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PubMed1991

From chimney sweeps to oncogenes: the quest for the causes of cancer.

Over the past 200 years, a bewildering array of chemical, physical, and viral agents has been identified that can cause cancer, but the mechanisms involved are only now becoming clear. In the leukemias and lymphomas, it appears that the activation of cellular oncogenes is important. The genes involved are present in all normal cells and are often associated with cell growth and regulation. When activated, they act in a dominant fashion to cause a cell to express the malignant phenotype. There is increasing evidence that in solid tumors, a more important mechanism may be the loss of a suppressor gene. The classic example is retinoblastoma, in which the retinoblastoma gene has been cloned and is also found to be associated with several other common cancers including sarcomas and small cell lung cancer. It is likely to be one of a family of such genes. It may well be that the activation of one or more oncogenes or the loss of one or more suppressor genes, or both, is required for a tumor to progress from initiation through promotion to a metastasizing malignancy.

Animals↗

PubMed1996

Postmenopausal intra-abdominal desmoplastic small cell tumor.

Intra-abdominal desmoplastic small cell tumor (DSCT) usually occurs in infants and young male adults. A case of DSCT occurring in a 60 year old female is described. No other apparent primary origin was detected. A mesocolon tumor, measuring 23 x 12 x 10 cm, was composed predominantly of round to spindle cells which showed epithelioid- and focally sarcomatous arrangements. Immunohistochemically, the tumor cells showed perinuclear dot-like staining of CAM5.2, many cells expressed HHF35, and some cells contained vimentin, epithelial membrane antigen, desmin, alpha-smooth muscle actin, neuron-specific enolase, or Leu 7. Electron microscopic examination showed that the tumor cells had mesenchymal-fibroblastic features. The tumor had an aneuploid DNA content with high S-phase fraction. The patient, who was treated with adjuvant chemotherapy, was alive, having had three recurrences in 36 months. In the second and third recurrent lesions, increased cellular atypia and fascicular arrangements of spindle cells were observed. DSCT should be included in differential diagnoses of post-menopausal pelvic tumors which show light-microscopically and immunohistochemically divergent phenotypes.

Abdominal Neoplasms↗

PubMed2005

Periosteal Ewing's sarcoma treated by photodynamic therapy with acridine orange.

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.

Acridine Orange↗

PubMed2000

Primary primitive neuroectodermal tumor of the kidney.

Primitive neuroectodermal tumor (PNET) is a small round cell sarcoma that mainly develops in the central nervous system and soft tissues of childhood; however recently, primary occurrence of this tumor in the kidney has been reported. We experienced one case of PNET primarily arose in the kidney without metastasis. The patient was a 28-year-old man whose chief complaint was abdominal pain, especially on exercise. On computed tomography scan and magnetic resonance imaging, a solid lesion was found in the left kidney, and a left nephrectomy was performed based on the diagnosis of a tumor in the left kidney. The tumor was within the parenchyma of lower end of left kidney protruding into the abdominal cavity. Histologically, diffuse proliferation of primitive small round cells with rosette formation was found. Immunohistochemically, MIC2 gene product, neuron-specific enolase and S-100 protein were positive. No metastasis to the regional lymph nodes was found. From these observations, the tumor was diagnosed as PNET primarily arising in the left kidney. Although chromosome analysis was not performed, EWS-FLI1 chimera gene was identified by reverse transcriptase-polymerase chain reaction on the freshly frozen specimen and fluorescence in situ hybridization on paraffin sections.

12E7 Antigen↗

PubMedDate not supplied

Desmoplastic small round cell tumor presenting in the ovaries: report of a case and review of the literature.

Desmoplastic small round cell tumor is a recently recognized clinical entity with specific morphologic, immunocytochemical, and genetic features. Though this tumor is mostly described to involve serosal surfaces, we report a case with ovarian involvement. The clinical presentation and differential diagnoses as well as the treatment including aggressive surgical debulking and multiagent chemotherapy are discussed.

Abdominal Neoplasms↗

PubMed1994

[Askin's tumor in a young adult: a rare occurrence. Apropos one case observed].

