A case of adrenal myelolipoma associated with adrenogenital syndrome

Myelolipoma is a benign non-functioning tumor, and the number of documented cases is increasing in recent years. We report a case of adrenal myelolipoma associated with adrenogenital syndrome. A 65-year-old woman presented with a complaint of abdominal discomfort. CT showed a mass with abundant fatty density in the left adrenal gland suggesting myelolipoma. She was small and thin. External genitalia had a female appearance, but showed type III abnormality in Prader's classification with clitoral hypertrophy. She had a history of primary amenorrhea. Endocrinological examination revealed marked increase in the 17 alpha-hydroxyprogesterone (or 17-OHP) and pregnanetriol levels. On the basis of clinical features and laboratory values, simple virilizing type congenital adrenal hyperplasia due to 21-hydroxylase deficiency was made. The left adrenal tumor was surgically removed and histologically proved to be myelolipoma. Tumor-uninvolved adrenal area adjacent to myelolipoma showed adrenocortical hyperplasia. To our knowledge, this is the first reported case of adrenal myelolipoma to be associated with congenital adrenal hyperplasia, due to 21-hydroxylase deficiency in Japan. The etiology of myelolipoma remains to be not clarified but our case suggests involvement of endocrine disorder in the development of this tumor.     

Clinical Spectrum of Adrenal Myelolipoma: Analysis of 8 Tumors in 7 Patients

Adrenal myelolipoma is a rare, benign, and biochemically nonfunctioning tumor. Most patients with adrenal myelolipoma are asymptomatic and the condition is discovered incidentally at autopsy. Review of the literature reveals 70 patients with surgically documented adrenal myelolipoma. We report 7 additional patients with 8 adrenal myelolipomas, of whom 5 underwent surgical removal because of tumor size, unusual imaging features or symptomatology. Of our patients 4 had symptomatic lesions requiring surgical removal, while 1 had an adrenal myelolipoma that was discovered intraoperatively. Of the remaining 2 patients 1 had a small (3 cm.) lesion discovered incidentally on excretory urography and 1 had bilateral adrenal myelolipoma discovered incidentally on computerized tomography.     

Combined adrenal adenoma and myelolipoma in a patient with Cushing''s syndrome: Case report and review of the literature

Myelolipoma is an uncommon benign tumor of unknown etiology and adrenal myelolipoma is rarely associated with endocrine disorders. We report a 67-year-old woman with Cushing's syndrome due to left adrenal adenoma associated with myelolipoma. The patient underwent laparoscopic left adrenalectomy and pathological examination revealed an adrenocortical adenoma associated with myelolipoma. To the best of our knowledge, 25 cases of endocrine dysfunction associated with myelolipoma have been reported in the English and Japanese literature. We review and discuss the pathogenesis of adrenal myelolipoma.     

肾上腺髓性脂肪瘤的诊断与治疗

目的：探讨肾上腺髓性脂肪瘤的影像学特点和诊治方法.方法：肾上腺肿瘤患者12例.男5例,女7例.平均年龄48（33～64）岁.左侧4例,右侧8例.血压高2例.腰腹部疼痛1例,无特异性症状9例.行B超、CT及MRI检查.术前提示为肾上腺占位,其中肾上腺髓性脂肪瘤10例.嗜铬细胞瘤1例,肾上腺肿瘤1例.12例均行肾上腺肿瘤切除术,其中开放手术7例.腹腔镜手术5例.结果：12例手术均顺利完成.平均手术时间1.5 h,平均术中出血量50 ml.术后无明显外科并发症,平均7天顺利出院.切除肿瘤最小3.0 cm×3.5 cm×4.0 cm.最大6.5 cm×7.5 cm×8.0 cm.病理诊断均为肾上腺髓件脂肪瘤.术后随访0.5～8年（平均2年）.所有患者临床症状消失,B超及CT检查末见肿瘤复发及恶性变.结论：肾上腺髓件脂肪瘤多无特异性临床表现,术前诊断主要靠影像学检查,确诊依赖于病理检查 手术切除预后良好,其中腹腔镜手术创伤小,疗效满意,为治疗本病的理想方法.     

