Two rare tumours involving the infratemporal fossa: alveolar soft part sarcoma and haemangiopericytoma By G. BUCHANAN (London)
Introduction
THE infratemporal fossa is an irregularly shaped anatomical space in the lateral facial region situated below and medial to the zygomatic arch. Few people gain much experience in treating tumours in this region and perhaps the largest personal series is thirty-eight cases lately reported by Conley (1970). This author classified tumours of the infratemporal fossa as: (1) Primary at this site; (2) Direct extension into the infratemporal fossa from an adjacent site; (3) Metastases to the infratemporal fossa from a distant site. Two examples of rare forms of mesenchymal tumours extending into the infratemporal fossa have recently been encountered. These tumours occurred in young females. Each provided intriguing problems from the standpoints of both diagnosis and treatment. These cases, both treated surgically with widely different outcomes in the Professorial Unit at the Royal National Throat, Nose and Ear Hospital, form the basis for this report. Case reports Case 1
The patient, a 17-year-old female, was referred to the Professorial Unit in June 1972 on account of a swelling of the right lower jaw (Fig. 1). This had been present for two years and was slowly increasing in size. It was firm in consistency involving the ramus from below the angle up to the zygomatic arch. The scar of a recent biopsy at another hospital was visible. The tumour bulged into the buccal cavity and adjacent oropharynx but the overlying mucosa was intact. There were no neurological signs. Pain was not a noticeable feature and she could still open her mouth and eat. Investigations: Hb 12.8 g./ioo ml. WBC 6700/mm3 with normal film and differential count. ESR 10 mm. in 1 hour (Westergren). Results of liver function tests and serum electrophoresis were normal. No Bence Jones protein was detectable in her urine. Plain X-ray films and rotational tomography (Fig. 2) showed a soft tissue mass invading the ramus of the mandible. The tumour * Read at the Section of Laryngology Meeting, Royal Society of Medicine, 3 May 1974.
375
G. Buchanan
FIG. I.
Case i. Full face photograph to show swelling of right lower jaw.
evidently extended to and had caused bone erosion of the greater wing of the sphenoid lateral to the foramen ovale on the submentovertical radiograph. A right carotid angiogram demonstrated a highly vascular neoplasm, compatible with a chemodectoma, which displaced the internal carotid artery posteromedially. Chest X-ray examination showed no abnormality. A further biopsy was performed. Microscopically this was a malignant mesenchymal tumour but there was no firm agreement as to its origin. The biopsy was at some stage variously described as plasmacytoma, chemodectoma, alveolar soft part sarcoma and adenocarcinoma. For this reason it was felt that before surgery was undertaken she should have a trial of radiotherapy. She received 3500 r without noticeable tumour response and radiotherapy was abandoned. Operation: The tumour was resected under hypothermia on 28 September 1972 with the assistance of a vascular surgeon in case carotid arteiy reconstruction should be required. Moderate hypothermia of 30 ° C. was achieved by surface cooling with refrigerating blankets (Harrison, 1965). Access to the 376
Two rare tumours involving the infratemporal fossa
FIG. 2. An orthopantomogram of the jaws of Case I showing extensive destruction of the right ascending ramus of the mandible.
tumour was gained via a parotidectomy type incision. It was firmly fixed to the ascending ramus of the mandible, the main bulk lying deeply. A composite resection with the ramus was carried out, freeing the mass from the surrounding tissues of the parapharyngeal region. Superiorly it extended towards the base of the skull and was adherent to the roof of the infratemporal fossa from which it was finally freed. In the event the carotid vessels were not involved. A mucosal defect into the buccal cavity was sutured. Primary repair of the mandibular defect at the time of surgery was not thought wise. A temporary tracheostomy was constructed. Postoperative course: She made an uneventful recovery having only a transient facial paralysis after the operation. Although very upset by the visual effect we encouraged her to return to work (Fig. 3). One year later a lymph node metastasis was excised from the wound area. Despite the poor prognosis the question now arose whether she should be offered some form of cosmetic surgery. A plastic surgeon saw her and it was agreed that the facial depression should be corrected using a dermofat graft taken from the buttock. Before this could be initiated she developed recurrent disease in the neck. Metastatic deposits appeared in the lungs and vertebrae followed by paraplegia. Death occurred on 2 May 1974, four years after the tumour was first noticed. No autopsy was carried out. Gross appearances: The resected specimen consisted of a large rounded tumour mass of firm consistency together with the ramus of the mandible with which it appeared to be intimately associated (Fig. 4). Cut surface showed yellowish-grey tumour. Histological findings: Microscopically (Fig. 5) the tumour was highly cellular, the component cells being arranged in pseudoalveolar groups surrounded by thin reticulin membrane. The abundant cytoplasm was granular and occa377
G. Buchanan sionally vacuolated but no striations were observed. Mitotic activity was minimal. The appearances were these of alveolar soft part sarcoma. Histology of the lymph node showed it to be composed almost entirely of tumour with the same appearance.