Askin's tumour is a rare malignant small cell neoplasia of the thoracic wall; it most often effects females during childhood and is characterised by limited survival. The authors report a recent case of Askin's tumour in a young adult male. The case was characterised by multiple recidivation but a long survival. The authors discuss its diagnosis and treatment.

Adult↗

PubMed2002

Distinctive soft tissue tumors of the head and neck.

Among soft tissue tumors as a whole, those in the head and neck region are relatively uncommon, and the proportion of all soft tissue sarcomas that arise in this region is <or=5-10% (although this figure is higher in children). With the exceptions of nasopharyngeal angiofibroma and sinonasal hemangiopericytoma, most mesenchymal lesions are not truly distinctive to this site. This overview focuses on tumors that show a relative predilection for this region, including principally benign fibroblastic/myofibroblastic lesions, nerve sheath tumors, vascular tumors (both benign and malignant), and small round cell sarcomas, especially rhabdomyosarcoma in young patients. Important generalizations to bear in mind include the facts that (1) among spindle cell malignancies in the head and neck of adults, spindle cell carcinoma and melanoma are by far more common than sarcomas; (2) because of anatomic constraints and dramatic clinical presentation, the index of clinical suspicion for malignancy is often high; (3) certain subsets of benign mesenchymal lesions in this anatomic region frequently show pseudosarcomatous morphology; and (4) the ability to define or obtain an adequate surgical resection margin in this region is often very limited, hence issues of local control may supercede the importance of overall biologic/metastatic potential in some cases.

Head and Neck Neoplasms↗

PubMedDate not supplied

[Peripheral primitive neuroectodermal tumors of the soft tissues and bones].

Large group of small-round-cell tumours of soft tissues and bone represents a complex diagnostic problem for the pathologists. Neuronal nature of many tumours from this group is proven by means of new methods--immunophenotypic analysis, tissue culture, cytogenetics. Peripheral neuroepithelioma, Ewing tumour, primitive neuroectodermal tumour (PNET), Askin tumour belong to these neoplasms. These tumours anatomically have no connection with the structures of the central nervous system or autonomous sympathetic nervous system.

Adolescent↗

PubMedDate not supplied

Recurrent intrapulmonary malignant small cell tumor of the thoracopulmonary region with metastasis to the oral cavity: review of literature and case report.

Malignant small cell tumor of the thoracopulmonary region (MSCT) was first described in 1979 and has been referred to as the Askin tumor. This malignant neoplasm is a member of the peripheral primitive neuroectodermal tumor (PPNET) family and typically involves the periosteum, soft tissue, and extrapulmonary tissue of the thoracic wall. MSCT may also involve the lung parenchyma by local extension or may arise de novo in peripheral lung tissue. Local recurrence, abdominal involvement by tumor extravasation across the diaphragm, and skeletal metastatic disease are relatively common. However, metastasis to the head and neck region and in particular to the oral cavity is extremely rare. We present a recurrent intrapulmonary MSCT with metastasis to the oral cavity in an adolescent Hispanic boy, and review the literature regarding this member of the PPNET family. Differentiation from neuroblastoma may be made based on immunoreactivity for beta 2 microglobulin and HBA71 and lack of immunoreactivity for chromogranin in PPNET and MSCT. Ultrastructural features commonly seen in MSCT and PPNET are round to ovoid tumor cells with occasional cytoplasmic processes with relatively few pleomorphic dense core granules. These tumors lack the gangliocytic and Schwann cell differentiation that is characteristic of neuroblastoma. MSCT and PPNET have a common reciprocal cytogenetic translocation [t(11;22)q(24;q12)], which is shared with Ewing's sarcoma. Prognosis in MSCT is quite dismal, with a 2-year survival of 38% and a 6-year survival of only 14%.

Adolescent↗

PubMedDate not supplied

Pathology quiz: small cell osteosarcoma.

