卵巢原发原始神经外胚层肿瘤4例临床分析并文献复习

原始神经外胚层肿瘤(primitive neuroectodermal tumor,PNET)分为中枢型和外周型,外周型PNET属于尤文肉瘤家族肿瘤,其发生是由于基因调控失常,原始干细胞向神经上皮各不同阶段分化,成为形态上类似于儿童中枢神经系统的肿瘤,其组织学及临床生物学行为类似于骨外的尤文肉瘤。卵巢原发PNET为外周型PNET,属于卵巢单胚层畸胎瘤,极为罕见,疾病早期即可发生转移、容易复发,预后极差。其临床表现缺乏特异性,患者常以腹痛、腹胀、盆腔包块就诊,病理是诊断的金标准。目前无明确诊治指南,国内外相关文献多为病例报道,1975—2017年英文文献报道的原发于卵巢的PNET仅17例。本文报道4例我院诊治的卵巢原发PNET,同时复习相关文献,描述此种罕见疾病的临床表现、治疗方法及预后情况。     

卵巢原发原始神经外胚层肿瘤诊治进展

卵巢原发原始神经外胚层肿瘤(PNETs)是向原始神经方向分化的小圆细胞恶性肿瘤,临床罕见,侵袭性强、且死亡率高,病理是诊断的金标准。目前无明确诊治指南,文献多为个案报道,其治疗方案常借鉴尤文氏肉瘤家族肿瘤,多采用根治性切除手术联合全身性化疗和(或)放疗的综合方案。卵巢原发PNETs在首次就诊时常表现出转移,临床预后极差,生存期从10.8个月到3年不等。     

外阴原始神经外胚层肿瘤一例报告及文献复习

原始神经外胚层肿瘤(primitive neuroectodermal tumor,PNET)是一种小圆细胞恶性肿瘤,属于尤文肿瘤家族,临床上较为少见且恶性度极高,预后不良.虽然近年来PNET的病例报道逐渐增加,但原发于外阴的PNET报道较少.中南大学湘稚医院2010年1月收治了1例外阴PNET患者,本文结合国内外有关PNET及外阴PNET的文献,对本病的临床病理特点、诊断、治疗及预后进行初步探讨.     

子宫颈原始神经外胚层肿瘤两例临床分析

外周原始神经外胚层肿瘤(peripheral primitiveneuroectodermal tumor,pPNET)是一类向神经方向分化、高度恶性的小圆细胞肿瘤。虽然近年来该肿瘤的报道逐渐增多,但临床仍较少见,其占成人软组织肉瘤的1％,儿童软组织恶性肿瘤的4％[1]。pPNET可发生于全身各个部位,发生于宫颈的原始神经外胚层肿瘤十分罕见,1987-2005年间英文文献先后报道了10例宫颈原始神经外胚层肿瘤,近年来国内也见到宫颈原始神经外胚层肿瘤的个例报道。2009年中国医学科学院肿瘤医院收治2例宫颈原始神经外胚层肿瘤,均经临床和病理检查证实。现结合临床表现、病理学特点、治疗预后情况并复习有关文献报道如下。     

宫颈原始神经外胚层肿瘤一例及文献复习

为探讨宫颈原始神经外胚层肿瘤[primitive neuroectodermal tumor,PNET;又称尤文氏肉瘤（Ewing sarcoma）]的组织起源、临床特征及诊治进展,提高对该种特殊类型宫颈恶性肿瘤的认识,回顾性分析2016年1月吉林大学第二医院收治的1例PNET患者,该患者体检发现宫颈肿物,无特殊临床表现,经手术病理及免疫组织化学（免疫组化）染色确诊,免疫组化显示人白细胞分化抗原99（cluster of differentiation 99,CD99）、波形蛋白（vimentin,VIM）阳性。提示该类肿瘤极为罕见,生长迅速、恶性程度高、侵袭性强、死亡率高、预后极差,目前尚无标准治疗方案,诊断主要依据病理、免疫组化及细胞遗传学。     

宫颈原始神经外胚层肿瘤一例及文献复习

为探讨宫颈原始神经外胚层肿瘤[primitive neuroectodermal tumor,PNET;又称尤文氏肉瘤(Ewing sarcoma)]的组织起源、临床特征及诊治进展,提高对该种特殊类型宫颈恶性肿瘤的认识,回顾性分析2016年1月吉林大学第二医院收治的1例PNET患者,该患者体检发现宫颈肿物,无特殊临床表现,经手术病理及免疫组织化学(免疫组化)染色确诊,免疫组化显示人白细胞分化抗原99(cluster of differentiation 99,CD99)、波形蛋白(vimentin,VIM)阳性。提示该类肿瘤极为罕见,生长迅速、恶性程度高、侵袭性强、死亡率高、预后极差,目前尚无标准治疗方案,诊断主要依据病理、免疫组化及细胞遗传学。     

