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

Hydatid disease is endemic in parts of India, yet genitourinary involvement is rare. Laparoscopic management of such cases is uncommonly reported. We present a case of an adrenal hydatid and its management by laparoscopic aspiration, instillation of scolicidal solution, and partial excision of the cyst.
Adrenal Gland Diseases/pathology/radiography/*therapy ; Albendazole/therapeutic use ; Anticestodal Agents/therapeutic use ; Combined Modality Therapy ; Echinococcosis/pathology/radiography/*therapy ; Female ; Humans ; Laparoscopy/*methods ; Middle Aged ; Tomography, X-Ray Computed

OBJECTIVETo explore the clinical characteristics of Wolman disease and diagnostic methods using enzymatic and molecular analysis.

METHODLysosomal acid lipase activity was measured using 4-methylumbelliferyl oleate in the leukocytes of an infant suspected of Wolman disease and LIPA gene mutational analysis was performed by PCR and direct sequencing in the proband and his parents. After the diagnosis was confirmed, the clinical, biochemical, radiological and histopathological findings in this case of Wolman disease were retrospectively reviewed.

RESULTThe sixteen-day-old boy was failing to thrive with progressive vomiting, abdominal distention and hepatosplenomegaly. Abdominal X-ray revealed adrenal calcifications which were confirmed on abdominal CT scan. Xanthomatosis were observed on enlarged liver, spleen and lymph nodes during abdominal surgery. Liver and lymph node biopsy showed foamy histiocytes. The lysosomal acid lipase activity in leukocytes was 3.5 nmol/(mg·h) [control 35.5 - 105.8 nmol/(mg·h)]. Serum chitotriosidase activity was 315.8 nmol/(ml·h) [control 0 - 53 nmol/(ml·h)]. The patient was homozygote for a novel insert mutation allele c.318 ins T, p. Phe106fsX4 in exon 4 on LIPA gene. His both parents were carriers of the mutation.

CONCLUSIONThe clinical features of Wolman disease include early onset of vomiting, abdominal distention, growth failure, hepatosplenomegaly and bilateral adrenal calcification after birth. A plain abdominal X-ray film should be taken to check for the typical pattern of adrenal calcification in suspected cases of Wolman disease. The enzymatic and molecular analyses of lysosomal acid lipase can confirm the diagnosis of Wolman disease.

Adrenal Gland Diseases ; etiology ; pathology ; Exons ; Humans ; Infant, Newborn ; Leukocytes ; enzymology ; Lipase ; blood ; genetics ; Liver ; pathology ; Lysosomes ; enzymology ; genetics ; Male ; Mutation ; Polymerase Chain Reaction ; Splenomegaly ; pathology ; Sterol Esterase ; genetics ; Tomography, X-Ray Computed ; Wolman Disease ; diagnosis ; enzymology ; genetics ; pathology

OBJECTIVETo analyze the change in the incidence of pheochromocytomas in adrenal medulla or extra-adrenal and multiple endocrine neoplasm type 2 (MEN2), to summarize the clinical characteristics of benign, potentially malignant and malignant pheochromocytomas and to investigate the correlation between clinical manifestations and pathological changes.

METHODSStatistic analysis was performed to detect the incidence, constituent ratio, mean diagnostic age, sex proportion and correlation between clinical manifestions and pathologic changes in pheochromocytomas in adrenal medulla or extra-adrenal gland and MEN2 from 1993 to 2008 in the Department of Pathology, the General Hospital of Tianjin Medical University with Runs test, ANOVA, t test and chi-square test.

