Home About us Contact | |||
Adrenal Tumors (adrenal + tumor)
Kinds of Adrenal Tumors Selected AbstractsWhat is the best approach to an apparently nonmetastatic adrenocortical carcinoma?CLINICAL ENDOCRINOLOGY, Issue 5 2010Martin Fassnacht Summary In suspected nonmetastatic adrenocortical carcinoma (ACC) a careful preoperative diagnostic work up is needed including comprehensive endocrine analysis as recommended by the European Network for the Study of Adrenal Tumors (http://www.ENSAT.org/ACC.htm). Staging prior surgery, in particular chest CT, is indispensable to exclude distant metastases. Open surgery is still the recommended approach in ACC. However, in localized non-invasive ACC with a diameter <10 cm laparoscopic adrenalectomy by an expert surgeon is probably similarly effective and safe. As many patients will suffer from tumor recurrence after seemingly complete removal of ACC, adjuvant treatment based on the individual risk status is recommended. Key factors for risk assessment are tumor stage, resection status and the proliferation marker Ki67. All patients considered at high risk for recurrence should receive adjuvant mitotane for a minimum of 2 years aiming at a drug level of 14,20 mg/l. In selected patients (e.g. R1 resection) we recommend additional radiotherapy of the tumor bed. Patients with a low/intermediate risk for recurrence should be included in the Adiuvo trial comparing adjuvant mitotane with observation only (http://www.adiuvo-trial.org). In low/intermediate risk patients who cannot be included in this trial observation only can be justified in cases with a tumor diameter of <8 cm and no microscopic evidence for invasion of blood vessels or tumor capsule. In all patients a structured follow-up for 10 years is strongly recommended. [source] Portless endoscopic adrenalectomy via a single minimal incision using a retroperitoneal approach: Experience with initial 30 casesINTERNATIONAL JOURNAL OF UROLOGY, Issue 9 2004YUKIO KAGEYAMA Abstract Aim: To assess the feasibility of portless endoscopic adrenalectomy via a single minimum incision that narrowly permits extraction of the specimen. Methods: For, 30 cases of adrenal tumor, portless endoscopic surgery through a single flank incision (3,9 cm; mean, 5.6 cm) was performed without gas inflation or trocar port placement. All of the instruments used during surgery were reusable. The cases included primary aldosteronism (12), Cushing's syndrome (6), preclinical Cushing's syndrome (3), pheochromocytoma (1), non-functioning cortical adenoma (6), adrenocortical carcinoma (1) and adrenocortical hemorrhage (1). Results: Resection of the tumor was successfully completed, without complications, in all of the cases. Operative time was between 83 and 240 min (mean, 147 min). Estimated blood loss was 5,470 mL (mean, 139 mL). None of the patients required blood transfusion. Postoperative course was uneventful. Wound pain was mild and walking and full oral feeding were resumed on the first and second postoperative day, respectively, in the majority of cases. Conclusions: Adrenal tumors are good candidates for portless endoscopic surgery, which is safe, cost-effective, minimally invasive and matches favorably with laparoscopic surgery. [source] Letter to the Editor: Primary retroperitoneal mature cystic teratoma presenting as an adrenal tumor in an adultINTERNATIONAL JOURNAL OF UROLOGY, Issue 9 2010Fuminori Sato phd md No abstract is available for this article. [source] Renal cell carcinoma with a huge solitary metastasis to the contralateral adrenal gland: A case reportINTERNATIONAL JOURNAL OF UROLOGY, Issue 12 2008Takanobu Utsumi Abstract Renal cell carcinoma (RCC) is capable of metastasizing to several organs. Synchronous isolated contralateral adrenal metastasis of the primary RCC is, however, very rare. Herein we report a case of RCC with a huge solitary metastasis to the contralateral adrenal gland that was surgically treated. We scheduled nephrectomy for the left primary RCC and adrenalectomy for the right adrenal tumor. However, at surgery we found a huge right adrenal tumor that had invaded the right kidney, right renal vein, and inferior vena cava. Therefore right nephrectomy was