阴道尤文肉瘤1例

阴道尤文肉瘤罕见, 术前缺乏特异性的临床表现, 易误诊。本文报道1例2018年9月就诊于本院的阴道尤文肉瘤患者, 从其术前影像学检查、术中情况及手术方案、病理诊断等方面阐述诊治经过, 并参考相关文献研究进行讨论。阴道尤文肉瘤的好发年龄为30～39岁, 临床症状不典型, 可表现为无痛性阴道肿块、阴道溢液;盆腔彩超表现为阴道实质性肿块, 磁共振成像(MRI)检查可表现为阴道内混杂横向弛豫时间(T2)信号肿块;病理诊断是关键, 手术完整切除病灶并辅以术后放化疗是预防阴道尤文肉瘤复发的主要方式。     

阴道壁原始神经外胚层肿瘤1例及文献复习

原始神经外胚层肿瘤（primitive neuroectdermal tumor,PNET）少见,发生于阴道壁者则更罕见,国内鲜见报道,我们收治1例,现结合文献复习报告如下.     

宫颈未分化子宫肉瘤1例报告

<正>宫颈肉瘤发病率低,预后不良,文献少有报道,而原发于宫颈的未分化子宫肉瘤更是罕见报道~([1])。该病临床表现主要是异常阴道流血、宫颈肿物、盆腹腔包块及其引起的压迫症状。病理诊断是确诊的金标准。手术治疗是主要的治疗方式,术后可辅助放化疗。青岛市市立医院近期诊治1     

妇科原发性原始神经外胚层瘤4例病理诊断与临床治疗

目的:探讨原发于生殖器官的原始神经外胚层瘤患者的临床表现、病理特点、治疗方法及预后。方法:回顾分析北京协和医院2005年内收治的原发于妇科的原始神经外胚层瘤患者病例资料。结果:4例均经过病理诊断确诊。1例来源于卵巢的原始神经外胚层瘤,2例来源于子宫(宫颈和圆韧带),1例来源于外阴。其中1例接受了化疗。1例肿瘤进展非常迅速,术后15天死亡。3例治疗后于随诊中。结论:病理及免疫组化有助于对原始神经外胚层瘤的确诊。原始神经外胚层瘤可以发生在生殖器官的任何部位,对于早期患者手术是首选的治疗方法,晚期患者可以辅助性地行联合化疗,目前原始神经外胚层瘤妇科少见,对预后尚没有评估依据。     

Primary metastasized extraskeletal Ewing sarcoma of the vulva: report of a case and review of the literature

Purpose  

Ewing’s sarcoma (ES) and primitive neuroectodermal tumor (PNET) are closely related malignant neoplasms, usually affecting the skeletal system. Extraosseous ES/PNETs are uncommon, with occasional reports of tumors affecting the genitourinary tract. Only few cases of primary vulvar Ewing’s sarcoma/PNET have previously been reported.     

Primary ovarian Burkitt’s lymphoma: a rare oncological problem in gynaecology: a review of literature

Purpose

This review presents the information about epidemiology, clinical manifestation, diagnosis and treatment of primary ovarian Burkitt’s lymphoma (BL), including a literature search of available BL cases. The purpose of this review is to draw clinicians’ attention to the possibility of ovarian BL occurrence, which may be important in the differential diagnosis of ovarian tumours.

Methods

PubMed and Web of Science databases were searched using the keywords ‘‘Burkitt’s’’, ‘‘Lymphoma’’, ‘‘Ovarian’’, ‘‘Primary’’, ‘‘Burkitt’s lymphoma’’. Only cases with histopathologically confirmed diagnosis of primary ovarian BL were included in this review.

Results

Fifty articles, reporting cases with an ovarian manifestation of primary non-Hodgkin’s lymphoma, were found. Twenty-one cases with a histopathologically confirmed BL were evaluated to compare various manifestations, treatment and prognosis in ovarian BL.

