3例肾脏原始神经外胚层肿瘤临床分析

目的：提高临床上对肾脏原始神经外胚层肿瘤的认知。方法：回顾性分析2004年1月～2008年4月收治的3俩肾脏原始神经外胚层肿瘤患者的临床资料。结果：3例术前均未明确诊断,皆为术后病理证实。其中2例接受术后辅助性化疗,随访无瘤生存达3年,1例未行术后辅助治疗,失访。结论：。肾脏原始神经外胚层肿瘤临床上极为少见,应结合影像学检查、病理及遗传学特点明确诊断,治疗以根治性手术为首选,综合治疗为主。     

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.     

Metastatic primitive neuroectodermal tumor to the kidney

Primitive neuroectodermal tumors of the kidney are rare neoplasms that may mimic renal cell carcinoma, especially in the presence of locally advanced or metastatic disease. Although several cases have been identified as primary renal tumors, this is the first report of metastatic spread of a primitive neuroectodermal tumor to the kidney.     

Primitive neuroectodermal tumor of the heart

We present a case of primitive neuroectodermal tumor of the left atrium with involvement of the coronary sinus. The initial presentation was of cardiac tamponade resulting from the size of the tumor. There was no evidence of tumor elsewhere, and after complete resection and without adjuvant chemotherapy the patient is well at 2-year follow-up. There has been no evidence of tumor recurrence. This is a rare reported case of resection of a cardiac primitive neuroectodermal tumor without adjuvant chemotherapy. Other cases in the literature have been treated by orthoptic transplantation and resection with chemotherapy.     

Desmin positivity in primitive neuroectodermal tumors of childhood.

In this report, we describe two rosette-forming primitive neuroectodermal tumors that were found to contain desmin by both immunohistochemistry and Western blotting. Electron microscopy on both cases was consistent with primitive neuroectodermal tumors and revealed that the tumor cells contained cytoplasmic bundles of intermediate filaments. In both cases, studies for MyoD1 protein using immunohistochemistry and Western blotting were negative. Thus, the detection of desmin in a pediatric neoplasm does not absolutely exclude the diagnosis of primitive neuroectodermal tumor and should not be considered as prima facie evidence that a small-cell tumor is a rhabdomyosarcoma.     

Primitive neuroectodermal tumor of the pulmonary hilum in an adult

A rare case of a thoracic primitive neuroectodermal tumor in an adult is presented. In this case, the aggressive, rapid progression characteristic of the disease was evident.     

Laparoscopic removal of extraosseous Ewing's sarcoma of the kidney in a pediatric patient

In the pediatric population, to the best of our knowledge, only 2 cases of renal extraosseous Ewing's sarcoma/primitive neuroectodermal tumor (EES/PNET) have been published. We report the initial case of renal EES/PNET occurring in a 10-year-old girl treated by a laparoscopic radical nephrectomy. The regimen used is the first documented use of neoadjuvant chemotherapy prior to laparoscopic radical nephrectomy for PNET. This approach obviated the need for a large incision and a prolonged postsurgical recovery. The minimally invasive nature of the laparoscopic procedure allowed for a rapid convalescence and resumption of her chemotherapy regimen within 14 days of the surgery.     

Management of Primitive Neuroectodermal Tumor of the Kidney with Inferior Vena Cava Thrombus

Primitive neuroectodermal tumors (PNET) are an aggressive group of small round cell tumors usually arising in the nervous system and affecting children. They have a tendency for local invasion, distant spread and formation of tumor thrombi. The kidney is a rare primary location for these tumors. Outcomes are frequently poor due to late diagnosis (Wilms tumor is a more common tumor in this population) and early spread. Immunohistochemistry is invaluable in making the diagnosis of PNET. We report a case of a primary renal PNET with extensive tumor thrombus into the inferior vena cava, and lung metastasis in a pediatric patient, and its successful management. Our 14-year-old patient with renal PNET was managed with radical nephrectomy, thrombectomy and chemotherapy and remains disease free to date. The diagnosis of renal PNETs should be considered in young adult patients who present with aggressive renal masses at initial presentations. Despite its aggressive nature, good outcomes can be achieved by a multimodality therapeutic strategy.Key Words: Primitive neuroectodermal tumors, Kidney cancer, Immunohistochemistry, Thrombosis     

Chemotherapy-Induced Neuronal Maturation in Sinonasal Teratocarcinosarcoma—a Unique Observation

Sinonasal teratocarcinosarcoma (SNTCS) is a rare and highly malignant tumour with combined features of a teratoma and carcinosarcoma. We report the first case of a SNTCS in 23 year old male treated with neo-adjuvant chemotherapy followed by cranio-facial resection. The resection specimen displayed cellular maturation in the neuroectodermal component. The patient presented with a short history of nasal obstruction, epistaxis and headache. On imaging, a bone destroying lesion of left paranasal sinuses and nasal cavity was identified. The diagnosis of SNTCS could be offered only on the third biopsy which showed heterogeneous admixture of primitive neuroectodermal, epithelial and mesenchymal elements. An adequate sampling with high index of suspicion is needed to catch hold this rare tumor. Tumor was excised after 4 cycles of neo-adjuvant chemotherapy. On microscopic examination, it showed similar epithelial and mesenchymal components as the pretreatment biopsies. However, the primitive neuroectodermal component displayed extensive neuronal maturation. The undifferentiated neuroectodermal cells were completely absent in the post chemotherapy specimen. This case throws light on the morphologic evidence of chemotherapy induced maturation in the neuroectodermal component within SNTCS, an event hitherto not reported in the literature in case of SNTCS.     

