1991, The British Journal of Radiology, 64, 171-172 Case reports MOURAD, K., GUGGIANA, P. & MINASIAN, H., 1987. Superior
mesenteric artery aneurysm diagnosed by ultrasound. British Journal of Radiology, 60, 287-288. REUTER, S. R., FRY, W. J. & BOOKSTEIN, J. J., 1968. Mesenteric
artery branch aneurysms. Archives of Surgery, 97, 497-499.
STANLEY, J. C , WAKEFIELD, T. W., GRAHAM, L. M., WHITEHOUSE, W. M., ZELONOCK, G. B. & LINDENAUER,
S. M., 1986. Clinical importance and management of splanchnic artery aneurysms. Journal of Vascular Surgery 3 836-840. '
Radiation induced extraskeletal osteosarcoma J. P. Logue, MB, MRCP, FRCR and F. Cairnduff, MB, MRCP Department of Radiotherapy and Oncology, Christie Hospital, Wilmslow Road Manchester M20 9BX, UK (Received June 1990 and in revised form July 1990) Keywords: Osteosarcoma, Radiation induced neoplasms
Radiation induced neoplasms are a well recognized complication of radiation therapy. Among these neoplasms osteosarcoma is not uncommon, classically affecting the skeleton. We report a case of extraskeletal osteosarcoma occurring in the nasal cavity following kilovoltage radiotherapy.
haematogenous and solid tumours are recognized and of the latter sarcomas are well described. It has been reported that 5.5% of osteosarcomas presenting in one centre are the result of previous therapeutic or incidental irradiation (Huvos et al, 1984). The majority of
Case report A 62-year-old woman presented with a 1 x 1 cm Basal cell carcinoma on the skin of her left upper nose. She was treated with a single exposure of radiotherapy to a skin dose of 20 Gy using 100 Kv (3 mm Al) at a focus-skin distance of 15 cm to a circular area of a diameter of 2 cm. She was reviewed 6 weeks later at which time the lesion had completely resolved. She represented 14 years later with an 8-month history of painless swelling of her left upper nose, nasal obstruction and epiphora (Fig. 1). Examination revealed a hard fixed mass adjacent to the bridge of her nose. The overlying skin showed signs of radiation stigmata. Examination under anaesthetic demonstrated fullness of the upper part of the lateral nasal wall anterior to the middle turbinate. The clinical appearance was of a primary nasal cavity tumour. Biopsies were taken of this area. Histology of these revealed normal nasal mucosa with an infiltrate of malignant tumour composed of spindle, oval and polygonal cells forming abundant osteoid and woven bone consistent with osteosarcoma. It is our belief that the nature of the presentation and histology, together with the subsequent growth pattern, are consistent with this osteosarcoma being extraskeletal in origin (although ultimately the exact origin cannot be proven). Three weeks later on admission for palliative radiotherapy the lesion had progressed rapidly with fleshy tumour exuding from the left nasal cavity and a proliferative ulcerated lesion arising from the upper nose and occluding the left eye (Fig. 2). She was treated with palliative intent utilizing a single megavoltage anterior field encompassing all gross disease to a dose of 27.5 Gy in 4 fractions. Following treatment there was an initial partial response but unfortunately this was not sustained and she died 6 months later with progressive local disease. Discussion
Secondary neoplasms are an unfortunate but accepted complication of cancer therapy. Both *Author for correspondence. Vol. 64, No. 758
Figure 1. Clinical photograph showing swelling of left upper nose and nasal obstruction.