Conclusions

Primary ovarian BL is a rare condition, included in the entity of non-Hodgkin lymphoma. The tumour can occur uni- or bilaterally in the ovaries with major symptoms such as abdominal pain or a large abdominal mass. Differential diagnosis, based on imaging features and pathological examination of the specimens, is essential for further treatment due to various aetiology of ovarian tumours. Although most of the patients suffering from ovarian BL underwent surgery after the ovarian tumour had been detected, surgical treatment is not the treatment of choice in patients with ovarian lymphoma. The mainstay of therapy is chemotherapy without further surgery. The prognosis is better if the chemotherapy protocol is more aggressive and followed by prophylactic central nervous system chemotherapy. Nowadays, multiagent protocols are administered, which improves the survival rate.

     

The use of Palma''s procedure in the salvage therapy for a leiomyosarcoma of the right pelvic sidewall: an intraoperative multidisciplinary approach

Leiomyosarcomas of the ovary and broad ligament are relatively rare. Less than 50 cases of primary ovarian and broad ligament leiomyosarcomas have been reported. The prognosis is poor, with late-stage disease being a problem in the majority of cases. It is difficult to determine the exact role of surgery, chemotherapy, and radiotherapy in the management of these tumors. So far, no cases of ovarian or broad ligament leiomyosarcomas have been reported where primary surgery for the sarcoma had to be combined with a Palma's procedure. We report a case of a right pelvic sidewall leiomyosarcoma with involvement of 10 cm of the right external iliac vein. A review of the imaging preoperatively did not suggest involvement of the venous system. Resection of the pelvic mass was carried out and a Palma's procedure was performed by the vascular surgeon. Histology reported an incompletely excised high-grade leiomyosarcoma. Chemotherapy was given postoperatively. Imaging at 12 months after surgery showed a patent vascular graft and no evidence of recurrence. There was no clinical evidence of recurrence at 21 months postsurgery.     

子宫肉瘤研究进展

子宫肉瘤是发生于女性生殖器官的恶性肿瘤，其发病率低、预后差，主要有异常阴道出血、腹痛、腹部包块等临床表现。血清乳酸脱氢酶、血清中性粒细胞与淋巴细胞比值（NLR）等血清学指标及超声和磁共振成像等影像学检查可协助子宫肉瘤的术前诊断，但目前其诊断仍主要依靠术后病理结果。子宫肉瘤主要有子宫平滑肌肉瘤、子宫内膜间质肉瘤及子宫腺肉瘤3种病理类型。全子宫及双附件切除是标准手术方式，术后可根据不同病理类型及手术分期选择辅助治疗方式。通过查阅国内外文献，综述子宫肉瘤的临床表现、辅助检查、病理特征、治疗及预后等。     

子宫静脉内平滑肌瘤临床病理分析

目的 探讨子宫静脉内平滑肌瘤的临床生物学特征及治疗手段对预后的影响。方法 对北京协和医院和江西省妇幼保健院1992年6月至2003年6月间收治并经手术及病理检查证实的子宫静脉内平滑肌瘤7例患者的临床和病理资料进行回顾性分析,全部病例均随访。结果 临床表现：7例患者均以盆腔包块为主诉,其中3例盆腔包块超过妊娠12周子宫大小,最大者妊娠16周大小;2例表现为经期延长、经量增多,1例表现为绝经后出血。超声检查：4例提示子宫肌瘤,1例提示子宫肉瘤,2例提示卵巢肿瘤,术前确诊率为0。术中冰冻检查：2例中1例确诊。手术治疗：4例行全子宫及双侧附件切除术,2例行全子宫及一侧附件切除术,1例仅行肌瘤剔除术;1例行子宫及一侧附件切除术患者术后2年复发,瘤栓沿下腔静脉转移至右心房及右心室再次手术治疗。结论 子宫静脉内平滑肌瘤具有特殊的不良生物学行为,提高术中确诊率与正确选择手术方式及术后密切随诊可以改善其预后。     

A case of malignant lymphoma of the ovary manifesting like an advanced ovarian cancer

BACKGROUND: In recent years, true primary ovarian lymphoma has been considered to carry a favorable prognosis, although most studies of supposedly primary ovarian lymphoma have reported a poor outcome. CASE: A 47-year-old woman presented with signs and symptoms suggestive of an advanced ovarian cancer. Ultrasonography and magnetic resonance imaging revealed bilateral abdominal tumors, each measuring 10 cm in diameter, thickened omentum, and a large amount of ascitic fluid, but no enlarged lymph nodes. The diagnosis of malignant lymphoma was established from the biopsy specimen after exploratory laparotomy. Six years following chemotherapy, the patient is alive and disease free without additional surgery. CONCLUSION: The prognosis of ovarian lymphoma was evaluated according to clinical stage, modality of onset, histologic type, and phenotype. It remains controversial whether this case can be considered truly primary ovarian lymphoma and not merely a localized initial manifestation of a generalized disease. But if this case of advanced ovarian lymphoma were not primary, it could still be managed successfully with chemotherapy appropriate for the specific histology.     