FIG. 3. Postoperative photograph of Case 1.
Case 2 This 30-year-old woman's symptoms began in April 1970 with gradual loss of vision and mild proptosis of the left eye. She was initially seen at another hospital where examination revealed a visual field defect in the left lower temporal quadrant. No abnormality was found in the nose or postnasal space. Radiographs showed a space-occupying lesion in the left pterygomaxillary fissure. This was explored transantrally when the posterior wall of the antrum was found to be pushed forwards by a swelling which was so clearly pulsatile that only a needle biopsy was taken; blood was aspirated but no malignant cells were found. Carotid angiography demonstrated a large vascular tumour 378
Two rare tumours involving the infratemporal fossa
CMl 1 | 2 | 3 [4 1 5 | 6 | 7 | 8[ 9 FIG. 4. Alveolar soft pait sarcoma. The surgical specimen (Case 1).
FIG. 5. Case 1. Alveolar soft part sarcoma. Photomicrograph showing large cells with pseudoalveolar arrangement (H. & E. X144).
379
G. Buchanan
FIG. 6. Case 2. Preoperative photograph demonstrating unilateral left proptosis which is concealed by the ptosed eyelid.
compressing the contents of the orbit, presumed to be a rare example of an angiofibroma in a female. Working on this assumption she was given a course of radiotherapy amounting to 6,000 r. The proptosis disappeared and the field defect partly recovered. The patient remained well for three years, when proptosis gradually recurred with progressive loss of vision in the left eye. She was referred to the Professorial Unit for further treatment in July 1973 (Fig. 6). The left eye was now completely blind and neurological examination revealed a well-defined sensory loss over V1-11 on the left. She denied any history of epistaxis and the nose and postnasal space remained clear. Investigations: A full blood examination was normal. Plain X-ray films (Fig. 7) followed by tomography showed involvement of the apex of the orbit. There was gross enlargement of the superior orbital fissure which had enlarged at the expense of the greater wing of the sphenoid with elevation of the anterior clinoid process on the left side (Fig. 8). 380
Two rare tumours involving the infratemporal fossa
FIG. 7. A postero-anterior radiograph of the skull of Case 2 showing gross enlargement of the left superior orbital fissure.
The maxillary sinus was reopened and a biopsy was taken from the tumour bulging through its posterior wall. There was negligible bleeding. The histology showed a malignant mesenchymal tumour initially interpreted as malignant change in an extradural meningioma. Further angiographic studies revealed tumour extension into the middle cranial fossa possibly displacing the carotid siphon from its normal position (Fig. 9). The bulk of the tumour's blood supply was arising from the maxillary artery. On selective left internal carotid angiogram no major arterial blood supply was seen arising from the carotid siphon. Neurosurgical advice was that we should go ahead and try to remove the tumour from below. If it was not too firmly adherent to dura, intradural extension was unlikely and it should be amenable to complete removal. Should it appear to have gone through dura a subtotal removal would be carried out; after an interval at a second operation the residue would be removed as a neurosurgical procedure. Operation: On 20 September 1973 a cheek flap was elevated through a Weber Fergusson approach, the incision extending laterally into the palpebral fissure. It appeared justified to enucleate the blind eye to provide adequate exposure and to allow a controlled removal of the lesion. The maxilla was then removed, special care being exercised in mobilizing it from the underlying tumour. This occupied the pterygopalatine fossa impinging on the cavernous sinus medially and extending laterally out into the infratemporal fossa (Fig. 10). After the main vessel on its lateral aspect was secured blood loss was slight. Posterosuperiorly it penetrated deeply through the defect in the greater wing of the sphenoid to be attached in a curve to the middle fossa dura from which it
G. Buchanan peeled off readily by a combination of sharp and blunt dissection. A small dural tear was closed by rotating the cone of muscle from the apex of the orbit to provide a muscle seal. A clean extirpation was achieved and there was nothing to suggest intradural involvement.
FIG. 8. A coronal tomogram of skull of Case 2 demonstrating elevation of the anterior clinoid process on the left side (arrow) by the tumour.
Postoperative course: She made an excellent recovery following her operation. At the present time the patient is symptom-free and there is no evidence of the tumour. She wears an obturator on a dental plate and an artificial eye attached to spectacle frames over the reapproximated eyelids (Fig. n ) . Gross appearances: The surgical specimen comprised an oval grey tumour measuring 6 cm. in its greatest diameter. The surface was somewhat bosselated and the tumour was fairly well encapsulated except anteriorly at the site of its maxillary attachment (Fig. 12). Cut surface showed homogeneous grey rather fibrous tumour. 382
Two rare tumours involving the infratemporal fossa Histological findings: A stretched out nerve intimately connected with the surface of the tumour was thought to represent fibres of the maxillary nerve. Microscopically (Fig. 13) the tumour was seen to be a haemangiopericytoma with numerous endothelial-lined vascular channels separated by sheets of spindle-shaped and rounded cells. An occasional mitotic figure was present. Sections of the optic nerve showed demyelination up to approximately o. 5 cm. from the optic disc probably due to pressure. There was no abnormality in the structures of the eye itself.
