Pheochromocytoma of the urinary bladder (report of 2 cases with review of literature).SS Punekar, AA Gulanikar, MK Sobti, SY Sane, DS Pardanani
Keywords: Adult, Bladder Neoplasms, diagnosis,pathology,surgery,Case Report, Child, Female, Human, Male, Pheochromocytoma, diagnosis,pathology,surgery,
Although pheochromocytoma of the urinary bladder is a rare but well known clinical entity, it is invariably a fascinating clinical encountrance. During a short span of 7 months (November 84 to April 1985) two cases were treated in the Department of Urology at the K.E.M. Hospital, Bombay. Here we are presenting our two cases, discussing their mode of presentation and histopathological diagnosis along with a brief review of literature.
A twelve year old male was admitted in the medical ward in October 1984 with history of severe hypertension. Detailed interrogation revealed occasional attacks of throbbing headache at the time of defaecation and micturition. He was asymptomatic in between these episodes. Four months prior to admission, he started getting attacks of excessive sweating with headache and palpitation. His family physician detected hypertension (180/120 mm Hg) and treated him with alpha methyl dopa and hydrochlorthiazide but had no relief and was therefore referred to our institution.
On examination, he was fully conscious with the pulse 86/min and B.P. 170/120 mm of Hg. There was no abdominal mass or bruit. Systemic examination was not contributory. Fundoscopic examination showed hypertensive retinopathy. A provisional clinical diagnosis of pheochromocytoma or reno-vascular hypertension was made.
The biochemical investigations were within normal limits except VMA, which was significantly raised (19.6 mg with a control of 1.8-7 mg). Ultrasonography showed a mass in the left postero-lateral wall of the urinary bladder measuring 5 x 4 cm. Aortogram showed a large hypervascular mass (5 x 4 cm.) in the left lateral wall of the urinary bladder supplied by the inferior vesical artery. Immediately after injection of contrast, the blood pressure rose to 240 mm Hg systolic, which was controlled with a sodium nitropruside drip. The adrenal glands were normal.
A week before surgery he was treated with phenoxybenzamine 30 mg b.d. and propranolol 20 mg b.d. Inspite of this treatment his B.P. remained 160/110-150/110 mm Hg. He was sedated pre-operatively with diazepam. Anaesthesia was induced with thiopentonic sodium and pancuronium bromide.
Through an infra-umbilical midline incision the bladder was approached extraperitoneally. The bladder was opened. A mass 5 x 3 x 2 cm in the region of the left lateral wall around the left ureteral orifice was excised and the ureter reimplanted. The B.P. dropped to 80 mm Hg immediately after removal of tumour. This was treated with rapid infusions intravenous fluid and blood.
He was normotensive for 1 week after surgery but later required low dosage of antihypertensive drugs to control his hypertension.
A forty year old female presented with profuse painless haematuria in a state of shock in a surgical unit in April 1985. She was transferred from a private gynaecological nursing home after being resuscitated from a similar episode 48 hour earlier. She was thought to have bleeding per vaginum but on urethral catheterization was detected to be a case of haematuria. She was given 4 units of blood.
On examination she was pale, with cold extremities, pulse 120/min and B.P. 100/80 mm of Hg. The urinary bladder was palpable upto the umbilicus. Her Hb was 5 gm% for which two units of blood transfusion were given. Renal chemistry was normal. On cystoscopy 700 ml of blood clots were evacuated and due to bleeding obsuring vision, details could not be visualized. The urine cleared in 48 hours and other parameters also improved. An intravenous urography showed normal upper urinary tracts with multiple filling defects the bladder suggestive blood clots. Ultrasonography was not available. Four days later she developed another episode of haematuria. She was therefore prepared for surgery with 24 hour bowel preparation and blood transfusions.
Cystoscopic examination performed just before surgery showed a solid growth near the dome of the bladder measuring 1.5 x 2 cm with ill-defined margins, smooth surface and ulcerated mucosa in the centre. An active bleeding point was seen in the ulcerated area. In view of the patient's condition and nature of growth transurethral resection or biopsy was deffered and a decision for open surgery was made.
The bladder was approached through a midline infraumbilical incision. A firm mass 7 x 5 x 2 cm was felt towards the dome of the bladder. The bladder was opened and the mass was excised along with a 1.5 cm margin.
During these manipulations of the tumour the patient's B. P. rose to 200-220 mm Hg systolic which dropped to 80 mm Hg after excision of tumour. This lead us to clinically suspect this tumour to be pheochromocytoma. However. frozen section of the specimen showed evidence of spindle shaped cells with features suggestive of malignancy. In view of fluctuations in B. P. and having performed excision with good margin of healthy tissue we closed the bladder and the wound. The formal histopathology report showed large polygonal and spindle cells arranged in sheets and packets separated by a thin vascular stroma. The cells showed vesicular nuclear, abundant cytoplasm and scanty mitoses. Scattered groups of large bizzare cells were seen suggesting ganglionic differentiation like neuroblasts [Fig. 1 ].
The post-operative period was uneventful. The gross specimen showed positive dichromatic reaction. The final histopathology showed mixed features of pheochromocytoma with neuroblastic elements.
To rule out any ectopic or metastatic tumours bone marrow biopsy, C.T. scan, and VMA levels were performed. All these were within normal limits. She is now normotensive with-cut drugs till today.
Zimerman published the first case of pheochromocytoma of urinary bladder in 1953. It was nearly 4 years later that the second case was published by Roserberg. Thereafter every case reported in world literature was being claimed as the third, fourth and so on. In 1966 Higgins and Tresider published their three cases and stated that only 17 cases were reported until then. By the year 1977 over 63 cases were reported. Today it has not remained such an unknown entity but still is regarded as an interesting clinical presentation.
Though the exact aetiology is not understood it is accepted that pheochromocytoma arises from paraganglia related to the autonomic nerves of the urinary bladder. Chromaffin tissue has been found in the wall of urinary bladder.
Clinically the relation of throbbing headache and micturition is reported in about 50% of the cases by various authors., Higgins and Tresider have pointed out that the significance of such symptoms was often realised much later when histopathological proof was available. Haematuria is described as a common presenting symptom in about 50% of the cases over the age of 50 years.
A variety of operative procedures were performed for pheochromocytoma of bladder like transurethral resection, total cystectomy or hemicystectomy., However, in most of these cases the diagnosis was missed initially and on subsequent review of histopathological material pheochromocytoma was diagnosed. Transurethral resection of tumour is not the ideal treatment since haemorrhage can be severe and uncontrollable.
In spite of the availability of modern investigative modalities one can still meet a situation when pheochromocytoma of the bladder is suspected or diagnosed for the first time during surgery as in our second case. However, both the patients in our experience were subjected to partial cystectomy which is considered the ideal treatment for pheochromocytoma of bladder.
The histopathological diagnosis may be quite difficult at times. It was shown in the past how patients were subjected to more mutilating and major procedures with the initial picture simulating malignancy., In the second case presented here the frozen section from the excised tumour had also shown spindle shaped cells suggestive of malignancy, but the associated fluctuations in blood pressure and excision with wide margin made us stop at partial cystectomy and it proved to be the right decision since the final histological diagnosis was pheochromocytoma. The histological presence of tumour cells in muscle layers indicates the site of origin of the pheochromocytoma and not malignancy. The only sure way to diagnose malignant pheochromocytoma is detection of distant metastases.
In conclusion, it is very important to suspect the possibility of a pheochromocytoma in a case of bladder tumour or hypertension to reduce the complications and mismanagement.
We thank the Dean of K.E.M. Hospital for allowing us to publish the hospital data.
[Figure - 1]