Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0027627 (metastases)
 103,950 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Primitive neuroectodermal tumor of the sternum is rare. A 59-year-old woman referred to our department with anterior chest pain and a tumor in the sternum. The patient was diagnosed as primitive neuroectodermal tumor of the sternum by core biopsy of the lesion. She received 2 cycles of preoperative chemotherapy with vincristine, doxorubicin, cyclophosphamide, ifosfamide, etoposide. She underwent a total sternectomy with resection of adjacent bilateral costal cartilages and sternal ends of the clavicles. The skeletal defect of chest wall was reconstructed by polypropylene mesh-resin sandwich. The myocutaneus defect was reconstructed by the pedicled latissimus dorsi myocutaneus flap and the bilateral breast flaps. The postoperative course was uneventful and adjuvant radiotherapy was started 6 weeks after the operation. She died of distant metastases 3 months after the operation, although this patient was free from local recurrence.
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PMID:[Sternal resection and chest wall reconstruction for primitive neuroectodermal tumor of the sternum]. 1878 70

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.
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PMID:Therapy and outcome of orbital primitive neuroectodermal tumor. 1909 May 47

We present the very unusual case of a young woman suffering from a brain tumor 22 years after a stage IV spinal neuroblastoma as an infant, demonstrating the difficulties of differentiating late neuroblastoma relapse from secondary supratentorial primitive neuroectodermal tumor (sPNET). Lacking specific immunohistochemical features, the first cerebral tumor at the age of 21 was regarded as sPNET, and we pursued a therapeutic approach consisting of neurosurgical resection as well as irradiation and high-dose alkylator-based chemotherapy according to the HIT2000 protocol. Two years later the patient suffered from a diffusely infiltrating local recurrence, changing its imaging appearance as well as its immunohistochemical characteristics, now revealing disseminated positivity for neuron-specific enolase and neural cell adhesion molecule. Moreover, the lack of PNET-specific translocations (EWS/FLI1 gene fusion) in both brain tumors as well as the development of hepatic metastases was more compatible with the diagnosis of a very late relapse 22 years after initial stage IV spinal neuroblastoma.
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PMID:CNS tumor 22 years after spinal neuroblastoma IV: diagnostic dilemma between recurrence and secondary malignancy. 1925 32

Primitive neuroectodermal tumor is an invasive neoplasm with neuronal differentiation, which frequently results in metastasis in various organs. We report the case of a patient with primitive neuroectodermal tumor whose primary site was the axilla. The patient presented with metastases in the lung, pleura, bone, iliac muscle and bone marrow. We highlight the uncommon finding in the pleural fluid cytology.
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PMID:Uncommon pleural effusion: pleuropulmonary metastasis from primitive neuroectodermal tumor. 1961 40

Medulloblastomas were originally classified under gliomas of the cerebellum until Bailey and Cushing in 1925 named these tumors as medulloblastoma. At present these tumors are classified under primitive neuroectodermal tumor. Surgical excision followed by craniospinal irradiation is the treatment of choice. A 13-year-old-girl operated for posterior fossa medulloblastoma 5 years ago presented with history of headache and vomiting on and off for 4 days in late August 2008. The MRI showed left frontal tumor which on excision was reported as medulloblastoma. Even after optimal treatment reports of recurrence abound in literature. The most common location is in the posterior fossa, followed by spinal, supratentorial, and uncommonly, systemic metastases. We conclude that medulloblastomas are highly aggressive tumor with high local recurrences if the initial excision is incomplete and that recurrence in the supratentorial area although uncommon is still a possibility. This mandates regular follow up of these children till adulthood to catch early recurrences and metastatic disease.
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PMID:Frontal recurrence of medulloblastoma five years after excision and craniospinal irradiation. 1967 68

We report a 21-year-old man with a primitive neuroectodermal tumor (PNET) of the kidney that was difficult to distinguish from other small round cell tumors, e.g., small cell carcinoma, malignant lymphoma, and Wilms' tumor. Pathologically, a primitive rosette-like pattern was shown by H&E staining; expression of MIC-2 was indicated by an immunohistochemical test; rather primitive organelles were observed by an ultrastructural method; and translocation of chromosome 22 was confirmed by FISH. We therefore diagnosed the current case as PNET. The patient had undergone a right radical nephrectomy more than 1.5 years earlier. After neither metastases nor recurrences for 0.5 year, imaging examinations revealed masses in his liver. He received chemotherapy and underwent surgery again, but the masses were not composed of viable tumor cells. PNET of the kidney is extremely rare; fewer than 30 cases have been reported in the English literature, and there are few data on the expression of p53, ki67, and bcl2. We investigated the relationships between these markers in the current case using immunohistochemical tests and observed strong expression of p53, Ki-67, and bcl-2. Such results generally indicate poor prognosis, and the patient eventually had some masses in his liver, but no viable tumor cells were found. The prognostic significance of these various markers in PNET of the kidney still remains unclear, but p53, ki-67, and bcl-2 might not be so important as indicators of prognosis in the kidneys as they are in other organs. Further studies are needed to investigate this, and we hope that the patient recovers completely.
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PMID:Primitive neuroectodermal tumor of the kidney. 1978 45