FIG. 9. Subtraction film of left common carotid angiogram (lateral view) of Case 2 reveals a vascular tumour in the left pterygoid region supplied by the maxillary artery (double arrow). There is extension through the greater wing of the sphenoid into the middle cranial fossa (white arrow) in relation to the carotid siphon. Anteriorly the tumour extends along the orbital floor (oblique arrow). Note the crescentic shadow of the retinal vessels (single arrow).
Discussion This paper brings together two rare soft tissue tumours of the head and neck. The cases are similar because of the location of the neoplasms, both of which extended into the infratemporal fossa from an adjacent site. A further similarity exists in the diagnostic difficulties each presented at both the gross and microscopic level. An interesting fact is that of the 38 infratemporal fossa tumours encountered by Conley (1970) only nine arose primarily at that site, twenty-seven had extended from adjacent sites, and two were metastases from distant sites. Christopherson et al. (1952) chose to use the non-committal term alveolar soft part sarcoma for this distinctive class of tumours, because of the obscure histogenesis and the characteristic pseudoalveolar or organoid 383
G. Buchanan
FIG. IO.
Photograph taken during operation on Case 2 after removal of the eye and the maxilla to show the extent of the tumour.
arrangement of the cells. This pattern is somewhat reminiscent of that found in carotid body tumours. Other authors (Smetana and Scott, 1951; Karnauchow and Magner, 1963) were firmly convinced that the likely source of origin is from deposits of chemoreceptor tissue in various parts of the body which have not been anatomically documented as yet. There is little doubt that in the past similar tumours were designated malignant granular cell myoblastoma (Ravich et al., 1945), or were mistaken for metastatic adenocarcinoma (Smetana and Scott, 1951) In a recent paper, Lieberman et al (1966) surveyed fifty-three cases of alveolar soft part sarcoma and presented survival data on forty-six patients. These tumours occurred particularly in the deep tissues of the extremities and retroperitoneal region. The disease pursued an indolent 384
Two rare tumours involving the infratemporal fossa but inexorable course with a pronounced tendency to metastasize to lungs, liver, skeleton and less frequently to lymph nodes. There were three examples of lung metastases fifteen years after the onset of the illness. Twenty-nine patients were either dead from or living with incurable disease. A review of the literature has yielded nine instances of alveolar soft part sarcoma presenting in the head and neck. These cases are tabulated in Table I. In the present case the tumour probably arose in the soft
FIG. I I .
Case 2 fully rehabilitated six months after operation.
tissues adjacent to the mandible which it later invaded with extensive destruction of the neighbouring bone. It seems unlikely that it originated in the alveolar body as described by Burman (1956) as the mandibular foramen was not involved. Despite the absence of cranial nerve involve3*5
G. Buchanan
CM| 1 | 2 | 3 | 4 I 5 I 6 FIG. 12. Gross specimen of haemangiopericytoma (Case 2).
FIG. 13. Case 2. Haemangiopericytoma. Photomicrograph showing sheets of spindle-shaped and rounded cells in relation to vascular spaces (H. & E. X576).
386
Smetana and Scott
Smetana and Scott
Christopherson, F'oote and Stewart (Patient number 8 of Lieberman et al.) Caldwell, Hughes and Fadell
Ushijima and Tamura
Vakil and Sirsat
Vakil and Sirsat
Gingrass, Mladick, Pickrell and Punyahotra Varghese, Nair and Joseph
Year
1951
1951
1952
1956
1957
1963
1963
1967
1968
Authors
F
13
24 M
10 [7 1'
26 F 45 M
TVT 1V1
4
i.
12 17
F
28
M
27
Age/sex
L. orbit
Unknown
Recurrence after 6 months
Recurrence after few months. Orbit exenterated Died
Unknown
Unknown
No recuirence after 5 ysars
Died 1 year. Metastases lungs, heait, biain No recurrence 4 years after surgery
Course
No recurrence after 6 months Orbital exenteration Recurrence after few months
Excision
FZxcision
Unknown
Tongue 3 cm.
Excision
Unknown
R. orbit
L. temporal region
Excision
FZxcision
Radiation (unspscified amount) Excision 3 years thereafter F^xcision
FZxcision
Treatment
5 cm.
4 cm.
5 cm.
1 '5 cm.
Weight 51 g
Size
Tongue
Base of tongue
Base of tongue
R. posterior cervical triangle Inferior to angle L. mandible
Location
PREVIOUSLY REPORTED ALVEOLAR SOFT PART SARCOMAS IN THE HEAD AND NECK REGION
TABLF: 1.
