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.     

Early diagnosis of and surgical strategy for adrenal medullary disease in MEN II gene carriers

Sixteen multiple endocrine neoplasia type II (MEN II) gene carriers--12 who had undergone thyroidectomy because of medullary carcinoma of the thyroid and 4 whose thyroid glands had been removed because of C cell hyperplasia--were examined for the presence of pheochromocytomas. No patient had sought medical advice for pheochromocytoma symptoms. Fourteen patients had MEN IIa syndromes, one patient had a MEN IIb and another patient had a mixed syndrome of von Recklinghausen's neurofibromatosis and MEN II. Eight patients had undergone unilateral adrenalectomy for pheochromocytoma 11 +/- 4 years before. The patients underwent clinical examination, determination of the urinary excretion of catecholamines and metabolites, and 131I-metaiodobenzylguanidine (131I-MIBG) and CAT scans. 131I-MIBG scanning was performed with images 1, 4, and 7 days after the radionuclide injection. In seven of eight patients who had undergone unilateral adrenalectomies, the 131I-MIBG scans showed accumulation of the radionuclide in the remaining adrenal gland. Bilateral adrenal accumulation of the radionuclide was demonstrated in seven of eight MEN IIa gene carriers who had not undergone adrenalectomy. Five patients, two of whom had undergone adrenalectomy, were found to have unilateral pheochromocytomas less than 2 cm in diameter. Only one of these five patients had an elevated excretion of urinary catecholamines. Between day 4 and day 7 after 131I-MIBG injection, adrenal glands with pheochromocytomas increased their relative accumulation of the radionuclide significantly more (p less than 0.02) than did adrenal glands without any demonstrable pheochromocytomas. All the pheochromocytomas were viewed by means of CAT scans. Only one MEN IIa patient had bilateral pheochromocytomas, but our findings indicate that there is a tendency to bilateral adrenal medullary hyperfunction in most MEN II gene carriers. As 131I-MIBG and CAT scans can facilitate the early diagnosis of pheochromocytomas, unilateral adrenalectomy can safely be performed in most MEN IIa patients. Bilateral pheochromocytomas develop in a majority of patients with MEN IIb syndromes. Bilateral adrenalectomy should therefore be performed in these patients.     

Sipple's syndrome: a case report

A 41-year-old woman was hospitalized for evaluation of diabetes mellitus and hypertension. The hormonal and radiological examinations revealed that she had pheochromocytoma of bilateral adrenal gland and medullary carcinoma of thyroid gland. Therefore, she was diagnosed as having Sipple's syndrome. She had no definite familial history, but her two sisters, already dead, had been strongly suspected of having had pheochromocytoma. First, bilateral adrenalectomy was performed and secondly, total thyroidectomy, excision of parathyroid and cervical lymph node dissection were performed. Histopathological diagnosis was pheochromocytoma of bilateral adrenal gland, medullary carcinoma of thyroid gland and chief cell hyperplasia of parathyroid gland. We report a case of Sipple's syndrome, which probably is the 88th case in Japan, with the review of the previous Japanese literature.     

Diagnosis of adrenal medullary diseases in patients with sporadic or hereditary medullary thyroid carcinoma. A report of 37 cases with 8-year follow-up study

Thirty-seven patients with medullary thyroid carcinoma were investigated to determine the status of adrenal medulla by computed tomography and 131I-metaiodobenzylguanidine (131I-MIBG) scintigraphy as well as measurements of urinary catecholamine excretion. Patients were followed up for 8 years in maximum. Fifteen patients belonged to multiple endocrine neoplasia type 2 including patients with incomplete phenotype. Computed tomography demonstrated adrenal tumors or enlargement in all 6 patients with urinary epinephrine (E) more than 30 micrograms/day, 4 of them were confirmed to have pheochromocytoma or adrenal medullary hyperplasia by surgery. In 2 patients with E less than 30 micrograms/day and epinephrine to norepinephrine (E/N) ratio more than 0.3 suggesting adrenal medullary hyperfunction, computed tomography revealed small adrenal tumors. Three of the remaining 7 patients with E less than 30 micrograms/day and E/N ratio less than 0.3 had equivocal enlargement of unilateral gland on computed tomography. 131I-MIBG scintigraphy demonstrated tracer uptake in adrenal glands with tumor more than 1cm in diameter. One of 2 adrenal glands with medullary hyperplasia showed a faint adrenal image on the scintiscan, but the other showed no tracer uptake. Pheochromocytoma became manifest in 4 patients during the follow-up period, 4, 13, 14 and 34 years after thyroid surgery. None of 22 patients with sporadic medullary thyroid carcinoma showed adrenal abnormalities on the examinations mentioned above.     

