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.     

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.     

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。     

Aortoesophageal fistula: report of a successfully managed case and review of the literature.

Aortoesophageal fistula is a rare, frequently fatal cause of upper gastrointestinal bleeding. Although several causes have been described, it appears that the most common cause is rupture of a thoracic aortic aneurysm into the esophagus, occurring in approximately 12% of thoracic aneurysm ruptures. Although the entity was originally described in 1818, the diagnosis has rarely been made before death, and until 1983 no one survived surgery for aneurysm-associated aortoesophageal fistulas. Since that report of two cases, only two other survivors, including this patient, have been reported. Included herein is the report of a successfully managed case, along with a discussion of the causes, clinical features, and diagnostic approach to the disease, as well as the management of both the aortic and esophageal components of the fistula.     

Anaplastic seminoma.

The clinical and morphological features of 7 cases of anaplastic seminoma are reviewed. Although this tumor appears to behave more aggressively than classical seminoma, presenting with earlier metastases, it has the same prognosis when staging is taken into consideration. Radiotherapy remains the treatment of choice.     

Phyllodes tumor of the prostate. A case report and review of the literature

Prostatic cystic epithelial-stromal tumors, commonly described as phyllodes tumors of the prostate, are extremely rare lesions. They exhibit a spectrum of histological features similar to their better known counterpart in the breast. Although a benign clinical course has been emphasized in some reports, cumulative evidence in the literature indicates that these lesions should be considered neoplasms rather than atypical hyperplasia due to the frequent early recurrences with possible dedifferentiation, infiltrative growth and potential for extraprostatic spread in some cases. An individualized approach to complete excision of the tumor is needed. A case record of a patient recently treated at our medical center with a low-grade phyllodes tumor is presented and a review of the literature was done.     

A case of retroperitoneal teratoma difficult to distinguish from adrenal myelolipoma

A 59-year-old man presented to our hospital suspected of having cholelithiasis. Computed tomography (CT) scan revealed a left retroperitoneal solid tumor cephalad to the kidney, 7 x 8 x 9 cm in size with mostly a fatty density area and focal calcification. Magnetic resonance imaging (MRI) on T1 and T2 weighted images showed a high intensity mass. Angiography revealed the hypovascular tumor. Although we had suspected it to be an adrenal myelolipoma, tumorectomy was performed because of its size. Pathological diagnosis was mature teratoma. Although retroperitoneal teratoma contains fas, cyst, soft tissue and calcification, the proportion of fat in the tumor is usually less than that of adrenal myelolipoma. In our case, the tumor contained more fat than the 'typical' retroperitoneal teratoma, which led to the misdiagnosis.     

肾上腺髓质脂肪瘤(附九例报告)

目的：提高肾上腺髓质脂肪瘤的诊断与治疗水平。方法：总结9例肾上腺髓质脂肪瘤患者的临床资料,其中右侧7例、左侧2例,均行肿瘤切除术。结果9例术后随访1－15年,均健在,无肿瘤复发。结论本组肾上腺髓质脂肪瘤影像学诊断准确率为100％,手术是治愈本病的唯一方法,但不应以肿瘤直径大小为唯一手术指征。     

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.     

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.     

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.     

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.     

Giant adrenal myelolipoma and testicular interstitial cell tumor in a man with congenital 21-hydroxylase deficiency.

The occurrence of a giant myelolipoma of the adrenal gland reported in a patient with congenital adrenal hyperplasia (21-hydroxylase deficiency). Associated significant findings include a massive proliferation of adrenocortical cells as an integral part of the myelolipoma and coincidental tumor of the interstitial cells of the testis. The clinical, radiologic, endocrinologic, and pathologic features of this case are correlated with a review of the literature. The additional myelolipomas are also reported here for the first time. Similar lesions have been induced experimentally in rats and provide further evidence suggesting a hyperplastic rather than a neoplastic nature for this complex lesion, at least in its earlier stages.     

