Tumors of the adrenal cortex

Three cases of adrenal cortical disease have been presented. These three demonstrate every variety of symptom complex which has sometimes been called the adreno-syndrome with the last case falling into the category of Cushing's syndrome.     

Tumors of the adrenal cortex and Cushing''s syndrome.

Fifty-nine patients with Cushing's syndrome, due to adrenocortical tumor, were studied and treated during the period 1953 through 1983 at Vanderbilt University Medical Center. Cushing's syndrome is caused by hypercortisolism that can be due to (1) medicinal use of steroids, (2) excess pituitary secretion of adrenocorticotropin (ACTH) (Cushing's disease), (3) adrenocortical tumor, benign or malignant, and (4) the ectopic ACTH syndrome. Clinical and endocrinologic features of Cushing's syndrome are described, and differential diagnosis of adrenocortical tumor by precise endocrinologic studies is detailed. Computerized axial tomographic (CAT) scan is currently the most accurate imaging modality for preoperative localization of tumors. Preoperative differential diagnosis between adrenocortical adenoma and carcinoma has become fairly accurate. Operative approaches in each category are described. Follow-up from 1 to 30 years has been completed for all patients, except for one who was lost after 7 years. Results of surgical treatment of adrenocortical adenomas are excellent, but the salvage from adrenocortical carcinomas is poor.     

Non-functioning carcinoma of the adrenal cortex

A case of non-functioning carcinoma of the adrenal cortex is reported. This type of neoplasm is rarely seen; up to 1974 only 173 similar cases had been reported. The patient has survived for nine months following diagnosis and operation.     

Tumors of the adrenal gland in childhood. Report of 38 patients

During the past 17 years at the Department für Pediatric Surgery, University of Heidelberg, 38 children aged 1.6 to 14 years were adrenalectomized (unilateral 3.3, bilateral 5). Individual diagnoses were: neuroblastoma 23; pheochromocytoma 5; adrenocortical carcinoma 8; adrenocortical adenoma 4; bilateral nodular hyperplasia 2 cases. Patients with histologically benign lesions are alive and without recurrence more than 5 years after surgery, except one patient who developed Nelson's tumor after bilateral adrenalectomy for Cushing's disease. Of the patients with malignant adrenal tumors 21 died within 18 months after therapy was started, a 7 years old girl with an adrenal carcinoma died after a period of 3.6 years of combined treatment. In most cases of adrenocortical tumors virilization was the prominent feature.     

Primary nonfunctioning carcinoma of adrenal cortex.

Two cases of nonfunctioning adrenocortical carcinomas are presented. The clinical features, operative findings, and histopathologic appearances are discussed. It is believed that these tumors are difficult to diagnose because of their vague presentation. Intravenous urography with nephrotomography is the most valuable method of detecting these tumors, and surgery remains the best treatment.     

Metastatic carcinoma of the adrenal gland from large cell neuroendocrine carcinoma; report of a case

A 69-year-old man had undertaken left upper lobectomy (ND 2 a) with partial resection of the left lower lobe under the diagnosis of a primary lung cancer, T2N0M0, stage IB in June, 2002. The histopathological diagnosis was large cell neuroendocrine carcinoma (LCNEC), T3N0M0, stage IIB. The patient was discharged on postoperative day 25. Abdominal computed tomography (CT) revealed an enhanced tumor in the left adrenal lesion, 3 cm in diameter, in October, 2003. Magnetic resonance imaging (MRI) showed a slightly low intensity mass on T1-weighted imaging and slightly high intensity on T2-weighted imaging without invasion to other organs. Tumor marker, NSE was slightly elevated on blood examination. Left adrenalectomy was performed with a surgical margin. Histopathologically, the tumor was diagnosed as metastasis of LCNEC. There is no evidence of recurrence for a year after surgery. Although a prognosis of LCNEC is poor in general, we should consider the resection of metastatic carcinoma of the adrenal gland from LCNEC for long-term survival expectantly.     

儿童肾上腺皮质癌的诊断与治疗

目的：探讨儿童肾上腺皮质癌的临床特征及其诊断、治疗和预后。方法：对2例儿童肾上腺皮质癌患者均行手术治疗,并对其发病及诊治过程进行分析。结果：2例肾上腺皮质癌中,1例为柯兴综合表现,1例为无功能性、小腿软组织肿块表现。结论：儿童肾上腺皮质癌生长迅速,可直接播散。无功能性肾上腺皮质癌症状隐蔽,早期诊断、及时治疗对改善预后起决定性作用。     

