Primary adrenal lymphoma is extremely rare. We describe a case of non-Hodgkin's lymphoma of diffuse large B-cell type with right adrenal involvement. The patient received chemotherapy and external irradiation and achieved complete remission of the disease. We describe the case of primary adrenal lymphoma with a review of the literature on this unusual neoplasm. Primary adrenal lymphoma should be included in the differential diagnosis of adrenal mass.
Adrenal Gland Neoplasms/*diagnosis/therapy ; Human ; Lymphoma, Large-Cell/*diagnosis/therapy ; Male ; Middle Aged ; Treatment Outcome

Ectopic Cushing’s syndrome caused by pheochromocytoma is rare. We reported a 15-year-old female patient who was admitted to hospital with typical Cushing’s syndrome. She had not started menstruation. Her plasma adrenocorticotropic hormone (ACTH) and 24-hour urinary free cortisol levels were extremely high. Gonadal and progestational hormone levels were also abnormal. Abdominal computed tomography scans and enhanced scans revealed multiple irregular tumors in the right adrenal. Pelvic echogram showed an infantile uterus, while the ovaries were at an immature stage of development. Retroperitoneal laparoscopic right adrenalectomy was performed without intraoperative complications. Histology and immunohistochemistry of the tumor were consistent with pheochromocytoma. Retroperitoneal laparoscopic adrenalectomy is a safe procedure with satisfactory outcomes and allows for rapid recovery.
Adolescent ; Adrenal Gland Neoplasms ; diagnosis ; secretion ; therapy ; Adrenocorticotropic Hormone ; secretion ; Female ; Humans ; Pheochromocytoma ; diagnosis ; secretion ; therapy

OBJECTIVETo evaluate the diagnosis and treatment of adrenal incidentalomas.

METHODSOne hundred and twenty-six patients were analyzed, in which 98 patients underwent operation.

RESULTSOf all types of the adrenal incidentalomas, 52 (41.3%) of them were adenomas and they were the most often; 43 (34.1%) of them were hypersecretory adrenal tumors, most of them were pheochromocytomas and Conn adenomas; all nonhypersecretory adrenal adenomas were under 6 cm, and all adrenal carcinomas were above 6 cm.

CONCLUSIONSIt is important to search for hypersecretory adrenal tumors and to detect malignant adrenal tumors. To nonhypersecretory adrenal adenomas, the sizes of tumors are the most important indexes in determining weather the tumors are benign or malignant and weather the tumors need to be treated with operation or not.

Adolescent ; Adrenal Gland Neoplasms ; classification ; diagnosis ; therapy ; Adrenocortical Adenoma ; diagnosis ; therapy ; Adult ; Aged ; Child ; Female ; Humans ; Hyperaldosteronism ; diagnosis ; therapy ; Male ; Middle Aged ; Pheochromocytoma ; diagnosis ; therapy

Pheochromocytoma in pregnancy is very rare but it is associated with very high maternal and fetal mortality. Therefore, it is important to include pheochromocytoma in the differential diagnosis of hypertension associated with pregnancy. It is difficult to make a diagnosis of pheochromocytoma in pregnancy before delivery. The characteristic symptoms of pheochromocytoma could be initiated during delivery because the process of delivery, general anesthesia, fetal movement, induce acute surge of catecholamine release, which could also induce cardiomyopathy. Early diagnosis and intensive care can affect the prognosis of cardiomyopathy induced by pheochromocytoma. Proper management with alpha-blockade, beta-blockade and angiotension converting enzyme inhibitor could acutely reverse the course of cardiomyopathy.
Adrenal Gland Neoplasms/surgery ; Adrenal Gland Neoplasms/diagnosis* ; Adrenal Gland Neoplasms/complications ; Adult ; Cardiovascular Agents/therapeutic use ; Disease-Free Survival ; Echocardiography ; Electrocardiography ; Female ; Human ; Myocardial Diseases/ultrasonography ; Myocardial Diseases/etiology* ; Myocardial Diseases/drug therapy ; Pheochromocytoma/surgery ; Pheochromocytoma/diagnosis* ; Pheochromocytoma/complications ; Pregnancy ; Pregnancy Complications, Cardiovascular/etiology* ; Pregnancy Complications, Neoplastic/surgery ; Pregnancy Complications, Neoplastic/diagnosis* ; Pregnancy Outcome* ; Puerperium ; Tomography, X-Ray Computed ; Substances: Cardiovascular Agents

We report a case of non-Hodgkin's lymphoma(NHL) with simultaneous involvement of both thyroid and bilateral adrenal glands. Literature review on a computerized search showed that this is an extremely rare condition. The final diagnosis of diffuse large B cell lymphoma was confirmed by biopsies of thyroid gland, enlarged cervical lymph node, and adrenal gland. The significant endocrine dysfunction of the thyroid, adrenal or other endocrine glands was absent in our case. The patient responded dramatically to three cycles of chemotherapy with no complication or endocrine dysfunction and continues to be followed.
Adrenal Gland Neoplasms/drug therapy ; Adrenal Gland Neoplasms/diagnosis+ACo- ; Aged ; Antineoplastic Agents, Combined/therapeutic use ; Biopsy, Needle ; Case Report ; Cyclophosphamide/administration +ACY- dosage ; Doxorubicin/administration +ACY- dosage ; Female ; Follow-Up Studies ; Human ; Lymphoma, Large-Cell/drug therapy ; Lymphoma, Large-Cell/diagnosis+ACo- ; Prednisolone/administration +ACY- dosage ; Thyroid Neoplasms/drug therapy ; Thyroid Neoplasms/diagnosis+ACo- ; Treatment Outcome ; Vincristine/administration +ACY- dosage

