Journal of the Royal Society of Medicine Volume 72 August 1979
615
Evans D J, William D G, Peters D K, Sissons J G P, Boulton-Jones J M, Ogg C S, Cameron J S & Hoffbrand B I (1973) British Medical Journal iii, 326 Maurice T D (1978) British Medical Journal ii, 831 Ozowa T, Pluss R, Lacher J, Boedecker E, Guggenheim S, Hammond W & McIntosh R (1975) Quarterly Journal of Medicine 44, 523 Row P G, Cameron J S, Turner D R, Evans D J, White R H R, Ogg C S, Chantler C & Brown C B (1975) Quarterly Journal of Medicine 44, 207
Massive chondroma of skull base' N S B Tanner MA FRCS H J Shaw MA FRCS P Clifford MD MCh Head and Neck Unit, Royal Marsden Hospital, Fulham Road, London SW3
This case is reported to illustrate certain problems in the investigation and management of tumours of the postnasal space. Chondromas in the head and neck region have been reported originating from the nasal cartilages and septum, eustachian cushions and larynx. Those arising from the basisphenoid and basiocciput are extremely rare. Two cases have been reported in recent literature (Timmis 1959, Falconer et al. 1968). The postnasal space is difficult to examine. Straight X-rays may show a soft tissue mass and polytomography will demonstrate bone destruction. Computerized tomography has the advantage of high contrast discrimination. Slight differences in density between adjacent soft tissues become visible (Thawley et al. 1978). Operative access to the postnasal space is difficult. Transnasal, transantral and transpalatal operative approaches give limited exposure. A transcranial approach combined with a Moures lateral rhinotomy achieves good surgical exposure (Clifford 1977). Case report A 27-year-old man presented with acute sinusitis and deafness. He was found to have nasal speech, blockage of both posterior choanae and gross retraction of the left tympanic membrane. Examination under general anaesthesia revealed a firm submucosal mass, mainly left-sided, which arose from the posterior nasopharyngeal wall. The lesion extended along the nasopharyngeal vault down to the level of the eustachian cushions, blocking the left eustachian tubal orifice. A biopsy was taken and a chondroma was diagnosed (Figure 1). The findings of straight Xray, xerography, and polytomography were confirmed by computerized axial tomography at the levels of the orbits and left maxillary antrum (Figure 2B). A dense tumour was shown to extend from the anterior ethmoidal area through the posterior ethmoidal cells, sphenoid sinus, basisphenoid, and basiocciput, to end at the dura of the posterior cranial fossa. The tumour caused the lateral wall of the left ethmoidal labyrinth to bulge into the left orbit. Inferiorly the tumour extended to the level of the palate and into the left maxillary antrum. The tumour had destroyed the posterior nasal septum, the medial wall of the left maxillary antrum, the posterior ethmoidal labyrinth, the lateral wall of the left sphenoidal sinus and the left pterigoid plates. Initially conservative operative procedures were adopted. In July 1976, using the transnasal, transantral and transpalatal approaches, 39 g of tumour was removed. An anterior myringotomy revealed thin yellow fluid and a grommet was inserted. Postoperatively his condition 1 Case presented to Clinical Section 12 May 1978. Accepted 16 June 1978
0 1 41-0768/79/080615-04/$O 1.00/0
,."