H
i-n
B'
n
1-1
c
3
r-
%
1-1
1
*—*
i-t
O
•atei
fossa
G. Buchanan ment the most likely origin remains the vagal body, a collection of chemoreceptor tissue adjacent to the ganglion nodosum of the vagus nerve. The treatment of alveolar soft part sarcoma is essentially surgical and complete extirpation should be attempted unless there is evidence of distant metastases. Lieberman et al. (1966) concluded that for the majority extensive surgery proved of no value except for palliation. Regrettably the present case did not present early enough for a complete and effective removal of her neoplasm. Special mention should be made of the use of total body hypothermia at operation in case a period of temporary occlusion of the carotid vessels should be necessary because it increases the tolerance of the brain to the ischaemia of vascular occlusion. Westbury (i960) has recommended this precaution in the removal of carotid body tumours which may involve a vascular procedure other than dissection. Haemangiopericytoma is a comparatively rare vascular tumour first described by Stout and Murray (1942), since when over 300 cases have been reported in the literature. It can occur anywhere in the body where there are capillaries since the parent cell is the pericyte, a contractile cell forming a network on the outer capillary wall. Approximately 9 per cent arise in the orbit, sinuses, nasal and oropharyngeal regions (Backwinkel and Diddams, 1970). These authors recorded that involvement of these anatomical regions is fraught with a recurrence rate of 57 per cent. They emphasized that this tumour is highly malignant not in the usual five-year survival sense but over a lifetime. When metastases occur they are predominantly blood-borne and lymph node involvement is uncommon. Histologically this tumour must be differentiated from glomangioma glomus tumour, haemangioendothelioma, angioblastic meningioma and vascular sarcomas. The successful management of a haemangiopericytoma depends to a great extent on its size and location. When feasible the treatment of choice is wide local excision with adequate exposure. There is some indication that irradiation may exert some control over the tumour. Backwinkel and Diddams (1970) reported a 13 per cent cure rate in patients treated with radiotherapy alone, but they also inferred that adjunctive radiotherapy did not significantly improve the results of surgery. These cases underline the need for a multidisciplinary approach to rare tumours in the head and neck region which cut across the borders between specialties. The rarity of these tumours not only may lead to diagnostic difficulties but their surgical management may be hazardous. They require a high order of judgement since there is little established precedent for their treatment.
Two rare tumours involving the infratemporal fossa Summary
Two patients with malignant mesenchymal tumours encroaching on the infratemporal fossa are described. The extreme rarity of these tumours and the difficulties in differential diagnosis and treatment are discussed. Acknowledgements I am indebted to Professor D. F. N. Harrison for allowing me to report these patients. Thanks are also given to Professor L. Michaels for reporting the histology; to Mr. J. Connolly of the Department of Clinical Photography; and to Miss Damps for typing the manuscript. REFERENCES BACKWINKEL, K. D., and DIDDAMS, J. A. (1970) Cancer, 25, 896.
BURMAN, S. O. (1956) International Abstracts of Surgery, 102, 330. CALDWELL, J. B., HUGHES, K. W., and FADELL, E. J. (1956) Journal of Oral Surgery,
14, 342. CHRISTOPHERSON, W. M., FOOTE, F. W., and STEWART, F. W. (1952) Cancer, 5, 100.
CONLEY, J. (1970) Concepts in Head and Neck Surgery. George Thieme Verlag, Stuttgart, pp. 24-43. GINGRASS, R., MLADICK, R., PICKRELL, K., and PUNYAHOTRA, V. (1967)
Plastic
and Reconstructive Surgery, 40, 463. HARRISON, D. F. N. (1965) Journal of Laryngology and Otology, 81, 173. KARNAUCHOW, P. N., and MAGNER, D. (1963) Journal of Pathology and Bacteriology, 86, 169. LIEBERMAN, P. H., FOOTE, F. W., STEWART, F. W., and BERG, J. W. (1966) The
Journal of the American Medical Association, 198, 1047. RAVICH, A., STOUT, A. P., and RAVICH, R. A. (1945) Annals of Surgery, 121, 361.
SMETANA, H. F., and SCOTT, W. F. (1951) Military Surgeon, 109, 330. STOUT, A. P., and MURRAY, M. R. (1942) Annals of Surgery, 116, 26. USHIJIMA, H., and TAMURA, Z. (1957) Ada Pathologica Japonica, 7, 851. VAKIL, V. V., and SIRSAT, M. V. (1963) Indian Journal of Pathology and Bacteriology, 6, 19. VARGHESE, S., Xair, B., and JOSEPH, T. A. (1968) British Journal of Ophthalmology, 52, 713WESTBURY, G. (i960) British Journal of Surgery, 47, 605. The Institute of Laryngology and Otology 330-336 Gray's Inn Road London WCiX 8EE.