肾上腺髓性脂肪瘤的诊疗(附9例报告)

目的:探讨肾上腺髓性脂肪瘤的诊断与治疗方法.方法:回顾性分析9例肾上腺髓性脂肪瘤患者的临床资料,并总结其诊断和治疗经验.9例中,7例无症状,1例有腰部不适,1例血压增高.B超与CT诊断8例,1例误诊为肾错构瘤.肿瘤发生于左侧肾上腺区4例,右侧肾上腺区5例,开放手术8例,腹腔镜手术1例.结果:肿瘤直径5～11cm,术后病理检查证实均为肾上腺髓性脂肪瘤.术后随访6个月～5年,无肿瘤复发.结论:肾上腺髓性脂肪瘤可依靠B超、CT或MRI等影像学检查诊断,手术切除肿瘤安全有效,腹腔镜手术较开放手术更有优越性.     

肾上腺髓样脂肪瘤3例报告并文献复习

目的探讨肾上腺髓样脂肪瘤的临床、影像学及病理特点。方法回顾性分析2000年1月～2011年12月3例肾上腺髓样脂肪瘤患者的临床资料并复习相关文献。患者年龄分别为53、67、73岁,平均64岁,男性1例,女性2例,左侧2例,右侧1例,肿瘤直径2.5～11cm。结果 2例患者经后腹腔镜肾上腺肿瘤切除,1例经腹开放手术切除,术后病理检查均证实为肾上腺髓样脂肪瘤。随访6个月至2年未见复发。结论肾上腺髓样脂肪瘤的诊断主要依靠B超、CT和MRI,确诊需经病理学检查证实。手术切除是主要的治疗方法,症状明显或瘤体直径〉4cm者应尽早手术。     

Bilateral adrenal myelolipoma associated with adrenogenital syndrome

Adrenal myelolipoma is a rare benign tumor, occasionally reported in association with endocrine disorders. We report herein a case of bilateral adrenal myelolipoma associated with adrenogenital syndrome caused by 21-hydroxylase deficiency. A diagnosis of 21-hydroxylase deficiency was confirmed by mutation analysis of the CYP21 gene. Our case represents only the second case of bilateral adrenal myelolipoma associated with adrenogenital syndrome caused by 21-hydroxylase deficiency.     

Adrenal myelolipoma: a report of a case

This is a case report of an adrenal myelolipoma, accidentally diagnosed during a work-up for bladder tumor. A 67-year-old male presented with the chief complaint of gross hematuria. He was subsequently diagnosed as having a bladder tumor, which was resected transurethrally and was found to be a transitional cell carcinoma of Grade 2 and Stage pT2. During further examination for metastasis computed tomography (CT) scan revealed a round tumor (approximately 5 cm in diameter) in the left adrenal. A tentative diagnosis was reached based on the scan, and surgery was undertaken to remove the tumor. A well-encapsulated tumor, yellowish and partly dark brown in color and 60 grams in weight, was retrieved. The tumor consisted of mature lipoid cells with myeloid cells scattered among them which verified the pathological diagnosis of adrenal myelolipoma. The present case is the 16th clinical case of adrenal myelolipoma reported in the Japanese literature.     

Adrenal myelolipoma: report of a case

We report a case of adrenal myelolipoma occurring in a 56-year-old man. The patient complained of lumbago and gross hematuria in October, 1981 and consulted a local doctor who pointed out a right ureteral stone and downward deviation of the left kidney. The patient was referred to our hospital for further examination. An echogram showed a high echogenic mass in the left flank which was compressing the spleen and the kidney. A CT scan revealed a large mass with heterogeneous low density contents. The mass was lobulated into several parts and their density suggested fat tissue. An aortogram showed an avascular mass and an adrenal venogram showed multiple veins which radiated to the periphery. Judging from these findings, a tentative diagnosis of adrenal myelolipoma was made and left adrenalectomy was performed. Histological examination revealed that this tumor was composed of adrenal tissues, fatty tissues and myeloid tissues. The postoperative course was uneventful. We discuss the necessity of aortography and selective adrenal venography to evaluate the origin of the tumor. To our knowledge, this is the tenth case report of adrenal myelolipoma in Japan and we have made a brief review of Japanese literature of adrenal myelolipoma.     