A 15-year-old black male presented with shortness of breath, leg weakness, and pain in his back and rib cage. Four years previously he had noticed a lump in his upper back and complained of pain when playing basketball, especially on contact to that area. Recently, the pain had become more constant and increased in intensity. This was associated with loss of control in his legs, weakness, and paraesthesia. General physical examination revealed a palpable mass in the right midline upper back. Laboratory results were within normal limits. Radiographic scans demonstrated a destructive soft tissue mass at T6 vertebral body with scattered stippled calcification (Figure 1). The patient underwent a biopsy followed by excision of the mass (Figure 2) and decompressive laminectomy with reconstruction.

Adolescent↗

PubMed2007

Response to vinorelbine and low-dose cyclophosphamide chemotherapy in two patients with desmoplastic small round cell tumor.

We report two cases of abdominal desmoplastic small round cell tumor (DSRCT) that showed a clinical response to the vinorelbine/low-dose cyclophosphamide combination that has been claimed to be effective for rhabdomyosarcoma. This observation may prompt further investigation into the activity of such a regimen in DSRCT patients with recurrent or refractory disease, with a view to a possible future role as maintenance therapy in controlling minimal residual disease in patients who achieve complete remission with intensive induction multimodality therapy.

Abdominal Neoplasms↗

PubMed2003

[Intra-abdominal desmoplastic small round cell tumor in childhood: case report and review of the literature].

Desmoplastic small round cell tumor (DSRCT) correspond to a recent clinicopathological entity, individualized in 1989 into the group of tumors with small round cells. This pathology puts ethiopathogenic, diagnostic, therapeutic and prognosis problems. Indeed, the ethiopathogenic is still unknown, diagnosis is asserted only by immuno-histochimic and cytogenetic study because of the big number of differential diagnoses and the anatomopathologic polymorphism. Its treatment is not well codified and its outcome remains dark in spite of therapeutic progress. The objective of this work is to report a personal observation of a DSRCT and to proceed to a review of the literature to clarify the epidemiological, clinical, paraclinical and therapeutic aspects of this rare tumor.

Abdominal Neoplasms↗

PubMed1996

Preresection chemotherapy improves survival for children with Askin tumors.

OBJECTIVE: To test whether patients with Askin tumor treated with aggressive neoadjuvant chemotherapy have a better clinical outcome. DESIGN: Retrospective case series. SETTING: Pediatric referral center. PATIENTS: All children diagnosed with malignant small-cell tumors of the chest wall (Askin tumor) and treated from 1975 to September 1987 (phase 1, n = 6) and from September 1987 to the present (phase 2, n = 9). MAIN OUTCOME MEASURES: Survival as a function of extent of disease and response to therapy as measured by tumor volume, survival, and recurrence. RESULTS: All phase 2 patients had significant reduction of tumor volume and improved survival by Kaplan-Meier estimates compared with phase 1 patients. No phase 1 patients are still alive. CONCLUSION: Patients with Askin tumor treated with aggressive preresection chemotherapy have smaller tumors to resect and improved survival.

Adolescent↗

PubMed1997

Malignancy-associated membranoproliferative glomerulonephritis.

An 11-year-old girl with an abdominal desmoplastic round cell tumor, treated with chemotherapy, presented with gross hematuria and proteinuria. Renal biopsy revealed type I membranoproliferative glomerulonephritis (MPGN). The association of a malignant tumor and MPGN is extremely unusual in children, and the pathogenesis of the renal lesion under these circumstances is unknown.

Abdominal Neoplasms↗

PubMedDate not supplied

Genetic and molecular abnormalities in tumors of the bone and soft tissues.

BACKGROUND: Malignant transformation requires the accumulation of multiple genetic alterations such as chromosomal abnormalities, oncogene activation, loss of tumor suppressor genes, or abnormalities in genes that control DNA repair and genomic instability. Sarcomas are a heterogeneous group of malignant mesenchymal tumors of difficult histologic classification and strong genetic predisposition. This article provides a comprehensive review of the cytogenetic abnormalities observed in bone and soft-tissue tumors, emphasizing known downstream molecular changes that may play a role in oncogenesis. METHODS: The database of the National Library of Medicine was searched for literature relating to genetic and molecular mechanisms in sarcomas in general and in each of the main tumor entities. RESULTS: Recent techniques in chromosome analysis and molecular cytogenetics have improved our ability to characterize genetic changes in mesenchymal tumors. Some changes are so characteristic as to be virtually pathognomonic of particular histologic types, while others are complex, difficult to characterize, and of unknown relevance to pathogenesis. The implications to the cell of some of these abnormalities are now being recognized. CONCLUSIONS: The study of sarcomas will benefit from the information derived from genetic studies and translational research. The human genome project and new methodologies, such as computer-based DNA microarray, may help in the histogenetic classification of sarcomas and in the identification of molecular targets for therapy.

