Dumbbell-shaped peripheral primitive neuroectodermal tumor of the spine—case report and review of the literature

Primary spinal peripheral primitive neuroectodermal tumors (pPNETs) are extremely rare. Here, we present a case study of a 29-year-old male with a dumbbell-shaped pPNET at the T9-10 spine level, including details of his examination, surgical procedures applied, histological and genetic findings, and his subsequent treatment. We discuss the clinical course, the pathology and treatment for this disease, the surgical approach to thoracic dumbbell tumors and we review the literature. To our knowledge, this is the first report of a case of a dumbbell-shaped intradural and spinal peripheral PNET.     

Combined pancreatoduodenectomy and orthotopic liver transplantation for hepatocellular carcinoma with portal vein tumor thrombus： A case report

Hepatocellular cancer (HCC) is the most common primary malignant hepatic tumor that accounts for over 80% of primary liver tumors. The outlook for HCC is dismal if it is left untreated and the treatment for patients with HCC evolved into a complex task. The treatments for HCC are mainly surgical therapies including hepatic resection (HR) and liver transplantation. Although HR is a well accepted therapy for HCC, it is not suitable for patients with advanced cirrhosis. Orthotopic liver transplantation (OLT) is considered more appropriate in cases with HCC related to cirrhosis, because it may eliminate both the tumor and the underlying liver disease. In this study, we reported a patient with HCC and portal vein tumor thrombus underwent combined pancreatoduodenectomy with OLT and survived 23 months in our center.     

Prognositic factors and clinicopathologic characteristics of small gastrointestinal stromal tumor of the stomach： a retrospective analysis of 31 cases in one center

Objective： To analyze the clinicopathologic characteristics and prognostic factors of small gastrointestinal stromal tumor （GIST） of the stomach. Methods： A total of 31 small gastric GIST patients, including 10 males and 21 females, with a median age of 58 years （37- 81 years）, who underwent surgery at any time from 1999 to 2012 were included in this study. The clinical records of the patients were analyzed retrospectively. Results： Abdominal discomfort and pain （10 cases, 32.3%, respectively） were the two most common complaints among the patients. All patients received surgery, 11 received gastric wedge resection, 11 received subtotal gastrectom）5 5 received laparoscopic gastric wedge resection, and 4 received endoscopic submucosal dissection. No severe adverse complication was observed. A total of 29 patients （93.5%） were followed up. During the follow-up, 2 patients were found to exhibit tumor recurrence, and 1 patient had liver metastases. One patient died of tumor progressionwhile another died of another malignant tumor. Median progression free survival （PFS） time was 120.3 months, and median overall survival （OS） time was 130.4 months. Conclusion： Small gastric GIST has better prognosis. Surgery is the best choice for therapy. Micro-invasive procedures are safe and effective for elective patients. Tumor necrosis, tumor bleeding, and muscle invasion are potential prognostic factors of small gastric GIST.     

Primary clear cell carcinoma of the nasopharynx： a case report

Primary dear cell carcinoma of the nasopharynx is a rare and locally invasive minor salivary gland neoplasm, only two cases have been reported yet in the literature. Now a 57-year man, with primary nasopharyngeal clear carcinoma,received radiotherapy and chemotherapy. After treatment, the mass of nasopharynx visibly reduced. Follow-up two months and repeated MRI, CT, abdominal B-ultrasonograpy, electronic nasopharyngoscopy, no tumor recurrence or metastasis.     

Collision tumor of the kidney composed of clear cell carcinoma and collecting duct carcinoma：report of a case with unusual morphology and clinical follow-up

We report the case of a 67-year-old female who presented with a large renal mass. Gross examination of the nephrectomy specimen demonstrated a 6-cm renal mass that invaded into the renal sinus and perinephric fat. Histologic examination revealed two distinct tumor types. The first type was a conventional （clear cell） renal cell carcinoma that was of low nuclear grade and comprised the minority of the overall tumor. The second type was a high-grade collecting duct carcinoma with glandular/tubular differentiation and composed the majority of the tumor. Immunohistochemical studies demonstrated distinctive patterns of the two tumor types, thus confirming two distinct lineages. Five months postoperatively, the patient developed metastasis to the lungs and right hilar lymph node region. A fine needle aspiration of a lung nodule demonstrated a metastatic, poorly differentiated carcinoma, similar to the collecting duct carcinoma component in the kidney. Collision tumors of the kidney are rare with fewer than 10 cases reported in the literature. Our report further expands the spectrum of this rare phenomenon.     

