子宫囊性淋巴管瘤1例并文献复习

目的 探讨子宫囊性淋巴管瘤的临床病理特征,提高临床医师对子宫囊性淋巴管瘤的认识。方法 分析1例子宫囊性淋巴管瘤的临床表现、辅助检查、病理学特点、鉴别诊断及治疗,并进行相关文献复习。结果 患者,女性,45岁,因“间歇性腹部胀痛2年,发现腹部肿块1年余”入院,结合入院相关检查,术前诊断为子宫肌瘤囊性变,行全子宫切除术。病理检查：子宫体前壁可见一囊性肿瘤,囊液呈淡黄色、清亮,囊内壁光滑,囊腔与子宫腔不通;镜下见囊壁内衬一层被覆单层扁平细胞,囊壁平滑肌间见淋巴网状结构,囊腔内见纤维蛋白物及少许淋巴细胞。免疫组化示D2-40阳性、CKpan弱阳性。术后病理诊断为子宫囊性淋巴管瘤。术后未行特殊治疗,随访6个月未见异常征象。结论 子宫囊性淋巴管瘤少见,临床以阴道不规则流血及盆腔包块多见,影像学无特异性表现,血清肿瘤标志物水平不升高,治疗以手术为主,确诊需要根据术后组织病理学及免疫组化检查。     

肝肉瘤样癌1例并文献复习

目的：探讨肝肉瘤样癌患者的临床及病理学特征、诊断、鉴别诊断、治疗和预后。方法：结合我院收治的肝肉瘤样癌1例临床资料,分析国内外文献报道的该病临床特点、影像学表现、病理学特点,治疗和预后。结果：大部分该病患者均有腹痛和发热的临床表现,病理学上肿瘤都包含明确的恶性上皮性成分和梭形细胞肉瘤样成分且上皮和间叶性两种标记物表达阳性,愈后差。结论：肝肉瘤样癌是一种罕见的恶性肿瘤,恶性程度高,浸润性强,病因不明,目前无有效治疗,预后很差。     

Peripheral primitive neuroectodermal tumor of the posterior mediastinum：A case report

Peripheral primitive neuroectodermal tumor （pPNET） is an extremely rare disease entity of malignant tumors belonging to the Ewing sarcoma family that usually occurs in children and adolescents. We describe a 41-year-old female who presented with right upper abdominal pain. Surgical resection and biopsy revealed small round-cell tumor. Combined with immunohistochemical analysis, pPNET was diagnosed. No evidence of recurrence was noted at 18 months postoperatively. Even thought pPNET is a highly malignant tumor, Wide tumor-free resection and multi-agent chemotherapy can also obtain good clinical outcomes.     

多中心型Castleman病临床病理分析并文献复习

目的:探讨多中心型Castleman病（multiple Castleman disease,MCD）的临床表现、病理学特征、鉴别诊断、病因及治疗,并分析其预后。方法:对9例多中心型Castleman病的临床资料、病理形态学特征进行分析,并行免疫组化检测及相关文献复习。结果:9例多中心型Castleman病例中,透明血管型5例,浆细胞型2例,透明血管浆细胞混合型2例。免疫组化无特征性表现,但可辅助诊断及鉴别诊断。9例多中心型患者均有完整随访资料,经治疗后其中有1例复发,1例死于心脏疾病,其余患者症状均有所缓解。结论:多中心型Castleman病是一种少见的淋巴组织增生性疾病,临床易与其他疾病相混淆,确诊需依靠病理学检查,准确的临床分型和病理类型对患者的治疗和预后具有重要意义。     

Abdominal soft tissue sarcoma: a multicenter retrospective study

Background  

Soft tissue sarcomas (STS) are rare mesenchymal neoplasms with a variety of histological subtypes. However, in Japan, data on the clinical characteristics and prognostic profiles of these tumors are lacking. The purpose of the present study was to clarify the clinical features and outcomes of Japanese patients with retroperitoneal and abdominal STS.     

