A Giant Lymphatic Cyst of the Adrenal Gland: Report of a Rare Case and Review of the Literature

Lymphatic type of adrenal cysts is most common; however, this type of endothelial cyst is quite rare in excessively large adrenal cysts. A 37-year-old Japanese woman was admitted to our institution with distension of her left flank and the upper quadrant of her abdomen. Abdominal ultrasonography revealed a cystic lesion with a homogenous anechoic texture, and measuring 21 cm in diameter. Computed tomography and magnetic resonance imaging displayed a giant cystic lesion adjacent to the liver, pancreas, kidney, and spleen. The origin of the cyst was not identified. We were not able to make a preoperative diagnosis; therefore, the patient underwent resection of the mass by open laparotomy for therapeutic diagnosis. Intraoperatively, the mass was identified to be cystic and adhered to the left adrenal gland. It was filled with more than 2000 mL of serous brown-red fluid. The content of the cyst contained no atypical cells on cytological examination. The wall of the cyst was composed of a lining of a single layer of lymphatic vessel–derived cells, and the cyst was pathologically classified as a true cyst. No abdominal symptoms were observed and a postoperative radiological work-up showed no evidence of recurrence during a 6-year follow-up period. We describe a case of a patient with a giant lymphatic cyst of the adrenal gland. The preset data suggest that surgeons should decide treatment strategy for large adrenal cysts in consideration of hormonal function, degree of size, and possibility of malignancy.Key words: Giant adrenal cyst, Adrenal gland, Lymphatic cyst, Endothelial cystCystic lesions of the adrenal gland are relatively rare. In 1903, Doran suggested that the first case of an adrenal cyst was described by Greiselius in 1670.¹ In an autopsy series, the incidence of adrenal cysts was reported to be 0.06 to 0.18%.² The actual frequency remains unknown; however, the detection rate of adrenal cysts has risen dramatically due to frequent application of new imaging modalities such as computed tomography (CT) and magnetic resonance imaging (MRI), which accounts for the identification of approximately 5% of incidental adrenal lesions.³ As patients with small adrenal cysts seldom have any symptom, these cysts are rarely recognized. Small adrenal cysts are usually clinically silent and incidental,⁴ whereas large adrenal cysts—especially greater than 10 cm in diameter—are associated with symptoms because of the mass effect and compression of adjacent organs.⁵ Cases of excessively large adrenal cysts have been reported sporadically, only few dozens of adrenal cysts over 20 cm in diameter have been found.^5–28 Moreover, the lymphatic type of endothelial cyst of the adrenal gland is quite rare in excessively large adrenal cysts.^20,22 Here, we report a rare case of a giant lymphatic cyst of the adrenal gland in a middle-aged woman and summarize the current clinicopathological information on excessively large adrenal cysts and lymphatic type of endothelial cysts.     

Intravascular Pyogenic Granuloma Arising in an Acquired Arteriovenous Malformation: Report of a Case and Review of the Literature

BACKGROUND: Pyogenic granuloma, also known as lobular capillary hemangioma, is a benign vascular skin tumor. Intravascular variant of this vascular tumor is rare and such a variant arising in another hemangioma is extremely unusual. OBJECTIVE: The objective was to present a patient with an intravascular pyogenic granuloma arising in an acquired arteriovenous malformation of the palm. METHODS: We report a case and review the literature. RESULT: A 44-year-old woman presented with an easy-bleeding and ulcerative nodule with a hyperkeratotic collaret on her right palm for 3-4 months. The nodule measured 6 x 6 mm in size and was tender. Under the clinical impression of pyogenic granuloma, the lesion was totally excised and the microscopic examination revealed an intravascular pyogenic granuloma in the papillary dermis with an underlying arteriovenous malformation. CONCLUSION: A rare composite vascular tumor is reported, and our case presented as a red eroded nodule surrounded by a keratotic collaret, which was easily misdiagnosed as pyogenic granuloma clinically. We suggest that any pyogenic granuloma-like lesion should be palpated carefully, and if a nodule is present, surgical excision rather than cryotherapy or laser ablation should be considered.     