389
Introduction
THE infratemporal fossa is an irregularly shaped anatomical space in the lateral facial region situated below and medial to the zygomatic arch. Few people gain much experience in treating tumours in this region and perhaps the largest personal series is thirty-eight cases lately reported by Conley (1970). This author classified tumours of the infratemporal fossa as: (1) Primary at this site; (2) Direct extension into the infratemporal fossa from an adjacent site; (3) Metastases to the infratemporal fossa from a distant site. Two examples of rare forms of mesenchymal tumours extending into the infratemporal fossa have recently been encountered. These tumours occurred in young females. Each provided intriguing problems from the standpoints of both diagnosis and treatment. These cases, both treated surgically with widely different outcomes in the Professorial Unit at the Royal National Throat, Nose and Ear Hospital, form the basis for this report. Case reports Case 1
The patient, a 17-year-old female, was referred to the Professorial Unit in June 1972 on account of a swelling of the right lower jaw (Fig. 1). This had been present for two years and was slowly increasing in size. It was firm in consistency involving the ramus from below the angle up to the zygomatic arch. The scar of a recent biopsy at another hospital was visible. The tumour bulged into the buccal cavity and adjacent oropharynx but the overlying mucosa was intact. There were no neurological signs. Pain was not a noticeable feature and she could still open her mouth and eat. Investigations: Hb 12.8 g./ioo ml. WBC 6700/mm3 with normal film and differential count. ESR 10 mm. in 1 hour (Westergren). Results of liver function tests and serum electrophoresis were normal. No Bence Jones protein was detectable in her urine. Plain X-ray films and rotational tomography (Fig. 2) showed a soft tissue mass invading the ramus of the mandible. The tumour * Read at the Section of Laryngology Meeting, Royal Society of Medicine, 3 May 1974.
375
G. Buchanan
FIG. I.
Case i. Full face photograph to show swelling of right lower jaw.
evidently extended to and had caused bone erosion of the greater wing of the sphenoid lateral to the foramen ovale on the submentovertical radiograph. A right carotid angiogram demonstrated a highly vascular neoplasm, compatible with a chemodectoma, which displaced the internal carotid artery posteromedially. Chest X-ray examination showed no abnormality. A further biopsy was performed. Microscopically this was a malignant mesenchymal tumour but there was no firm agreement as to its origin. The biopsy was at some stage variously described as plasmacytoma, chemodectoma, alveolar soft part sarcoma and adenocarcinoma. For this reason it was felt that before surgery was undertaken she should have a trial of radiotherapy. She received 3500 r without noticeable tumour response and radiotherapy was abandoned. Operation: The tumour was resected under hypothermia on 28 September 1972 with the assistance of a vascular surgeon in case carotid arteiy reconstruction should be required. Moderate hypothermia of 30 ° C. was achieved by surface cooling with refrigerating blankets (Harrison, 1965). Access to the 376
Two rare tumours involving the infratemporal fossa
FIG. 2. An orthopantomogram of the jaws of Case I showing extensive destruction of the right ascending ramus of the mandible.
tumour was gained via a parotidectomy type incision. It was firmly fixed to the ascending ramus of the mandible, the main bulk lying deeply. A composite resection with the ramus was carried out, freeing the mass from the surrounding tissues of the parapharyngeal region. Superiorly it extended towards the base of the skull and was adherent to the roof of the infratemporal fossa from which it was finally freed. In the event the carotid vessels were not involved. A mucosal defect into the buccal cavity was sutured. Primary repair of the mandibular defect at the time of surgery was not thought wise. A temporary tracheostomy was constructed. Postoperative course: She made an uneventful recovery having only a transient facial paralysis after the operation. Although very upset by the visual effect we encouraged her to return to work (Fig. 3). One year later a lymph node metastasis was excised from the wound area. Despite the poor prognosis the question now arose whether she should be offered some form of cosmetic surgery. A plastic surgeon saw her and it was agreed that the facial depression should be corrected using a dermofat graft taken from the buttock. Before this could be initiated she developed recurrent disease in the neck. Metastatic deposits appeared in the lungs and vertebrae followed by paraplegia. Death occurred on 2 May 1974, four years after the tumour was first noticed. No autopsy was carried out. Gross appearances: The resected specimen consisted of a large rounded tumour mass of firm consistency together with the ramus of the mandible with which it appeared to be intimately associated (Fig. 4). Cut surface showed yellowish-grey tumour. Histological findings: Microscopically (Fig. 5) the tumour was highly cellular, the component cells being arranged in pseudoalveolar groups surrounded by thin reticulin membrane. The abundant cytoplasm was granular and occa377
G. Buchanan sionally vacuolated but no striations were observed. Mitotic activity was minimal. The appearances were these of alveolar soft part sarcoma. Histology of the lymph node showed it to be composed almost entirely of tumour with the same appearance.
