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

A peripheral primitive neuroectodermal tumor (pPNET), most consistent with a human Ewing's sarcoma, is described in a 5-month-old male Australian Shepherd puppy. The first tumor site detected was in the left frontal bone of the skull with apparent subsequent rapid metastases to multiple sites in the axial and appendicular skeleton and bone marrow, kidneys, and perihyphophyseal meninges. Radiographically, all bone lesions were lytic and there was also a humeral bone fracture. Histologically, the tumor was diagnosed as a small round blue cell tumor. At this stage, the differential diagnosis included a lymphoma, rhabdomyosarcoma, and a PNET of the peripheral nervous system. However, the cells had positive expression of triple neurofilament antigens as detected immunocytochemically. The cells were negative for a broad panel of canine-specific leucocyte cell marker antigens for desmin, smooth muscle actin, synaptophysin, and CD99. Ultrastructurally, the cells contained occasional dense core neurosecretory granules and intermediate filaments with intercellular desmosomal-like junctions and abundant glycogen clusters. Based on the age of the dog, the clinical history, the distribution of gross lesions, histologic characteristics of a small round blue cell tumor, and immunocytochemical and ultrastructural evidence of neuroectodermal differentiation, a diagnosis of a pPNET similar to a human Ewing's sarcoma was made.
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PMID:A peripheral primitive neuroectodermal tumor with generalized bone metastases in a puppy. 1523 49

Primitive neuroectodermal tumor/Ewing sarcoma (PNET/ES) rarely occurs in the skin and subcutaneous tissues. We present a case of a 16-year-old girl with primary cutaneous and subcutaneous PNET/ES of the abdominal wall. Despite wide local excision and chemotherapy, she rapidly developed cranial bone and brain metastases, followed by lung and skeletal metastases, and died shortly thereafter. The recurrent tumor exhibited light microscopic features of a small, round, blue cell tumor with intracytoplasmic glycogen. Immunohistochemical analysis showed positivity for CD99, CD56, S100, and glial fibrillary acid protein, and ultrastructural features included cytoplasmic glycogen and focal complex interdigitating synaptic junction-like cytoplasmic folds. Cytogenetic analysis of the relapsed tumor showed a complex karyotype: 47,XX,i(1)(q10), der(4)t(4;19) (q33 approximately q35;q13.1), + 8,t(15;17)(q24;p11.2 approximately p12),der(19)t (19;20)(q13.1;p11.2),der(22)t(20;22)(q13;q13). Cytogenetic, interphase fluorescence in situ hybridization, and molecular genetic analyses failed to show t(11:22) (q24;q12) or abnormalities of chromosome region 22q12. The clinical behavior and atypical and complex cytogenetic abnormalities exhibited by the tumor in this patient are unusual and represent the most aggressive end of the clinical spectrum of cutaneous and subcutaneous PNET/ES.
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PMID:Primary subcutaneous primitive neuroectodermal tumor with aggressive behavior and an unusual karyotype: case report. 1554 79

We recently encountered a very rare case of periosteal Ewing's sarcoma (PES), which was treated by surgery followed by photodynamic therapy using acridine orange with radiodynamic therapy. No more than 15 cases of PES have been reported previously in literature. In our case, MRI revealed the tumor to be localized on the cortical surface of the proximal humerus. Histopathological examination of biopsy specimens revealed a small round cell sarcoma suggestive of Ewing's sarcoma or PNET, and immunohistochemistry showed positive staining for MIC2. Although fusion genes EWS-FLI1 and EWS-ERG were undetectable, PES was still considered to be the most likely diagnosis. Therefore, we administered preoperative chemotherapy, as a result of which the tumor shrank to 48% of its original volume. With a view to preserve excellent shoulder and upper limb function, we attempted intralesional tumor resection supported by photodynamic therapy using acridine orange with radiodynamic therapy. After surgery followed by postoperative chemotherapy, the patient has, until the time of writing, had no local tumor recurrence and no evidence of metastatic disease, and can move his shoulder fully and throw a ball well. Since it has been reported that PES has a better prognosis and responsiveness to chemotherapy than intramedullary Ewing's sarcoma, we believe that such reduction surgery with photodynamic therapy might be the strategy of choice to obtain satisfactory limb function in cases of PES.
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PMID:Periosteal Ewing's sarcoma treated by photodynamic therapy with acridine orange. 1564 11

