肾上腺嗜酸细胞腺瘤一例报告并文献复习

目的报道1例肾上腺嗜酸细胞腺瘤患者的临床及病理特点。方法患者,男,37岁,体检发现左肾上腺肿物15 d入院。既往否认高血压、低血钾等病史。实验室检查血钾、血儿茶酚胺、血皮质醇、立卧位肾素血管紧张素、醛固酮、尿3-甲基-4-羟基苦杏仁酸、尿17-羟皮质类固醇、17-酮皮质类固醇均正常。B超及CT检查示左肾上腺区肿物,直径约7 cm。术前诊断为左肾上腺区肿物,行后腹腔镜下左肾上腺肿物切除术。结果术中发现肿物体积较大,包膜完整光滑,与周围组织无粘连,肿物表面血管较为丰富,术中碰触肿物,血压无波动,完整切除肿物。术后病理检查示肿瘤细胞嗜酸性,内部充满颗粒,诊断为肾上腺嗜酸细胞腺瘤。随访2个月无复发。结论肾上腺嗜酸细胞腺瘤是一种具有潜在恶性的良性肿瘤,可手术切除,但需密切随访。     

Surgery for adrenal tumours: is operation for the small incidental tumour appropriate?

The last 15 years have seen a change in the indications for adrenalectomy. On the one hand there has been a decline in the number of operations for metastatic breast cancer due principally to the introduction of effective medical therapy. Moreover, the introduction of trans-sphenoidal techniques has reduced the number of adrenalectomies for Cushing's Disease. In contrast the introduction of CT scanning has seen an increase in the diagnosis of incidental adrenal masses, most of which turn out to be benign non-functioning adrenal cortical adenomas. During the period 1970-86, 124 adrenalectomies were performed at Royal North Shore Hospital. From 1970 to 1976 the primary indication for adrenalectomy was metastatic breast carcinoma (84%). Over the past three years one of the primary indications has been the discovery of an asymptomatic, non-functional adrenal mass on CT scan (26%) and, of these tumours, 95% have been less than 5 cm in size. Yet no incidentally discovered adrenal mass was an adrenocortical carcinoma and no adrenocortical carcinoma was smaller than 7 cm in diameter. We recommend that an asymptomatic adrenal mass less than 6 cm in size, with no evidence of function, should be managed conservatively.     

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.     

Thoracoscopic transdiaphragmatic adrenalectomy for isolated locally recurrent adrenal carcinoma

A 58-year-old man who had undergone left adrenalectomy 2 years previously for adrenocortical carcinoma was diagnosed to have a left suprarenal solid mass. Thoracoscopic transdiaphragmatic excision of the tumor was conducted under the diagnosis of isolated local recurrence of adrenal carcinoma. There were no intraoperative or postoperative complications. The patient subsequently received three courses of adjuvant chemotherapy. There have been no signs of tumor recurrence during 3 years follow up after surgery. This approach provides a minimally invasive alternative to an open thoracoabdominal procedure after prior open surgery.     

False positive 18F-FDG PET scan in adrenal oncocytoma

18F-FDG whole-body positron emulsion tomography (18F-FDG PET) has become an established imaging modality for a variety of cancers. Today, 18F-FDG PET is utilized to differentiate benign from malignant non-functioning adrenal masses. In this report, a 25-year-old woman presented with a 2-month history of left flank pain. Abdominal CT revealed a left 6.5 x 4.5 cm adrenal mass with regular margins and focal calcification. To make a differential diagnosis, 18F-FDG PET was performed. Preoperative laboratory studies showed that the mass was non-functioning. A left adrenalectomy was performed through a left subcostal incision. The final pathologic evaluation revealed adrenal oncocytoma. We present the disassociation between preoperative 18F-FDG PET and pathologic findings of a benign adrenocortical oncocytoma.     

A case of infantile virilizing adrenocortical tumor

The patient was a 16-month-old girl, born by mature natural delivery and weighing 3,320 g. Hirsutism was noted on birth. Development of pubic hair and hypertrophy of the labia minora were noted after 8 months. At the time of admission, the height was 80 cm and body weight 14.5 kg. Systemic obesity, facial acne, systemic hirsutism, low pitched voice and hypertrophied clitoris were observed. Androstenedione, dehydroepiandrosterone-sulfate and cortisol showed high levels in the blood, and the urinary 17-KS was obviously high, along with an increase in urinary 17-OHCS. The subject did not respond to either the dexamethasone inhibition test or ACTH load test. The abdominal CT revealed a tumor in the front upper position of the left kidney, and adrenal scintigraphy disclosed an obvious accumulation image in the adrenal gland on the left side. Based on the diagnosis of a left adrenal tumor, left adrenalectomy was performed. The tumor measured 5.0 x 4.5 x 3.7 cm, and weighed 57 g. Histopathologically it was diagnosed as adrenocortical adenoma. The infantile virilizing adrenocortical tumor is reported together with some discussion of the literature.     

