A case of Sipple syndrome whose bilateral pheochromocytomas were resected separately after a 3-year interval

A 23-year-old woman who had undergone total thyroidectomy and parabronchial lymphadenectomy at the Department of Otolaryngology in our hospital was suspected to have a thyroid carcinoma. Histological examination revealed a medullary carcinoma of the thyroid and normal parathyroid glands. Because a computed tomographic scan after surgery revealed a tumor of the left adrenal gland, the patient was examined at our department two months after the thyroidectomy, 1994. Endocrinic examinations and 123I-MIBG scintigraphy revealed a left adrenal pheochromocytoma of Sipple syndrome. She then underwent left adrenalectomy. Histological diagnosis was pheochromocytoma of the left adrenal gland. Three years after the surgery, a right adrenal tumor was detected by computed tomography. Although results of endocrine examinations were normal, 131I-MIBG scintigraphy showed a hot spot in the right adrenal gland. Right adrenal pheochromocytoma was diagnosed 3 years after surgery for a contralateral adrenal pheochromocytoma. Although surgery was recommended, the patient consented to right adrenalectomy, 1 year later after marriage and before becoming pregnant. Histological examination of the resected specimen revealed pheochromocytoma of the right adrenal gland.     

Hématome spontané de la surrénale (à propos de 2 cas)

Spontaneous hematoma of the adrenal glands is a very rare condition and may have multiple etiologies. It is usually asymptomatic and may easily be confused with a malignant tumor of the adrenal glands. We herein report two cases: the first patient was a 77-year-old male patient with an uneventful medical history who was admitted for abdominal pain. Ultrasonography and CT-scan revealed a heterogeneous mass of the right adrenal gland. The second patient, a 31-year-old woman presented with intermittent left lumbar pain. Ultrasonography and CT-scan revealed a cystic tumor of the left adrenal gland. In both cases laboratory tests were normal and adrenalectomy was performed. Histopathological evaluation revealed an isolated hematoma without adrenal abnormalities. The clinical, diagnostic and therapeutical aspects are discussed     

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.     

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.     

Idiopathic adrenal hematoma: a case report

We report a rare case of idiopathic adrenal hematoma. Including our case, 13 such cases have been described in Japan. A 63-year-old [correction of 65] woman was admitted to our hospital for further examination of a right adrenal mass on ultrasonography. Laboratory tests including hormonal assay were within the normal ranges. Computed tomography showed a tumor with calcification measuring 3.0 x 2.0 cm in the right adrenal gland. Magnetic resonance imaging (MRI) revealed a mass with heterogeneous low to iso signal intensity on T1-weighted images and high signal intensity on T2-weighted images. A peripheral rim of the mass was slightly enhanced on dynamic MRI. The patient underwent laparoscopic adrenalectomy. Histopathological examination revealed an old hematoma without neoplastic cells or vascular lesions and these findings were evidence of idiopathic adrenal hematoma.     

Myelolipoma of the adrenal gland presenting as upper abdominal symptoms: a case report

A case of adrenal myelolipoma is presented. A 39-year-old woman was admitted to our hospital for further examination of a right adrenal mass, which was found by examination for nausea, vomiting and upper abdominal pain. Imaging analyses by computed tomography and magnetic resonance imaging revealed a round fatty mass. Endocrine study of the adrenal gland showed normal results. Right adrenalectomy was performed. The tumor weighed 240 g and the histological diagnosis was adrenal myelolipoma. Postoperative course was uneventful and upper abdominal symptoms disappeared after surgery.     

Rupturierte vaskuläre Nebennierenzyste durch Badeunfall

Endothelial cysts of the adrenal gland are a rare entity of adolescents. We here present the case of a 19-year-old woman, who developed right abdominal pain after trauma. Magnetic resonance imaging showed a tumor in the right adrenal gland with suspicious signs for malignancy. Histopathological examination revealed the diagnosis of an endothelial (vascular) adrenal cyst. In most cases, cysts of the adrenal gland remain clinically inapparent. In cases of clinically manifest symptoms, surgical intervention represents the standard for therapy.     

Adrenal pseudocyst; a case report

We report a case of adrenal pseudocyst. A 35-year-old woman presented with palpation of right upper abdominal mass without tenderness. Abdominal computed tomographic scan showed a right retroperitoneal cystic mass 20 cm in diameter. The patient underwent complete resection of the mass, including the normal adrenal gland. The cyst contained 3100 ml of dark brown thrombotic liquid. Histopathological examination revealed adrenal pseudocyst with a thick figrocollagenous wall. The normal adrenal gland was compressed by the wall. Adrenal pseudocyst is a rare disease. The mechanisms of adrenal pseudocyst formation and their expanding nature are discussed.     

