Successful chemotherapeutic decompression of primary endodermal sinus tumor presenting with severe spinal cord compression

Management of spinal cord compression from a primary paraspinal endodermal sinus tumor (EST) is described. A 17-month-old child presented for treatment with near-complete paraplegia secondary to spinal cord compression from a primary paraspinal EST. The child was treated with cisplatin-based chemotherapy without laminectomy or radiation therapy. Rapid resolution of symptoms was observed. The child had an excellent tumor response and complete neurologic recovery with no sequelae. Chemotherapy alone is an alternative to laminectomy or radiation therapy in the management of epidural cord compression from EST, even when the cord compression is severe.     

儿童颅外恶性生殖细胞性肿瘤诊断治疗方案临床报告

国际上通过多中心协作的临床研究使儿童恶性生殖细胞性肿瘤(GT)诊断治疗技术有了很大的进步,长期元病生存率已达60%～90%.     

Successful chemotherapeutic treatment of diencephalic syndrome with continued tumor presence

A 7-month-old infant with typical features of diencephalic syndrome (DES) associated with a hypothalamic mass, most probably a glioma, was treated with chemotherapy. The tumor showed clear shrinkage, but after more than 2 years regrowth was noted. During the treatment period the child regained normal growth and became free of symptoms. As radiation therapy, especially at a young age, has significant adverse effects and a neurosurgical approach to the diencephalic region also has the potential to cause significant sequelae, a chemolherapeulic option, when it exists, is preferred. Thus, in an infant in whom a glioma is suspected to be the cause of the DES, based on the clinical picture and the neuroimaging appearance, chemotherapy should be considered the primary therapeutic modality. Even if its effect is temporary, its use is well justified. The most appropriate treatment protocol still needs to be determined.     

The unique association of malignant histiocytosis and a primary gonadal germ cell tumor

Previous reports of the association between hematologic malignancies and germ cell tumors have been limited to patients with nonseminomatous tumors, exclusively of mediastinal origin. Among the various hematologic disorders, a large proportion have involved histiocytic tumors, either acute monocytic leukemia or malignant histiocytosis. We now report the first case of simultaneously occurring malignant histiocytosis and testicular embryonal carcinoma. The patient, an 18-year-old male, presented with hepatosplenomegaly due to malignant histiocytosis and was found on further evaluation to have a stage I testicular cancer consisting of teratocarcinoma with endodermal sinus elements. Despite aggressive chemotherapy, the patient died of malignant histiocytosis 6 months after the original diagnosis. The autopsy revealed widespread organ involvement with malignant histiocytosis and no evidence of residual germ cell tumor. This case demonstrates that germ cell tumors associated with hematologic malignancy do not arise solely in extragonadal sites.     

C-PEB方案联合手术治疗儿童恶性生殖细胞瘤3例并文献复习

目的探讨儿童恶性生殖细胞瘤的临床特点及治疗方法。方法回顾性分析我中心收治的3例恶性生殖细胞瘤病例,均经C-PEB方案联合手术治疗。结果目前3例随访时间分别达2年、2年半、3年,均为完全缓解。结论 C-PEB方案联合手术治疗恶性生殖细胞瘤安全有效。     

Langerhans cell histiocytosis after therapy for a malignant germ cell tumor of the central nervous system

Langerhans cell histiocytosis (LCH) is a clonal neoplastic disorder that results in a spectrum of clinical manifestations. Known to be associated with a variety of malignant diseases, LCH may precede, coincide with, or develop after the diagnosis of cancer. A child with a malignant germ cell tumor of the brain who subsequently experienced LCH is reported. The 8-year-old boy was treated for an immature teratoma of the posterior fossa with gross total resection and craniospinal irradiation preceding bleomycin, etoposide, and vinblastine chemotherapy for four cycles. Seven months after completion of therapy, he experienced multifocal bone disease with LCH.     

儿童恶性生殖细胞肿瘤25例临床治疗评估

目的评价AVAP方案治疗儿童恶性生殖细胞肿瘤的临床疗效。方法选择2005年8月—2010年2月住院治疗的恶性生殖细胞肿瘤患儿25例,所有病例诊断均经手术病理活检证实,术后予AVAP方案按不同分期化疗6～12个月。分别计算Ⅰ、Ⅱ期及Ⅲ、Ⅳ期患儿生存率,及不同原发部位的生存率。结果Ⅰ、Ⅱ期生存率100%,Ⅲ期、Ⅳ期生存率55.6%,Ⅲ、Ⅳ期生存率明显低于Ⅰ、Ⅱ期(P=0.016)。性腺肿瘤生存率85.7%,性腺外肿瘤生存率66.7%,骶尾部肿瘤生存率60%。结论Ⅰ、Ⅱ期恶性生殖细胞肿瘤经手术及术后AVAP方案化疗,可获得理想的生存率;Ⅲ、Ⅳ期疗效不理想,需探寻更合适的治疗手段。性腺恶性生殖细胞肿瘤预后好于性腺外恶性生殖细胞肿瘤,骶尾部恶性生殖细胞肿瘤预后尤其不佳。     

The unique association of malignant histiocytosis and a primary gonadal germ cell tumor.

