成人鼻腔腺泡状横纹肌肉瘤一例并文献学习

本文为回顾性研究,经过兰州大学第二医院医学伦理委员会批准(批准文号:2021A-577),免除受试者知情同意. 患者男,39岁,因"左侧持续性鼻塞半年,加重一月"就诊于兰州大学第二医院.患者于入院前半年无明显诱因出现左侧持续性鼻塞,鼻流涕,涕为白色清水样,嗅觉略减退,伴打喷嚏,晨起后加重,无头痛.副鼻窦CT检查示:左侧...     

超声诊断儿童眼眶横纹肌肉瘤并复发1例(附文献复习)

目的：探讨儿童眼眶横纹肌肉瘤超声声像图特点。方法：回顾性分析1例儿童眼眶横纹肌肉瘤及复发的临床资料并复习相关文献。结果：患者女岁6。发现右眼肿物半月余。入院术前超声检查：右眼眶近鼻侧探及大小约3.0cm×1.5cm的实性低回声团块边界尚清,欠规则回声不均匀,右眼球受压向外移位,双眼球内未见明显异常回声。CDFI:团块内探及丰富血流信号,PW可探及低速高阻动脉频谱,PSV:30cm/s,RI:0.8。诊断意见：右眼眶近鼻侧实性肿块考虑横纹肌肉瘤可能性大,泪腺多形性腺瘤(恶变）待排。患儿全麻下行右眼眶肿瘤摘除术,术后病理：(右眼肿物)恶性肿瘤,符合横纹肌肉瘤。结论：儿童眼眶横纹肌肉瘤较少见,其超声表现具有一定的特征性,掌握这些特征有助于超声医师对此病的诊断。     

睾丸旁胚胎性横纹肌肉瘤1例报告并文献复习

目的:提高睾丸旁横纹肌肉瘤诊断与治疗水平。方法:回顾分析收治的1例睾丸旁胚胎性横纹肌肉瘤患者的临床资料,结合文献复习进行讨论。结果:本例患者行经腹股沟睾丸根治性切除术,病灶直径2.5cm,病理检查证实为睾丸旁胚胎性横纹肌肉瘤。术后采用2个疗程辅助放疗,随访6个月,现无瘤生存。结论:睾丸旁横纹肌肉瘤是罕见的男性生殖系统恶性肿瘤,病理上胚胎型最为常见,早期患者应进行原发灶根治性切除和腹膜后淋巴结清扫。     

Metastatic thoracic and lumbar intramedullary and extramedullary Ewing’s sarcoma: a rare case report and literature review

Ewing sarcoma (ES) is a highly aggressive bone and soft tissue tumor that occurs mainly in young children and adolescents and is associated with primary and metastatic disease. Intramedullary ES (either primary or secondary) is rare, and the ideal management remains inconclusive. We herein report intramedullary and extramedullary metastatic ES in a single patient. A 46-year-old woman was referred to our outpatient clinic from the oncology clinic with progressive paraparesis and paresthesia for 1 week prior to presentation. She had developed left clavicular ES 2 years earlier for which surgery and chemoradiotherapy had been performed. At the present evaluation, she was diagnosed with intramedullary thoracic and lumbar extradural masses. Thoracic surgery was performed, and a biopsy of the lesion was obtained. The diagnosis of ES was confirmed histopathologically, and she underwent adjuvant chemotherapy. Her neurological status did not improve after surgery, and she underwent rehabilitation and physical therapy. The lumbar lesion resolved with chemotherapy. Metastasis of ES to the spinal cord, especially intramedullary lesions, is extremely rare, and there is no standard management guideline. However, surgical decompression and adjuvant chemotherapy are the main treatments in these cases.     

