Neuroblastoma with intraspinal (dumbbell) extension

Twenty-three patients with neurogenic tumors having extradural extension (dumbbell tumors) were treated over a 30-year period. Three had ganglioneuroma, 6 had ganglioneuroblastoma, and 14 had neuroblastoma. Nineteen patients with malignant tumors had symptoms of spinal-cord compression; three patients with ganglioneuroma and one with a malignant tumor had no neurologic symptoms. Delays in diagnosis were frequent ranging from 6 weeks to 3 years in 10 patients. Sixteen patients had abnormal x-rays of the spine. All patients with malignant tumors had positive myelograms. Three patients with ganglioneuroma and 13 with malignant tumors are alive and free of disease. Seven patients with malignant tumors died: five with stage IV and 1 with stage III tumors from disease, and 1 with stage II tumor during laminectomy. Age and stage were important prognostic factors; location was not. Treatment consisted of excision and radiation or radiation only. Patients receiving lower-dose (less than 2000 rad) radiation did as well as those receiving much larger doses. Morbidity in patients with malignant tumors was high; 4 have spinal deformity (3 severe) and 8 of 13 surviving patients have residual neurologic deficits. Earlier diagnosis, a surgical approach which does not introduce morbidity, and low-dose radiation in younger patients are important factors in reducing morbidity.     

Primary mediastinal tumors in children

From 1950–1986, a total of 159 children (age 1 day–16 years) were treated for primary mediastinal tumors at our hospital. There were 77 malignant and 82 benign tumors. Tracheal compression causing respiratory distress was a significant symptom in 45.3% (24/53) of the children under 2 years of age. The diagnosis was based on the chest X-ray and the findings at surgery. Malignant lymphoma was usually diagnosed by cervical lymph node biopsy (23/39) but the treatment protocol was non-surgical. Non-lymphatic malignant tumors were completely or partially excised in 59.0% (23/39) of the cases. There was no early or late mortality in patients with benign tumors. At follow-up (0.5–24 years; mean 6.0 years), 62.3% (48/77) of the patients with malignant tumors were alive and symptom free. About half of the mediastinal tumors in children are malignant. Mediastinal tumors in small children can cause severe respiratory symptoms demanding urgent treatment. The treatment of choice is surgery (except in lymphomas) and the results are good even in malignant tumors.     

Algorithms for the surgical management of benign and malignant presacral tumors

Presacral (or retrorectal) tumors are rare tumors which arise in the space between the mesorectum and the sacrum. This space contains tissues derived from all three germ cell layers and from which presacral tumors may arise. Presacral tumors can be broadly categorized as congenital or acquired, and benign or malignant, and morphologically they may be cystic, solid or heterogeneous. This group of tumors are often asymptomatic and may be identified incidentally on clinical examination or pelvic imaging. Magnetic resonance imaging (MRI) is the gold standard imaging modality as it can accurately demonstrate the relationship between the tumor and adjacent structures and helps to delineate between benign and malignant lesions. PET/CT can be useful when assessing malignant and indeterminate lesions in order to identify aggressive pathology and exclude metastatic disease. Radiologically-guided percutaneous biopsy of malignant or indeterminate tumors can be performed safely in order to obtain a histological diagnosis and determine whether there is a role for adjuvant therapies. Surgery is the mainstay of treatment of presacral tumors. Benign lesions can be monitored with regular MRI, particularly smaller lesions which are asymptomatic. If resection is required, benign lesions can often be “shelled out” with preservation of adjacent structures and therefore function, whereas malignant tumors generally require radical excision with wide margins and sacrifice of involved pelvic organs or soft tissues, bone and neurovascular structures.     

Knochen- und Weichteiltumoren des Fußes und Sprunggelenkes

BACKGROUND: This study reports the epidemiology of bone and soft-tissue tumors of the foot and ankle, presents therapy strategies, and evaluates mid-term clinicofunctional outcome after surgery for malignant tumors.METHODS: Two hundred four patients with tumors of the foot and ankle were analyzed (163 benign and 41 malignant). Epidemiology and surgical therapy are reported.RESULTS: The most frequent tumors were exostosis, bone cyst, and osteoid osteoma for benign tumors and metastases and chondrosarcoma and Ewing's sarcoma for malignant tumors. In more than 90% of the benign tumors, local resection could be carried out, whereas in malignant tumors, ablative procedures and arthrodeses were almost as common as limb- and joint-sparing techniques. However, follow-up revealed good functional results and a 5-year survival rate of 84% for patients with primary malignant tumors.CONCLUSION: Tumors of the foot and ankle require a thorough therapeutic strategy. Mid-term functional results and survival rate after surgical treatment are good, although a high percentage of ablative procedures or fusions could not be avoided in our patients.     