Giant cystic pheochromocytoma: a case report

The patient, a 59-year-old woman, was referred to our University Hospital for evaluation of a left mass. Ultrasonography revealed a left adrenal cystic mass. On excretory urograms, the left kidney was pressed downward by a suprarenal mass, and computerized tomography (CT) and magnetic resonance imaging (MRI) confirmed an adrenal cyst. 131I-meta-iodo-benzylguanidine (MIBG) scintigraphy showed prominent accumulation in the left adrenal mass and the capsule. Considering the elevation of catecholamines in both blood and urine samples, we performed a left adrenalectomy with a presumptive diagnosis of pheochromocytoma (tumor size: 11.6 x 7.5 x 6.5 cm, tumor weight 720 g). The subsequent pathological examination confirmed a left giant cystic pheochromocytoma. 131I-MIBG scintigraphy was the most useful tool in the diagnosis of the cystic pheochromocytoma.     

原发性色素性结节状肾上腺皮质病一例报告并文献复习

目的 探讨原发性色素性结节状肾上腺皮质病(PPNAD)的临床表现及诊治方法.方法 PPNAD患者1例,男性,52岁.因血压升高1年余入院.库欣征面容.血、尿皮质醇升高,大、小剂量地塞米松抑制试验均不被抑制.B超检查示双侧肾上腺区未见肿块回声.CT检查左侧肾上腺可见1.6 cm×2.0 cm肿块,右侧正常.检索国内外医学数据库相关文献并复习. 结果 患者行腹腔镜下左肾上腺切除术.术后病理标本切面中央见灰黑色细带,镜下示肾上腺皮质各带均增生、网状带细胞胞质内可见脂褐素颗粒.结合临床诊断为PPNAD.术后患者血压降至正常.目前仍在随访. 结论 PPNAD临床较罕见,其症状体征可不典型,影像学检查多无特异性,临床上极易误诊.由于该病部分可合并多发性内分泌肿瘤综合征,故需对其加强认识.     

A case of pre-Cushing's syndrome]

A 66-year-old female was admitted to Chiba University Hospital for the evaluation of a left adrenal mass which was incidentally discovered by computerized tomography. The patient had no clinical signs of Cushing's syndrome. Although the plasma ACTH level was suppressed, serum cortisol and urinary 17-OHCS levels were normal. Serum cortisol was not suppressed by dexamethasone and loss of diurnal rhythm of cortisol was observed. Uptake of 131I-aldosterone in the left adrenal gland was noted, but no accumulation was observed in the right one. Left adrenalectomy was performed. The tumor resected was 20 x 22 x 26 mm in size. Pathological diagnosis was adreno-cortical adenoma. Whether slight abnormality of adrenocortical function without clinical symptoms observed in the present case would develop into a clinically typical Cushing's syndrome remains to be solved.     

Adrenocortical carcinoma: report of a case]

A 24-year-old man visited our hospital complaining of hypertension and headache. Endocrinological findings revealed no abnormalities except for a slight decrease in serum adrenocorticotropic hormone (ACTH), a slight increase in urine 17-ketosteroid (17-KS), and a marked increase in serum pregnenolone. Computed tomography and magnetic resonance imaging revealed a 3 x 3 cm mass in the right adrenal area and I131-aldosterol scintigraphy demonstrated a high absorption of the isotope in the right adrenal area. Vena cavography suggested a 2 x 2 cm tumor thrombus originating in the right adrenal. Under the diagnosis of the right adrenocortical carcinoma, adrenalectomy and removal of the tumor thrombus were performed. Both serum pregnenolone and urine 17-KS returned to the normal level within a week after the operation and blood pressure was well controlled without any medication 3 months after the operation. Thus, the tumor seemed to be endocrinologically active.     

The surgical management of adrenal cortical carcinoma

Experience with the surgical treatment of 12 patients with carcinoma of the adrenal cortex is presented. They were 6 men and 6 women. Their ages ranged from 23 to 66 years old (mean 38 years). Four carcinomas had detected hormonal activity. The location of the carcinoma involved the right adrenal in 8 cases and the left in 4 cases. The mean diameter of the mass was 15 cm. Excision of the adrenal cancer with the ipsilateral kidney was the usual procedure. Lymph nodes were involved in 5 cases and interestingly the kidney was only involved in 3 cases. One patient died during hospitalization from pulmonary embolus, whereas another one required re-exploration for postoperative hemorrhage. Nine patients developed pulmonary, hepatic and bone metastasis within 3 to 10 months, postoperatively. Radiation treatment and chemotherapy had poor results. Nevertheless, one patient in whom left adrenalectomy, splenectomy and partial pancreatectomy was performed, is doing well, despite the presence of hepatic metastases, with the addition of o,p' DDD, 24 months postoperatively. Another patient underwent reoperation and excision of recurrent local disease 12 months after adrenalectomy/nephrectomy. She is now alive 16 months following her second surgery. Based on the above, an aggressive surgical approach is advocated in the management of adrenocortical carcinoma.     

