Approximately 10–15% of pheochromocytomas are malignant. There are insufficient histologic criteria for the diagnosis of malignant pheochromocytoma. Thus, the term malignant pheochromocytoma is restricted to tumors with local invasion or distant metastases. We experienced a case of malignant pheochromocytoma recurred with spinal metastasis 4 years after the surgery for huge benign pheochromocytoma. A 68-year-old female was admitted for trunk and back pain. The patient had a history of surgery 4 years ago for a 10.0×9.5×7.5 cm sized benign pheochromocytoma at the left adrenal gland. A thoracolumbar magnetic resonance imaging showed a tumor in the 7th thoracic vertebral body and a 24-hour urinary norepinephrine increased, suggesting metastatic recurrence of malignant pheochromocytoma. After metastasectomy in the 7th thoracic vertebral body, urine catecholamine was normalized and pain also disappeared. However, a metastatic lesion was found in the paraaortic area on a follow-up abdominal computed tomography scan and an additional metastasectomy was performed. The pathology confirmed the diagnosis of metastatic pheochromocytoma in the paraaortic lymph nodes. She is supposed to be treated with adjuvant iodine 131-meta-iodobenzylguanidine therapy. In our experience, a close follow-up should be considered in patients who had a huge benign pheochromocytoma due to the possibility of malignant metastases.
Adrenal Gland Neoplasms ; Adrenal Glands ; Aged ; Back Pain ; Catecholamines ; Diagnosis ; Female ; Follow-Up Studies ; Humans ; Iodine ; Lymph Nodes* ; Magnetic Resonance Imaging ; Metastasectomy ; Neoplasm Metastasis* ; Norepinephrine ; Pathology ; Pheochromocytoma* ; Recurrence ; Spine*

Approximately 10–15% of pheochromocytomas are malignant. There are insufficient histologic criteria for the diagnosis of malignant pheochromocytoma. Thus, the term malignant pheochromocytoma is restricted to tumors with local invasion or distant metastases. We experienced a case of malignant pheochromocytoma recurred with spinal metastasis 4 years after the surgery for huge benign pheochromocytoma. A 68-year-old female was admitted for trunk and back pain. The patient had a history of surgery 4 years ago for a 10.0×9.5×7.5 cm sized benign pheochromocytoma at the left adrenal gland. A thoracolumbar magnetic resonance imaging showed a tumor in the 7th thoracic vertebral body and a 24-hour urinary norepinephrine increased, suggesting metastatic recurrence of malignant pheochromocytoma. After metastasectomy in the 7th thoracic vertebral body, urine catecholamine was normalized and pain also disappeared. However, a metastatic lesion was found in the paraaortic area on a follow-up abdominal computed tomography scan and an additional metastasectomy was performed. The pathology confirmed the diagnosis of metastatic pheochromocytoma in the paraaortic lymph nodes. She is supposed to be treated with adjuvant iodine 131-meta-iodobenzylguanidine therapy. In our experience, a close follow-up should be considered in patients who had a huge benign pheochromocytoma due to the possibility of malignant metastases.
Adrenal Gland Neoplasms ; Adrenal Glands ; Aged ; Back Pain ; Catecholamines ; Diagnosis ; Female ; Follow-Up Studies ; Humans ; Iodine ; Lymph Nodes ; Magnetic Resonance Imaging ; Metastasectomy ; Neoplasm Metastasis ; Norepinephrine ; Pathology ; Pheochromocytoma ; Recurrence ; Spine

OBJECTIVETo evaluate the CT and MR findings of retroperitoneal ectopic pheochromocytoma.

METHODSTo analyze retrospectively the CT and MR images of 32 patients with retroperitoneal ectopic pheochromocytoma proved by pathology.

RESULTSThe lesions (benign=28, malignant=4) were located in the anterior pararenal space (ARS) (n=12), the perirenal space (PS) (n=13) and the posterior pararenal space (PRS) (n=7). The tumors showed heterogeneous density on unenhanced CT (n=25). Among the 23 cases with enhanced CT imaging, 19 cases had marked contrast and 4 had mild contrast. The enhancement patterns included whole enhancement (n=9), solid area enhancement (n=12), peripheral enhancement (n=1), and spotted enhancement (n=1). The tumors had heterogeneous signal on unenhanced MR (n=23), and usually showed enhancement at arterial, portal and delayed phases on 22 enhanced MR, while cystic area with no enhancement. The lesions usually had cystic changes (n=18), septa (n=16), vessels inside (n=9), hemorrhage (n=3), and calcification (n=3). Besides that the morphology had statistical significance (P=0.013), other indexes had no statistical significance (P>0.05) in differential diagnosis of benign and malignant retroperitoneal ectopic pheochromocytomas.

CONCLUSIONSRetroperitoneal ectopic pheochromocytomas have some CT and MR features, usually revealed as an oval mass, growing along the paravertebral axis, and often with cystic changes. Those signs combined with hypertension and elevated catecholamine level may lead to a correct diagnosis.

