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Background: Extraosseous Ewing's sarcoma/peripheral primitive neuroectodermal tumours(EWS/pPNETs) of the kidney are rare. Signs and symptoms are atypical in EWS patients. Presenting symptoms include haematuria, abdominal pain, or a palpable mass. A comprehensive review of the literature shows that it is difficult to make an accurate diagnosis based on physical examination alone. The imaging findings of EWS/pPNETs are nonspecific. We used contrast-enhanced ultrasound (CEUS) to diagnose an EWS/pPNET in our patient, which had never been reported previously to our knowledge., Case Presentation: This article reports the case of a 20-year-old female with an abdominal mass and gross haematuria for 1 month. The ultrasound revealed a hypoechoic mass with a clear margin at the lower pole in the left kidney. CEUS demonstrated signs of annular enhancement and heterogeneous enhancement of the tumour, and simultaneous wash-in was predominant. Computed tomography images showed an elliptical low-density tumour. The patient underwent a left kidney resection, and the pathological diagnosis was an EWS/pPNET. Twenty-one days after the kidney operation, the patient underwent 8 cycles of a CAV (vinorelbine, ifosfamide, epirubicin) + IE (isocyclophosphamide, etoposide) chemotherapy regimen. Subsequently, radiotherapy (dose: 45 Gy, radiation field:the tumour bed following surgical resection) was administered for nearly 30 days. The patient had no signs of local recurrence or metastasis within a follow-up of 4 years., Conclusions: As a radiation-free, inexpensive, convenient, and repeatable examination method, ultrasound was the primary choice for kidney examination. Early CEUS was helpful to make an accurate diagnosis. Surgery and adjuvant radiation or chemotherapy administered in a timely manner can prevent further deterioration., (© 2022. The Author(s).)

Introduction: Primitive neuroectodermal tumors arise from the progenitor cells of the neural crest, in the central nervous system or other peripheral locations., Case Presentation: We report a rare case of a congenital malignant tumor, diagnosed as a primary orbital primitive neuroectodermal tumor on histopathological examination., Conclusion: Multidisciplinary management with adjuvant chemotherapy needed for the management of these cases.

Introduction: Ewing sarcoma (ES), the neuroectodermal derived tumour typically occurs in the bone and soft tissue of children and young adults. Primary ES of the kidney is strikingly rare and only a few cases and small case series have been documented. Due to the highly aggressive nature of this neoplasm, distinction from other morphological mimickers is truly indispensable in terms of treatment and prognosis. Here we describe the clinicopathological features of four cases of primary ES of the kidney with special emphasis on one case having extensive neural differentiation postneo- adjuvant chemotherapy (NACT). Extensive neural differentiation in renal ES has not been documented to date., Case Series: Four patients (age range from 15-35 years) had kidney mass and multiple distant metastases at first presentation. Primary diagnosis of Ewing sarcoma was rendered by histopathology with the help of immunohistochemistry on core biopsy material. Tumour cells in all cases showed diffuse membranous CD99, nuclear FLI-1 and NKX2.2. Two of the patients had undergone radical nephrectomy followed by combination chemotherapy. Another two patients were first treated with neo-adjuvant chemotherapy (NACT) followed by radical nephrectomy. In one of them, histopathological examination of nephrectomy specimens revealed extensive neural differentiation. The adrenal gland was free in all four cases. The follow-up period was 12 -24 months. Three patients had survived and one of them became disease-free., Conclusion: Primary ES of the kidney is a rare and lethal entity. Due to overwhelming rarity, chemotherapy protocol has not been standardised and followed as ES in bone/soft tissue. Histopathological confirmation and prompt initiation of treatment may improve patient survival and outcome.

Background: The primitive neuroectodermal tumors (PNETs) are a family of highly malignant tumors with a multidirectional differential potential. The tumors are characterized by aggressive small round tumor cells that originate from the spinal cord of the central and sympathetic nervous systems. Cases involving the pericardium are extremely rare. Herein, we present a case of peripheral primitive neuroectodermal tumor (pPNET) that originated in the pericardium., Case Presentation: A 23-year-old woman presented with cough and progressive dyspnea for 1 month, followed by eyelid and facial edema for 10 days, without any apparent cause. Significantly elevated tumor markers were detected in her blood. A cardiac ultrasound revealed a 74 mm × 61 mm spherical mass that was attached to the left pericardium, as well as massive pericardial effusion. Positron emission tomography-CT (PET-CT) showed focal hypermetabolism in the left pericardium. Via histopathology and immunohistochemistry, the spherical mass was identified as PNETS. The patient was successfully treated with a combination of surgical resection via thoracotomy and postoperative chemotherapy, and she was disease-free for 7 years at follow-up. Unfortunately, at 7 years after the treatment, the patient's pPNET recurred. Positron emission tomography-MRI (PET-MRI) and 64-slice coronary CTA revealed that the aorta and multiple coronary arteries were involved. Subsequently, the patient refused a heart transplant and voluntarily left the hospital., Conclusions: This paper reports on a rare and recurrent case of PNET in the parietal pericardium. With respect to the different biologic characteristics and prognoses of pPNETs (compared to other known pericardium tumors), it is essential to consider this entity as a differential diagnosis in pericardium tumors.

