Rritih 0
Journal
of Oral and hfarillofacial
1990 The British
Association
Surgery (1990) 28, 117-121
of Oral and Maxillofacial
SU~~UXU
Rhabdomyosarcoma, presenting as a facial swelling in a child. A case report and review of the literature C. N. Brookes, D. van Velzen Department of Oral and Maxillofacial Surgery and Department of Pathology, Royal Liverpool Children’s Hospital (Alder Hey), Liverpool
A case of the very rare rhabdomyosarcoma as the more common second primary in a young SUMMARY. child who had been treated for bilateral, genetic retinoblastoma is described and the importance early diagnosis emphasised. A review of the literature is presented with special reference to the prognosis of head and neck rhabdomyosarcoma and the various treatment alternatives, related to both new primary and secondary turnout-s in children with retinoblastoma. -. _.-
INTRODUCTION
The head and neck is the commonest site for malignant tumours in childhood (Jones & Campbell, 1976). Rhabdomyosarcoma may be defined as a malignant tumour of rhabdomyoblasts. It is the commonest of all soft tissue sarcomas in childhood (Mahour et al., 1967) and is estimated to comprise from 12 to 56% of all solid malignant tumours in the paediatric age group. These figures reflect the increased incidence of the tumour as diagnostic techniques have improved (Bale & Reye, 1970; Santamaria et al., 1970). Early diagnosis is essential as the extent of the disease at presentation has an important relationship with survival (Jaffe et al., 1973). Case report A 2.5-year-old child was referred to the Royal Liverpool Children’s Hospital (Alder Hey) from a peripheral unit, with a gross facial swelling. This had been present for approximately 6 weeks and had persisted despite formal incision and drainage under general anaesthesia 1 week previously. Routine culture and sensitivity of the sample obtained failed to reveal any significant growth of organisms. Following drainage the lesion had at first resolved slightly and then once more increased in size. As a neonate, the patient had been treated with locahsed radiotherapy for genetic bilateral retinoblastoma. Ophthalmic follow-up on regular basis had revealed no recurrence. The medical history was otherwise noncontributory. On examination the child was pyrexial (38.2%). There was a large, erythematous, non-fluctuant tender swelling of the right maxillary and mandibular soft tissues (Fig. 1). There was no associated palpable lymphadenopathy. Intra-oral examination (Fig. 2) revealed a gross haemorrhagic soft-tissue swelling, extending from the right buccal sulcus and retromolar trigone area into the sublingual space. This was displacing the tongue and embarrassing the airway.
Pia. 1 - Clinical presentation of the case.
Embedded in the swelling anteriorly was a deciduous molar tooth (E 1) displaced from its socket. This was readily removed digitally. A small piece of tissue attached to its root apex was submitted for urgent histopathological diagnosis. Plain radiographs revealed a large, poorly circumscribed osteolytic lesion extending from the right body of the mandible and eroding the right body distally, the ascending ramus and the condylar head (Fig. 3). CT scan of the head and neck revealed that in addition to the bony lesion of the mandible there was a large mass obliterating the infratemporal fossa (Fig. 4). This mass was displacing the 117
118 British Journal of Oral and Maxillofacial Sureerv
Fig. 3 - A PA mandible radiograph demonstrating bony destruction of the right body of the mandible distally, the right ramus and the right condylar head (between arrows).
Fig. 2 - Intra-oral view demonstrating
large haematoma in right
sublingual space. tongue and oropharynx to the left and extending to the midline prevertebrally. By the use of intra-venous contrast it was demonstrated that the great vessels were displaced posteriorly. There was no focal orbital or cerebral lesion. Plain films and CT scanning of the chest revealed no metastases and ultrasound of the liver, kidney and spleen confirmed their normal size and texture. However, a whole body scan provided evidence of metaphyseal flaring of both femurs distally and both tibia proximally. This appearance was felt to be compatible with metastases. Histopathological evidence demonstrated a rhabdomyosarcoma. In view of the local spread, bony involvement and probable distant metastases the lesion was classified as Stage III (T-3, N-0) (Donaldson et al., 1973). A cytoxic regime was suggested to the child’s parents. However, in view of the poor prognosis offered they decided against treatment. The lesion resulted in a fatal outcome approximately 2 weeks later. Permission for post-mortem was not received. Right and left iliac crest bone marrow aspirations taken prior to death showed normal marrow with no evidence of malignant infiltration. Histopathological examination of the tooth and attached tissue revealed the following: . Macroscopical examination: Tooth resection specimen with 0.4 cm diameter piece of tissue attached to it with variable consistency. The tissue was haemorrhagic and partly necrotic. Light microscopy: Tissue in part made up of necrosis, haemorrhage and inflammatory tissue with part of the tooth cavity squamous epithelium preserved. The tumour is composed of infiltrating bands and nests of small
Fig. 4 - CT scan showing extent of lesion (arrowed) obliterating the infratemporal fossa.