The importance of recognizing malignant giant cell tumor of soft parts.

Four tumors in the extremities were initially diagnosed as extraskeletal osteogenic sarcoma. Recent review of these tumors had led to their reclassification. Three of these are now recognized as malignant giant cell tumors of soft tissue and one as a malignant fibrous histiocytoma. All four patients have been cured. Some special features of malignant giant cell tumors of soft parts are described. The importance of treatment planning is stressed.     

A case of meningeal melanocytoma in the cerebellopontine angle

Meningeal melanocytomas are uncommon intracranial tumors and extremely rare in the cerebellopontine angle (CPA). The tumors are generally considered to be benign because they lack malignant features in histological examination, but several literatures describe malignant behavior of the tumors such as high frequency of local recurrence, malignant transformation with leptomeningeal seeding. We describe a case of meningeal melanocytoma in the CPA and discuss the features of the tumor. The case was a 43-year-old woman with a right CPA exta-axial mass suffering from vertigo and nausea. Computed tomography (CT) and magnetic resonance imaging (MRI) showed a mass in the right CPA. The mass was hyperintense on T1-weighted images and hypointense on T2-weighted images. Surgical removal was done and pathological diagnosis was made as meningeal melanocytoma. Twenty months after the first surgery, MRI revealed local recurrence of the tumor and subtotal resection was performed.     

Three types of spindle cell tumors of the pleura. Fibroma, sarcoma, and sarcomatoid mesothelioma

The clinicopathologic features of 17 fibrous tumors of the pleura are presented. Eight were benign localized fibrous tumors; all of these were negative when stained with antibodies to keratin. Eight were diffuse malignant tumors that demonstrated intense immunohistochemical staining of the spindle cells with antibodies to keratin. One case presented as a histologically malignant spindle cell tumor that was initially localized but recurred subpleurally three times in 12 years. This spindle cell tumor was consistently negative when stained for keratin. We conclude that there are three types of fibrous tumors of the pleura: (a) a localized, histologically benign, keratin-negative spindle cell tumor that might be termed "fibroma," (b) a diffuse, histologically malignant, keratin-positive neoplasm that might appropriately be termed a "sarcomatoid mesothelioma," and (c) a histologically malignant, keratin-negative, spindle cell tumor with the potential to spread under the pleura that might appropriately be termed a "sarcoma." Immunohistochemistry proved to be useful in distinguishing the sarcomatoid mesothelioma from the sarcoma.     

Transformación maligna secundaria de teratomas testiculares: serie de casos y revisión de la literatura

BackgroundTeratomas are a spectrum of neoplasms that can undergo malignant transformation. In the World Health Organization (WHO) classification of tumors, this entity was classified as «teratoma with somatic-type malignancy», was defined as a malignant neoplasm of non-germinal phenotype that originates in a teratoma.Materials and methodsWe present a serie of nine cases of testicular teratomas with secondary malignant transformation. From January 1995 to December 2011, we found a total of 306 cases of testicular tumors. Mixed germ cell tumors were the most frequently diagnosed malignancy with 45.7%.ResultsTeratoma with secondary malignant transformation, represented 2.9% of all germinal tumors. Five cases originated within a mixed germ cell tumor, two cases from mature teratomas, and two from immature teratomas. The predominante malignant somatic component were sarcomas; two cases of chondrosarcoma, one rhabdomyosarcoma, and one case showing foci of chondrosarcoma and rhabdomyosarcoma. The case of osteosarcoma is notable for its rarity. Two cases showed epithelial malignancy in the form of an adenocarcinoma, and finally, two cases were primitive neuroectodermal tumors. At the time of diagnosis, five patients had metastases.ConclusionThe transformation of germ cell tumors to somatic type malignancies is rare. The malignant component can originate from any of the three germ lines. These tumors are resistant to standard chemotherapy for a germ cell tumor and the clinical stage is the most important prognostic factor. At our institution, the malignant component that appeared most frequently was chondrosarcoma.     