Adrenal myelolipoma associated with Cushing's syndrome: a case report]

A 42-year-old woman was referred to our hospital for evaluation of severe hypertension. A right adrenal tumor was revealed by CT scan, and the elevation of cortisol and u-17OHCS was found. We made a diagnosis of adrenal adenoma with Cushing's syndrome and performed right adrenalectomy. However, a small myelolipoma (1.5 mm in diameter) was found beside the cortical adenoma by histopathological examination. Adrenal myelolipoma is not a rare disease now, because it is easily-detected as an incidental by CT scan. The present case of adrenal myelolipoma, however, is interesting and uncommon in its connection with functioning cortical adenoma. Only 3 cases have been previously reported so far in English and Japanese publications. We discuss the etiology of adrenal myelolipoma, and suggest that myelolipoma would develop in the course of regressive or necrotic degeneration of cortical cells by hormonal disorders, stress, circulatory disturbance or other unknown factors.     

肾上腺髓样脂肪瘤50例报告

目的 探讨肾上腺髓样脂肪瘤的临床表现、实验室检查、影像学及病理学特点与诊治方法.方法 回顾性分析50例肾上腺髓样脂肪瘤患者的临床资料,并结合文献复习,分析其特点.结果 50例患者均经手术切除肿瘤,并经病理检查证实为肾上腺髓样脂肪瘤,随访6~66个月未见复发.结论 肾上腺髓样脂肪瘤术前诊断主要依据超声、CT和MRI等影像学检查,确诊需经病理学检查证实.对于无明显症状、肿瘤体积较小的肾上腺髓样脂肪瘤患者,短期内可随访观察,但手术切除仍是其最有效的治疗方法.     

Adrenal myelolipoma: A case report and review of the literature

A case of a 73 year old man who was preoperatively diagnosed as possibly having adrenal myelolipoma is presented herein. Adrenal myelolipoma is an uncommon benign tumor of which only 74 foreign cases and 43 Japanese cases have been previously reported in the literature dealing with surgically removed myelolipomas. The most consistent complaint of the adrenal myelolipoma sufferer is abdominal pain, caused by hemorrhaging in the tumor, and many cases are associated with obesity, hypertension and/or diabetes mellitus. There is now an increasing number of such cases being diagnosed during abdominal scanning with ultrasonography or computerized tomography for unrelated problems, whereupon the myelolipoma is usually surgically resected. With the availability of modern scanning techniques and fine needle biopsy, however, it should be possible to adopt a more conservative approach to the management of asymptomatic adrenal myelolipoma. Nevertheless, symptomatic or large tumors, must be removed since there is a high risk of spontaneous hemorrhage.     

Retroperitoneal hemorrhage due to spontaneous rupture of adrenal myelolipoma

BACKGROUND: A very rare case of retroperitoneal bleeding due to spontaneous rupture of a large adrenal myelolipoma in a 62-year-old woman is reported. METHODS/RESULTS: She consulted the emergency room of the Nagano Red Cross Hospital with a complaint of sudden left flank pain. A computerized tomography (CT) scan revealed a tumor with areas of fat density and hematoma in the left retroperitoneal space. After her general condition improved, an operation was performed. The tumor strongly adhered to the left kidney and a left nephrectomy with the tumor was curative. Histologic diagnosis was adrenal myelolipoma. No blood transfusion was required. CONCLUSIONS: A CT scan is very useful in the pre-operative diagnosis of adrenal myelolipoma with retroperitoneal hemorrhage. Watch and wait treatments before operation and nephrectomy with adhered tumor are safe and curative.     

A case of adrenal myelolipoma

A case of adrenal myelolipoma is presented. The patient was a 61-year-old woman who complained of lumbago this time. A tumor of the left adrenal gland, however, had been found by computed tomography 4 years earlier. Judging from the CT, the size of this tumor had not changed at all, although the density of the mass on admission had reduced, compared with that of 4 years earlier. Laboratory examinations of adrenal function was normal. Left adrenalectomy was performed. Histologically, this tumor consisted of adipose and some hematopoietic tissue. We reviewed 43 cases of adrenal myelolipoma resected surgically in Japan.     