performed simultaneously with resection and reconstruction of the inferior vena cava. Pathological findings demonstrated that the left renal tumor and right adrenal tumor had the same histology. Although the patient required hemodialysis, he remains well at six months postoperatively. So far, there have been only two cases of a solitary contralateral metastatic adrenal tumor that was larger than the primary RCC, thus the present case is the third one. [source] Indications for laparoscopic adrenalectomy for non-functional adrenal tumor with hypertension: Usefulness of adrenocortical scintigraphyINTERNATIONAL JOURNAL OF UROLOGY, Issue 6 2006HIROFUMI IZAKI Aim:, Laparoscopic adrenalectomy is currently indicated for biochemically and clinically functional adrenal tumors and potentially malignant tumors of the adrenal glands. Non-functional adenomas greater than 5 cm in diameter of the adrenal gland are generally considered to represent potentially malignant tumors. The present study shows indications of laparoscopic adrenalectomy for non-functional adrenal tumors with hypertension in a retrospective fashion. Methods:, Between 1994 and 2004, 110 laparoscopic adrenalectomies were performed at Tokushima University Hospital. All 110 patients underwent detailed endocrinological examination before surgery. Medical and operative records of these 110 patients (57 men, 53 women), including operative parameters, histopathological findings and pre- and postoperative hypertension, were reviewed. Forty-five patients underwent laparoscopic adrenalectomy for non-functional adrenal tumors, and [131I]6,-iodomethyl-19-norcholest-5(10)-en-3,-ol (NP-59) scintigraphy was performed for patients with preoperative hypertension. Results:, Mean patient age was 55.0 years (range, 22,77 years). Mean maximum tumor diameter was 42 mm (range, 20,105 mm). All adrenal tumors were removed successfully by laparoscopic surgery. Hypertension was postoperatively improved in seven of the 11 patients with preoperative hypertension, without subclinical Cushing syndrome. Importantly, all patients who improved hypertension after adrenalectomy displayed strong accumulation in adrenal tumors with visualization of the contralateral gland on NP-59 scintigraphy. Conversely, blood pressure did not improve in four patients for whom scintigraphy yielded negative results. Conclusions:, The indication of laparoscopic adrenalectomy for non-functional adrenal tumors is generally considered for lesions more than 5 cm diameter. However, the present study suggests that laparoscopic surgery should be considered even in patients with tumors less than 5 cm in diameter, if both hypertension and accumulation in tumors on NP-59 scintigraphy are present. [source] Gastric diverticulum preoperatively diagnosed as one of two left adrenal adenomasINTERNATIONAL JOURNAL OF UROLOGY, Issue 1 2006AKIHIRO ARAKI Abstract, A 47-year-old man was diagnosed with primary aldosteronism due to two left adrenal adenomas, suggested on computed tomography (CT) to be located at the upper and lower adrenal portion. However, adosterol scintigraphy revealed negligible uptake at the upper portion of the left adrenal. Laparoscopic left adrenalectomy was performed, but macroscopic examination of the specimen revealed only one adrenal tumor. Continued surgical exploration detected another mass between the spleen and the stomach, which was demonstrated to be continuous with the stomach and was eventually diagnosed as a gastric diverticulum. Postoperatively, aldosteronism resolved and repeat CT revealed staining of the adrenal pseudotumor when oral contrast was administered. Since organs located near the adrenals can simulate adrenal tumors, caution must be exercised in interpreting suprarenal masses on CT. To our knowledge, this is the first reported case of concurrent pseudotumor and true tumor of the ipsilateral adrenal. [source] Clinical outcomes of laparoscopic adrenalectomy according to tumor sizeINTERNATIONAL JOURNAL OF UROLOGY, Issue 12 2005ISAO HARA Objectives: In order to evaluate the indication and usefulness of laparoscopic adrenalectomy, clinical outcomes of laparoscopic adrenalectomy for patients with adrenal tumors were