NOT THE USUAL CAUSE OF SUPERIOR VENA CAVA OBSTRUCTION
A. Jamieson, D.G. Ingham, A.F. Dominiczak, J.M.C. Connell Department of Medicine and Therapeutics, MRC Blood Pressure Unit, Western Infirmary, Glasgow
Abstract: Superior vena cava obstruction has a classical association with bronchial carcinoma. In the presence of cortisol excess, the diagnosis of small-cell carcinoma and ectopic ACTH production seems likely. We present a case where the clinical features did not accurately reflect the underlying pathology.
Key words: Vena cava, adrenal carcinoma
Case report
A 52-year-old woman was referred for investigation of hypertension and hypokalaemia. At the time of assessment a 4-week history of hoarseness and facial plethora was reported. There was a 10-year history of hypertension previously well controlled by enalapril. The patient had smoked 30 cigarettes per day for 35 years. At presentation the neck veins were distended and non-pulsatile, and plethora of the upper limbs, neck and face were noted. A chest X-ray suggested the presence of superior mediastinal adenopathy. A clinical diagnosis of superior vena caval obstruction was made. Further investigation was undertaken to elucidate the potential cause (Table I).
Hypokalaemia at presentation suggested the possibility of ectopic ACTH syndrome. Hypercortisolism was confirmed but ACTH was undetectable in serum. The elevated serum cortisol did not suppress with high dose dexamethasone, and in addition serum androgens were also elevated suggesting the possibility of an adrenal source for the steroid production. CT of thorax and abdomen was performed (Fig 1). A mass of right paratracheal lymph nodes compressing the superior vena cava, and right hilar nodes were detected. A 3.5 cm lesion in the right adrenal was detected, as were multiple lesions within the liver consistent with metastases.
An ultrasound guided biopsy of one of the liver metastases was undertaken. Microscopic and immunohistochemical examination revealed the tumour to be a primary adrenocortical carcinoma. The patient received therapy with mitotane, aminoglutethimide and metyrapone and intravenous cisplatin and etoposide. She died four months later of disseminated carcinomatosis.
Discussion
Superior vena cava obstruction and ectopic ACTH secretion are well-defined associations with bronchial carcinoma.’ Indeed. the occurrence of both of these clinical syndromes in the same patient is not uncommon. In this case, the occurrence of hypertension, hypokalaemia, hypercortisolism, and superior vena cava obstruction in a cigarette smoker made the diagnosis of metastatic small cell bronchial carcinoma highly likely.
| Serum Cortisol | ACTH | Testosterone | |
|---|---|---|---|
| Day -1 | 1032 nmol/l | <5 mU/1 | 4.7 |
| Day 0 0800 | 1184 nmol/1 | <5 mU/I | 5.9 |
| Day 1 0800 | 1081 nmol/1 | <5 mU/1 | 4.4 |
| Day 2 0800 | 1075 hmol/l | <5 mU/1 | 4.8 |
Histology
Necrotic islands of adrenocortical carcinoma. Stains positively with cytokeratin, vimentin and synapthophysin.
Biochemical testing confirmed cortisol excess in the presence of undetectable ACTH. Elevated adrenal androgens and failure of high dose dexamethasone to suppress cortisol secretion further confirmed the likelihood of an adrenal source for cortisol secretion.
Correspondence to: Dr Andrew Jamieson, Consultant Physician, Queen Margaret Hospital, Dunfermline KY12 0SU
Tel .: 01383 623623 Email: ajamieson_qmh@yahoo.co.uk
However, the absence of detectable ACTH using a highly sensitive assay was unexpected and raised the possibility of a primary adrenal lesion with disseminated metastases as the underlying cause.
Adrenal carcinoma is uncommon, accounting for less than 10% of all adrenal lesions.2 Adrenal carcinoma most commonly presents with symptoms and signs of hormone excess, principally cortisol giving rise to Cushingoid-features, or adrenal androgens leading to virilisation. In addition, there is often evidence of local invasion or metastatic spread, principally to the liver, with pain as the most common symptom. Due to the anatomical location of these tumours, infiltration of the inferior vena cava may occur.3
In this case, treatment of the tumour with the adrenolytic agent mitotane in addition to aminoglutethimide and metyrapone led to an initial improvement in the patient’s biochemistry and signs of superior vena cava obstruction. However, despite intensive management including platinum based chemotherapy, the aggressive nature of the tumour led to her death within four months of presentation, significantly worse than that of small cell carcinoma treated with combination chemotherapy.4
Whilst ectopic ACTH secretion or superior vena cava obstruction may occur in the context of bronchial carcinoma, each case merits a thorough clinical and biochemical evaluation. In this case, thorough clinical and biochemical evaluation resulted in an unusual diagnosis which radically altered the therapeutic approach to the clinical problem
ACKNOWLEDGEMENTS: We thank Dr NS Reed. Consultant Clinical Oncologist for his input in this case.
REFERENCES
I Home NW. Spiro SG. Tumours of the lung, mediastinum and pleura. In Oxford Textbook of Medicine 2nd Ed. 1987. (eds DJ Weatherall, JGG Ledingham, and DA Warrell). 15.145-158.
2 Kloos RT, Gross MID, Francis R, Korobkin M, Shapiro B. Incidentally discovered adrenal masses. Endocrine Research 1995; 16:460-484.
