睾丸原发性神经内分泌癌1例报告并文献复习

目的：探讨睾丸原发性神经内分泌癌的起源、诊断要点和治疗方法,提高对该疾病的认识。方法：回顾性分析我院收治的1例睾丸原发性神经内分泌癌患者的资料,并复习有关文献。结果：患者左侧睾丸有一无痛肿块,质硬。彩超扫描证实左侧睾丸内存在一2．7cmX2．3crux3．1cm低回声实性团块,包膜完整,界限清楚,肿块内血流信号丰富;腹膜后、腹股沟彩超检查未见肿大淋巴结。血清肿瘤标记物pHCG、CEA和AFP未见异常。手术完整切除肿瘤,术后病检提示为睾丸神经内分泌癌。病变组织起源于神经内分泌细胞,癌细胞类圆形,胞质伊红染,核圆形,颗粒状,癌细胞呈巢状或岛状排列。癌细胞弥漫性表达突触素、嗜铬颗粒A和细胞角蛋白。结论：睾丸原发性神经内分泌癌罕见,临床表现缺乏特异性,组织学特点和免疫组化标记物是诊断该病的主要依据,需与转移性类癌和混合性类癌相鉴别。早期诊断并手术效果良好,术后应密切随访预防复发和转移。     

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目的探讨原发性腹膜后内胚窦瘤的治疗和预后方法回顾性分析1991～2002年收治的11例碍发性腹膜后内胚窦瘤．结果该组病例占同期收治恶性原发性腹膜后肿瘤的8.8％(1l／125)和内胚窦瘤的20.3％(1l／54)。术前B超、CT或MR1检查发现腹膜后肿瘤。术前血清甲胎蛋白升高5例全组手术切除全部或大部分肿瘤。术后用PVH(顺铂、长春新碱、博菜霉素)或BEP方案(博菜霉素、足叶乙甙、顺铂)化疗随访8个月至8年，生存10例，死亡1例。结论以顺铂为基础的联合化疗改善了内胚窦瘤的疗效，测定血清AFP确助内胚窦瘤的诊治，应选择恰当的手术方式并辅以联合化疗来救治病人     

Granular Cell Tumor as an Unusual Cause of Obstruction at the Hepatic Hilum: Report of a Case

A diagnosis of malignancy is reasonably assumed when a lesion is found at the hilum or bile ducts in a patient with jaundice who has never undergone biliary surgery. Although benign tumors occasionally develop in this location, preoperative recognition is difficult and most are treated as malignant lesions. We illustrate this clinical scenario in this case report of a granular cell tumor (GCT) that developed at the biliary bifurcation, necessitating right hemi-hepatectomy with extrahepatic biliary tree excision. We describe the clinical presentation, imaging findings, treatment, and histological findings of this tumor. Although rare, a GCT can develop at the hilum and mimic a malignant lesion such as cholangiocarcinoma (CC) radiologically. To our knowledge, this is the fourth report of a GCT at the hilum of the liver. However, the possibility of this tumor should be considered in the differential diagnosis of a lesion in this location.     

Teratoid Wilms' tumor: case report of a rare variant that can mimic aggressive biology during chemotherapy

Teratoid Wilms' tumor is considered by some as a variant of Wilms' tumor containing at least 50% heterologous differentiated tissue. Fewer than 30 cases have been described. We report a 9-month-old boy with bilateral Wilms' tumors who did not respond to multiagent chemotherapy and underwent right nephrectomy that showed a teratoid Wilms' tumor. The patient continued to survive despite cessation of treatment. The overall predominance of differentiated stromal elements in this subtype of Wilms' tumor might explain the poor response to chemotherapy yet generally favorable outcome. Recognition of this subtype on biopsy might justify earlier surgical intervention that, for bilateral tumors, might allow for greater nephron sparing.     

A solid and papillary (solid-cystic) tumor of the pancreas occurring in a 36-year-old man: Report of a case

A case of a solid and papillary (solid-cystic) tumor of the pancreas occurring in a 36-year-old man is herein reported. This patient was admitted with left hypochondrial pain and diagnostic imaging detected a large tumor consisting of both solid and cystic components in the tail of the pancreas. A distal pancreatectomy with splenectomy was performed. The resected tumor was diagnosed as a solid and papillary tumor of the pancreas by pathological examination. Detailed immunohistochemical studies showed that the tumor consisted of cells both exocrine and endocrine in character. Thus, this case appears interesting from the point of cellular differentiation in solid and papillary tumors, since they are generally considered to have an acinar cell origin.     

