A case of non-functional adrenocortical adenoma showing enlargement in size during follow-up period

A case of non-functional adrenocortical adenoma is reported. The patient was a 62-year-old woman with the chief complaint of right hypochondoralgia. The small right adrenal tumor was found incidentally by ultrasonography on examination for cholecystolithiasis. Since there was no evidence of malignancy from biochemical data, observation of the tumor was performed for 3 months. However, the lesion was gradually increased in size. Therefore, right adrenalectomy was performed. Histological findings revealed benign adrenocortical adenoma. In the Japanese literature this case seems to be the 36th case of non-functional adrenocortical adenoma.     

A case of deoxycorticosterone-producing benign adrenocortical tumor

A case of deoxycorticosterone-producing benign adrenocortical tumor is presented. A review of the English literature revealed that this is the second case of deoxycorticosterone-producing adrenocortical adenoma.     

巨大肾上腺皮质嗜酸细胞腺瘤(附1例报告)

目的 探讨肾上腺皮质嗜酸细胞腺瘤的诊断与治疗方法.方法 报道经治的一例巨大肾上腺皮质嗜酸细胞腺瘤的临床资料,并结合文献复习进行讨论.患者术前血压、血皮质醇等检查均正常,行彻底肿瘤切除.结果 完整切除巨大肿瘤,术后病理证实为右侧肾上腺皮质嗜酸细胞腺瘤,患者术后恢复良好,无并发症发生.结论 肾上腺皮质嗜酸细胞腺瘤属罕见肿瘤,多为良性,缺乏典型的临床表现及实验室检查,手术切除是首先的治疗手段.     

Hemorrhagic adrenocortical adenoma with myelolipoma: a case report

We present a case of hemorrhagic adrenocortical adenoma with myelolipoma. A 66-year-old woman was admitted to our hospital for left retroperitoneal mass. Based on abdominal computed tomography, magnetic resonance imaging and blood tests, preoperative diagnosis was a sarcoma of renal capsule origin. En bloc resection of adrenal gland, tumor, and the kidney with lymph node dissection was performed. Histologically, the mass was diagnosed as hemorrhagic adrenocortical adenoma with myelolipomatous foci.     

肾上腺功能性色素性皮质腺瘤1例报告并文献复习

目的：探讨肾上腺功能性色素性皮质腺瘤的临床及病理学特征。方法：回顾性分析1例肾上腺功能性色素性皮质腺瘤患者的诊治情况,并复习有关文献探讨其临床特征。结果：肾上腺功能性色素性皮质腺瘤临床上十分罕见,无特异性表现,影像学检查提示有肾上腺腺瘤病变,病理检查可见肿瘤细胞胞质内有特异性脂褐质存在。结论：肾上腺功能性色素性皮质腺瘤的诊断主要依靠病理检查,治疗以手术为主。     

Nonfunctioning adrenocortical adenoma: a report of two cases

Two cases of nonfunctioning adrenocortical adenoma are herein reported. The adrenal tumor was found incidentally by computed tomography in both patients. Preoperative evaluation revealed that both patients had neither abnormal values in a variety of adrenal function studies nor any recognizable clinical signs associated with adrenal tumor. Following a definitive diagnosis of nonfunctioning adrenal tumors, surgical extirpation was performed in both cases. Histopathological finding was adrenocortical adenoma. Nonfunctioning adrenocortical tumor will be found more frequently with the increasing use of computed tomography in ordinary clinical practice. The management of nonfunctioning adrenocortical tumor is briefly discussed.     

Cushing's syndrome with cortisol hypersecretion from one of bilateral adrenocortical adenomas: Report of a case

We report herein the case of a 40-year-old man with Cushing's syndrome, diagnosed by clinical manifestations and endocrinological studies, who was found to have bilateral adrenocortical adenomas, one of which hypersecreted cortisol. The Cushing's syndrome was therefore attributed to primary adrenocortical disease, and the right adrenal tumor was resected and histologically diagnosed as a so-called black adenoma. After resection of the right tumor, the left adrenal tumor showed no signs of cortisol hypersecretion for the 23 months of follow-up until the patient died of peritonitis subsequent to the rupture of a duodenal ulcer. The left adrenal tumor was examined at autopsy and found to be a cortical adenoma. These data imply that the adrenal adenomas developed primarily from the adrenal gland itself, and that one of the tumors was well differentiated and secreted excess hormones, while the other remained in cell proliferation without hypersecretion.     

