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)

Cutaneous small blue cell tumors are relatively uncommon and include primary lesions of either adnexal or neuroendocrine differentiation, as well as metastatic disease. Extraosseous Ewing's sarcoma/malignant primitive neuroectodermal tumor (MPNET) rarely may occur as a primary, superficially based neoplasm in children and young adults. We describe a series of five cases of Ewing's sarcoma/malignant primitive neuroectodermal tumor occurring as a primary cutaneous malignancy supported diagnostically both by immunohistochemical stains and fluorescence in situ hybridization (FISH). All five cases occurred as a solitary dermal nodule and were located in the lower extremities (3 cases), the axilla (1 case), and the flank (1 case). Three of the cases were clinically polypoid. Four of the five patients were female, and age at presentation ranged form 8 to 50 years of age (median, 18 years). All five tumors consisted of nodular proliferations of monomorphous, small blue cells with round, vesicular nuclei, and scant to moderate cytoplasm that were uniformly immunoreactive for the CD99 cell surface glycoprotein in a characteristic membranous pattern. Fluorescence in situ hybridization analysis of paraffin-embedded tissue revealed that three of four tumors were positive for a chromosomal translocation involving the EWS locus at 22q12, seen in more than 90% of cases of Ewing's sarcoma/malignant primitive neuroectodermal tumor. One case was not analyzable. All five patients were treated using local excision, and two patients additionally received postoperative chemotherapy and radiotherapy. Clinical follow-up is available in three cases (median duration, 33 months) and to date none has shown evidence of either local recurrence or metastasis. Because similar cases reported in the literature have likewise had favorable clinical courses after excision, primary cutaneous Ewing's sarcoma/malignant primitive neuroectodermal tumor may represent a clinically favorable subset of this otherwise highly aggressive neoplasm.
 ...
PMID:Primary cutaneous Ewing's sarcoma: immunophenotypic and molecular cytogenetic evaluation of five cases. 950 Jul 72

Bone marrow metastases from small round cell tumors can present diagnostic difficulties. In this study, we assessed the value of immunohistochemistry, using two monoclonal antibodies to CD99, for the diagnosis of metastatic disease in bone marrow trephine specimens from patients with Ewing's sarcoma or primitive neuroectodermal tumor (PNET). The proportions of specimens showing metastases were 10.3% with routine staining and 20.7% with immunohistochemistry. The specimens that were negative on conventional light microscopy and positive with immunohistochemistry all showed other abnormalities. The results do not support the routine use of immunohistochemistry in specimens that are normal by conventional light microscopy, but indicate that useful information may be gained in cases where marrow histology is obscured by fibrosis, necrosis, or distortion artefact. Neither of the two antibodies tested was superior for this purpose.
 ...
PMID:Bone marrow metastasis in Ewing's sarcoma and peripheral primitive neuroectodermal tumor: An immunohistochemical study. 950 36

The use of fine-needle aspiration biopsy (FNAB) has been infrequently described as a diagnostic modality for Ewing's sarcoma (ES) patient follow-up and management. The purpose of this study is to examine the use and accuracy of FNAB combined with MIC2 immunocytochemistry for evaluating metastases in patients with ES. Records from Saint Louis University Health Sciences Center and Indiana University Medical Centers identified patients with known ES who had undergone FNAB for evaluation of potential metastases. Immunocytochemical analysis for MIC2 (CD99) was performed retrospectively on cell blocks and direct aspirate smears. FNABs from nine patients were procured either percutaneously or under radiologic guidance and in all cases a definitive cytologic diagnosis of metastatic ES was rendered. Aspirates were cellular with many single discohesive small round cells and occasional loosely cohesive clusters. The nuclei were round with a fine chromatin pattern and small nucleoli. The cytoplasm was scanty and the nuclear-cytoplasmic ratio was high. Six of six cases showed strongly positive immunocytochemical labeling for MIC2. Immunocytochemistry with MIC2 in FNAB aspirate smears can provide supportive evidence of ES in patients with known disease.
 ...
PMID:Fine-needle aspiration biopsy of metastatic Ewing's sarcoma with MIC2 (CD99) immunocytochemistry. 981 36

Mesenchymal chondrosarcoma is a rare tumor that is distinctly different from classic chondrosarcoma. The prognosis of this tumor is poor, with a high incidence of locoregional and distant metastases. It shows a predilection for the head and neck, however mesenchymal chondrosarcoma of hyoid bone has rarely been reported. We experienced a case of mesenchymal chondrosarcoma of the hyoid bone in a 39-year-old woman. She underwent excision of the tumor by right hemihyoidectomy. Histologically, a combination of cellular zones composed of undifferentiated small cells and chondroid zones typically presented a bimorphic appearance. CD99 (DN16) immunohistochemical stain demonstrated that all undifferentiated small cells had strong reactivity with a distinct membranous pattern. There was microscopic tumor extension to the resection margin of the hyoid bone, however, no evidence of reccurence is noted at follow-up of 4 months with neck CT.
 ...
PMID:Mesenchymal chondrosarcoma of the hyoid bone: a case report. 988 85