Successful treatment of primary aldosteronism due to computed tomography-negative microadenoma

We report a case of aldosterone-producing microadenoma that was correctly diagnosed and thus treated less invasively by laparoscopic adrenalectomy. A 58-year-old woman presented with palpitation and muscular weakness. She exhibited hypertension, hypokalemia and increased aldosterone excretion with suppressed renin activity. Therefore, primary aldosteronism was suggested. Although adrenal scintigram and computed tomography findings in the adrenal glands were normal, adrenal venous sampling tests indicated an overproduction of aldosterone in the right adrenal gland. We diagnosed an aldosterone-producing microadenoma in the right adrenal gland and performed an adrenalectomy. The patient became normotensive postoperatively and histopathological examination demonstrated a microadenoma, 5 mm in diameter.     

Nonfunctioning malignant pheochromocytoma associated with dermatomyositis: case report and literature review

A 42-year-old man with dermatomyositis presented with right-upper-quadrant dull pain and normal blood pressure for 10 days. Abdominal ultrasonography, abdominal computed tomography, and angiography revealed a retroperitoneal tumor with direct invasion to the liver and the diaphragm. The diagnosis of nonfunctioning malignant pheochromocytoma was made on the basis of clinical evidence. The tumor was removed en bloc with part of the diaphragm, the right lobe of the liver, the right adrenal gland, and the right kidney. Pathologic examination with immunohistochemical staining revealed a malignant pheochromocytoma growing exophytically from the right adrenal gland and invading the right lobe of the liver and the diaphragm. The postoperative course has been uneventful and no recurrence has been noted over a 6-month follow-up period.     

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.     

肾上腺嗜铬细胞瘤引起库欣综合征一例报告并文献复习

目的探讨肾上腺嗜铬细胞瘤引起库欣综合征的临床诊断和治疗。 方法结合文献复习,回顾性分析2015年12月我院收治的1例右侧肾上腺嗜铬细胞瘤引起库欣综合征患者的资料。患者女性,45岁,因反复头痛半年,加重伴头晕、多尿、消瘦2个月收入我院内分泌科。术前诊断考虑为肾上腺嗜铬细胞瘤引起库欣综合征,同时按库欣综合征和嗜铬细胞瘤做术前准备,行腹膜后入路腹腔镜右侧肾上腺肿瘤切除术。 结果内分泌检查示促肾上腺皮质激素（ACTH）、皮质醇高于检测值上限,且大、小地塞米松抑制试验不受抑制。血儿茶酚胺三项均升高,以肾上腺素为主,可乐定试验不受抑制。甲氧基肾上腺素及甲氧基去甲肾上腺素均升高。胸部CT平扫及PET-CT未发现可疑异位ACTH来源。肾上腺MR示双侧肾上腺内、外支弥漫性增厚,右侧肾上腺内、外侧支之间可见一类圆形肿块,边界清,考虑肾上腺嗜铬细胞瘤可能。予口服甲吡酮抑制皮质醇生成,常规剂量酚苄明作术前准备,行腹膜后入路腹腔镜右侧肾上腺肿瘤切除术。病理提示右侧肾上腺嗜铬细胞瘤,合并肾上腺皮质区带性增生。免疫组化CgA（+）,ACTH（+）。术后ACTH及皮质醇降至正常范围。术后6个月复查提示患者高血压、糖尿病等表现消失。 结论嗜铬细胞瘤引起库欣综合征是一种罕见疾病,手术切除是有效治疗手段,术前需同时按照嗜铬细胞瘤和库欣综合征完善术前准备。     

Adenocarcinoma of the renal pelvis and transitional cell carcinoma of the ureter occurring 11 years after radical cystectomy for bladder cancer: a case report

We report a case of upper urinary tract carcinoma which recurred 11 years after total cystectomy. A 52-year-old man presented with complaints of a sense of residual urine and terminal miction pain. Urinary cytology, cystoscopic examination and intravenous pyelography revealed normal findings. Twenty months later, because class V urinary cytologic findings were detected, transurethral biopsy was performed. Carcinoma in situ was diagnosed pathologically. Therefore, total cystectomy and ileal conduit urinary diversion were performed. The pathological diagnosis was transitional cell carcinoma, grade 3, pTis. At 127 months postoperatively, laboratory examination revealed an extremely high serum level of LDH (3,084 U/l). The right kidney was not visualized on IVP and computed tomography revealed a right renal irregular mass. On the suspicion of a renal pelvic tumor, right total nephroureterectomy was done. The pathologic diagnosis was renal pelvic adenocarcinoma and ureteral transitional cell carcinoma. The patient was treated postoperatively with 3 cycles of systemic chemotherapy and radiotherapy. The serum level of LDH returned to normal. However, one year later, the serum level of LDH elevated to 1,118 U/l. He died of retroperitoneal lymph node, left adrenal gland and pulmonary metastases.     