Adrenal Gland Neoplasms ; diagnostic imaging ; pathology ; Calcinosis ; Diagnosis, Differential ; Humans ; Hypertension ; Image Enhancement ; Pheochromocytoma ; diagnostic imaging ; pathology ; Retroperitoneal Space ; Retrospective Studies ; Tomography, X-Ray Computed

Adrenal Cortex Neoplasms ; complications ; Adrenal Gland Neoplasms ; complications ; Adrenal Glands ; pathology ; Adrenocortical Adenoma ; complications ; Humans ; Pheochromocytoma ; complications

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

We report a case of a 74-year-old female with myxoid adrenocortical adenoma which showed different magnetic resonance imaging findings compared to those of a typical adrenocortical adenoma. The myxoid change in the adrenocortical adenoma is a rare form of degeneration. It presents a considerable diagnostic challenge to both radiologists and clinicians because it can mimic other adrenal tumor types on imaging. The MRI findings of the presented case included a high signal intensity on T2-weighted images similar to that of fluid and delayed progressive enhancement.
Adenoma/*diagnosis/pathology ; Adrenal Gland Neoplasms/*diagnosis/pathology ; Adrenocortical Adenoma/*diagnosis/pathology ; Aged ; Diagnosis, Differential ; Female ; Humans ; Magnetic Resonance Imaging ; Rare Diseases/*diagnosis/pathology

We present two cases of adrenocortical oncocytomas that were well-delineated on multi-detector computed tomography and magnetic resonance imaging. The images showed a well-enhanced large mass with multiple stippled calcifications in a 10-yr-old girl who was consulted due to precocious puberty. A well-enhanced solid mass with necrotic components was incidentally noticed in a 54-yr-old man. These lesions were resected and diagnosed as adrenocortical oncocytomas through immunohistochemical studies and electron microscopy. Adrenocortical oncocytomas are rare disease entities, therefore, we report these interesting, rare adrenocortical oncocytomas here with radiologic findings, and discuss differential diagnosis.
Adenoma, Oxyphilic/*diagnosis/pathology/radiography ; Adrenal Gland Neoplasms/*diagnosis/pathology/radiography ; Child ; Diagnosis, Differential ; Female ; Hemorrhage ; Humans ; Immunohistochemistry ; Magnetic Resonance Imaging ; Male ; Middle Aged ; Tomography, X-Ray Computed

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

Pheochromocytoma is a rare cause of hypertension in children. Hypertension is one of the common reasons of posterior reversible encephalopathy. Intracerebral hemorrhage is a serious and unexpected complication of hypertensive encephalopathy due to pheochromocytoma, and very rarely seen in the childhood. Intracerebral hemorrhages should be searched if there are hypertensive reversible signal changes on the brain. Susceptibility weighted imaging (SWI) is a more sensitive method than conventional MRI when demonstrating cerebral microhemorrhagic foci. This is the first report of SWI findings on intracerebral hemorrhages in basal ganglia, brain stem and periventricular white matter due to hypertensive encephalopathy in a child with pheochromocytoma.
Adolescent ; Adrenal Gland Neoplasms/*complications/diagnosis ; Brain/*pathology ; Diagnosis, Differential ; Female ; Humans ; Hypertensive Encephalopathy/*diagnosis/etiology ; Magnetic Resonance Imaging/*methods ; Pheochromocytoma/*complications/diagnosis

OBJECTIVETo investigate the molecular genetic abnormalities of N-myc and C-myc, and their clinical pathological implications in pediatric neuroblastic tumors (NTs).

METHODSAbnormalities of N-myc were detected by interphase fluorescence in situ hybridization (FISH) technique in 246 cases of NTs, including neuroblastoma (NB,188 cases), ganglioneuroblastoma (GNB, 52 cases), ganglioneuroma (GN, 6 cases), and their association with the histological typing of the tumors and prognosis was analyzed. Abnormalities of C-myc were detected by FISH in 133 cases of NTs.

RESULTSOf the 246 cases of NTs, N-myc amplification was only found in 27 cases (11.0%, 27/246) of NB, but not in any cases of GNB or GN (P < 0.05). 89.0% (219/246) N-myc non-amplification were found in NTs, and it included N-myc gain in 175 cases (71.1%, 175/246) and normal N-myc in 44 cases (17.9%, 44/246). Univariate analysis indicated significantly (P = 0.012) poorer outcome in patients with N-myc amplification than N-myc non-amplification. However no significant difference was observed between N-myc gain cases and normal N-myc cases (P = 0.057). C-myc gain was found in 74 of 133 cases (55.6%) of NTs; no C-myc amplification or translocation was detected. Forty percent (6/15) of cases with N-myc amplification and 57.6% (68/118) of cases with N-myc non-amplification were accompanied by C-myc gain. The difference between N-myc amplification and non-amplification with C-myc gain was not significant (P > 0.05). Univariate analysis indicated that the outcome difference was not statistically significant between C-myc gain cases and normal C-myc cases (P = 0.357).

CONCLUSIONSThe incidence of N-myc amplification only found in NB is low in pediatric NTs in China. Patients with N-myc amplification predict poorer outcome. No amplification or translocation of C-myc is detected in NTs, whereas C-myc gain is relatively common in NTs. There is no obvious association between N-myc amplification and C-myc gain.

Adrenal Gland Neoplasms ; genetics ; pathology ; Child ; Child, Preschool ; Female ; Follow-Up Studies ; Ganglioneuroblastoma ; genetics ; pathology ; Ganglioneuroma ; genetics ; pathology ; Gene Amplification ; Genes, myc ; Humans ; In Situ Hybridization, Fluorescence ; Infant ; Male ; Mediastinal Neoplasms ; genetics ; pathology ; Neuroblastoma ; genetics ; pathology ; Survival Rate