The aim of this study was to determine the outcome for patients with Chang stage M2-3 medulloblastoma (MB) treated with surgery and pre-radiotherapy (RT) chemotherapy (CT). Between 1992 and 2000, 68 patients aged 2.8-16.4 years (median 7.8 years) with M2-3 MB were treated with CT comprising vincristine, etoposide, carboplatin and cyclophosphamide. For 61 patients, CT was followed by craniospinal RT 35 Gy/21 fractions with a posterior fossa (PF) boost, 20 Gy/12 fractions. Twenty-four (35%) irradiated patients received a metastatic boost (mean dose to metastases 47.4 Gy, range 40.0-55.1 Gy). With 7.2-years of median follow-up, overall survival (OS) rates at 3 and 5 years were 50.0% (95% Confidence Interval (CI): 38.1-61.9%) and 43.9% (95% CI: 32.0-55.7%), respectively, event-free survival (EFS) rates at 3 and 5 years were 39.7% (95% CI: 28.1-51.3%) and 34.7% (95% CI: 23.2-46.2%), respectively. Univariate analysis did not demonstrate an impact of age, gender, M stage, extent of resection, RT duration or metastatic boost. For patients commencing RT within 110 days of surgery, EFS was significantly (P=0.04) worse than for those who commenced RT later than this. Response to pre-RT CT was assessable from institutional reports for 44 (65%) patients, and 17 (39%) had a complete response (CR), 15 (34%) a partial response (PR), 4 (9%) stable disease (SD) and 8 (18%) progression. Although CT improved outcome for M0-1 patients in the primitive neuroectodermal tumour (PNET-3) randomised study, and resulted in a high response rate in this study, there has been no apparent improvement in outcome for M2-3 patients when compared with earlier multi-institutional series. Newer approaches such as more intensive CT and RT need to be explored.
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PMID:Outcome for patients with metastatic (M2-3) medulloblastoma treated with SIOP/UKCCSG PNET-3 chemotherapy. 1576 49

For the subgroup of patients with inoperable gastrointestinal stromal tumors, progress has been made by the rapid development and approval of the targeted therapy imatinib mesylate. Small round cell sarcomas (SRCT), such as Ewing/primitive neuroectodermal tumor, desmoplastic SRCT, and rhabdomyosarcoma, are chemotherapy-sensitive and potentially curable malignancies that are treated with multimodality dose-intensive neoadjuvant protocols regardless of size or overt metastatic disease. However, the number of effective cytotoxic agents for the treatment of patients with metastatic so-called adult soft tissue sarcoma is limited, especially when patients have failed anthracycline- and ifosfamide-based chemotherapy.
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PMID:New drug developments for patients with metastatic soft tissue sarcoma. 2237 Oct

In 1918, Stout defined the lesion in which small round cells originating from the ulnar nerve formed a rosette as neuroepithelioma. It was claimed that this tumor originated from neuroectodermis and was different from the classical neuroblastoma. The term primitive neuroectodermal tumor (PNET) involves a group of tumors of the soft tissue originating from neural crest and resulting from the brain, spinal cord and branches of the sympathetic nervous system. Extracranial primitive neuroectodermal tumors originate from neural crest cells outside the sympathetic and central nervous system. PNET also has some distinctive histological, immunohistochemical and ultrastructural features. It is usually encountered in children and young adults; most frequently located in thoracopulmonary region (Askin's tumor). The second most commonly involved body part is the extremities. It is very rarely located on the face. PNET is an aggressive tumor. In fact, the disease has a rapid progression, causes local or distant metastases and 50% of the patients die within two years of the presentation. It is treated with aggressive surgery as well as chemotherapy and radiotherapy. In this report, we presented a case of PNET located on the right cheek with multiple distant metastases. Clinicians should be on alert when treating facial tumors, not to skip PNET, which is a very aggressive one.
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PMID:A primitive neuroectodermal tumor on the face: case report. 1603 49

