No abstract available.
Adrenal Cortex Function Tests ; *Adrenal Cortex Neoplasms/complications/diagnosis/metabolism/surgery ; *Adrenal Gland Neoplasms/complications/diagnosis/metabolism/surgery ; Adrenalectomy ; *Adrenocortical Adenoma/complications/diagnosis/metabolism/surgery ; Biopsy ; Cushing Syndrome/diagnosis/etiology ; Female ; Humans ; Immunohistochemistry ; *Incidental Findings ; Middle Aged ; *Neoplasms, Multiple Primary/complications/diagnosis/metabolism/surgery ; *Pheochromocytoma/complications/diagnosis/metabolism/surgery ; Predictive Value of Tests ; Tomography, X-Ray Computed ; Treatment Outcome ; Tumor Markers, Biological/metabolism

OBJECTIVETo investigate the efficacy of combined modality therapy for neuroblastoma in children associated with opsoclonus-myoclonus syndrome (OMS-NB).

METHODFrom May 2011 to December 2013, 6 consecutive patients (4 boys and 2 girls) diagnosed as OMS-NB underwent surgery and chemotherapy in the First Hospital, Peking University. The median age of onset was 19.5 months (range 13-24 months) and misdiagnosis occurred 7.5 months (range 2-14 months) ago. A retrospective analysis for the location, stage, pathological type, treatment way and outcome of neuroblastoma was done.

RESULT(1) All patients were misdiagnosed as simply opsoclonus-myoclonus syndrome (OMS) at the time of onset. They had been receiving treatment with adrenocorticotropic hormone and intravenous immunoglobulin within 1-13 months.OMS-NB was diagnosed by means of enhanced abdominal CT image which was delayed to be given after the poor efficacy or relapse. (2) The primary tumors were almost all small, stage I-II, located in adrenal, retroperitoneal or pelvis. The pathology of tumors included ganglioneuroblastoma (5/6) and neuroblastoma (1/6). (3) All these cases underwent surgery, 4/6 cases with complete tumor resection, 2/6 cases with tumor around the aorta and induced local residue. Preoperative and postoperative chemotherapy was given to 2 and 5 cases, respectively. (4) The patients were followed up for 3-31 months, except 1 patient lost, the other 5 are currently surviving disease-free (3 having been at the end of chemotherapy, 1 still in chemotherapy, and another had local recurrence and is receiving radiotherapy and chemotherapy after the second operation and now also stopped taking the medicine). The symptoms of nervous system have been significantly improved during postoperative chemotherapy.

CONCLUSIONTo reduce the misdiagnosis, regular CT imaging of the abdomen or pelvic should be ordered for all cases with OMS. The children with OMS-NB need to be actively treated with the combined modality therapy including surgery, chemotherapy or radiotherapy, to reduce recurrence and reduce the symptoms of nervous system.

Adrenal Gland Neoplasms ; complications ; diagnosis ; surgery ; therapy ; Antineoplastic Agents ; administration & dosage ; Antineoplastic Combined Chemotherapy Protocols ; therapeutic use ; Biomarkers ; analysis ; Chemotherapy, Adjuvant ; Child, Preschool ; Combined Modality Therapy ; Female ; Follow-Up Studies ; Humans ; Infant ; Male ; Neoplasm Recurrence, Local ; surgery ; therapy ; Neoplasm Staging ; Neuroblastoma ; complications ; diagnosis ; surgery ; therapy ; Opsoclonus-Myoclonus Syndrome ; diagnosis ; etiology ; therapy ; Retroperitoneal Neoplasms ; complications ; diagnosis ; surgery ; therapy ; Retrospective Studies ; Tomography, X-Ray Computed ; Treatment Outcome

Actins ; metabolism ; Adrenal Gland Neoplasms ; diagnostic imaging ; metabolism ; pathology ; surgery ; Desmin ; metabolism ; Diagnosis, Differential ; Female ; Humans ; Immunohistochemistry ; Leiomyosarcoma ; diagnostic imaging ; metabolism ; pathology ; surgery ; Middle Aged ; Neoplasm Recurrence, Local ; surgery ; Reoperation ; Tomography, X-Ray Computed ; Vimentin ; metabolism