undifferentiated cells with slightly bloated nuclei and inconspicuous but clearly defined nucleoli. In some places a pseudopapillary arrangement of the remaining viable tumour tissue is seen with the live cells lining a papillary outgrowth with a vascular centre. In larger sheets of tumour cells there is extensive tumour cell degeneration with cleft shrinkage revealing an alveolar pattern. Reticulin stains showed the solid tumour fields to be completely devoid of any reticulin. A reticulin rich tissue lined these tumour spaces. In PAS stains the cells were positive in a granular fashion, the granules being occasionally diastase resistant (Fig. 5). Immunocytochemistry showed the tumour to be negative for cytokeratins, LCA, SW, TFAP, NSE, and neurofilamen&. Occasionally cells turned positive with antibodies
Rhabdomyosarcoma,
presenting as a facial
swellingin a child
119
DISCUSSION
Fig. 5 - Microphotograph of 4 q paraffin sections stained with reticulin stain. Note alveolar pattern of solid tumour fields within fibre rich connective tissue. (Original magnification x20.) Inset: Strongly atypical nuclei with a lobulated appearance and numerous mitoses. 4 pm paraffin section stained with haematoxylin and eosin. (Original magnification x500.)
Fig. 6 - Microphotograph
Soft tissue sarcomas present a particular diagnostic difficulty. Each type is individually uncommon as there are a number of soft-tissue lesions which may resemble a sarcoma clinically. A fibrosarcoma may be difficult to distinguish from an aggressive and highly cellular, but benign, fibromatosis (Jones & Campbell, 1976). These authors emphasise the difficulty of excluding the benign conditions of intramuscular haematoma and intramuscular abscess as a diagnosis. However, they suggest that if a supposed inflammatory lesion is drained surgically and no pus is obtained, or if encephaloid material is extruded, definitive biopsy for histopathological diagnosis should be performed. O’Day et al. (1965) studied 11 patients with embryonal rhabdomyosarcoma confirming that the most common presenting complaint is a patient with a painless mass. These workers considered that strabismus, dysphonia, dysphagia, cough and a deviation of the jaws described by Schafer et al. (1974) were much later presenting features. However, in
of 4 km paraffin section stained with the APAAP method of monoclonal antibody based immuno-peroxidase
technique. a: Vimentin b: Dcsmin c: Haemoglobin Note diffuse cytoplasmic staining of many cells with antibodies to Vimentin indicating sarcomatous origin (a). Individual cells in tumour groups show strong cytoplasmic staining for Desmin indicating myogenous origin (b). Cells in lining of alveolar spaces show strong cytoplasmic staining for Haemoglobin indicating rhabdomyosarcomatous lesion (c). (Original magnification ~500 for (a), (b) and (c).)
to vimentin. The tumour was, in its more viable parts, clearly positive for desmin. Positivity for myoglobin was outspoken especially in more papillary parts of the tumour. However, in tumour no development of more mature cells
with cross striations was seen even on additional sections. Morphologically discriminatable strap cells were absent (Fig. 6). Conclusion: Poorly differentiated alveolar type.
rhabdosarcoma.
