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Hemorrhagic Shock from the Spontaneous Rupture of an Adrenal Cortical Carcinoma. A Case Report
J. S. Stamoulis, Z. Antonopoulou & M. Saiioleas
To cite this article: J. S. Stamoulis, Z. Antonopoulou & M. Saiioleas (2004) Haemorrhagic Shock from the Spontaneous Rupture of an Adrenal Cortical Carcinoma. A Case Report, Acta Chirurgica Belgica, 104:2, 226-228, DOI: 10.1080/00015458.2004.11679543
To link to this article: http://dx.doi.org/10.1080/00015458.2004.11679543
Published online: 14 Mar 2016.
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Haemorrhagic Shock from the Spontaneous Rupture of an Adrenal Cortical Carcinoma. A Case Report
J. S. Stamoulis, Z. Antonopoulou, M. Safioleas
Second Department of Propedeuticsurgery, University of Athens, Laiko Hospital, Athens, Greece.
Key words. Adrenal gland ; adrenal cortical carcinoma ; retroperitoneal haemorrhage.
Abstract. Adrenal cortical carcinoma is a rare endocrine neoplasm which can be either functioning or non-functioning. Usually, patients refer to the doctor because of abdominal pain or symptoms associated with the mass effect. We pre- sent an unusual case of a patient with adrenal cortical carcinoma who was immediately operated due to massive retroperitoneal haemorrhage following the spontaneous rupture of the tumour. Adrenal cortical carcinoma should enter in differential diagnosis of retroperitoneal haemorrhage. Surgeons should be familiar with this clinical entity and attempt complete resection if possible.
Introduction
Spontaneous massive retroperitoneal haemorrhage is usually associated with the rupture of an aortic aneu- rysm. When the patient is haemodynamically stable, abdominal CT-scanning is the examination of choice to delineate the haemorrhage cause. If a mass is found occupying the renal space, the most possible diagnosis is that of a renal tumour.
We report a rare case of a patient with an adrenal cor- tical carcinoma (ACC), first presenting as haemorrhagic shock due to spontaneous rupture and massive retroperi- toneal haemorrhage.
We discuss the difficulties in diagnostic evaluation and operative procedure. The prognosis seems to be dis- mal.
Case report
A 41-year old male was urgently admitted due to inten- se loin pain and fading. At clinical examination, the patient was pale, ill and a mass could be felt at the left upper quadrant of the abdomen. Abdominal X-ray exhi- bited an enlarged left kidney with irregular borders. Serum blood tests were normal with the exception of the haematocrit, which was lowered at 20%. Ultrasound of the abdomen revealed a bulky mass, situated between the spleen and the left kidney, with mixed echo structu- re. Computed tomography of the abdomen confirmed ultrasound findings (Fig. 1). At the time, a renal tumour coupled with retroperitoneal haemorrhage was the pro- posed diagnosis. The patien’s clinical condition deterio- rated over the next few hours and an emergency opera-
tion was decided. A large soft friable tumour with necro- sis areas and active haemorrhage from multiple sites within the mass was found, occupying the upper pole of the left kidney which seemed infiltrated and was exci- sed. Histological examination revealed an ACC with a calculated maximal diameter of 8 cm. The patient recei- ved chemotherapy : etoposide and cisplatin. Seven months after the operation, lung metastatis appeared.
Discussion
ACC is a rare neoplasm ; its incidence in the general population is of two per million (1). It can be either functioning - hormone producing tumour (more than 50% ) - or non-functioning. Cortisol is the most com- mon hormone produced in at least 30% of the patients (2). Functioning tumours usually present as a Cushing syndrome, often with virilizing features. The most common symptoms in non-functioning tumours are abdominal pain and pressure symptoms associated with the mass effect (3). Other clinical presentations are malaise, haematuria, varicocele, dyspnea, palpable abdominal mass and fever (3-4). Our patient did not pre- sent any symptoms suggestive for an adrenal tumour. Acute massive haemorrhage from spontaneous rupture of the tumour was the presenting symptom.
Differential diagnosis of a retroperitoneal haematoma consists of a variety of morbid circumstances (Table I). It is of interest that tumour haemorrhage finally necessi- tated an emergency operation. Peroperatively, the efforts were concentrated towards the control of the haemor- rhage while no surgical oncological principals could be met in such emergent situation. Soon after operation, the
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Fig. 1 A bulky tumour measuring almost 15 cm in maximal diameter, with irregular borders, mixed consistency, irregular- destroyed structure obviously due to haemorrhage was found, occupying the left renal space.
patient developed distant metastasis. We feel thus safe to conclude that haemorrhage in ACC patients is a dismal prognostic factor.
Complete surgical removal is the cornerstone to out- come in ACC. With complete resection, a five-year sur- vival is reported (ranging from 36 to 58% of the cases) (2, 4-7). On the contrary incomplete resection is asso- ciated with poor prognosis and a median survival of less than one year (2, 7). Besides complete resection, HARRISON L. E. et al. analysed other pathological poten- tial prognostic factors. The presence of intra-tumour haemorrhage was a poor prognostic factor (5). Our case confirms these findings. Chemotherapy is ineffective in case of distant metastasis of ACC. Our observation, with its dismal outcome, confirms the fact. CT-scan is an important examination in evaluating adrenal masses (8).
| 1. Trauma | Blunt Penetrating Iatrogenic (angioplasty, myo- cardial catheterization) |
| 2. Abdominal Aneurysm Rupture | Aortic, splenic, renal |
| 3. Coagulopathies | Haemophilia |
| 4. Anticoagulation agents | Heparin therapy, thrombolysis |
| 5. Benign tumours | Renal angiomyolipoma, adrenal adenoma, Others |
| 6. Malignant tumours | Renal adenocarcinoma |
| 7. Haemorrhagic pancreatitis | |
| 8. Idiopathic spontaneous |
Neither CT-scan nor ultrasound examination could reve- al the nature or even considered the mass to be from the adrenal. Even histological examination is sometimes difficult to discriminate between renal cells and adrenal cortical carcinoma (3). Tumour cells and tumour archi- tecture can look quite similar and the distinction between the two cannot be made on routine haematoxy- lin and eosin staining. Immunohistochemical stains were instructive for the diagnosis (9).
Conclusion
ACC should enter the differential diagnosis of retroperi- toneal haemorrhage associated with a mass. We believe that a two-stage procedure is justified in the case of acute haemorrhage from ACC. At first, every effort should be made to achieve control of the haemorrhage and drainage of the haematoma, while an attempt for complete surgical removal should be undertaken in a second operation, if there is no evidence of distant meta- stasis. In case of distant metastasis, chemotherapy regi- ments are totally ineffective.
References
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7. CRUCITTI F., BELLANTONE R., FERRANTE A., BOSCHERINI M., CRUCITTI P. The Italian Registry for adrenal cortical carcinoma : analysis of a multiinstitutional series of 129 patients. Surgery, 1996, 119 : 161-70.
8. LOCKHART M. E., SMITH J. K., KENNEY P. J. Imaging of adrenal mas- ses. Eur J Radiol, 2002, 41 : 95-112.
9. WICK M. R., CHERWITZ D. L., McGLENNEN R. C., DEHNER L. P. Adrenocortical carcinoma : an immunohistochemical comparison with renal cell carcinoma. Am J Pathol, 1986, 122 : 343-52.
J. S. Stamoulis Sd Department of Propedeuticsurgery Laiko Hospital Ag. Thoma, 17 GR-11527 Athens, Greece
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