1979 The Royal Society of Medicine
616
Journal of the Royal Society of Medicine Volume 72 August 1979
Figure 1. The tumour is composed of clumps of chondrocytes set in a cartilaginous matrix. There is little cellular pleomorphism and mitosis are not seen. H&E. xlOO
Figure 2. Computerized axial tomographs at the level of the maxillary antrum. A, normal. B, chondroma in the post nasal space. c, appearances after transcranial operation
was improved though he developed a small palatal fistula. This was sealed with a dental plate. One year later, in July 1977, the patient returned with symptoms suggestive of tumour compression of the left orbit (proptosis, partial paresis of the abducent nerve, and swollen optic disc). Repeated computerized axial tomography at the orbital level showed a 2 mm proptosis and distortion of the left medial orbital wall (Figure 3). Radical surgery was deemed necessary to save the left eye and extirpate the tumour. A block resection of the tumour was accomplished using a combined transcranial and left lateral rhinotomy approach (Clifford 1977). A total of 60 g of tumour was removed. The posterior resection line was indeed found to be the dura of the posterior cranial fossa. The patient made an uneventful postoperative recovery and was discharged sixteen days later. Repeat
Journal of the Royal Society of Medicine Volume 72 August 1979
617
Figure 3. Enlarged computerized axial tomograph at
the orbital level one year after incomplete tumour _ _ excision using transnasal, transantral and tran-
spalatal operative approaches
computerized tomography showed the extensive surgical resection and complete removal of tumour (Figure 2c). The patient remains well and at work one year after the transcranial operation. He has diplopia on distant vision due to a divergence weakness, thought to indicate incomplete recovery of the left abducent nerve. Symptomatic relief is afforded by prism perspex incorporated into a pair of spectacles. Discussion The differential diagnosis of lesions arising in the basisphenoid and basiocciput include well differentiated chondrosarcomas (Jones 1973), chordomas (especially chondroid chordomas: Heffelfinger et al. 1973) and juvenile nasopharyngeal angiofibromas (Wirth & Shimkin 1943). Benign chondromas in the head and neck region destroy adjacent tissues and should be regarded clinically as locally invasive. Adequate surgical removal is required, as most are radioresistant (Jones 1973). The basisphenoid and basiocciput are preformed in cartilage, unlike the membrane bones of the skull vault and face. Growth in length of the skull base at the spheno-occipital synchrondrosis keeps pace with the backward growth of the palate. Ossification begins about the 18th year and fusion about the 25th year. Chondromas are thought to arise either from clearly differentiated cartilage cells (Lichtenstein 1965) or from embryological cartilage 'cell rests' which escaped resorption during enchrondrial ossification (Ringertz 1938). In this case, where all the basisphenoid and most of the basiocciput was involved by tumour, it is suggested that the chondroma arose, before ossification began in the 18th year, from clearly differentiated cartilage cells. This supports Lichtenstein's hypothesis. The value of computerized tomography in the management of this difficult case was immense. The noninvasive, repeatable nature ofthe technique allowed the full extent of the soft tissue lesion and its anatomical relations to be shown. This acted both as a guide to treatment and to follow up. The transcranial approach, modified by Clifford, is relatively straightforward. It gives good access to the postnasal space and has a low morbidity. During the operation the intracranial pressure is reduced to facilitate traction on the frontal lobe by an infusion of mannitol 20% in water. This obviates cerebrospinal fluid drainage through a ventricular or lumbar subarachnoid catheter. Hypotensive anaesthesia is not needed, and indeed may be dangerous due to retraction anaemia on the frontal lobe (Aserman 1953). Facial deformity is minimal and the olfactory nerves are the only cranial nerves to be sacrificed. This operation can be used for lesions of the frontal sinus, the ethmoidal sinuses, and sphenoidal sinus, even if the dura overlying the cribriform plate is involved. The technique can be used alone, as in the case described, or in combination with orbital exenteration or maxillectomy.
618
Journal of the Royal Society of Medicine Volume 72 August 1979
References Aserman D (1953) British Medical Journal i, 961-964 Clifford P (1977) Clinical Otolaryngology 2, 115-130 Falconer M A, Bailey I C & Duchen L W (1968) Journal ofNeurosurgery 29, 261-275 (Case 5) Heffelfinger M J, Dahlin D C, MacCarthy C S & Beabout J W (1973) Cancer 32, 410-420 Jones H M (1973) Journal ofLaryngology and Otology 87, 135-151 Lichtenstein L (1965) Bone Tumours. 3rd edn. C V Mosby, St Louis; p 202 Ringertz N (1938) Acta Otolaryngologica Suppl. 27; pp 187-188 Thawley S E, Gado M & Fuller T R (1978) Laryngoscope 88, 451-459 Timmis P (1959) Journal of Laryngology and Otology 73, 383-387 (Case 1) Wirth J E & Shimkin M B (1943) Archives of Pathology 36, 83-88
Fibrosarcoma of bladder exhibiting endocrine characteristics of phaeochromocytoma' R A Keenan FRCSEd2 J D Buchanan MRcPath Royal Naval Hospital, Haslar, Hampshire Mr R B, aged 53, a caretaker, was referred to hospital with a diagnosis of chronic urinary retention and overflow incontinence. He had a three-month history of 2-hourly frequency by day, hourly nocturia, and poor urinary stream. There was also marked hesitancy and terminal dribbling, and he had the feeling he was not emptying his bladder. His previous medical history did not contribute but he had lost more than 5 kg weight in the last year despite a healthy appetite. On clinical examination he looked well and there was no superficial lymphadenopathy. The lungs appeared clear and there were no signs of cardiac failure. Blood pressure was 130/80 mmHg. There was a non-tender swelling arising from the pelvis in the midline which reached the umbilicus. On rectal examination the prostate appeared to be large and smooth. Retention of urine was suspected, but only 100 ml of clear urine was obtained.