Adrenal epithelioid angiosarcoma: a case report

We report what to the best of our knowledge is the first case of primary angiosarcoma of the adrenal gland. A 49-year-old woman, with blunt abdominal trauma, was admitted to our department. Computed tomography revealed a suspected rupture of a liver neoplasm and surgical treatment was performed. At laparotomy there was no evidence of liver tumour but a large mass was found in the right adrenal gland. The mass was removed along with the entire periadrenal fat tissue and locoregional lymph nodes. Microscopically, we found diffuse neoangiogenesis with large, hyperchromatic cells. This cellular proliferation, together with the widespread necrosis, distorted the normal appearance of the adrenal gland. No adjuvant therapy was administered. Six months and 1 year later thoracic- abdominal computed tomography and positron emission tomography were performed, and no signs of local recurrence or metastases were observed.     

Ganglioneuroma with calcification mimicking adrenal tumor: a case report

A 28-year-old woman was referred to our hospital complaining of upper abdominal discomfort. The patient had been receiving medical treatment for hypertension. Computed tomography revealed a 30 mm solid tumor with calcification in the left adrenal gland and a 8 mm nodule in the right adrenal gland. Endocrinological examinations revealed no activity of either adrenal mass. The left adrenal tumor was extirpated, because malignancy of the tumor was not ruled out. Histopathological examination proved that the tumor was ganglioneuroma arising from the extra-adrenal retroperitoneum.     

A case of retroperitoneal neurinoma

Retroperitoneal neurinoma is a rare disease, although retroperitoneal tumor is rather common. A 40-year-old man without any clinical symptoms was admitted to our hospital for a medical examination. Ultrasonogram demonstrated a cystic mass over the right kidney. This cystic mass was suggested to be a right adrenal tumor by CT scan and angiogram. All of the laboratory data including endocrinological data were within the normal limits. Under the diagnosis of right non-functioning adrenal tumor, an operation was performed through a right lumbal incision. A round and tennis ball sized tumor with a fibrous capsule was identified and freed from the right kidney and the right adrenal gland, and the tumor was extirpated uneventfully. The surgical specimen was 9.5 X 8.0 X 6.5 cm in size, 260 g in weight, and the histological diagnosis was neurinoma (Antoni-B type).     

Unilateral asymptomatic adrenal tuberculoma: the role of percutaneous biopsy

We report the case of a 55-year-old male with a large left adrenal mass that conformed to the normal shape of the adrenal gland. Percutaneous biopsy revealed tuberculosis and, following anti-tuberculosis therapy, there was a >75% decrease in the size of the mass. In large tumefactions of the adrenal glands in which a normal adrenal contour is maintained, a percutaneous biopsy may obviate the need for major intervention.     

Two case reports of unilateral adrenal hyperplasia with contralateral renal cell carcinoma

We report two cases of unilateral adrenal hyperplasia with contralateral renal cell carcinoma. First case was a 66-year-old man with right renal mass who came to our hospital for study. Although no major symptoms were complained, blood pressure was consistently above normal. Hypokalemia, low plasma renin activity and high plasma aldosterone level were detected. CT revealed that he had a right renal mass and a left adrenal tumor. Preoperative diagnosis was right renal tumor and primary aldosteronism due to left adrenal adenoma. Second case was a 59-year-old man with right renal mass who also came to our hospital for study. He also had a left adrenal tumor, however his blood pressure was normal and serum hormonal analysis showed normal adrenal cortical function. Preoperative diagnosis was right renal tumor and non-functional left adrenal adenoma. Two patients were performed right radical nephrectomy with contralateral adrenarectomy. Pathological diagnosis were right renal cell carcinoma and left adrenocortical hyperplasia. One Patient clinically showed primary aldosteronism and the other had no clinical symptoms. These patients were rare cases with unilateral adrenal hyperplasia.     

Massive retroperitoneal hemorrhage from adrenal gland metastasis.

We report an unusual case of spontaneous massive retroperitoneal hemorrhage from an adrenal gland metastasis. After medical therapy failed to stabilize the patient's condition, surgical exploration revealed a large retroperitoneal hematoma arising from a right adrenal gland metastasis. At the time of thoracoabdominal exploration in the lower lobe of right lung a small tumor nodule was palpated and resected. Pathologic examination of both lung and abdominal lesions revealed squamous cell carcinoma thought to have been primary in the lung. A review of the literature reveals that metastatic lesions to the adrenal gland are infrequently encountered clinically and rarely hemorrhage; the first such case in which massive retroperitoneal hemorrhage was a complication is reported in the urologic literature.     

Multiple calcified adrenal myelolipoma suggestive of association of primary aldosteronism: report of a case

We report a case of adrenal myelolipoma with multiple calcification and hypertension. A 69-year-old woman visited our hospital with a complaint of right flank pain. Computed tomography demonstrated a right adrenal tumor which was a spherical mass with fat density and multiple calcification. Adrenal scintigraphy of I-131 adosterol demonstrated predominant accumulation of the right adrenal gland. Selective venous sampling disclosed a high aldosterone level (303.7 ng/dl) from the affected side. Right adrenalectomy was performed. Pathological diagnosis revealed adrenal myelolipoma with calcification. After the operation the patient became normotensive. The characteristics of the 12 cases of myelolipoma with calcification we found in the Japanese literature are also briefly reviewed.