肾上腺横纹肌肉瘤1例及文献复习

目的：探讨肾上腺横纹肌肉瘤（RMs）的临床特征及诊治疗方法。方法：对1例肾上腺横纹肌肉瘤患者的临床资料进行总结,检索Pubmed和CBM数据库相关文献进行复习讨论。结果：患儿通过手术治疗,术后病理检查为右肾上腺区横纹肌肉瘤,免疫组化：Vim（＋）,Myogenin（＋）,axtin-横（＋）,CgA（-）,Syn（-）,S-100（-）,HHF35（-）,F8（-）,CD34（-）,EMA（-）。结论：肾上腺RMS是非常罕见的恶性肿瘤,其肿瘤结构、细胞形态都有一定的特征,确诊需免疫组化染色的帮助,横纹肌肉瘤的治疗和预后在很大程度上取决于手术切除的彻底性和组织类型。     

Isolated Superficial Sylvian Vein Thrombosis with Long Cord Sign: Case Report and Review of the Literature

Isolated cortical vein thrombosis (ICVT) is extremely rare. Only single case or small series of ICVT have been reported; clinical details are still uncertain. We report a case of isolated superficial sylvian vein thrombosis with exceedingly long cord sign. A 14-year-old female with severe sudden onset headache visited our hospital. Fluid attenuated inversion recovery and echo-planar T₂* susceptibility-weighted imaging (T₂*SW) showed a long cord sign on the surface of the sylvian fissure. The patency of dural sinuses and deep cerebral veins were confirmed by magnetic resonance venography (MRV), and diagnosis of ICVT was made. She recovered completely without anticoagulant agents. To clarify the clinical characteristics of ICVT, we reviewed 51 ICVT cases in the literature. In many cases, T₂*SW was the most useful examination to diagnose ICVT. In contrast with general cerebral venous thrombosis, MRV and conventional angiography were either supporting or useless. Anastomotic cortical veins were involved frequently; symptoms of gyri around the veins were common. It also suggested that ICVTs of the silent area might have been overlooked because of nonspecific symptoms, and more patients with ICVT may exist. In cases involving patients with nonspecific symptoms, the possibility of ICVT should be considered.     

Preputial Calculi: a Case Report and Review of Literature

Preputial calculi are the rarest form of urolithiasis seen usually in an uncircumcised male with phimosis. We report a case in an elderly male who presented with azotaemia. Calculi are evident on clinical examination by palpation. Late presentation by the patient leads to complications. Treatment is by dorsal slit or circumcision. The case highlights the need of early recognition and treatment of this rare condition to prevent any complication.     

Superficial Siderosis of the Central Nervous System Caused by Hemorrhagic Intraventricular Craniopharyngioma: Case Report and Literature Review

Superficial siderosis is a rare condition caused by hemosiderin deposits in the central nervous system (CNS) due to prolonged or recurrent low-grade bleeding into the cerebrospinal fluid (CSF). CNS tumor could be one of the sources of bleeding, both pre- and postoperatively. We report an extremely rare case of superficial siderosis associated with purely third ventricle craniopharyngioma, and review previously reported cases of superficial siderosis associated with CNS tumor. A 69-year-old man presented with headache, unsteady gait, blurred vision, and progressive hearing loss. Brain magnetic resonance (MR) imaging with gadolinium revealed a well enhanced, intraventricular mass in the anterior part of the third ventricle. T₂*-weighted gradient echo (GE) MR imaging revealed a hypointense rim around the brain particularly marked within the depth of the sulci. Superficial siderosis was diagnosed based on these findings. The tumor was diffusely hypointense on T₂*-weighted GE imaging, indicating intratumoral hemorrhage. The lateral ventricles were dilated, suggesting hydrocephalus. [¹⁸F]fluorodeoxyglucose positron emission tomography revealed increased uptake in the tumor. The whole brain surface appeared dark ocher at surgery. Histological examination showed the hemorrhagic tumor was papillary craniopharyngioma. His hearing loss progressed after removal of the tumor. T₂*-weighted GE MR imaging demonstrated not only superficial siderosis but also diffuse intratumoral hemorrhage in the tumor. Superficial siderosis and its related symptoms, including hearing loss, should be considered in patients with hemorrhagic tumor related to the CSF space. Purely third ventricle craniopharyngioma rarely has hemorrhagic character, which could cause superficial siderosis and progressive hearing loss.     

