Congenital primitive neuroectodermal tumor (neuroepithelioma) of the chest wall

Primitive neuroectodermal tumor (neuroepithelioma) is a relatively common central nervous system tumor in children. Those arising from a peripheral nerve are extremely rare in childhood. There is only one reported case in a 6-year-old where the tumor arose from the sciatic nerve. A case of neuroectodermal tumor of the chest wall, arising from the intercostal nerve, in a newborn is presented. The tumor metastasized to the brain. Prominent Homer-Wright rosettes, with central eosinophilic fibrillar substance similar to that seen in neuroepithelioma of the central nervous system, were present in the primary tumor and brain metastases. Ultrastructure, as revealed by transmission electron microscopy, is also described.     

Monoclonal antibody MB2: a potential marker for Ewing's sarcoma and primitive neuroectodermal tumor

The small round-cell tumors of childhood present difficulties in diagnosis when differentiation is not apparent. Immunohistochemistry is helpful; however, the only antigen consistently detected in Ewing's sarcoma is vimentin, which may also be detected in the other types of small-cell neoplasms. The monoclonal antibody (MAb) MB2 is marketed as a B-lymphocyte marker that can be used on paraffin-embedded tissue. To determine its specificity, we performed immunohistochemical staining on pediatric tumors with MB2. These included 55 cases of small round-cell tumors (lymphomas, Ewing's sarcoma, peripheral primitive neuroectodermal tumors [PNET], neuroblastomas, rhabdomyosarcomas, and nephroblastomas). MB2 positivity was detected in all B-cell lymphomas and in seven of nine cases of Ewing's sarcoma and three PNET. In neuroblastomas only differentiating ganglion cells were positive. In rhabdomyosarcomas only large rhabdomyoblasts were positive. Blastema of nephroblastomas was negative. Thus, in cases of poorly differentiated small round-cell tumors, MB2 was positive in all B-cell lymphomas, most Ewing's sarcomas and all cases of PNET. Lymphomas were distinguished by staining for leukocyte-common antigen and PNET by neuron-specific enolase. Therefore, the addition of MB2 to a discrete panel of antibodies may prove useful in the diagnosis of Ewing's sarcoma and PNET.     

Ewing sarcoma/primitive neuroectodermal tumor of the kidney treated with chemotherapy including ifosfamide

Ewing sarcoma/primitive neuroectodermal tumor (ES/PNET) of the kidney is extremely rare, and is usually diagnosed after nephrectomy without neoadjuvant chemotherapy. Although ifosfamide and etoposide improve survival to a great extent in ES/PNET, the use of nephrotoxic agent, particularly ifosfamide, is a concern after nephrectomy. We describe the case of a 14‐year‐old female patient with abdominal mass who was diagnosed with ES/PNET of the right kidney after nephrectomy. Adjuvant chemotherapy including ifosfamide and etoposide were given. The estimated glomerular filtration rate decreased to 75% after the end of therapy. There was no evidence of recurrence 70 months after initial diagnosis.     

Ewing sarcoma/primitive neuroectodermal tumor arising from the adrenal gland in an adolescent

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.     

Extracranial primitive neuroectodermal tumor

An 11-year-old white girl was admitted to The Children's Hospital of Philadelphia in July 1983 for evaluation of metastatic tumor. She had been well until July 1982, when a mass developed over the right scapula. Treatment with warm compresses and antibiotics resulted in no improvement, and incision and drainage were unproductive. A biopsy at another hospital was interpreted as showing a primitive neuroectodermal tumor. There was no evidence of metastatic disease. She then underwent excision of the tumor with the underlying scapula. No further treatment was administered. She remained well until February 1983, when she began to complain of occasional lower back and thigh pains. Her symptoms worsened over the succeeding 3 months, despite treatment with analgesics and physical therapy. By May 1983, she was no longer able to attend school because of weakness and pain, and had sustained a 10% weight loss during the previous 2 months. She was admitted to her original hospital, where bone scan and bone marrow biopsy showed disseminated tumor; she then came to The Children's Hospital of Philadelphia. On examination, she appeared acutely and chronically ill. It was very uncomfortable for her to move, and she walked with a slow, stooped, shuffling gait. She complained of tenderness in the lower back and both sides. There were no other abnormalities on examination. The hemoglobin level was 9.7 gm/dl, following transfusion at the other hospital; white blood cell count was 6,900/mm3 with a normal differential, and the platelet count was 480,000/mm3. A 24-hr urine test for VMA excretion was normal. She underwent bone marrow aspiration and biopsy, and the radiographs and pathology slides from the other hospital were reviewed.     

