Multiple primary brain tumors of different histological types--report of two cases.

Simultaneous development of histologically different primary brain tumors aside from phacomatoses or previous irradiation is rare, and its preoperative diagnosis is still difficult. We report two such cases, a 49-year-old male with an acoustic neurinoma and a cerebellar hemangioblastoma and a 69-year-old female with a parietal convexity meningioma and an ipsilateral frontal lobe astrocytoma. The tumors in the first case developed closely, so some local stimulation probably acted as a developmental factor. However, the tumors in the second case developed in distant areas and were considered coincidental.     

Late occurrence of additional ocular and intracranial pathologies in the linear naevus sebaceous (Feuerstein-Mims) syndrome

Summary The linear naevus sebaceous syndrome of Feuerstein-Mims belongs to the phacomatoses and classically is composed of a linear cutaneous lesion associated with mental retardation and epilepsy, sometimes also with ocular dystrophies.During an unusually long follow-up of 32 years of such a case, which is reported here, the late occurrence of additional ocular and intracranial pathology are demonstrated: conjunctival and bulbar tumours with progressive deterioration of visual acuity, bilateral intracranial arachnoidal cysts, dilatation and torstuosity of the middle cerebral artery.As a consequence of these observations the prognosis in cases with this syndrome has to be given with special caution.     

Renal artery stenosis and aneurysmatic dilatation of arteria carotis interna in tuberous sclerosis complex

Hypertension in children with neurocutaneous disorders (phacomatoses) is a well-recognized complication of these diseases and the cause of hypertension is fairly specific within each group. In patients with neurofibromatosis, hypertension is mainly caused by renovascular disease, whereas in tuberous sclerosis (TSC) reasons for hypertension are renoparenchymal lesions, such as angiomyolipoma or cysts. We report on a girl with TSC and hypertension due to unilateral renal artery stenosis associated with aneurysmatic changes of internal carotid artery. This unusual combination of symptoms in our patient supports the importance of thorough and complete investigation of hypertension in children with phacomatoses. Received: 20 July 2001 / Revised: 23 October 2001 / Accepted: 23 October 2001     

Vascular hyperpermeability as a hallmark of phacomatoses: is the etiology angiogenesis comparable with mechanisms seen in inflammatory pathways? Part I: historical observations and clinical perspectives on the etiology of increased CSF protein levels,CSF clotting,and communicating hydrocephalus: a comprehensive review

Phacomatoses are a special group of familial hamartomatous syndromes with unique neuro-cutaneous manifestations as well as disease characteristic tumors. Neurofibromatosis 2 (NF2) and tuberous sclerosis complex (TSC) are representatives of this family. Vestibular schwannoma (VS) and subependymal giant cell tumor (SGCT) are two of the most common intracranial tumors associated with NF2 and TSC, respectively. These tumors can present with obstructive hydrocephalus due to their location adjacent to or in the ventricles. However, both tumors are also known to have a unique association with an elevated protein concentration in the cerebrospinal fluid (CSF), sometimes in association with non-obstructive (communicating) hydrocephalus (HCP), the causality of which has been unclear. Furthermore, SGCTs have repeatedly been shown to have a predisposition for CSF clotting, causing debilitating obstructions and recurrent malfunctions in shunted patients. However, the exact relation between high protein levels and spontaneous clotting of the CSF is not clear, nor is the mechanism understood by which CSF may clot in SGCTs. Elevated protein levels in the CSF are thought to be caused by increased vascular permeability and dysregulation of the blood–brain barrier. The two presumed underlying pathophysiologic mechanisms for that, in the context of tumorigenesis, are angiogenesis and inflammation. Both mechanisms are correlated to the Pi3K/Akt/mTOR pathway which is a major tumorigenesis pathway in nearly all phacomatoses. In this review, we discuss the influence of angiogenesis and inflammation on vascular permeability in VSs and SGCTs at the phenotypic level as well as their possible genetic and molecular determinants. Part I describes the historical perspectives and clinical aspects of the relationship between vascular permeability, abnormal CSF protein levels, clotting of the CSF, and communicating HCP. Part II describes different cellular and molecular pathways involved in angiogenesis and inflammation in these two tumors and the correlation between inflammation and coagulation. Interestingly, while increased angiogenesis can be observed in both VS and SGCT, inflammatory processes seem more prominent in SGCT. Both pathologies are characterized by different subgroups of tumor-associated macrophages (TAM): the pro-inflammatory, M1 type is predominating in SGCTs while pro-angiogenetic, M2 type is predominating in VSs. We suggest that lack of NF2 protein in VS and lack of TSC1/2 proteins in SGCT determine this fundamental difference between the two tumor types, by defining the predominant TAM type. Since inflammatory reactions and coagulation processes are tightly connected, a “pro-inflammatory state” of SGCT can be used to explain the observed associated enhanced CSF clotting process. These distinct cellular and molecular differences may have direct therapeutic implications on tumors that are unique to certain phacomatoses or those with similar genetics.     