Spindle cell carcinoma of the breast as complex cystic lesion： a case report

Spindle cell carcinoma of the breast is a rare tumor. This tumor can proliferate rapidly and cause cystic changes because of internal tissue necrosis. We evaluated a 54-year-old woman with right breast lump. Mammography showed a category four mass with a diameter of 2.5 cm. Ultrasonography（US） revealed a complex cystic lesion, and fine-needle aspiration（FNA） cytology demonstrated bloody fluid and malignant cells. Partial breast resection and sentinel lymph node biopsy were performed. Immunohistology revealed spindle cells with positive results for cytokeratin（AE1/AE3） and vimentin, partially positive results for s-100, and negative results for desmin and α-actin. The pathological stage was IIA, and biochemical characterization showed that the tumor was triple negative. Six courses of FEC-100 chemotherapy（5-fluorouracil 500 mg/m2, epirubicin 100 mg/m2, and cyclophosphamide 500 mg/m2） were administered. Radiotherapy was performed. This case is discussed with reference to the literature.     

Effect of chemotherapy on autoimmune hepatitis in thymoma： a case report and literature review

Autoimmune hepatitis （AIH） has rarely been described as an autoimmune paraneoplastic syndrome ofthymoma. This case is the seventh case of AIH revealed by cholestasis few years after the diagnosis of thymoma and the first case treated with chemotherapy alone. We report in this paper a new approach to this rare severe condition. A 29 year-old man presented with chest pain and dyspnea with a history of thymoma surgically removed 4 years ago. CT scan showed the recurrence of an anterior mediastinal mass. Biology showed elevated liver enzymes and profound cholestasis. No sign of viral or toxic hepatitis or bile duct abnormalities were observed. Autoimmune antibodies, except for the anti-nuclear antibody, were negative. Liver biopsy showed active chronic AIH. The patient was diagnosed with recurrent thymoma with AIH and underwent 6 cycles of chemotherapy. A complete response on thymoma and cholestasis was obtained after 10 months of follow-up. Steroids and immunosuppressors are the standard treatment for AIH. The effect of chemotherapy as a specific treatment of this paraneoplastic syndrome needs to be considered.     

Epithelioid haemangioendothelioma of the lung： one case report with positive pulmonary hilar lymph nodes

Pulmonary epithelioid hemangioendothelioma (PEH) is a rare tumor that occurs among young women and typically presents as bilateral multiple nodules. In this report, we described an unusual case of PEH presented as a single mass in a 46-year-old man, with 6 positive pulmonary hilar lymph nodes. Histologic findings were compared with conventional PEH, except those features such as round to oval-shaped nodules, hypocellular/cellular peripheral zone, hyaline, myxomatous intercellular stroma and intracellular vacuoles, and we found the patient’s pulmonary hilar lymph nodes were metastasis and cholesterol crystal were discovered in the focal infarction areas. Immunohistochemically, the tumor cells were strong positive for CD34, CD31, factor VIII related endothelial antigen and vimentin. The patient died 3 and half months after the surgery without receiving any radiotherapy or chemotherapy. This report may contribute to the data on clinical and histological finding of this rare tumour.     

Neuroendoscopic management of a colloid cyst at the third ventricle using the high-definition flexible neuroendoscopic system： report of two cases

Colloid cysts are benign intracranial lesions located at the anterior roof of the third ventricle, which can result in the obstruction of the foramen of Monro and subsequent hydrocephalus or sudden death. Colloid cysts have been treated by mierosurgical resection or by stereotaetic aspiration. More recently, the use of rigid neuroendoscopy for colloid cysts has been attempted, but the efficacy of the flexible neuroendoscopic system is undetermined except for limited reports.     