Purpose: The purpose is to highlight the primary intracranial (meningeal-based) occurrence of Ewing sarcoma/primitive neuroectodermal tumor (ES/PNET)., Methods: This report is a collation of clinicopathological features of eight cases of molecularly and clinicoradiologically confirmed primary (non-metastatic) intracranial (non-osseous) meningeal ES/PNET., Results: The age range was 1 to 33 years with a median age of 9 years. Male to female ratio was 0.6:1. All patients were diagnosed on the debulking surgical material (gross total resection, 2 cases; subtotal resection, 6 cases) and showed primitive embryonal histomorphology with diffuse membranous CD99 immunoexpression and EWSR1 gene rearrangement by fluorescence in situ hybridization. Seven of them showed a typical FISH pattern of split signals with break-apart probe, while one showed an unusual signal pattern of loss of green signals. EFT-2001 adjuvant protocol was followed along with focal radiotherapy (RT) in all cases (except case 8, full course of chemotherapy could not be completed). Two cases had local recurrence-one of them died of disease recurrence before the administration of further treatment., Conclusion: This series adds non-osseous intracranial site to the list of uncommon sites of occurrence for ES/PNET and more importantly emphasizes the need to be considered in a differential list of primary intracranial primitive embryonal tumors before embarking as primary central nervous system (CNS) embryonal tumor, NOS.

Peripheral primitive neuroectodermal tumors (PNETs) constitute very rare and aggressive malignancies. To date, there are no standard guidelines for management of peripheral PNETs due to the paucity of cases arising in various body sites. Therapeutic approach is derived from Ewing sarcoma family, which currently remains multimodal. Our study retrospectively analyzed 86 PNET patients from February 1, 1998 to February 1, 2018 at Peking Union Medical College Hospital with an additional 75 patients from review of literature. The clinicopathologic and treatment plans associated with survival was investigated. Surgery, chemotherapy, female sex, small tumor size, no lymph node metastasis, R0 surgical resection, (vincristine + doxorubicin + cyclophosphamide)/(isophosphamide + etoposide) regimen, and more than 10 cycles of chemotherapy were associated with improved overall survival in univariate analysis. Surgery, more than 10 cycles of chemotherapy, and small tumor size were independent prognostic factors for higher overall survival. Our data indicates that multimodal therapy is the mainstay therapeutic approach for peripheral PNET.

Ewing sarcoma and peripheral primitive neuroectodermal tumor constitute the Ewing family of tumors (EFT). EFTs primarily arising in the ovary are extremely rare. We report the case of a 22-yr-old nulliparous woman with a primary EFT in the ovary that initially presented as a 3-cm teratoma-like ovarian tumor, with rapid progression to a 15-cm-sized tumor with liver metastasis in 3 mo. The patient underwent suboptimal debulking surgery and salvage chemotherapy with vincristine, doxorubicin, and cyclophosphamide alternating with ifosfamide and etoposide. In conclusion, primary EFT in the ovary is extremely rare with highly aggressive behavior and poor outcome for metastatic disease. Demonstration of EWSR1 rearrangement, observed in a variety of soft tissue tumors, is very helpful in the diagnosis of EFT when interpreted on the basis morphology and immunohistochemistry.

Purpose: Ewing Sarcoma/Primitive Neuroectodermal Tumor (ES/PNET) is a malignant small round cell tumor belonging to the Ewing Sarcoma Family of Tumors. It occurs more commonly in children and young adults. Its localization in the adrenal gland is extremely rare. We reviewed 35 cases of ES/PNET of the adrenal gland reported in the literature and presented our case., Methods: Data were collected by searching for ES/PNET and adrenal gland key words on Google Scholar and PubMed in March 2018, including a case diagnosed in our department. We analyzed all reviewed cases for diagnosis, surgical and systemic therapy and outcome., Results: To date 24 articles presenting cases of ES/PNET of the adrenal gland are reported in the literature. We included in our review 35 cases previously described and one new case. Histologically all cases consisted of sheets of small round cells. Immunohistochemistry was also performed in all cases. Most cases stained positive for CD99 and negative for lymphocytic markers. Markers of epithelial differentiation displayed variable results. In all cases tested, characteristic translocations were displayed supporting the diagnosis. All patients but four were treated surgically and the majority received adjuvant therapy. Only very few cases received neoadjuvant chemotherapy., Conclusions: Primary ES/PNET of the adrenal gland is a rare tumor, showing specific morphological, immunohistochemical and cytogenetic characteristics. Treatment consists of surgery, chemotherapy and radiotherapy. Further investigations paired with long term follow-up are necessary to define prognosis for this rare entity.

Objective: Primary intracranial Ewing sarcoma (ES)/peripheral primitive neuroectodermal tumors (pPNETs) are extremely rare, and only a few studies have reported >4 cases of this disease. The purpose of this study was to explore the clinical features, treatment, and outcome of primary intracranial ES/pPNETs., Methods: The clinical data of 14 patients who had been surgically treated from February 2003 to November 2017 and in whom immunohistochemical staining results had confirmed the diagnosis of primary intracranial ES/pPNETs were retrospectively analyzed. Kaplan-Meier survival analysis was used to estimate the survival rate and the median survival time (MST)., Results: Gross total resection (GTR) was achieved in 7 cases, and subtotal resection was performed in 7 cases. During follow-up, 10 (71.4%) patients had local recurrence and 3 (21.4%) patients had distant metastasis. The overall 1-, 2-, and 5-year survival rates were 78.6%, 47.6%, and 19.0%, respectively. Kaplan-Meier survival analysis showed that postoperative radiotherapy was a significant prognostic factor for longer MST (P = 0.034). GTR and radiotherapy with or without adjuvant chemotherapy yielded the highest 2-year survival rate (100%). Three patients who underwent GTR, radiotherapy, and chemotherapy had the highest 2-year survival rates (100%) and the longest MST (48 months)., Conclusions: Primary intracranial ES/pPNETs have an aggressive clinical course, with a high tendency for local recurrence and distant metastasis. Radiotherapy plays a significant role in improving the survival of patients. GTR combined with radiotherapy and chemotherapy may be the most beneficial treatment modality., (Copyright © 2018 Elsevier Inc. All rights reserved.)

