Unusual early recurrence of a cerebellar pilocytic astrocytoma following complete surgical resection

Summary Pilocytic cerebellar astrocytomas are usually benign tumors with generally an excellent prognosis following complete surgical resection. The goal of surgery is total resection to minimize the risk of recurrence. In this case report, a 5-year old boy who had undergone total resection of a posterior fossa pilocytic cerebellar astrocytoma (as documented by a contrast-enhanced computed tomography (CT) scan within 24 hours following surgery), developed a massive recurrence of the tumor within four months. Both the initial histology and the sections examined after the second resection revealed features typical for a pilocytic astrocytoma with no suspicion of malignancy. This case is unusual in that it is contrary to other reports suggesting that CT-documented complete surgical resection of pilocytic astrocytomas is without recurrence, and suggests the need for vigilant radiographic and clinical follow-up of these patients even if apparent complete resection of the tumor has been achieved.     

Hypertrophic olivary degeneration after resection of a cerebellar tumor

We report a case of hypertrophic olivary degeneration due to cerebellar surgery for a low-grade tumor. A 27-year-old female presented with right-sided paresthesias and intermittent leg paresis following a right cerebellar resection of a tumor 2 weeks prior. One month later, her symptoms remained stable while her neurological examination demonstrated slight right hemi-body hypoesthesia and subtle appendicular ataxia in her right upper extremity. An MRI scan revealed a hypertrophied left anterolateral medulla with increased T2 signal and no diffusion abnormality. The T2 hyperintensity and hypertrophy slowly resolved and she clinically improved without further intervention. Hypertrophic olivary degeneration may be mistaken for tumor progression, post-operative vasculopathy or granulation tissue and should be considered in patients undergoing cerebellar surgery.     

成人嗅神经母细胞瘤的临床策略探讨

嗅神经母细胞瘤是一类少见的恶性肿瘤,治疗方式有手术、放疗和化疗,但缺乏标准的治疗规范。现介绍1 例天津医科大学肿瘤医院多学科协作治疗的成人嗅神经母细胞瘤,以探讨和制定出相应的诊疗标准,使临床治疗更为规范,患者更多获益。     

Stage IV-S neuroblastoma involving the liver and ectopic liver. Report of an unusual case

Stage IV-S (special) neuroblastoma involving the liver and ectopic liver attached to the left adrenal gland of a 38-day-old girl was reported. At surgery, the ectopic liver with neuroblastoma metastases was excised, and the liver was biopsied. The histologic features of neuroblastoma involving hepatic tissue were quite similar in the liver and the ectopic liver. Eighteen months after surgery, the patient was doing well after treatment with less intensive chemotherapy. No similar case has been reported in the literature, and study of this case indicates that the hypothesis of fetal hematogenous metastasis proposed by Wieberdink does not suffice to explain the pathogenesis of extensive involvement of the liver in Stage IV-S neuroblastoma.     

Ultrastructural study of neuronal and related tumors in the ventricles

Intraventricular tumors may arise from a variety of cells in the region. There are some difficulties in diagnosing these tumors because of their histologically similar appearance. We analyzed intraventricular tumors, including central neurocytoma, oligodendroglioma, cerebral neuroblastoma, and cerebellar neuroblastoma, the neuronal characters of which were established based on their ultrastructural findings, except for oligodendroglioma. Central neurocytoma and cerebellar neuroblastoma showed synaptic formation, and cerebral neuroblastoma possessed immature neurites. Oligodendroglioma showed similar structures to that of a normal oligodendrocyte. Furthermore, we review the literature and evaluate the usefulness of analyzing ultrastructures.     

Recurrent olfactory neuroblastoma treated by radiation, surgery, and chemotherapy: "a case report"

Olfactory neuroblastoma is a rare tumor. The role of CT Scan, morphological details and the electron microscopic findings have been highlighted in the diagnosis of the lesion. The present patient, in which the tumor had recurred following surgical excision, was treated successfully with a combination of radiation, further surgery, and maintenance chemotherapy. We also feel that such a planned multidisciplinary approach is justified as the initial treatment for all advanced lesions in view of high recurrence rates reported following use of radiotherapy or surgery alone.     

Gefitinib (‘Iressa’, ZD1839) is active against brain metastases in a 77 year old patient

This report highlights the case of a symptomatic 77-year-old non-smoking female patient who was diagnosed with advanced non-small-cell lung cancer (NSCLC), metastatic to the liver and contralateral lung. After tumor progression in the liver and lung following polychemotherapy, multiple diffuse brain and cerebellar metastases were apparent. Oral treatment with the epidermal growth factor receptor tyrosine kinase inhibitor gefitinib (‘Iressa’) 250 mg/day resulted in progressive and durable symptom relief, and improvements in quality of life and performance status. Reductions in the size of the primary pulmonary tumor and brain, cerebellar, and liver metastases were observed. Furthermore, gefitinib was well tolerated with an absence of adverse events. These results provide evidence that oral gefitinib is active in patients with advanced NSCLC and central nervous system metastases. An erratum to this article is available at .     