Emergency management of severe right ventricular inflow obstruction secondary to a metastatic cardiac tumor

We report the case of a 21-year-old female with previous history of primitive neuroectodermal tumor of the anterior abdominal wall who developed severe manifestations of right heart failure due to a mass obliterating the right ventricular cavity. She underwent emergent resection of the mass with histopathologic confirmation of metastatic neuroectodermal tumor. To the best of our knowledge, this is the first case of emergent surgical excision of a metastatic cardiac tumor of primitive neuroectodermal origin.     

A rare,highly aggressive primitive neuroectodermal tumor of the kidney: Case report and literature review

We report a case of a 14-year-old boy who initially suffered from a sudden onset of abdominal pain for 2 weeks with a protrusive soft mass over the left upper abdomen. No obvious symptomatic symptoms or body weight loss were observed. However, early lung metastasis was detected after an initial computed tomographic examination. Even after we performed salvage en bloc resection of the huge retroperitoneal tumor after primary neoadjuvant chemotherapy, the final outcome was still poor. A diagnosis according to radiologic findings was uncharacteristic. Finally, a pathologic diagnosis based on histologic and immunohistochemical results revealed a rare renal peripheral primitive neuroectodermal tumor.     

Polypoid renal pelvic lesions in children

A case is presented of gross hematuria and a large polypoid renal pelvic mass in a 15-month-old boy. Operation revealed a Wilms tumor with limited parenchymal involvement and rupture into the collecting system. This rare presentation of nephroblastoma and its differentiation from other rare benign and malignant renal pelvic lesions occurring in the pediatric patient are discussed. The advantages and disadvantages of radical and conservative surgical approaches to these rare lesions are reviewed. Preoperative cystoscopy and retrograde pyelography are mandatory in all patients with renal tumors presenting with gross hematuria, regardless of age. Radical nephrectomy with total ureterectomy is recommended for Wilms tumor with extension into the collecting system.     

Primitive neuroectodermal tumor in a 57-year-old man

Primitive neuroectodermal tumors are rare cerebral neoplasms previously described only in children and young adults. This report describes such a tumor arising in the left frontal lobe of a 57-year-old man. After surgical resection and radiation therapy to the primary site, the patient developed extensive central nervous system metastases that led to his death. The histopathologic, radiographic, and clinical features of this case suggest that future therapeutic protocols for primitive neuroectodermal tumor should be similar to those for childhood medulloblastoma or neuroblastoma.     

Extraosseous Ewing's sarcoma of the kidney

Extraosseous Ewing's sarcoma/primitive neuroectodermal tumor (EES/PNET) of the kidney is an uncommon entity and usually arises from the retroperitoneum and paraspinal region. We report a case of renal EES/PNET and discuss its clinical features, histological findings and management.     

Melanotic neuroectodermal tumor of infancy

A case of melanotic neuroectodermal tumor of infancy has been presented, and the etiology, clinical presentation, and management of this rare tumor have been discussed. The tumor may not be familiar to most otolaryngologists and should be included in the differential diagnosis of pediatric head and neck neoplasms.     

Primitive neuroectodermal tumor of the kidney confirmed by fluorescence in situ hybridization

We report a rare case of primitive neuroectodermal tumor of the kidney. The diagnosis was confirmed by the immunohistochemical profile and fluorescence in situ hybridization in formalin-fixed, paraffin-embedded tissues. The patient received intensive chemoradiotherapy after radical surgery and remains alive without recurrence 1 year after initial presentation.     

Primitive neuroectodermal tumor. Ewing's sarcoma

Primitive neuroectodermal tumor is an extraordinarily rare primary tumor in the kidney and can be mistaken for a variety of other round cell tumors. It is important to recognize each of these entities, because each carries unique therapeutic and prognostic implications. However, accurate diagnosis of these tumors is hindered by their significant morphologic overlap and complicated by their rarity. These neplasm are highly aggressive that tend to recurence and to metastatize. Standard therapy combining surgery, chemotherapy, radiation and genetic therapy. We report a case of primitive neuroectodermal tumor of the kidney in a 50 year old female patient.     

Peripheral primitive neuroectodermal tumor associated with the anterior mandible: a case report and review of the literature.

Neuroectodermal tumors may arise in many places throughout the body including the diverse tissues of the head and neck. The primitive neuroectodermal tumor is a predominately neural, nonepithelial neoplasm similar to Ewing sarcoma. This article describes an 18-year-old female patient with a highly malignant peripheral primitive neuroectodermal tumor located in the soft tissue anterior to the mandibular symphysis. The clinical and radiographic presentation as well as the histopathology and immunohistochemistry of this rare entity is discussed. A review of the literature with respect to this tumor, as well as the current management of this tumor, is presented.     

Successful penile reconstruction after multimodal therapy in patients with primitive neuroectodermal tumor originating from the penis

We herein present an extremely rare case of primitive neuroectodermal tumor originating in the penis. A 16‐year‐old male adolescent presented with painful penile swelling. Pathological, immunohistochemical and cytogenetical examinations of the specimens obtained from total penectomy confirmed the diagnosis of primitive neuroectodermal tumor. After total penectomy, the patient received adjuvant chemotherapy with ifosfamide‐based regimen for 48 weeks. As a series of therapies, the patient underwent penile reconstruction surgery after completing adjuvant chemotherapy. The patient has not shown any evidence of recurrence for the 7 years after penile reconstruction surgery, and voiding function is completely normal. A favorable outcome was observed by multimodal therapy including aggressive resection for local control, intensive adjuvant chemotherapy, and penile reconstruction with cosmetic and functional success. Similar therapeutic approaches might be selected for children with primary malignant tumors of the penis.