Objective: We sought to assess the use of surgical treatment, the effect of postoperative adjuvant therapy, and the prognostic factors for survival of patients with primary spinal peripheral primitive neuroectodermal tumors (pPNETs)., Patients and Methods: The clinical data of 24 patients, who had been surgically treated from April 2003 to February 2018 and in whom immunohistochemical staining results had confirmed the diagnosis of primary spinal pPNETs, were retrospectively analyzed. To analyze the factors related to prognosis, the Kaplan-Meier method was used for univariate analysis, the log-rank method was used to test the significance of difference, and multivariate analysis was performed using Cox regression., Results: The overall 1-year, 2-year, and 5-year survival rates were 73.2%, 48.1%, and 12.0%, respectively. The median survival time (MST) of all patients was 21 months. Univariate analysis showed that the extent of tumor resection, adjuvant radiotherapy, and chemotherapy were the factors influencing patient prognosis after surgery (all P < 0.05); sex, age, tumor location, and preoperative Karnofsky performance scale (KPS) scores were not the influential factors for prognosis of patients after surgery (all P > 0.05). Multivariate analysis showed that gross total resection (GTR) of tumors and adjuvant radiotherapy were independent factors influencing the prognosis of patients with pPNETs (all P < 0.05)., Conclusions: Primary spinal pPNETs are extremely rare, and they have a poor prognosis. Microsurgical GTR of the tumor is the preferred method of treatment. Radiotherapy plays an important role in improving the prognosis of patients with pPNETs. GTR combined with radiotherapy and chemotherapy may be the best treatment modality., (Copyright © 2018 Elsevier B.V. All rights reserved.)

A 26-year-old male with a personal history of schizophrenia initially presented with a 13 cm pelvic mass corresponding to a cryptorchidic testis. The patient was treated with primary and second-line chemotherapy for metastatic germ-cell tumor followed by surgical consolidation. Final pathology revealed a primitive neuroectodermal tumor (PNET) mixed with mature teratoma. Despite multidisciplinary management, significant patient non-compliance led to inadequate follow up and treatment delays ultimately resulting in death. To our knowledge, this is the only reported case of teratoma with malignant transformation arising from an untreated congenital undescended testicle.

Purpose of Review: The purpose of this review is to examine the historical context alongside contemporary studies in order to provide the most current recommendations for the management of patients with metastatic teratoma with malignant somatic transformation (MST)., Recent Findings: The main themes in the recent literature covered herein include prognostic features, the management of early-stage disease, recommended chemotherapeutic and surgical strategies as well as recognized patterns of late relapse., Summary: Recent literature, combined with a significant contribution from historical studies, suggests that while MST is uncommon, its aggressive nature coupled with its resistance with traditional germ cell tumor chemotherapies makes it very difficult to manage. The key message is that surgery is recommended in all resectable MST from primary retroperitoneal lymph node dissection for clinical stage I, to radical removal of disease after chemotherapy and when chemotherapy fails. In advanced cases with documented spread of the transformed histologic subtype, systemic therapies targeted to the identified tumor type should be considered.

Background: We report our experience and outcomes about the management of Askin's tumors [AT], which are rare primitive neuroectodermal tumors (PNETs) that develop within the soft tissue of the thoracopulmonary region, typically in children and adolescents., Methods: We retrospectively analyzed the charts of 9 patients affected by AT (aged 6-15 years), treated at the Paediatric Oncology Unit of Gemelli University Hospital in Rome between January 2001 and December 2016., Results: All nine patients underwent to biopsy followed by neoadjuvant chemotherapy. At the end of the neoadjuvant chemotherapy, they underwent to surgical removal of the residual tumor. Five patients with positive tumor margins and/or necrosis< 90% received local radiotherapy. Two patients with metastasis received an intensified treatment, with the addition of high dose adjuvant chemotherapy followed by peripheral blood stem cells rescue. No statistically significant correlation was found between outcome and gender; the presence of any metastasis and the radiotherapy. The overall survival was 65.14 months (95% confidence interval [95%CI], 45.81-84.48), and the 5 years survival was 60%, at a median follow-up of 53.1 months., Conclusion: Our study confirms that a multimodal treatment with surgery, chemotherapy, and radiotherapy may increase the survival in AT pediatric patients.

Background: Ewing sarcoma (ES) is a malignant bone tumor that is most commonly observed in the long bones, the pelvis, and the chest. Primary intracranial localization is extremely rare., Objective: In the aim of identifying the prognostic factors of this rare localization, we conducted a systematic review of the literature for patients with primary intracranial peripheral primitive neuroectodermal tumor/ES., Methods: A complete MEDLINE search was undertaken for all articles reporting data for primary intracranial peripheral primitive neuroectodermal tumor/ES. We divided the patient population into 2 groups: group I, patients who were free of disease; and group II, patients who died or had uncontrolled disease at the last available follow-up. We conducted a comparative statistical analysis of data between the 2 groups., Results: A total of 48 patients were included in the review. There were 32 patients in group I and 12 in group II. Fifty percent of tumors in group II and only 16% of tumors in group I were infratentotorial (P = 0.03). Signs of bone involvement were observed in 19% of patients in group I and 54% in group II (P = 0.03). Total removal was accomplished in 29% of patients in group I and in no patients in group II (P = 0.03). Radiotherapy was performed in 73% of patients in group II and 81% in group I (P = 0.43). Chemotherapy was administered in 36% of patients in group II and 74% in group I (P = 0.03)., Conclusions: Infratentorial localization and the presence of bone involvement were associated with poor prognosis; Surgery seems to be a predictive factor of prognosis; radiotherapy and chemotherapy must be performed whenever the tumor is not totally removed., (Copyright © 2018. Published by Elsevier Inc.)