There are nearly 50 PNET cases in the literature with primary renal localization. The mean age for renal PNET is 28 but it can be seen in a wide range between 4 and 69 years. In this atypical localization PNET may be mistaken with a variety of small round cell tumors, particularly with blastema predominant Wilms' tumor and due to its distinctive prognostic and therapeutic features differential diagnosis is very important. Immunohistochemical studies are useful in discriminating PNET from other renal small round cell tumors. Renal PNETs tend to metastasize at early stages and they have a high rate of recurrence. Our case is a 45-year-old male presented with left flank pain and hematuria. In computerized tomography a mass in left kidney was observed and nephrectomy was performed. Histopathologic examination revealed a small round cell tumor with rosettes and pseudorosettes. Immunohistochemically the lesion was diffuse strong positive with CD99 and NSE, negative with LCA, pancytokeratin, vimentin, desmin, smooth muscle actin, chromogranine A and S-100. At the time of diagnosis the pathologic stage of the tumor was T1aN0Mx. The patient did not take any further therapy and in 1-year follow-up no local recurrence or distant metastasize occurred.
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PMID:Primary primitive neuroectodermal tumor of the kidney: a case report. 1686 82

Neuroblastoma (NB) is uncommon in the adolescent age group. It is located at any site containing sympathetic neural tissue, with retroperitoneal and adrenal lesions being the most common. Cutaneous and subcutaneous metastases of NB in children are infrequent. Primary thyroid NB with regional lymph node and distant multiple subcutaneous metastases was diagnosed in an 11-year-old boy. The differential diagnosis was primitive neuroectodermal tumor. Our case demonstrates the unique presentation of NB in an adolescent.
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PMID:The thyroid: an extremely rare primary site of neuroblastoma. 1694 5

Medulloblastoma is a primitive neuroectodermal tumor arising in the posterior fossa usually in the first decade of life. Systemic metastases are infrequent at diagnosis and usually occur after surgical resection or shunt placement. We report a rare case of medulloblastoma in an 18-year-old woman who presented with headache, leukopenia, and anemia. Neurologic examination was normal. Bone marrow evaluation revealed primitive cells morphologically resembling blasts. By flow cytometry, these cells lacked CD45 and expressed CD13/33, CD15, CD34, HLA-DR, and strong CD56. The presence of myeloid antigens and CD34 suggested acute myeloid leukemia; however, the bone marrow core biopsy architecture and tumor cells in cerebrospinal fluid were more compatible with a nonhematopoietic tumor. Further workup revealed a cerebellar mass, and a diagnosis of desmoplastic medulloblastoma was made. To our knowledge, this is the first reported case of a nonhematopoietic small round blue-cell tumor expressing multiple myeloid antigens and CD34 by flow cytometry.
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PMID:Medulloblastoma simulating acute myeloid leukemia: case report with a review of "myeloid antigen" expression in nonhematopoietic tissues and tumors. 1748 18

The authors describe the case of a 19-year-old female patient with a primary primitive neuroectodermal tumor (PNET) of the thoraco-lumbar spinal cord, who presented with acute urinary retention and back pain for 2 months. Magnetic resonance imaging revealed an intradural extramedullary tumor, 6.5 cm long, in the region of the conus medullaris. Histological examination disclosed a small round cell tumor with immunohistochemical characteristics of a peripheral PNET. Metastatic workup showed no evidence of an intracranial tumor or metastases outside the neuroaxis. The patient received multidisciplinary treatment, including surgical excision, irradiation of the entire cranio-spinal axis, and high-dose chemotherapy with autologous stem cell rescue. Presently, 24 months after diagnosis, the patient remains in complete remission.
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PMID:Primary intraspinal primitive neuroectodermal tumor treated with autologous stem cell transplantation: case report and review of the literature. 1713 Jan 14


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