British Journal of Oral Surgery
14 (1976) 112-l 15
HAEMANGIOPERICYTOMA
OF THE A CASE
FLOOR
OF THE
MOUTH:
REPORT
S. MUKERJEE, F.R.C.S.(Eng.), F.R.C.S.(Ed.), F.A.C.S., RAKESH DIXIT, M.S. and H. B. MAHESHWARI, M.D., Ph.D., F.C.A.P.(U.S.A.), M.A.M.S. Department of Surgery, Willingdon Hospital, New Delhi, and Lady Hardinge Medical College, Department of Pathology, New Delhi Summary. A case of haemangiopericytoma of the floor of the mouth is discussed together with its treatment. Introduction Haemangiopericytomas are rare soft tissue tumours in which many proliferating blood vessels are present in a stroma of spindle shaped cells resembling the pericytes described by Zimmermann (quoted by Stout & Murray, 1942). Only two such cases have previously been reported as occurring in the floor of the mouth.
Case report Mr H. L., a 60-year-old male, was admitted for treatment of a swelling in the floor of the mouth and right submandibular region which had been present for six months. The patient had been without symptoms until six months previously when he had noticed a small swelling in the floor of the mouth. This had gradually increased in size but remained painless. Three months later the right submandibular region also appeared swollen. It was now difficult for him to swallow food adequately and over the next two months the patient lost some weight. There was no previous history of diabetes mellitus, haemoptysis or jaundice. His family and personal history were noncontributory. Physical examination revealed a man of average build, vital parameters being within normal limits. There was no lymphadenopathy. Cardiovascular and respiratory systems showed no abnormality and the liver was not palpable. Examination of the oral cavity revealed a smooth soft swelling on the right side of the floor of the mouth, displacing the tongue to the opposite side. It measured 10 cm in diameter and was extending into the right submandibular region. Bimanual palpation showed that it was soft, smooth, slightly tender and without any ulceration or evidence of infection. It did not appear to be attached either to bone or to skin. The tonsils were not enlarged and examination of the pharynx was normal. The haemoglobin was 12.5 g/d1 and the total leucocyte count was 10 x log/1 of which 71 per cent were polymorphonuclear cells. Urine and stools were normal. Serum proteins were 71 g/l with albumin of 40.8 g/l, thymol turbidity of 20 units and serum cholesterol 210 mg/dl of which 88 per cent was esterified. Serum alkaline phosphatase was 8 K.A. units. Blood urea and sugar were normal. X-ray of the mandible and chest were taken during his stay in hospital and were normal. (Received
6
January 1976; accepted 2 June 1976)
HAEMANGIOPERICYTOMA
OF
THE
FLOOR
OF
THE
MOUTH
113
FIG. 1. Excised specimen.
He was operated on under general anaesthesia but when endotracheal intubation was performed profuse haemorrhage occurred from the swelling. This was immediately controlled by ligation of the external carotid artery. After this the mucosa at the anterior and lateral margins of the swelling was incised and the tumour was shelled out quite easily (Fig. 1). The mucosa was sutured after securing complete haemostasis. The post-operative period was uneventful and patient was discharged from the hospital on the eighth post-operative day. Microscopic examination revealed that the tumour mass was lobulated and encapsulated, interspersed with dark haemorrhagic areas. It measured 11 cm in diameter. Microscopically the tumour mass was covered by oral mucosa with normal stratified squamous epithelium which was ulcerated in places. Microscopically the tumour was seen to be poorly encapsulated and consisted largely of spindle-shaped cells distributed around thin-walled vascular spaces (Fig. 2). These capillaries were lined by a single layer of endothelial cells (Fig. 3). Reticulin stain confirmed that these tumour cells lay outside the vascular lumen. No vascular or capsular invasion was noted. These findings are consistent with a diagnosis of haemangiopericytoma. Discussion The haemangiopericytoma was first described by Stout and Murray (1942). Haemangiopericytomas have been reported in many sites throughout the body, and constitute about 1.3 per cent of vascular tumours. Approximately 45 per cent are found in the musculoskeletal system and 33 per cent are found in the head and neck. Since 1942 approximately 300 cases have been identified and nearly 100 cases have been reported in the literature. Haemangiopericytomas of the oral cavity have been reported as arising from the base of the tongue (Stout, 1949), tongue (Kauffmann & Stout, 1960), buccal sulcus (Das & Gan, 1965) and gingivae (Rewell, 1968). The first case of haemangiopericytoma
114
BRITISH
JOURNAL
OF ORAL
FIG. 2. Histological appearance of haemangiopericytoma around thin-walled capillaries.