Locally advanced cervical rhabdomyosarcoma in adults: A case report

BACKGROUNDRhabdomyosarcoma is a soft tissue tumor of primitive mesenchymal cells origin, occurring predominantly in children and adolescents, but extremely rare in adults and the data regarding its treatment are sparse. Here, we would like to share our experience in the treatment of a locally advanced primary embryonal rhabdomyosarcoma of cervix in a 39-year-old female.CASE SUMMARYThe patient was admitted with symptoms of intermenstrual bleeding and postcoital bleeding for six months. Physical examination revealed a friable, polyp-like mass (5 cm × 5 cm) in her cervix protruding into the vagina, while the uterus was mobile and normal-sized. Colposcopy-directed biopsy was performed, and a pathological diagnosis of embryonal rhabdomyosarcoma was made. Magnetic resonance imaging of the pelvis showed that the cervical volume was significantly increased, with a hypointense and hyperintense soft tissue mass on the right side, invading the cervical stroma; the mass was 5 cm × 5 cm with a clear boundary and confined to the cervix; there were no obvious findings indicating tumor invasion in the vaginal wall, parametrium, or pelvic wall; no enlarged lymph nodes were observed in the pelvic cavity. Based on our findings, the tumor was classified as stage IA according to the intergroup rhabdomyosarcoma studies criteria and IB3 stage according to The International Federation of Gynecology and Obstetrics 2018. The patient underwent two courses of neoadjuvant chemotherapy and a partial remission was achieved. Subsequently, she underwent laparoscopic radical hysterectomy, bilateral salpingo-oophrectomy and pelvic lymph node dissection and there were no risk factors revealed by postoperative pathological examination. Adjuvant chemotherapy was performed after surgery. The patient was disease-free until the last follow-up, 49 mo after completing the entire treatment.CONCLUSIONOur experience suggests that neoadjuvant vincristine, dactinomycin, and cyclophosphamide chemotherapy followed by radical surgery and adjuvant chemotherapy might be reasonable therapeutic option for bulky cervical rhabdomyosarcoma in adults without fertility desire. Since large-scale studies on such rare conditions are rather impossible, further case reports and systematic reviews could help optimize the treatment of primary, bulky cervical rhabdomyosarcoma in adults.     

Primary leiomyosarcoma of the middle ureter: A rare case report with literature review

Leiomyosarcoma is a rarely seen neoplasm of the ureter. Malignant tumors of smooth muscle of the ureter are extremely rare, and about 22 cases of leiomyosarcoma of ureter have been reported to date. A 57‐year‐old diabetic Pakistani man presented with a dull ache pain in the right flank. Past surgical history was three ureteroscopic surgeries for a ureteric stricture. Computed tomography showed a stricture with a peri‐ureteral soft tissue mass of 11 mm x 5 mm at the middle third of the ureter at the level of common iliac vessels. laparoscopic excision with safety margin and right ureterovesical reimplantation is performed. Diagnosis of leiomyosarcoma of the right ureter was made, and one iliac lymph node was excised and was positive for tumor by pathologic examination. Although leiomyosarcoma is rarely seen in urinary tract, it should be considered in the differential diagnosis of ureteral stricture disease and retroperitoneal tumors.     

Embryonic cervical rhabdomyosarcoma complicated with uterine inversion with cerebral venous sinus thrombosis as the first symptom: a case report and literature review

The probability of rhabdomyosarcoma occurring in the cervix is less than 0.5% and may be associated with a pathogenic dicer 1, ribonuclease III (DICER1) gene variation. Tumour-induced hypercoagulability and high levels of cancer antigen (CA) 125 are risk factors for cerebral venous sinus thrombosis (CVST). In addition, although nonpuerperal uterine inversion is very rare and is usually caused by leiomyomas from the uterus, large cervical masses can also be the cause. This case report describes a 24-year-old woman with uterine inversion caused by an embryonic cervical rhabdomyosarcoma that presented with CVST as her first symptom. The patient underwent laparoscopic total uterus and bilateral salpingectomy, during which the uterus was found to be completely inverted. Postoperative pathology confirmed embryonic cervical rhabdomyosarcoma. The patient quickly developed lung and para-aortic lymph node metastases. Two months later, the patient died of complications. When coagulation indices in patients with tumours are abnormal, especially when the levels of D-dimer and CA125 increase, it is recommended that anticoagulant therapy is administered in a timely manner to prevent the occurrence of CVST. Furthermore, for large cervical tumours, physicians should also be alert to the occurrence of uterine inversion.     