Hyperadrenocorticism, a disorder characterized by excessive production of cortisol by the adrenal cortex, is wellrecognized in dogs. A 10-year-old, intact male, Yorkshire terrier dog was evaluated because of corneal ulceration and generalized alopecia. Diagnosis was made based on history taking, clinical signs, physical examination, and results of routine laboratory testing (complete blood count, serum biochemical analysis, and urinalysis). In addition, adrenocorticotropic hormone (ACTH) stimulation test and abdominal ultrasonography were also used to diagnose this case. The patient was diagnosed as adrenal gland neoplasia and medical therapy using the adrenocorticolytic agent, mitotane, was initiated. An ACTH stimulation test was performed after initial therapy. After successful induction was obtained, maintenance therapy with mitotane still continued.
Adrenal Gland Neoplasms/complications/diagnosis/drug therapy/*veterinary ; Adrenocortical Hyperfunction/diagnosis/drug therapy/etiology/*veterinary ; Adrenocorticotropic Hormone/blood ; Animals ; Antineoplastic Agents, Hormonal/*therapeutic use ; Dog Diseases/*drug therapy ; Dogs ; Male ; Mitotane/*therapeutic use ; Radiography, Abdominal/veterinary ; Tomography, X-Ray/veterinary

OBJECTIVETo analyze the clinical features of adrenal metastasis.

METHODSFrom January 1993 to December 2004, 103 cases of adrenal metastasis were reviewed.

RESULTSLung and hepatocellular carcinoma were the most common primary tumor of adrenal metastatic tumor, which about 36.9% (38/103) and 42.7% (44/103) of all cases, followed by renal carcinoma 6.8% (7/103), colorectal carcinoma 4.9% (5/103), stomach carcinoma 3.9% (4/103), breast cancer 1.9% (2/103), unknown primary tumor 2.9% (3/103). Most of these were low differentiation. The mean diameter of adrenal metastasis was 3.9 cm. The mean interval from detection of primary tumor to adrenal metastasis was 9.5 months. And 79.6% (82/103) were detected as a part of multiorgan metastasis. Only 5 cases (4.9%) were presented with pain in the back. There was little characterization of ultrasonography, CT and MRI, color-Doppler and selective arterial imaging showed little blood supply. All of patients were treated with synthetic methods, 16 cases (15.5%) who had undergone adrenalectomy for metastasis disease had a improved survival compared with those non-adrenalectomy.

CONCLUSIONSThere is no particular presentation of clinic and imaging, diagnosis depending on history, follow-up and the pathological presentation of primary tumor. There are no standard treatment guidelines for this group of patients. When the primary tumor could be resected or be well controlled, and there is no other evidence of metastasis, adrenalectomy is recommended. Transarterial chemoembolization (TACE) could not actually be performed.

Adrenal Gland Neoplasms ; diagnosis ; secondary ; therapy ; Adult ; Aged ; Aged, 80 and over ; Carcinoma, Hepatocellular ; pathology ; Combined Modality Therapy ; Female ; Humans ; Liver Neoplasms ; pathology ; Lung Neoplasms ; pathology ; Male ; Middle Aged ; Retrospective Studies ; Survival Analysis ; Treatment Outcome

BACKGROUND/AIMS: We investigated the clinical characteristics and follow-up findings of subjects with adrenal incidentalomas in a single, tertiary-care hospital in South Korea. METHODS: The study consisted of a retrospective analysis of 282 adrenal incidentaloma patients who underwent radiographic and endocrinological evaluations at Samsung Medical Center in Seoul, South Korea, between January 2004 and July 2011. RESULTS: Most (86.2%) of the subjects were found to have nonfunctioning tumors. Functioning tumors were seen in 39 patients (13.8%). Among them, 28 (9.9%) had subclinical Cushing syndrome (SCS), six (2.1%) had pheochromocytoma, and five (1.8%) had primary hyperaldosteronism. Malignant adrenal tumors were discovered in three cases: two (0.7%) were primary adrenal cancers, and one (0.4%) was a secondary metastasis from a lung cancer. Significant risk factors for functional tumors were female gender (odds ratio [OR], 3.386; 95% confidence interval [CI], 1.611 to 7.117; p = 0.0013) and a noncontrast attenuation value of > 10 Hounsfield units (OR, 2.806; 95% CI, 1.231 to 6.397; p = 0.0141). During follow-up (mean, 22.5 months) of 72 of the patients, three (4.2%) developed hormonal changes due to functional tumors. One was confirmed as pheochromocytoma by histopathology, and the others were diagnosed with SCS and followed routinely without surgical intervention. No malignant transformation was found in these patients. CONCLUSIONS: Based on these findings, initial hormonal and radiographic evaluations for adrenal incidentalomas appear to be more important than follow-up tests because functional or malignant changes are rare.
*Adrenal Gland Neoplasms/blood/diagnosis/epidemiology/therapy ; Aged ; *Cushing Syndrome/blood/diagnosis/epidemiology/therapy ; Disease Progression ; Female ; Hormones/blood ; Humans ; *Hyperaldosteronism/blood/diagnosis/epidemiology/therapy ; Logistic Models ; Male ; Middle Aged ; Odds Ratio ; *Pheochromocytoma/blood/diagnosis/epidemiology/therapy ; Predictive Value of Tests ; Prognosis ; Republic of Korea/epidemiology ; Retrospective Studies ; Risk Factors ; Tertiary Care Centers ; Time Factors ; Tomography, X-Ray Computed ; Tumor Markers, Biological/blood