Primary carcinoma of the adrenal cortex: An institutional surgical perspective

Background During the decades 1960 to 1980, we surgically treated 62 patients with primary adrenocortical carcinoma (ACC). In that study, operative mortality was 9.7% and the 5-year Kaplan-Meier survival 19%. In the decade 1981 to 1990, we have treated 31 patients with ACC. Methods A retrospective review of these more recent patients was undertaken in an attempt to answer 3 questions: Has: 1) the operative mortality been reduced? 2) the distribution of stages changed at operation? and 3) adjuvant therapy with mitotane (OPDD) been beneficial? 14 men and 17 women with a mean age of 51 years were retrospectively studied. 16 patients (52%) were placed on postoperative mitotane in a nonrandomized fashion with no difference in the distribution of stages between those patients receiving and those not receiving mitotane. Results Operative mortality was 9.7%. More patients in the current study had stage II disease (52 versus 32%) and fewer patients stage IV disease (35 versus 53%) at operation. 5-year Kaplan-Meier survival was 39%. 18 patients died more than 30 days following operation secondary to metastatic ACC, and 5 of the 10 patients currently living have metastatic disease. There was no statistically significant difference in the overall survival of the group treated versus the group not treated with mitotane (p=0.55), nor in the survival of treated versus non-treated stage II patients (p=0.16). Conclusions Although survival in the more recent study has improved, ACC remains a lethal disease. Operative mortality remains high with a large number of patients being operated upon with stage IV disease. The earlier promise of benefits from adjuvant therapy with mitotane are not substantiated.     

Tumors of the parotid gland

The clinicopathologic behavior of 153 primary tumors of the parotid gland is reviewed. Of these 153 tumors, 111 were malignant and forty-two were benign. None of the benign tumors was fatal, and only two of the forty-two patients had a single recurrence. Of the 111 patients with malignant tumors, fifty-eight (53 per cent) are currently living and free of disease at five years or longer. Thirty-two of the 111 patients had one or more local recurrences (29 per cent) and only four of these are living and free of disease. Thirty-six patients (31 per cent) of those with malignant tumor died of their disease; the majority of these had uncontrolled local disease. Seventeen patients died of unrelated causes; of these, six lived more than five years without a recurrence.The treatment of choice for all primary tumors of the parotid gland is parotidectomy, generally total, in continuity with the first echelon of lymph nodes. Unless invaded by tumor, the facial nerve is preserved. A radical neck dissection is recommended only in the presence of clinically or histologically proved metastasis to the regional or cervical lymph nodes.     

Tumors of the submaxillary gland

Study of 128 cases of neoplasms of the submaxillary gland in which operation was performed at the Mayo Clinic from 1936 through 1955 with careful attention to classification of the lesions and comparison of findings in the literature on other salivary tumors yielded the following observations and conclusions.

• 1.1. Benign mixed tumor is the most common tumor found in the submaxillary gland. Its predominance, however, is less than in the parotid gland.
• 2.2. Cylindroma is the most common malignant tumor in the submaxillary gland and is relatively more common in the submaxillary gland than in the parotid gland.
• 3.3. Benign tumors of the submaxillary gland are found in younger patients than are malignant tumors.
• 4.4. Benign tumors tend to be smaller, more stationary in size, give less pain and show less tendency to local invasion than do malignant tumors.
• 5.5. Both benign and malignant tumors, that later recur, begin at an earlier age than tumors that do not recur.
• 6.6. Removal of the entire submaxillary gland with the tumor is the treatment of choice for all benign tumors and well encapsulated malignant tumors. More radical surgical procedures are preferable for locally invasive malignant tumors.
• 7.7. Recurrence is rare after surgical treatment of benign tumors of the submaxillary gland.
• 8.8. Following adequate surgical treatment, approximately half of the patients with malignant tumors of the submaxillary gland will live five years without evidence of recurrence.
• 9.9. Recurrence may develop in patients with cylindroma more than five years postoperatively, but a ten year follow-up without recurrence should be synonomous with cure.

     

Ganglioneuroma of the adrenal gland associated with a contralateral renal carcinoma]

A case of ganglioneuroma of the adrenal gland associated with contralateral renal cell carcinoma is presented. Both the lesions underwent surgical therapy: of left adrenalectomy and right nephrectomy. The benign nature of the ganglioneuroma and the low staging (T2) of the renal neoplasm explain the positive exitus of the treatment: the patient is alive and free from disease after five years from the operation.     

Giant nonfunctioning carcinoma of the adrenal cortex mimicking renal cell carcinoma: a diagnostic dilemma

Adrenocortical carcinoma is a rare, highly malignant neoplasm that originates in the adrenal cortex and is difficult to differentiate from renal cell carcinoma, especially if it is gigantic and nonfunctional. We report the case of a 40-year-old man with an incidental mass in the right upper abdomen. Magnetic resonance imaging revealed that the mass originated from the right kidney and was highly suggestive of renal cell carcinoma. However, histologic examination after radical nephrectomy confirmed the mass to be an adrenocortical carcinoma compressing the kidney. We discuss the obscurity and implications of such a diagnosis.     

Myelolipoma of the adrenal gland

Adrenal myelolipomas are rare, nonfunctioning, benign neoplasms of the adrenal gland. The authors describe their experience of a case and they report the review of the literature. They illustrate what's etiopathogenetic theories, modern diagnostic technology "of imaging" and different surgical approaches need to be adapted to the excision of the adrenal myelolipomas.