肾移植术后并发恶性肿瘤的临床分析

目的：总结肾移植术后恶性肿瘤的发病情况，并探讨其预防及治疗措施。方法：回顾性分析30例肾移植术后恶性肿瘤患者的临床资料。结果：在2500例肾移植患者中，发生恶性肿瘤30例，发病率为1．2％，其中泌尿系肿瘤所占比例最大为33．3％(10／30)，其次为消化系统肿瘤30％(9／30)，再次为皮肤癌26．7％(8／30)。结论：肾移植术后患者的肿瘤发生率显著提高，以泌尿系肿瘤最为常见。早发现、早诊断、早期采取手术治疗是提高患者生存率的关键。     

Thyroid surgery

In a 5-year period we treated 233 patients referred to us with a thyroid swelling. Among them, 39 patients (17%) had a malignant tumor. Seventy-eight percent of the patients were women. The preoperative examination procedure is discussed. Preoperatively, recurrent nerve palsy was found in 2.6% of patients with benign tumors and in 28% of patients with malignant tumors. The histologic features of the benign and the malignant tumors are described. The operative procedure in the benign group consisted of enucleation, resection, or lobectomy. In the patients with malignant tumors a radical operation was performed, consisting of lobectomy (unilateral or bilateral) when possible, eventually combined with radical neck dissection. The operative complications are discussed.     

Diagnostik und Therapie primärer Knochentumoren

Primary bone tumors can be either benign or malignant. Metastization is a characteristic feature ofmalignant bone tumors. Malignant tumors are characterized by a local aggressive and destructive behavior. The behavior of a tumor is dependent on its entity, the differentiation grade and localization and these factors are of decisive importance for the correct therapy. Even benign tumors can behave very aggressively. Different stages are defined. Patient history and conventional radiographs are the most powerful primary diagnostic tools. Many tumors show typical characteristics and if a malignant lesion is suspected a biopsy should be carried out. Several quality standards have to be respected when making the biopsy. The approach to malignant tumors is always interdisciplinary. Several biological as well as alloplastic reconstruction techniques exist. The treatment of primary malignant bone tumors requires a lot of experience and should only be done in specialized centers.     

Prim?r maligne Knochentumoren

Among human neoplasms, primary malignant bone tumors are fairly rare. They present an incidence rate of roughly 10 cases per 1 million inhabitants per year. During childhood (<15 years), the percentage of malignant bone tumors amounts to 6% of all infantile malignancies. Only leukemia and lymphoma show a higher incidence in adolescence. Of all primary malignant bone tumors, 60% affect patients younger than 45 years and the peak incidence of all bone tumors occurs between 15 and 19 years. The most common primary malignant bone tumors are osteosarcoma (35%), chondrosarcoma (25%), and Ewing's sarcoma (16%). Less frequently (≤?5%) occurring tumors are chordoma, malignant fibrous histiocytoma of bone, and fibrosarcoma of bone. Vascular primary malignant tumors of bone and adamantinoma are very rare. Staging of the lesion is essential for systemic therapeutic decision-making and includes complete imaging and histo-pathological confirmation of the suspected entity. In most cases, this is established by open- or image-guided biopsy. Based on this information, an interdisciplinary tumor board will determine the individual therapeutic approach. Endoprosthetic or biological reconstruction following wide tumor resection is the most common surgical therapy for primary malignant bone tumors. There is vital importance in a thorough postoperative follow-up and continous after-care by a competent tumor center which is permanentely in charge of therapy.     

肝移植术后新发恶性肿瘤四例临床分析

目的 探讨肝移植术后新发恶性肿瘤的临床特征、危险因素和防治措施.方法 回顾性分析2003年8月至2008年12月间行肝移植术的726例受者中新发恶性肿瘤的临床资料.结果 在726例肝移植受者中,术后新发恶性肿瘤4例,发生率为0.6%;患者均为男性;新发恶性肿瘤的类型分别为:急性髓性白血病、胃癌、肺癌和未分化肝肉瘤;患者肝移植时年龄为42～57岁,中位年龄52岁,肿瘤确诊时的年龄为45～60岁,中位年龄53岁;从接受肝移植手术至发生肿瘤的时间为6～38个月,中位时间31个月.4例患者均死于肿瘤进展和多器官功能衰竭.肝移植术至死亡时间为12～48个月,中位时间39.5个月;确诊为新发恶性肿瘤至死亡时间为6～10个月,中位时间8.5个月.结论 肝移植术后新发恶性肿瘤国内发病率低于国外;确诊时间较晚是患者死亡的主要原因;重视癌前病变和高危因素,早期诊断,早期治疗是提高疗效的关键.     