Vascular hyperpermeability as a hallmark of phacomatoses: is the etiology angiogenesis related to or comparable with mechanisms seen in inflammatory pathways? Part II: angiogenesis- and inflammation-related molecular pathways,tumor-associated macrophages,and possible therapeutic implications: a comprehensive review

Phacomatoses are a special group of familial hamartomatous syndromes with unique neurocutaneous manifestations as well as characteristic tumors. Neurofibromatosis type 2 (NF2) and tuberous sclerosis complex (TSC) are representatives of this family. A vestibular schwannoma (VS) and subependymal giant cell tumor (SGCT) are two of the most common intracranial tumors associated with these syndromes, related to NF2 and TSC, respectively. These tumors can present with an obstructive hydrocephalus due to their location adjacent to or in the ventricles. Remarkably, both tumors are also known to have a unique association with elevated protein concentrations in the cerebrospinal fluid (CSF), sometimes in association with a non-obstructive (communicating) hydrocephalus. Of the two, SGCT has been shown to be associated with a predisposition to CSF clotting, causing a debilitating recurrent shunt obstruction. However, the exact relationship between high protein levels and clotting of CSF remains unclear, nor do we understand the precise mechanism of CSF clotting observed in SGCT. Elevated protein levels in the CSF are thought to be caused by increased vascular permeability and dysregulation of the blood–brain barrier. The two presumed underlying pathophysiological processes for that in the context of tumorigenesis are angiogenesis and inflammation. Both these processes are correlated to the phosphatidylinositol-3-kinase/Akt/mammalian target of rapamycin pathway which is tumorigenesis related in many neoplasms and nearly all phacomatoses. In this review, we discuss the influence of angiogenesis and inflammation pathways on vascular permeability in VSs and SGCTs at the phenotypic level as well as their possible genetic and molecular determinants. Part I described the historical perspectives and clinical aspects of the relationship between vascular permeability, abnormal CSF protein levels, clotting of the CSF, and communicating hydrocephalus. Part II hereafter describes the different cellular and molecular pathways involved in angiogenesis and inflammation observed in both tumors and explores the existing metabolic overlap between inflammation and coagulation. Interestingly, while increased angiogenesis can be observed in both tumors, inflammatory processes seem significantly more prominent in SGCT. Both SGCT and VS are characterized by different subgroups of tumor-associated macrophages (TAMs): the pro-inflammatory M1 type is predominating in SGCTs, while the pro-angiogenetic M2 type is predominating in VSs. We suggest that a lack of NF2 protein in VS and a lack of TSC1/TSC2 proteins in SGCT significantly influence this fundamental difference between the two tumor types by changing the dominant TAM type. Since inflammatory reactions and coagulation processes are tightly connected, the pro-inflammatory state of SGCT may also explain the associated tendency for CSF clotting. The underlying cellular and molecular differences observed can potentially serve as an access point for direct therapeutic interventions for tumors that are specific to certain phacomatoses or others that also carry such genetic changes.     

