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)

The blastemal component of Wilms' tumor (WT) might be indistinguishable histologically from other small, blue, round-cell tumors of childhood, including alveolar rhabdomyosarcoma (RMS), particularly in small biopsy specimens and in the setting of metastatic disease. Furthermore, there are currently no reliable blastemal markers. Deparaffinized sections of 9 formalin-fixed blastema-predominant WTs and 46 RMSs were immunostained with antibodies to desmin (D33), myogenin (F5D), MyoD1 (5.8A), and muscle-specific actins (HHF35), after heat-induced epitope retrieval. WE defined as positive those cases with more than 5% of cells immunostained (only nuclear staining was considered as positive for myogenin and MyoD1). Antibodies to desmin were positive in eight (89%) of nine cases of blastema-predominant WT; in contrast, no case was positive for any of the other muscle-associated proteins. Of the 46 cases of RMS, all were positive for desmin, 42 were positive for myogenin and MyoD1, and 43 were positive for muscle actins. Desmin immunoreactivity, of and by itself, cannot be considered specific for RMS, but when accompanied by immunoreactivity for other myogenic proteins, it is highly characteristic of RMS. Our data also suggest that desmin immunoreactivity, in the absence of other muscle-associated protein expression, might be considered a clue to the diagnosis of the blastemal WT. Particularly in the context of small biopsy specimens or in metastatic settings, the use of a panel of antibodies to desmin as well as to other myogenic proteins, such as MyoD1 or myogenin, can help to discriminate between WT and RMS. Additional studies are required to determine whether desmin immunoreactivity in the blastemal component of WT represents true desmin expression.
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PMID:Antibodies to desmin identify the blastemal component of nephroblastoma. 931 Sep 52

A case of endometrial stromal sarcoma (ESS) showed cystic pulmonary metastases mimicking lymphangiomyomatosis (LAM). A 58-year-old female, who had undergone total hysterectomy for low-grade ESS 16 years previously, had repeated bouts of pneumothorax. Multiple thin-walled cysts in the peripheral lung were revealed by radiological examinations. In an open-lung biopsy specimen, cystic lesions were surrounded by layers of spindle-shaped cells of varying thickness that resembled LAM. However, in addition to subtle histologic differences from LAM, HMB45 (antimelanoma antibody) showed positive in LAM (n = 3), but was negative in ESS (n = 2) and the cystic lesions of this case. Using myogenic markers (desmin and alpha-smooth muscle actin), metastatic ESS could be immunohistochemically differentiated from mesenchymal cystic hamartoma (n = 1). HMB45 immunohistochemistry is useful in the differential diagnosis of cystic pulmonary lesions.
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PMID:Cystic pulmonary metastases of endometrial stromal sarcoma of the uterus, mimicking lymphangiomyomatosis: a case report with immunohistochemistry of HMB45. 936 Nov 9

Childhood kidney tumors seldom metastasize into the cranial cavity unless it is a special histological variant. We report a 4-year-old boy with multiple intracranial metastases in the left parietotemporal and right cerebellar area from primary clear cell sarcoma of the kidney without evidence of bony metastases. Metastatic tumor revealed nests of uniformly polygonal cells with clear cytoplasm demarcated by delicate fibrovascular arcades. Tumor cells were positive for vimentin and negative for cytokeratin, S-100 protein, desmin, and myoglobin. Cellular proliferation rate measured by PCNA, and Ki-67 was not significantly different between primary tumor mass and metastatic brain lesion. Expression of p53 oncoprotein was not evident in both lesions. These findings suggested that the relapse and metastasis of clear cell sarcoma of the kidney was probably due to regrowth of micro-metastases which were present at an early stage of disease.
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PMID:Intracranial metastasis from clear cell sarcoma of the kidney--a case report. 936 10