Investigations Urinalysis was normal. Results of haematological examination were: Hb 13 g/dl, WBC 10 000/mm3, ESR 25 mm in the first hour (Westergren). Blood urea, electrolytes and acid phosphatase were all normal. Chest X-ray showed clear lung fields. An intravenous urogram showed prompt and equal excretion of the contrast, and normal upper tracts; there was no evidence of back pressure and the ureters were seen throughout their length but were displaced laterally by a large, soft tissue mass within the pelvis which compressed the bladder. Under general anaesthetic, bimanual examination revealed a large fixed tumour occupying the entire pelvis. The prostate was diminutive and the seminal vesicles were palpable on each side. On cystourethroscopy the penile urethra was normal but the prostatic urethra could only be entered and visualized by acutely depressing the eye-piece end of the instrument. As the instrument was advanced in this plane into the bladder it was apparent that the bladder cavity was reduced to a mere slit by a large extrinsic mass posterior to it. There was oedema of the posterior wall. Through a small suprapubic incision, cystotomy was performed to define the tumour, which was found to be inseparable from the posterior wall of the bladder and fixed to the right side of the pelvis. During handling of the tumour, there was a dramatic rise in the blood pressure, from 140/90 to 240/140 mmHg. This suggested the possibility of a phaeochromocytoma, since none 1 Case presented to Section of Urology, 26 January 1978. Accepted 4 October 1978 2 Present address: Dumfries and Galloway Royal Infirmary, Dumfries, Scotland
0141-0768/79/080618-03/$01.00/0
If"
1979 The Royal Society of Medicine
615
Evans D J, William D G, Peters D K, Sissons J G P, Boulton-Jones J M, Ogg C S, Cameron J S & Hoffbrand B I (1973) British Medical Journal iii, 326 Maurice T D (1978) British Medical Journal ii, 831 Ozowa T, Pluss R, Lacher J, Boedecker E, Guggenheim S, Hammond W & McIntosh R (1975) Quarterly Journal of Medicine 44, 523 Row P G, Cameron J S, Turner D R, Evans D J, White R H R, Ogg C S, Chantler C & Brown C B (1975) Quarterly Journal of Medicine 44, 207
Massive chondroma of skull base' N S B Tanner MA FRCS H J Shaw MA FRCS P Clifford MD MCh Head and Neck Unit, Royal Marsden Hospital, Fulham Road, London SW3
This case is reported to illustrate certain problems in the investigation and management of tumours of the postnasal space. Chondromas in the head and neck region have been reported originating from the nasal cartilages and septum, eustachian cushions and larynx. Those arising from the basisphenoid and basiocciput are extremely rare. Two cases have been reported in recent literature (Timmis 1959, Falconer et al. 1968). The postnasal space is difficult to examine. Straight X-rays may show a soft tissue mass and polytomography will demonstrate bone destruction. Computerized tomography has the advantage of high contrast discrimination. Slight differences in density between adjacent soft tissues become visible (Thawley et al. 1978). Operative access to the postnasal space is difficult. Transnasal, transantral and transpalatal operative approaches give limited exposure. A transcranial approach combined with a Moures lateral rhinotomy achieves good surgical exposure (Clifford 1977). Case report A 27-year-old man presented with acute sinusitis and deafness. He was found to have nasal speech, blockage of both posterior choanae and gross retraction of the left tympanic membrane. Examination under general anaesthesia revealed a firm submucosal mass, mainly left-sided, which arose from the posterior nasopharyngeal wall. The lesion extended along the nasopharyngeal vault down to the level of the eustachian cushions, blocking the left eustachian tubal orifice. A biopsy was taken and a chondroma was diagnosed (Figure 1). The findings of straight Xray, xerography, and polytomography were confirmed by computerized axial tomography at the levels of the orbits and left maxillary antrum (Figure 2B). A dense tumour was shown to extend from the anterior ethmoidal area through the posterior ethmoidal cells, sphenoid sinus, basisphenoid, and basiocciput, to end at the dura of the posterior cranial fossa. The tumour caused the lateral wall of the left ethmoidal labyrinth to bulge into the left orbit. Inferiorly the tumour extended to the level of the palate and into the left maxillary antrum. The tumour had destroyed the posterior nasal septum, the medial wall of the left maxillary antrum, the posterior ethmoidal labyrinth, the lateral wall of the left sphenoidal sinus and the left pterigoid plates. Initially conservative operative procedures were adopted. In July 1976, using the transnasal, transantral and transpalatal approaches, 39 g of tumour was removed. An anterior myringotomy revealed thin yellow fluid and a grommet was inserted. Postoperatively his condition 1 Case presented to Clinical Section 12 May 1978. Accepted 16 June 1978
0 1 41-0768/79/080615-04/$O 1.00/0
,."