Ewing sarcoma/peripheral primitive neuroectodermal tumor (ES/PNET) typically occurs in long or flat bones, soft tissues, or less often, solid organs. Ewing sarcoma/peripheral primitive neuroectodermal tumor arising from the adrenal gland is extremely rare, especially in children, and only limited cases are reported previously. Herein, we review a case of a 22-month-old girl who presented to our department with abdominal pain, bulging of the left flank, and a nonfunctioning adrenal lesion which was found to be an adrenal ES/PNET. The patient was successfully treated with surgery and adjuvant chemotherapy. Since delayed diagnosis may result in metastatic lesions, this case underscores the importance of considering ES/PNET in the differential diagnosis of large adrenal masses.

Background: Peripheral primitive neuroectodermal tumor isolated in the heart, presenting as a primary cardiac tumor is considered as extremely rare., Methods: We present a 53-year-old Chinese female with a cardiac tumor which was discovered by CT., Results: A hypo-intense tumorous mass was shown extending from the left ventricle by Cardiac CT, and fused FDG positron emission tomography demonstrated no other abnormal FDG active lesions in the body. We performed a total resection surgery of the tumor subsequently and the patient recovered well and discharged from hospital 6 d after surgery., Conclusion: The pathological diagnosis was primary cardiac peripheral primitive neuroectodermal tumor. No tumor recurrence was shown by echocardiography during the 24 months follow-up visits.

Ewing's sarcoma (EWS) and peripheral primitive neuroectodermal tumor (pPNET) are small round cell malignancies that develop in soft tissue and bone. They very rarely affect newborns. A diagnosis of EWS/pPNET depends mainly on immunohistochemistry and molecular/genetic assays. Since these tumors are highly aggressive, patient prognosis is typically very poor, and treatment remains a challenge. Here, we report a 13-day-old newborn diagnosed with congenital EWS/pPNET and describe its treatment., (Copyright © 2014. Published by Elsevier B.V.)

Primary spinal peripheral primitive neuroectodermal tumors (pPNETs) are extremely rare entities that predominantly occur in children and young adults. Few studies have reported more than three cases. There are no current optimum treatment strategies due to the paucity of data. Here, we present 13 patients (nine females and four males) with primary intraspinal pPNETs who were surgically treated from April 2008 to February 2014. Histopathologic findings revealed the expression of CD99 in all cases. Limb weakness was the most common initial symptom (11/13, 85 %). The tumors were located mainly at the cervical level (6/13, 46 %) and in the epidural space (10/13, 77 %). The radiological diagnosis was neurinoma or meningioma in most cases (10/13, 77 %). Gross total resection was achieved in 77 % (10/13) of patients. During a mean follow-up of 25.5 months, local relapse occurred in 8 (61.5 %) patients and distant metastases occurred in 8 (61.5 %) patients. The overall 1-year survival rate was 77 % (10/13), and the overall 2-year survival rate was 54 % (7/13). The 2-year survival rate was 57.1 % in patients with adjuvant chemotherapy and 50 % in those without chemotherapy. Gross total resection and adjuvant radiotherapy with or without chemotherapy demonstrated a longer survival period (1-year survival rate: 100 %; 2-year survival rate: 86 %). Our data showed that primary spinal pPNETs are extremely rare and aggressive tumors with a poor prognosis. Radical resection is advocated. Gross total resection combined with adjuvant radiation may help to significantly improve patient survival period. Chemotherapy may also help to slightly prolong patient life.

A 50 year old white man received an incidental ultrasound diagnosis of hypoechoic mass interesting the right seminal vesicle. A CT scan showed the presence of a 7.8 cm roundish cyst, originating from the right seminal vesicle. He had been followed by the removal of the right seminal vesicle and both the cystic lesion. The histological findings of the specimen documented the presence of small round cells compatible with Ewing's sarcoma/PPNET. The patient received also adjuvant chemotherapy and radiation treatment. After 10 years, the follow-up is still negative.

Neonatal tumors comprise less than two percent of childhood malignancies. Most are solid tumors, most common histologies being teratoma and neuroblastoma. We encountered a child who was detected to have a right arm mass on antenatal sonogram, which was diagnosed to be a primitive neuroectodermal tumor involving the triceps on fine needle aspiration cytology performed in the post-natal period. The child was successfully treated with multimodality treatment consisting of surgery, chemotherapy and radiotherapy. We also discuss briefly the problems associated with therapy in neonatal period. A review of all cases reported to have congenital Ewing's sarcoma family of tumors is presented. Novel therapies are needed to improve efficacy and decrease the devastating side effects of treatment in this age group., (Copyright © 2014. Production and hosting by Elsevier B.V.)