Primary retroperitoneal liposarcoma: a rare case report

Primary retroperitoneal liposarcoma (PRPLS) is a rare malignant tumor with a low incidence. A 34-year-old female patient presented to our department with abdominal pain, nausea, and vomiting for 2 days. Abdominal computed tomography (CT) indicated a huge mass between the liver and kidney, with a clear boundary and measuring approximately 202 mm × 155 mm ×106 mm. The mass was considered a retroperitoneal lipoma or liposarcoma. The entire tumor was completely resected without auxiliary injury, and histopathology of the resected specimen indicated liposarcoma. The patient recovered well and was discharged from our department on the 6th postoperative day. No signs of relapse were seen during 1-year of follow-up. PRPLS is rare and without obvious symptoms in the early stage. CT plays a vital role in the diagnosis of PRPLS, and surgical resection is considered the most suitable treatment. Radiotherapy and chemotherapy might also be treatment options to improve the overall survival of PRPLS patients.     

Porocarcinoma of the left arm: A report of a rare skin cancer case and literature review

Porocarcinoma is a rare type of skin cancer that develops from the intraepidermal ductal part of eccrine sweat glands. It can arise de novo or from poroma ground with high potential for locoregional metastatic spread, morbidity, and mortality. It mostly occurs in elderly patients.     

Pleomorphic adenoma of the trachea: A case report and review of the literature

BACKGROUNDPleomorphic adenoma (PA) is the most common benign tumor that occurs in the salivary glands; however, tracheobronchial PA is rarely observed. To the best of our knowledge, fewer than 50 cases have been reported in the literature. We report a 49-year-old woman who had been treated for asthma for 2 years before being diagnosed with PA of the trachea.CASE SUMMARYA 49-year-old woman was referred to our hospital due to dyspnea upon exertion and chronic cough with wheezing for 2 years. Laboratory tests showed an elevated white blood cell count, absolute neutrophil count, and percentage of neutrophils. A chest computerized tomography scan showed a well-defined, soft-tissue density lesion measuring 2.4 cm × 2.1 cm in the lower trachea. Flexible bronchoscopy revealed that nearly 90% of the tracheal lumen was obstructed. The histopathological and immunohistochemistry features suggested PA of the trachea. Furthermore, we review the characteristics of 29 patients with tracheobronchial PA over the last 30 years.CONCLUSIONTracheobronchial PA occurs without gender predominance, mostly in the lower or upper trachea, and has a low recurrence rate. The median age at diagnosis is 48 years. The most common symptoms are cough, stridor, dyspnea, and wheezing.     

伴有横纹肌肉瘤成分的肝细胞癌1例报道并文献复习

目的分析伴有横纹肌肉瘤成分的肝细胞癌的临床及病理组织学特征。方法应用光镜观察及免疫组化染色对1例肝细胞癌伴有横纹肌肉瘤成分的病例进行病理分析，同时复习相关文献。结果肿瘤有明确的肝细胞癌及横纹肌肉瘤成分。免疫组化：肝细胞癌成分CK7( )／(一)，CK8、CK19、hepetocyte、AFP( )；横纹肌肉瘤成分Vim、myoglobin、desmin( )，MyoD1( )／(-)，AFP( )。结论肝细胞癌伴有横纹肌肉瘤成分是一种独特的肝恶性肿瘤，肿瘤中的横纹肌肉瘤成分可能来自肝细胞癌。     

Sarcoidosis during treatment of pulmonary tuberculosis: a rare case report and review of the literature