Madelung Disease: Report of a Case and Review of the Literature

Madelung disease is a rare disorder characterized by the presence of multiple, symmetric, nonencapsulated fatty accumulations diffusely involving the cheeks, the neck, the upper trunk, the shoulder girdle area, and the upper extremities. The cause of this syndrome is unknown, but it has been associated with alcoholism in 60% to 90% of patients. The long-term lipomatous deposits are often large and cosmetically deforming, and the upper aerodigestive tract and great veins may be compressed. We report the case of a man with MD, involving the cervical and upper dorsal regions, who underwent surgical treatment at our Department.     

Neurinoma of the Greater Superficial Petrosal Nerve Developed Acute Facial Palsy: Case Report and Review of the Literature

The authors report on a patient with a greater superficial petrosal nerve neurinoma. The patient developed severe acute facial palsy and the palsy improved soon after removal of the tumor. We review of the literature and describe its rarity and surgical treatment with regard to other facial nerve neurinomas originating from the main trunk of the facial nerve.     

Traumatic Hemipelvectomy: A Case Report and a Review of the Literature

Abstract Traumatic hemipelvectomy is a life threatening, devastating pelvic injury with very low survival rates. Excessive loss of blood, related systemic problems and additional injuries about the gastrointestinal and genitourinary systems increase mortality of this severe sort of injury. A young, male farmer injured violently by an agricultural heavy machine; a survivor of traumatic hemipelvectomy is described in this case report.     

Transmesosigmoid Hernia: Report of a Case and Review of the Literature

We report a case of a transmesosigmoid hernia in a 6 weeks postpartum woman. We found 14 previous reports of this rare type of internal hernia. Our patient presented with acute abdominal pain and developed a small intestinal obstruction. History, clinical and radiographic examination were not diagnostic. An early laparoscopy was performed and a herniation of a small intestine loop through a hole in the sigmoid mesocolon was seen. The hernia was reduced and the defect in the sigmoid mesocolon was closed laparoscopically. The small intestine was viable and enterectomy could be avoided. The role of laparoscopy and potential causes of this type of hernia are discussed.     

Sinonasal Hemangiopericytoma: Case Report and Literature Review

Hemangiopericytoma (HPC) is a rare vascular tumor that can arise in any organ system, but occurs most frequently in skeletal muscle. We present a case of a primary sphenoid sinus HPC unusual for its spontaneous remission after biopsy. There have been approximately 55 cases of sinonasal HPC reported in the literature, representing less than 5% of all lesions. In general, HPC behaves aggressively, demonstrating greater than 50% local recurrence and 10% metastatic disease. Although extended surgical resection is traditionally considered the most effective therapy for all HPC, critical literature review does not support this method of treatment for HPC occurring in the paranasal sinuses and skull base. Long-term follow-up indicates that the majority of sinonasal HPC have a benign clinical course regardless of treatment. As a result, we do not recommend extended resection as the initial therapy for sinonasal HPC. Instead, a period of observation coupled with serial MRI or CT scans should be used to detect tumor progression. These indolent tumors should undergo extended resection only after progression has been confirmed. In addition to the case report, a synopsis of the reviewed literature and a summary of treatment recommendations are also presented.     

Shone’s综合征一例报告及文献复习

目的探讨Shone’s综合征的一般规律、治疗策略及其对预后的影响因素,为临床诊治提供借鉴。方法 2009年7月中山大学附属第一医院收治1例主动脉缩窄合并先天性二尖瓣狭窄患者,女,年龄12岁。属于广义的Shone’s综合征,包括主动脉缩窄、动脉导管未闭、二尖瓣瓣上环、二尖瓣狭窄等畸形。采用体外循环、胸骨正中切口,切除主动脉狭窄段,剪开二尖瓣下与乳头肌粘连的腱索,充分松解两组乳头肌,剪除瓣上纤维环。计算机检索PubMed（1963~2009年）、Elsevier Science（1963~2009年）、Wiley Online Library（1963~2009年）、Ovid（1963~2009年）数据库,收集有关Shone’s综合征的临床研究（前瞻性或回顾性）、病例报告和综述,分析其诊治特点。结果本例患者手术时间350 min,体外循环时间156 min,主动脉阻断时间48 min。手术矫治了所有畸形。患者术后2 d拔除气管内插管,3 d转出监护室,心肺功能恢复好;术后第7 d复查超声心动图提示：降主动脉与肺动脉间未见血流相通,降主动脉处未见狭窄和血流障碍,二尖瓣瓣口面积1.9 cm2,肺动脉压降至28 mm Hg;术后2周顺利出院。经检索共纳入19篇文献：其中典型Shone’s综合征回顾性研究5篇,包括112例患者;有关先天性二尖瓣狭窄或左心室流出道狭窄的临床研究14篇。各文献报道的病例类型各异,对手术策略基本达成共识：尽量矫正所有畸形。结论 Shone’s综合征一经诊断,则应手术治疗,左心室流入道梗阻矫正是影响患者预后的重要因素。     

Spinal Angiolipoma: Case Report and Literature Review

Abstract

Background/Objective: Spinal angiolipoma (SAL) is an uncommon clinico-pathological entity.