Ewing sarcoma/peripheral primitive neuroectodermal tumors (ES/pPNETs) typically occur in the long or flat bones, the chest wall, extraskeletal soft tissue, or less frequently, in solid organs. They can arise from anywhere in the body; however, ES/pPNETs arising from the adrenal gland are very rare, especially in children and adolescents. Herein, the authors report a case of an ES/pPNET in the adrenal gland of a 17-year-old girl, who was successfully treated with a multimodal treatment, with a brief review of the pertinent literature.

Objective: The aim of the study was to investigate the clinical manifestations, diagnosis, treatment, and prognosis of primitive neuroectodermal tumors (PNETs) in the female genital tract., Methods: From April 2001 to May 2013, the clinicopathologic characteristics, treatments, outcomes, and prognosis of 11 patients with PNET in the female genital tract were analyzed retrospectively at our hospital., Results: The location of PNET in the 11 patients presented here included vulva (2 patients), cervix (2 patients), uterus and its ligament (5 patients), and the ovaries (2 patients). Ages ranged from 18 to 59 years (median, 31 years).The main clinical manifestations of PNET in the female genital tract are irregular vaginal bleeding (6 patients), pelvic mass, uterine enlargement, and rapidly increasing vulvar mass (8 patients), and vulvar pain and lower abdominal pain (5 patients). The CA125 levels of 8 patients were elevated before the operations and reduced to normal when the diseases were controlled, while the levels increased as the tumor was progressive. Results for the most commonly used immunohistochemistry studies revealed CD99 in 11 of the 11 tumors, synaptophysin in 6 of the 7 positive tumors, and neuron-specific enolase in 6 of the 6 tumors. Ten patients underwent surgical resection. Nine of them underwent preoperative or/and postoperative combination chemotherapy. The follow-up of 10 patients were available and ranged from 1 to 145 months (median, 30.5 months), 3 of whom experiencing recurrence., Conclusions: Primitive neuroectodermal tumor is very rare and can originate from any part of the female genital tract. The tumors had different manifestations but the same pathologic features. CA125 may be an important marker for prognosis and follow-up of PNET of the female internal genital tract.

Ewing's sarcoma, a primitive neuroectodermal tumour is a rare entity belonging to a family of neoplasms of neuroectodermal origin. These highly aggressive neoplasms primarily affect older children and young adults. They primarily involve the bones or soft tissues. A quarter of Ewing's sarcomas occur principally in the soft tissues with primary involvement of the kidney being exceptionally rare. We hereby, present a case of 6 years old girl child with primary Ewing's sarcoma of the kidney who presented with abdominal mass and posed much diagnostic challenge. The patient underwent complete surgical excision of tumour, and is being treated with aggressive adjuvant chemotherapy.

Primitive neuroectodermal tumors of the cervix are very rare. A 28-year-old pregnant woman presented with a cervical mass. The tumor was staged as IB2. The biopsy from tumor was suggestive of malignant small round cell tumor. She then underwent termination of pregnancy followed by radical hysterectomy. Based on morphologic and immunohistochemical profile, a diagnosis of peripheral primitive neuroectodermal tumor of the cervix was made. The patient received adjuvant chemotherapy and radiotherapy. The patient is alive and disease-free 33 months post-surgery. The present case highlights the importance of keeping primitive neuroectodermal tumors in the differential diagnosis of small cell neoplasms of the uterine cervix. Pregnancy should not be a barrier to early detection and treatment of this potentially aggressive tumor. The optimal treatment methods have not yet been established because of the rarity of the tumor.

Data on prognostic factors in pelvic PNET are minimal. We analyzed patients with pelvic PNET treated between June 2003 and November 2011 for prognostic factors. Forty-eight (13%) of 374 patients with PNET were pelvic PNET with median age 14.5 years (range: 5-33); 31 (65%) had metastases. After median follow-up of 20.4 months (range: 1.3-64.9), 3-year EFS, OS, and local-control-rate were 13.5 ± 5.5%, 15.4 ± 9%, and 41.3 ± 14.9%, respectively. Hypoalbuminemia (≤3.4 g/dl) predicted inferior EFS and OS for both entire cohort and metastatic group. All patients with hypoalbuminemia (n = 10) had low BMI as compared to 23/38 without hypoalbuminemia (P = 0.02)., (Copyright © 2013 Wiley Periodicals, Inc.)

Objectives: Thoracic primitive neuroectodermal tumour is an aggressive malignancy with poor survival despite multimodality treatment regimens. Early diagnosis of the tumour by histological, immunohistochemical, ultrastructural and cytogenetic techniques and early total surgical resection of the tumour with intensive chemoradiation may improve outcomes., Methods: Over 30 years, 23 patients (median age 29.5) with primitive neuroectodermal tumours (15 chest wall, 4 lung, 3 costovertebral sulcus and 1 anterior mediastinum) were diagnosed by transthoracic needle biopsy (43%) or excisional biopsy (57%). Treatment of a localized disease (Stage I and II) in 19 patients included surgery (wide excision of chest lesions in 11, 4 lung resections, excision of 3 costovertebral sulcus and 1 anterior mediastinal tumours, and resection of adjacent tissues involved by tumour en bloc) with adjuvant chemoradiation. Four metastatic chest wall tumours (Stage III) had chemotherapy and radiation alone., Results: Tumour recurred in 5 (2 chest wall, 2 costovertebral sulcus and 1 lung) requiring further chemotherapy, radiation and completion pneumonectomy for a lung recurrence. The incidence of recurrent tumour in 7 years for Stage I was 21 vs 40% (P=0.4) for Stage II lesions and 16% after the neoadjuvant chemotherapy vs 30% (P=0.4) after adjuvant chemoradiation. Four with recurrence, except one with a chest recurrence, succumbed to second relapse (78-96 months). All four Stage III chest tumours succumbed to advanced disease (30 months). The Kaplan-Meier disease-free survival of the overall group (23 patients) was 82±2% at 5 years and 64±3% at 10 years. The 10-year disease-free survival of 19 patients with localized tumours was 76%, but was high at 90% for chest wall tumours and low 33% for costovertebral sulcus tumours (P≤0.01). The 10-year disease-free survival was 86% for Stage I vs 60% (P=0.02) for Stage II tumours; and 83% for neoadjuvant vs 76% (P=0.06) for adjuvant chemotherapy and radiation., Conclusions: The primitive neuroectodermal tumours are aggressive neoplasms with poor prognosis. Early diagnosis and total surgical excision of localized tumours with neoadjuvant or adjuvant chemotherapy and radiation improved disease-free survival.