Previous reports of the association between hematologic malignancies and germ cell tumors have been limited to patients with nonseminomatous tumors, exclusively of mediastinal origin. Among the various hematologic disorders, a large proportion have involved histiocytic tumors, either acute monocytic leukemia or malignant histiocytosis. We now report the first case of simultaneously occurring malignant histiocytosis and testicular embryonal carcinoma. The patient, an 18-year-old male, presented with hepatosplenomegaly due to malignant histiocytosis and was found on further evaluation to have a stage I testicular cancer consisting of teratocarcinoma with endodermal sinus elements. Despite aggressive chemotherapy, the patient died of malignant histiocytosis 6 months after the original diagnosis. The autopsy revealed widespread organ involvement with malignant histiocytosis and no evidence of residual germ cell tumor. This case demonstrates that germ cell tumors associated with hematologic malignancy do not arise solely in extragonadal sites.     

Acute limb ischemia due to malignant arterial embolism from a metastatic germ cell tumor

Arterial tumor embolization is a rare but serious complication of neoplastic disease. The majority of these tumors are associated with primary or secondary lung malignancies, originating from pulmonary vein metastasis or from an atrial mass. Malignant germ cell tumors primarily disseminate to the retroperitoneal lymph nodes and lung, and to the brain and liver later in the course of the disease. A germ cell tumor metastasis embolizing to the iliac-femoral arterial system has not yet been reported. We report a metastatic embolism in a patient with disseminated embryonal cell carcinoma causing acute limb ischemia, managed by surgical embolectomy. The sudden development of limb ischemia in a patient with a germ cell tumor should alert the physician to the possibility of tumor embolism. © 1995 Wiley-Liss, Inc.     

儿童生殖细胞肿瘤标志物研究进展

肿瘤标志物是肿瘤学领域的研究热点,儿童生殖细胞肿瘤标志物主要涉及甲胎蛋白、磷酸酰肌醇蛋白聚糖-3、SALL4、胶质细胞系源性神经营养因子受体α-1(GFRα-1)、Ki-67、八聚体结合转录因子4、CD117、D2-40、CD30等.其中,SALL4可作为生殖细胞肿瘤新的标志物,尤其是卵黄囊瘤;GFRα-1有助于未成熟畸胎瘤的诊断.     

Spontaneous extracranial decompression of epidural hematoma

Epidural hematoma (EDH) is a common sequela of head trauma in children. An increasing number are managed nonsurgically, with close clinical and imaging observation. We report the case of a traumatic EDH that spontaneously decompressed into the subgaleal space, demonstrated on serial CT scans that showed resolution of the EDH and concurrent enlargement of the subgaleal hematoma.     

Askin tumor : A malignant small cell tumor

Malignant tumours of the chest wall are rare in the pediatric age group. Askin tumour, a primitive neuroectodermal tumour with aggressive nature, is extremely rare. Clinico-pathological features make it quite distinct from Ewing’s sarcoma. Aggressive nature of the tumour results in short clinical presentation of the patient. Diagnosis of Askin tumour is mainly by histo-pathology. Imaging modalities play only a complimentary role. We present a case diagnosed to have this rare malignant tumour in the thoraco-pulmonary area in a child.     

Mediastinal germ cell tumor in a young woman

Primary mediastinal germ cell malignancies are uncommon neoplasms, usually presenting in young men. We review a case of a primary mediastinal seminoma occurring in a 23-year-old woman. The differential diagnosis, work-up and therapy for these lesions is discussed with emphasis on the role of magnetic resonance imaging in the evaluation of anterior mediastinal masses.     

Postoperative chemotherapy for primary intracranial germ cell tumor

Two children with primary intracranial mixed germ cell tumors are described who were successfully treated by partial resection of the tumor followed by sequential combination chemotherapy without radiation therapy. The chemotherapy consisting of VP-16 and cisplatin alternating with vincristine, methotrexate, and bleomycin resulted in apparent complete response after 6 to 7 months of treatment. Disease-free remission has continued 30–34 months off therapy. A small residual mass in one patient continues to decrease in size on magnetic resonance imaging and is presumed to represent postsurgical change rather than malignant tumor. This report demonstrates that chemotherapy may be effective in primary germ cell tumors of the suprasellar and pineal regions and could be considered for primary treatment instead of radiotherapy.     

Pediatric malignant germ cell tumors: A comparison of the neuro‐oncology and solid tumor experience

Malignant germ cell tumors (GCT) arise from abnormal migration of primordial germ cells and are histologically identical whether they occur inside or outside the central nervous system (CNS). However, the treatment strategy for GCTs varies greatly depending on the location of the tumor. These differences are in part due to the increased morbidity of surgery in the CNS but may also reflect differential sensitivity of the tumors to chemotherapy and radiation therapy (RT) or not‐yet‐understood biologic differences between these tumors. Historically, specialists caring for extracranial and intracranial GCT in the United States have practiced separately without much cross communication. The focus of this review is a discussion of differences between the management of CNS and extra‐CNS GCTs and opportunities for collaboration and future research.