171
Case reports
Figure 2. Clinical photograph showing tumour exuding from the left nasal cavity and lesion arising from the upper nose. osteosarcomas arise within the skeleton with extraskeletal to skeletal ratio quoted as 1 : 25 (Allan & Soule, 1971). Radiation induced extraskeletal osteosarcomas are rare with only 17 cases reported in the world literature by 1988. The majority of these cases occurred on the trunk with only two previous cases in the head and neck; one of which also arose in the nasal cavity. Only two previous cases have been reported following therapeutic radiation directed at skin conditions (Huvos et al, 1984). The criteria for accepting the diagnosis of radiation induced osteosarcoma have been outlined by Cahan et al (1948) and are (1) the sarcoma must have arisen in an irradiated area; (2) irradiation must be followed by a relatively long latent period; and (3) there must be histological documentation. All these criteria are fulfilled by our patient. Boyer and Navin (1965)
172
additionally suggested that there should be chronic radiodermatitis overlying the sarcoma. This latter criteria is also fullfilled but we would refute this as a requirement of diagnosis, particularly when skin sparing is now often sought in radiotherapy planning. The mean latent period from exposure to development of sarcoma is approximately 12 years with cases arising from 6 to 24 years (Huvos et al, 1984). The reported case arose 14 years following treatment. In the present case the tumour followed a virulent course with progressive local disease. Of the previously reported cases 9 out of 17 died of progressive local and or metastatic disease. The others are reported to be alive and diseases free from 1 month to 19 years following treatment. Treatment of post-radiation osteosarcomas should be selected according to individual cases, but primary surgery is the treatment of choice in resectable lesions. The reported case represents a serious late sequelae of radiotherapy. Quantification of the risk is necessary but unfortunately is unsatisfactory. Hadfield and Schultz (1970) estimated the risk of developing radiation induced bone sarcoma following radiotherapy for carcinoma of the breast to be about 0.2% of patients surviving for 10 years after therapy. There are however many possible sources of error leading to underreporting of cases. The possibility of spontaneous sarcoma must also be remembered. Only three cases of extraskeletal osteosarcoma, including this one, have arisen subsequent to radiotherapy directed at primary skin lesions. In our clinical practice we treat up to 1000 patients with skin lesions by radiotherapy each year and would strongly argue that the efficacy and convenience of this treatment heavily counterbalances the risk of development of this reported and other complications. Acknowledgments We thank Miss K. Hawksworth for typing the manuscript. References ALLAN, C. & SOULE, E., 1971. Osteogenic sarcoma of the
somatic soft tissues. Cancer, 27, 1121-1133. BOYER, C. & NAVIN, J., 1965. Extraskeletal osteogenic sarcoma. Cancer, 18, 628-633. CAHAN, W.
G.,
WOODWARD, H. Q.,
HIGINBOTHAM, N. L.,
STEWART, F. W. & COLEY, B. L., 1948. Sarcoma arising in
irradiated bone. Report of 11 cases. Cancer, 1, 3-29. HADFIELD, P. M. & SCHULTZ, M. D., 1970. Post irradiation
sarcoma including 5 cases after x-ray therapy of breast carcinoma. Radiology, 96, 593-602. Huvos, A. G., WOODWARD, H. Q., CAHAN, W. G., HIGINBOTHAM, N. L., STEWART, F. W., BUTLER, A. &
BRETSKY, S. S., 1984. Post-radiation osteogenic sarcoma of bone and soft tissue. A clinico-pathological study of 66 patients. Cancer, 55, 1244-1255.
The British Journal of Radiology, February 1991
mesenteric artery aneurysm diagnosed by ultrasound. British Journal of Radiology, 60, 287-288. REUTER, S. R., FRY, W. J. & BOOKSTEIN, J. J., 1968. Mesenteric
artery branch aneurysms. Archives of Surgery, 97, 497-499.
STANLEY, J. C , WAKEFIELD, T. W., GRAHAM, L. M., WHITEHOUSE, W. M., ZELONOCK, G. B. & LINDENAUER,
S. M., 1986. Clinical importance and management of splanchnic artery aneurysms. Journal of Vascular Surgery 3 836-840. '
Radiation induced extraskeletal osteosarcoma J. P. Logue, MB, MRCP, FRCR and F. Cairnduff, MB, MRCP Department of Radiotherapy and Oncology, Christie Hospital, Wilmslow Road Manchester M20 9BX, UK (Received June 1990 and in revised form July 1990) Keywords: Osteosarcoma, Radiation induced neoplasms
Radiation induced neoplasms are a well recognized complication of radiation therapy. Among these neoplasms osteosarcoma is not uncommon, classically affecting the skeleton. We report a case of extraskeletal osteosarcoma occurring in the nasal cavity following kilovoltage radiotherapy.