Possibly
character of
one of the series of O’Day et al. (1965) paraesthesia and pain were noted when the tumour had invaded the mandible via the mental foramen. Most pathological conditions where the underlying lesion is in the mandible or maxilla can be satisfactorily demonstrated by routine radiographs. Furthermore, such radiographs often provide clear evidence of metastatic spread to the jaws from other tissues. CT scanning (Hounsfield, 1973) is now recognised
120 British Journal of Oral and Maxillofacial Sureerv
as a valuable diagnostic tool and has an accepted role in the evaluation of head and neck tumours (Rapidis et al., 1980), demonstrating oro-facial tissues in considerable detail relative to osseous structures (Frame & Wake, 1981). Intravenous contrast may be used to demonstrate the relationship of tumours to major vessels (McGahon et al., 1984). In the case described CT scanning provided important information regarding the extent of the lesion and the absence of pulmonary metastases at an early stage. Posterior displacement of the great vessels was demonstrated by intravenous contrast. Jaffe ef al. (1973) reported on childhood rhabdomyosarcoma arising in the extremities and genitourinary system as well as the head and neck (including orbits). They detailed the treatment of these rhabdomyosarcomas with surgery or radiotherapy but without chemotherapy. This revealed an overall survival rate of approximately 12%. Chemotherapeutic agents when incorporated into a multi-disciplinary approach were found by these authors to yield survival rates of 75% for Stage I (localised tumours) and 25% for Stage II lesions (tumours extending into adjacent tissues or regional lymph nodes). Definitive staging of our patient was complicated by the absence of a post-mortem report. Thus the importance of a multi-disciplinary approach is emphasised. However, Jaffe et al. (1973) qualify their approach by suggesting that in some lesions of the head and neck, surgery should be reserved for recurrence after primary treatment with chemotherapy and irradiation. In the series of Jaffe et al. (1973) patients with tumours of the orbit had the best prognosis (65% survival rate). The worst prognosis was associated with tumours of the extremities (13% survival rate). This is in contrast to the data of Sutow et al. (1970) who suggested that the worst prognosis was in tumours arising in structures of the head and neck excluding the orbit. In both the reports of Jaffe et al. (1973) and Sutow et al. (1970) tumours of the genitourinary system carried an intermediate prognosis. The earlier occurrence in the same patient of the genetic form of retinoblastoma is known in the literature (Draper et al., 1986) who reported a total of 39 secondary tumours. Radiotherapy seems to play a role in the tumour genesis. True second tumours seem only to occur in the genetic forms of retinoblastoma. In our case, the mandible was not included in the primary field of radiotherapy. However, the proximity of the second, primary site to the radiation field suggests that this factor along with the known susceptibility of patients with the genetic form of retinoblastoma to radiation-induced second tumours may have played a part in the genesis of the rhabdomyosarcoma. The reported cases of true second tumours were mostly osteosarcomas (21 patients); 8 osteosarcomas were sited within the field of radiation. The other 13 patients developed osteosarcomas in the leg. Other second neoplasms were brain tumours (3 patients; all of these lesions developing within the radiation
field), leukaemia (one patient who had received radiotherapy) and melanoma (two patients). One of these patients developed a melanoma within the radiation field, 17 years after treatment. Six epithelial tumours (none of which could be attributed to radiation treatment) and six soft tissue sarcomas were also reported. None of the sarcomas was a rhabdomyosarcoma, therefore to the best of our knowledge, this case is the first of its kind published in the British literature. Donaldson et al. (1973) suggested that rhabdomyosarcoma originating in the head and neck may be considered separately from other rhabdomyosarcoma. It is their opinion that children with this lesion originating in the head and neck can be effectively treated by combined surgery, radical radiation therapy and aggressive chemotherapy. In six of their 19 patients the primary site of origin was the cheek or adjacent area (tonsil, retromolar trigone or maxillary antrum). Generally, when compared to orbital lesions, tumours of this area have been found to have a poor prognosis. Donaldson et al. (1973) advance abundant lymphatics, ease of extension into neighbouring structures and lack of anatomical confines to the tumour as factors for this. However, with a systematic aggressive combination approach to treatment these authors report a local control rate of 86% (follow-up period ranging from 11 to 63 months). Jones and Campbell (1976) summarise the treatment of rhabdomyosarcomas as follows:
1) Surgical
excision of the primary tumour where feasible. They emphasise though that this surgery should be as complete as possible, but not radical or mutilating. 2) Radiotherapy to the site of the primary tumour and regional lymph nodes. Due attention should be paid to shielding adjacent vulnerable tissues. with a combination of three 3) Chemotherapy cytoxic agents as a primary course. Secondary courses at intervals of 1 to 3 months should continue for upto 2 years. However, in view of the suspected staging none of these forms of therapy was applied. In summary, the authors feel that although this is a rare case of rhabdomyosarcoma occurring as a second primary in a patient with the congenital variant of retinoblastoma, the tumour in its clinical presentation and histopathology was not different from patterns described for uncomplicated rhabdomyosarcoma of the oro-facial tissues. As such, even in patients with congenital retinoblastoma, a swelling as described should be approached with the same differential diagnosis, including that of a malignancy to allow for early diagnosis before spread has occurred. Thus, successful treatment may be a possibility. Acknowledgments The authors would like to thank Mr R. Cook, Consultant Paediatric Surgeon, Royal Liverpool Children’s Hospital (Alder Hey) for permission to describe a patient under his care. We arc
Rhabdomyosarcoma, also grateful to Mr J. C. Cooper for his helpful comments.