1979 The Royal Society of Medicine
616
Journal of the Royal Society of Medicine Volume 72 August 1979
Figure 1. The tumour is composed of clumps of chondrocytes set in a cartilaginous matrix. There is little cellular pleomorphism and mitosis are not seen. H&E. xlOO
Figure 2. Computerized axial tomographs at the level of the maxillary antrum. A, normal. B, chondroma in the post nasal space. c, appearances after transcranial operation
was improved though he developed a small palatal fistula. This was sealed with a dental plate. One year later, in July 1977, the patient returned with symptoms suggestive of tumour compression of the left orbit (proptosis, partial paresis of the abducent nerve, and swollen optic disc). Repeated computerized axial tomography at the orbital level showed a 2 mm proptosis and distortion of the left medial orbital wall (Figure 3). Radical surgery was deemed necessary to save the left eye and extirpate the tumour. A block resection of the tumour was accomplished using a combined transcranial and left lateral rhinotomy approach (Clifford 1977). A total of 60 g of tumour was removed. The posterior resection line was indeed found to be the dura of the posterior cranial fossa. The patient made an uneventful postoperative recovery and was discharged sixteen days later. Repeat
Journal of the Royal Society of Medicine Volume 72 August 1979
617
Figure 3. Enlarged computerized axial tomograph at
the orbital level one year after incomplete tumour _ _ excision using transnasal, transantral and tran-
spalatal operative approaches
computerized tomography showed the extensive surgical resection and complete removal of tumour (Figure 2c). The patient remains well and at work one year after the transcranial operation. He has diplopia on distant vision due to a divergence weakness, thought to indicate incomplete recovery of the left abducent nerve. Symptomatic relief is afforded by prism perspex incorporated into a pair of spectacles. Discussion The differential diagnosis of lesions arising in the basisphenoid and basiocciput include well differentiated chondrosarcomas (Jones 1973), chordomas (especially chondroid chordomas: Heffelfinger et al. 1973) and juvenile nasopharyngeal angiofibromas (Wirth & Shimkin 1943). Benign chondromas in the head and neck region destroy adjacent tissues and should be regarded clinically as locally invasive. Adequate surgical removal is required, as most are radioresistant (Jones 1973). The basisphenoid and basiocciput are preformed in cartilage, unlike the membrane bones of the skull vault and face. Growth in length of the skull base at the spheno-occipital synchrondrosis keeps pace with the backward growth of the palate. Ossification begins about the 18th year and fusion about the 25th year. Chondromas are thought to arise either from clearly differentiated cartilage cells (Lichtenstein 1965) or from embryological cartilage 'cell rests' which escaped resorption during enchrondrial ossification (Ringertz 1938). In this case, where all the basisphenoid and most of the basiocciput was involved by tumour, it is suggested that the chondroma arose, before ossification began in the 18th year, from clearly differentiated cartilage cells. This supports Lichtenstein's hypothesis. The value of computerized tomography in the management of this difficult case was immense. The noninvasive, repeatable nature ofthe technique allowed the full extent of the soft tissue lesion and its anatomical relations to be shown. This acted both as a guide to treatment and to follow up. The transcranial approach, modified by Clifford, is relatively straightforward. It gives good access to the postnasal space and has a low morbidity. During the operation the intracranial pressure is reduced to facilitate traction on the frontal lobe by an infusion of mannitol 20% in water. This obviates cerebrospinal fluid drainage through a ventricular or lumbar subarachnoid catheter. Hypotensive anaesthesia is not needed, and indeed may be dangerous due to retraction anaemia on the frontal lobe (Aserman 1953). Facial deformity is minimal and the olfactory nerves are the only cranial nerves to be sacrificed. This operation can be used for lesions of the frontal sinus, the ethmoidal sinuses, and sphenoidal sinus, even if the dura overlying the cribriform plate is involved. The technique can be used alone, as in the case described, or in combination with orbital exenteration or maxillectomy.