RESULTSThe total number of biopsies within the 16 years was 167 702 cases (average 10 481 cases per year). The numbers (detectable rate) of total adrenal diseases, pheochromocytomas in adrenal medulla and extra-adrenal glands were 910 (0.54%), 139 (0.08%), and 42 (0.03%) cases, respectively. The numbers (constituent ratio) of benign, potentially malignant and malignant of pheochromocytomas in adrenal medulla were 102 cases (73.4%), 29 cases (20.9%) and 8 cases (5.7%), respectively; in the 102 cases of benign tumors, patients with MEN2 were 8 (7.8%); the three groups of the tumors in extra-adrenal sites were 18 (42.8%) cases, 12 (28.6%) cases and 12 (28.6%) cases. There were no changes in the detectable rate and constituent ratio of adrenal diseases, benign, potential malignant and malignant pheochromocytomas in adrenal medulla or extra-adrenal glands and patients with MEN2 during the past 16 years (P > 0.05), but there was a tendency that malignant transformation was gradually increased with age, which was more commonly found in male patients than females. The mean age at diagnosis of patients with benign and potentially malignant pheochromocytomas was 42.7 years (ranged from 10 - 74 years), and 40.1 years (13 - 66 years), respectively, which were younger than patients with malignant pheochromocytomas (51.6 years, P < 0.05); the mean age of patients with benign and potentially malignant pheochromocytomas in extra-adrenal sites was 43.1 years (ranged from 20 - 75 years) and 45.2 years (28 - 65 years) that were older than those with malignant (37.8 years, ranged from 14 - 58 years, P < 0.05). It was spectacular that patients with malignant pheochromocytoma in adrenal medulla (51.6 years) were older than that in extra-adrenal sites (37.8 years); all the patients with MEN2 were female benign pheochromocytoma in adrenal medulla, whose age (38.9 years) was younger than that of benign lesions (42.7 years, P < 0.05), in which thyroid medullary carcinoma appeared early than pheochromocytomas in adrenal medulla. The detectable rate of hypertension in patients with malignant pheochromocytomas in adrenal medulla and in extra-adrenal sites were less than that in benign and potentially malignant ones (P < 0.05). The bilateral lesions more commonly found in malignant pheochromocytoma (25.0%) than benign (15.7%) and potentially malignant pheochromocytomas (6.9%) only in adrenal medulla. Relapse rates in both adrenal and extra-adrenal tumors were rising from benign (11.8%, 0), potentially malignant (13.8%, 25.0%), to malignant (33.3%, 37.5%) groups; the average diameter of pheochromocytomas in both adrenal and extra-adrenal sites was increasing from benign (4.2 cm, 4.0 cm), potentially malignant (5.3 cm, 5.6 cm) to malignant (7.3 cm, 6.9 cm) groups (P < 0.05).

CONCLUSIONSThe diagnostic criteria of benign, potentially malignant and malignant pheochromocytomas in adrenal medulla and in extra-adrenal sites are well established according to the WHO classification of endocrine tumors (2004). The closer relationship is found between clinical manifestations and pathologic changes. The definite type and nature of pheochromocytomas are mainly rested upon the pathologic examination.

Adolescent ; Adrenal Gland Diseases ; pathology ; Adrenal Gland Neoplasms ; complications ; pathology ; Adrenal Medulla ; pathology ; Adult ; Age Factors ; Aged ; Carcinoma, Neuroendocrine ; Child ; Female ; Humans ; Hypertension ; etiology ; Male ; Middle Aged ; Multiple Endocrine Neoplasia Type 2a ; pathology ; Neoplasm Recurrence, Local ; Pheochromocytoma ; complications ; pathology ; Retroperitoneal Neoplasms ; pathology ; Thyroid Neoplasms ; pathology ; Urinary Bladder Neoplasms ; pathology ; Young Adult

Adrenal Gland Diseases ; etiology ; surgery ; Aged ; Bacteremia ; microbiology ; Fatal Outcome ; Hemorrhage ; etiology ; pathology ; surgery ; Humans ; Lung Neoplasms ; complications ; diagnosis ; pathology ; Male ; Methicillin-Resistant Staphylococcus aureus ; Retroperitoneal Space ; Risk Factors

OBJECTIVE: To analyze the relationship between adrenal hemorrhage and the cause of death, age and gender. METHODS: Eighty-two cases of adrenal hemorrhage were statistically analyzed. RESULTS: Adrenal hemorrhage occurred mostly in cases of sudden death, infection, trauma and asphyxia. Male had more chance than female to have adrenal hemorrhage. Adrenal hemorrhage caused by sudden death, trauma and poisoning was more frequently seen in young adults, whereas adrenal hemorrhage in children as well as in fetus and newborns was often caused by infection as well as sudden death and asphyxia respectively. Adrenal hemorrhage caused by sudden death and asphyxia was mainly located in medulla, while the infection usually induced hemorrhage in cortex. Adrenal hemorrhage caused by trauma showed an equal opportunity in either the cortex or medulla. CONCLUSION Our data indicate that adrenal hemorrhage might provide some clues in searching for the cause of death.
Adolescent ; Adrenal Gland Diseases/pathology* ; Adult ; Autopsy ; Cause of Death ; Child ; Child, Preschool ; Death, Sudden ; Forensic Pathology ; Hemorrhage/pathology* ; Humans ; Infant ; Infant, Newborn ; Middle Aged ; Young Adult

Adrenal Gland Diseases ; diagnosis ; pathology ; therapy ; Female ; Hemorrhage ; diagnosis ; pathology ; therapy ; Humans ; Infant, Newborn ; Magnetic Resonance Imaging ; Male ; Prognosis ; Tomography, X-Ray Computed