References Bale, P. M. & Rcye, R. D. K. (1975). Rhabdomyosarcoma in childhood. Pathology, 7, 101. Donaldson, S. S., Castro, J. R., Wilbur, J. R. & Jesse, R. H. (1973). Rhabdomvosarcoma of the head and neck in children. Cuncer, 31, 26. Draoer. G. J.. Sanders. B. M.. & Kineston. J. E. (1986). Second ‘primary neoplasms in patients with retinoblastoma.‘Brirish Journal of Cancer, 53,661.
Frame, J. W.&Wake, M. J. C. (1981).Thevalueofcomputerized tomography in oral surgery. Oral Surgery, 52, 357. Hounsfield, E. N. (1973). Computerised transverse axial scanning. British Journal of Radiology, 46, 1016. Jaffe, N., Filler, R., Farber, S. & Murray, J. E. (1973). Rhabdomyosarcoma in children; improved outlook with a multidiscipliniary approach. American Journal of Surgery, 125,482.
Jones, P. G. & Campbell, P. E. (1976). In: Turnours of Infancy & Childhood, pp. 295-833, Melbourne: Blackwell Scientific Publications. Mahour, G. H., Soule, E. H., Mills, S. D. & Lynn, H. B. (1967). Rhabdomyosarcomas in infants and children: a clinicopathologic study of 75 cases. Journal of Paediatric Surgery,
presenting as a facial swelling in a child
121
Rapidis, A. D., Angelopoulos, A. P., Langdon, J. D. & Scouteris, C. A. (1980). Computer&d axial tomography in the diagnosis of head and neck tumours. International Journal of Oral Surgery, 9,387.
Santamaria, J. N., Colebatch, J. H. & Campbell, P. E. (1970). Rhabdomyosarcoma: a study of 27 cases. Australasia Radiology, 14, 438.
Shafer, G. W., Hine, M. K. & Levy, B. M. (1974). In: A Textbook of Oral Pathology (3rd edition). pp. 186-187. Philadelphia: Saunders. Sutow, W. W., Sullivan, M. P., Taylor, A. G. & Griffith, R. H. (1970). Prognosis in childhood rhabdomyosarcoma. Cancer, 25,13x4.
The Authors C. N. Brookes BDS, FDSRCS Department of Oral and Maxillofacial Surgery D. van Velzen MD, PhD Department of Pathology Royal Liverpool Children’s Hospital (Alder Hey) Eaton Road Liverpool L12 2AP
2,402.
McGahan, J. P., Walter, J. P. & Bernstein, L. (1984). Evaluation of the parotid gland. Radiology, 152,‘453. O’Day, R. A., Soule, E. H. & Gazes, R. J. (1965). Embryonal rhabdomyosarcoma of the oral soft tissues. Oral Surgery, Oral Medicine, Oral Pathology, 20,85.