Adrenocortical tumor in a patient with untreated congenital adrenocortical hyperplasia owing to 21-hydroxylase deficiency: characterization of steroidogenic lesions

We report a case of congenital adrenal hyperplasia complicated by a right adrenal adenoma. The congenital adrenal hyperplasia was confirmed by extremely high levels of basal serum 17 alpha-hydroxy-progesterone and urinary pregnantriol as well as an exaggerated response of 17 alpha-hydroxyprogesterone to adrenocorticotropic hormone. On roentogenographic examinations and an echogram a huge calcified tumor was seen in the right adrenal gland. Histological findings indicated an adrenocortical adenoma. Culture of the tumor cells showed an accentuated response in the ratio of 17 alpha-hydroxy-progesterone to desoxycortisol with adrenocorticotropic hormone as a stimulator, suggesting that such adenoma cells also lack 21-hydroxylase activity. Our study clearly demonstrates that tumor cells have the same steroidogenic lesions as hyperplasia.     

Combined tumor consisting of non-functioning adrenocortical adenoma and pheochromocytoma in the same gland.

A 59-year-old woman was admitted to East Tokyo Metropolitan Hospital for further examination of a left adrenal mass. Catecholamine levels in the plasma and urine were within normal limits. Neither (131)I-metaiodobenzylguanidine (MIBG) nor norcholestenol iodomethyl ((131)I) had accumulated in the left adrenal gland. A left adrenalectomy was performed through a retroperitoneal endoscope. Sections showed a tumor consisting of two parts. Histologically one part of the tumor was completely encapsulated, with clear cells comprising a honeycomb-like mass, regarded as adrenocortical adenoma. The other part consisted of rich cytoplasma and these basophilic, hyperchromatic cells included alveolar patterns, resulting in the diagnosis of pheochromocytoma. Thus, an extremely rare case of non-functioning adrenal incidentaloma consisting of an adrenocortical adenoma and a concomitant pheochromocytoma in the same gland is reported here.     

A case of non-Cushingoid Cushing's syndrome

A 42-year-old female was admitted to our hospital with a chief complaint of hypertension. Endocrinologically, the plasma cortisol level was increased, but its diurnal rhythm had disappeared and the plasma cortisol level was not suppressed by dexamethasone loading at 2 mg or 8 mg. The plasma ACTH level was low. Computerized tomographic scan, echography and adrenocortical scintigraphy showed an adrenocortical tumor on the right adrenal gland. Physical examination did not reveal typical Cushingoid symptoms such as moon face, central obesity and violaceous striae. Thus we diagnosed this case as non-Cushingoid Cushing's syndrome and performed right adrenalectomy. Histological examination showed adreno-cortical adenoma without malignancy.     

Concomitant renal cell carcinoma with pituitary adenoma

Renal cell carcinoma has a complex and variable natural history. We report a case underlining this who presented concomitant renal cell carcinoma metastasis with pituitary and adrenocortical adenomas. A 62-year-old woman presented with visual loss. Imaging revealed a large sellar mass with suprasellar extension. Four years before, nephrectomy and adrenalectomy had been performed for a renal cell carcinoma with metastasis in a coexistent adrenocortical adenoma. Faced with progressive visual loss and the questionable pituitary pathology, the patient underwent trans-sphenoidal surgery. Due to profuse tumor bleeding, only a biopsy was possible. In a second operation, the patient underwent craniotomy with subtotal resection of the tumor. Histological examination of the specimen revealed a metastasis of the renal cell carcinoma and a pituitary adenoma. The case presented here and a review of the reports suggest that there are some differences between the clinical features and outcomes of metastases of renal cell carcinoma and those of pituitary gland metastases from other primary sites.     