FIG. 3. Postoperative photograph of Case 1.
Case 2 This 30-year-old woman's symptoms began in April 1970 with gradual loss of vision and mild proptosis of the left eye. She was initially seen at another hospital where examination revealed a visual field defect in the left lower temporal quadrant. No abnormality was found in the nose or postnasal space. Radiographs showed a space-occupying lesion in the left pterygomaxillary fissure. This was explored transantrally when the posterior wall of the antrum was found to be pushed forwards by a swelling which was so clearly pulsatile that only a needle biopsy was taken; blood was aspirated but no malignant cells were found. Carotid angiography demonstrated a large vascular tumour 378
Two rare tumours involving the infratemporal fossa
CMl 1 | 2 | 3 [4 1 5 | 6 | 7 | 8[ 9 FIG. 4. Alveolar soft pait sarcoma. The surgical specimen (Case 1).
FIG. 5. Case 1. Alveolar soft part sarcoma. Photomicrograph showing large cells with pseudoalveolar arrangement (H. & E. X144).
379
G. Buchanan
FIG. 6. Case 2. Preoperative photograph demonstrating unilateral left proptosis which is concealed by the ptosed eyelid.
compressing the contents of the orbit, presumed to be a rare example of an angiofibroma in a female. Working on this assumption she was given a course of radiotherapy amounting to 6,000 r. The proptosis disappeared and the field defect partly recovered. The patient remained well for three years, when proptosis gradually recurred with progressive loss of vision in the left eye. She was referred to the Professorial Unit for further treatment in July 1973 (Fig. 6). The left eye was now completely blind and neurological examination revealed a well-defined sensory loss over V1-11 on the left. She denied any history of epistaxis and the nose and postnasal space remained clear. Investigations: A full blood examination was normal. Plain X-ray films (Fig. 7) followed by tomography showed involvement of the apex of the orbit. There was gross enlargement of the superior orbital fissure which had enlarged at the expense of the greater wing of the sphenoid with elevation of the anterior clinoid process on the left side (Fig. 8). 380
Two rare tumours involving the infratemporal fossa
FIG. 7. A postero-anterior radiograph of the skull of Case 2 showing gross enlargement of the left superior orbital fissure.
The maxillary sinus was reopened and a biopsy was taken from the tumour bulging through its posterior wall. There was negligible bleeding. The histology showed a malignant mesenchymal tumour initially interpreted as malignant change in an extradural meningioma. Further angiographic studies revealed tumour extension into the middle cranial fossa possibly displacing the carotid siphon from its normal position (Fig. 9). The bulk of the tumour's blood supply was arising from the maxillary artery. On selective left internal carotid angiogram no major arterial blood supply was seen arising from the carotid siphon. Neurosurgical advice was that we should go ahead and try to remove the tumour from below. If it was not too firmly adherent to dura, intradural extension was unlikely and it should be amenable to complete removal. Should it appear to have gone through dura a subtotal removal would be carried out; after an interval at a second operation the residue would be removed as a neurosurgical procedure. Operation: On 20 September 1973 a cheek flap was elevated through a Weber Fergusson approach, the incision extending laterally into the palpebral fissure. It appeared justified to enucleate the blind eye to provide adequate exposure and to allow a controlled removal of the lesion. The maxilla was then removed, special care being exercised in mobilizing it from the underlying tumour. This occupied the pterygopalatine fossa impinging on the cavernous sinus medially and extending laterally out into the infratemporal fossa (Fig. 10). After the main vessel on its lateral aspect was secured blood loss was slight. Posterosuperiorly it penetrated deeply through the defect in the greater wing of the sphenoid to be attached in a curve to the middle fossa dura from which it
G. Buchanan peeled off readily by a combination of sharp and blunt dissection. A small dural tear was closed by rotating the cone of muscle from the apex of the orbit to provide a muscle seal. A clean extirpation was achieved and there was nothing to suggest intradural involvement.
FIG. 8. A coronal tomogram of skull of Case 2 demonstrating elevation of the anterior clinoid process on the left side (arrow) by the tumour.
Postoperative course: She made an excellent recovery following her operation. At the present time the patient is symptom-free and there is no evidence of the tumour. She wears an obturator on a dental plate and an artificial eye attached to spectacle frames over the reapproximated eyelids (Fig. n ) . Gross appearances: The surgical specimen comprised an oval grey tumour measuring 6 cm. in its greatest diameter. The surface was somewhat bosselated and the tumour was fairly well encapsulated except anteriorly at the site of its maxillary attachment (Fig. 12). Cut surface showed homogeneous grey rather fibrous tumour. 382
Two rare tumours involving the infratemporal fossa Histological findings: A stretched out nerve intimately connected with the surface of the tumour was thought to represent fibres of the maxillary nerve. Microscopically (Fig. 13) the tumour was seen to be a haemangiopericytoma with numerous endothelial-lined vascular channels separated by sheets of spindle-shaped and rounded cells. An occasional mitotic figure was present. Sections of the optic nerve showed demyelination up to approximately o. 5 cm. from the optic disc probably due to pressure. There was no abnormality in the structures of the eye itself.