examined. Whether tumor size affects surgical outcome was analysed, along with the long-term clinical outcome for these patients. Patients and methods: A total of 63 patients with adrenal tumor underwent laparoscopic adrenalectomy in our institute between 1999 and 2003. A laparoscopic transperitoneal approach was used in all cases. Underlying pathologies comprised Cushing syndrome (n = 12), pheochromocytoma (n = 13), primary aldosteronism (n = 21), non-functioning adenoma (n = 12) and others (n = 5). Results: No open conversion was performed. Mean operative duration was 239 min, and mean estimated blood loss was 134 mL. Tumor diameter was significantly smaller for primary aldosteronism than for Cushing syndrome, which in turn was significantly smaller than for adrenocorticotropic hormone-independent macronodular hyperplasia (AIMAH). No significant differences in surgical outcome and postoperative recovery were noted between large (,5 cm) and small (<5 cm) tumors. Long-term clinical outcome was better for patients with pheochromocytoma or primary aldosteronism than for patients with Cushing syndrome. Conclusions: Laparoscopic adrenalectomy for benign tumor offers excellent surgical outcomes and convalescence. This is true for both small and large tumors. [source] Portless endoscopic adrenalectomy via a single minimal incision using a retroperitoneal approach: Experience with initial 30 casesINTERNATIONAL JOURNAL OF UROLOGY, Issue 9 2004YUKIO KAGEYAMA Abstract Aim: To assess the feasibility of portless endoscopic adrenalectomy via a single minimum incision that narrowly permits extraction of the specimen. Methods: For, 30 cases of adrenal tumor, portless endoscopic surgery through a single flank incision (3,9 cm; mean, 5.6 cm) was performed without gas inflation or trocar port placement. All of the instruments used during surgery were reusable. The cases included primary aldosteronism (12), Cushing's syndrome (6), preclinical Cushing's syndrome (3), pheochromocytoma (1), non-functioning cortical adenoma (6), adrenocortical carcinoma (1) and adrenocortical hemorrhage (1). Results: Resection of the tumor was successfully completed, without complications, in all of the cases. Operative time was between 83 and 240 min (mean, 147 min). Estimated blood loss was 5,470 mL (mean, 139 mL). None of the patients required blood transfusion. Postoperative course was uneventful. Wound pain was mild and walking and full oral feeding were resumed on the first and second postoperative day, respectively, in the majority of cases. Conclusions: Adrenal tumors are good candidates for portless endoscopic surgery, which is safe, cost-effective, minimally invasive and matches favorably with laparoscopic surgery. [source] Case of combined adrenal cortical adenoma and myelolipomaPATHOLOGY INTERNATIONAL, Issue 9 2004Takaharu Matsuda We report a case of myelolipoma 10 mm in size within a functional cortical adenoma that was 33 × 22 × 17 mm in size. A 29-year-old woman was referred to hospital for transient hypertension. A right adrenal tumor was detected by computed tomography (CT) scan and magnetic resonance imaging (MRI). Her cortisol levels indicated a loss of the normal diurnal pattern, and urinary 17-hydroxycorticosteroids was elevated. She underwent a right adrenalectomy under the diagnosis of adrenal adenoma with Cushing's syndrome. The tumor was fairly well encapsulated by a thin layer of connective tissue. The major tumor portion was composed of two distinct cell types, clear cells and eosinophilic cells, arranged in an alveolar structure. These findings were representative of cortical adenoma. The adrenal cortical adenoma centrally included well-demarcated adipose tissue admixed with scattered islands of myelopoietic elements: erythroblasts, myeloid cell series and lymphocytic cells, which was eventually recognized as myelolipoma. Recently, adrenal myelolipoma has commonly been found because of the ease of detecting it as an incidentaloma by CT scan or MRI. However, the present adrenal myelolipoma case is uncommon because it is combined with a functioning cortical adenoma. Only six similar cases have previously been reported in English and Japanese