Morphology and biology of cerebellar neuroblastomas

Summary A nodular neuroblastoma was partially removed from the cerebellar vermis of a 15-month-old boy. Postoperative irradiation and chemotherapy were performed. More than 5 years later, re-operation revealed a mature ganglioglioma. Problems relating to the maturation of ganglionic tumors are discussed. The present case emphasizes that it is not justified to regard all neuroblastomas of the central nervous system as highly malignant neoplasms (grade IV).     

Relapsed stage I neuroblastoma successfully treated by allogeneic bone marrow transplantation in second remission: case report of a 7-year-old girl

Stage I neuroblastoma in a seven-year-girl have had local recurrence with bone metastasis. Second surgery was performed after the aggressive chemotherapy including cisplatin, VP16, cyclophosphamide, vincristine and doxorubicin. She underwent allogeneic bone marrow transplantation from her HLA identical brother, following the preparative regimen consisting of high-dose melphalan and total body irradiation (12 Gy). Clinical course after the transplantation was uneventful. She is still alive with no evidence of disease for twenty-eight months after the transplantation. It is suggested that bone marrow transplantation is an effective way of therapy even in a patient with relapsed neuroblastoma.     

Large cell medulloblastoma with myogenic and melanotic differentiation: a case report with molecular analysis

We present a case of large cell medulloblastoma with myogenic and melanotic differentiation arising in the cerebellar vermis of a 2-year-old boy and following an aggressive clinical course. Histologic and immunohistochemical features of this tumor include primitive neuronal, rhabdomyoblastic, and pigmented epithelial elements, along with large cell features. Immunohistochemical and molecular data (c-myc gene amplification and the presence of isochromosome 17q) support the contention that this histologically diverse tumor represents a pattern of medulloblastoma with striated muscle and pigmented epithelial differentiation, rather than a teratoma or a cerebellar variant of melanotic neuroectodermal tumor of infancy (‘progonoma’).     

Long-term Cognitive and MRI Findings in a Patient with Paraneoplastic limbic Encephalitis

This case study describes the long-term course of behavioral alterations and MRI findings in a patient with a combined limbic and cerebellar paraneoplastic syndrome, which was associated with a squamous lung carcinoma. Clinical equivalents were cerebellar ataxia, as well as severe anterograde memory loss, frontal executive dysfunction and behavioral alterations. MRI revealed inflammatory changes followed by progressive atrophy affecting the cerebellum and both temporal lobes. Tumor surgery yielded only a partial and transient recovery of neurological symptoms, and paraneoplastic atrophy continued to progress despite radical excision of the carcinoma. This case of paraneoplastic encephalitis suggests that the related atrophy may present as a chronic, progressive, multifocal encephalopathy and that the associated cognitive impairments may include several cognitive domains.     

Rational selection of adjuvant chemotherapy after cytoreduction surgery for murine neuroblastoma

Improving the prognosis of advanced neuroblastoma remains an important yet unachieved goal of pediatric oncology, a fact which may be related to an insufficient analysis of the role played by cytoreductive surgery. Utilizing strain A mice bearing C-1300 syngeneic neuroblastoma, tumor biology and host immunocompetence were studied after cytoreduction surgery and adjuvant chemotherapy. Cell kinetic analysis in the residual tumor demonstrated an increase of the proliferative fraction 18 to 42 h after operation, but the same peak proliferation was delayed in bone marrow cells to 24 to 96 h. The potential for drug distribution to the tumor after cytoreduction surgery was assessed by injecting Na251CrO4 and measuring tumor uptake. There were two significant (P less than 0.05) peaks of activity at 6 h and 3 days, suggesting local edema and neovascularity, respectively. Injection of both cell cycle specific and nonspecific adjuvant chemotherapeutic agents in a dosage of one-fourth of their 50% lethal dose at 24 or 72 h following surgical cytoreduction did not induce any antitumor activity at either injection time. However, when cyclophosphamide was given in this dose, the C-1300 tumor growth was impaired, an effect which was largely abrogated by first subjecting the tumor bearer to thymectomy and irradiation. The transfer of spleen cells from adjuvant cyclophosphamide-treated mice to tumor-inoculated normal mice significantly delayed tumor appearance when comparison was made with animals treated by operation alone, and such recipients also exhibited a more prolonged survival. These data suggest that the antitumor activity of cyclophosphamide following cytoreduction surgery of C-1300 neuroblastoma is mediated by both pharmacological and immunological mechanisms.     