FIG. 9. Subtraction film of left common carotid angiogram (lateral view) of Case 2 reveals a vascular tumour in the left pterygoid region supplied by the maxillary artery (double arrow). There is extension through the greater wing of the sphenoid into the middle cranial fossa (white arrow) in relation to the carotid siphon. Anteriorly the tumour extends along the orbital floor (oblique arrow). Note the crescentic shadow of the retinal vessels (single arrow).
Discussion This paper brings together two rare soft tissue tumours of the head and neck. The cases are similar because of the location of the neoplasms, both of which extended into the infratemporal fossa from an adjacent site. A further similarity exists in the diagnostic difficulties each presented at both the gross and microscopic level. An interesting fact is that of the 38 infratemporal fossa tumours encountered by Conley (1970) only nine arose primarily at that site, twenty-seven had extended from adjacent sites, and two were metastases from distant sites. Christopherson et al. (1952) chose to use the non-committal term alveolar soft part sarcoma for this distinctive class of tumours, because of the obscure histogenesis and the characteristic pseudoalveolar or organoid 383
G. Buchanan
FIG. IO.
Photograph taken during operation on Case 2 after removal of the eye and the maxilla to show the extent of the tumour.
arrangement of the cells. This pattern is somewhat reminiscent of that found in carotid body tumours. Other authors (Smetana and Scott, 1951; Karnauchow and Magner, 1963) were firmly convinced that the likely source of origin is from deposits of chemoreceptor tissue in various parts of the body which have not been anatomically documented as yet. There is little doubt that in the past similar tumours were designated malignant granular cell myoblastoma (Ravich et al., 1945), or were mistaken for metastatic adenocarcinoma (Smetana and Scott, 1951) In a recent paper, Lieberman et al (1966) surveyed fifty-three cases of alveolar soft part sarcoma and presented survival data on forty-six patients. These tumours occurred particularly in the deep tissues of the extremities and retroperitoneal region. The disease pursued an indolent 384
Two rare tumours involving the infratemporal fossa but inexorable course with a pronounced tendency to metastasize to lungs, liver, skeleton and less frequently to lymph nodes. There were three examples of lung metastases fifteen years after the onset of the illness. Twenty-nine patients were either dead from or living with incurable disease. A review of the literature has yielded nine instances of alveolar soft part sarcoma presenting in the head and neck. These cases are tabulated in Table I. In the present case the tumour probably arose in the soft
FIG. I I .
Case 2 fully rehabilitated six months after operation.
tissues adjacent to the mandible which it later invaded with extensive destruction of the neighbouring bone. It seems unlikely that it originated in the alveolar body as described by Burman (1956) as the mandibular foramen was not involved. Despite the absence of cranial nerve involve3*5
G. Buchanan
CM| 1 | 2 | 3 | 4 I 5 I 6 FIG. 12. Gross specimen of haemangiopericytoma (Case 2).
FIG. 13. Case 2. Haemangiopericytoma. Photomicrograph showing sheets of spindle-shaped and rounded cells in relation to vascular spaces (H. & E. X576).
386
Smetana and Scott
Smetana and Scott
Christopherson, F'oote and Stewart (Patient number 8 of Lieberman et al.) Caldwell, Hughes and Fadell
Ushijima and Tamura
Vakil and Sirsat
Vakil and Sirsat
Gingrass, Mladick, Pickrell and Punyahotra Varghese, Nair and Joseph
Year
1951
1951
1952
1956
1957
1963
1963
1967
1968
Authors
F
13
24 M
10 [7 1'
26 F 45 M
TVT 1V1
4
i.
12 17
F
28
M
27
Age/sex
L. orbit
Unknown
Recurrence after 6 months
Recurrence after few months. Orbit exenterated Died
Unknown
Unknown
No recuirence after 5 ysars
Died 1 year. Metastases lungs, heait, biain No recurrence 4 years after surgery
Course
No recurrence after 6 months Orbital exenteration Recurrence after few months
Excision
FZxcision
Unknown
Tongue 3 cm.
Excision
Unknown
R. orbit
L. temporal region
Excision
FZxcision
Radiation (unspscified amount) Excision 3 years thereafter F^xcision
FZxcision
Treatment
5 cm.
4 cm.
5 cm.
1 '5 cm.
Weight 51 g
Size
Tongue
Base of tongue
Base of tongue
R. posterior cervical triangle Inferior to angle L. mandible
Location
PREVIOUSLY REPORTED ALVEOLAR SOFT PART SARCOMAS IN THE HEAD AND NECK REGION
TABLF: 1.