Bone Neoplasms↗

PubMed1996

Molecular genetics of small round cell tumors.

The small round cell tumors of children and young adults constitute part of a group of undifferentiated tumors, the precise diagnosis of which is often a challenge for the pathologist because of their uniform morphological appearance. Diagnostic cytogenetic analysis and identification of specific chromosomal abnormalities have been especially useful for the classification of some of these tumors. The cloning of the genes and molecular characterization of the associated genetic anomalies have led to the discovery of the mechanisms involved in their neoplastic transformation and identified a variety of tumor-specific molecular genetic markers. Information has also been provided regarding the histogenetic origin and mechanisms of differentiation in these tumors. This review focuses on the tumor-specific genetic markers, particularly those of clinical relevance, and the recently identified genes and deregulation mechanisms associated with them. The availability of these markers provides auxiliary methods with increasingly improved resolution for primary diagnosis and classification of histologically similar tumors and tools for monitoring patients and identifying potential antineoplastic therapy targets.

Adolescent↗

PubMed1992

Secretogranin II expression in Ewing's sarcomas and primitive neuroectodermal tumors.

Production of chromogranins, the acidic components of the chromaffin granules regarded as specific neuroendocrine markers, was analyzed by immunocytochemistry and hybridization (Northern blotting and in situ hybridization) in primary lesions and cell lines of Ewing's sarcomas, primitive neuroectodermal tumors (PNETs), and neuroblastomas. Antibodies and probes specific for chromogranin A (CgA), chromogranin B (CgB), and secretogranin II (SgII) were used. Ewing's sarcomas and PNETs, unlike neuroblastomas, were negative for CgA and CgB. Two primary Ewing's sarcomas, one primary PNET (an Askin tumor), and one PNET cell line (TC32) were found to strongly express the SgII gene, as shown by the presence of specific mRNA. This result supports the hypothesis that some Ewing's sarcomas represent a most primitive form of neuroectodermal tumor; in addition, it indicates a diagnostic role of SgII in cases of Ewing's sarcomas and PNETs.

Adolescent↗

PubMed1999

Clinically critical impact of molecular genetic studies in pediatric solid tumors.

BACKGROUND: Standard cytogenetic techniques are time-consuming and often not informative with solid tumors. In contrast, the reverse transcriptase-polymerase chain reaction (RT-PCR) is a readily available technique that can rapidly detect tumor-specific chromosomal rearrangements, even in small biopsy specimens. We present cases depicting the importance of including molecular diagnostic studies in the routine evaluation of pediatric solid tumors. PROCEDURE: We used RT-PCR to detect chimeric transcripts specific for major pediatric solid tumors, including peripheral primitive neuroectodermal tumor (pPNET), alveolar rhabdomyosarcoma (ARMS), and desmoplastic small round-cell tumor (DSRCT). We reviewed six recent cases in which the initial diagnosis was changed by the results of RT-PCR. RESULTS: Highly unusual or nonspecific clinical and/or histopathologic findings led to the initial diagnoses of neuroblastoma in three patients and DSRCT, leukemia, and carcinoma in one patient each. The final diagnoses after RT-PCR studies were pPNET in three patients, ARMS in two patients, and DSRCT in one patient. RT-PCR results led to early corrections in the diagnosis in two patients, but four patients received treatment not considered optimal for the neoplasms ultimately diagnosed, including three who, despite presenting with localized tumors that have a >70% cure rate with standard therapy, have died or are dying of disease. CONCLUSIONS: Molecular genetic studies on solid tumors can clarify the diagnosis in seemingly straightforward as well as in overtly problematic cases. These diagnostic distinctions are now critical as disease-specific and risk-directed therapies have emerged.

Adolescent↗