Diagnostic imaging of primitive neuroectodermal tumour of the chest wall (Askin tumour)

Objectives. To describe the radiological features of primitive neuroectodermal tumour (PNET) of the chest wall (Askin tumour) at diagnosis and to analyse the radiological changes occurring as a consequence of treatment and during follow-up. Materials and methods. Nine children with histologically proven PNET were studied. At diagnosis, all patients underwent chest X-ray (CXR), chest CT and bone scintigraphy; three patients also had MR and three had US. During treatment and follow-up, CT was performed in all patients. Results. CT demonstrated a solid heterogeneous chest wall mass in all children at diagnosis and six had a rib lesion. Small nodular densities in the extra-pleural fat were identified in three patients at diagnosis. US, performed in three patients, excluded tumour infiltration of the lung or diaphragm, which had been suspected on CT. On MR, the lesions showed high signal intensity in T1-weighted/proton-density images and intermediate/high signal intensity in T2-weighted images compared with muscle. Minimal chest wall involvement was demonstrated in one case by MRI. Extensive necrosis of tumour mass with pseudo-cystic appearance was documented in the five patients who underwent chemotherapy. Macroscopically complete resection was performed in five patients but there was early local recurrence after surgery in two, identified by CT in one and by MR in the other. Conclusions. PNET of the chest wall should be considered in a child with a chest wall mass. CT is valuable for evaluating tumour extension at diagnosis, the effects of chemotherapy and assessing tumour recurrence after surgery. However, CT can overestimate pleural, lung or diaphragmatic infiltration, which are better evaluated by US. MR was superior to CT in the evaluation of tumour extension in one of three patients and may be considered complementary to CT, particularly in very large chest wall tumours. Received: 23 May 1997 Accepted: 23 February 1998     

Localized sarcoma of the chest wall

This 9-year-old white male suffered mild trauma to the left side of the chest on two occasions in April 1981, and then developed a low-grade fever. A chest x-ray film showed pneumonia. He was then given oral ampicillin for 2 weeks, and the fever and left chest pain resolved. Repeat chest radiograph one month later showed a persistent density, and a bone scan revealed increased uptake in the left fifth rib. Osteomyelitis was diagnosed, and intravenous antibiotics were administered. In June 1981, the density had become larger on radiographic examinations, and a left chest wall mass was noted for the first time. He was referred to the Children's Hospital of Philadelphia, with the suspected diagnosis of Ewing's sarcoma of the left fifth rib. On arrival at the Children's Hospital of Philadelphia on June 13, 1981, the physical examination was unremarkable except for a 1 X 2-cm mass overlying the fifth rib anterolaterally. Dr Hugh Watts of the Division of Orthopedic Surgery reviewed the radiographs and bone scan and felt that the appearance was not consistent with primary Ewing's sarcoma of the rib. The boy was then taken to surgery by Dr. Moritz Ziegler on June 23, 1981, at which time biopsy of the lesion was accomplished. The tumor had by then grown to a size of 3 X 5 cm.     

Primary primitive neuroectodermal tumor of the lumbar extradural space

Primary spinal primitive neuroectodermal tumors (PNETs) of the extradural space are very rare; only 10 cases have been reported in the English language literature. The histopathological diagnosis of primary spinal PNETs has been discussed for many years. These tumors have a rapidly progressive course, and there is no current consensus on the optimal therapeutic approach for these patients. The authors present a case of primary PNET located in the lumbar extradural space in a 13-year-old girl and report the clinical, radiological, histopathological, and surgical findings. They compare their findings with those from the other 10 cases reported in the literature and review relevant literature.     

Therapy and outcome of orbital primitive neuroectodermal tumor

Primary orbital primitive neuroectodermal tumor (PNET) is rare with no reported series. We report six cases of orbital PNET treated at a tertiary care oncology center in northern India from 2003 to 2008. None of them had distant metastases. All were treated with neoadjuvant chemotherapy followed by exenteration in two, radiotherapy and adjuvant chemotherapy in five cases. Three out of six achieved complete remission at end of therapy with globe salvage in three and vision in two cases. Chemoradiotherapy may help us to avoid mutilating surgery in large or locally advanced tumors, allowing preservation of vision or the globe. Pediatr Blood Cancer 2009;52:544–547. © 2008 Wiley‐Liss, Inc.     

p21 (WAF1/Cip1/Sdi1/Pic1) mRNA is expressed in neuroblastoma cell lines but not in Ewing's sarcoma and primitive neuroectodermal tumor cell lines

The p21 protein inhibits the activity of cyclin-Cdk complexes and suppresses cell cycle progression. Wild type p53 can induce p21, but mutated p53 cannot. Previous studies have demonstrated that mutation of p53 is absent in neuroblastoma (NB). These reports prompted us to examine whether p53 induced p21 in NB. We examined the expression of p21 and p53 mRNA in eight NB, two Ewing's sarcoma (ES) and two primitive neuroectodermal tumor (PNET) cell lines by Northern blot analysis, and sequenced p53 cDNA of these cells. Although p53 mRNA was detected in all analyzed cell lines by Northern blot analysis, p21 mRNA was detected in six NB but not in two NB, two ES and two PNET cell lines. We detected the point mutation of p53 at codon 273 (CGT to TGT) in one NB and two ES cell lines. The non-transforming substitution at codon 72 (CCC to CGC) was detected in all analyzed cell lines. One PNET cell line had a large deletion of p53 cDNA. These results showed that p21 mRNA was usually expressed in NB but not in ES and PNET. This may suggest that the down stream of the p53 signal transduction pathway in NB is different from that of the closely related tumors of ES and PNET.     