Plexiform fibrohistiocytic tumor of the hand: a case with late recurrence

A case of a rare and recently described neoplasm of the superficial soft tissue that occurs mainly in children and young adults is described. The lesion demonstrates morphological features reminiscent of both fibrous histiocytoma and fibromatosis. Microscopically, this tumor is characterized as a multinodular or plexiform proliferation of histiocyte- and fibroblast-like cells associated with multinuclear giant cells. Because of its unpredictable biological behavior, high recurrence rate, and potential for regional lymphatic metastasis, the diagnosis of plexiform fibrohistiocytic tumor raises important treatment considerations.     

Primitive neuroectodermal tumor of the adrenal gland

We report a rare case of primitive neuroectodermal tumor arising from adrenal gland in adulthood, diagnosed preoperatively as having non-functional adrenocortical adenoma. Laparoscopic adrenalectomy was performed. Immunohistological examination revealed the definite diagnosis as primitive neuroectodermal tumor of the adrenal gland. Although primitive neuroectodermal tumor is a highly malignant neoplasm, there is no evidence of local recurrence and distant metastasis 16 months after surgery.     

A rare case report of an adenomatoid odontogenic tumor associated with odontoma in the maxilla

IntroductionThe adenomatoid odontogenic tumor (AOT) associated with odontoma has been described as a new entity with overlapping features of adenomatoid-like structures and odontoma. Presentation of the case: We report the second case of AOT associated with odontoma in the posterior maxillary region. A 22-year-old patient presented expansion of the vestibular and palatine cortical wall of maxilla. Radiographic examination and computed tomography confirmed erosion of maxillary walls, root resorption, displacement of a neighboring tooth and radiopacities within the lesion. Histopathological analysis showed a cystic cavity lined by odontogenic epithelium, organized as duct like structures and tubular dentin. The diagnosis established was AOT associated with odontoma.DiscussionAs fewer cases have been described, the aim of this study is to report clinical behavior and evolution of adenomatoid odontogenic tumor associated with odontoma, as it have not yet been described and its origin is not completely established. The hamartomatous or neoplastic origin of this tumor is under debate.ConclusionsTherefore, it is necessary similar cases to be published to increase the knowledge about the clinical behavior and evolution of this tumor, to enable such lesions to be more clearly defined in the next classification of odontogenic tumors.     

Malignant triton tumor of the gluteal region in a patient unaffected by neurofibromatosis: A case report

Malignant triton tumor (MTT) is a rare variant of malignant peripheral nerve sheath tumor (MPNST) made up of both malignant schwannoma cells and malignant rhabdomyoblasts.A 26-years-old male patient was admitted with an asymptomatic gluteal mass. Magnetic resonance imaging showed heterogeneous soft tissue mass and he underwent open biopsy. Malignant peripheral nerve sheath tumor was diagnosed. He was given adjuvant chemotherapy following the removal of the tumor with hip disarticulation. The tumor was diagnosed as “malignant triton tumor” based on pathological examination including immunohistochemical studies. There were no signs of metastasis but recurrence was observed at 9 months follow up.MTT is usually associated with Neurofibromatosis 1 and located in head, neck region. In this case sporadic involvement of gluteal region and aggressive behavior of the lesion despite radical surgery was demonstrated.     

Primitive neuroectodermal tumor of the kidney: A rare entity

Primitive neuroectodermal tumor (PNET) is usually aggressive and rapidlyprogressing and metastasizing tumor. Occurrence of the this type of tumorin the kidney is considered as unusual, and few cases have been reportedso far. We present a PNET arising from the kidney in a 23-year-old femalepatient.     

脊髓肿瘤合并腰椎间盘突出症的临床诊疗体会：附5例报告

脊髓肿瘤合并腰椎间盘突出症的临床诊疗体会（附５例报告）于晓华，张玉发，刘军脊髓肿瘤与腰椎间盘突出症二种疾病同时存在的病例较少，但误、漏诊率却较高，近年来这方面的文献报告有所增加。我院在这方面也有一些经验教训，本文报告典型病例，分析诊断失误的原因及提高...     

