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)

Leiomyosarcomas of the spermatic cord are very rare tumours, which, in contrast to the rhabdomyosarcoms of childhood, occur almost exclusively in adults. We report the case of a 51-year-old male patient with a metastasizing, pleomorphic leiomyosarcoma of the spermatic cord. The tumour showed paraneoplastic secretion of beta-chain human chorionic gonadotropin (beta-hCG), as was demonstrated by the elevated levels of beta-hCG in serum samples and by immunohistochemistry. Histologically, the tumour was a high-grade leiomyosarcoma which showed an aggressive course with pulmonary metastases appearing 4 months after primary surgery. A concomitant rise in the serum levels of beta-hCG was also noted at this time. A wide spectrum of tumours with a choriocarcinomatous component or paraneoplastic production of beta-hCG has been described, the vast majority being carcinomas. Only two leiomyosarcomas producing beta-hCG have so far been reported. The paraneoplastic production of beta-hCG should prevent confusion with germ-cell tumours, especially teratomas.
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PMID:[Leiomyosarcoma of the spermatic cord with paraneoplastic beta-hCG production]. 955

Dermatofibrosarcoma protuberans (DFSP) is a locally aggressive fibrohistiocytic tumor of intermediate malignancy with potential for local recurrences and with a small, but well recognized risk of distant metastases. We report a case of a 43-year-old Filipino male with an otherwise typical cutaneous DFSP followed 4 years later by a local recurrence of tumor with subsequent pulmonary metastasis at year 5, and 3 sequential central nervous system (CNS) metastases at years 5, 6, and 12 following the original diagnosis of DFSP. Recurrent and metastatic tumors showed areas of fibrosarcomatous change, high cellularity, high mitotic rate (10 - 15 per 10 high power fields), loss of CD34 expression, and aneuploidy. DFSP has not been previously reported to metastasize to the CNS. In addition, we report complex karyotypic aberrations within the metastasis, which have not been previously associated with DFSP. The dural-based location of the intracranial metastasis, together with the light- and electron-microscopic appearance, could cause diagnostic confusion with an anaplastic syncytial meningioma.
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PMID:Metastatic dermatofibrosarcoma protuberans mimicking meningioma. 970 32

Three patients with hydrocephalus secondary to central nervous system (CNS) metastases from lung or breast cancer are reviewed representing less than 5% of patients with CNS metastases seen at The Cancer Center of Boston over a 10-year period. The clinical picture is characterized by ataxia and mental confusion with dilated ventriculi on computed tomography (CT) scan of the brain. Computerized tomography of the brain and microscopic analysis of the cerebrospinal fluid are complementary in establishing the diagnosis of meningeal carcinomatosis. Surgical management by ventricular peritoneal shunt is an important component to multimodality therapy. The clinical course and extended survival in three patients provides a basis for recommending palliative surgical bypass as a therapeutic intervention with or without intrathecal chemotherapy, radiation, or both.
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PMID:Malignancy-related hydrocephalus: clinical features and results of ventricular peritoneal shunt procedure in three patients. 970 35

There are six major steps in the management of patients with neuroendocrine tumors (NETs) (carcinoids and pancreatic endocrine tumors). One of the steps that is increasing in its importance is the need to assess primary tumor location and tumor extent in these patients. Without such information, it is not possible to adequately manage these patients. Conventional imaging studies (CT scan, MRI, ultrasound, angiography), functional localization studies measuring hormonal gradients, endoscopic ultrasound, and most recently, somatostatin receptor scintigraphy (SRS) with [125I-DTPA-DPhe1]-octreotide have all been advocated to localize NETs in different studies. Whereas it is now established that for all NETs, except insulinomas, SRS has the greatest sensitivity, it remains unclear whether this increased sensitivity translates into increased clinical usefulness. It, therefore, remains unclear based on fiscal and clinical considerations what should be the recommended algorithm for the use of the different localization methods. To address this issue, we have recently performed two prospective studies on patients with gastrinomas. In this paper, the methods and results of each are summarized and based on these results, an algorithm for localization studies in NETs is proposed. One study assessed the role of SRS in management in 122 patients and shows that the use of SRS changed management in 47 percent of patients according to six different criteria when the patients were stratified according to their principal management problem. Determining whether liver metastases were present is one of the major goals of tumor localization studies and is frequently a source of confusion because of the difficulty in distinguishing small NETs liver metastases from hemangiomas. In the second study, the ability of SRS and other tumor localization methods to distinguish these two possibilities was assessed in 15 patients with small hemangiomas and 15 patients with small hepatic metastases (mean size 1.3 cm). SRS correctly identified 93 percent of the patients with liver metastases and was not positive in any patient with a hemangioma, suggesting it was not a liver metastases. SRS had greater negative and positive predictive value than conventional studies. Based on these two studies, and SRS's greater sensitivity and fiscal considerations, it is proposed that SRS should be the initial tumor imaging study in all NETs except insulinomas, and algorithms for the use of other localization studies in both NETs and insulinomas are proposed.
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PMID:Definition of the role of somatostatin receptor scintigraphy in gastrointestinal neuroendocrine tumor localization. 982 76