Correspondence
and requests for offprints to Mr C. N. Brookes
Paper received 14 November 1988 Accepted 24 March 1989
Journal
of Oral and hfarillofacial
1990 The British
Association
Surgery (1990) 28, 117-121
of Oral and Maxillofacial
SU~~UXU
Rhabdomyosarcoma, presenting as a facial swelling in a child. A case report and review of the literature C. N. Brookes, D. van Velzen Department of Oral and Maxillofacial Surgery and Department of Pathology, Royal Liverpool Children’s Hospital (Alder Hey), Liverpool
A case of the very rare rhabdomyosarcoma as the more common second primary in a young SUMMARY. child who had been treated for bilateral, genetic retinoblastoma is described and the importance early diagnosis emphasised. A review of the literature is presented with special reference to the prognosis of head and neck rhabdomyosarcoma and the various treatment alternatives, related to both new primary and secondary turnout-s in children with retinoblastoma. -. _.-
INTRODUCTION
The head and neck is the commonest site for malignant tumours in childhood (Jones & Campbell, 1976). Rhabdomyosarcoma may be defined as a malignant tumour of rhabdomyoblasts. It is the commonest of all soft tissue sarcomas in childhood (Mahour et al., 1967) and is estimated to comprise from 12 to 56% of all solid malignant tumours in the paediatric age group. These figures reflect the increased incidence of the tumour as diagnostic techniques have improved (Bale & Reye, 1970; Santamaria et al., 1970). Early diagnosis is essential as the extent of the disease at presentation has an important relationship with survival (Jaffe et al., 1973). Case report A 2.5-year-old child was referred to the Royal Liverpool Children’s Hospital (Alder Hey) from a peripheral unit, with a gross facial swelling. This had been present for approximately 6 weeks and had persisted despite formal incision and drainage under general anaesthesia 1 week previously. Routine culture and sensitivity of the sample obtained failed to reveal any significant growth of organisms. Following drainage the lesion had at first resolved slightly and then once more increased in size. As a neonate, the patient had been treated with locahsed radiotherapy for genetic bilateral retinoblastoma. Ophthalmic follow-up on regular basis had revealed no recurrence. The medical history was otherwise noncontributory. On examination the child was pyrexial (38.2%). There was a large, erythematous, non-fluctuant tender swelling of the right maxillary and mandibular soft tissues (Fig. 1). There was no associated palpable lymphadenopathy. Intra-oral examination (Fig. 2) revealed a gross haemorrhagic soft-tissue swelling, extending from the right buccal sulcus and retromolar trigone area into the sublingual space. This was displacing the tongue and embarrassing the airway.
Pia. 1 - Clinical presentation of the case.
Embedded in the swelling anteriorly was a deciduous molar tooth (E 1) displaced from its socket. This was readily removed digitally. A small piece of tissue attached to its root apex was submitted for urgent histopathological diagnosis. Plain radiographs revealed a large, poorly circumscribed osteolytic lesion extending from the right body of the mandible and eroding the right body distally, the ascending ramus and the condylar head (Fig. 3). CT scan of the head and neck revealed that in addition to the bony lesion of the mandible there was a large mass obliterating the infratemporal fossa (Fig. 4). This mass was displacing the 117
118 British Journal of Oral and Maxillofacial Sureerv
Fig. 3 - A PA mandible radiograph demonstrating bony destruction of the right body of the mandible distally, the right ramus and the right condylar head (between arrows).
Fig. 2 - Intra-oral view demonstrating
large haematoma in right
sublingual space. tongue and oropharynx to the left and extending to the midline prevertebrally. By the use of intra-venous contrast it was demonstrated that the great vessels were displaced posteriorly. There was no focal orbital or cerebral lesion. Plain films and CT scanning of the chest revealed no metastases and ultrasound of the liver, kidney and spleen confirmed their normal size and texture. However, a whole body scan provided evidence of metaphyseal flaring of both femurs distally and both tibia proximally. This appearance was felt to be compatible with metastases. Histopathological evidence demonstrated a rhabdomyosarcoma. In view of the local spread, bony involvement and probable distant metastases the lesion was classified as Stage III (T-3, N-0) (Donaldson et al., 1973). A cytoxic regime was suggested to the child’s parents. However, in view of the poor prognosis offered they decided against treatment. The lesion resulted in a fatal outcome approximately 2 weeks later. Permission for post-mortem was not received. Right and left iliac crest bone marrow aspirations taken prior to death showed normal marrow with no evidence of malignant infiltration. Histopathological examination of the tooth and attached tissue revealed the following: . Macroscopical examination: Tooth resection specimen with 0.4 cm diameter piece of tissue attached to it with variable consistency. The tissue was haemorrhagic and partly necrotic. Light microscopy: Tissue in part made up of necrosis, haemorrhage and inflammatory tissue with part of the tooth cavity squamous epithelium preserved. The tumour is composed of infiltrating bands and nests of small
Fig. 4 - CT scan showing extent of lesion (arrowed) obliterating the infratemporal fossa.
