Adrenocortical carcinoma detected by retroperitoneal hemorrhage : a case report

Spontaneous massive retroperitoneal hemorrhage from an adrenal tumor is rare and is usually fatal if unrecognized. We report a case of spontaneous rupture of a primary adrenocortical carcinoma that occurred in a 79-year-old man. He visited our hospital with left abdominal pain. Computed tomography (CT) showed a left retroperitoneal hemorrhage. We could not find the origin of this hemorrhage. Two months later, CT showed the left adrenal tumor, and left adrenalectomy and nephrectomy were performed successfully. The histological diagnosis was adrenocortical carcinoma. He rejected adjuvant therapy. Local recurrence of the tumor was found, and right adrenal gland, brain, and mediastinal lymph node metastases were recognized 6 months after the operation. He died 11 months after the operation.     

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。     

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。     

肾球旁细胞瘤的诊治(附五例报告)

目的：提高肾球旁细胞瘤的诊治水平。方法：回顾分析5例肾球旁细胞瘤患者的临床特点、诊断、治疗方式及治疗效果等临床资料。男2例、女3例。平均年龄31岁。5例临床表现均为低血钾，4例行血浆肾素、醛固酮测定，表现为高肾素、高醛固酮血症3例，血浆肾素、醛固酮正常1例。结果：5例患者中1例术前怀疑右肾上腺肿瘤、右肾肿瘤，行手术探查，术中冰冻病理示肾脏良性肿瘤行肾脏肿瘤剜除，右肾上腺活检术，术后病理示：右肾球旁细胞瘤，正常肾上腺组织。4例术前明确诊断者行肿瘤剜除术治疗。5例患者术后血钾、血浆肾素、醛固酮、血压均恢复正常，随访1年3个月至10年未见肿瘤复发及高血压再发。结论：肾球旁细胞瘤为良性肾脏肿瘤。术前应与原发性醛固酮增多症、肾动脉狭窄等相鉴别。肿瘤根治性切除效果满意。     

肾上腺皮质肿瘤的诊断与治疗(附230例报告)

目的:提高肾上腺皮质肿瘤的诊断与治疗水平。方法:对230例肾上腺皮质肿瘤的临床表现、术后病理及随访结果进行分析。结果:原发性醛固酮增多症皮质瘤161例(2例为恶性醛固酮瘤),其中140例获得3个月～6年随访,均无复发;皮质醇症皮质腺瘤21例,其中19例获1～6年随访,治愈未见复发;无功能性肾上腺皮质腺瘤48例(4例为皮质腺癌),其中38例获6个月～6年随访,已治愈。结论:良性肾上腺皮质肿瘤手术疗效满意,恶性肾上腺皮质肿瘤应早期诊断,及时手术探查。     

Giant suprarenal tumor

Adrenal tumors evoke considerable interest and represent various diagnostic challenges. Adrenal tumors can be stratified into adrenal medullary and adrenocortical tumors. Approximately 60% of adrenocortical tumors are hormonally active and show specific signs and symptoms. Patients with a nonfunctioning adrenal tumor usually present with abdominal discomfort due to the mass effect of the tumor. An imaging feature that differentiates benign from malignant adrenal neoplasms is the tumor size. Thus, for the differential diagnosis of adrenal lesions, measurement of the Hounsfield units on an unenhanced computed tomographic scan is of great value when differentiating malignant from benign lesions. Herein, we describe a young female patient who presented with a huge left suprarenal tumor. She underwent complete resection of the adrenal tumor. The final pathological diagnosis was an adrenocortical carcinoma. There has been no evidence of recurrence for the last 4 years.     

Synchronous Testicular Seminoma and Adrenocortical Carcinoma: A Case Report

We report a rare case of synchronous testicular seminoma and adrenocortical carcinoma. A 57-year-old man had a left testicular seminoma (clinical stage 1MB) with metastases to the lung and paraaortic lymph node. A complete response was obtained after 3 courses of chemotherapy with single-agent carboplatin. However, a left adrenal tumor was detected 1 2 months later and demonstrated a tumor volume doubling time of 2.1 months. Chemotherapy with bleomycin, etoposide and cisplatin failed to stop the tumor growth. A laparoscopic adrenalectomy was performed and pathology revealed an adrenocortical carcinoma. The patient has been free of recurrence for 42 months postoperatively.     

Cis-platinum used for the prevention of the recurrence of adrenal cortical carcinoma: report of a case

The right suprarenal mass was found in a 21-year-old housewife. Her major clinical features were amenorrhea, polydipsia and buffalo hump obesity. Endocrinological and roentgenological studies suggested the presence of Cushing's syndrome due to adrenocortical carcinoma in addition to ipsilateral renal stone. The huge adrenal tumor and renal calculus were successfully removed. The histological diagnosis was adrenocortical carcinoma. Seventeen days after the operation, cis-platinum was administered to prevent the recurrence of tumor development. No recurrence has been observed for approximately 2 years after the surgery. Long follow-up must be pursued to clarify the real efficacy of cis-platinum treatment.     