Clinicopathological review of 46 primary retroperitoneal tumors

OBJECTIVES: To clarify the clinical factors including diagnostic imaging findings that may correlate with the histopathological malignancy in primary retroperitoneal tumors. METHODS: The clinical backgrounds and imaging findings of 22 benign and 24 malignant primary retroperitoneal tumors were retrospectively investigated, and the prognosis of patients with malignant retroperitoneal tumors was assessed. RESULTS: There were significant correlations between the presence of symptoms and malignancy (P < 0.01), as well as between the irregularity of tumor margins and malignancy (P < 0.01). On dynamic magnetic resonance imaging (MRI), 90% of malignant tumors showed early enhancement either with quick or slow washout, while 75% of benign tumors showed delayed and no enhancement (P < 0.002). All malignant and benign paraganglioma showed the same early enhancement with quick washout. Malignant lymphoma showed various enhancement patterns. The 2-year and 5-year cause-specific survival rates of the patients with malignant retroperitoneal tumors were 68.0% and 43.2%, respectively. All malignant lymphoma patients were mainly treated with chemotherapy after being diagnosed histologically. Malignant paraganglioma patients who could not meet complete resection needed chemotherapy for promising survival. CONCLUSION: The symptoms, the irregularity of the margins, and the specific enhancement pattern on the dynamic MRI may be important predictive factors of the primary malignant retroperitoneal tumors. Histological diagnosis was needed for malignant definition of paraganglioma because both benign and malignant paraganglioma showed similar clinical and imaging findings. Preoperative biopsy should be considered for selection of the appropriate treatment particularly in patients that are likely to have malignant lymphoma that could not be diagnosed definitively by the clinical and imaging findings.     

肝移植术后新发恶性肿瘤四例临床分析

目的 探讨肝移植术后新发恶性肿瘤的临床特征、危险因素和防治措施.方法 回顾性分析2003年8月至2008年12月间行肝移植术的726例受者中新发恶性肿瘤的临床资料.结果 在726例肝移植受者中,术后新发恶性肿瘤4例,发生率为0.6%;患者均为男性;新发恶性肿瘤的类型分别为:急性髓性白血病、胃癌、肺癌和未分化肝肉瘤;患者肝移植时年龄为42～57岁,中位年龄52岁,肿瘤确诊时的年龄为45～60岁,中位年龄53岁;从接受肝移植手术至发生肿瘤的时间为6～38个月,中位时间31个月.4例患者均死于肿瘤进展和多器官功能衰竭.肝移植术至死亡时间为12～48个月,中位时间39.5个月;确诊为新发恶性肿瘤至死亡时间为6～10个月,中位时间8.5个月.结论 肝移植术后新发恶性肿瘤国内发病率低于国外;确诊时间较晚是患者死亡的主要原因;重视癌前病变和高危因素,早期诊断,早期治疗是提高疗效的关键.     

What adrenal incidentalomas are to be operated?

AIM OF THE STUDY: The goal of this retrospective study was to recognize the incidence of adrenal tumors called incidentalomas, that are in fact symptomatic or hormone secreting tumors, to assess if the diagnostic criteria proposed in the literature are reliable for adrenal tumors found incidentally. PATIENTS AND METHOD: The records concerned 147 patients operated between April 1981 and December 2000 for an adrenal mass measuring 3 cm in diameter and larger on CT scan. Clinical and laboratory findings as well as hormone levels and imaging examinations (CT Scan, IRM, scintigraphy) were correlated with operative findings and results of histologic examinations. One hundred and three patients were operated by a traditional approach and forty four by videoendoscopic techniques. RESULTS: In 41 patients (28%), the tumors had to be classified as false incidentalomas (group 1) postoperatively. In 106 patients (72%), the nature of tumor was non definite. In this group 2, there were ten malignant tumors, two pheochromocytomas and 94 benign lesions. Age of patient, size of tumor, increase in size over time and radiological appearance represent criteria that are not reliable to separate malignant from benign tumors. CONCLUSIONS: Tumors of indeterminated and non specific nature only should be classified as incidentalomas. Imaging techniques as well as other criteria of malignancy cannot be relied upon to separate benign from malignant tumors. The risk even small of cancer or pheochromocytoma leads us to recommend excision of tumors > or = 3 cm in radiological diameter. The videoendoscopic approach is a good alternative to open operation in centers with this special expertise.     