Retinoblastoma is a relatively rare malignant pediatric tumor accounting for approximately 3% of childhood cancers and 1% of all cancer deaths in children under 15 years of age. During the clinical course of the disease, a metastasis usually occurs within the first year of diagnosis and is seen in 2% of retinoblastoma patients. Metastases to the intracranial region are common and account for approximately 50% of the metastatic cases. Metastasis to the soft tissue is very rare. Herein, we report a case of metastatic retinoblastoma presenting as a left shoulder soft tissue mass in a 14-year-old female with a 14-year history of familial bilateral retinoblastoma status post radiation therapy. In our case, the FNA cytology shows some features of the small round blue cell tumor group with inconspicuous Flexner-Wintersteiner or Homer-Right rosette formation. The unusual clinical presentation and morphology give rise to a diagnostic dilemma, with the differential diagnosis centering on the small round blue cell tumors such as lymphoma, rhabdomyosarcoma, nephroblastoma (Wilms' tumor), Ewing's sarcoma/PNET, and desmoplastic small round cell tumor. It also prompts concern for the development of a second primary tumor. The purpose of our study is to discuss the FNA cytology of metastatic retinoblastoma, its differential diagnoses, and the utility of immunohistochemistry. An accurate diagnosis is imperative due to the differences in prognosis and treatment implications for the various diseases.
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PMID:Metastatic retinoblastoma presenting as a left shoulder soft tissue mass: FNA findings and review of the literature. 1993 45

PNETs of the spinal cord are aggressive and local recurrence and/or leptomeningeal spread is common. Primary spinal PNETs are extremely rare and most cases involving the spinal cord are drop metastases from primary intracranial tumors by cerebrospinal fluid. Herewith, we present a 40-day-old infant with multilevel primary spinal PNET at Th12-L1 and L5-S1 levels associated with hydrocephalus occurring nearly 15 days after the operation. According to our knowledge this is probably the first case harboring all these pathologies. Multilevel primary intraspinal PNET in an infant is even rarer and can be associated with hydrocephalus that occurs during the postoperative period.
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PMID:Multilevel primary intraspinal PNETs in an infant associated with hydrocephalus. 2006 29

Surgical excision has been the mainstay of treatment for neuroendocrine tumors of the pancreas (PNET). Compounds like streptozocin and dacarbazin have been traditionally used in inoperable cases and somatostatin to treat syndromes deriving from functional tumors. However, a lot of progress has taken place in the area of molecular characterization of these tumors, revealing activation of mammalian target of rapamycin (mTOR) and VEGF pathways. Recent data from the 2010 ASCO Gastrointestinal Cancers Symposium demonstrate antitumor activity of everolimus, an mTOR inhibitor in combination with temozolomide in a phase I/II trial and of sunitinib versus placebo in a randomized double blinded phase III trial. The role of modern biologic compounds in the treatment of PNET is not clear yet. In addition, combination of resection and transarterial chemoembolization (TACE) has been proven effective over either modality alone in the treatment of PNET metastatic to the liver in a retrospective analysis. This comes to address the problem of selecting local intervention in a metastatic disease, which has been a reasonable choice for this group of tumors in the past. Last but not least the role of Ki-67 in decision-making in PNET is being discussed.
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PMID:Neuroendocrine tumors of the pancreas: what's new. Highlights from the "2010 ASCO Gastrointestinal Cancers Symposium". Orlando, FL, USA. January 22-24, 2010. 2020 21

Ewing's sarcoma is the second most common primary tumour of bone in childhood. Less frequently it occurs in soft tissues. Ewing's sarcoma/primitive neuroectodermal tumour (ES/PNET) is an extra ordinarily rare primary tumour in the kidney. Only very few cases of primary renal Ewing's sarcoma have been reported in the literature to date. We present a case of primary right renal Ewing's sarcoma in a 13-year-old girl who was diagnosed as a case of stage IV ES/PNET of kidney with metastases to lung and liver. Right nephrectomy was done followed by adjuvant radiotherapy and chemotherapy with complete response to local and distant area.
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PMID:Ewing's sarcoma/PNET of kidney in 13-year-old girl. 2041 80


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