Objective: To collect and analyze clinical and pathological features of primitive neuroectodermal tumor (PNET)/Ewing sarcoma (EWS), a rare tumor occurring most commonly in bone and soft tissues of young people, which rarely occurs as a primary renal neoplasm and exhibits highly aggressive biological behavior., Methods: All cases of PNET/EWS published from 1975 to February 2012 were collected. When available, clinical and pathological data were extracted for each case. Survivals were estimated with the Kaplan-Meier method and compared with the log-rank test with 95% confidence interval (CI)., Results: A total of 116 cases were found. All patients had clinical symptoms as first presentation of disease such as pain (54%), hematuria (29%), and bulky renal mass (28%). Sixty-six percent of patients had stage IV disease at diagnosis. Median disease-free survival (DFS) was 5.0 months (95% CI 2.4-7.6). The probability to be alive at 18 months was 60% and 85% for patients with metastatic disease (M1) or not (M0) at diagnosis, respectively. Median overall survival (OS) was 24 months (95% CI 4.5-15.1) in patients with M1 disease, whereas it was not reached in patients with M0 disease (P <.001). In patients with M0 disease, 50% received neoadjuvant chemotherapy and the 12-month OS was 93% compared to 75% of untreated patients (P = .092). In patients with M1 disease who underwent treatment, the median progression-free survival (PFS) was 22.0 months (95% CI 17.9-26.1) with a clinical benefit in 74% of cases., Conclusion: Our findings suggest that PNET/EWS is a rare aggressive tumor affecting principally young people, with a poor prognosis for patients with M1 disease; chemotherapy is an effective strategy in M1 disease and probably also in M0 disease., (Copyright © 2013 Elsevier Inc. All rights reserved.)

We report a rare case of Ewing's sarcoma (ES)/primitive neuroectodermal tumor (PNET) arising from the adrenal gland. A 17-year-old Japanese woman presented with left upper abdominal pain and high fever. Computed tomography and magnetic resonance imaging revealed a 15 × 10 cm tumor replacing the adrenal gland. Preoperative diagnosis was an adrenocortical carcinoma. Resection of the tumor was performed. We obtained the final diagnosis of ES/PNET by immunohistochemical molecular study with positive staining for the MIC2 gene product (CD99) and a Ewing sarcoma breakpoint region 1 (EWSR1) gene rearrangement. Local recurrence was observed one month after the surgery. The patient was then treated with systemic chemotherapy and localized radiotherapy.

The Ewing family of tumors and peripheral primitive neuroectodermal tumor (pPNET) represent different manifestations of the same entity. Immunohistochemical and cytogenetic studies suggest that these tumors have a common origin. Ewing sarcoma is more common in bone, while pPNET is more common in soft tissues. Extraosseous Ewing sarcoma (EoES) is rare. We present the case of a 48-year-old man who presented with acute obstructive respiratory failure secondary to a large thyroid swelling. The patient was initially diagnosed with giant B-cell non-Hodgkin lymphoma and treated with chemotherapy. However, subsequent immunohistochemical staining of biopsy specimens revealed that the patient actually had EoES/pPNET of the thyroid gland. We performed a nearly complete surgical resection of the tumor plus a total laryngectomy and resection of five tracheal rings. However, the patient died of a cerebral metastasis 1 month later after he had completed one cycle of postoperative chemotherapy.

Primitive neuroectodermal tumour (PNET) is a neural crest tumour derived from neuroectoderm. Renal PNET is a very rare tumour occurring during childhood or adolescence. We report two cases of PNET involving kidney in adults. Presenting signs and symptoms include abdominal/flank pain and/or haematuria. Microscopy reveals the tumour consisted of small round cells with round nuclei and scant cytoplasm. Diagnosis was confirmed by immunohistochemistry with diffuse membranous positivity of tumour cells with CD99. As these tumours have an aggressive clinical course with rapid death in many reported cases, it is important to differentiate them from other small round-cell tumours.

Reported here is a case of Müllerian adenosarcoma of the ovary which contained a primitive neuroectodermal tissue component within the stroma. The adenosarcoma coexisted with clear cell adenocarcinoma in an endometriotic cyst. The patient was a 33-year-old woman with a large unilocular endometriotic cyst of the right ovary. On the internal wall of the cyst, both a plaque-like protrusion with a papillary surface and a dome-like mural nodule were noted. The former exhibited features of Müllerian adenosarcoma, and the latter showed those of clear cell adenocarcinoma. In the deeper portion of adenosarcoma, teratoma-like tissue which contained various tissue components including primitive neuroectodermal tissue was found. The presence of primitive neuroectodermal tissue in the stroma of adenosarcoma suggested the diagnosis of 'adenosarcoma with neuroectodermal differentiation' ('teratoid adenosarcoma'), although the possibility of the incidental occurrence of an immature teratoma could not be completely excluded., (© 2011 The Authors. Pathology International © 2011 Japanese Society of Pathology and Blackwell Publishing Asia Pty Ltd.)