肾上腺嗜酸细胞腺瘤1例报告并文献复习

目的:探讨肾上腺嗜酸细胞腺瘤的临床及病理特点。方法:回顾性分析1例肾上腺嗜酸细胞腺瘤患者的临床资料:男,54岁,体检时B超发现左肾上腺区肿物,CT检查发现6.0cm×5.3cm肾上腺区肿物。肾素、醛固酮、儿茶酚胺实验值均正常。行肾上腺肿物切除术。结果:术中发现肿物体积较大,包膜完整光滑,与周围组织无粘连,肿物表面血管较为丰富,术中触碰肿物,血压无波动,遂完整切除肿物。术后病理检查显示肿瘤细胞嗜酸性,内部充满颗粒,诊断为肾上腺嗜酸细胞腺瘤。随访6个月无复发。结论:肾上腺嗜酸细胞腺瘤是一种具有潜在恶性的良性肿瘤,可手术切除,但术后需密切随访。     

A case report of Cushing's syndrome due to adrenocortical nodular dysplasia, with remission period of 6 years after unilateral adrenalectomy

A 29 y.o. female had been associated with Cushing's syndrome, gradually, from 1977. She was advised to get further examination at our hospital on September 1980. A pre-operative diagnosis of Cushing's syndrome due to bilateral adrenocortical tumors was made and right adrenalectomy was performed on December 22, 1980, as the 1st of two stage operation. The right adrenal, 1.8 cm in diameter, had a pigmented nodule and multiple pigmented micronodules, which were diagnosed pathologically as primary adrenocortical nodular dysplasia. It was not necessary for this patient to maintain steroid therapy, but after 3 months she complained of the withdrawal syndrome and was administered dexamethasone. Since July, 1981, she had no symptom without adrenocortical steroid administration. In November 1986, she became cushingoid and on June 1, 1987, we performed left adrenalectomy on her. The appearance of the left adrenal was similar to the right. We have found no reports of the cases of adrenocortical nodular dysplasia such as delayed appearance of withdrawal syndrome after unilateral adrenalectomy. In this case, plasma ACTH had relatively poor correlation with cortisol. Her plasma ACTH was not always suppressed and sometimes within normal range. Her adrenals had not only autonomy but also pituitary dependency. It is concluded that in this case there may have been a pituitary-adrenal dual control.     

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.     

Long-Term Survival After Resection of Metachronous Bilateral Adrenal Metastases of Mucinous Gastric Carcinoma: Report of a Case

We report a case of metachronous bilateral adrenal metastases from mucinous adenocarcinoma of the stomach. A 68-year-old man who had undergone surgery for advanced gastric cancer 5 months earlier had a follow-up computed tomography (CT) scan, which showed a right adrenal tumor. We performed a right adrenalectomy, and histopathological examination revealed a mucinous adenocarcinoma with features consistent with those of gastric cancer. A routine follow-up CT scan done 41 months after the right adrenalectomy showed a left adrenal mass. Chemotherapy had no apparent effect, and left adrenalectomy was performed 65 months after the right adrenalectomy. Histopathological examination also revealed a metastasis from gastric cancer. The patient was alive without recurrence 40 months after the left adrenalectomy. This case suggests that resection of adrenal metastasis from gastric cancer is an effective treatment option that may prolong survival in selected patients.     