undifferentiated cells with slightly bloated nuclei and inconspicuous but clearly defined nucleoli. In some places a pseudopapillary arrangement of the remaining viable tumour tissue is seen with the live cells lining a papillary outgrowth with a vascular centre. In larger sheets of tumour cells there is extensive tumour cell degeneration with cleft shrinkage revealing an alveolar pattern. Reticulin stains showed the solid tumour fields to be completely devoid of any reticulin. A reticulin rich tissue lined these tumour spaces. In PAS stains the cells were positive in a granular fashion, the granules being occasionally diastase resistant (Fig. 5). Immunocytochemistry showed the tumour to be negative for cytokeratins, LCA, SW, TFAP, NSE, and neurofilamen&. Occasionally cells turned positive with antibodies
Rhabdomyosarcoma,
presenting as a facial
swellingin a child
119
DISCUSSION
Fig. 5 - Microphotograph of 4 q paraffin sections stained with reticulin stain. Note alveolar pattern of solid tumour fields within fibre rich connective tissue. (Original magnification x20.) Inset: Strongly atypical nuclei with a lobulated appearance and numerous mitoses. 4 pm paraffin section stained with haematoxylin and eosin. (Original magnification x500.)
Fig. 6 - Microphotograph
Soft tissue sarcomas present a particular diagnostic difficulty. Each type is individually uncommon as there are a number of soft-tissue lesions which may resemble a sarcoma clinically. A fibrosarcoma may be difficult to distinguish from an aggressive and highly cellular, but benign, fibromatosis (Jones & Campbell, 1976). These authors emphasise the difficulty of excluding the benign conditions of intramuscular haematoma and intramuscular abscess as a diagnosis. However, they suggest that if a supposed inflammatory lesion is drained surgically and no pus is obtained, or if encephaloid material is extruded, definitive biopsy for histopathological diagnosis should be performed. O’Day et al. (1965) studied 11 patients with embryonal rhabdomyosarcoma confirming that the most common presenting complaint is a patient with a painless mass. These workers considered that strabismus, dysphonia, dysphagia, cough and a deviation of the jaws described by Schafer et al. (1974) were much later presenting features. However, in
of 4 km paraffin section stained with the APAAP method of monoclonal antibody based immuno-peroxidase
technique. a: Vimentin b: Dcsmin c: Haemoglobin Note diffuse cytoplasmic staining of many cells with antibodies to Vimentin indicating sarcomatous origin (a). Individual cells in tumour groups show strong cytoplasmic staining for Desmin indicating myogenous origin (b). Cells in lining of alveolar spaces show strong cytoplasmic staining for Haemoglobin indicating rhabdomyosarcomatous lesion (c). (Original magnification ~500 for (a), (b) and (c).)
to vimentin. The tumour was, in its more viable parts, clearly positive for desmin. Positivity for myoglobin was outspoken especially in more papillary parts of the tumour. However, in tumour no development of more mature cells
with cross striations was seen even on additional sections. Morphologically discriminatable strap cells were absent (Fig. 6). Conclusion: Poorly differentiated alveolar type.
rhabdosarcoma.