The coexistence of pulmonary tuberculosis and pulmonary sarcoidosis is rare. Further, the morphological features of pulmonary tuberculosis with comorbid pulmonary sarcoidosis are similar to those of tuberculosis alone. There are obvious clinical, histological, and radiological similarities between sarcoidosis and tuberculosis, which makes differential diagnosis very challenging, particularly in countries with a high burden of tuberculosis. Here, a rare case of computed tomography (CT) findings of sarcoidosis that developed during tuberculosis treatment is reported. The 46-year-old male patient had no significant symptoms and was undergoing treatment for Mycobacterium tuberculosis infection. Chest CT revealed enlargement of multiple lymph nodes, without cystic or necrotic changes, in the mediastinum and both hili, and post-infectious changes consistent with the sequelae of tuberculosis infection in the left upper lobe. Chest radiographic evidence was accompanied by compatible clinical features and noncaseating granulomas on biopsy. As the patient was clinically stable, corticosteroid treatment was not initiated. To date, the patient remains without specific symptoms and outpatient follow-ups continue. Although rare, sarcoidosis may occur during treatment of pulmonary tuberculosis, and requires attention for diagnosis and treatment. The present case draws a radiological picture of how tuberculosis evolved to sarcoidosis.     

肺淋巴管平滑肌瘤病1例并文献复习

对1例肺淋巴管平滑肌瘤病(pulmonary lymphangioleiomyomatosis,PLAM)的病理形态特点及免疫组织化学进行观察分析,并复习相关文献。在PLAM镜下可见肺组织较多囊腔样结构,内衬单层扁平上皮,囊壁、支气管及肺泡周围多灶性平滑肌样梭形细胞增生,正常至轻度异型,肺泡间隔、胸膜增厚。免疫组织化学示:平滑肌样梭形细胞中平滑肌肌动蛋白(smooth muscle actin,SMA)、人类黑色素瘤单克隆抗体(human melanoma black-45,HMB45)、孕激素受体(progestogen receptor,PR)均为阳性表达,雌激素受体(estrogen receptor,ER)弱阳性,囊壁衬附单层扁平上皮D2-40阳性。PLAM是一种持续进展的罕见肺部疾病,病理光学显微镜结合免疫组织化学表型可帮助确诊。     

膀胱原发透明细胞癌1例报告及文献复习

目的 阐明膀胱透明细胞癌病理形态学特征和诊断依据。方法 运用光镜、电镜及免疫组化方法观察分析，结合文献资料进行研究探讨。结果 瘤细胞胞质透明，呈腺管状与实性巢状浸润生长。间质红染的团块状结构。瘤细胞PAS染色阳性，免疫组化CK、EMA及CA125阳性，vimentin、CEA、CgA阴性。电镜见胞质中大量糖原颗粒聚集，并见特征性的天才密电子小体。结论 膀胱原发透明细胞癌是一种少见的膀胱低分化癌，可以来源于苗勒管上皮。     

Occult vaginal cancer recurrence after hysterectomy: a case report and literature review

Vaginal cancer is a rare disease of the lower genital tract. We present the case of a 54-year-old woman with occult vaginal cancer after hysterectomy for cervical intraepithelial neoplasia (CIN) III. Despite persistently negative cytology and colposcopy results, a lesion was finally detected by vagino-recto-abdominal examination and she underwent radical parametrectomy and lymph node dissection. We consider the possibility that transabdominal suturing of the vaginal cuff after hysterectomy may reduce the ability to detect subsequent vaginal lesions, and discuss the benefits of a vaginal suture approach. We recommend that suturing the vagina apex transvaginally instead of transabdominally would benefit patients during follow-up.     

Primary lumbar hernia: A rare case report and a review of the literature

Primary lumbar hernia is a rarely encountered hernia. Surgical correction is the standard treatment for lumbar hernia, and either an open or laparoscopic procedure can be performed with equivalent success. However, open repairs are most commonly performed. Here, we present a case of a primary lumbar hernia that was successfully treated laparoscopically and discuss surgical modalities of treatment reported in the literature. There are two laparoscopic approaches: transabdominal and extraperitoneal. The main advantage of the laparoscopic approach is that it helps the surgeon to precisely locate and evaluate the characteristics of the defect. It also has a lower morbidity rate, shorter length of hospital stay, less postoperative pain, and an earlier return to daily routine activities than the open approach. In the present study, a patient with a superior lumbar hernia was treated laparoscopically by the transabdominal approach. Laparoscopic repair in such cases is feasible and achieves a good result when done by an experienced laparoscopic hernia surgeon.