Extraosseous Ewing's sarcoma/primitive neuroectodermal tumor (ES/PNET) is an uncommon, aggressive, and malignant tumor with a poor patient outcome. Its occurrence in the lesser sac is a rare event and to the best of our knowledge, has not been previously described. The present case was clinically and radiologically misdiagnosed as a pancreatic tumor/gastrointestinal stromal tumor. Histopathology revealed a tumor with "small round cells" that were positive for CD99, confirming the diagnosis of ES/PNET. This report highlights the importance of considering Ewing's sarcoma in the differential diagnosis of intraabdominal, extraintestinal masses.

Background Aims: Adoptive T-cell therapy with tumor-specific T cells has emerged as a potentially useful approach for treating patients with advanced malignancies. We have demonstrated previously the feasibility of obtaining large numbers of autologous anti-tumor-specific cytotoxic T lymphocytes (CTL) generated by stimulation of patients' peripheral blood mononuclear cells with dendritic cells pulsed with apoptotic tumor cells. Methods. Six patients with progressing metastatic solid tumors (one renal cell carcinoma, two ovarian cancers, two extraosseous peripheral neuroectodermal tumors, one soft tissue sarcoma) not eligible for conventional therapies were treated with adoptive immunotherapy. Anti-tumor CTL, proven to be reactive in vitro against patient tumor cells, but not against normal cells, were infused following lymphodepleting chemotherapy administered to favor T-cell proliferation in vivo., Results: Patients received a median of nine CTL infusions (range 2-19). The median number of CTL administered per infusion was 11 × 10(8) (range 1-55 × 10(8)). No patient experienced acute or late adverse events related to CTL infusion, even when large numbers of cells were given. Post-infusion laboratory investigations demonstrated an increase in the frequency of circulating anti-tumor T-cells and, in patients with a longer follow-up receiving two CTL infusions/year, a stabilization of these values., Conclusions: Our study demonstrates that autologous ex vivo-generated anti-tumor CTL can be administered safely in patients with advanced solid tumors and can improve the immunologic reactivity of recipients against tumor. These preliminary results provide a rationale for evaluating the clinical efficacy of this immunotherapeutic approach in phase I/II studies.

Ewing tumor is the second most common bone tumor in children. Its variant, malignant small cell tumor of the thoracopulmonary region, is infrequent in children and extremely rare in adults. A multimodal treatment approach is preferred for this tumor. We describe a rare case of primitive neuroectodermal tumor/Ewing sarcoma arising from the thoracic wall in a 54-year-old man. He underwent extensive resection of the tumor followed by adjuvant chemotherapy and radiotherapy.

Objective: Though classically thought of as an osseous-based sarcoma of children and adolescents, it is important to recognize that approximately 20% of all cases of Ewing sarcoma family of tumors (ESFT) occur in patients older than 18 years, often with extraskeletal disease. Understanding the specific imaging features, manners of presentation, and patterns of behavior of ESFT is therefore important in all settings., Conclusion: This article presents the imaging findings of ESFT in the adult, with specific attention to extraskeletal manifestations.

Askin tumor is an uncommon malignant neoplasm in the thoracopulmonary region mainly occurring in children and adolescents. Four young patients with histologically proven Askin tumors were treated in our hospital. In all patients, chest computed tomography imaging demonstrated a chest wall mass with or without destruction of ribs. All patients underwent radical mass resection and postoperative chemotherapy. By the time this article was completed, two of the patients had died with local chest wall recurrences. Here we focus on the imaging features, differential diagnosis, pathology and prognosis of this rare disease.

We review the case of an adolescent who presented with flank pain, fatigue and a discrete nonfunctioning adrenal lesion which was found to be an adrenal Ewing sarcoma/primitive neuroectodermal tumor (ES/PNET). The patient was treated with a minimally invasive adrenalectomy as a component of multimodal therapy, including seven courses of chemotherapy and whole abdominal radiation. She is currently disease free 14 months after the operation and 3 months off therapy., (Copyright © 2011 Wiley-Liss, Inc.)

Peripheral primitive neuroectodermal tumours (pPNETs) are a group of soft-tissue tumours of neuroepithelial origin that arise outside the central and sympathetic nervous system. Orbital location is infrequent, and to the best of the authors' knowledge only 16 cases have been reported in the literature. With this article, the authors report the demographics and clinical characteristics, diagnostic features, differential diagnosis, prognosis and therapeutic options of primary orbital peripheral primitive neuroectodermal tumour, based on their patients and on the cases reported in the literature to date. A differential diagnosis should be made with other small round cell tumours; immunohistochemical and ultrastructural techniques are essential for this purpose. Although bone invasion and extraorbital extension are possible, systemic metastases are uncommon in the cases of orbital pPNETs. Surgery has been the initial treatment in most cases; chemotherapy with or without radiotherapy is considered the best additional treatment. The orbital pPNET could be less aggressive than other forms of pPNETs, since most of the patients reported were alive after the follow-up period (at least 6 months).