Possibly
character of
one of the series of O’Day et al. (1965) paraesthesia and pain were noted when the tumour had invaded the mandible via the mental foramen. Most pathological conditions where the underlying lesion is in the mandible or maxilla can be satisfactorily demonstrated by routine radiographs. Furthermore, such radiographs often provide clear evidence of metastatic spread to the jaws from other tissues. CT scanning (Hounsfield, 1973) is now recognised
120 British Journal of Oral and Maxillofacial Sureerv
as a valuable diagnostic tool and has an accepted role in the evaluation of head and neck tumours (Rapidis et al., 1980), demonstrating oro-facial tissues in considerable detail relative to osseous structures (Frame & Wake, 1981). Intravenous contrast may be used to demonstrate the relationship of tumours to major vessels (McGahon et al., 1984). In the case described CT scanning provided important information regarding the extent of the lesion and the absence of pulmonary metastases at an early stage. Posterior displacement of the great vessels was demonstrated by intravenous contrast. Jaffe ef al. (1973) reported on childhood rhabdomyosarcoma arising in the extremities and genitourinary system as well as the head and neck (including orbits). They detailed the treatment of these rhabdomyosarcomas with surgery or radiotherapy but without chemotherapy. This revealed an overall survival rate of approximately 12%. Chemotherapeutic agents when incorporated into a multi-disciplinary approach were found by these authors to yield survival rates of 75% for Stage I (localised tumours) and 25% for Stage II lesions (tumours extending into adjacent tissues or regional lymph nodes). Definitive staging of our patient was complicated by the absence of a post-mortem report. Thus the importance of a multi-disciplinary approach is emphasised. However, Jaffe et al. (1973) qualify their approach by suggesting that in some lesions of the head and neck, surgery should be reserved for recurrence after primary treatment with chemotherapy and irradiation. In the series of Jaffe et al. (1973) patients with tumours of the orbit had the best prognosis (65% survival rate). The worst prognosis was associated with tumours of the extremities (13% survival rate). This is in contrast to the data of Sutow et al. (1970) who suggested that the worst prognosis was in tumours arising in structures of the head and neck excluding the orbit. In both the reports of Jaffe et al. (1973) and Sutow et al. (1970) tumours of the genitourinary system carried an intermediate prognosis. The earlier occurrence in the same patient of the genetic form of retinoblastoma is known in the literature (Draper et al., 1986) who reported a total of 39 secondary tumours. Radiotherapy seems to play a role in the tumour genesis. True second tumours seem only to occur in the genetic forms of retinoblastoma. In our case, the mandible was not included in the primary field of radiotherapy. However, the proximity of the second, primary site to the radiation field suggests that this factor along with the known susceptibility of patients with the genetic form of retinoblastoma to radiation-induced second tumours may have played a part in the genesis of the rhabdomyosarcoma. The reported cases of true second tumours were mostly osteosarcomas (21 patients); 8 osteosarcomas were sited within the field of radiation. The other 13 patients developed osteosarcomas in the leg. Other second neoplasms were brain tumours (3 patients; all of these lesions developing within the radiation
field), leukaemia (one patient who had received radiotherapy) and melanoma (two patients). One of these patients developed a melanoma within the radiation field, 17 years after treatment. Six epithelial tumours (none of which could be attributed to radiation treatment) and six soft tissue sarcomas were also reported. None of the sarcomas was a rhabdomyosarcoma, therefore to the best of our knowledge, this case is the first of its kind published in the British literature. Donaldson et al. (1973) suggested that rhabdomyosarcoma originating in the head and neck may be considered separately from other rhabdomyosarcoma. It is their opinion that children with this lesion originating in the head and neck can be effectively treated by combined surgery, radical radiation therapy and aggressive chemotherapy. In six of their 19 patients the primary site of origin was the cheek or adjacent area (tonsil, retromolar trigone or maxillary antrum). Generally, when compared to orbital lesions, tumours of this area have been found to have a poor prognosis. Donaldson et al. (1973) advance abundant lymphatics, ease of extension into neighbouring structures and lack of anatomical confines to the tumour as factors for this. However, with a systematic aggressive combination approach to treatment these authors report a local control rate of 86% (follow-up period ranging from 11 to 63 months). Jones and Campbell (1976) summarise the treatment of rhabdomyosarcomas as follows:
1) Surgical
excision of the primary tumour where feasible. They emphasise though that this surgery should be as complete as possible, but not radical or mutilating. 2) Radiotherapy to the site of the primary tumour and regional lymph nodes. Due attention should be paid to shielding adjacent vulnerable tissues. with a combination of three 3) Chemotherapy cytoxic agents as a primary course. Secondary courses at intervals of 1 to 3 months should continue for upto 2 years. However, in view of the suspected staging none of these forms of therapy was applied. In summary, the authors feel that although this is a rare case of rhabdomyosarcoma occurring as a second primary in a patient with the congenital variant of retinoblastoma, the tumour in its clinical presentation and histopathology was not different from patterns described for uncomplicated rhabdomyosarcoma of the oro-facial tissues. As such, even in patients with congenital retinoblastoma, a swelling as described should be approached with the same differential diagnosis, including that of a malignancy to allow for early diagnosis before spread has occurred. Thus, successful treatment may be a possibility. Acknowledgments The authors would like to thank Mr R. Cook, Consultant Paediatric Surgeon, Royal Liverpool Children’s Hospital (Alder Hey) for permission to describe a patient under his care. We arc
Rhabdomyosarcoma, also grateful to Mr J. C. Cooper for his helpful comments.