经闭孔行闭孔神经阻滞对预防膀胱侧壁肿瘤电切时闭孔神经反射的作用

目的探讨经闭孔行闭孔神经阻滞对预防膀胱侧壁肿瘤电切时闭孔神经反射的作用。方法回顾性分析67例膀胱侧壁浅表性肿瘤行经尿道膀胱肿瘤电切术的患者资料,根据术中是否行闭孔神经阻滞分为闭孔神经阻滞组（35例）和对照组（32例）,其中闭孔神经阻滞组术中辅以经闭孔法闭孔神经阻滞,而对照组未行闭孔神经阻滞。比较两组术中闭孔神经反射发生率、膀胱穿孔率、手术时间、及出血量,并术后随访观察肿瘤的复发情况。结果两组术中电切时间、出血量、术后1年肿瘤复发率均无统计学差异,但闭孔神经阻滞组闭孔神经反射率及膀胱穿孔率较对照组明显降低。结论经闭孔行闭孔神经阻滞能有效预防膀胱侧壁肿瘤电切时闭孔神经反射,可降低膀胱穿孔率,其操作要点是选择准确的穿刺点、掌握好穿刺方向和深度。     

An inexpensive reconstruction method after resection in tumors of the proximal humerus with extensive involvement of the diaphysis

Limb salvage is now the norm for a large majority of malignant bone tumors. The proximal humerus is the third most common site for the occurrence of malignant tumors of bone. In tumors of the proximal humerus with large volume disease, extensive involvement of the diaphysis is not infrequent. Resection may often extend as far as the distal metaphysis in order to obtain adequate oncologic margins. A very small distal stump of bone poses unique challenges in reconstruction where conventional reconstruction modalities may be difficult to apply. We describe the use of a customised plate to reconstruct these defects which offers an inexpensive, easy to use, durable reconstruction option. It provides adequate shoulder and arm stability and ensures excellent hand and elbow motion with good functional results.     

Bilateral Wilms' tumor and secondary malignancies

A series of six patients with bilateral Wilms' tumor (nephroblastoma) is presented. Multimodal therapy yielded a survival rate of 83% at 2 yr of follow-up. However, at the end of a later follow-up period only two patients (33%) were alive. of the 4 patients who died. Only 1 died of Wilms' tumor. One died of complications of aggressive chemotherapy and two patients died after 12 and 16 yr following treatment of secondary malignant tumors arising in the irradiated region. Patients with bilateral tumor should be followed at regular intervals for the duration of life for the occurrence of secondary malignant tumors.     

FACTORS AFFECTING THE OCCURRENCE OF UROTHELIAL TUMORS IN DYE WORKERS EXPOSED TO AROMATIC MINES

Background: Past studies have analyzed individual jobs in dyestuff factories, materials manufactured and handled, age at exposure, and the duration of exposure in factories as factors related to the occurrence of urothelial tumors. None of these studies was based on long-term observation, and the factors involved in the occurrence of urothelial tumors remain controversial. In this study, various factors that may affect the occurrence of urothelial tumors in dye workers were assessed by multivariate analysis. Methods: Three hundred and sixty-three workers in nine member factories of the Dyestuff Industrial Cooperative associated were included the study. Factory A is a large dyestuff chemical factory in Wakayama City with 218 dye workers. The other eight smaller factories employ a total of 145 dye workers. Correlations of tumor occurrence with a variety of factors, such as dyestuff -intermediates manufactured and handled, types of job in the factory, age at the beginning of occupational exposure, and the duration of exposure were examined by multivariate analysis using multiple logistic models. Results: Urothelial tumors were found in 58 (16.0%) of the 363 dye workers in the nine member factories of the Cooperative associated examined in the present study. The incidence in workers in Factory A, 5.5% (12 patients), was significantly (P 0.01) lower than the overall incidence, while that in the eight small factories, 31.7% (46 patients), was significantly (P 0.01) higher than the overall incidence. The risk factors significantly related to tumor occurrence in the 363 dye workers were benzidine (odds ratio, 8.302) as a dyestuff intermediate, manufacturing work (odds ratio, 4.631), and a long period of exposure (odds ratio, 1.018). Correlations of the tumor occurrence with the various factors were examined by multivariate analysis using multiple logistic models. In the total of 363 workers, benzidine as an intermediate (P 0.05), manufacturing work (P 0.01) and the duration of exposure (P 0.01) were found to have contributed to the urothelial tumor occurrence. In Factory A, benzidine as an intermediate (P 0.01) and duration of exposure (P 0.05) contributed significantly to tumor occurrence. Conclusions: (1 The manufacturing and handling of benzidine and duration of exposure contribute significantly to the occurrence of occupational urothelial tumor, the former more strongly than the latter; 2) the contribution of different job types to tumor occurrence may be dependent upon the industrial health and safety practices in each factory.     