Laparoscopic approach for hepatocellular carcinoma： where is the limit？

Hepatocellular cancer （HCC） is the sixth most common malignant tumor worldwide and the most common primary liver cancer （1）. Liver resection or liver transplantation is the therapeutic gold standards in patient with HCC. Due to advanced disease, extrahepatic metastases, or inadequate liver reserve, only 15% to 30% of patients with HCC can undergo to surgery （2）. Five-year risk of recurrence of HCC after resection is as high as 70% because the underlying chronic liver disease continues to put the patient at risk for the development of new cancer nodules （3）. Starting from the assumption that recurrence may be newly treated with surgery, laparoscopic approach is recommended,     

Chylous ascites arises after chemotherapy of gastric signet ring cell carcinoma：A case report and review

Chylous ascites, a rare clinical condition resulting from the disruption of the abdominal lymphatic system, usually diagnosed by paracentesis when the patients suffer ascites as primary symptom. The conditions, in which chylous ascites arise after chemotherapy of solid tumor, are rarely reported. In this paper we present a quite rare case of chylous ascites aris- ing after chemotherapy of gastric signet ring cell carcinoma.     

巨大纵隔原始神经外胚瘤1例并文献复习

原始神经外胚层瘤(primitive neuroectodermal tumor PNET)是一种少见高度恶性软组织肿瘤,好发于儿童及青少年,可发生于神经系统及全身软组织。起源于外周神经系统的被称之为外周性PNET,以躯干、四肢和中轴软组织多见。发生于纵隔的PNET较为罕见。兰州军区兰州总医院普胸外科近来收治1例儿童巨大纵隔PNET,经手术完整切除,较为罕见,现报告如下。1临床资料患儿,男,3岁6个月,因"左胸痛11天,发现胸腔     

The clinicopathological characteristics and surgical treatment of retroperitoneal angiomyolipoma： a case report and literature review of both English and Chinese

Angiomyolipoma usually present as incidental findings on routine imaging or laparotomy, but rarely they may give rise to massive hemorrhage. If bleeding occurs, the treatment of choice is to save life either by angiography with selective embolisation or surgery. Herein, we reported a case of a 32-year-old man who came to our hospital complaining of sudden colicky pain in the right hypochondria region, associated with nausea and vomiting. On physical examination, the patient was in a state of shock; blood pressure of 60/36 mmHg, heart rate of 160 beats/min, high abdominal distention, and non-coagulated blood was extracted from abdominal puncture. The abdominal computed tomography (CT) and arteriography showed a 20 × 15 × 15 cm mass suggestive of spontaneous rupture of liver neoplasm and intraperitoneal hemorrhage. On laparotomy, 1500 mL of non-coagulated blood was found, and the tumor located at right upper abdomen, which originated from retroperitoneum. The tumor was resected totally with hematoma and sutured the bleeding vessels. The histological study of the resected mass revealed the presence of angiomyolipoma.     

Primary Malignant Amelanotic Melanoma in the Female Genital Tract：A Report of Six Cases and a Review of the Literature

OBJECTIVE To analyze the clinical characteristics,pathologic diagnosis,treatment and prognosis of amelanotic melanoma in the female genital tract (AMFGT).METHODS The medical records of 6 patients with AMFGT between 1991 and 2006 in our hospital were reviewed.RESULTS Of these cases,4 were preliminarily misdiagnosed as chorioepithelioma,sarcoma,adenocarcinoma or lymphoma.Two patients were determined to have AMFGT preoperatively after positive immunohistochemical staining for both S-100 protein and HMB-45.Specimens removed from all 6 cases were tested for immunohistochemical staining,as well as H & E histochemical stains.S-100 and vimentin were both positive in all patients,and HMB-45 was positive in 3 out of 5 patients.Four patients recurred (at 6,6,12 and 19 months) after primary treatments.Three patients died (at 13,18 and 19 months) after the initial diagnosis.CONCLUSION Because of an absence of pigmentation AMFGT is extremely difficult to diagnose.Combined immunohistochemical staining,such as the S-100 protein,HMB-45 and vimentin etc,is important in the evaluation of AMFGT.Correct diagnosis plays a crucial role in the treatment of this disease.     