Metastatic solid-pseudopapillary tumour of the pancreas: clinico-biological correlates and management

Solid-pseudopapillary tumour of the pancreas is a rare neoplasm of young women, currently categorised in the World Health Organization classification under exocrine pancreatic tumours. Increased awareness of this condition correlated recently with an apparent rise in incidence as well as recognition of more aggressive clinical courses. We describe two patients with solid-pseudopapillary tumour of the pancreas. A smaller, localised tumour in an unusually young white man was surgically excised with no evidence of recurrence after 2 years. The other case also had an uncommon presentation, with an aggressive course resulting in vascular encasement of the superior mesenteric bundle and aorta, and local involvement of the mesenteric lymph nodes. A literature review was carried out, and the main clinico-pathological features and strategies of treatment of solid-pseudopapillary tumour of the pancreas are presented. Pathological, genetic and molecular features distinguish solid-pseudopapillary tumours from pancreatic ductal adenocarcinoma. Furthermore, neuroendocrine differentiation can be found focally in occasional cases of solid-pseudopapillary tumour. Patients with localised disease are usually cured by surgery. Prolonged survival can be seen in the presence of distant metastasis, if such lesions are resected surgically. Chemotherapy and radiation therapy are used in rare cases when resection is not possible. No current chemotherapy regimens are considered standard in the treatment of this tumour. A rational chemotherapy protocol for such a rare tumour needs to consider its origin and clinical behaviour. However, the indolent clinical progression of solid-pseudopapillary tumours is similar to that of pancreatic neuroendocrine tumour.     

前腹壁脓肿为首发症状结肠癌的临床策略探讨

  目的  探讨前腹壁脓肿为首发症状的结肠癌的临床病理特点。  方法  报告1例前腹壁脓肿为首发症状的结肠癌的诊治过程,并结合文献分析其临床病理特点。  结果  前腹壁脓肿为首发症状结肠癌男女发病率基本一致,中老年多发,病变部位可分布在全结肠,以右半结肠为主;早期CT和肠镜检查能及早诊断,病理类型上以黏液腺癌及中高分化腺癌为主,生长缓慢,直接浸润周围组织,合并细菌感染形成前腹壁脓肿,较少出现淋巴结转移及远处转移。  结论  联合脓腔的结肠癌整块切除具有潜在根治可能。      

Collecting ducts carcinoma: An orphan disease. Literature overview and future perspectives

Collecting ducts carcinoma (CDC) is a rare and aggressive histological subtype of renal cancer accounting for only 1% of renal tumors. Usually patients present in bad clinical conditions due to a symptomatic disease with synchronous metastasis. Due to the rarity of CDC, data from prospective trials evaluating the best treatment for these patients are limited. The prognosis is poor with a median overall survival of around 11 months for patients with metastatic disease. The best treatment option today is considered a doublet chemotherapy with platinum salt plus gemcitabine as a result from a prospective phase II trial, but survival outcomes remain unsatisfactory.The interest in the in-depth understanding the biology of this orphan disease is growing, leading to find potential new biological-driven treatment approaches. Here we review the up-to-date literature evidences to address the best management of this rare and unfavorable clinical condition.     

Advanced image analytics predicting clinical outcomes in patients with colorectal liver metastases: A systematic review of the literature

BackgroundTo better select patients with colorectal liver metastases (CRLM) for an optimal selection of treatment strategy (i.e. local, systemic or combined treatment) new prognostic models are warranted. In the last decade, radiomics has emerged as a field to create predictive models based on imaging features. This systematic review aims to investigate the current state and potential of radiomics to predict clinical outcomes in patients with CRLM.MethodsA comprehensive literature search was conducted in the electronic databases of PubMed, Embase, and Cochrane Library, according to PRISMA guidelines. Original studies reporting on radiomics predicting clinical outcome in patients diagnosed with CRLM were included. Clinical outcomes were defined as response to systemic treatment, recurrence of disease, and survival (overall, progression-free, disease-free). Primary outcome was the predictive performance of radiomics. A narrative synthesis of the results was made. Methodological quality was assessed using the radiomics quality score.ResultsIn 11 out of 14 included studies, radiomics was predictive for response to treatment, recurrence of disease, survival, or a combination of outcomes. Combining clinical parameters and radiomic features in multivariate modelling often improved the predictive performance. Different types of individual features were found prognostic. Noticeable were the contrary levels of heterogeneous and homogeneous features in patients with good response. The methodological quality as assessed by the radiomics quality score varied considerably between studies.ConclusionRadiomics appears a promising non-invasive method to predict clinical outcome and improve personalized decision-making in patients with CRLM. However, results were contradictory and difficult to compare. Standardized prospective studies are warranted to establish the added value of radiomics in patients with CRLM.     