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.     

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.     

Histologically benign but telomerase positive adrenal pheochromocytoma.

Adrenalectomy was performed on a 37-year-old woman with right adrenal pheochromocytoma. Although the histological diagnosis was benign, telomerase repeat amplification protocol (TRAP) demonstrated positive telomerase activity. Thirty-five months after adrenalectomy, a recurrent tumor was found. Forty-seven months after adrenalectomy, bone metastases also appeared. Malignant pheochromocytoma was clinically diagnosed. Our experience suggests that telomerase-positive tumor cells are highly likely to repeat cell proliferation when they are left in the patient's body, and lead to recurrence. Therefore, telomerase-positive tumors should be clinically treated as malignant even if they have been histologically diagnosed as benign.     

Long-term survival after bilateral adrenalectomy for metachronous adrenocortical cancer

We report the case of a female patient with bilateral metachronous adrenocortical cancer who survived long-term after adrenalectomy. In 1991, the patient underwent left adrenalectomy to remove a huge adrenal mass (10 x 9 cm) displaying no hormonal abnormality. Histological diagnosis was adrenocortical cancer. A right adrenal mass (7 x 6 cm) was found 4 years after left adrenalectomy. Right adrenalectomy was performed, and histological diagnosis was again adrenocortical cancer. The patient remains alive with no evidence of disease 8 years after last surgery.     

黏液样肾上腺皮质腺瘤一例报告及文献复习

目的 探讨黏液样肾上腺皮质腺瘤的临床病理特点及治疗方法.方法 分析1例黏液样肾上腺皮质腺瘤患者的临床资料,总结其临床表现、影像学特点、病理学结果及治疗方法,结合文献复习讨论.患者,女,43岁.主诉反复发作头痛、乏力10年,伴高血压、低血钾症.B超提示左肾上腺4.1 crux 3.4 cm低回声实性占位,CT提示肾上腺内2.5 cm×3.8 cm×4.0 cm肿物,CT值24HU,增强后瘤体外周CT值41 HU,中心未见明显强化.结果 患者行腹腔镜下手术,完整切除肿瘤及同侧肾上腺.病理报告:肿物灰粉灰红色相间,质地较硬.肿物剖面呈灰粉灰黄相间的胶冻样物,可见散在出血点.镜下肿瘤细胞大小一致,细胞境界清楚,核膜及核仁无明显异型性,未见核分裂象.肿瘤细胞内可见黏液样物质,部分区域可见细胞周围黏液样基质.免疫组织化学及特殊染色结果:波形纤维蛋白(+)、黑色素蛋白一A(+),阿尔辛蓝/对氨基水杨酸染色(+).病理诊断:黏液样肾上腺皮质腺瘤.术后患者血压及血钾等各项生化指标恢复正常,随访6个月未见肿瘤复发.结论 黏液样肾上腺皮质腺瘤是少见的肾上腺皮质肿瘤,确诊需依靠病理学检查,手术切除肿瘤及同侧肾上腺为首选治疗.     

Left adrenocortical cancer with inferior vena cava tumor thrombus--a case report

An 62-year-old male was admitted to our hospital for an evaluation of high grade fever, body weight loss and lumbago. He was diagnosed as having a left adrenal tumor with intracaval extension and underwent a radical surgery, including resection of the tumor, left kidney, spleen and IVC tumor thrombus. Histopathological diagnosis was adrenocortical carcinoma with tumor thrombus. To our knowledge, our case seems to be the 8th case report of left adrenocortical cancer with tumor thrombus extension into IVC. Average survival of reported cases was about 15 months. At 4 months after surgery, the patient died due to lung metastasis.     

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.     

Primary malignant giant cell tumor of bone: a series of three rare cases

The purpose of this research was to study incidence; clinical, histological, and radiological features; and outcome of primary malignant giant cell tumor (PMGCT). The authors retrospectively reviewed all cases of giant cell tumor (GCT) in which a diagnosis of GCT was related to sarcoma treated in their department between 1997 and 2004. Three cases of PMGCT were found according to the criterion of Hutter and Dahlin. Histological and radiological records of all the three cases were reviewed. In these three cases of PMGCT, the initial clinical and radiological findings were the same as those for benign giant cell tumor. Wide excision of the tumor was performed in all three cases. In two cases, knee arthrodesis was performed, and in one case a custom-made total knee replacement was performed. PMGCT was diagnosed on initial biopsy in one patient, in the second patient it was diagnosed in the excised specimen, and in third case it was only diagnosed after local recurrence 6 months after initial treatment. All the patients died within 5 months of detection of recurrence and metastasis. PMGCT has a very poor prognosis. Histological examination is highly significant in such cases. Awareness about this entity, adequate biopsy, and sampling of specimen can aid in early diagnosis, which may improve the overall prognosis.     