A case of adrenal myelolipoma

We report a case of surgically resected adrenal myelolipoma. Myelolipoma of the adrenal gland is a rare, benign and nonfunctioning tumor. The present patient represents the 57th reported clinical case of this tumor. It consists of fatty and hematopoietic tissue. It is asymptomatic and usually found only at autopsy incidentally. Ultrasonography, computed tomography and fine needle biopsy help in the preoperative diagnosis of adrenal myelolipoma. Especially fine needle biopsy is recommended when the diagnosis is doubtful.     

Adrenal myelolipoma: report of a case and review of the literature

Adrenal myelolipoma is a rare benign tumor composed of mature lipomatous and hemopoietic tissue. Although it is often discovered as an incidental finding on imaging of the abdomen for some other reason or at autopsy, adrenal myelolipoma has been reported to present with symptoms such as flank pain resulting from tumor bulk, necrosis, or spontaneous retroperitoneal hemorrhage. Myelolipomas are hormonally inactive but have also been reported to coexist with other hormonally active tumors of the adrenal gland. They are usually unilateral but may be bilateral and may also develop in extraadrenal sites like the retroperitoneum, thorax, and pelvis. We report a patient with symptomatic adrenal myelolipoma diagnosed on computed tomography scan and confirmed on computed tomography-guided biopsy. The patient underwent surgical resection for symptomatic relief. We also review the literature to evaluate the presentation and optimal management of this rare adrenal tumor that is not encountered by most general surgeons and therefore not well known to most surgeons.     

Myelolipoma of the adrenal gland: a case report

A case of myelolipoma of the adrenal gland is reported. The patient was a 50-year-old male who was obese and robust, and complained of right flank pain. Laboratory investigation of adrenal functions were within normal levels. Excretory urography showed a large radiolucent mass in the right upper quadrant, displacing the right kidney to a lower position. Angiography demonstrated a large hypovascular mass. CT scan showed a large mass with low density in the right retroperitoneal space. The tumor was surgically removed through a lumbar incision, and was 800 gm in weight and well capsulated. A pathological study of the tumor disclosed adrenal myelolipoma consisting of mature fat cells and myeloid elements. Myelolipoma of the adrenal gland is rare and 33 cases of adrenal myelolipoma from the English and Japanese literature including this case were reviewed and analyzed statistically.     

Laparoscopic treatment of adrenal myelolipoma: case report and review of the literature

Adrenal myelolipoma is a rare benign, non-functioning tumor consisting of fat and hematopoietic tissues. In January 2005 we had observed an adrenal myelolipoma in 70 year old man. During the follow-up for bladder urothelioma, an abdominal CT revaled a well delineated 4x4 cm homogeneous fatty mass in the right suprarenal area with negative attenuation values. The functional study of adrenal gland was normal. The patient underwent videolaparoscopic right adrenalectomy (Gagner technique). Postoperative course was uneventful. The istological diagnosis showed adrenal myelolipoma. We conclude that videolaparoscopic adrenalectomy should be considered the gold standard treatment for benign adrenal lesions.     

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.     

Adrenal myelolipoma: a case report and review of the literature

A case of a 73 year old man who was preoperatively diagnosed as possibly having myelolipoma is presented herein. Adrenal myelolipoma is an uncommon benign tumor of which only 74 foreign cases and 43 Japanese cases have been previously reported in the literature dealing with surgically removed myelolipomas. The most consistent complaint of the adrenal myelolipoma sufferer is abdominal pain, caused by hemorrhaging in the tumor, and many cases are associated with obesity, hypertension and/or diabetes mellitus. There is now an increasing number of such cases being diagnosed during abdominal scanning with ultrasonography or computerized tomography for unrelated problems, whereupon the myelolipoma is usually surgically resected. With the availability of modern scanning techniques and fine needle biopsy, however, it should be possible to adopt a more conservative approach to the management of asymptomatic adrenal myelolipoma. Nevertheless, symptomatic or large tumors, must be removed since there is a high risk of spontaneous hemorrhage.