References Bale, P. M. & Rcye, R. D. K. (1975). Rhabdomyosarcoma in childhood. Pathology, 7, 101. Donaldson, S. S., Castro, J. R., Wilbur, J. R. & Jesse, R. H. (1973). Rhabdomvosarcoma of the head and neck in children. Cuncer, 31, 26. Draoer. G. J.. Sanders. B. M.. & Kineston. J. E. (1986). Second ‘primary neoplasms in patients with retinoblastoma.‘Brirish Journal of Cancer, 53,661.
Frame, J. W.&Wake, M. J. C. (1981).Thevalueofcomputerized tomography in oral surgery. Oral Surgery, 52, 357. Hounsfield, E. N. (1973). Computerised transverse axial scanning. British Journal of Radiology, 46, 1016. Jaffe, N., Filler, R., Farber, S. & Murray, J. E. (1973). Rhabdomyosarcoma in children; improved outlook with a multidiscipliniary approach. American Journal of Surgery, 125,482.
Jones, P. G. & Campbell, P. E. (1976). In: Turnours of Infancy & Childhood, pp. 295-833, Melbourne: Blackwell Scientific Publications. Mahour, G. H., Soule, E. H., Mills, S. D. & Lynn, H. B. (1967). Rhabdomyosarcomas in infants and children: a clinicopathologic study of 75 cases. Journal of Paediatric Surgery,
presenting as a facial swelling in a child
121
Rapidis, A. D., Angelopoulos, A. P., Langdon, J. D. & Scouteris, C. A. (1980). Computer&d axial tomography in the diagnosis of head and neck tumours. International Journal of Oral Surgery, 9,387.
Santamaria, J. N., Colebatch, J. H. & Campbell, P. E. (1970). Rhabdomyosarcoma: a study of 27 cases. Australasia Radiology, 14, 438.
Shafer, G. W., Hine, M. K. & Levy, B. M. (1974). In: A Textbook of Oral Pathology (3rd edition). pp. 186-187. Philadelphia: Saunders. Sutow, W. W., Sullivan, M. P., Taylor, A. G. & Griffith, R. H. (1970). Prognosis in childhood rhabdomyosarcoma. Cancer, 25,13x4.
The Authors C. N. Brookes BDS, FDSRCS Department of Oral and Maxillofacial Surgery D. van Velzen MD, PhD Department of Pathology Royal Liverpool Children’s Hospital (Alder Hey) Eaton Road Liverpool L12 2AP
2,402.
McGahan, J. P., Walter, J. P. & Bernstein, L. (1984). Evaluation of the parotid gland. Radiology, 152,‘453. O’Day, R. A., Soule, E. H. & Gazes, R. J. (1965). Embryonal rhabdomyosarcoma of the oral soft tissues. Oral Surgery, Oral Medicine, Oral Pathology, 20,85.
Correspondence
and requests for offprints to Mr C. N. Brookes
Paper received 14 November 1988 Accepted 24 March 1989