Desmoid tumor of the chest wall following chest surgery: Report of a case

Desmoid tumors of the chest wall following chest surgery are a rare occurrence. A case of this disease is reported herein together with a review of the literature. A 74-year-old man, who had previously undergone a right lower lobectomy for squamous cell carcinoma of the lung, was referred to our hospital with an abnormal shadow on his chest X-ray. The tumor, located in the right lateral chest wall, was successfully resected by an aggressive, wide extirpation, and a final diagnosis of a desmoid tumor originating in the chest wall was made. When following up patients after surgery for lung cancer, the possibility of desmoid tumors developing in the incised chest wall should therefore be kept in mind.     

Extraskeletal mesenchymal chondrosarcoma of the forearm: a case report

Mesenchymal chondrosarcomas represent less than 10% of all chondrosarcomas. The majority of these tumors arise from the skeleton; only 22% have an extraosseous origin. Of the extraskeletal locations, the meninges, brain, and thigh are the most common. Involvement of the forearm is a rare occurrence. This case report describes an extraskeletal mesenchymal chondrosarcoma involving the forearm.     

Desmoplastic Fibroma Arising in the Distal Phalanx of the Great Toe: A Case Report

Desmoplastic fibroma (DF) of the bone is a rare locally aggressive tumor usually occurring in adolescents and young adults. These tumors most commonly occur in the mandibles and metaphyses of long bones but are extremely rare in small bones, often resulting in diagnostic problems. The occurrence of these tumors in the foot is especially limited. We report the clinical, radiographic, and histologic features of DF arising in the distal phalanx of the great toe and a review of the published data.     

Spinal cord arteriovenous malformations and the Klippel-Trenaunay-Weber syndrome.

Five cases in which the Klippel-Trenaunay-Weber syndrome was associated with a spinal cord arteriovenous malformation are reported: they formed part of a series of 150 spinal arteriovenous malformations. Hypertrophic lesions, dilated arteries and varicose veins were present in every case, but cutaneous angiomatosis was found in only two. In each case, there was an intramedullary AVM. The relationship of the Klippel-Trenaunay-Weber syndrome to the regional angiomatous phacomatoses is discussed.     

Primary gastric tumors of infancy and childhood: 54-year experience at a single institution

Background/Purpose

Primary gastric tumors are rare in infancy and childhood. Because of the infrequent occurrence of these tumors, the clinician may be unfamiliar with optimal management strategies. We review our experience over the past 54 years and the current literature.

Methods

During the period extending from 1952 to 2006, 21 infants and children with primary gastric tumors were treated at Children's Hospital Los Angeles. The series includes 8 cases previously reported and 13 additional cases seen since the initial report. Follow-up information is included.

Results

There were 12 males and 9 females, aged 12 days to 18 years, who were diagnosed with gastric tumors. The patients presented primarily with weight loss, vomiting, or an abdominal mass. Morphological analysis revealed gastric stromal tumors (n = 6), teratomas (n = 4), lymphomas (n = 4), adenocarcinomas (n = 2), inflammatory myofibroblastic tumors (n = 2), embryonal rhabdomyosarcoma (n = 1), and hamartomas (n = 3). There were 16 patients still alive (mean follow-up, 22.3 months), whereas 6 died from active disease despite multimodal treatment. The deaths occurred in patients with stromal tumors, adenocarcinomas, lymphomas, and rhabdomyosarcoma.

Conclusions

Gastric tumors in children are rare. A high index of suspicion is needed to diagnose these tumors. Most malignant tumors present at an advanced stage and carry a substantial rate of mortality. They should be completely resected whenever feasible. In the case of some malignancies, chemotherapy may play a major role. Metastatic evaluation should be performed in all patients with malignant gastric tumors.     

