神经-皮肤黑色素沉着序列症1例

神经 皮肤黑色素沉着序列症 ,是外胚层黑色素细胞先天性发育异常所致的全身多处巨大黑色素痣、斑和脑脊膜黑色素细胞增生浸润产生的一种临床综合征[5] 。自 186 1年报告以来 ,查阅近十年国外文献报告尸解证实 40例 ,国内仅 4例。本病极为罕见 ,现将我院 1例报告如下。病例　患儿 ,男 ,98年 11月 18日于我院产科剖宫产娩出 ,孕 39周 ,羊水清 ,体重 32 80ｇ ,出生阿氏评分 ,1分钟、5分钟均评 10分 ,出生时即见皮肤有大片黑褐色色素痣。其母30岁 ,硕士毕业 ,医生 ;父 34岁 ,大学毕业 ,工程师。双亲在孕前、孕时均未接触有害物质 ,母在孕后期患…     

瘤样钙质沉着症病理及免疫组化研究

瘤样钙质沉着症系病因不明的罕见病症,Duret于1899年论述,称内皮钙化。1943年Inlan命名瘤样钙质沉着症。文献可查者不过数十例。我院1987年10月～1991年3月收治2例。例1,男,53岁。右大腿外上方肿块,渐增大伴胀痛及腰酸痛3年。体检:右股外上方可及15cm×10cm×5cm肿块,境界清楚,轻度压痛,能推移,稍有囊性感,腰椎明显后凸畸形,活动受限。X线片示右股外上方软组织内有18cm×10cm×10cm块影,内见钙化影,     

肺泡蛋白沉着症1例

肺泡蛋白沉着症１例陈新，王仪，唐秀如，魏霖患者男，５０岁。２个月前受凉后出现咳嗽、咳痰、气促症状。痰量少，灰白色，体温３７．５～３８℃。随着气促逐渐加剧，出现进行性呼吸困难、紫绀明显、无寒战、高热咯血。胸片示：双肺弥漫性病变。痰液细菌培养阴性。当地医...     

中枢神经系统黑色素细胞瘤临床病理分析

目的：探讨中枢神经系统黑色素细胞瘤的临床病理特征。方法：对1例发生于脊髓的黑色素细胞瘤的临床表现、组织形态、免疫组织化学等进行分析,并复习相关文献。结果：患者女性,52岁,右侧肢体活动不灵活9个月。MRI示颈3、4椎体水平见一软圆形界限清楚肿块,大小约3.7 cm ×0.8 cm ×1.3 cm。镜检示肿瘤细胞排列呈巢团状、漩涡状及束状;瘤细胞呈软圆形或梭形,部分细胞呈上皮样,大部分细胞质内见多量黑色素颗粒;瘤细胞无明显异型,无核分裂像。免疫组织化学Vimentin、HMB45、S-100、Melan-A均为阳性,Ki67指数5%~10%;其余Ckpan、EMA、GFAP、CD34、SMA、Desmin和P53均为阴性。结论：黑色素细胞瘤是一种罕见的低度恶性中间型肿瘤,临床上易与弥漫性黑色素细胞增多症和黑色素瘤病、恶性黑色素瘤以及伴有黑色素分化的其他肿瘤混淆。其诊断主要靠病理形态学特征、免疫组化、影像学及电镜检查有助于诊断与鉴别诊断。治疗主要依靠肿块切除,必要时可局部放疗,术后随访非常重要。     

胆囊胆固醇沉着症手术122例

本文对胆囊胆固醇沉着症作临床及手术观察,女多于男,男女比1∶6.2,临床特点为右上腹痛,恶心、呕吐,少数畏寒、发热、黄疸等。术中常见胆囊肿大或正常,少数萎缩,多数伴有慢性胆囊炎、胆石症,胆囊切除术效果良好。     