SURGERY
showing spindle-shaped (H&E. x 100)
pericytes arranged
FIG. 3. Histological appearance of haemangiopericytoma under high magnification. Note the thinwalled capillaries lined by a single layer of endothelial cells and spindle to oval-shaped cells lying outside in a loose matrix. (H&E. x 240)
of the floor of the mouth which was described by Small and Bloom (1959) had many similarities with the present case and the treatment was also similar. The tumour was shelled out in both instances, as also in the case reported by O’Brien and Brasfield (1965). Haemangiopericytomas are potentially malignant tumours. When they acquire malignant characteristics metastases are produced in the lungs and liver. Recurrence
HAEMANGIOPERICYTOMA
OF THE FLOOR
OF THE MOUTH
115
at the primary site is also common but the present patient was followed up for one year without any evidence of local recurrence or metastases. Histologically this tumour has to be differentiated from haemangioendothelioma, sclerosing angioma and fibrosarcoma. In the haemangiopericytoma the characteristic cells lie outside the vascular lumen, thus differentiating it from the haemangioendothelioma. A close relationship between proliferating blood capillaries and tumour cells can also be seen - a further distinguishing feature from the other two tumours. Haemangiopericytoma is one of those rare mesodermal tumours which are occasionally associated with hypoglycaemia (Cracker & Veith, 1965) although only a few such cases have been reported in the medical literature (Paullada et al., 1968). No case of haemangiopericytoma of the floor of the mouth associated with hypoglycaemia appears to have been described. The treatment in most of these tumours has been simple excision or enucleation since they are slow growing and usually well encapsulated, but recurrence at the primary site is common. There has been as yet no evidence of recurrence or metastases in the present case. References O’Brien, P. & Brasfield, R. D. (1965). Cancer, 18, 249. Cracker, D. W. & Veitch, F. J. (1965). AnnaIs ofSurgery, 110,215. Das, A. K. & Gans, B. J. (1965). Journal of Oral Surgery, 23, 456. Ferguson, J. O., Clagett, 0. T. & McDonald, J. R. (1954). Surgery, 36, 320. Kauffmann. S. L. & Stout. A. P. (1960). Cancer. 13. 695. Paullada, J: J., Lisci-Garmilla, A., Gonzales-A&&, A., Jurado-Mendoza, J., Quijano-Narezo, Gomez-Peralta, L. & Dorrja-Medina, M. (1968). American Journal of Medicine, 44, 990. Rewell, R. E., (1968). Journal OfLaryngology and Orology, 82,261. Small, I. A. & Bloom, H. J. (1959). Journal of Oral Surgery, 17(2), 65. Stout, A. P. (1949). Cancer, 2, 1027. Stout, A. P. & Murray M. R. (1942). Annals of Surgery, 116, 26. Zimmermann, K. W. (1923). Zeitschrif fur Anatomie und Entwicklungsgeschicte, 68, 29.
14/2-B
M.,
14 (1976) 112-l 15
HAEMANGIOPERICYTOMA
OF THE A CASE
FLOOR
OF THE
MOUTH:
REPORT
S. MUKERJEE, F.R.C.S.(Eng.), F.R.C.S.(Ed.), F.A.C.S., RAKESH DIXIT, M.S. and H. B. MAHESHWARI, M.D., Ph.D., F.C.A.P.(U.S.A.), M.A.M.S. Department of Surgery, Willingdon Hospital, New Delhi, and Lady Hardinge Medical College, Department of Pathology, New Delhi Summary. A case of haemangiopericytoma of the floor of the mouth is discussed together with its treatment. Introduction Haemangiopericytomas are rare soft tissue tumours in which many proliferating blood vessels are present in a stroma of spindle shaped cells resembling the pericytes described by Zimmermann (quoted by Stout & Murray, 1942). Only two such cases have previously been reported as occurring in the floor of the mouth.
Case report Mr H. L., a 60-year-old male, was admitted for treatment of a swelling in the floor of the mouth and right submandibular region which had been present for six months. The patient had been without symptoms until six months previously when he had noticed a small swelling in the floor of the mouth. This had gradually increased in size but remained painless. Three months later the right submandibular region also appeared swollen. It was now difficult for him to swallow food adequately and over the next two months the patient lost some weight. There was no previous history of diabetes mellitus, haemoptysis or jaundice. His family and personal history were noncontributory. Physical examination revealed a man of average build, vital parameters being within normal limits. There was no lymphadenopathy. Cardiovascular and respiratory systems showed no abnormality and the liver was not palpable. Examination of the oral cavity revealed a smooth soft swelling on the right side of the floor of the mouth, displacing the tongue to the opposite side. It measured 10 cm in diameter and was extending into the right submandibular region. Bimanual palpation showed that it was soft, smooth, slightly tender and without any ulceration or evidence of infection. It did not appear to be attached either to bone or to skin. The tonsils were not enlarged and examination of the pharynx was normal. The haemoglobin was 12.5 g/d1 and the total leucocyte count was 10 x log/1 of which 71 per cent were polymorphonuclear cells. Urine and stools were normal. Serum proteins were 71 g/l with albumin of 40.8 g/l, thymol turbidity of 20 units and serum cholesterol 210 mg/dl of which 88 per cent was esterified. Serum alkaline phosphatase was 8 K.A. units. Blood urea and sugar were normal. X-ray of the mandible and chest were taken during his stay in hospital and were normal. (Received
6
January 1976; accepted 2 June 1976)
HAEMANGIOPERICYTOMA
OF
THE
FLOOR
OF
THE
MOUTH
113
FIG. 1. Excised specimen.