publications. Furthermore, in the present case, the myelolipoma formed a tumor nodule, and to our knowledge, this is the first reported case of a radiographically recognizable tumor nodule. We discuss the etiology of myelolipoma and suggest that myelolipoma can develop in the course of endocrine hyperfunction. [source] A composite pheochromocytoma/ganglioneuroblastoma of the adrenal gland,PEDIATRIC BLOOD & CANCER, Issue 7 2010Elizabeth Lee Thiel MD Abstract A 9-year-old female presented with a large abdominal mass. At surgery, the mass was noted to arise from the right adrenal gland. As the mass was manipulated, the patient developed severe hypertension. The final diagnosis was a cystic composite-pheochromocytoma/ganglioneuroblastoma. This compound adrenal tumor is only the fourth case reported in a child. Because composite pheochromocytomas are rare in the pediatric population, the management, optimal surveillance schedule and outcomes have not been characterized. Pediatr Blood Cancer 2010;54:1032,1034 © 2010 Wiley-Liss, Inc. [source] Laparoscopic adrenalectomy: Troublesome casesINTERNATIONAL JOURNAL OF UROLOGY, Issue 5 2009Gaku Kawabata Among 143 cases of laparoscopic adrenalectomy carried out from 1993 to the present, 13 patients in whom the surgical manipulation presented problems were examined. Problems occurred due to the condition of the adrenal tumors themselves in six patients, whereas problems occurred due to the operative history in four patients. There were three patients with no operative history but with strong intraperitoneal adhesion. In patients with a history of laparotomy in other fields such as open cholecystectomy, gastrectomy or colostomy, operations were possible in most patients by examining the trocar site preoperatively. Patients with strong adhesion even without a history of surgery could be handled by full separation of the adhesion during surgery. In patients with bleeding in the adrenal tumors, large adrenal tumors, or tumors impacted in the liver, methods such as changing the sequence of separation procedures were required. In patients with a history of renal subcapsular hematomas due to extracorporeal shock wave lithotripsy (ESWL), it was not possible to understand the conditions of adrenal or perinephritic adhesion in preoperative imaging diagnosis, but resection was possible by changing the order of separation procedures and by using optimal instruments and devices. As with any surgery, including open surgeries, it is necessary to obtain knowledge on how to deal with variations in laparoscopic adrenalectomy to assure safe outcomes and to always consider effective methods for coping with unexpected difficulties. [source] Clinical outcomes and learning curve of a laparoscopic adrenalectomy in 103 consecutive cases at a single instituteINTERNATIONAL JOURNAL OF UROLOGY, Issue 6 2006MASATOSHI ETO Objective:, We examined the clinical outcomes and the learning curve for a laparoscopic adrenalectomy (LA) in 103 consecutive cases performed by three surgeons at our institute, according to the type of adrenal disorder. Patients and Methods:, One hundred and three patients with adrenal tumors, including 38 cases of primary aldosteronism, 33 cases of Cushing syndrome (including preclinical Cushing syndrome), 15 cases of pheochromocytoma, and nine cases of non-functioning adenoma were evaluated, while focusing on the approaches, intraoperative and postoperative data, and the learning curve of LA, according the type of adrenal disorder. Results:, There was no significant difference in the operation time, estimated blood loss, incidence of conversion to open surgery and blood transfusion, or postoperative recovery among the patients treated by LA for aldosteronoma, Cushing adenoma, pheochromocytoma, and non-functioning adenoma. In the cases of aldosteronoma and Cushing adenoma, the learning curve for the operation time and blood loss in each operator tended to decrease as the number of operations increased. On the other hand, in the cases treated by LA for pheochromocytoma, no trends in either the operation time or blood loss were observed. However, there has been neither any conversion to open surgery nor blood transfusion in cases