Therapy and outcome of primitive neuroectodermal tumor of the jaw

Data pertaining to outcomes in jaw primitive neuroectodermal tumor (PNET) are lacking. Eleven cases of jaw PNET (five maxillary and six mandibular) were treated at our cancer center with the same chemotherapeutic agents from June 2003 to January 2009. Four patients underwent surgery after neoadjuvant chemotherapy and all received local radiotherapy. At median follow-up of 56 months (range: 19-77 months), 7/11 patients are in sustained remission. There was no difference in outcome with respect to site of tumor, and whether surgery was performed or not. These results support the use of chemoradiation as initial modality of treatment rather than going for extensive and mutilating surgery.     

Late events in pediatric patients with Ewing sarcoma/primitive neuroectodermal tumor of bone: the Dana-Farber Cancer Institute/Children's Hospital experience

The outcome for 82 pediatric patients with Ewing sarcoma (ES) and primitive neuroectodermal tumor (PNET) of bone is reported; the patients were treated at the Dana-Farber Cancer Institute (DFCI) and Children's Hospital (CH) in Boston, MA (USA) from 1971-1988. The charts of all patients with ES/PNET of bone treated during this period were reviewed for disease status, therapy, sites of relapse, information on second malignancies, and survival status. Eighty-two patients with ES/PNET of bone treated at DFCI/CH were identified. The 10-year event-free survival (EFS) rates were 12% (95% confidence interval [CI] 0, 27%) and 38% (95% CI 26, 51%) for patients with and without metastases, respectively (P = 0.002); the overall survival (OS) rates were 17% (95% CI 1, 33%) and 48% (95% CI 35, 61%) for patients with and without metastases (P = 0.001). Median follow-up for surviving patients is 10.2 years. Primary site in the pelvis also was associated with a poor outcome for patients with no metastatic disease (P = 0.006 OS, P = 0.03 EFS). Thirty-one patients survived in first remission at least 5 years from diagnosis, and of these, five experienced relapse of original disease, and five experienced secondary malignancies. Pediatric patients treated for ES/PNET of bone remain at risk for life-threatening events into the second decade of follow-up. After 5 years, the risk of second malignant neoplasm is at least as high as the risk of late relapse. Prolonged follow-up of patients with ES and PNET of bone is indicated.     

So-called primitive neuroectodermal tumor in aborted previable fetuses

We have described the gross anatomic and histopathologic characteristics of 36 tumorlike lesions found in previable fetuses. Although these lesions appear to be invasive, metastasizing, and likely contributing to the death of the fetus, they represent a frequent finding only among severely macerated retained fetuses of 9-16 developmental weeks and have never been found in fresh nonretained fetuses. No family history of neurofibromatosis could be elicited in any of these cases to support the suggestion in the literature that these tumors may represent fetal manifestations of neurofibromatosis. The most likely explanation is that they represent an artifact owing to squeezing of brain tissue into the spinal canal and along the spinal nerves into retroperitoneal and retropleural spaces or the neck area. As this artifact appears to be confined to a specific developmental period (9-16 weeks), it may be related to certain developmental characteristics such as a very soft consistency of brain tissue or incomplete development of vertebral foramina. A higher incidence of these lesions among heteroploid fetuses remains unexplained.     

新生儿纵隔原始神经外胚层肿瘤1例报告

<正>患儿男,因窒息后复苏21 h入院。患儿为第2胎第2产,胎龄39+2周,顺产出生。患儿母亲孕早期无用药史、放射接触史,定期产检。羊水、胎盘未见异常,脐带绕颈2周,出生体重3.4 kg。Apgar评分1 min 4分,5 min 5分,10 min 6分。出生后患儿逐渐出现呻吟样呼吸,皮肤紫绀,抽搐1次,予抗惊厥、镇静、纠正酸中毒、气管插管呼吸机辅助呼吸等急救处理。经过治疗后,患儿症状无好转,继而出现肺出血,予以止血、输注新鲜血浆及抗感染等处理。体查:T 36.8℃,P 140次/分,R 35次/min,Bp 50/35 mm Hg,头     

Congenital peripheral primitive neuroectodermal tumor refractory to treatment

The authors report an unusual case of an infant presenting with a congenital peripheral primitive neuroectodermal tumor (PPNET) of the left hand refractory to treatment. A newborn girl was born with a large bluish-red mass of 4.5 cm diameter protruding into the palm and the dorsum of the left hand. Tumor biopsy confirmed the diagnosis of PPNET. The initial metastatic workup for the detection of metastases was negative. Four cycles of chemotherapy according to CCSG 7881/POG 8850 regimen B were given. Despite this aggressive chemotherapy the tumor grew to involve the entire left hand. The left hand was amputated, and then two cycles of topotecan/cyclophosphamide were given. Five months later extensive metastases developed, involving the brain, lungs, liver, and skeleton, and the child died at the age of 2.5 years. PPNET presenting at birth is uncommon; presentation in the hand is unusual, and the fact that it did not respond to treatment is still more uncommon.