Concurrent invasive ductal carcinoma and chronic lymphocytic leukemia manifesting as a collision tumor in breast

Collision tumors are rare clinical entities in which two histologically distinct tumor types show involvement in the same site. The occurrence of these tumors in the breast is extremely rare. Here, we present a case of a patient with both invasive ductal carcinoma and chronic lymphocytic leukemia in the breast. Wide excision with sentinel lymph node biopsy revealed palpably abnormal lymph nodes negative for breast carcinoma on frozen section. Histopathological examination of these lymph nodes showed extensive involvement by lymphoma and review of the breast specimen demonstrated the same lymphoma at the periphery of the ductal carcinoma. We review the literature and discuss possible etiologies for the dual presentation of both cancers.     

Bifocal temporal ganglioglioma

The authors present the case of a 33-year-old patient with a bifocal ganglioglioma located in the right superior temporal gyrus. He had a history of tonic–clonic seizures and developed intermittent nausea and vertigo later on. Magnetic resonance imaging showed two distinct, small lesions in the right temporal lobe. Both tumors were removed microsurgically with ultrasound guidance. Intraoperatively, two distinct tumors were found. Histological diagnosis of both tumors was of ganglioglioma WHO II. Postoperatively, the patient was free of symptoms. Bifocal occurrence or the coincidence of two distinct gangliogliomas is a very uncommon finding. So far, it has not yet been reported in benign gangliogliomas. Received: 16 December 1998 / Accepted: 12 January 1999     

Congenital brain tumors

Two cases of congenital brain tumor present at birth are reported. One patient was a baby girl with a huge teratoma measuring 8 × 6 × 5 cm; she died on her fourth day of life. The other patient had a large cystic astrocytoma, which was successfully removed; the patient is alive and in good health at 2 years of age and has no sign of recurrence of the tumor. Each baby had a large head, which caused dystocia at delivery. In addition to these two cases, we reviewed 200 cases of brain tumors reported in the literature presenting or producing symptoms or found incidentally at autopsy within 2 months after birth. We analyzed these cases in terms of initial symptoms and signs, sex distribution, histologic types and sites of tumors, prognosis, associated anomalies, familial occurrence, and hemorrhage from the tumor. The results of these analyses are described in detail, and the etiology of neonatal brain tumors is discussed.     

腹部肿瘤联合脾切除212例临床分析

目的探讨腹部肿瘤联合脾切除适应证的选择、围手术期最佳处理以及脾切除对机体的影响。方法对1993年1月至2004年7月经手术治疗的212例腹部肿瘤联合脾切除病人的临床资料进行回顾性分析。结果腹部原发肿瘤以胃癌最为多见(117/212,55·2%),其次为肝癌合并肝硬化脾功能亢进(28/212,13·2%)和胰体尾癌(23/212,10·8%)。212例病人共行324例次不同类型的手术,共有33例(15·6%)术后发生59例次并发症,发生率为27·8%,以感染最为多见。结论联合脾切除术后并发症的发生与原发疾病和手术种类密切相关,应严格掌握手术适应证,加强围手术期处理是提高近期疗效的关键。     

Kidney carcinoma in Bourneville-Pringle disease]

We report a case of renal cell carcinoma in a 28-year-old woman with Bourneville-Pringle's disease. A review of the literature revealed 16 cases of renal cell carcinoma associated with tuberous sclerosis. The incidence of angiomyolipoma among patients with Bourneville-Pringle's disease is cited in the literature as 40-80%, but this is based solely on two early publications, while other publications suggest a considerably higher incidence of renal cell carcinoma in Bourneville-Pringle's disease, as in other phacomatoses (Hippel-Lindau's disease). We therefore recommend screening for renal cell carcinoma in patients with Bourneville-Pringle's disease.     

Giant cell tumor of distal phalanx of great toe. A case report

Giant cell tumors are locally aggressive benign osseous neoplasms of unknown origin. They mostly occur after skeletal maturity in 3rd or 4th decade and commonly involve long bones although occasional occurrence at other sites has been reported. It is rare to see these tumors involving the phalangeal bone of foot. We report a case of giant cell tumor involving the distal phalanx of great toe in a 27 year old female who presented with swelling of great toe of right foot. Radiography showed an expansile lesion in distal phalanx of great toe. En bloc resection of phalanx was done. Biopsy showed giant cell tumor and regular follow up of this patient for two years showed no recurrence of tumor. Giant cell tumor at such a location is unusually aggressive and needs regular follow up to detect local recurrence.