CAR-T在甲状腺癌免疫治疗中的研究进展

大多数甲状腺癌患者进行以外科治疗为主的综合治疗可以取得良好的预后,但目前晚期甲状腺癌治疗手段匮乏。采用体外基因编辑技术的嵌合抗原受体T细胞（chimeric antigen receptor T-cell,CAR-T）免疫疗法在血液系统肿瘤中得到应用并取得了良好的治疗效果。以此为基础,CAR-T开始在实体肿瘤中尝试使用,但效果欠佳。CAR-T在晚期甲状腺癌中的研究已有开展,目前仍局限在临床前的基础研究,相关临床试验较少且缺乏试验结果报道。本文将相关报道进行综述,旨在为今后的研究提供参考。     

Intraspinal Metastasis of Neuroblastoma -- Report of a Case Detected at Autopsy --

Metastasis to the brain or spinal cord parenchyma is extremelyrare in cases of neuroblastoma. We present a 3-year-7-month-old boy with neuroblastoma, stageIV, with intraspinal metastasis. He had no neurologic manifestationexcept incontinentia urinae and ataxia at the terminal stage.His cranial computed tomography scan showed high density areasin both cerebellar hemispheres which seemed to be distant metastaticmasses. At autopsy, the metastases in the cerebellum were foundto be due to dural infiltration but in the spinal cord parenchymaof the lumbar spine metastases were detected macroscopically.There were multiple distant metastatic areas near the roots,anterior and posterior. The neuroblastoma seemed to have spreadalong the roots by direct invasion from the meninges. In the future, the number of patients with metastasis into thebrain or spinal cord parenchyma will increase because patientswith progressive disease could live for a long time as a resultof intensive chemotherapy. Observation of these cases will behelpful to clarify the routes of metastasis to these areas.     

邻近腋窝的上皮样血管内皮瘤1例报道

We described a case of a 71-year-old woman with an epithelioid hemangioendothelioma (EHE) in her left axilla,a rare location which hasn't been reported yet. The patient suffered from numbness, pain and decreased muscle strength of her left upper extremity. Sonography revealed a hypoechoic mass surrounded the axillary artery and brachial artery. No obvious capsule was demonstrated. CT showed a soft-tissue mass with some calcifications and peripheral ring-like en-hancement. The MRI indicated a mass with mainly intermediate signal intensity on Tl-weighted imagine and intermediate signal intensity on T2-weighted imagine. The diagnosis was confirmed by histopathologic examination after surgery. There are some correlations of these imaging features with its histopathologic characters.     

Neuroblastoma as a Neurobiological Disease

While neuroscientists are often involved in the assessment and care of patients with central nervous system tumors, they are only rarely involved in the case of peripheral nervous system neoplasia. Neuroblastoma is a childhood tumor of the primitive sympathetic nervous system. It is at once one of the most common and one of the most deadly tumors of childhood. The prognosis for children with this tumor has not changed in the past two decades. Clearly, a fresh approach to neuroblastoma is needed. The neuroscientist has much to add to our understanding and treatment of neuroblastoma and its sequelae. Conversely, neuroblastoma has much to teach us regarding the normal development of the neural crest and the aberrant loss of neurons in this lineage. A neuroscientist's approach to neuroblastoma, its biology and clinical features, is presented herein.     

Intracranial olfactory neuroblastoma

A rare case of intracranial olfactory neuroblastoma presenting with epistaxis and intracranial symptoms is presented and relevant literature reviewed. The patient was treated by surgery and radio-therapy.     

Cytoreductive surgery and hyperthermic intraperitoneal chemotherapy: a review of factors contributing to morbidity and mortality

Cytoreductive surgery (CRS) and hyperthermic intraperitoneal chemotherapy (HIPEC) is associated with prolonged survival for appropriately selected patients with peritoneal dissemination of abdominal malignancies. CRS and HIPEC has been criticized for perceived high rates of morbidity and mortality. Morbidity and mortality rates of CRS and HIPEC, however, do not appear dissimilar to those of other large abdominal surgeries, particularly when relevant patient and operative factors are accounted for. The risk of morbidity and mortality following this surgery for a given individual can be predicted in part by a variety of patient and operative factors. While strong data are lacking, the limited data that exists on the matter suggests that the independent contribution of the heated intraperitoneal chemotherapy to CRS and HIPEC morbidity is relatively small. A more thorough understanding of the patient and operative factors associated with CRS and HIPEC morbidity and mortality, as well as the specific complications related to the intraperitoneal chemotherapy, can better inform clinicians in multidisciplinary teams and patients alike in the decision-making for this surgery.     

Paraneoplastic papilloedema in a child with neuroblastoma

Non-metastatic neurological disease complicating neuroblastoma is well recognized. Gross papilloedema in the absence of intracranial disease as initial manifestation of neuroblastoma is reported in adults. We report for the first time a case of bilateral papilloedema in a child with neuroblastoma in the absence of intracranial disease and hypertension.