脑脊膜黑色素细胞瘤的临床病理分析

目的：探讨脑脊膜黑色素细胞瘤的临床病理特征、组织发生及预后。方法：报道2例罕见的椎管内软脊膜黑色素细胞瘤（0.05％），并结合国内外文献中报道的58例脑脊膜黑色素细胞瘤进行讨论。结果：2例均为妇性，分别为14岁和46岁，肿瘤均发生于脊髓软脊膜，大体上呈黑色，包膜完整。光镜下见瘤细胞由上皮样细胞和梭形细胞混合而成，细胞无异型，大部分瘤细胞质内含有多量黑色素颗粒，核分裂象无偶见（0－1个／10HPF），无出血坏死。免疫表型HMB45和S－100蛋白呈阳性表达。1例颈髓单发者全切除术后生存14个月，无肿瘤复发，另1例开始为颈髓单发性，本切除术后12年复发，为颈、胸髓多发性，再次行肿瘤部分切除术。结论：脑脊膜黑色素细胞瘤生物学行为相对良性，好发于后颅凹及脊髓的软脑脊膜，具有明显的临床和组织学特征。免疫组化和电镜检查有助于本瘤与其他黑色素性肿瘤的鉴别诊断早期完全切除有望治愈或减少复发，术后放疗对控制复发并无意义。     

18例儿童特发性肺含铁血黄素沉着症的临床分析

目的 探讨儿童特发性肺含铁血黄素沉着症（IPH）的临床特点。方法 结合以前相关研究对确诊为IPH的18例患儿的临床表现、治疗及预后进行分析。结果 IPH好发于学龄前儿童,主要症状为面色苍白、咳嗽及咯血等,主要体征为呼吸音增粗。糖皮质激素治疗能有效改善患儿的症状,减轻肺部病变。结论 IPH临床表现多样,易被漏诊,长疗程激素治疗是本病治疗的重要方法。     

脑脊膜黑色素细胞瘤的临床与病理学观察

脑脊膜黑色素细胞瘤为中枢神经系统 (ＣＮＳ)非常罕见的相对良性的肿瘤 ,其发病率是ＣＮＳ肿瘤的 0 .0 6 %～1% [1] 。以往大多数病例作为恶性黑色素瘤、黑色素性脑膜瘤及黑色素性神经鞘瘤被报道[2 ,3 ] 。ＣＮＳ不同的黑色素性肿瘤的生物学行为和治疗是不相同的 ,因此作出正确的病理诊断尤为重要。作者报道 2例椎管内软脊膜黑色素细胞瘤 ,占我院同期ＣＮＳ肿瘤的 0 .0 5 %。一、病例资料例 1　女 ,14岁。因四肢乏力 ,行走不稳 ,双手持物困难5个月 ,进行性加重于 1999年 7月 14日入院。体检 :感觉平面障碍位于Ｔ1平面 ,双侧上下肢肌力Ⅳ级。…     

A histogenetic consideration of ovarian mucinous tumors based on an analysis of lesions associated with teratomas or Brenner tumors

To Investigate the histogenesis of ovarian mucinous tumors, clinicopathologic and histologic studies of mucinous tumors associated with teratomas or Brenner tumors were performed. Of 458 ovarian mucinous tumors, 13 (2.8%) and 8 (1.7%) were associated wlth teratomas or Brenner tumors, respectively. Of patients younger than 40 years, 5.1% had teratomas. Of patients 50 years or older, 3.5% had Brenner tumors. Histologically, nine lesions with teratomas and six lesions with Brenner tumors showed transitions or intimate admixtures of both eiements. The findings of the present study suggest that teratomas and Brenner tumors make small contributions to the histogenesis of ovarian mucinous tumors but may give rise to some mucinous tumors, especially in younger patients and older patients, respectively.     

Peritoneal melanosis combined with serous cystadenoma of the ovary: a case report and literature review

Benign peritoneal melanosis is extremely rare and traditionally occurs in association with ovarian dermoid cysts, but rarely with peritoneal cyst, enteric duplication cyst or gastric triplication. The pathogenesis of peritoneal melanosis, in particular, the origin of the pigment-producing cells is unclear. We describe a case of peritoneal melanosis that was associated with ovarian serous cystadenoma in a young woman. Ovarian serous cystadenoma has not been previously described as a combined lesion of peritoneal melanosis. Based on the extremely rare incidence of this lesion and heterogeneous combined lesions, the possibility of an incidentally found, coexisting lesion couldn't be excluded. Here, we suggest that peritoneal mesothelial cells pinched off during the developmental period might be a source of pigment-producing cells.     

Peritoneal washing in borderline epithelial ovarian tumors in women under 25: The use of cell block preparations

Serous tumors of low malignant potential are uncommon in women under 30-years-old peritoneal washings play an important role in the diagnosis and prognosis of ovarian neoplasms. Accurate diagnosis of peritoneal washings is important in determining therapeutic regimens and in determining patient prognosis. In peritoneal washings, these tumors can be difficult to distinguish from reactive benign mesothelial cells. The cases of four women with borderline serous ovarian tumors, all of whom were under the age of 25, and had peritoneal washings which were positive for papillary tumors were reviewed. Diagn. Cytopathol. 1998:212– 214. © 1998 Wiley-Liss, Inc.     