Peripheral primitive neuroectodermal tumor of the chest wall is a rare malignant tumor usually occurring in children. The tumor shares a similar histology, immunohistology and cytogenetics to Ewing's sarcoma. The location of the tumor in the chest wall poses a major challenge with respect to the diagnostic workup and treatment which involves multidisciplinary management. Neoadjuvant chemotherapy is given initially and is followed by complete surgical resectioning of the mass followed by repeat chemotherapy with or without radiotherapy. We report a case of peripheral primitive neuroectodermal tumor of the chest wall in a 5-year-old boy and review the literature regarding its diagnosis and management.

Purpose: To evaluate the prognostic factors and treatment outcome of patients with Askin-Rosai tumor of the chest wall treated at a single institution., Methods and Materials: Treatment comprised multiagent chemotherapy and local therapy, which was either in the form of surgery alone, radical external-beam radiotherapy (EBRT) alone, or a combination of surgery and EBRT. Thirty-two patients (40%) were treated with all three modalities, 21 (27%) received chemotherapy and radical EBRT, and 19 (24%) underwent chemotherapy followed by surgery only., Results: One hundred four consecutive patients aged 3-60 years were treated at the Tata Memorial Hospital from January 1995 to October 2003. Most (70%) were male (male/female ratio, 2.3:1). Asymptomatic swelling (43%) was the most common presenting symptom, and 25% of patients presented with distant metastasis. After a median follow-up of 28 months, local control, disease-free survival, and overall survival rates were 67%, 36%, and 45%, respectively. Median time to relapse was 25 months, and the median survival was 76 months. Multivariate analysis revealed age ≥18 years, poor response to induction chemotherapy, and presence of pleural effusion as indicators of inferior survival. Fifty-six percent of patients with metastatic disease at presentation died within 1 month of diagnosis, with 6-month and 5-year actuarial survival of 14% and 4%, respectively., Conclusion: Primary tumor size, pleural effusion, response to chemotherapy, and optimal radiotherapy were important prognostic factors influencing outcome. The combination of neoadjuvant chemotherapy, surgery, and radiotherapy resulted in optimal outcome., (Copyright © 2011 Elsevier Inc. All rights reserved.)

Objective: To observe the expression of FLI-1 in primitive neuroectodermal tumors (PNET), explore the value of immunohistochemical staining of FLI-1 in combination with other neural markers in diagnosis of PNET, and analyze the prognostic factors in PNET patients., Methods: 35 cases of PNET, of which 33 cases with complete clinical data, were included in this study. Immmunohistochemistry (The En Vision method) was applied to detect the expression of FLI-1, CD99, Syn, NSE, S-100, NF, Vim in the tumor tissues. The clinicopathological data of 33 cases were analyzed by Cox regression., Results: The positive expression rate of FLI-1 were 51.4% and that of CD99 was 88.6%. The sensitivity of FLI-1 combined with CD99 was up to 100%. The positive rates of Vim, Syn, NSE, s-100 and NF were 91.4%, 48.6%, 45.7%, 22.9% and 0, respectively. Cox regression analysis showed that the impact of primary location and treatment modality were of statistical significance (P < 0.05), but the age, sex, stage or size of tumors did not (P > 0.05)., Conclusion: Immunohistochemical detection of FLI-1 and neural markers is a preferred method for clinical diagnosis of PNET. The main factors affecting the prognosis are the primary location of PNET and treatment modality.

Ewing's sarcomas/peripheral primitive neuroectodermal tumors (ES/pPNETs) are high-grade malignant neoplasms rarely found outside the skeletal system. Only 12 cases of vulvar ES/pPNET have so far been reported, all involving children or women of child-bearing age. We describe the case of a 52-year-old woman who was admitted to our hospital for the local excision of a 4cm vulvar mass, originally thought to be a Bartholin's gland cyst. It was subsequently found to consist of small round cells positive for anti-CD99 antibody, thus suggesting a diagnosis of ES/pPNET. The demonstration of EWSR1 gene translocations by means of fluorescent in situ hybridization excluded small-cell carcinoma, squamous cell carcinoma of the small type, Merkel cell carcinoma, and lymphoblastic lymphoma. After surgery, the patient received six cycles of polychemotherapy and radiotherapy; she is still alive and well after 1 year of follow-up. Our findings underline the crucial role of molecular biology techniques in the differential diagnosis of small round cell tumors in these unusual locations., (Copyright 2009. Published by Elsevier GmbH.)

Ewing tumours are characterised as tumours consisting of small, blue, round malignant cells that may exhibit varying degrees of neural differentiation. Most of them arise in bony sites, and they represent the second commonest primary osseous malignancy in and adolescence and young adults. During the past 30 years, chemotherapy has increased survival from less than 5% to 65-70% in localized tumours and to 25-30% in primary metastatic tumours. Surgery is a major tool, whereas advances in imaging techniques have improved treatment indication and optimization. Radiotherapy remains useful, either alone or in addition to surgery, and new techniques (conformational RT and IMRT) will reduce short-term toxic effects. However, long-term toxic effects are also of major concern. Clinical and biological prognostic factors has been clearly identified and should guide the therapeutic choice for these patients. The metastatic Ewing tumours are of extremely poor prognosis, and impose the development of new therapeutic agents. This article is a review of the data available in 2009 concerning Ewing's sarcoma either as biologic aspects or as therapeutic aspects.

We describe a rare case of primitive neuroectodermal tumor arising from the thoracic wall in a 48-year-old man. He underwent extensive resection of the tumor en bloc with the diaphragm and the thoracic wall, followed by adjuvant chemoradiotherapy. Primitive neuroectodermal tumor may be included in a multidisciplinary protocol incorporating neoadjuvant chemotherapy, surgery, and adjuvant chemoradiotherapy.