Papillary glioneuronal tumor: case report and review of literature

Papillary glioneuronal tumor (PGNT) is a recently described central nervous system neoplasm that mostly occurs in the supratentorial system, adjacent to the lateral ventricles.In 2007, WHO classified PGNT as grade I neuronal-glial tumor because of the characteristic papillary architecture and bipartite (astrocytic and neuronal/neurocytic) cell population. As a newly established entity of mixed glioneuronal tumor family, PGNT attracted extensive attention recently.In our report we discuss the clinical, neuroradiological and surgical features. The final result is compared with literature data.     

膀胱孤立性纤维瘤1例报告并文献复习

目的:探讨膀胱孤立性纤维瘤(SFT)的临床病理特征、诊断及治疗方法。方法:回顾性分析1例膀胱SFT患者的的临床资料,临床及影像学诊断为膀胱肿瘤,在腰麻下行经尿道膀胱肿瘤电切术,术后出院。1个月后再次来我院复查,在全麻下行经腹腹腔镜下膀胱肿瘤部分切除术。结果:病理检查提示为梭形细胞肿瘤,免疫组织化学检查倾向于SFT。患者腹腔镜术后痊愈出院。结论:膀胱SFT是一种罕见肿瘤,其诊断主要依靠病理学及免疫组织化学检查,手术是其主要治疗方法。     

Contralateral breast cancer: factors associated with stage and size at presentation

Few reports have evaluated factors associated with the stage at presentation of contralateral breast cancer and whether contralateral cancer presentation has changed in recent years, during which increased screening and enhanced adjuvant therapy of the initial tumor has been introduced. Patients with initial cancers staged 0-IIIB diagnosed between 1980 and 1999 who subsequently developed contralateral breast cancer were evaluated for stage, primary tumor size and histologic features, patient age and year of diagnosis, synchronous or metachronous presentation, and time from initial tumor to diagnosis of contralateral breast cancer. Tumor presentation was compared with contemporaneously diagnosed unilateral breast cancer patients. A total of 171 patients were diagnosed with contralateral breast cancer; 161 cases if lobular carcinoma in situ for either the initial or the contralateral tumor were excluded. Contralateral tumors were of significantly smaller size and of earlier stage than initial tumors. Contralateral cancers demonstrated earlier stage distributions with more recent year of diagnosis; initial tumors in contralateral patients did not. Earlier stage at presentation of contralateral tumors was associated in a multivariate model with more recent year of contralateral tumor diagnosis and earlier stage at presentation of the initial tumor; contralateral tumor size was associated with the size of the initial tumor. More recently diagnosed contralateral breast cancers are presenting with earlier stages of disease. The stages of initial tumors in these patients have not undergone such an improvement. Initial tumor size and stage is strongly associated with contralateral breast cancer size and stage; this association is independent of the year of diagnosis. Increased compliance with screening might be expected to improve both initial tumor stage and tumor size presentations in contralateral tumor patients, as well as the presentation of contralateral cancers.     

原发性附睾肿瘤(附32例报告)

目的为了提高对原发性附睾肿瘤的认识。方法回顾性总结已治疗的32例原发性附睾肿瘤。结果其中良性30例，恶性2例，行肿瘤切除16例，附睾切除11例，患侧睾丸附睾切除3例，患侧根治性切除加腹膜后淋巴清除术2例。术前确诊仅为11例，余均经手术后病理确诊。结论原发性附睾肿瘤术前明确诊断困难，治疗有怀疑附睾肿瘤者应首选手术探查，良性者可行肿瘤或附睾切除，如为恶性则需行根治性睾丸切除加腹膜后淋巴清除术。     

Mixed granulosa-Sertoli sex cord-stromal tumor of the testis: clinical,ultrasonic and histopathological features: a case report

Testicular sex cord-stromal tumors are less common in men, while mixed sex cord-stromal tumors (MSCSTs) are rarer. Recently, we found a MSCST in an adult male testis [adult granulosa cell tumor (AGCT) with Sertoli cell tumor]. He was admitted to the hospital based on “left testicular bloating and dull pain for 20 years and aggravating for 10 days”. Routine examination of color Doppler ultrasound showed a size of approximately 1.09 cm × 0.79 cm in the left testis with a low echo area, clear outline, and color flow in it. The patient underwent a radical left orchiectomy to remove the tumor. Pathological results showed that the tumor was diagnosed as testicular MSCST (AGCT with Sertoli cell tumor). He was in good health after the operation and showed no signs of recurrence or metastasis after 6 months of follow-up. We summarized the clinical, ultrasonic, and histopathological characteristics of this case. And immunohistochemical staining was very important in the pathological diagnosis of testicular MSCSTs, which can distinguish different tumor types. MSCSTs were usually mixed Sertoli-Leydig cell tumors, while this case is a MSCST of AGCT with Sertoli cell tumor, which is unique from other cases. Moreover, in this case, the doctors could not clearly diagnose the tumor through pre-operative physical, ultrasonic and laboratory examinations until the postoperative pathological examination. This further reflected the importance of pathological examination in the diagnosis of such tumors.     