treated by LA since 1998 (our 42nd case), even after the changes in the operators. Conclusions:, Our results clearly indicate that LA is becoming safer than before, probably due to improvements in the technique, education, and training of surgeons, in addition to the increased number of cases now treated by LA. [source] Indications for laparoscopic adrenalectomy for non-functional adrenal tumor with hypertension: Usefulness of adrenocortical scintigraphyINTERNATIONAL JOURNAL OF UROLOGY, Issue 6 2006HIROFUMI IZAKI Aim:, Laparoscopic adrenalectomy is currently indicated for biochemically and clinically functional adrenal tumors and potentially malignant tumors of the adrenal glands. Non-functional adenomas greater than 5 cm in diameter of the adrenal gland are generally considered to represent potentially malignant tumors. The present study shows indications of laparoscopic adrenalectomy for non-functional adrenal tumors with hypertension in a retrospective fashion. Methods:, Between 1994 and 2004, 110 laparoscopic adrenalectomies were performed at Tokushima University Hospital. All 110 patients underwent detailed endocrinological examination before surgery. Medical and operative records of these 110 patients (57 men, 53 women), including operative parameters, histopathological findings and pre- and postoperative hypertension, were reviewed. Forty-five patients underwent laparoscopic adrenalectomy for non-functional adrenal tumors, and [131I]6,-iodomethyl-19-norcholest-5(10)-en-3,-ol (NP-59) scintigraphy was performed for patients with preoperative hypertension. Results:, Mean patient age was 55.0 years (range, 22,77 years). Mean maximum tumor diameter was 42 mm (range, 20,105 mm). All adrenal tumors were removed successfully by laparoscopic surgery. Hypertension was postoperatively improved in seven of the 11 patients with preoperative hypertension, without subclinical Cushing syndrome. Importantly, all patients who improved hypertension after adrenalectomy displayed strong accumulation in adrenal tumors with visualization of the contralateral gland on NP-59 scintigraphy. Conversely, blood pressure did not improve in four patients for whom scintigraphy yielded negative results. Conclusions:, The indication of laparoscopic adrenalectomy for non-functional adrenal tumors is generally considered for lesions more than 5 cm diameter. However, the present study suggests that laparoscopic surgery should be considered even in patients with tumors less than 5 cm in diameter, if both hypertension and accumulation in tumors on NP-59 scintigraphy are present. [source] Gastric diverticulum preoperatively diagnosed as one of two left adrenal adenomasINTERNATIONAL JOURNAL OF UROLOGY, Issue 1 2006AKIHIRO ARAKI Abstract, A 47-year-old man was diagnosed with primary aldosteronism due to two left adrenal adenomas, suggested on computed tomography (CT) to be located at the upper and lower adrenal portion. However, adosterol scintigraphy revealed negligible uptake at the upper portion of the left adrenal. Laparoscopic left adrenalectomy was performed, but macroscopic examination of the specimen revealed only one adrenal tumor. Continued surgical exploration detected another mass between the spleen and the stomach, which was demonstrated to be continuous with the stomach and was eventually diagnosed as a gastric diverticulum. Postoperatively, aldosteronism resolved and repeat CT revealed staining of the adrenal pseudotumor when oral contrast was administered. Since organs located near the adrenals can simulate adrenal tumors, caution must be exercised in interpreting suprarenal masses on CT. To our knowledge, this is the first reported case of concurrent pseudotumor and true tumor of the ipsilateral adrenal. [source] Laparoscopic adrenalectomy for functioning and non-functioning adrenal tumors: Analysis of surgical aspects based on histological typesINTERNATIONAL JOURNAL OF UROLOGY, Issue 12 2005JA H KU Background: The aim of this study was to evaluate whether hormonal functions of the tumor influence the operative results of laparoscopic adrenalectomy, and to analyse the clinical outcomes in patients with various hormonally active adrenal tumors. Methods: Clinical