He was operated on under general anaesthesia but when endotracheal intubation was performed profuse haemorrhage occurred from the swelling. This was immediately controlled by ligation of the external carotid artery. After this the mucosa at the anterior and lateral margins of the swelling was incised and the tumour was shelled out quite easily (Fig. 1). The mucosa was sutured after securing complete haemostasis. The post-operative period was uneventful and patient was discharged from the hospital on the eighth post-operative day. Microscopic examination revealed that the tumour mass was lobulated and encapsulated, interspersed with dark haemorrhagic areas. It measured 11 cm in diameter. Microscopically the tumour mass was covered by oral mucosa with normal stratified squamous epithelium which was ulcerated in places. Microscopically the tumour was seen to be poorly encapsulated and consisted largely of spindle-shaped cells distributed around thin-walled vascular spaces (Fig. 2). These capillaries were lined by a single layer of endothelial cells (Fig. 3). Reticulin stain confirmed that these tumour cells lay outside the vascular lumen. No vascular or capsular invasion was noted. These findings are consistent with a diagnosis of haemangiopericytoma. Discussion The haemangiopericytoma was first described by Stout and Murray (1942). Haemangiopericytomas have been reported in many sites throughout the body, and constitute about 1.3 per cent of vascular tumours. Approximately 45 per cent are found in the musculoskeletal system and 33 per cent are found in the head and neck. Since 1942 approximately 300 cases have been identified and nearly 100 cases have been reported in the literature. Haemangiopericytomas of the oral cavity have been reported as arising from the base of the tongue (Stout, 1949), tongue (Kauffmann & Stout, 1960), buccal sulcus (Das & Gan, 1965) and gingivae (Rewell, 1968). The first case of haemangiopericytoma
114
BRITISH
JOURNAL
OF ORAL
FIG. 2. Histological appearance of haemangiopericytoma around thin-walled capillaries.
SURGERY
showing spindle-shaped (H&E. x 100)
pericytes arranged
FIG. 3. Histological appearance of haemangiopericytoma under high magnification. Note the thinwalled capillaries lined by a single layer of endothelial cells and spindle to oval-shaped cells lying outside in a loose matrix. (H&E. x 240)
of the floor of the mouth which was described by Small and Bloom (1959) had many similarities with the present case and the treatment was also similar. The tumour was shelled out in both instances, as also in the case reported by O’Brien and Brasfield (1965). Haemangiopericytomas are potentially malignant tumours. When they acquire malignant characteristics metastases are produced in the lungs and liver. Recurrence
HAEMANGIOPERICYTOMA
OF THE FLOOR
OF THE MOUTH
115
at the primary site is also common but the present patient was followed up for one year without any evidence of local recurrence or metastases. Histologically this tumour has to be differentiated from haemangioendothelioma, sclerosing angioma and fibrosarcoma. In the haemangiopericytoma the characteristic cells lie outside the vascular lumen, thus differentiating it from the haemangioendothelioma. A close relationship between proliferating blood capillaries and tumour cells can also be seen - a further distinguishing feature from the other two tumours. Haemangiopericytoma is one of those rare mesodermal tumours which are occasionally associated with hypoglycaemia (Cracker & Veith, 1965) although only a few such cases have been reported in the medical literature (Paullada et al., 1968). No case of haemangiopericytoma of the floor of the mouth associated with hypoglycaemia appears to have been described. The treatment in most of these tumours has been simple excision or enucleation since they are slow growing and usually well encapsulated, but recurrence at the primary site is common. There has been as yet no evidence of recurrence or metastases in the present case. References O’Brien, P. & Brasfield, R. D. (1965). Cancer, 18, 249. Cracker, D. W. & Veitch, F. J. (1965). AnnaIs ofSurgery, 110,215. Das, A. K. & Gans, B. J. (1965). Journal of Oral Surgery, 23, 456. Ferguson, J. O., Clagett, 0. T. & McDonald, J. R. (1954). Surgery, 36, 320. Kauffmann. S. L. & Stout. A. P. (1960). Cancer. 13. 695. Paullada, J: J., Lisci-Garmilla, A., Gonzales-A&&, A., Jurado-Mendoza, J., Quijano-Narezo, Gomez-Peralta, L. & Dorrja-Medina, M. (1968). American Journal of Medicine, 44, 990. Rewell, R. E., (1968). Journal OfLaryngology and Orology, 82,261. Small, I. A. & Bloom, H. J. (1959). Journal of Oral Surgery, 17(2), 65. Stout, A. P. (1949). Cancer, 2, 1027. Stout, A. P. & Murray M. R. (1942). Annals of Surgery, 116, 26. Zimmermann, K. W. (1923). Zeitschrif fur Anatomie und Entwicklungsgeschicte, 68, 29.
14/2-B
M.,