Pseudomyxoma peritonei usually originates from the appendix: a review of the evidence

Pseudomyxoma peritonei (PMP) is a rare condition, said to be more common in females during the fourth or fifth decade of life with an incidence believed to be in the region of one per million per year. Although PMP has been reported as originating from many intra-abdominal organs, in the majority of cases an ovarian or appendix cystadenoma or cystadenocarcinoma has been implicated as the primary site. Our experience suggests that most cases arise from the appendix. We have reviewed the clinical and scientific evidence. In the four largest reported series of 393 patients, 181 (46%) were males. Immunohistochemistry techniques in women with both appendical and ovarian tumours favour an appendiceal primary in most cases. The distinction between "benign" adenomucinosis and mucinous adenocarcinoma is important in both treatment and prognosis. Experience suggests that there may well be a spectrum of disease and possibly an "adenoma carcinoma sequence".     

Primary ovarian angiosarcoma--review of the literature and report of a case with coexisting chylothorax

BACKGROUND: Primary ovarian angiosarcoma is a very rare gynaecologic malignancy with poor prognosis and uncertain, up-to-date, treatment options. Its exact diagnosis is challenging for surgeons and difficult for pathologists. There are only a few cases reported in the international literature. CASE: We report a case of primary pure ovarian angiosarcoma with coexisting chylothorax which is, to the best of our knowledge, the first reported case. An extensive review of the literature analyzing all clinical and pathological parameters related to this condition is presented. RESULT: In spite of all therapeutic efforts, surgical and medical, prognosis of ovarian angiosarcoma remains very poor in most cases. CONCLUSION: Primary ovarian angiosarcoma is a rare and aggressive malignancy. The report of such cases is interesting in order to exchange knowledge and experience, and possibly to further improve our diagnostic and therapeutic capabilities.     

Synchronous primary neoplasms of the uterine corpus and the ovary: a case report

OBJECTIVE: To determine the frequency of synchronous primary neoplasia of the ovaries in patients with primary malignant neoplasia of the uterus, and to analyze the clinical and histological characteristics of these cases. MATERIALS AND METHODS: Clinicopathological data from a series of patients treated for primary malignant neoplasia of the uterus between 1985 and November 2003 have been studied retrospectively. RESULTS: Synchronous primary neoplasia of the ovaries was found in 13 out of 173 patients (7.5%) treated for primary malignant neoplasia of the uterus. In four patients (2.3%) the histological findings suggested ovarian metastases from primary endometrial adenocarcinoma. In four other cases (2.3%) there was extension of the primary uterine sarcoma to the ovaries. In the remaining five cases (2.9%) primary endometrial adenocarcinoma coexisted with: a) ovarian cystadenocarcinoma in two cases, b) ovarian fibromathecoma in two cases, and c) ovarian tumor of borderline malignancy in one case. CONCLUSIONS: Coexistence of distinct primary neoplasias in the uterus and ovaries is rare. Diagnosis of two primary malignancies in the uterus and ovaries should be based on histological examination. Treatment should be appropriate for both tumors, taking into consideration that treatment of one tumor will not lead to subtreatment of the other.     

Breast metastasis two years after pelvic surgery and adjuvant chemotherapy for serous ovarian cancer

Ovarian cancer is considered the leading cause of death among gynecologic neoplasias. Breast metastases from primary ovarian cancer have been reported in only 0.03–0.6% of all breast cancers. A 38-year-old female, multipara, affected by advanced ovarian cancer and undergone a total abdominal hysterectomy with bilateral salpingo-oophorectomy, omentectomy, and pelvic peritonectomy two years earlier, was discovered to have a breast metastasis. She underwent a quadrantectomy (QSI) with an open biopsy of nodularity attached to the pectoral muscle fascia: on definitive histological characterization the breast lesions showed a high-grade invasive carcinoma, with papillary serum differentiation, and the immunohistochemistry study of breast lesion showed positivity for cytokeratin 7 and an extensive positivity for Wilm’s tumor (WT)-1 and paired box 8 (PAX8). These aspects are indicative of mammary metastasis from carcinoma of ovarian origin. Despite their rarity, metastases to breast from ovarian cancer should be considered possible because their recognition and differentiation compared to primary tumors are crucial for prognosis. Future clinical studies on MOCB should be performed to discover new specific markers for a more accurate histopathological diagnosis and to establish the real need of surgical treatment.