haematogenous and solid tumours are recognized and of the latter sarcomas are well described. It has been reported that 5.5% of osteosarcomas presenting in one centre are the result of previous therapeutic or incidental irradiation (Huvos et al, 1984). The majority of
Case report A 62-year-old woman presented with a 1 x 1 cm Basal cell carcinoma on the skin of her left upper nose. She was treated with a single exposure of radiotherapy to a skin dose of 20 Gy using 100 Kv (3 mm Al) at a focus-skin distance of 15 cm to a circular area of a diameter of 2 cm. She was reviewed 6 weeks later at which time the lesion had completely resolved. She represented 14 years later with an 8-month history of painless swelling of her left upper nose, nasal obstruction and epiphora (Fig. 1). Examination revealed a hard fixed mass adjacent to the bridge of her nose. The overlying skin showed signs of radiation stigmata. Examination under anaesthetic demonstrated fullness of the upper part of the lateral nasal wall anterior to the middle turbinate. The clinical appearance was of a primary nasal cavity tumour. Biopsies were taken of this area. Histology of these revealed normal nasal mucosa with an infiltrate of malignant tumour composed of spindle, oval and polygonal cells forming abundant osteoid and woven bone consistent with osteosarcoma. It is our belief that the nature of the presentation and histology, together with the subsequent growth pattern, are consistent with this osteosarcoma being extraskeletal in origin (although ultimately the exact origin cannot be proven). Three weeks later on admission for palliative radiotherapy the lesion had progressed rapidly with fleshy tumour exuding from the left nasal cavity and a proliferative ulcerated lesion arising from the upper nose and occluding the left eye (Fig. 2). She was treated with palliative intent utilizing a single megavoltage anterior field encompassing all gross disease to a dose of 27.5 Gy in 4 fractions. Following treatment there was an initial partial response but unfortunately this was not sustained and she died 6 months later with progressive local disease. Discussion
Secondary neoplasms are an unfortunate but accepted complication of cancer therapy. Both *Author for correspondence. Vol. 64, No. 758
Figure 1. Clinical photograph showing swelling of left upper nose and nasal obstruction.
171
Case reports
Figure 2. Clinical photograph showing tumour exuding from the left nasal cavity and lesion arising from the upper nose. osteosarcomas arise within the skeleton with extraskeletal to skeletal ratio quoted as 1 : 25 (Allan & Soule, 1971). Radiation induced extraskeletal osteosarcomas are rare with only 17 cases reported in the world literature by 1988. The majority of these cases occurred on the trunk with only two previous cases in the head and neck; one of which also arose in the nasal cavity. Only two previous cases have been reported following therapeutic radiation directed at skin conditions (Huvos et al, 1984). The criteria for accepting the diagnosis of radiation induced osteosarcoma have been outlined by Cahan et al (1948) and are (1) the sarcoma must have arisen in an irradiated area; (2) irradiation must be followed by a relatively long latent period; and (3) there must be histological documentation. All these criteria are fulfilled by our patient. Boyer and Navin (1965)
172
additionally suggested that there should be chronic radiodermatitis overlying the sarcoma. This latter criteria is also fullfilled but we would refute this as a requirement of diagnosis, particularly when skin sparing is now often sought in radiotherapy planning. The mean latent period from exposure to development of sarcoma is approximately 12 years with cases arising from 6 to 24 years (Huvos et al, 1984). The reported case arose 14 years following treatment. In the present case the tumour followed a virulent course with progressive local disease. Of the previously reported cases 9 out of 17 died of progressive local and or metastatic disease. The others are reported to be alive and diseases free from 1 month to 19 years following treatment. Treatment of post-radiation osteosarcomas should be selected according to individual cases, but primary surgery is the treatment of choice in resectable lesions. The reported case represents a serious late sequelae of radiotherapy. Quantification of the risk is necessary but unfortunately is unsatisfactory. Hadfield and Schultz (1970) estimated the risk of developing radiation induced bone sarcoma following radiotherapy for carcinoma of the breast to be about 0.2% of patients surviving for 10 years after therapy. There are however many possible sources of error leading to underreporting of cases. The possibility of spontaneous sarcoma must also be remembered. Only three cases of extraskeletal osteosarcoma, including this one, have arisen subsequent to radiotherapy directed at primary skin lesions. In our clinical practice we treat up to 1000 patients with skin lesions by radiotherapy each year and would strongly argue that the efficacy and convenience of this treatment heavily counterbalances the risk of development of this reported and other complications. Acknowledgments We thank Miss K. Hawksworth for typing the manuscript. References ALLAN, C. & SOULE, E., 1971. Osteogenic sarcoma of the
somatic soft tissues. Cancer, 27, 1121-1133. BOYER, C. & NAVIN, J., 1965. Extraskeletal osteogenic sarcoma. Cancer, 18, 628-633. CAHAN, W.
G.,
WOODWARD, H. Q.,
HIGINBOTHAM, N. L.,
STEWART, F. W. & COLEY, B. L., 1948. Sarcoma arising in
irradiated bone. Report of 11 cases. Cancer, 1, 3-29. HADFIELD, P. M. & SCHULTZ, M. D., 1970. Post irradiation
sarcoma including 5 cases after x-ray therapy of breast carcinoma. Radiology, 96, 593-602. Huvos, A. G., WOODWARD, H. Q., CAHAN, W. G., HIGINBOTHAM, N. L., STEWART, F. W., BUTLER, A. &
BRETSKY, S. S., 1984. Post-radiation osteogenic sarcoma of bone and soft tissue. A clinico-pathological study of 66 patients. Cancer, 55, 1244-1255.
The British Journal of Radiology, February 1991