and pathological records of 68 patients were reviewed. The average age of patients was 40 years (range 20,75); 39 were women and 29 men. For the comparison, patients were divided into the non-functioning tumor group (n = 22) and the functioning tumor group (n = 46). Results: All laparoscopic adrenalectomies were finished successfully, and no open surgery was necessary. The median operative time and blood loss in the two groups were similar; however, in subgroup analysis, operative time for pheochromocytoma was significantly longer than that for non-functioning tumor (P = 0.044). No difference was noted in intra- and postoperative data between the groups. Of the 22 patients with aldosteronoma, 18 (81.8%) became normotensive and no longer required postoperative blood pressure medications. Adrenalectomy led to an overall reduction in the median number of antihypertensive medications (P < 0.001). All patients with Cushing adenoma had resolution or improvement of the signs and symptoms during follow-up periods. There was no evidence of biochemical or clinical recurrence in any patient with pheochromocytoma. Conclusion: The results of this retrospective review document that laparoscopic adrenalectomy is a safe and effective treatment for functioning as well as non-functioning adrenal tumors, although endocrinologic features may play a significant role. [source] Clinical outcomes of laparoscopic adrenalectomy according to tumor sizeINTERNATIONAL JOURNAL OF UROLOGY, Issue 12 2005ISAO HARA Objectives: In order to evaluate the indication and usefulness of laparoscopic adrenalectomy, clinical outcomes of laparoscopic adrenalectomy for patients with adrenal tumors were examined. Whether tumor size affects surgical outcome was analysed, along with the long-term clinical outcome for these patients. Patients and methods: A total of 63 patients with adrenal tumor underwent laparoscopic adrenalectomy in our institute between 1999 and 2003. A laparoscopic transperitoneal approach was used in all cases. Underlying pathologies comprised Cushing syndrome (n = 12), pheochromocytoma (n = 13), primary aldosteronism (n = 21), non-functioning adenoma (n = 12) and others (n = 5). Results: No open conversion was performed. Mean operative duration was 239 min, and mean estimated blood loss was 134 mL. Tumor diameter was significantly smaller for primary aldosteronism than for Cushing syndrome, which in turn was significantly smaller than for adrenocorticotropic hormone-independent macronodular hyperplasia (AIMAH). No significant differences in surgical outcome and postoperative recovery were noted between large (,5 cm) and small (<5 cm) tumors. Long-term clinical outcome was better for patients with pheochromocytoma or primary aldosteronism than for patients with Cushing syndrome. Conclusions: Laparoscopic adrenalectomy for benign tumor offers excellent surgical outcomes and convalescence. This is true for both small and large tumors. [source] Proliferative activity and genetic changes in adrenal cortical tumors examined by flow cytometry, fluorescence in situ hybridization and immunohistochemistryINTERNATIONAL JOURNAL OF UROLOGY, Issue 2 2005KOUSUKE TAKEHARA Abstract Background: To determine differences in biological features among different adrenal tumors, we investigated the DNA ploidy, numerical chromosomal aberration and proliferative activity in human adrenal cortical neoplasms. Methods: Our study included six adrenal cortical adenomas with Cushing syndrome, 12 adenomas with hyperaldosteronism, three non-functioning adenomas and three adrenal cortical carcinomas. Isolated nuclei from frozen samples were used for fluorescence in situ hybridization (FISH) analysis, and formalin-fixed, paraffin-embedded tissues from the same materials were analyzed using flow cytometry (FCM) for DNA ploidy. Sections from paraffin blocks were stained immunohistochemically with antibodies against Ki-67 and p53. For FISH analysis, we used an ,-centromeric enumeration probe for chromosome 17. Results: The mean Ki-67 labeling index (LI) of adrenal cortical carcinomas was markedly higher than that of adrenal cortical adenomas (209.4 vs 8.7). In functional adrenal cortical adenomas, the