Design: Single case report.

Methods: Retrospective data analysis.

Findings: An obese woman with a 1-year history of progressive spastic paraparesis and acute deterioration underwent magnetic resonance imaging of the thoracic spine, the results of which suggested a tumor compressing the thoracic spinal cord. The histopathological examination of the completely resected tumor revealed an epidural angiolipoma.

Conclusions: This case report offers a reminder that SAL should be considered in the differential diagnosis of long-standing, slowly progressive paraparesis. It remains unclear whether an increased body mass index might be a contributing factor to the development of SAL.     

Treatment of a Large Superficial Basal Cell Carcinoma with 5% Imiquimod: A Case Report and Review of the Literature

BACKGROUND: Large superficial basal cell carcinomas (BCCs) may be difficult to treat surgically due to the potentially large resulting defect after removal. Imiquimod, an immune response modifier, when applied topically, has been demonstrated to be successful in treating superficial BCCs. There is no published experience on the treatment of larger superficial BCCs (greater than 2 cms2) with imiquimod at this time. OBJECTIVE: To assess the clinical and histologic regression, as well as the short- and long-term effects of topical imiquimod on large superficial BCC. METHOD: A 52-year-old white female with a 30 cm2 biopsy proven superficial BCC of 28 years duration on the right dorsal arm was treated with 5% imiquimod three times a week for 12 weeks. Clinical follow-up was conducted 1, 4, 6, and 10 months after treatment, as well as histologic assessment of recurrence at 4 months after treatment. RESULTS: Apparent clinical and histologic clearance was achieved. The treatment was well-tolerated and the patient completed the treatment schedule without a rest period, despite erosion of the lesion in the last week of treatment. CONCLUSION: This report describes the first case of a large superficial BCC (30 cm2) successfully treated with topical imiquimod and with a 10 month follow-up.     

肾髓质癌1例报告及文献复习

目的:探讨起源于肾盏上皮的肾髓质癌,分析其临床,病理形态学,影像学,鉴别诊断及分子生物学特征,以提高该病的诊疗水平。方法:对1例肾髓质癌的诊治过程进行回顾性分析,结合国内外文献对本病的临床,病理学,影像学,鉴别诊断及分子生物学等特征以及治疗和预后进行总结分析。结果:本病好发于中青年,伴有镰状细胞病或镰状红细胞特征是其主要特点。与其他肾肿瘤相比影像学上没有明显的特异表现。CK、Vim和EMA均(+)有助于同肾集合管癌相鉴别。拓扑异构酶2α有望成为肾髓质癌治疗的新靶点。结论:肾髓质癌是一种少见的侵袭性恶性肿瘤,影像学上无特异性,确诊主要靠病理诊断,主要应与肾集合管癌鉴别。探索该病的新靶点,尽量早期诊断治疗是提高其疗效的关键。     

Pleomorphic Lipoma: Case Report and Literature Review

BACKGROUND: Pleomorphic lipoma is a rare, benign, pseudosarcomatous, soft tissue neoplasm typically involving the subcutis of the neck and shoulder in middle-aged to elderly men. It is characterized histologically by atypical, multinucleated giant cells and grossly as a well-circumscribed mass. Since this neoplasm can resemble a sarcoma, histopathologic diagnosis is critical in preventing unnecessary surgery. OBJECTIVE: To describe a case of pleomorphic lipoma in the neck and to review the clinical and histopathologic characteristics of this neoplasm. METHODS: We present a case report and review of the literature. RESULTS: Local excision to completely extirpate this neoplasm has proven curative at 10 months of follow-up. CONCLUSION: Pleomorphic lipoma is superficially located and may be encountered in a dermatologic setting. Thus the dermatologist should become familiar with its clinical presentation and microscopic appearance.