Nonfunctioning adrenal tumors. Dilemmas in management

A retrospective study conducted from 1975-1987 at Vanderbilt University Medical Center and affiliated hospitals identified 28 patients, ranging in age from 22 to 74 years, who were noted to have "nonfunctioning" adrenocortical tumors. Eighteen (64%) were men, and ten (36%) were women. A left adrenal mass was present in 16 (57%) patients, a right in 11 (39%) patients, and one (4%) patient had bilateral adrenal enlargements. Nineteen patients underwent an adrenalectomy. Ten were found to have adrenocortical adenomas, ranging is size from 2.5-4.0 cm in greatest diameter. Three were adrenocortical carcinomas (3.0, 5.5, and 8.0 cm). A necrotic mass (14.0 cm) was found in one patient. Two patients had myelolipoma, one had ganglioneuroma, and one had a suspected primary melanoma. Five patients were not operated upon and were followed by serial computed tomographic scans for variable periods; two died of unrelated cardiac problems, and one was lost to follow-up. Three patients underwent biopsy with benign pathology. The data indicate a high incidence of adenoma and carcinoma in patients with nonfunctioning adrenal tumors that measure more than 3.0 cm in diameter. These tumors have the potential to progress to malignant degeneration without any recognizable clinical or endocrine abnormalities. The authors thus conclude that all patients with "nonfunctioning" adrenal tumors that are larger than 3.0 cm in diameter should undergo surgical exploration and excision.     

Laparoscopic Bilateral Partial Adrenalectomy for Adrenocortical Adenomas Causing Cushing's Syndrome: Report of a Case

Laparoscopic total adrenalectomy has become a standard technique for small adrenal tumors; however, bilateral adrenalectomy results in postoperative adrenal insufficiency, necessitating lifelong steroid replacement. To preserve adrenocortical function in a 41-year-old woman with bilateral adrenocortical adenoma (BAA) causing Cushing's syndrome, we performed laparoscopic bilateral partial adrenalectomy. We based our preoperative diagnosis of bilateral adrenocortical tumors causing Cushing's syndrome on the results of endocrinological investigations and imaging findings. Thus, we performed lateral transperitoneal laparoscopic bilateral partial adrenalectomy, preserving the adrenal glands, which were normal. Pathological examination of both tumors confirmed the diagnosis of adrenocortical adenoma. The patient had no postoperative complications, and her adrenocortical function was normal without steroid replacement at her 10-month follow-up. This report shows that Cushing's syndrome resulting from bilateral adenomas can be effectively treated by laparoscopic bilateral partial adrenalectomy as a minimally invasive, adrenocortical-preserving operation.     

11-Deoxycorticosterone-producing adrenocortical carcinoma.

A woman presented with a history of weight loss and muscle weakness. A laboratory test revealed hypokalemia and elevation of plasma 11-deoxycorticosterone (DOC). CT showed a left adrenal mass. A left adrenalectomy was performed. The histological and immunohistochemical diagnosis showed a DOC-producing adrenocortical carcinoma. This cancer is very rare; only 10 cases including the present case have appeared in the literature.     

Giant non-functioning adrenocortical carcinoma effectively treated with preoperative transarterial embolization: a case report

An 80-year-old women consulted a physician because of weight loss and slight fever. Since a large retroperitoneal tumor was found, the patient was referred to our hospital. Based on abdominal computed tomography, magnetic resonance imaging and blood tests, preoperative diagnosis was non-functioning left adrenal tumor, 13 cm in diameter. Preoperative abdominal angiography revealed that the tumor was supplied blood by the left adrenal artery, left renal artery through left renal upper segment, splenic artery, pancreas tail artery, and middle colic artery. We performed transcatheter arterial embolization (TAE) of some of these tumor-supplying vessels. Three days after the TAE, adrenalectomy was performed without blood transfusion. Histopathological examination of the tumor was adrenocortical carcinoma of low grade malignancy.     

Operable lung cancer and synchronous adrenal masses: role of laparoscopic adrenalectomy combined with pulmonary resection

STUDY AIM: Assessment of laparoscopic adrenalectomy in the management of operable non-small cell lung cancer (NSCLC) associated with solitary and synchronous adrenal mass. PATIENTS AND METHODS: In a retrospective study, we reviewed 3 patients with operable NSCLC proved by pulmonary biopsy and an isolated synchronous adrenal mass shown by abdominal CT scan. We first performed a laparoscopic adrenalectomy followed by pulmonary resection. RESULTS: All patients had a laparoscopic adrenalectomy without any conversion or treatment-related death. Hospitalization stay ranged from 5 to 6 days. A left pneumonectomy has been performed immediately after this first hospitalization in 2 cases and after a first cycle of chemotherapy in the third case. Pathologic examination showed a NSCLC adrenal metastasis in 2 cases and an adrenocortical adenoma in the last case. During the follow-up 2 patients died of other distant metastasis and a mediastinal lymph node recurrence has been diagnosed in the third patient, actually treated by a second line chemotherapy. CONCLUSION: Despite those bad results that concern patients T3 M+ in 2 cases, laparoscopic adrenalectomy seems to be very interesting in selected cases. Considering that pulmonary resection can be done after, it represents a mean of diagnosis at least better than fine needle aspiration biopsy. Laparoscopic adrenalectomy might also be considered in the resection of a synchronous and isolated metastasis as a way to improve survival.