Adrenal pheochromocytoma with multiple neurofibromatosis on the trunk

We report a case of adrenal pheochromocytoma in a patient with neurofibromatosis type 1 (NF1). A 65-year-old female patient was admitted to our hospital for examination of a right adrenal mass. The adrenal tumor was incidentally discovered by abdominal computed tomography during examination for hypertension in another hospital. She had large multiple neurofibromatous lesions and café-au-lait spots on the trunk. We thought that it was difficult to make a skin incision on normal skin. Serum and urinary catecholamines were markedly increased. Magnetic resonance imaging revealed a solid round tumor 3 cm in diameter, located in the right adrenal gland. Laparoscopic right adrenalectomy was performed. Serum and urinary catecholamines returned to the normal range on post-operative day 10. Laparoscopic surgery may be a good option for NF1 patients with pheochromocytoma, especially those who had multiple neurofibromatosis on the trunk.     

A case of composite pheochromocytoma-ganglioneuroblastoma in the adrenal gland with primary hyperparathyroidism

We report a case of composite pheochromocytoma-ganglioneuroblastoma in the adrenal gland with primary hyperparathyrodisim. A 55-year-old woman consulted our hospital for an examination of a right adrenal tumor, incidentally found by screening abdominal ultrasound sonography. On the clinical diagnosis of pheochromocytoma in the right adrenal gland from the findings of enhanced abdominal computed tomography, endocrinal examinations and 123I-metaiodobenzyl-guanidine scintigram, right adrenalectomy was performed transperitoneally. Histopathological diagnosis was an adrenal composite pheochromocytoma-ganglioneuroblastoma. This combination of compound adrenal tumor is extremely rare, and to date this case may be the seventh reported in Japan. Moreover, since her serum calcium level and intact parathyroid hormone level were high, so we considered the existence of multiple endocrine neoplasia type 2A. 99mTc-methoxyisobutylisonitrile scintigram and ultrasound of the neck revealed hyperparathyroidism, but medullary thyroid carcinoma was not detected. One year later, she was readmitted for parathyroid tumor excision, and histopathological finding was parathyroid adenoma. We concluded that she had both adrenal composite pheochromocytoma and hyperparathyroidism incidentally.     

Tumeurs des deux surrénales. A propos d’un cas

Bilateral adrenal tumors are rare. We report a case of adrenal pheochromocytoma associated with a contralateral adrenocortical carcinoma in a 65-year-old woman. Her presenting symptoms, consisting of hypertension associated with headache, sweating and palpitations, suggested the diagnosis of pheochromocytoma, which was confirmed by measurement of catecholamine metabolites in her urine. Ultrasonography and computed tomography revealed bilateral solid adrenal masses with tissue enhancement after the injection of contrast medium. Based on these clinical, biological and morphological findings, the diagnosis of bilateral adrenal pheochromocytoma was made and bilateral adrenalectomy was performed. Histopathological examination revealed a pheochromocytoma in the right adrenal and an unsuspected adrenocortical carcinoma in the left adrenal gland. The postoperative course was uneventful, without recurrence at 4 years follow-up. Based on the present case and a literature review, we discuss the various characteristics of this unusual entity.     

Renal cell carcinoma with asynchronous contralateral adrenal metastasis and liver cirrhosis (four years after surgery): a case report

We report a case of adrenal metastasis from renal cell carcinoma. A 52-year-old man was referred to our hospital for a left renal mass. A computed tomography revealed a left renal tumor. Liver cirrhosis and splenomegaly were observed. Blood tests revealed pancytopenia; platelet count was 2.5 x 10(4)/mm3. The patient was treated by partial splenic embolization (PSE) in an attempt to ensure a safe nephrectomy. After the embolization, his platelet count increased to 6.1 x 10(4)/mm3, and left nephrectomy was performed successfully. Histopathological finding was renal cell carcinoma (RCC). We concluded that PSE before surgery was useful for the patients with thrombocytopenia due to hypersplenism. Four years after surgery, computed tomography revealed the presence of a mass on the right adrenal gland. He was suspected of having a non-functioning adrenal tumor. Metastasis of the RCC was suspected and right adrenalectomy was performed by a laparoscopic procedure. Histologically, the mass was identified as a RCC metastasis. It is clinically rare for an RCC metastasis to the contralateral adrenal gland to occur.     

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.     

Laparoscopic resection of periadrenal paraganglioma in a patient with multiple endocrine neoplasia type 2A

We report here a case of recurrent pheochromocytoma successfully managed by laparoscopic surgery in a patient with multiple endocrine neoplasia type 2A. A 25-year-old man presented with the contralateral adrenal mass after earlier right adrenalectomy. For the preoperative diagnosis of left adrenal pheochromocytoma, adrenal sparing surgery was considered. From the intraoperative laparoscopic view, we found that the tumor originated in periadrenal sympathetic ganglia, and laparoscopic excision of paraganglioma was successfully performed that preserved the integrity of normal adrenal gland. Extra-adrenal pheochromocytoma is rather rare in MEN 2A and it is important to identify precise intraoperative localization of pheochromocytoma with laparoscopic surgery.     