Modern Interpretation of Giant Cell Tumor of Bone: Predominantly Osteoclastogenic Stromal Tumor

Owing to striking features of numerous multinucleated cells and bone destruction, giant cell tumor (GCT) of bone, often called as osteoclastoma, has drawn major attractions from orthopaedic surgeons, pathologists, and radiologists. The name GCT or osteoclastoma gives a false impression of a tumor comprising of proliferating osteoclasts or osteoclast precursors. The underlying mechanisms for excessive osteoclastogenesis are intriguing and GCT has served as an exciting disease model representing a paradigm of osteoclastogenesis for bone biologists. The modern interpretation of GCT is predominantly osteoclastogenic stromal cell tumors of mesenchymal origin. A diverse array of inflammatory cytokines and chemokines disrupts osteoblastic differentiation and promotes the formation of excessive multi-nucleated osteoclastic cells. Pro-osteoclastogenic cytokines such as receptor activator of nuclear factor kappa-B ligand (RANKL), interleukin (IL)-6, and tumor necrosis factor (TNF) as well as monocyte-recruiting chemokines such as stromal cell-derived factor-1 (SDF-1) and monocyte chemoattractant protein (MCP)-1 participate in unfavorable osteoclastogenesis and bone destruction. This model represents a self-sufficient osteoclastogenic paracrine loop in a localized area. Consistent with this paradigm, a recombinant RANK-Fc protein and bisphosphonates are currently being tried for GCT treatment in addition to surgical excision and conventional topical adjuvant therapies.     

Solitary Fibrous Tumor of the Breast and Mammary Myofibroblastoma: The Same Lesion?

Abstract:  Benign stromal tumors of the breast are rare mesenchymal neoplasms that have significant clinical and morphologic overlap. We report the case of a spindle cell tumor occurring in the mammary gland with mixed features of solitary fibrous tumor and mammary myofibroblastoma. The significance of this finding as well as the pathologic and radiologic diagnostic strategies used to differentiate these two lesions are presented based on a review of pertinent literature.     

Glomus Tumor in a Segmental Bronchus: A Case Report

Glomus tumors originate from a neuroarterial structure called the glomus body, and grow mostly in soft tissue. It is rare for glomus tumors to develop in the respiratory system. The patient of the present case had an abnormal shadow in the right lung on chest X-ray, and computed tomography (CT) findings displayed a lung tumor in the right S6. Bronchoscopy was performed for the diagnosis of the lung tumor, and a polypoid bronchial tumor was unexpectedly found to occupy the right B3. The bronchial tumor was diagnosed as a glomus tumor, and the lung tumor was diagnosed as an adenocarcinoma. The bronchial glomus tumor was cauterized by argon plasma coagulation (APC). Three weeks after the cauterization by APC, the right lower lobectomy was performed for the treatment of the lung adenocarcinoma. The patient has remained disease free for 2 years.     

Primary carcinoid of the testis associated with carcinoid syndrome

Testicular carcinoid is a rare disease accounting for less than 1% of all testicular neoplasms. It rarely manifests symptoms of carcinoid syndrome. Recent reports have noted that only 1.1-3.1% of testicular carcinoid tumors are complicated by carcinoid syndrome. In general, large tumor size and the presence of carcinoid syndrome are features associated with a malignant course. In the present case, pathological findings revealed pure carcinoid of the testis without metastasis. Moreover, watery diarrhea due to carcinoid syndrome disappeared and the serum serotonin level normalized following orchiectomy. The patient was followed up for 12 months with whole body computed tomography scan and assessment of serotonin levels. To date, there is no evidence of tumor recurrence. These findings suggest that monitoring serum serotonin levels may be useful as a marker during follow up of this type of tumor.