Adrenal gland tumours in pregnancy are very rare occurrences and have highly variable clinical presentations. The timely diagnosis of adrenal tumours in pregnancy is extremely important, as failure to do so may lead to fatality. As there is limited published literature on adrenal tumours in pregnancy and no consensus on its management, the management of such patients with regard to medical and surgical treatments, as well as timing of delivery, must therefore be individualised and carried out with multidisciplinary expertise. We present two cases of adrenal tumours in pregnancy, both with favourable outcomes and variable gestations. Our first and second cases discuss a large phaeochromocytoma and a cortisol-secreting adrenal cortical adenoma in pregnancy, respectively.
Adrenal Gland Neoplasms ; diagnosis ; surgery ; Adrenalectomy ; Adult ; Biomarkers ; blood ; urine ; Diagnosis, Differential ; Female ; Humans ; Laparoscopy ; Magnetic Resonance Imaging ; Pheochromocytoma ; diagnosis ; surgery ; Pregnancy ; Pregnancy Complications, Neoplastic ; diagnosis

In patients with primary aldosteronism who have bilateral adrenal incidentalomas, it is important to identify which adrenal gland is secreting excess aldosterone. Traditionally, adrenal vein sampling (AVS) has been performed for lateralization despite its invasiveness. Here we report a case of bilateral adrenal incidentaloma in which 18-Fluorodeoxyglucose (FDG)-positron emission tomography (PET) was used to identify the functional adrenal mass. A 53-yr-old man was referred to our clinic due to bilateral adrenal incidentalomas (right: 1 cm, left: 2.5 cm) on computed tomography (CT). Given his history of colon cancer, FDG-PET/CT scanning was used to rule out metastasis. Although there was focal hot uptake lesion in the right adrenal gland, the patient was suspected primary aldosteronism clinically more than metastasis because of the patient's underlying hypertension with hypokalemia. It was consistent with the results of AVS. Based on these findings, we propose that FDG-PET/CT can be used instead of AVS to identify the source of primary aldosteronism between two bilateral adrenal incidentalomas.
Adrenal Gland Neoplasms/*diagnosis/pathology/radionuclide imaging ; Adrenal Glands/pathology/surgery ; Fluorodeoxyglucose F18/diagnostic use ; Humans ; Hyperaldosteronism/*diagnosis/pathology ; Hypertension/diagnosis ; Hypokalemia/diagnosis ; Male ; Middle Aged ; Positron-Emission Tomography and Computed Tomography

Although the adrenal gland is a common site of metastasis from hepatocellular carcinoma (HCC), adrenal metastases are rarely seen in clinical practice because of its lower metastatic potential compared to the other malignancies. Adrenal metastases usually were detected at the time of diagnosis of primary HCC or simultaneously with intrahepatic recurrence after curative management of HCC. It is very rare that only metastatic HCC is detected without evidence of intrahepatic recurrence. Hereby, we report two cases of adrenal metastasis from HCC without intrahepatic recurrence after hepatic resection.
Adrenal Gland Neoplasms/*diagnosis/radionuclide imaging/secondary ; Aged, 80 and over ; Carcinoma, Hepatocellular/*pathology/surgery ; Humans ; Liver Neoplasms/*pathology/surgery ; Male ; Positron-Emission Tomography ; Tomography, X-Ray Computed

Adrenal Gland Neoplasms ; pathology ; surgery ; Adrenalectomy ; Child ; Diagnosis, Differential ; Follow-Up Studies ; Ganglioneuroblastoma ; pathology ; Hematopoiesis, Extramedullary ; Hodgkin Disease ; pathology ; Humans ; Leukemia ; pathology ; Male

BACKGROUNDAdrenal ganglioneuroma is a rare adrenal pathogenic disease with difficult differential diagnosis from adrenal pheochromocytoma. Currently, very limited literature is available to allow a differential diagnosis of these two conditions from each other. This study aimed to evaluate the clinical profile, differential diagnosis and surgical treatments of both conditions.

METHODSClinical characteristics of 36 patients with adrenal pheocheomocytoma and 18 patients with adrenal ganglioneuroma were analyzed. Data from CT scans and surgical treatments from 1999 to 2007 were collected. Endocrine hormone tests and (131)I-metaiodobenzylguanidine (MIBG) were performed. Neither (131)I-MIBG nor endocrine hormone tests were available in 9 cases of asymptomatic adrenal ganglioneuroma with tumor size less than 4 cm and there were negative findings from contrast enhanced CT scans. The level of urine catecholamine of patients was compared by one-way analysis of variance.