618
Journal of the Royal Society of Medicine Volume 72 August 1979
References Aserman D (1953) British Medical Journal i, 961-964 Clifford P (1977) Clinical Otolaryngology 2, 115-130 Falconer M A, Bailey I C & Duchen L W (1968) Journal ofNeurosurgery 29, 261-275 (Case 5) Heffelfinger M J, Dahlin D C, MacCarthy C S & Beabout J W (1973) Cancer 32, 410-420 Jones H M (1973) Journal ofLaryngology and Otology 87, 135-151 Lichtenstein L (1965) Bone Tumours. 3rd edn. C V Mosby, St Louis; p 202 Ringertz N (1938) Acta Otolaryngologica Suppl. 27; pp 187-188 Thawley S E, Gado M & Fuller T R (1978) Laryngoscope 88, 451-459 Timmis P (1959) Journal of Laryngology and Otology 73, 383-387 (Case 1) Wirth J E & Shimkin M B (1943) Archives of Pathology 36, 83-88
Fibrosarcoma of bladder exhibiting endocrine characteristics of phaeochromocytoma' R A Keenan FRCSEd2 J D Buchanan MRcPath Royal Naval Hospital, Haslar, Hampshire Mr R B, aged 53, a caretaker, was referred to hospital with a diagnosis of chronic urinary retention and overflow incontinence. He had a three-month history of 2-hourly frequency by day, hourly nocturia, and poor urinary stream. There was also marked hesitancy and terminal dribbling, and he had the feeling he was not emptying his bladder. His previous medical history did not contribute but he had lost more than 5 kg weight in the last year despite a healthy appetite. On clinical examination he looked well and there was no superficial lymphadenopathy. The lungs appeared clear and there were no signs of cardiac failure. Blood pressure was 130/80 mmHg. There was a non-tender swelling arising from the pelvis in the midline which reached the umbilicus. On rectal examination the prostate appeared to be large and smooth. Retention of urine was suspected, but only 100 ml of clear urine was obtained.
Investigations Urinalysis was normal. Results of haematological examination were: Hb 13 g/dl, WBC 10 000/mm3, ESR 25 mm in the first hour (Westergren). Blood urea, electrolytes and acid phosphatase were all normal. Chest X-ray showed clear lung fields. An intravenous urogram showed prompt and equal excretion of the contrast, and normal upper tracts; there was no evidence of back pressure and the ureters were seen throughout their length but were displaced laterally by a large, soft tissue mass within the pelvis which compressed the bladder. Under general anaesthetic, bimanual examination revealed a large fixed tumour occupying the entire pelvis. The prostate was diminutive and the seminal vesicles were palpable on each side. On cystourethroscopy the penile urethra was normal but the prostatic urethra could only be entered and visualized by acutely depressing the eye-piece end of the instrument. As the instrument was advanced in this plane into the bladder it was apparent that the bladder cavity was reduced to a mere slit by a large extrinsic mass posterior to it. There was oedema of the posterior wall. Through a small suprapubic incision, cystotomy was performed to define the tumour, which was found to be inseparable from the posterior wall of the bladder and fixed to the right side of the pelvis. During handling of the tumour, there was a dramatic rise in the blood pressure, from 140/90 to 240/140 mmHg. This suggested the possibility of a phaeochromocytoma, since none 1 Case presented to Section of Urology, 26 January 1978. Accepted 4 October 1978 2 Present address: Dumfries and Galloway Royal Infirmary, Dumfries, Scotland
0141-0768/79/080618-03/$01.00/0
If"
1979 The Royal Society of Medicine