Observation of the Curative Effect of Mabthera in Combination with the CHOP Regimen in Treating Invasive B-Cell Lymphoma： A Report of 45 Cases

OBJECTIVE To observe the clinical effcacy and toxic effects of Mabthera (rituximab) in combination with the CHOP (R-CHOP) regimen for treating invasive B-cell non-Hodgkin’s lymphoma. METHODS A total of 45 patients with CD20 positive B-cell non-Hodgkin’s lymphoma were randomly divided into the R-CHOP (22 cases) and CHOP groups (23 cases for controls).They received the regimens of Mabthera in combination with CHOP or single CHOP therapy respectively.An appraisement of the curative effect could only be performed following 4 cycles of chemotherapy for the 45 patients.Follow-up was conducted to observe the conditions of survival. RESULTS The rate of complete remission(CR)in the R-CHOP group was 68.2%,with a total effective rate of 81.8%,and in the CHOP group these rates were 34.8% and 78.3% respectively.There was a significant difference in comparing the CR rates between the two groups (P<0.05).The 1,2 and 3-year overall survival (OS) rates of the RCHOP group were 90.9%,81.8% and 77.3%,respectively.In the CHOP group,the OS rates were respectively 91.3%,69.5% and 47.8%.The difference in the 3-year OS between the two groups was significant (P<0.05).The toxic effects of the two groups were mainly a slight and moderate bone marrow depression and a gastrointesinal reaction,with similar tolerable toxic effects in the two groups (P>0.05). Adverse effects related to the Mabthera infusions occurred in 6 cases of the R-CHOP group (27.2%).These effects lessened after symptomatic treatment. CONCLUSION The therapeutic regimen of Mabthera,in combination with CHOP (R-CHOP) has an obvious curative effect for treating invasive B-cell non-Hodgkin's lymphoma,with a favorable tolerance.It is highly recommended as the treatment of choice.     

The cardiotoxicity and long-term efficacy of different doses of epirubicin in the adjuvant treatment of breast cancer patients：A case control study

Abstract Objective： The aim of the study was to observe the cardiac toxicity caused by different doses of epirubicin in the adjuvant treatment of breast cancer and to evaluate the long-term efficacy. Methods： The 180 cases of breast cancer patients received epirubicin based adjuvant chemotherapy. The patients were randomly assigned to high-dosage group （90 rag/m^2）, medium-dosage group （70 mg/m^2） and low-dosage group （50 rag/m^2）, the primary endpoint was cardiac toxicity. The secondary outcomes were the 5-year overall survival （OS） and 5-year disease-free survival （DFS）. Results： During chemo- therapy, the clinical symptoms such as palpitation, dyspnea and paroxysmal nocturnal dyspnea occurred in 6 patients with the high-dosage group, 4 patients with the medium-dosage group and 3 patients with the low-dosage group. The number of patients who had changed in electrocardiogram （ECG） was 7, 5 and 4 in three groups, respectively. The echocardiographic showed each group had only one case with LVEF 〈 50%, there was no significantly difference （P 〉 0.05）. In the three groups, the 5-year DFS rates were 73.3% （44/60） in high-dose group, 53.3% （32/60） in medium-dose group and 41.6% （25/60） in low dose group. The 5-year OS rates were 85.0% （51/60）, 68.3% （41/60） and 58.3% （35/60） in three groups, respectively. The differences were statistically significant （P 〈 0.05）. Conclusion： The high-dose epirubicin in adjuvant chemotherapy with CEF （cyclophosphamide, epirubicin and fluorouracil） regimen could improve the 5-year OS rate and 5-year DFS rate on patients of breast cancer. The cardiotoxicity was mild-moderate and well tolerated.