of tendons may be a melanin-containing tumour and that there may be an entity "malignant melanoma of soft tissue" which is distinct from clear-cell sarcoma (Kubo 1969, Tsuneyoshi 1976, Bearman et al., 1975 and MacKenzie 1975.) We were unable to show the presence of melanin in our case.
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52, No. 615 Case reports
FIG. 2. Photomicrograph of the tumour, showing cells with indistinct pale cytoplasm and prominent nucleoli. A moderate number of small round cells is also present. Strands of fibrous tissue are present. (haematoxylin-eosin, X 500)
FIG. 3. Photomicrograph of the tumour, showing groups of cells surrounded by fibrous septa to give a pseudoacinar or alveolar appearance. (Gomori's silver impregnation, reticulin X 300)
240
MARCH 1979
Case reports The differential diagnosis of clear-cell sarcoma from synovial sarcoma has to be considered. Clinically the lack of pain during a major part of the long history favours clear-cell sarcoma, as does the site, antecedent trauma and slow growth with relentless multiple metastases. Histologically there was no evidence of the biphasic pattern seen in synovial sarcoma. There was a pseudoacinar alveolar arrangement in areas of the tumour and the PAS cytoplasmic granules were diastase sensitive, whereas they are diastase resistant in synovial sarcoma. Our patient's tumour showed a remarkable sensitivity to both radiotherapy and chemotherapy. With regard to the latter a substantial mass of tumour completely regressed after only 2 mg of vincristine and 20 mg of bleomycin had been given. Recent surveys of treatment for soft-tissue sarcomas (Chang, 1977; Wilbur et al., 1975) have shown a wide variation in response to various agents and combinations including vincristine but there have been no reports for the combination of vincristine and bleomycin. As the suggested regimes for the chemotherapy of soft-tissue sarcomas are more unpleasant and associated with a greater morbidity than this combination, the marked sensitivity
of this tumour to the combination of vincristine and bleomycin may therefore be of interest to other clinicians. REFERENCES BEARMAN, N. M., NOW, J., and KEMPSON, R. L.,
1975.
Clear cell sarcoma with melanin pigment. Cancer, 36, 977-984. BENNETT, G., 1947. Malignant neoplasms originating in synovial tissues (synoviomata). Journal Bone Joint Surgery, 29, 259-291. CHANG, P., 1977. Management of soft tissue sarcomas. American Journal of the Medical Sciences, 273, 244-257. DUTRA, F. W., 1970. Clear cell sarcoma of tendons and aponeuroses. Cancer, 25, 942-946. ENZINGER, F. M., 1965. Clear cell sarcoma of tendons and aponeuroses. Cancer, 18, 1163—1174. KUBO, T., 1969. Clear cell sarcoma of patellar tendon studied by electron microscopy. Cancer, 24, 948. MACKENZIE, D. H., 1974. Clear cell sarcoma of tendons and aponeuroses with melanin production. Journal Pathology, 7/4,231-232. SAMUEL, M. L., LANZOTTI, V. J., HOLOYE, P. Y., BOYLE, L. E., SMITH, T. L., and JOHNSON, D. E., 1976. Com-
bination chemotherapy in germinal cell tumours. Cancer Treatment Review, 3, 185-204. TSUNEYOSHI, M., 1975. Distinction of melanoma of soft parts from clear cell sarcoma of tendons and aponeuroses. Fukuoka Acta Medica, 66, 666-681. WILBUR, J. R., SUTOW, W. W., SULLIVAN, M. P., and
GOTTLIEB, J. A., 1975. Chemotherapy of sarcomas. Cancer, 36, 765-679.
Book reviews First year Physics for Radiographers. By George A. Hay and Donald Hughes, 1978, pp. xii + 271 (Bailliere Tindall, London), £3-95. ISBN 0-7020-0692-0. In the preface to the second edition of their excellent book which covers the physics syllabus for the diploma of the College of Radiographers, Messrs Hay and Hughes describe minor modifications and additions that they have made, notably with respect to solid state rectifiers, and their emphasis on expressing X-ray spectra in terms of photon energy rather than wavelength. The book is well laid out and in a similar style to the first edition, with many diagrams and with important points and laws emphasized in heavy type, a presentation which enables the student to use the book with ease. Two minor criticisms would be: the continued use in places of conventional current flow, which, even allowing for the authors' prior warning, some students will still find confusing; and their proof of the inverse square law using similar triangles, where the radii of different spheres is more correct. But these are minor criticisms of what is a well written, readable book and remarkable value at £3 -95. It is a book that most student radiographers will find invaluable in the preparation for their Part I examination.
Gastrointestinal Angiography. By S. R. Reuter and H. C. Redman, 1977, 2nd Edition, pp. ix + 390, illus., (W. B. Saunders Co., Sussex), £20-00. It is not uncommon to find oneself in difficulties to put major criticisms in polite words. It is very rare to experience difficulty in finding the right words in praise of an extraordinarily good text in radiology. This book falls into the latter category and is essential reading for all interested in abdominal angiography in any way. I am a little surprised that the authors have not used subtraction techniques more widely which may make some of the illustrated points more clear for the uninitiated or inexperienced. Although the standard of the illustrations is excellent the book is not just an atlas but is accompanied by a very good text covering technique, equipment and normal anatomy in addition to the multitude of clinical examples. Each chapter is concluded with a full and worthwhile bibliography. Too many words will dilute the praise. This is an excellent book.
R. S. FAIRBANKS.
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