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.     

Intrathoracic papillary thyroid carcinoma from occult primary disease

A 42-year-old woman undertook a chest radiograph for a routine evaluation prior to surgery for pelvic endometrioma, which revealed a right paratracheal mass slightly displacing the trachea to the left. CT of the thorax disclosed a well demarcated, heterogeneous, lobular, right paratracheal mass, bearing punctate, coarse, and curvilinear calcifications. MRI further revealed two components within the lesion: a larger, cystic, exhibiting thin septations, and a solid component at the lower part exhibiting strong enhancement. No continuity of the mass with the thyroid gland was demonstrated, which had normal size and no focal lesion. Histological examination of the resected mass disclosed lymph node tissue infiltrated by papillary thyroid carcinoma; subsequent total thyroidectomy revealed small foci of papillary carcinoma within both lobes of the thyroid gland. Ablative dose I-131 was administered and the patient was put on daily thyroid supplements.     

A case of pheochromocytoma associated with incidental multiple renal cell carcinoma originating from acquired cystic disease of the kidney in hemodialysis

The patient was a 52-year-old woman maintained on peritoneal dialysis for 9 years and on hemodialysis for 7 years. This patient complained of palpitations and hypertensive attack during the hemodialysis. Computed tomography and magnetic resonance imaging demonstrated the presence of right adrenal tumor(1.5 x 1.5 cm) and bilateral multiple renal cysts. Right adrenal pheochromocytoma was confirmed by elevated serum level of catecholamines and diagnosed by 131I-MIBG scintigram. This patient underwent right adrenalectomy and nephrectomy in consideration of future generation of cancer in the acquired cystic kidney. Histological examination confirmed malignant pheochromocytoma and small multiple renal cell carcinomas. The patient was alive without any recurrence 6 months postoperatively. To our knowledge, this is the fifth case of adrenal pheochromocytoma and renal cell carcinoma associated ACDK in a hemodialysis patient in the Japanese literature.     

Laparoscopic adrenalectomy for solitary metachronous adrenal metastasis from lung cancer: Report of a case

We report herein the case of a 69-year-old man who underwent laparoscopic adrenalectomy for a solitary adrenal metastasis 10 months after a left lower lobectomy for T2N1M0 lung cancer. A 30×20 mm tumor was found in the left adrenal gland, and dissected using an ultrasonically activated scalpel. Histological examination revealed metastatic squamous cell carcinoma. The patient recovered uneventfully and his condition is now stable 18 months after the second operation, with no evidence of local recurrence or metastatic disease. Although laparoscopic resection for malignant adrenal tumors is still controversial, we consider that laparoscopic adrenalectomy may be an optional treatment for metastatic adrenal tumors, provided the tumor is solitary, small in size, and well-localized. To our knowledge, only 14 cases of laparoscopic adrenalectomy for malignant tumors have been reported to date; however, this is the first case of successful laparoscopic adrenalectomy for a metastasis from lung cancer.     

Metastatic adrenal carcinoma suspected as a primary tumor: a case report

A 62-year-old man was admitted with chief complaints of fever and general fatigue. Enhanced computed tomography revealed a tumor (5 cm in diameter) in the right suprarenal space, and the right renoportal lymph nodes were swollen. An abdominal TI-weighted MR image showed a low-intensity tumor measuring 4.5 x 5.5 x 6.0 cm. Chest computed tomography revealed two tumors. One was 1.5 cm, on the hilum of left lung, the other was 1 cm in the S6 lung field near the pleura. These findings strongly indicated primary adrenal carcinoma and lung metastasis. Right adrenalectomy was performed. Histological examination of this tumor revealed diffuse tumor cells with irregular nuclei, and it was diagnosed as poorly differentiated adenocarcinoma. There was no possibility of primary adrenal carcinoma. The primary site of the tumor was suspected to be the left lung.     

Pedunculated hepatocellular carcinoma suspected of right adrenal tumor: a case report]

We report a rare case of extrahepatic growing hepatocellular carcinoma which was clinically diagnosed as right adrenal tumor. A 61-year-old woman was admitted for further examination of right flank pain and hypertension. Abdominal computed tomographic scan and echogram revealed a suprarenal mass. Hypercatecholaminemia was suspected from urinary analysis. Preoperative diagnosis was right adrenal tumor; suspected pheochromocytoma. On operation, we found the tumor was pedunculated from right lobe of liver and compressing normal right adrenal gland. Its clinical diagnosis was hepatocellular carcinoma. Ten months after operation she is still alive. We discuss the difficulty of differential diagnosis between extrahepatic growing hepatocellular carcinoma and adrenal tumor.     