肝移植术后新发恶性肿瘤四例临床分析

目的 探讨肝移植术后新发恶性肿瘤的临床特征、危险因素和防治措施.方法 回顾性分析2003年8月至2008年12月间行肝移植术的726例受者中新发恶性肿瘤的临床资料.结果 在726例肝移植受者中,术后新发恶性肿瘤4例,发生率为0.6%;患者均为男性;新发恶性肿瘤的类型分别为:急性髓性白血病、胃癌、肺癌和未分化肝肉瘤;患者肝移植时年龄为42～57岁,中位年龄52岁,肿瘤确诊时的年龄为45～60岁,中位年龄53岁;从接受肝移植手术至发生肿瘤的时间为6～38个月,中位时间31个月.4例患者均死于肿瘤进展和多器官功能衰竭.肝移植术至死亡时间为12～48个月,中位时间39.5个月;确诊为新发恶性肿瘤至死亡时间为6～10个月,中位时间8.5个月.结论 肝移植术后新发恶性肿瘤国内发病率低于国外;确诊时间较晚是患者死亡的主要原因;重视癌前病变和高危因素,早期诊断,早期治疗是提高疗效的关键.     

Primary tumors of the small bowel

Primary tumors of the small bowel are uncommon, representing less than 6 per cent of all gastrointestinal tumors and less than 2 per cent of all malignant gastrointestinal tumors. This report concerns a twenty-five year survey of our clinical records from 1946 to 1971 which revealed 140 primary small bowel tumors, excluding periampullary tumors. Fifty-two of the neoplasms (37 per cent) were benign; eighty-eight (63 per cent) were malignant and included twenty-eight adenocarcinomas (31.8 per cent), twenty-four lymphosarcomas (27.3 per cent), nineteen carcinoids (21.6 per cent), and ten leiomyosarcomas (11.4 per cent). The average age at the time of diagnosis was 56.9 years for patients with benign tumors and 55.9 years for those with malignant tumors. The illusive and obscure nature of small bowel tumors is illustrated by the fact that 63.3 per cent of patients with benign lesions and 47.6 per cent of those with malignant lesions had symptoms for more than six months before the diagnosis was made. Bleeding was the most common present complaint in patients with benign neoplasms (52.9 per cent) whereas patients with malignant lesions more often had symptoms of obstruction (50.6 per cent). Most of the benign lesions were located proximally in the small bowel (duodenum, 34.6 per cent; ileum, 11.5 per cent), and most of the malignant lesions were located distally (duodenum, 17.0 per cent; ileum, 61.4 per cent). Treatment of patients with malignant lesions was radical excision whenever possible. Adjunctive radiation therapy was used for those with lymphoma. A second benign or malignant tumor occurred in 42.9 per cent of the patients with primary small bowel tumors. The average period of survival after diagnosis of a malignant small bowel tumor was 5.03 years: for patients with adenocarcinoma, 3.6 years; lymphosarcoma, 1.3 years; carcinoid, 6.8 years; and leiomyosarcoma, 8.3 years.     

Primary tumors of the small intestine

Seventy-two malignant and 19 benign tumors were seen at the Veterans Administration Hospital, Hines, Illinois, from 1950 to 1976. Of these, 44 malignant and 14 benign tumors were seen in the clinical setting--the remainder were found at autopsy. Carcinoid tumors were the most common malignant tumors, followed by adenocarcinoma, lymphoma and leiomyosarcoma. Patients with malignant tumors usually presented with abdominal pain, vomiting and weight loss. The most common clinical signs were anemia, abdominal tenderness and abdominal distention. The most useful diagnostic tests were upper GI series and plain x-ray of the abdomen. Twenty-one (50%) five year survivals and 14 (33%) ten year survivals were obtained with small bowel malignancies. The majority of the five and ten year survivals were patients who had carcinoid tumors and lymphoma. Various theories on the causation of small bowel malignancies are discussed.