Sporadic malignant pheochromocytoma, a rare disease with poor prognosis, is always difficult to treat due in part to lack of effective agents. We presented three patients with advanced malignant pheochromocytoma treated by sunitinib, which indicates that sunitinib is an effective agent for this malignancy.
Adrenal Gland Neoplasms ; drug therapy ; Adult ; Female ; Humans ; Indoles ; therapeutic use ; Male ; Middle Aged ; Pheochromocytoma ; drug therapy ; Protein Kinase Inhibitors ; therapeutic use ; Pyrroles ; therapeutic use

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

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

OBJECTIVETo evaluate the clinical characteristics and prognosis of adrenal metastasis from breast cancer, and to explore methods to improve prognosis.

METHODSThirty-four breast cancer patients with adrenal metastasis were diagnosed and treated in our hospital from Jan. 1999 to Dec. 2010. SPSS 17.0 was used for survival analysis.

RESULTSDuring the Jan. 1999 to Dec. 2010 period, 13 595 patients with breast cancer were treated in our hospital. Among them, 34 cases had adrenal metastasis from breast cancer, with an incidence of 0.25%. The median time to progression (TTP) and overall survival of the 34 patients was 6.2 months (95%CI 3.1-9.3 months) and 21.4 months (95%CI 0-44.0 months), respectively. Eleven patients (34.4%) achieved partial response among 32 patients who received chemotherapy, and 10 (31.2%) achieved stable disease. Patients who achieved best response of PR or SD were superior in TTP and OS than patients with disease progression after chemotherapy (TTP: 18.1 months vs. 2.3 months, P < 0.001; OS: 35.2 months vs. 10.3 months, P = 0.003). Patients who received 1st or 2nd line chemotherapy were superior in TTP than patients who received over 2nd line chemotherapy (TTP: 15.7 months vs. 4.2 months, P = 0.005).

CONCLUSIONSThe incidence of adrenal metastasis from breast cancer is low. Chemotherapy-based systemic therapy should be recommended to improve the prognosis for these patients.

Adrenal Gland Neoplasms ; drug therapy ; secondary ; surgery ; Adrenalectomy ; Adult ; Aged ; Antineoplastic Combined Chemotherapy Protocols ; therapeutic use ; Breast Neoplasms ; drug therapy ; pathology ; Carcinoma, Ductal, Breast ; drug therapy ; pathology ; surgery ; Disease Progression ; Female ; Humans ; Middle Aged ; Remission Induction ; Retrospective Studies ; Survival Rate

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

Primary adrenal lymphoma is a very rare extranodal lymphoma; its clinical features consist of a high incidence of bilateral adrenal involvement and diffuse large B-cell lymphoma. We report a patient with primary bilateral adrenal diffuse large B-cell lyphoma who achieved complete remission with R-CHOP (rituximab-cyclophosphamide, doxorubicin, vincristine, and prednisone) chemotherapy. A 52-yr-old man presented with fever and progressive fatigue for 3 months. Computed tomography (CT) scans of the abdomen and pelvis demonstrated large bilateral adrenal masses, and a needle biopsy of the left adrenal mass revealed diffuse large B-cell lymphoma. After 6 cycles of R-CHOP chemotherapy, CT scans showed no residual disease. To our knowledge, this is the second report to date of a patient with primary bilateral adrenal diffuse large B-cell lymphoma who achieved complete remission using R-CHOP chemotherapy.
Adrenal Gland Neoplasms/*drug therapy/pathology/radiography ; Antibodies, Monoclonal/*therapeutic use ; Antineoplastic Combined Chemotherapy Protocols/*therapeutic use ; Cyclophosphamide/therapeutic use ; Doxorubicin/therapeutic use ; Drug Therapy, Combination ; Humans ; Lymphoma, Large B-Cell, Diffuse/*drug therapy/pathology/radiography ; Male ; Middle Aged ; Positron-Emission Tomography ; Prednisone/therapeutic use ; Tomography, X-Ray Computed ; Vincristine/therapeutic use

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