Rhabdoid tumor is a well-accepted clincopathologic entity among childhood renal neoplasms; similar tumors have been described in extrarenal locations. We present the clinicopathologic profile and the immunohistochemical features of a series of soft tissue rhabdoid tumors. Twenty-eight cases coded as extrarenal rhabdoid tumor (ERRT), RT, possible ERRT, and "large cell sarcoma" were retrieved from the Armed Forces Institute of Pathology soft tissue registry. The tumors were reclassified according to strict criteria by light microscopy, clinical information, immunohistochemistry, and, in some cases, electron microscopy. Soft tissue rhabdoid tumor (STRT) was defined as (1) a tumor composed of noncohesive single cells, clusters, or sheets of large tumor cells with abundant glassy eosinophilic cytoplasm, an eccentric vesicular nucleus, and an extremely large nucleolus; (2) positivity for vimentin and/or cytokeratin or other epithelial markers by immunostaining; and (3) exclusion of other tumor types with rhabdoid inclusions (melanoma, other sarcomas, carcinoma). Eighteen cases met our criteria for soft tissue rhabdoid tumors. The median patient age was 13 years (range, 6 months to 56 years). Ninety-four percent of STRT cases were positive for vimentin and 59% for pan-cytokeratin. Sixty-three percent and 60% were positive for CAM 5.2 and EMA, respectively. Seventy-nine percent stained for at least one epithelial marker; 76% stained for both vimentin and epithelial markers simultaneously. Forty-two percent stained for MSA, and 14% for CEA and SMA. CD99, synaptophysin, CD57 (Leu-7), NSE, and focal S100 protein were identified in 75%, 66%, 56%, 54%, and 31% of the STRT cases, respectively. All STRT cases examined were negative for HMB-45, chromogranin, BER-EP4, desmin, myoglobin, CD34, and GFAP. Follow-up examination in 61% of the STRT patients revealed that 64% of patients died of disease within a median follow-up interval of 19 months (range, 4 months to 5 years); 82% had metastases to lung, lymph nodes, or liver; 22% had local recurrences before metastasis; and 18% were alive without known disease status (median, 5.5 years). Soft tissue rhabdoid tumor is a highly aggressive sarcoma, predominantly of childhood. Besides having nearly consistent coexpression of vimentin and epithelial markers, STRTs show positivity for multiple neural/neuroectodermal markers that overlap with those of primitive neuroectodermal tumor.
 ...
PMID:Extrarenal rhabdoid tumors of soft tissue: a clinicopathologic and immunohistochemical study of 18 cases. 993 May 72

Synovial sarcoma is a rare soft tissue tumor of children and adults that is unrelated to synovium and can occur in almost any part of the body. The familiar biphasic synovial sarcoma has discernible glandular or solid epithelial structures, and monophasic forms have characteristic ovoid or spindle cells with only immunohistochemical or ultrastructural evidence of epithelial differentiation. There are several morphologic patterns, including myxoid and hemangiopericytic, and behaviorally distinct calcifying, ossifying, and poorly differentiated subtypes can be recognized. Most synovial sarcomas are immunoreactive for cytokeratin, epithelial membrane antigen, and bc12 protein, and negative for CD34, and many express S100 protein and CD99 (MIC2). Nearly all synovial sarcomas have a specific t(x;18) (p11.2;q11.2) chromosomal abnormality, resulting in fusion of either of two variants of the SSX gene with the SYT gene; the genetic features may relate to morphology and outcome. The differential diagnosis can include a wide range of spindled, polygonal, or round cell sarcomas. Clinically, there have been marked recent improvements in local control of disease and lesser ones in management of metastases. The pathology, differential diagnosis, and behavior of this unique tumor are reviewed.
 ...
PMID:Synovial sarcoma. 993 May 76

Focal myxoid change is a well-recognized feature of solitary fibrous tumor (SFT), but to date, predominantly myxoid examples of SFT have not been reported. We describe seven cases of SFT in which stromal myxoid change affected 50% or more of the tumor examined, thus obscuring typical diagnostic features. Patients ranged in age from 35 to 68 years old (median, 45 yr), with an equal sex distribution. Tumor locations included pleura, orbit, and periparotid subcutaneous tissue, as well as four cases in deep soft tissue (two in the abdominal wall and one each in the chest wall and thigh). Myxoid areas were identified grossly in four cases. Histologically, the lesions were composed of bland spindle cells disposed haphazardly or with a lacy or reticulated appearance in a myxoid, richly vascularized stroma These myxoid areas were punctuated by small cellular aggregates in four cases, and areas showing diagnostic features of SFT were present in five of seven primary excision specimens. Atypical features suggestive of malignancy were not present in any of the cases. Immunohistochemically, all of the seven cases stained positively for CD34 and CD99 (013), and all were negative for smooth muscle actin, desmin, S-100 protein, epithelial membrane antigen, and pan-keratin. There were no recurrences or metastases reported in four patients with limited follow-up (median duration, 19 mo). Recognition of this uncommon morphologic subset of SFT is important because of possible confusion, particularly in small biopsy specimens, with a variety of myxoid spindle cell neoplasms with different biologic potential. These include low-grade fibromyxoid sarcoma, myxoid synovial sarcoma, malignant peripheral nerve sheath tumor, low-grade myxofibrosarcoma, myxoid liposarcoma, myxoid spindle cell lipoma, myxoid neurofibroma, and so-called "hemangiopericytoma."
 ...
PMID:Myxoid solitary fibrous tumor: a study of seven cases with emphasis on differential diagnosis. 1034 83