Primitive neuroectodermal tumor of the adrenal gland

We report a rare case of primitive neuroectodermal tumor arising from adrenal gland in adulthood, diagnosed preoperatively as having non-functional adrenocortical adenoma. Laparoscopic adrenalectomy was performed. Immunohistological examination revealed the definite diagnosis as primitive neuroectodermal tumor of the adrenal gland. Although primitive neuroectodermal tumor is a highly malignant neoplasm, there is no evidence of local recurrence and distant metastasis 16 months after surgery.     

Adrenal adenoma with bilateral testicular Leydig cell tumor: a case report

A case of left adrenal adenoma with bilateral testicular Leydig cell tumor in a 38-year-old man is reported. He had received bilateral orchiectomy for testicular Leydig cell tumor at the age of 37. After operation computed tomography revealed left adrenal mass and aldosterone-secreting adrenal tumor was suspected. Left adrenalectomy was performed and histopathological diagnosis was adrenocortical adenoma. Serum ACTH, aldosterone and plasma renin activity were still high after operation. The adrenal cortex and gonads are of common embryologic origin and the histologic pattern may overlap. Examination of the adrenal gland is necessary for the patient with a testicular Leydig cell tumor.     

A case of adrenal pheochromocytoma with contralateral adrenocortical adenoma

A case of a pheochromocytoma in the right adrenal gland and adrenocortical adenoma in the left adrenal gland of a 58-year-old male is reported. The patient was incidentally found to have a right adrenal tumor by ultrasonographic study. A computerized tomographic (CT) study and magnetic resonance image (MRI) study revealed bilateral adrenal tumors. The sizes of the right tumor and left tumor were 2.5 x 3.5 cm and 1.2 x 1.0 cm, respectively. The intensity of each tumor was different on T2-weighted MRI. 131I-MIBG scintigram showed the uptake of right adrenal gland. The existence of pheochromocytoma was confirmed by the elevated levels of catecholamines. We performed venous sampling to be certain whether the patient had unilateral or bilateral pheochromocytoma. As a result, bilateral adrenal pheochromocytoma was diagnosed. Therefore, we performed bilateral adrenalectomy. However, histopathological examination revealed right pheochromocytoma and left non-functioning adrenocortical adenoma.     

Salvage computed tomography‐guided transhepatic radiofrequency ablation for unresected aldosteronoma of adrenohepatic fusion after adrenalectomy

Adrenalectomy is a treatment of choice for functioning adrenocortical adenoma. We experienced a case of unresected aldosteronoma after right adrenalectomy. Computed tomography‐guided transhepatic radiofrequency ablation was carried out to treat the unresected functioning adenoma. The purpose of our case report was to show the clinical findings, computed tomography imaging features and computed tomography‐guided transhepatic ablation techniques for adrenocortical adenoma arising from adrenohepatic fusion.     

Feminizing adrenocortical tumor]

Feminizing adrenocortical tumor is rare. Three patients with feminizing adrenocortical tumor (2 males and 1 female) were treated surgically. The age of the patients was 11, 21 and 32 years old respectively. The removed tumors weighing 70 g, 250g and 31g respectively and they all were adrenocortical adenoma. The chief symptoms of the patients were gynecomastia, orchiatrophy and sexual inadequacy. The results of follow-up for 1-11 years showed no recurrence in all 3 patients. We consider that the tumor should be resected as soon as the diagnosis was established and long-term follow-up should be made after operation.     

Clinical experience of adrenal incidentaloma with particular reference to adrenal cortical function