LI was significantly lower in adenomas with hyperaldosteronism than in those with Cushing syndrome (P = 0.004), although FCM results indicated that tetraploid patterns were more frequently observed in the former type. Tumor size was significantly smaller in adenomas with hyperaldosteronism than in those with Cushing syndrome (P = 0.004). Chromosome 17 showed disomy in all adrenal cortical adenomas, whereas chromosome 17 abnormalities were found in two of three adrenal cortical carcinomas. Only the latter two cases strongly expressed p53 protein. Conclusions: Our study characterized various biological features of benign and malignant adrenal cortical tumors. The use of a combination of markers might provide additional information to assist our understanding of the clinical behavior of an individual adrenal cortical tumor. [source] Efficacy and safety of laparoscopic surgery for pheochromocytomaINTERNATIONAL JOURNAL OF UROLOGY, Issue 2 2005YUKIO NAYA Abstract Objective: Laparoscopic surgery for primary aldosteronoma and Cushing's syndrome is well established. We report on our experiences with laparoscopic adrenalectomy for pheochromocytoma, and assess the efficacy and safety of the laparoscopic approach. Methods: Between April 1998 and April 2003, a total of 23 patients underwent laparoscopic adrenalectomy for pheochromocytoma at Chiba University Hospital and Yokohama Rosai Hospital, Japan. We compared the surgical outcomes of these patients with those of 106 patients with adrenal tumors due to other pathologies who underwent laparoscopic adrenalectomy during the same period. Results: The mean tumor size of pheochromocytoma was 4.96 cm. Mean operative time was 192.7 min, and mean estimated blood loss was 130 mL. Neither mean operative time nor mean estimated blood loss was greater for patients with pheochromocytoma. Intraoperative hypertension (systolic blood pressure > 180 mmHg) occurred in 39.1% (9/23) of patients with pheochromocytoma. During the follow-up period, there were no mortalities or recurrences of endocrinopathy. Conclusions: Laparoscopic adrenalectomy for pheochromocytoma is a safe and minimally invasive procedure. [source] Adrenal leiomyosarcoma extending into the right atriumINTERNATIONAL JOURNAL OF UROLOGY, Issue 1 2002YOSHIYUKI MATSUI Abstract Primary soft tissue sarcoma of the adrenal gland is very rare and aggressive. In right adrenal tumors, because of direct venous drainage into inferior vena cava, the tumor may invade the vena caval wall toward the right atrium. We present a case of adrenal leiomyosarcoma extending into the right atrium. [source] Nine cases of childhood adrenal tumour presenting with hypertension and a review of the literatureACTA PAEDIATRICA, Issue 6 2007Xiumin Wang Abstract Aim: To describe the clinical features, treatment and outcome of children adrenal tumors presenting with hypertension. Methods: The records of nine children under 16 years of age with adrenal tumours presenting with hypertension were analysed. Details were recorded for family history, clinical presentation, biochemistry, imaging, histology, treatment and outcome. Results: Abdominal mass was palpable only in one patient at diagnosis. Besides hypertension-associated symptoms, Cushing's syndrome was the common presentation form (n = 4). Abdominal computed topography showed adrenal mass in all patients. Tumours were completely resected for each patient. The median tumour weight was 73 g (11,530 g) and the size ranged from 1.5 × 1.5 to 12 × 14 cm2. Pheochromocytoma (n = 2), adrenocortical adenoma (n = 3), adrenocortical carcinoma (n = 1), neuroblastoma (n = 2) and ganglioneuromas (n = 1) were found. In one case, adrenal pheochromocytoma first occurred and non-functioning islet cell tumour successively occurred at pancreas. A better status is common at a median follow-up time of 3.5 years. Conclusions: Childhood adrenal tumours presented with hypertension showed an atypical course, variable presentation. We report a unique case of adrenal pheochromocytoma followed by the occurrence of non-functioning islet cell tumour. Reversal of hypertension by surgery is crucial. Imaging techniques are important to detect adrenal tumours. [source] |