We report a case of a patient who presented with hemophagocytic syndrome (HPS) and adrenal crisis associated with bilateral adrenal gland tuberculosis, and resulted in a poor outcome. A 50-year-old man was transferred to our hospital from a local clinic due to fever, weight loss, and bilateral adrenal masses. Laboratory findings showed leukopenia, mild anemia, and elevated lactate dehydrogenase. Computed tomography (CT) of the abdomen revealed bilateral adrenal masses and hepatosplenomegaly. CT-guided adrenal gland biopsy showed numerous epithelioid cells and infiltration with caseous necrosis consistent with tuberculosis. Bone marrow aspiration and biopsy showed significant hemophagocytosis without evidence of malignancy, hence HPS associated with bilateral adrenal tuberculosis was diagnosed. During anti-tuberculosis treatment the patient showed recurrent hypoglycemia and hypotension. Rapid ACTH stimulation test revealed adrenal insufficiency, and we added corticosteroid treatment. But pancytopenia, especially thrombocytopenia, persisted and repeated bone marrow aspiration showed continued hemophagocytosis. On treatment day 41 multiple organ failure occurred in the patient during anti-tuberculous treatment and steroid replacement.
Adrenal Gland Diseases/*complications/drug therapy/radiography ; Antitubercular Agents/therapeutic use ; Histiocytosis, Non-Langerhans-Cell/*etiology/pathology ; Human ; Isoniazid/therapeutic use ; Male ; Middle Aged ; Tomography, X-Ray Computed ; Tuberculosis, Endocrine/*complications/drug therapy/radiography

The features of the symptoms, laboratory tests and pathological characteristics of adrenal cortical and medullary hyperplasia were studied. In 6 cases of hypercatecholaminenia, plasma norepinephrine (NE), epinephrine (E), catecholamine (CA) and 24-h urinary vanillylmandelic acid (VMA), 17-hydroxycorticosteroid (OHCS) and 17-ketosteroid (KS) were determined. Adrenal glands were examined by CT scan and 131I-MIBG imaging. Pathological examination was performed after operation. The results showed that in 6 cases of hypercatecholaminenia (3 men and 3 women) aged from 34-50 years, the clinical features were just like "pheochromocytoma", for example, episodic headache, perspiration, palpitation, pallor, apprehension, nausea, tremor, anxiety and so on. Plasma levels of CA, NE and E were elevated in all 6 cases. 24-h urinary samples obtained at the onset revealed elevated VMA in 1 case. 24-h urinary cortisol was obviously elevated in all 6 cases. 24-h urinary 17-OHCS, 17-KS was normal. B-type ultrasound, CT, MRI and 131I-MIBG revealed 9 lateral adrenal gland diffuse or nodular enlargement in 6 cases. Pathologic examination showed adrenal cortical and medullary hyperplasia. Clinically, adrenal cortical and medullary hyperplasia resembled "pheochromocytoma". The most significant feature of this disease was both elevated plasma CA and 24-h urinary cortisol obviously. Pathologic examination showed adrenal cortex nodular hyperplasia and medullar diffuse or limit hyperplasia. Whether it is an independent disease or symptoms of the other disease has not final conclusion up till now.
Adrenal Cortex ; pathology ; Adrenal Gland Diseases ; pathology ; Adrenal Medulla ; pathology ; Adrenocortical Hyperfunction ; pathology ; Adult ; Catecholamines ; blood ; Female ; Humans ; Hyperplasia ; Hypertension ; etiology ; Male ; Middle Aged ; Retrospective Studies

The features of the symptoms, laboratory tests and pathological characteristics of adrenal cortical and medullary hyperplasia were studied. In 6 cases of hypercatecholaminenia, plasma norepinephrine (NE), epinephrine (E), catecholamine (CA) and 24-h urinary vanillylmandelic acid (VMA), 17-hydroxycorticosteroid (OHCS) and 17-ketosteroid (KS) were determined. Adrenal glands were examined by CT scan and 131I-MIBG imaging. Pathological examination was performed after operation. The results showed that in 6 cases of hypercatecholaminenia (3 men and 3 women) aged from 34-50 years, the clinical features were just like "pheochromocytoma", for example, episodic headache, perspiration, palpitation, pallor, apprehension, nausea, tremor, anxiety and so on. Plasma levels of CA, NE and E were elevated in all 6 cases. 24-h urinary samples obtained at the onset revealed elevated VMA in 1 case. 24-h urinary cortisol was obviously elevated in all 6 cases. 24-h urinary 17-OHCS, 17-KS was normal. B-type ultrasound, CT, MRI and 131I-MIBG revealed 9 lateral adrenal gland diffuse or nodular enlargement in 6 cases. Pathologic examination showed adrenal cortical and medullary hyperplasia. Clinically, adrenal cortical and medullary hyperplasia resembled "pheochromocytoma". The most significant feature of this disease was both elevated plasma CA and 24-h urinary cortisol obviously. Pathologic examination showed adrenal cortex nodular hyperplasia and medullar diffuse or limit hyperplasia. Whether it is an independent disease or symptoms of the other disease has not final conclusion up till now.
Adrenal Cortex/*pathology ; Adrenal Gland Diseases/*pathology ; Adrenal Medulla/*pathology ; Adrenocortical Hyperfunction/*pathology ; Catecholamines/blood ; Hyperplasia ; Hypertension/etiology ; Retrospective Studies