The adrenal function mainly cortical one, was evaluated in 16 patients with incidentally discovered adrenal masses. Pathological examination was possible in 15 cases. The finding consisted of adrenocortical adenoma in 9, adrenocortical nodular hyperplasia in 1, adrenal medullary hyperplasia in 1, metastatic tumor in 2 and adrenal cyst in 2. Another case of adrenal cyst was diagnosed by percutaneous puncture. In all cases peripheral levels of plasma cortisol, plasma aldosterone concentration and plasma renin activity were normal. Plasma catecholamine levels were also normal except in a case of adrenal medullary hyperplasia. On the other hand, the cases of adrenocortical adenoma displayed elevation of urinary 17-hydroxycorticosteroids in 6/9 (67%), a loss of plasma cortisol circadian rhythm in 3/7 (43%) and insufficient suppression on dexamethasone (DXM) suppression test in 6/9 (67%). Their adrenal scintigraphy (with 131I-6 beta-iodomethyl-9-nor-cholest-5 (10)-en-3 beta-ol) revealed an increased ipsilateral up-take and insufficient suppression after DXM in all, while a diminished contralateral up-take in 4/9 (44%). These data suggested that a considerable number of adrenal incidentalomas may not be truly "non-functioning". Two patients with cortical adenoma experienced post operative adrenal insufficiency (25%). It was suggested that a pre-operative loss of plasma cortisol circadian rhythm was the most prognosticating of the post operative adrenal insufficiency, rather than insufficient DXM suppression or scintigraphic absence of contralateral up-take. Among the patients with malignancy, differentiation of incidental adrenal adenoma from metastasis by size alone may not be reliable.     

Nonfunctioning adrenal cortical adenoma: a case report

A case of nonfunctioning adrenal cortical adenoma is presented. A 56-year-old woman was admitted with the chief complaint of asymptomatic microscopic hematuria. Adrenal tumor was found accidentally by computed tomography scan during evaluation for hematuria. A venogram and adrenal scan confirmed left adrenal neoplasm. Endocrine studies were normal. Surgical extirpation was performed and pathological examination revealed 2.2 X 2.0 X 2.0 cm left adrenal cortical adenoma. Reports of nonfunctioning adrenal cortical adenoma in the Japanese literature were reviewed and a discussion was made on this rare condition.     

A Child with Adrenocortical Adenoma Accompanied by Congenital Hemihypertrophy: Report of a Case

We report herein the findings of a 7-year-old male child with a ruptured adrenocortical adenoma and congenital hemihypertrophy which was incidentally detected after suffering a trauma. A review of 21 pediatric cases of adrenocortical neoplasms in the literature was made. The patient showed precocious puberty such as pubis and advanced bone age, but an endocrinological examination revealed no definite abnormalities. The right adrenal tumor with hematoma was resected after these evaluations. Adrenocortical adenoma is considered to occur more frequently in female children. However, the incidence of adrenocortical tumors accompanied by congenital hemihypertrophy does not differ between males and females. The outcomes were relatively good, although the observation periods were short in some patients. A large number of patients presented with a tumor and hemihypertrophy on the same side. This finding is of interest when considering the possible association between hemihypertrophy of the organs and tumor proliferation. However, their association in terms of development was unclear. It is necessary for patients with hemihypertrophy to have regular examinations for the possible development of malignant tumors, especially in the kidney, adrenal gland, and liver. Received: November 1, 1999 / Accepted: March 24, 2000     

Coexistence of three distinct adrenal tumors in the same adrenal gland in a patient with primary aldosteronism and preclinical Cushing's syndrome

A 62-year-old woman was admitted to our hospital because of hypokalemia. Physical examination revealed no signs of excessive adrenocortical steroid production, as are found in Cushing's syndrome. Her plasma renin activity (PRA) was suppressed (0.10 ng/ml per h), and her serum aldosterone level was high (30.0 ng/dl). PRA was not increased after a renin-releasing test. Her plasma adrenocorticotropic hormone (ACTH) level was low (<5 pg/ml), but her serum cortisol level was normal (21.0 μg/dl). Administration of 8 mg dexamethasone did not suppress her plasma cortisol level. Finally, she was diagnosed with clinical primary aldosteronism associated with preclinical Cushing's syndrome. Magnetic resonance image revealed three sequential nodular masses (each 15 mm × 15 mm) in the right adrenal gland. A right adrenalectomy was performed by endoscopy. The three removed tumors appeared to have different characteristics. Microscopic examination revealed that the upper and lower tumors were adrenocortical adenomas, and the middle tumor was a black adenoma. Immunohistochemical staining for the enzymes involved in cortisol biosynthesis suggested that the upper tumor secreted aldosterone, whereas either or both of the two other tumors secreted cortisol. Surprisingly, at 33 years of age, she had been diagnosed with Cushing's syndrome, due to a cortisol-producing adrenocortical adenoma, and she had received a left adrenalectomy. Clinically and pathophysiologically, this was a very rare case.