March 2007 Vol 7 Isuue 1 FINAL without suicide .indd Pulmonary Infarction A rare case of adrenal carcinoma *Sukhpal Sawhney1, Ikram Burney2, Rajeev Jain1 الرِّئَة تِشاءُ احْ ر ظْ الكُ رَطانَة سَ من نادرة حالة جني وراجيف بورني اكرام سوهني، سخبال انسداد مع املشخص غير الكظر سرطانة الدم، وكان لديها ضغط انخفاض لعدة مرات بسبب للمستشفى أدخلت شابة تقرير حالة امللخص: هذا األمين البطني األَميَن، ، األُذَينُ لِيّ ــفْ وَفُ السُّ الوَريدُ األَجْ إلى ممتدة اليمنى الكظر غدة في كبيرة كتلة أظهر ــب احملوس املقطعي التصوير الرئوي. ــريان الش املصابني املرضى عند األمين واألذين السفلي األجوف الوريد من كال توثق خثرة من تقارير حلاالت العديد هناك األدبيات الطبية، في األمين. الرئوي والشريان الرئوي. الشريان إلى الورم امتداد نتيجة الرئوي االحتشاء خاصة بصورة توثق هذه أول حالة علمنا حسب الكظر. بسرطانة عمان. حالة، تقرير ب، َوْسَ يُّ احملُ عِ طَ َقْ امل وِيْرُ التَّصْ الرئة، احتشاء الكظر، سرطانة الكلمات: مفتاح 1Department of Radiology and Molecular Imaging, Sultan Qaboos University Hospital, P. O. Box 35, Al-Khod 123, Sultanate of Oman 2Department of Medicine, Sultan Qaboos University, P. O. Box 35, Al-Khod 123, Sultanate of Oman *To whom correspondence should be addressed. Email: sukh@squ.edu.om SULTAN QABOOS UNIVERSITY MEDICAL JOURNAL APRIL 2007 VOL 7, NO. 1 SULTAN QABOOS UNIVERSITY© WE REPORT A CASE OF A PREVIOUSLY well young girl with newly diagnosed adrenal carcinoma, who presented with a pulmonary infarction due to a tumour thrombus ex- tending in-continuity from the inferior vena cava to the right atrium, right ventricle and pulmonary artery. She did not have any clinical or imaging studies in the past. There have been no previous reports specifically documenting pulmonary embolism from a tumour thrombus of adrenal carcinoma, though there are many reports in literature documenting inferior vena cava and right atrial thrombus in patients with adrenal carcinoma. This is the first reported case of adrenal cortical carcinoma presenting as pulmonary infarc- tion in which a computed tomography (CT) scan es- tablished the diagnosis. C A S E R E P O R T A 15-year-old girl was referred to a tertiary hospital in urban Oman with a two-month history of sudden onset of hirsutism, acne, and a three-day history of episodes of prolonged hypotension, with blood pressure drop- ping to 90/60 mmHg. Her past medical history was not significant for medical or surgical illness. However, the menstrual history was significant, in that she had not attained menarche. On examination, she had a pulse rate of 138/min, blood pressure of 110/70mmHg, had a coarse voice, hirsutism, acne, ascites, and bilateral C A S E R E P O R T ABSTRACT A case report of unsuspected adrenal carcinoma with pulmonary artery obstruction in a young girl who was admitted with recurrent episodes of hypotension is presented. Computed tomography (CT) scans demonstrated a large right adrenal mass extending into the inferior vena cava (IVC), right atrium, right ventricle and right pulmonary artery. There are many case reports in literature documenting IVC and right atrial thrombus in patients with adrenal carcinoma. To our knowledge, this is the first case report specifically documenting pulmonary infarction secondary to tumour extension into the pulmonary artery. Keywords: Adrenal carcinoma, Pulmonary infarction, Computed Tomography , Case report, Oman. S U K H PA L S AW H N E Y, I K R A M B U R N E Y, R A J E E V J A I N 56 pitting edema. Her external genitalia were normal. The oxygen saturation was 95%, and blood gases re- vealed pH 7.42, pCO2 2.4 KPa, PO2 11.9 KPa, HCO3 11.9 mmol/l. The results of the full blood count were as follows: Hb 16.1 g/dl, WBC 16.8x109/1, cytology platelets 208 x 109 / l. Ascitic tap revealed lymphocytic exudates, with a protein content of 22.8gm/l. Serum cortisol levels were 6284 nanomol/L. A CT scan of her abdomen and chest was per- formed. A contrast enhanced CT scan (CECT) of the abdomen showed a large enhancing mass in the right adrenal gland. The mass was extending into and within the extrahepatic and intrahepatic course of the inferi- or vena cava. The liver showed features of Budd-Chiari syndrome in the form of an enlarged liver with areas of patchy parenchymal enhancement and multiple en- hancing regenerative nodules. Hepatic veins could not be identified [Fig 1]. A CECT scan of her chest at the level of the heart showed a mass in the right atrium extending into the right ventricle [Fig 2]. The CT scan at the level of the main pulmonary arteries showed a extension of the tumour in the right main pulmonary artery, with diminished perfusion of the lung distally [Fig 3]. The patient died on the third day after admis- sion, before any surgical intervention could be con- templated. D I S C U S S I O N This is the first reported case of an adrenal tumour with an IVC thrombus extending into the pulmonary artery. The estimated incidence of adrenocortical car- cinomas in patients younger than 20 years of age