卵巢粘液性肿瘤组织发生的初步探讨

目的：初步探讨卵巢粘液性肿瘤（ＯＭＴ）的组织发生。方法：用组织化学染色方法观察９１例ＯＭＴ上皮的粘液分泌。按粘液性质将上皮分为３型：胃型、肠型和中间型。并将肿瘤分为简单型及混合型。结果：良性５６例中,４５例为混合型,其中２２例由３种上皮成份组成,２３例含２种上皮,另有１１例为简单型。中间型、胃型及肠型３种上皮在良性肿瘤中的出现频率分别是４０／５６、３５／５６、２９／５６。２１例交界性、１４例恶性Ｏ     

Case report: ultrastructure of mature neurogenic implants from ovarian immature teratoma

An ultrastructural study of mature (grade O) neurogenic peritoneal implants from an ovarian immature teratoma grade I is presented. The presence of different types of neuroectodermal cells challenges the current terminology of this lesion, which implies a single proliferating cell type. The ultrastructural features were indicative of maturity in all cell lines and even the neurons exhibited well formed synapses. Marked cytoplasmic fibrillary change, similar to that found in central nervous system gliosis, was observed in the astrocytes. The ependymal cells had abnormal cilia with an altered configuration and number of microtubules.     

Squamous cell carcinoma arising in a mature cystic ovarian teratoma with bladder invasion: a case report

Background

Malignant transformation in a mature cystic ovarian teratoma is rare. Except in cases with high index of suspicion or overt metastasis, oophorectomy is the mainstay of treatment for ovarian teratoma.

Method

A 46-year-old perimenopausal woman who had salpingo-oophorectomy following a clinical diagnosis of benign ovarian tumour that was subsequently reported histologically as mature cystic ovarian teratoma with malignant transformation is presented.

Results

She was referred to our facility based on the histopathology report and haematuria two weeks after surgery. Cystoscopic biopsy done was reported as metastatic squamous cell carcinoma most probably from the ovary. Patient was thereafter referred for radiotherapy but was lost to follow-up after the first course.

Conclusion

Adequate evaluation prior to surgery in suspected ovarian teratoma with malignant transformation is critical to determine extent of surgery and adjuvant therapy. Prognosis in advanced disease condition such as the case presented is generally poor although radical pelvic surgery with resection of the adjacent involved bladder before radiotherapy would probably have improved her prognosis.     

Glioblastoma multiforme in a mature ovarian teratoma with recurring brain tumours

AIMS: We report a case study to highlight the occurrence of glioblastoma multiforme in an ovarian teratoma. METHODS AND RESULTS: A 10-year-old girl presented with a left frontal lobe primitive neuroectodermal tumour which was successfully treated. After 6 uneventful years, she developed glioblastoma multiforme located posterior to the site of the initial tumour. Six years later, she presented with a mature cystic teratoma containing glioblastoma multiforme. CONCLUSIONS: Glioblastoma in an ovarian teratoma is an exceptional event, which might have an initial clinical presentation as a metastatic brain tumour. Alternatively, recurring glial tumours may occur in a genetically predisposed person; the role of radiation and chemotherapy in this context remains to be elucidated.     

Anti-N-methyl-D-aspartate receptor encephalitis with occult ovarian teratoma: a case report

A 31-year-old female was admitted with headache, memory disturbance, abnormal behavior, incontinence, confusion, complex partial seizures, decreased oxygen saturation and increased temperature. Anti-NMDAR antibodies were positive in serum and cerebrospinal fluid. Subsequently, a regimen of immunotherapy that included intravenous immunoglobulins, methylprednisolone, plasma exchange and their combinations were used. But the treatment was ineffective. Though both transvaginal ultrasonography and abdominal CT scan contrast revealed left ovarian cyst, the patient had left oophorectomy. And during surgery we found a small cyst mass contained fat-like liquid with air in her left ovarian. Pathological examination demonstrated mature cystic teratoma accompanied with brain tissue. She has made gradual and steady improvement after surgery, but not fully recovery. By combining this case with previous studies of others, we further discuss the clinical characteristics, treatment and prognosis of the disease.