We report a small-cell variant of synovial sarcoma examined by fine-needle aspiration (FNA) biopsy. The patient is a 23-yr-old female who had a synovial sarcoma involving the left infratemporal region, diagnosed at 7 yr of age, followed by a metastatic lesion involving the lung and chest wall 16 yr later. The chest wall metastases was sampled by FNA biopsy. The aspirate consisted of numerous, small, round cells with very high nuclear-to-cytoplasmic ratios. The cytomorphologic features could potentially be confused with other pediatric small round cell tumors. Ancillary studies demonstrated positive staining of the neoplastic cells for cytokeratin, epithelial membrane antigen (EMA), and CD99. The differential diagnosis of other small round cell tumors that may be mistaken for the small-cell variant of synovial sarcoma are presented. We believe that this is the first FNA report detailing the cytologic and ancillary features of the small-cell variant of synovial sarcoma.
 ...
PMID:Small-cell variant of synovial sarcoma: fine-needle aspiration with ancillary features and potential diagnostic pitfalls. 1088 58

Although it is classically a deep soft-tissue tumor of childhood, primitive neuroectodermal tumor (PNET) can occur at any age and may occasionally involve cutaneous sites. Merkel cell carcinoma (MCC) and basaloid neoplasms of cutaneous adnexa are the principal diagnostic alternatives to that tumor. The common expression of CD99 in PNET and cytokeratin-20 (CK20) in MCC suggests that these markers may be of value in this diagnostic setting, but they have not been rigorously examined in other small-cell and basaloid lesions of the skin. Accordingly, we evaluated CD99 and CK20 reactivity in formalin-fixed, paraffin-embedded sections of 30 MCC, five cutaneous metastases of pulmonary small-cell neuroendocrine carcinomas, 10 primary cutaneous adnexal carcinomas with basaloid features, 18 benign basaloid adnexal neoplasms of the skin (nine spiradenomas and nine cylindromas), and two cutaneous PNETs, using a standard immunohistologic technique and microwave-mediated epitope retrieval. Of the 30 MCC, 12 showed crisp membrane staining for CD99. Among the remaining tumors, only the two PNETs were positive for that marker. Although the majority of MCCs did not label for CD99, the pattern of reactivity in positive cases was indistinguishable from that observed in PNETs. Eighteen of 27 MCCs that were stained for CK20 were reactive for that protein, in contrast to metastatic small cell carcinomas, cutaneous PNETs, and appendageal skin tumors, which were uniformly negative for this marker. However, a subset of nine tumors, which were most consistent with MCC on clinical grounds, was CD99 positive and CK20 negative. Hence, reliance on CD99 alone as a diagnostic marker for PNET in this context cannot be recommended. Rather, careful assessment of the clinical presentation, together with extended immunophenotyping that includes other lineage markers and, when possible, cytogenetic analysis for characteristic chromosomal aberrations, remains the best means of separating MCC from PNET. Finally, the lack of CD99 reactivity in basaloid adnexal neoplasms of the skin suggests a utility in their differential diagnosis from cutaneous tumors with neuroendocrine or neuroectodermal differentiation.
 ...
PMID:CD99 and cytokeratin-20 in small-cell and basaloid tumors of the skin. 1093 47

Although considered a specific marker for Ewing's sarcoma/peripheral neuroectodermal tumour, the MIC2 gene product (CD99) has been immunolocalised in a variety of human tumours. The present study evaluated immunohistochemically the prevalence of CD99 expression in a series of 68 neuroendocrine tumours of different gastrointestinal and pulmonary sites. We now report on membrane and/or granular cytoplasmic immunoreactivity in 25% of these tumours, independent of their anatomical sites. In lung neuroendocrine tumours, CD99 was preferentially confined to typical carcinoids (P=0.009). A statistically significant relationship was observed between the number of CD99 positive cells but not the immunostaining patterns and the presence of local invasion and/or distant metastases (P<0.001). Moreover, there was a tendency for CD99-reactive tumours to show a reduced proliferative activity expressed by a Ki67 index of 2% (P=0.119). The number of CD99 immunoreactive cells or patterns of immunoreactivity did not correlate with the presence of associated clinical syndrome or particular hormonal immunostaining. Although the molecular basis underlying CD99 expression in neuroendocrine tumours is still poorly understood, our data suggest that CD99 may be involved in cell-to-cell adhesion of neuroendocrine tumour cells and in downregulation of their proliferative activity.
 ...
PMID:CD99 immunoreactivity in gastrointestinal and pulmonary neuroendocrine tumours. 1103 47


1 2 3 4 5 6 7 8 9 Next >>