is three per million of the population.1 Of adrenal tu- mours in children, adrenocortical neoplasms are far less common than neuroblastomas, but more com- mon than pheochromocytomas.2 It is now recognized that most children with an adrenocortical neoplasm show clinical evidence of an endocrine abnormality, in contrast to the behaviour of adrenocortical neoplasms in adults.3 Although the commonest cause of inferior vena cava extension of a tumour is from renal cell carcino- ma, various other tumours, such as leiomyosarcomas of the inferior vena cava, adrenal cortical carcinomas, pheochromocytoms, neuroblastomas, transitional cell carcinomas of the renal pelvis, metastatic small cell carcinomas and Wilms tumours have been described to extend into the inferior vena cava.4 Pulmonary ar- tery tumour thrombus has been reported in patients with renal cell carcinomas,5,6 but to our knowledge there is no reported case of an adrenal carcinoma with tumour thrombus extension into the pulmonary ar- tery. Adrenal carcinomas may extend into the renal veins and inferior vena cava, and are usually accompanied by a thrombus.7 Right atrial involvement is very rare.7 Evidence suggests that as many as a third of primary Figure 1: Contrast-enhanced CT scan of upper abdomen shows an enhancing mass in the right adrenal gland (arrowheads), extending into the intrahepatic inferior vena cava (IVC) see arrows. Geographic hepatic parenchymal enhancement together with IVC occlusion by enhancing tumour thrombus indicates hepatic venous outflow obstruction (Budd-Chiari syndrome) Figure 2: Contrast-enhanced CT scan of chest at the level of the right ventricle shows a large tumour thrombus as a filling defect in the right atrium and ventricle (arrowheads). Hardly any enhancing pulmonary arteries are identified on the right side P U L M O N A R Y I N FA R C T I O N 57 adrenocortical cancers may develop a tumour throm- bus within the inferior vena cava.8 The thrombus may be asymptomatic or may become manifest as ascites, nephrotic syndrome, hepatomegaly, acute tricuspid valve failure or the Budd-Chiari syndrome.9 A limitation of this case report is the lack of avail- ability of a tissue diagnosis. The patient was very ill at presentation, in a state of hypotension and pursued a rapid downhill course, succumbing to the disease within three days of admission. However, virilising clinical features, a very high serum cortisol, together with a radiological picture of an adrenal mass with ex- tension into the IVS, right atrium and right pulmonary artery are highly suggestive of an adrenal carcinoma. C O N C L U S I O N Patients with adrenocortical carcinomas may have an extensive inferior vena caval tumour thrombus, which can extend into the right cardiac chambers and occa- sionally into the pulmonary arteries. Such patients may present with clinical features of pulmonary infarction. Alternatively, cardiac and/or pulmonary extensions of tumour thrombi may be overlooked, if not assiduously looked for. Therefore, a complete evaluation of a pa- tient with adrenocortical carcinoma should include an evaluation of the cardiac chambers with echocardiog- raphy or contrast-enhanced CT scans. R E F E R E N C E S 1. Lack EE, Mulvihill JJ, Travis WD, Kozakewich HP. Ad- renal cortical neoplasms in the pediatric and adoles- cent age group: clinicopathologic study of 30 cases with emphasis on epidemiological and prognostic factors. Pathol Ann 1992; 27:1-53. 2. Stewart DR, Jones PH, Jolleys A. Carcinoma of the adre- nal gland in children. J Pediatr Surg 1974; 9:59-67. 3. Lack EE. Adrenal cortical neoplasms in childhood. In Lack EE, Ed. Atlas of tumors of the adrenal gland and extra adrenal paraganglia,. Fasc 19, Ser 3. Washington, DC: Armed Forces Institute of Pathology, 1997. p. 153- 168. 4. Chesson JP, Theodorescu. Adrenal Tumor with caval ex- tension. Case report and review of the literature. Scand J Urol Nephrol 2002; 36: 71-73 5. G Gayer, S. Mini, D. Olchovosky, et al. Pulmonary em- bolism - the initial manifestation of renal cell carcinoma in a young woman. Emerg. Radiol 2003; 10:43-45. 6. Wieder JA, Laks H, Freitas D, Marmureanu A, Bell- degrun A. Renal cell carcinoma with tumor thrombus extension into the proximal pulmonary artery. J Urol 2003; 169: 2296-2297. 7. Rosen B, Rozenman Y, Harpaz D. Extension of adreno- cortical carcinoma into the right atrium-echocardio- graphic diagnosis; A case report. Cardiovas Ultrasound 2003; 16:5. 8. Radecka E, Brekkan E, Juhlin C, Sundin A, Magnusson A. An unusual case of tumor thrombus in the inferior vena cava. Acta Radiol 2003; 44:160-161 9. Hugh TB, Jones RM, Shanahan MX. Intra-atrial exten- sion of renal and adrenal tumors: diagnosis, manage- ment, and prognosis. World J Surg 1986; 10:496-500. Figure 3: Contrast-enhanced CT scan of chest demonstrates a thrombus in the right main pul- monary artery (arrowheads) with diminished arterial perfusion of the right lung.