Pathological and clinical studies on primary aldosteronism

In the surgical treatment of patients with primary aldosteronism, differentiation of idiopathic hyperaldosteronism due to bilateral adrenal hyperplasia should be kept in mind. Controversies exist, on the other hand, concerning the diagnosis and management of multiple nodular hyperplasia which often coxists with adrenal adenoma. We studied correlations between pathological findings and clinical diagnosis on 13 patients with primary aldosteronism who were operated on during the 19 year period from 1963 to 1981, and the results were as follows; (1) Histologically, adrenal glands of these patients were classified into three subgroups, namely i) adrenocortical adenoma alone, ii) adrenocortical adenoma associated with multiple nodular hyperplasia, and iii) nodules of multiple nodular hyperplasia alone. (2) Clinically, the latter two subgroups showed increase in plasma renin activity following the combination test of volume depletion and spironolactone administration. From these results it is suggested that even in patients with primary aldosteronism multiple nodular hyperplasia of the adrenal cortex can coexist together with adrenal adenoma, and that coexistence of this hyperplasia may modify some clinical features of the disease.     

A case of adrenal cortical carcinoma

We report a case of left adrenocortical carcinoma in a 23-year-old man. His chief complaints were spike fever attack and general malaise. Imaging examination revealed a heterogeneously enhanced mass at the site of the left adrenal gland. Non-functional adrenocortical carcinoma was suspected from hormonal studies. The mass was removed together with the left kidney because of severe adhesion to the renal pedicle. The histopathological findings showed adrenocortical carcinoma (pT3N0M0). Three months after surgery, local recurrence appeared and the tumor grew larger rapidly. Spike fever attack reappeared. No distant metastasis was detected. Two courses of systemic chemotherapy with o, p'-DDD, VP-16, adriamycin and cisplatinum were performed. Computed tomography after the chemotherapy showed progression. Although o, p'-DDD was administered continuously, the patient died of cancerous cachexia 8 months after the surgery. Autopsy could not be done.     

Secondary chondrosarcoma in cartilage bone tumors: report of 32 patients

Background Secondary malignancies arising from benign bone tumors are rare. Their recognition and diagnosis are difficult, and their slow growth and late recurrence require long-term follow-up. In this study, malignant transformation rates of various histological types of benign cartilage-forming bone tumors in large series were evaluated. Methods Between 1986 and 2004, a retrospective analysis of 627 cartilage-forming benign bone tumors revealed that 32 patients had malignant transformation. Of the 32 patients, 14 had solitary osteochondromas, 10 had multiple osteochondromas, 6 had a solitary enchondroma, 1 had Ollier's disease, and 1 had Maffucci's syndrome. The patient with Ollier's disease had two chondrosarcomas, and one patient with multiple osteochondroma had three chondrosarcomas. The cases were included in the study only when complete clinical documentation, radiological records, and histological analyses were available. Results The rate of malignant transformation for cartilage-originating tumors was 5.1% (solitary osteochondromas 4.2%, multiple osteochondromas 9.2%, solitary enchondromas 4.2%). The average time between the initial diagnosis and malignant transformation was 9.8 years. The most common site of involvement was the proximal portion of the femur. The tumors generally were well differentiated. The mean follow-up period was 57.3 months. Five patients (15.6%) died of tumor recurrence or metastasis at an average of 20.6 months. One patient is alive with tumor at 104 months. Conclusions Cartilage-forming benign bone tumors are rather prone to undergo malignant transformation. Although malignant transformation of a benign bone tumor is a rarely encountered situation, orthopedic surgeons should be cautious while following patients with a benign bone neoplasm. Early recognition and appropriate surgical treatment are required to achieve successful outcomes. The rate of local recurrence in secondary chondrosarcomas depends not only on adequate surgical treatment but also on the localization and histological grade.     

Desmoplastic fibroma of the fibula. A difficult clinical, radiological and histological diagnosis

Desmoplastic fibroma of bone is a rare benign tumor that is made up of wavy fibroblasts and abundant collagenous tissue. The case of a 18-year-old patient is presented with a two months history of weightbearing pain in the left knee. Neither native x-ray, CT nor MRI could detect the kind of tumor. The histological findings lead finally to the diagnosis of a desmoplastic fibroma. Wide resection prevented recurrence of the tumor for 8 1/2 years until now. Considering the semi-malignant character of the desmoplastic fibroma and the recurrence rate marginal or wide resection for the primary treatment is recommended. The superior imaging quality of MRI facilitates preoperative planning.