Idiopathic Sclerosing Orbital Inflammation with Intranasal Extension

Introduction: To report a case of idiopathic orbital sclerosing inflammation (ISOI) with intranasal extension.

Material and Methods: The patient presented with a 6-month history of epiphora, upper eyelid swelling, ptosis and mild orbital pain. Ophthalmologic examination, CT, MRI and biopsy with surgical debulking were performed.

Results: MRI revealed a homogeneously enhancing diffuse right orbital mass in the inferonasal quadrant of the orbit, which extended to the nasal cavity up to inferior nasal concha, maxillary and ethmoid sinuses. Histological analysis showed dense collagenous tissue with sparse infiltration of mixed inflammatory cells. Inmunohistochemical analysis confirmed polyclonality. The diagnosis of idiopathic sclerosing orbital inflammation was made and 80?mg/day of oral prednisolone was prescribed. At last follow up, one year later, there was no clinical evidence of recurrent orbital disease.

Conclusion: ISOI can present with extraorbital extension. Corticosteroids are a reasonable first-line treatment, until the pathogenesis is better understood.     

肺硬化性血管瘤的多层螺旋CT表现及病理对照研究

目的探讨肺硬化性血管瘤（PSH）的多层螺旋CT表现与特点,提高对本病的认识。方法回顾性分析26例经手术或穿刺病理证实的PSH的多层螺旋CT表现。所有病例行胸部多层CT平扫和（或）增强扫描,三维重建后进行图像观察。结果病灶均为单发,右肺12例,左肺14例;大小以1．5-3．0cm多见（65．4％）,平均（25．5±15．4）mm;病灶形态以圆形或类圆形为主（80．8％）,少数呈不规则形;边缘多光滑（88．5％）;多数无分叶（73．1％）;密度多均匀（88．5％）,少数可见钙化（26．9％）;强化可均匀、可不均匀（比例约1：1）,平均增强程度约（41．1±27．4）HU;网格征（76．9％）及贴边血管征（38．4％）较多见。延时扫描表现为持续强化,灌注成像表现为高血流灌注。结论PSH具有肺内良性肿瘤的特点,明显强化、容易出现钙化、网格征、贴边血管征等征象,对诊断PSH具有重要提示意义。     

Sclerosing Sertoli cell tumor without expression of typical sex cord stromal tumor markers: case report and literature review

Sertoli cell tumor is a potential histologic mimic of other tumors, such as seminoma due to similar histology and overlapping clinical presentation. Sclerosing Sertoli cell tumor is a rare sex cord stromal tumor variant, with 16 cases reported in the English literature. We present an unusual case of sclerosing Sertoli cell tumor in a 33-year-old Caucasian male, which was negative or weakly reactive using immunohistochemical markers typically positive in Sertoli cell tumors. The tumor was positive for cytokeratin AE1/AE3, CAM 5.2, vimentin, CD56, CK8, synaptophysin and S100, and negative for inhibin, calretinin, WT1, CD99, CD117, CK5/6, CK7, chromogranin A, placental alkaline phosphatase, neuron specific enolase, D2-40, smooth muscle actin, Melan-A, epithelial membrane antigen and carbonic anhydrase IX. This is the second reported case of a Sertoli cell tumor with reactivity limited to neuroendocrine markers and the first such case of the sclerosing variant. A literature review of sclerosing Sertoli cell tumor, including English and non-English literature, is described. Our case highlights that expected immunohistochemical markers may be negative, and awareness of antigenically unreactive tumors is needed to avoid confusion between Sertoli cell tumor and other entities.     

