Abdullah Jan ( Department of ENT, Khyber Medical College, Peshawar. )
Iftikhar Ahmed ( Department of ENT, Khyber Medical College, Peshawar. )
Haemangiopericytomas are uncommon vascular tumours found in almost every region of the body. The common sites are the soft tissues of limbs and head and neck. The incidence in head and neck ranges from 15 to 25% of all haemangiopericytomas1,2. The uncertainty of the tumours is because of difficult histopathological nature and unpredictable biological behaviour. Its origin from the pericytes was described earlier by Stout and Murray3 who described these tumours clinically to be originating from the pericytes.
M.A. 50 years male was referred to us from Ophthalmic Department with the history of left sided swelling of cheek, nasal obstruction and proptosis for the last 5 months. Biopsy was done previously in a peripheral hospital but the report not made available to us. He was admitted on the same day for further management. On examination, he was found to have marked swelling of his left cheek with a small infra-orbital ulcer resulting from his previous biopsy (Figure):
He also had total left sided nasal obstruction alongwith marked proptosis. The hard palate was also pushed down on the left side. The nasopharynx was clear and the eye sight was normal. There were no palpable lymph nodes in the neck. Routine laboratory findings were within normal range. X-Rays of paranasal sinuses showed erosion of the roof and medial wall of left maxillary sinus. An exploratory Caidwell Luc operation was carried out and a large mass filling the whole of the maxillary sinus found, which was removed. The histology showed malignant haemangiopericytoma. After discussion with the patient and his relations, a subtotal maxillectomy was performed after 4 days. He did not consent to total maxillectomy with excentration of his eye because of his normal vision. The excised specimen was again sent for histology and the report was the same. The patient did very well for first six months after operation, then developed recurrence and because of his refusal to any further surgery, he was referred to the Radiotherapy department. He died during the course of Radiotherapy 10 months after surgery.
S. A., 18 years girl, presented with the history of right sided nasal obstruction, epistaxis and external deformity of her nose. Ten years back she had a surgery on her nose for asimilar problem and the mass removed at that time was reported as haemangioma. On examination, there was a large polypoid mass filling the whole of right nasal fossa with marked external deformity. The mass, after decongesting the nose with 4% lignocane and Adrenaline 1:100,000 was found to be firm, and not bleeding to touch. Nasopharynx was clear. X-Rays of her paranasal sinuses showed a hazy right maxillary sinus. The right lateral nasal bone was pushed laterally, but there was no erosion. The rest of investigations were normal. Examination under anaesthesia was carried out and the mass was found to be cQming from the nasal septurn and going into right middle meatus but not adherent to the lateral ~wull. It was too big to be removed through the nose and in order to ensure total clearance, a right lateral rhinotomy was carried out. There was no extensioninto ethmoids. The mass was removed in toto and was sent for histological examination. The report came as haemangiopericytoma. The postoperative course was uneventful except a band of adhesion which was divided. The external deformity is gradually reducing and she has no scar. She is being followed up regularly at one month intervals and has no sign of recurrence todate.
Haemangiopericytoma is a rare tumour which may arise at any site and in any age or sex; however some workers describe it as being most commonly seen in the middle age4. These tumours in general are universally accepted as highly malignant but the ones in head and neck region are less aggressive5. However these head and neck tumours have a high recurrence rate i.e. about 40%. Wide local excision is therefore recommended as the treatment of choice6. Radiotherapy is not helpful because of radioresistance7. On the other hand chemotherapy has not yet received the general consensus of approval8. Auguste and his coworkers9 reported success in treating several cases by a combination of Radiotherapy and chemotherapy. Gudrun10, as a result of his elaborate survey came to the conclusion that the treatment of choice is wide surgical excision followed by life-long follow up. This is also our routine in localised disease. However, tumours with distant metastasis have to be treated with combination therapy.
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