The case presents a 61 year old woman which came into the hospital with left heart failure. After an unsuccessful trial to treat the heart failure in an habitual method the echocardiographic showed a mass lesion which had filled out mostly of the left atrium. Histological features demonstrated a leiomyosarcoma with an origin from the wall of the left atrium. Immunohistochemical preparations revealed a positivity for actin, desmin and vimentin. Despite an operative resection and an attach of polychemotherapy it developed a local relapse and multiple lymph node metastases. Within the scope of this case report apart from an detailed bibliography it ought to be discussed the origin of the tumor into the atrium and the differential diagnosis from other sarcoma.
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PMID:[Primary leiomyosarcoma in the left atrium--a rarity. Case report and literature review]. 945 38

Sarcomas and related proliferative lesions of the specialized prostatic stroma have been the subject of case reports and, thus, have not been well characterized. We reviewed the clinicopathologic features of 22 cases and studied the immunohistochemical profile of 9. Patient age ranged from 25 to 86 years; mean age was 54 years, and peak incidence was in the 6th and 7th decades. The most common clinical presentation was urinary retention, then abnormal results of digital rectal examination, hematuria or hematospermia, and a palpable rectal mass. The cases were grouped into two categories: prostatic stromal proliferation of uncertain malignant potential (PSPUMP, 18 cases) and prostatic stromal sarcoma (PSS, 4 cases) based on the degree of stromal cellularity and the presence of mitotic figures, necrosis, and stromal overgrowth. Four histologic patterns of PSPUMP were identified: (1) hypercellular stroma with scattered cytologically atypical cells associated with benign glands, (2) hypercellular stroma with minimal cytological atypia associated with benign glands, (3) hypercellular stroma with or without cytologically atypical cells, associated with benign glands in a "leaflike" growth pattern that resembled phyllodes tumors of the mammary gland, and (4) hypercellular stroma without cytologically atypical stromal cells and without glands. Prostatic stromal sarcoma showed greater cellularity, mitoses, necrosis, and stromal overgrowth than PSPUMP and consisted either of stromal elements with benign glands in a pattern that resembled malignant phyllodes tumors of the mammary gland (3 cases) or of purely stromal elements (1 case). Positive immunohistochemical reactions were noted using vimentin in 9 of 9 cases, CD34 in 8 of 8, HHF-35 in 2 of 8, smooth muscle actin in 3 of 9, desmin in 4 of 8, S-100 protein in 0 of 9, estrogen receptor in 1 of 7, and progesterone receptor in 6 of 7. None of the cases classified as PSS were positive for HHF-35, smooth muscle actin, or desmin. Of the 13 patients classified as having PSPUMP who did not undergo definitive local therapy at the time of diagnosis, recurrent signs or symptoms were seen in six (46%), necessitating additional therapy. Distant metastases to lung and bone developed in one patient classified as having PSS. Clinical and pathologic findings in this patient suggested a progression from PSPUMP to PSS. We conclude that sarcomas and related proliferative lesions of the specialized prostatic stroma encompass a spectrum of histologic features and may be grouped into two clinicopathologic categories: PSPUMP and PSS. Based on their distinctive histologic appearance and immunohistochemical profile, PSPUMP and PSS can be differentiated from other mesenchymal lesions of the prostate.
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PMID:Sarcomas and related proliferative lesions of specialized prostatic stroma: a clinicopathologic study of 22 cases. 998 56