H
i-n
B'
n
1-1
c
3
r-
%
1-1
1
*—*
i-t
O
•atei
fossa
G. Buchanan ment the most likely origin remains the vagal body, a collection of chemoreceptor tissue adjacent to the ganglion nodosum of the vagus nerve. The treatment of alveolar soft part sarcoma is essentially surgical and complete extirpation should be attempted unless there is evidence of distant metastases. Lieberman et al. (1966) concluded that for the majority extensive surgery proved of no value except for palliation. Regrettably the present case did not present early enough for a complete and effective removal of her neoplasm. Special mention should be made of the use of total body hypothermia at operation in case a period of temporary occlusion of the carotid vessels should be necessary because it increases the tolerance of the brain to the ischaemia of vascular occlusion. Westbury (i960) has recommended this precaution in the removal of carotid body tumours which may involve a vascular procedure other than dissection. Haemangiopericytoma is a comparatively rare vascular tumour first described by Stout and Murray (1942), since when over 300 cases have been reported in the literature. It can occur anywhere in the body where there are capillaries since the parent cell is the pericyte, a contractile cell forming a network on the outer capillary wall. Approximately 9 per cent arise in the orbit, sinuses, nasal and oropharyngeal regions (Backwinkel and Diddams, 1970). These authors recorded that involvement of these anatomical regions is fraught with a recurrence rate of 57 per cent. They emphasized that this tumour is highly malignant not in the usual five-year survival sense but over a lifetime. When metastases occur they are predominantly blood-borne and lymph node involvement is uncommon. Histologically this tumour must be differentiated from glomangioma glomus tumour, haemangioendothelioma, angioblastic meningioma and vascular sarcomas. The successful management of a haemangiopericytoma depends to a great extent on its size and location. When feasible the treatment of choice is wide local excision with adequate exposure. There is some indication that irradiation may exert some control over the tumour. Backwinkel and Diddams (1970) reported a 13 per cent cure rate in patients treated with radiotherapy alone, but they also inferred that adjunctive radiotherapy did not significantly improve the results of surgery. These cases underline the need for a multidisciplinary approach to rare tumours in the head and neck region which cut across the borders between specialties. The rarity of these tumours not only may lead to diagnostic difficulties but their surgical management may be hazardous. They require a high order of judgement since there is little established precedent for their treatment.
Two rare tumours involving the infratemporal fossa Summary
Two patients with malignant mesenchymal tumours encroaching on the infratemporal fossa are described. The extreme rarity of these tumours and the difficulties in differential diagnosis and treatment are discussed. Acknowledgements I am indebted to Professor D. F. N. Harrison for allowing me to report these patients. Thanks are also given to Professor L. Michaels for reporting the histology; to Mr. J. Connolly of the Department of Clinical Photography; and to Miss Damps for typing the manuscript. REFERENCES BACKWINKEL, K. D., and DIDDAMS, J. A. (1970) Cancer, 25, 896.
BURMAN, S. O. (1956) International Abstracts of Surgery, 102, 330. CALDWELL, J. B., HUGHES, K. W., and FADELL, E. J. (1956) Journal of Oral Surgery,
14, 342. CHRISTOPHERSON, W. M., FOOTE, F. W., and STEWART, F. W. (1952) Cancer, 5, 100.
CONLEY, J. (1970) Concepts in Head and Neck Surgery. George Thieme Verlag, Stuttgart, pp. 24-43. GINGRASS, R., MLADICK, R., PICKRELL, K., and PUNYAHOTRA, V. (1967)
Plastic
and Reconstructive Surgery, 40, 463. HARRISON, D. F. N. (1965) Journal of Laryngology and Otology, 81, 173. KARNAUCHOW, P. N., and MAGNER, D. (1963) Journal of Pathology and Bacteriology, 86, 169. LIEBERMAN, P. H., FOOTE, F. W., STEWART, F. W., and BERG, J. W. (1966) The
Journal of the American Medical Association, 198, 1047. RAVICH, A., STOUT, A. P., and RAVICH, R. A. (1945) Annals of Surgery, 121, 361.
SMETANA, H. F., and SCOTT, W. F. (1951) Military Surgeon, 109, 330. STOUT, A. P., and MURRAY, M. R. (1942) Annals of Surgery, 116, 26. USHIJIMA, H., and TAMURA, Z. (1957) Ada Pathologica Japonica, 7, 851. VAKIL, V. V., and SIRSAT, M. V. (1963) Indian Journal of Pathology and Bacteriology, 6, 19. VARGHESE, S., Xair, B., and JOSEPH, T. A. (1968) British Journal of Ophthalmology, 52, 713WESTBURY, G. (i960) British Journal of Surgery, 47, 605. The Institute of Laryngology and Otology 330-336 Gray's Inn Road London WCiX 8EE.
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