The glucagon syndrome

Although the features of the glucagonoma syndrome had been described in isolated reports since 1942, this potentially curable condition has only recently been adequately defined. In 1974, MALLINSON collected nine cases and described the association of a peculiar skin rash with glucagon-secreting tumors of the pancreas. The typical skin rash, necrolytic migratory erythema, is accompanied by other disturbances including weight loss, anaemia, glossitis, cheilitis angularis, psychiatric symptoms. Diabetes is not a prominent feature, and the impairment of glucose tolerance can indeed be very slight. Up to 1979, about 50 cases have been reported. More than half of these patients had malignant tumors. The authors report on a 67-year-old man in whom a glucagonoma in the tail of the pancreas has been resected with apparent cure. The almost complete disappearance of the skin rash within three weeks of extirpation of the tumor has been most impressive. In June 1979 a similar tumor has been operated by one of the authors in a 60-year-old man. In this instance the tumor was located in the pancreatic corpus and could be radically resected by left-sided pancreatectomy.     

A study on alpha-fetoprotein and endodermal sinus tumor.

The phenomenon of alpha-fetoprotein production by testicular, ovarian, or sacrococcygeal teratocarcinomas is frequently observed but has not been well explained. This paper includes clinicopathologic studies of 19 cases of teratocarcinoma with positive AFP reactions. Sixteen of the 19 showed typical histologic features of endodermal sinus tumor (yolk sac tumor) of Teilum and one other was compatible with this diagnosis.^6,7 The occurrence of AFP in these tumors is best explained by the concept of endodermal sinus tumor; because it is known that large amounts of AFP are synthesized not only by the fetal liver but also by the yolk sac during early embryonic life, and because the diagnosis of endodermal sinus tumor itself implies that the tumor is of yolk sac origin morphologically.¹³More direct evidence of AFP synthesis was demonstrated by immunofluorescent technique in one of our cases. Immunofluorescence was seen only in that cell layer long defined as of yolk sac origin morphologically.Alpha-fetoprotein studies are valuable in following such patients, unless they are 1 mo of age or younger.     

Soft-tissue myoepithelioma of the hypothenar region: a case report

Myoepithelial tumors only recently have been recognized as occurring primarily in soft tissue, and only a few cases have been described in the literature. The occurrence of these tumors in the hand is even more limited. In this article, we review the clinical, radiologic, and histologic features of a benign myoepithelioma of the hypothenar region of the left hand.     

Etiology and diagnosis of severe retroperitoneal hematoma: therapeutic options and surgical indication

Retroperitoneal hematoma is a rare entity with clinical consequences that vary according to the speed and amount of bleeding. Thus, it may be asymptomatic, in which case it is usually diagnosed incidentally through imaging tests, or severe, in which case the most common symptoms are pain and hypovolemic shock. We report three cases of severe retroperitoneal hematoma. Although the literature on the subject describes a wide variety of causes, in all three patients the etiology was tumoral, consisting of one suprarenal myelolipoma, one renal adenocarcinoma and one suprarenal metastasis from bronchogenic adenocarcinoma. Computed tomography was the imaging test employed not only to determine the nature of the process (hematoma, abscess, tumor) but also to assess compression of adjacent structures and the occurrence of active bleeding. Conservative treatment consisting of volemic replacement and correction of coagulation was initially attempted. However, all three patients required emergency surgery due to hemodynamic instability in two patients and compression of the vena cava in the other.     

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.     

肾上腺髓性脂肪瘤误诊6例报告

目的:提高肾上腺髓性脂肪瘤术前确诊率。方法:分析1983年1月～1998年8月收治的肾上腺髓性脂肪瘤18例中6例误诊的原因,提出避免误诊的措施。结果:6例全部行手术治疗,组织病理报告均为髓性脂肪瘤。结论:肾上腺髓性脂肪瘤无特异的临床表现与特征,提高对该病的认识,通过辅助检查,综合分析,术前能作出正确诊断     

Solitary fibrous tumors of the pleura

Solitary fibrous tumor of the pleura is a mesenchymal tumor that has been increasingly recognized over the past few years. The tumor was initially described in the pleura, but it has been reported in many other sites lately. Although the majority of these tumors have a benign course, the malignant form still remains enigmatic. Indeed, the behavior of these tumors is often unpredictable and does not always correlate with histologic findings. In addition, benign tumors may remain unproblematic for several years before changing into a malignant form. In order to define more precisely the clinical behavior of solitary fibrous tumors of the pleura, we reviewed the literature with particular attention to the clinical presentation, histopathologic characteristics, and cytogenetic differentiation of these tumors. A staging system and an algorithm for the management and follow-up of these patients are proposed.