Cytoreductive surgery and intraperitoneal chemotherapy for peritoneal mesothelioma

Aims

Peritoneal mesothelioma is a rare disease and traditionally has been associated with a gloomy prognosis. The present study aimed to report the outcomes following surgery and intraperitoneal chemotherapy in selected patients with peritoneal mesothelioma.

Methods

Clinicopathological features, operative procedures, early outcomes and survival were analysed for 17 consecutive patients who underwent surgery for peritoneal mesothelioma between 1998 and 2007. Seventeen consecutive patients who underwent surgery for peritoneal mesothelioma between 1998 and 2007 were analysed for clinicopathological features, operative procedures, early outcomes and survival.

Results

Seventeen patients underwent 18 laparotomies. Most presented with abdominal distension (71%) and abdominal pain or discomfort (53%). Complete cytoreduction was achieved in 8 patients, major debulking in 8, and 1 patient had an exploratory laparotomy only due to extensive disease. One patient died on day 30 postoperatively due to a chest infection and pulmonary embolism. The median survival for 8 patients who underwent complete cytoreduction was 3.7 years (range, 0.7–6.9), whereas that for 8 patients with palliative debulking was 1.0 years year (range, 0.3–5.7). Among the 12 patients who had significant ascites as a presenting symptom, 10 reported good palliation of ascites.

Conclusions

Cytoreductive surgery combined with intraperitoneal chemotherapy appears to be the optimal treatment for selected patients with peritoneal mesothelioma. Increased familiarity with this condition's presentation and natural history, and knowledge of available treatment options, will hopefully facilitate treatment of these patients and expedite speedy referral to appropriate treatment centres.     

肾上腺皮质癌1例报道并文献回顾

目的:探讨肾上腺皮质癌的临床表现、诊断及治疗方法。方法:报道我科近期收治的1例肾上腺皮质癌患者的临床资料,并检索中国知网、PubMed等数据库,进行文献回顾,对该病的临床特征及诊断要点、治疗方法及最新的研究进展进行归纳总结。结果:本例患者以腹痛为首发临床表现,影像学提示肾上腺肿瘤,行腹腔镜切除,术后20天查PET/CT提示多发转移,给予依托泊苷+顺铂化疗4周期后进展,遂应用吉西他滨联合卡培他滨化疗1周期,出现脑转移后行放疗。结论:肾上腺皮质癌是一种罕见的泌尿系恶性肿瘤,且预后差。诊断应结合临床表现、肾上腺皮质激素水平、影像学检查及病理结果。治疗方面首推多学科会诊（MDT）,早期患者首选手术,术后需根据疾病分期、分级选择辅助治疗,复发转移的患者可给予化疗、放疗、米托坦、免疫治疗等。     

Presacral Ganglioneuroma: Diagnostic Considerations and Therapeutic Strategy

Presacral ganglioneuroma is an extremely rare tumor of neural crest origin. To the best of our knowledge, less than 20 cases have been reported previously. The present study reports on a presacral ganglioneuroma, 10.5 × 8 × 4 cm in size, that was found incidentally in a 35-year-old man with prior history of diverticulitis. He was admitted to our hospital due to lower left abdominal pain. Abdominal computed tomography and magnetic resonance imaging confirmed the extension of the lesion from the S2 level to the coccyx. The mass had low signal intensity on T1-weighted images and heterogeneous high signal intensity on T2-weighted images with no intraspinal or rectal extension. T2-weighted images demonstrated a compartmentalized solid tumor with cystic components. Complete tumor resection with free surgical margins was achieved using an abdominal approach. The patient remains asymptomatic 2 years after surgery. We emphasize on clinical features, radiologic appearance and surgical treatment of this rare entity. The clinical and pathologic features of previously reported studies are also briefly reviewed.Key Words: Ganglioneuroma, Presacral ganglioneuroma, Retrorectal tumors     