腹腔化疗后硬化性腹膜炎的营养支持治疗

目的:总结腹腔化疗后硬化性腹膜炎的治疗经验.方法:回顾分析2000年7月至2003年10月收治的7例腹腔化疗后硬化性腹膜炎的临床资料及治疗方法.该组7例病人均采用营养支持等非手术方法进行治疗.结果:所有病人症状均得到缓解,营养状况明显改善,平均治愈时间为(57.14±20.39)天.7例中有5例经肠外和肠内营养支持治疗后痊愈,2例在营养状况好转后手术,治疗原手术并发症(肠瘘)后痊愈出院.结论:采用营养支持等非手术方法治疗腹腔化疗后硬化性腹膜炎,并发症少,安全,疗效满意.     

Autoimmune pancreatitis and cholangitis

Autoimmune pancreatitis(AIP) is part of a systemic fibrosclerotic process characterized by lymphoplasmacytic infiltrate with immunoglobulin G subtype-4(Ig G4) positive cells. It characteristically presents with biliary obstruction due to mass-like swelling of the pancreas. Frequently AIP is accompanied by extra-pancreaticmanifestations including retroperitoneal fibrosis, thyroid disease, and salivary gland involvement. Auto-antibodies, hypergammaglobulemia, and prompt resolution of pancreatic and extrapancreatic findings with steroids signify its autoimmune nature. Refractory cases are responsive to immunomodulators and rituximab. Involvement of the biliary tree, termed IgG 4 associated cholangiopathy, mimics primary sclerosing cholangitis and is challenging to manage. High IgG 4 levels and swelling of the pancreas with a diminutive pancreatic duct are suggestive of autoimmune pancreatitis. Given similarities in presentation but radical differences in management and outcome, differentiation from pancreatic malignancy is of paramount importance. There is controversy regarding the optimal diagnostic criterion and steroid trials to make the diagnosis. Additionally, the retroperitoneal location of the pancreas and requirement for histologic sampling, makes tissue acquisition challenging. Recently, a second type of autoimmune pancreatitis has been recognized with similar clinical presentation and steroid response though different histology, serologic, and extrapancreatic findings.     

Adenocarcinoma in an Ileoanal Pouch Formed for Ulcerative Colitis in a Patient With Primary Sclerosing Cholangitis and a Liver Transplant: Report of a Case and Review of the Literature

This is a report of the first case of adenocarcinoma arising in an ileal pouch after proctocolectomy for ulcerative colitis in a patient who had also undergone orthotopic liver transplantation for primary sclerosing cholangitis. Previously reported cases of adenocarcinoma developing after formation of an ileoanal pouch are reviewed as is the evidence for neoplastic transformation of the ileal mucosa. The risk factors for the development of colorectal cancer in patients with ulcerative colitis and the possibility that these may be risk factors for the development of pouch malignancy are discussed. We conclude that this patient exemplifies a small group of patients who may be at increased risk of developing pouch malignancy and need endoscopic follow-up. There is also the need for longer-term follow-up data to determine the risk of this rare and potentially devastating complication of restorative proctocolectomy.     

Mitotically active cellular luteinized thecoma of the ovary and luteinized thecomatosis associated with sclerosing peritonitis: Case studies,comparison, and review of the literature

In this study, we distinguish two clinical and pathological entities that are similarly named: luteinized thecoma and luteinized thecoma associated with sclerosing peritonitis. Ovarian luteinized thecoma lacks definitive criteria for malignancy. Based on our case study of a mitotically active neoplasm without nuclear atypia in which the patient was living and well 19 years after operation and comparison with prior studies of luteinized thecoma and the closely related entity of cellular fibroma, we propose presumptive criteria for malignancy for this rare neoplasm. Increased mitotic activity in luteinized thecoma without significant nuclear atypia is not an indication of malignant behavior, and such cases should therefore be referred to as mitotically active cellular luteinized thecoma. We also contrast neoplasms in the luteinized thecoma category with the entity originally reported as luteinized thecoma associated with sclerosing peritonitis. In the latter, the ovarian stromal proliferations are typically bilateral, can have an exceedingly high mitotic rate as was seen in our illustrative case, often incorporate non-neoplastic ovarian structures at their periphery, and are responsive to medical therapy. In our patient with sclerosing peritonitis, both the ovarian masses and peritoneal sclerosis underwent complete regression following treatment with gonadotropin-releasing hormone agonist and high doses of steroids, and an ovarian biopsy taken 2 months after therapy showed a histologically normal ovary. The patient subsequently became pregnant and delivered a normal infant. This is, to our knowledge, the first case of successful medically conservative treatment of a young patient with this entity that led to complete relief of symptoms and allowed preservation of fertility. Because recent observations support the non-neoplastic nature of the ovarian stromal proliferations, we advocate use of the previously proposed term luteinized thecomatosis associated with sclerosing peritonitis for this entity.     