RESULTSTe mean age of patients in the adrenal ganglioneuroma group was 41.2 years (16 - 67 year) and in the adrenal pheochromocytoma patients 38 years (17 - 74 year). Contrast enhanced CT showed that the foci were intensified in 5 cases (27.8%) of adrenal ganglioneuroma and there were obvious contrast indications in 30 (83.3%) of the pheochromocytoma. Catecholamine levels in a 24-hour urine sample were normal in 4 patients with adrenal ganglioneuroma and increased in 36 (100%) cases with adrenal pheochromocytoma. (131)I-MIBG nuclear scan showed negative results in 4 patients (100%) with adrenal ganglioneuroma and positive results in 25 (96.2%) with adrenal pheochromocytom. Laparoscopy for adrenal tumors was performed through a transperitoneal or retroperitoneal approach during a follow-up period of (43 +/- 6) months, and all cases survived well.

CONCLUSIONSCT, urinary catecholamine and (131)I-MIBG are standard and efficient tools for differential diagnosis of adrenal ganglioneuroma from pheochromocytoma. Laparoscopic surgery can be performed through a transperitoneal or retroperitoneal approach depending on the finding of CT scans. Open surgery is necessary for patients with blood loss of more than 800 ml and violent fluctuation of intraoperative blood pressure.

Adolescent ; Adrenal Gland Neoplasms ; diagnosis ; surgery ; Adult ; Aged ; Female ; Ganglioneuroma ; diagnosis ; surgery ; Humans ; Laparoscopy ; methods ; Male ; Middle Aged ; Pheochromocytoma ; diagnosis ; surgery ; Retrospective Studies ; Young Adult

Paraganglioma is a rare neuroendocrine tumor arising from the neural crest, which includes tissues such as the adrenal medulla, carotid and aortic body, organs of Zuckerkandl, and other unnamed paraganglia. The head, neck, and retroperitoneum are the most common sites for paraganglioma. However, paraganglioma of the pancreas is extremely rare. We report our experience of this rare disease. A 70-year old female patient admitted for a pancreas tail mass detected by computed tomography (CT) scan checked for vague left flank pain. CT with contrast enhancement showed a 4.2-cm heterogeneously enhanced lesion in the tail of the pancreas. A well defined ovoid shape mass in left adrenal gland was suggested adenoma. Distal pancreatectomy with left adrenalectomy was performed. Any lymph node enlargement was not found. Pancreas mass did not invade adjacent organ. Microscopic examination with pancreas and adrenal gland revealed that the cells were arranged in a characteristic Zellballen pattern. Immunohistochemical staining revealed positivity for neuron-specific enolase, chromogranin A, synaptophysin, and S-100 protein. On the basis of these findings, we diagnosed the tumor as a paraganglioma of the pancreas and adrenal gland. We report the first case of pancreas paraganglioma in Korea.
Adenoma/diagnosis/surgery ; Adrenal Gland Neoplasms/*diagnosis/secondary/surgery ; Aged ; Chromogranin A/metabolism ; Female ; Humans ; Pancreatic Neoplasms/*diagnosis/pathology/surgery ; Paraganglioma/*diagnosis/secondary/surgery ; Phosphopyruvate Hydratase/metabolism ; S100 Proteins/metabolism ; Synaptophysin/metabolism ; Tomography, X-Ray Computed

OBJECTIVETo study and summarize the diagnosis and treatment for the corticomedullary mixed tumor of adrenal gland.

METHODSThe clinical data of 25 cases of adrenal corticomedullary mixed tumor from January 2000 to April 2008 were analyzed retrospectively, which including 9 males and 16 females. The ages were from 25 to 60 years old, and the average age was 39 years old. Thirteen cases had paroxysmal hypertension and 11 cases had central obesity, as well as 8 cases with hypokalemia. There were different degree abnormalities in plasma endocrine hormones in laboratory examination. Every case underwent b-ultrasound and CT normal plus extensive scan to make the diagnosis.

RESULTSAdrenalectomy was performed in the 25 cases, which contain 9 cases of open operations and 16 cases of endoscopic adrenalectomies. All of the cases had blood pressure fluctuation during dissection of the adrenal tumors, with the highest blood pressure reached to 230/140 mm Hg (1 mm Hg = 0.133 kPa). Postoperative histopathological study revealed that the pathological changes was corticomedullary mixed tumor of adrenal gland, which was supported by immunohistochemical study.

CONCLUSIONSIn cases with complex phenomenon that can't explain with single cortical or medullary changes, it must beware of the mixed pathological changes in adrenal gland.

Adrenal Gland Neoplasms ; diagnosis ; surgery ; Adrenalectomy ; Adult ; Female ; Humans ; Male ; Middle Aged ; Retrospective Studies