Primary vascular tumours of the kidney

Primary vascular tumours of the kidney are rare and may pose diagnostic difficulties because of their similar clinical, morphological, and immunohistochemical features. This article summarizes the clinical and pathological features of primary renal angiosarcoma and anastomosing haemangioma of the kidney including epidemiology, genetics, and prognosis. Renal anastomosing haemangiomas are benign neoplasms characterized by anastomosing capillary-sized vascular channels. These tumours are rare, with about 75 cases reported in the literature. Most anastomosing haemangiomas are found incidentally on ultrasound, computed tomography, or magnetic resonance imaging. Common symptoms include abdominal pain, haematuria, and abdominal mass. Renal anastomosing haemangiomas are characterized by recurrent mutations in GNAQ and GNA14 genes. The prognosis of anastomosing haemangioma is excellent. Primary renal angiosarcomas are malignant tumours showing endothelial differentiation. To date, 76 cases have been described in the literature. Primary renal angiosarcomas are frequently symptomatic. The clinical features of renal angiosarcomas are similar to those of renal anastomosing haemangiomas, including abdominal pain, haematuria, and abdominal mass. Angiogenesis-related genes and vascular-specific receptor tyrosine kinases such as KDR, TIE1, SNRK, TEK, and FLT1 are upregulated in angiosarcomas. Primary renal angiosarcomas are highly aggressive neoplasms with a poor prognosis despite surgical treatment, chemotherapy, radiotherapy, or targeted therapy.     

Locally advanced anal small cell carcinoma with durable complete response to chemoradiation followed by consolidation chemotherapy: case report and literature review

Extrapulmonary small cell carcinoma (EPSCC) is a rare and aggressive clinical entity that can involve a variety of anatomic locations, including the gastrointestinal tract. Involvement of the gastrointestinal tract is associated with a particularly poor prognosis with patients often presenting with widespread dissemination on initial clinical presentation or rapidly progressing to systemic disease from locoregional involvement. Primary small cell carcinoma of the anal canal is extremely rare, with limited published case reports in the literature. As a result, management of this disease is not well defined, and outcomes are poor with high rates of disease relapse. We report a patient with locally advanced anal small cell carcinoma after presenting with irregular bowel movements, changes in stool caliber, and rectal bleeding for two months and achieved a durable complete response to concurrent chemoradiation with cisplatin and etoposide followed by consolidation chemotherapy and discuss our current understanding of this disease. Specifically, we review the epidemiology, risk factors, clinical course, the treatment strategies over the past two decades, and prognosis for EPSCC. Finally, we conclude our discussion by reviewing the rationale of our treatment regimen and the potential role and benefit of consolidation therapy in the management of this rare and aggressive disease.     

Stereotactic Body Radiation Therapy for Abdominal Oligometastases: A biological and clinical review

ABSTRACT: Advances in imaging and biological targeting have led to the development of stereotactic body radiation therapy (SBRT) as an alternative treatment of extracranial oligometastases. New radiobiological concepts, such as ceramide-induced endothelial apoptosis after hypofractionated high-dose SBRT, and the identification of patients with oligometastatic disease by microRNA expression may yet lead to further developments. Key factors in SBRT are delivery of a high dose per fraction, proper patient positioning, target localisation, and management of breathing-related motion. Our review addresses the radiation doses and schedules used to treat liver, abdominal lymph node (LN) and adrenal gland oligometastases and treatment outcomes. Reported local control (LC) rates for liver and abdominal LN oligometastases are high (median 2-year actuarial LC: 61 -100% for liver oligometastases; 4-year actuarial LC: 68% in a study of abdominal LN oligometastases). Early toxicity is low-to-moderate; late adverse effects are rare. SBRT of adrenal gland oligometastases shows promising results in the case of isolated lesions. In conclusion, properly conducted SBRT procedures are a safe and effective treatment option for abdominal oligometastases.     