Encapsulating peritoneal sclerosis

Encapsulating peritoneal sclerosis(EPS) is a debilitating condition characterized by a fibrocollagenous membrane encasing the small intestine, resulting in recurrent small bowel obstructions. EPS is most commonly associated with long-term peritoneal dialysis, though medications, peritoneal infection, and systemic inflammatory disorders have been implicated. Many cases remain idiopathic. Diagnosis is often delayed given the rarity of the disorder combined with non-specific symptoms and laboratory findings. Although cross-sectional imaging with computed tomography of the abdomen can be suggestive of the disorder, many patients undergo exploratory laparotomy for diagnosis. Mortality approaches 50% one year after diagnosis. Treatment for EPS involves treating the underlying condition or eliminating possible inciting agents(i.e. peritoneal dialysis, medications, infections) and nutritional support, frequently with total parenteral nutrition. EPSspecific treatment depends on the disease stage. In the inflammatory stage, corticosteroids are the treatment of choice, while in the fibrotic stage, tamoxifen may be beneficial. In practice, distinguishing between stages may be difficult and both may be used. Surgical intervention, consisting of peritonectomy and enterolysis, is timeconsuming and high-risk and is reserved for situations in which conservative medical therapy fails in institutions with surgical expertise in this area. Herein we review the available literature of the etiology, pathogenesis, diagnosis, and treatment of this rare, but potentially devastating disease.     

Sclerosing cholangitis associated with autoimmune pancreatitis differs from primary sclerosing cholangitis

AIM： To clarify the characteristic features of biliary lesions in patients with autoimmune pancreatitis （AIP） and compare them with those of primary sclerosing cholangitis （PSC）.METHODS： The clinicopathological characteristics of 34 patients with sclerosing cholangitis （SC） associated with AIP were compared with those of 4 patients with PSC.RESULTS： SC with AIP occurred predominantly in elderly men. Obstructive jaundice was the most frequent initial symptom in SC with AIP. Only SC patients with AIP had elevated serum IgG4 levels, and sclerosing diseases were more frequent in these patients. SC patients with AIP responded well to steroid therapy. Segmental stenosis of the lower bile duct was observed only in SC patients with AIP, but a beaded and prunedtree appearance was detected only in PSC patients.Dense infiltration of IgG4-positive plasma cells was detected in the bile duct wall and the periportal area, as well as in the pancreas, of SC patients with AIP.CONCLUSION： SC with AIP is distinctly different from PSC. The two diseases can be discriminated based on cholangiopancreatographic findings and serum IgG4 levels.     

脾硬化性血管瘤样结节性转化的诊断分析

目的探讨脾硬化性血管瘤样结节性转化(SANT)的影像特征及鉴别诊断。方法分析1例脾SANT病人的MRI表现及临床病理特征,并复习相关文献。结果 MRI示脾的下极孤立性肿物,边界清,平扫T_1WI呈等信号,抑脂T_2WI呈不均匀低信号,DWI呈低信号,动态增强扫描呈渐进性向心性强化,延迟期相对周围脾实质呈稍高信号伴中心放射状低信号影。手术病理为脾SANT。结论脾SANT MRI表现具有一定特征,有助于本病的诊断及鉴别诊断。