Spindle cell rhabdomyosarcoma (RMS) is a recently described variant of embryonal RMS that carries a relatively favorable prognosis when compared with other forms of RMS. To date, spindle cell RMS has been described only in children. The authors have identified two unusual cases occurring in adults using the following criteria: tumors composed mainly of fascicular, relatively monomorphic spindle-shaped cells that show unequivocal immunohistochemical and ultrastructural evidence of myogenic differentiation. The tumors were identified in a 38-year-old woman and a 56-year-old man, arising in the cheek and left hemidiaphragm, respectively. Both were treated with surgical resection and chemotherapy. The first patient died of uncontrolled local recurrence of her tumor at 27 months after diagnosis, and the second died of metastatic disease at 13 months follow-up. The tumors were composed mainly of fascicles of spindle cells with palely eosinophilic cytoplasm admixed diffusely with sparse polygonal, rounded, or strap-shaped rhabdomyoblasts with brightly eosinophilic cytoplasm and with cross-striations in the first case only. Immunostaining for muscle-related antigens showed staining for smooth-muscle actin (focal), pan-actin HHF-35, desmin, fast myosin, myoglobin, and MyoD1. Both cases were negative for S-100 protein. On electron microscopy, both cases showed neoplastic rhabdomyoblasts with clear-cut sarcomeric differentiation in many of the tumor cells. Spindle cell RMS poses special problems in differential diagnosis when arising in adults and should be distinguished from leiomyosarcoma, malignant peripheral nerve sheath tumor with heterologous rhabdomyoblastic differentiation (malignant Triton tumor), and fibrosarcoma. In view of the good prognosis afforded children with spindle cell RMS and in light of the chemoresponsive behavior of RMS in general, we feel that it is important to identify tumors that meet the criteria for spindle cell RMS occurring in the adult population. However, based on these two cases, it is possible that spindle cell RMS occurring in adults may not be associated with such a favorable outcome.
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PMID:Spindle cell rhabdomyosarcoma (so-called) in adults: report of two cases with emphasis on differential diagnosis. 953 74

Alveolar soft-part sarcoma (ASPS) is a rare tumor of uncertain histogenesis, mainly localized in the extremities and less frequently found in the head, neck and trunk. The present report describes two cases of ASPS localized in the uterus. In general, this entity is very rarely encountered in the female genital tract. Including the two cases presented here, 27 patients have been described in the literature. Whereas the prognosis for ASPS in soft tissues is usually poor (most of the patients died of lung metastases), those localized in the female genital tract are associated with a much better survival. This could be explained by the fact that their diameter seldom exceeds 5 cm, the size which is regarded as being the critical prognostic limit. Histologically, both tumors showed the same characteristics as known for ASPS in other localizations: organoid or nest-like arrangement of tumor cells; rounded or polygonal cells with cytoplasm of varying density; thin-walled, sinusoidal vascular spaces between tumor cell nests. Immunohistochemically, we found positivity for desmin and, to a lesser extent, for vimentin. Cytokeratin was negative, which is important in differential diagnosis to other rare uterine neoplasias like clear cell (mesonephroid) adenocarcinomas or metastases.
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PMID:Alveolar soft part sarcoma of the uterine corpus. Report of two cases and review of the literature. 954 49

Three cases of intravenous leiomyomatosis (IVL) of the uterus, a rare benign smooth-muscle tumor, are described. A preoperative diagnosis of IVL was not made in any of the patients, all of which presented with a pelvic mass with the presumptive diagnosis of leiomyoma. Surgical exploration confirmed the presence of uterine mass and two of the three cases showed extra-uterine extension into the ovarian or uterine veins. Histological examination demonstrated a fascicular pattern of bland spindle-shaped smooth-muscle cells, which extended to veins inside the myometrium or to extrauterine veins. This was confirmed by immunohistochemical stain for desmin and factor VIII. Despite their histological benignity, these lesions have a tendency to metastasize and are closely related to the conditions called "benign metastasizing leiomyoma" and "intracaval mass and cardiac extension". The primary treatment of IVL is hysterectomy and excision of any extrauterine tumor, when technically feasible. Anti-estrogenic therapy has been suggested as potentially useful in controlling of unresectable tumor. According to the literature, the follow-up must be long and periodic postoperative ultrasonic or magnetic nuclear resonance imaging studies may be useful in detecting growth of residual intravascular tumor.
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PMID:Intravenous leiomyomatosis of the uterus. A report of three cases. 955 21