PURPOSE: Adrenocortical tumors are uncommon in children and comprise only a small proportion of primary adrenal neoplasms. The biologic behavior of these tumors may be very difficult to predict, and their rarity has hindered identification of clinical characteristics. Patients with functioning tumors have excessive steroid hormone production, and the clinical manifestation depends on the predominant hormone produced. The detection of nonfunctioning tumors is not easy and the diagnosis may be delayed. Benign tumors can be cured by complete surgical excision, but malignant cases have poor response to treatment and worse prognosis. Early diagnosis and proper management are very important because of the large proportion of functioning malignant tumors in children. We report clinical features of adrenocortical tumors in children that may be of help in the early detection, proper management, and assessment of prognosis of patients. METHODS: We reviewed the clinical characteristics of 14 cases of adrenocortical tumors, among 85 children diagnosed with adrenal tumors, who visited the Severance Hospital, College of Medicine, Yonsei University, from January 1970 to July 1996. RESULTS: 85 Patients were diagnosed with adrenal tumors. Among them, 71 cases 83.5%) were tumors of the adrenal medulla, neuroblastoma and pheochromocytoma, and 14 cases (16.5%) were adrenocortical tumors, consisting of 5 cases of adenoma, 7 cases of carcinoma, and 2 cases unspecified. The age distribution ranged from 16 months to 14 years of age, and the mean was 5 years & 11 months (median 4 years & 2 months). Sex distribution revealed a male to female ratio of 1:1.33. The left to right ratio was 3.7:1, showing a left side predominance. 13 Cases (92.9%) were functioning tumors: 12 cases (92.3%) had clinical evidence of androgen excess, among which 6 cases (46.2%) were associated with Cushing's syndrome, and 1 case was compatible with primary aldosteronism. Serum cortisol, urinary 17-ketosteroids and 17-hydroxycorticosteroids concentrations were measured in 11 cases and urinary concentrations of 17-ketosteroids were elevated in all 11 cases (100%), while 17-hydroxycorticosteroids were elevated in 4 cases (36.4%). Abnormalities of serum cortisol were found in all cases except 1: serum cortisol concentrations were abnormally elevated in 5 cases (45.5%), and the remainder (5 cases, 45.5%) showed loss of diurnal variation. Dexamethasone suppression test was performed in 9 cases, and all (100%) showed no suppression. Preoperative radiologic studies included abdominal sonograms, CT or MRI scans, and angiography. Histology showed carcinomas to be bigger and heavier than adenomas, and microscopically carcinomas had necrosis, calcifications, and invasions of vessels. Distant metastases were found in 4 cases (12.7%). Adrenalectomy with complete surgical excision was performed in 12 cases. Long-term follow-up was possible in 10 patients after operation: 3 patients initially diagnosed with adenoma survived without tumor recurrence over a year, and among 6 carcinoma patients, 4 expired within a year, and 2 survived, with one patient currently undergoing postoperative chemotherapy. Of the 10 patients currently under going follow-up, one patient was initially diagnosed with a histologically unspecified tumor, and has survived 4 years after operation. CONCLUSIONS: When adrenocortical neoplasms are suspected by clinical symptoms and laboratory findings, abdominal ultrasonogram, CT or MRI scans must be performed immediately. Early detection and proper management are important for better prognosis, but are often delayed in the majority of cases. Ultimately, pediatricians need to be familiar with clinical characteristics and laboratory findings of adrenocortical tumors, bearing in mind the possibility of diagnosis in children.
17-Hydroxycorticosteroids ; 17-Ketosteroids ; Adenoma ; Adrenal Gland Neoplasms ; Adrenal Medulla ; Adrenalectomy ; Age Distribution ; Angiography ; Child ; Cushing Syndrome ; Dexamethasone ; Diagnosis ; Drug Therapy ; Early Diagnosis ; Female ; Follow-Up Studies ; Humans ; Hydrocortisone ; Hyperaldosteronism ; Magnetic Resonance Imaging ; Male ; Necrosis ; Neoplasm Metastasis ; Neuroblastoma ; Pheochromocytoma ; Prognosis ; Recurrence ; Sex Distribution ; Ultrasonography

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