Primary aldosteronism and hypercortisolism due to bilateral functioning adrenocortical adenomas

A 50-year-old male patient with a 15-year history of hypertension was referred to our hospital for evaluation of bilateral adrenal tumors. No Cushingoid features were observed. Computed tomographic scan showed 10-mm masses in each adrenal gland. Preoperative endocrinological examinations revealed autonomous cortisol and aldosterone secretion in this patient. The results of a subsequent adrenal venous catheterization study were consistent with the presence of a left cortisol-producing tumor and a right aldosterone-producing tumor. A left partial adrenalectomy was performed initially, but cortisol and aldosterone over-secretion persisted. Accordingly, the patient underwent a right adrenalectomy. Pathological examination of the resected specimens, including immunohistochemical analysis, demonstrated that both adenomas possibly produced cortisol and aldosterone. This is an extremely rare case of bilateral adrenal tumors, in which the left adrenocortical tumor produced and secreted cortisol or both cortisol and aldosterone and the right one produced and secreted both aldosterone and cortisol, as confirmed by clinical findings and pathological studies using immunohistochemical analysis.     

Laparoscopic Cortical-Sparing Adrenalectomy for Bilateral Pheochromocytoma

A 32-year-old female with asthma was hospitalized for pneumonia in 2/06. She underwent a CT scan of the chest which revealed an incidental finding of bilateral adrenal masses. On further questioning, she admitted to palpitations and flushing. She was normotensive. Biochemical workup was significant for elevated urinary norepinephrine and normetanephrines, and plasma catecholamine level. MIBG scan showed positive uptake in the left adrenal gland consistent with pheochromocytoma. T2 weighted MRI showed bilateral adrenal masses, left greater than right. After adequate alpha blockade with phenoxybenzamine, the patient underwent a laparoscopic left adrenalectomy. Pathology revealed a 3.5 cm pheochromocytoma. The patient then underwent a right cortical-sparing adrenalectomy to avoid complete adrenal insufficiency and Addisonian crisis. The choice of operation was made realizing the potential for increased bleeding, which was further complicated by the patient’s Jehovah’s Witness beliefs, which prohibit transfusion of any blood products. At surgery, a small, well-circumscribed mass of the inferior right adrenal gland was found, and excised in its entirety. A postoperative ACTH-stimulation test showed appropriate cortisol response. Pathology revealed a 1.5 cm pheochromocytoma, and the patient recovered uneventfully. Cortical-sparing adrenalectomy has been reported with success rates of 65–100% in avoiding exogenous steroid dependence.^1,2 Bilateral pheochromocytoma remains the most common indication. Risks for both recurrence and malignancy require lifelong follow-up in these patients. Electronic supplementary material  The online version of this article (doi:) contains supplementary material, which is available to authorized users.     

Pheochromocytoma associated with neurofibromatosis 1: a report of two cases

Pheochromocytomas may occur as either sporadic or familial tumors. Clinical features of pheochromocytomas in neurofibromatosis 1 (NF1) patients are similar to those in patients with sporadic pheochromocytomas, unlike pheochromocytomas associated with other hereditary syndromes. Here we report two cases of pheochromocytoma associated with NF1. Case 1: A 29-year-old man with previously undiagnosed NF1, was admitted to our hospital for hypertension and a right adrenal tumor. On physical examination, café-au-lait spots and neurofibromas were observed on his body. Serum and urine catecholamine levels were markedly elevated. Case 2: The patient was a 46-year-old man with NF1. The tumor was incidentally detected by ultrasonography. Serum and urine catecholamine levels were similarly elevated. 131I-MIBG scintigraphy showed an abnormal accumulation in the right adrenal gland. After this diagnosis of pheochromocytoma associated with NF1, open adrenalectomy was performed. No evidence of malignancy was seen in either case.     

A case of multiple endocrine neoplasia type 2A (MEN2A) with a mutation in the RET gene]

A 44-year-old woman complained of headache and palpitation. Magnetic resonance imaging showed bilateral adrenal tumors 10 x 9 cm in size on the left side and 8 x 4 cm in size on the right side. CT scan revealed a 0.7 x 0.7 cm mass in the thyroid. Hormonal examinations showed high values of urinary cathecholamines and serum calcitonin. DNA sequence analysis of peripheral white blood cells revealed that codon 634 in exon 11 of the RET gene was mutated from TGC (Cys) to TAC (Tyr). From these findings, a diagnosis was made of MEN2A with bilateral adrenal pheochromocytomas and medullary thyroid carcinoma. Bilateral adrenalectomy and thyroidectomy were performed. The same mutation of the RET gene was detected in all her 3 children, in two of whom, early stage medullary thyroid carcinoma was detected and thyroidectomy was performed. DNA analysis of the RET gene was useful for the diagnosis of carriers of MEN2A and the early detection of medullary thyroid carcinoma.