3 Tritos NA, Cushing GW, Heatley G. Libertino JA. Clinical features and prognostic factors associated with adrenocortical carcinoma: Lahey Clinic Medical Center experience. American Surgery 2000;66(1):73-9.
4 Williamson SK, Lew D. Miller G-1, Balcerzok SP, Baker LH, Crawford ED. Phase II evaluation of cisplatin and etoposide followed by mitotane at disease progression in patients with locally advanced or metastatic adrenocortical carcinoma: a Southwest Oncology Group Study. Cancer. 2000;88:1159-65.
0036-9330/01/00800/052
@ 2001 Scottish Medical Journal
Scot Med J 2001; 46: 052-053
EMBOLISATION OF RENAL ARTERIOVENOUS MALFORMATION (AVM) IN PREGNANCY
S. Macdonald, R.D. Edwards Interventional Radiology Unit, Gartnavel General Hospital, Great Western Road, Glasgow
Abstract: A case of congenital renal arteriovenous malformation (AVM), presenting with profuse haematuria in the second trimester of pregnancy is reported. Selective embolisation with polyvinyl alcohol particles and absolute alcohol successfully ablated the lesion and a healthy infant was delivered at term five months later. Renal angiography at three years showed no evidence of the lesion.
Key words: Embolisation, vascular malformation, congenital, renal artery, pregnancy.
Introduction
A total of nine cases of symptomatic renal AVM have been reported in pregnancy. Surgical management was elected in eight1-6 and endovascular treatment in only one.7 In this case, non-selective renal arterial embolisation with the temporary embolic agent, gelatin sponge was performed but follow up angiography one month post delivery of a healthy infant showed a small recurrent fistula. The lesion reported in our case was in the superior peri-pelvic portion of the right kidney and total nephrectomy was considered the only feasible surgical option. Selective embolisation provided a safe, effective and durable treatment with minimal renal parenchymal loss.
Case report
A 24-year-old primiparous woman presented in the 16th week of pregnancy with profuse painless haematuria requiring transfusion of three units of blood. There was no significant previous medical history. Ultrasound examination demonstrated a vascular lesion in the right kidney and selective angiography confirmed the presence of a vascular malformation in the peri-pelvic area (fig. la).
Three small arterial branches supplied the lesion and prominent, rapid venous drainage was demonstrated. Each feeding vessel was catheterised with a coaxial catheter system and embolised with polyvinyl alcohol particles (250-500 microns) and three 0.5ml aliquots of absolute alcohol. To enhance the local thrombotic effect of the alcohol the venous return was partially obstructed with an occlusion balloon placed selectively in the draining renal vein. Complete ablation of the lesion was achieved but a small upper polar branch was sacrificed. The patient tolerated the procedure well and had no further haematuria.
The remainder of her pregnancy was uneventful. Three years later she was referred for investigation of minor haematuria associated with right loin pain. In view of her previous history, renal angiography was performed which demonstrated no residual vascular malformation and recanalisation of the occluded small upper pole branch (fig. lb). Her symptoms settled without active treatment.
Discussion
Renal arteriovenous malformations (AVMs ) are rare lesions
with an estimated incidence of 0.04%& and can be classified as congenital, idiopathic or acquired.9 Our case highlights the typical manifestations of a congenital or high flow arteriovenous malformation. The angiographic features include multiple small tortuous arterial feeding vessels with arteriovenous shunting and early visualisation of the draining veins.10
There is a female predilection, right kidney predominance8.11 and an association with polycystic disease, fibromuscular dysplasia8 and Hereditary Haemorrhagic Telangiectasia. Although the majority of congenital lesions are clinically silent due to their small size, they may become symptomatic on enlarging. In high flow vascular malformations, haematuria is the presenting complaint in 75% of cases and hypertension occurs in 25%.12 Patients with large lesions may have an abdominal or flank bruit or present with symptoms of high out-put cardiac failure.
More than 70% of renovascular lesions heal spontaneously, but complications may occur in 4-9% of cases.13 Congenital vascular malformations rarely, if ever, disappear without treatment.
Conservative management is appropriate for small, peripheral lesions without haemodynamic effects or significant symptoms.14 Indications for intervention include significant haemorrhage, cardiac failure, refractory hypertension and ischaemic renal failure. Intravenous urography is often the initial investigation as many cases present with haematuria, but the diagnostic yield is low as upper urinary tract clot may only be only demonstrated in the bleeding phase. Colour duplex ultrasound can provide an accurate diagnosis of renal vascular malformation and should be performed as a primary investigation in the pregnant patient.
The high incidence of haematuria in congenital renal AVMs is thought to be due to their superficial location in the lamina propria immediately below the transitional epithelium of the collecting system and also due to the focal absence of the elastic lamina.14 Pregnancy may influence both the development and progression of arteriovenous malformations and bleeding, although unpredictable, is more likely in the second trimester. Primiparous patients between 20-25 years of age are most commonly affected.
Correspondence to: Dr Sumaira Macdonald MRCP, FRCR, Interventional Radiology Unit, Gartnavel General Hospital, 1053 Great Western Road, Glasgow G12 OYN. Tel: 0141 211 3113 Fax: 0141 211 3471