We report 51 cases of a previously undescribed tumor of the distal extremities that is often mistaken for an inflammatory or infectious process, Hodgkin's disease, or various sarcomas. These lesions developed in patients of all ages (range, 4-81 yr; median, 40 yr) and affected the sexes nearly equally (27 men, 24 women). They presented as a painless mass of the fingers (14 cases), hand (11 cases), wrist or arm (10 cases), toe or foot (8 cases), or lower leg (5 cases), usually within the subcutaneous tissues. Grossly, they were infiltrative, multinodular masses characterized by a dense chronic inflammatory infiltrate that merged with a stroma, which varied from densely hyaline to focally myxoid and contained sheets of short spindled to rounded epithelioid cells. Focally, the epithelioid cells were extremely large with bizarre, vesicular nuclei and macronucleoli resembling Reed-Sternberg cells or virocytes. Despite the level of atypia, mitotic activity was low. The tumor cells consistently expressed vimentin but lacked a variety of other mesenchymal, epithelial markers, e.g., S100 protein, desmin, actin, neuron-specific endolase, epithelial membrane antigen, HMB-45, CD34) and leukocyte markers (CD15, CD30, CD45). Keratin was noted focally and weakly in four cases and CD68 focally in six cases, the latter suggesting that the cells had acquired phagocytic properties. Immunostains for cytomegalovirus were negative. Polymerase chain reaction for Epstein-Barr virus showed amplification levels consistent with latent infection in 4 of 10 cases, but no cases showed levels consistent with active infection. All of the bacterial and viral cultures were negative. Follow-up information was available in 27 cases. Recurrences developed in six patients (interval, 15 mo-10 yr), but there were no metastases or tumor-related deaths. In one patient, progressive proximal extension up the arm was noted. Although the most common submitting diagnosis was that of an inflammatory or infectious process, the negative studies for infectious agents, clinical behavior with local recurrences, immunophenotypic profile, and cytologic atypia support the idea that these are unusual mesenchymal neoplasms with at least the potential for local recurrence. It remains to be investigated whether with time these lesions will prove to have metastatic potential.
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PMID:Inflammatory myxohyaline tumor of distal extremities with virocyte or Reed-Sternberg-like cells: a distinctive lesion with features simulating inflammatory conditions, Hodgkin's disease, and various sarcomas. 957 90

A follow-up investigation of 25 cases of extraskeletal osteosarcomas diagnosed at the Center for Bone and Soft Tissue Tumors, Aarhus University Hospital, Denmark, in the period from 1970-1995 was undertaken. The immunohistochemical profile of these tumors was evaluated using a panel of 10 antibodies, and the value of alkaline phosphatase staining in differential diagnostic situations also was considered. The study revealed that this tumor is high-grade malignant and affects adults (median age, 67 years; range, 35-82 years) at diagnosis. The thigh (52%) was the most common tumor location. Seven tumors were superficial, whereas the remaining 18 were intramuscular. Two patients with superficial tumors previously received radiation to the area. Local recurrences developed in 9 (36%) patients and distant metastases developed in the lungs in 15 (60%) patients as the most common site. Median survival time was 24 months, and the cause-specific survival rate at 5 years was less than 25%. Thirteen (52%) intramuscularly located extraskeletal osteosarcomas were of the fibroblastic subtype, often with sparse amounts of osteoid. They could be separated from malignant fibrous histiocytoma on the basis of a strongly positive alkaline phosphatase reaction. Immunohistochemistry did not reveal characteristic features because positivity for vimentin, occasional positivity for desmin, actin, S-100, epithelial membrane antigen, cytokeratin, and p-53 may be observed in many other pleomorphic sarcomas. Various histopathologic factors, such as tumor size, tumor depth, histopathologic subtype, malignancy grade (IIIA versus IIIB), MIB-1, and p53 reactivity were analyzed in relation to clinical course. Only MIB proliferation was correlated to prognosis, with significantly longer survival in patients with tumors with MIB-1 values less than 24%. Our study has shown extraskeletal osteosarcoma to behave in a highly aggressive fashion. Alkaline phosphatase staining compared with immunohistochemistry proved to be superior in the differentiation from other pleomorphic sarcomas.
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PMID:Extraskeletal osteosarcomas: a clinicopathologic study of 25 cases. 959 29


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