Clinical characteristics of struma ovarii

Objective

To evaluate the clinical characteristics of struma ovarii.

Methods

Twenty-five cases of struma ovarii were reviewed retrospectively from June 1994 to April 2007. The presenting clinical, radiologic, and pathologic features of the patients were reviewed.

Results

The mean age of the patients in this study was 45.3 years. The majority was of premenopausal status. Sixteen patients had clinical symptoms such as low abdominal pain, palpable abdominal mass and vaginal bleeding. Although one patient had an abnormal thyroid function test, the laboratory findings normalized after operative treatment. CA-125 levels were elevated in 6 cases. Diagnosis by preoperative imaging studies were 8 dermoid cysts, while only 3 cases were diagnosed as struma ovarii. There were 4 cases of malignant struma ovarii, and no patients with recurrent disease.

Conclusion

Struma ovarii is a rare tumor. The presented clinical, laboratory and radiological findings of patients are very diverse. The diagnosis was confirmed by pathologic findings. The treatment of benign struma ovarii is surgical resection only. The cases of malignant struma ovarii may need adjuvant treatment, but recurrence is uncommon.     

Hepatoid Adenocarcinoma of the Colon: What Should We Target?

Hepatoid adenocarcinoma is a rare extra hepatic neoplasm that displays morphological and phenotypic features similar to those of hepatocellular carcinoma. We report a case of a 75-year-old woman, presenting with abdominal pain and complaints of weakness and lost of appetite, who was found to have a mass on her right colon. She underwent right hemicolectomy for a pT3N2M0, stage IIIC colon cancer. The tumor phenotype and immunophenotype, as documented by alpha-fetoprotein immunoreaction positivity, were consistent with adenocarcinoma of hepatoid origin. The patient received FOLFOX-4 regimen as adjuvant treatment, relapsed after six cycles, then was switched to FOLFIRI regimen plus Bevacizumab and progressed after only four cycles. She died 1 month later, eight months after the diagnosis. The lack of any clinical benefit despite an aggressive and multimodal therapeutic strategy, raises a question about what should be targeted when we face this rare disease associated with a very poor prognosis.     

Experience of surgical treatment of webbed penis in children

The webbed penis (WP) belongs to a rare and little-known defect of the external genitalia. The term denotes the penis of normal for age size hidden in the adjacent scrotal and pubic tissues. The authors describe clinical symptoms of the disease, consider anatomomorphological features underlying development of the WP, propose the WP classification including three basic clinical forms of this malformation, outline principles of diagnosis and differential diagnosis with the conditions associated with a small-size penis, analyze surgical treatment of 30 boys with WP, surgical techniques in each form of the disease with adjustment for anatomic features, provide follow-up outcomes in the treated patients.     

Extranodal non-Hodgkin's lymphoma presenting as an abdominal wall mass. A case report and review of the literature

Soft tissue lymphoma is a very rare clinical entity with varying presentation characteristics and atypical clinical and imaging features. The present report describes a patient who presented with a painless soft tissue mass on the posterolateral surface of the abdominal wall, simulating a neoplasm of mesenchymal origin. After complete surgical excision, the tumor was diagnosed as a diffuse large B-cell lymphoma. No B-symptoms were present and clinical staging did not reveal other sites of disease (stage I EA). The International Prognostic Index score was equal to 1 and classified the patient to the good risk group. Post-operatively the patient was treated with immuno-chemotherapy consisting of rituximab plus cyclophosphamide, epirubicin, vincristine and prednisolone and is currently free of disease for 10 months. The case is discussed with a brief review of the literature on the diagnosis, treatment and outcome of soft tissue lymphomas.