Neuroendocrine neoplasms of the larynx have been divided into those of epithelial or neural origin. The latter consist of paragangliomas while the epithelial origin group can be divided into the typical and atypical carcinoids and small cell neuroendocrine carcinomata, the latter consisting of the oat cell type, the intermediate cell type and the combined cell type. There are now over 500 cases of neuroendocrine neoplasms of the larynx in the literature. The diagnosis is primarily based on light microscopy, and, in some instances, it may be supported by special histochemical studies. It should be confirmed by immunocytochemical and/or ultrastructural investigation. The different biological behaviour of neuroendocrine neoplasms of the larynx makes a specific diagnosis of paramount importance, since treatment depends on diagnostic accuracy. Typical carcinoid is an extremely rare lesion. It is treated preferably by conservative surgery; elective neck dissection is not necessary because of the lack of lymph node metastases at diagnosis. Chemotherapy and/or radiotherapy have not been effective in the limited number of patients treated thus far. Prognosis is excellent with cure following surgery. Atypical carcinoid is the most frequent non-squamous carcinoma of the larynx. The mainstay of treatment is surgery. Elective neck dissection should be performed because of the high likelihood of cervical lymph node metastases. Primary radiation therapy with adjuvant chemotherapy is not indicated. The survival rate is 48 per cent at five years and 30 per cent at 10 years. Although the larynx is one of its most common extrapulmonary sites, small cell neuroendocrine carcinoma is still a rare tumour. Surgical results for this tumour have been disappointing and is reserved for cases of local relapse with no evidence of metastasis. Chemotherapy and radiotherapy currently appear to offer the least disabling and most effective forms of therapy. The two- and five-year survival rates are 16 per cent and five per cent, respectively. Paraneoplastic syndromes have occasionally been reported in association with carcinoid tumours (typical and atypical) and small cell neuroendocrine carcinoma. There have been also rare reports of an elevated neuropeptide serum level. Paraganglioma is the only laryngeal neuroendocrine neoplasm with a female preponderance (3:1). Confusion with atypical carcinoid has led to incorrect diagnosis and inappropriate classification schemes, erroneously suggesting that laryngeal paraganglioma has the potential for aggressive behaviour. Conservative surgery represents the treatment of choice; elective neck dissection is not necessary, and the prognosis is excellent.
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PMID:A review of neuroendocrine neoplasms of the larynx: update on diagnosis and treatment. 987 71

The term haemangioendothelioma has been used in the past for a number of vascular lesions, which vary not only by their morphological features, but more importantly, also by their biological behavior. In the recent WHO-classification of mesenchymal tumours haemangioendotheliomas have been defined as vascular tumours of "intermediate" or "borderline" malignancy, and spindle cell haemangioendothelioma (SHE), epithelioid haemangioendothelioma (EHE), and rare malignant endovascular papillary angioendothelioma (Dabska's tumour) were included in this category. To this list might be added the more recently delineated kaposiform (KHE), retiform (RHE), polymorphous (PHE), and composite haemangioendothelioma (CHE). Although very popular, the concept of "borderline" or "intermediate" malignancy encompasses a wide variety of clinical situations, prognosis, and biological behavior. Therefore uncritical use of the term haemangioendothelioma represents a potential source of confusion to patients and oncologists, and it should not be used without further clarification. SHE was originally described as low-grade angiosarcoma, however, the study of large series with expanded follow-up information clarified that these lesions are often multicentric in one anatomic region, whereas true recurrences are rather rare, and systemic metastases and tumour progression do not occur. Therefore redesignation of these lesions as spindle cell haemangioma has been proposed. EHE of skin and soft tissues represents a distinctive vascular neoplasm characterized by nests and cords of epithelioid endothelial tumour cells with characteristic cytoplasmic vacuoles, which are set in a myxohyaline matrix. The reported rates of systemic metastases (20-30%), and tumour related death of patients (13-17%) in EHE, and the occurrence of multicentric EHE argue against the classification of EHE as a low-grade or "borderline" malignant neoplasm; EHE should be better regarded as a clearly malignant vascular tumour (G2). Although it seems that KHE is associated with a high mortality rate, the deaths are almost always related to locally invasive effects or as result of bleeding and consumption coagulopathy. So far no metastasizing case of KHE has been reported, and it seems that the prognosis in KHE is mainly related to size, anatomical site and depth of the lesion. KHE should be classified as a locally aggressive, non-metastasizing vascular tumour. The remaining entities (RHE, Dabska's tumour, PHE, and CHE) are characterized by an infiltrative growth, a high rate of (often repeated) local recurrences, and a definitive risk of metastases. Therefore these lesions fulfil criteria for low-grade malignant vascular neoplasms.
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PMID:[Hemangioendotheliomas--evolution of a concept of a heterogeneous group of vascular neoplasms]. 1009 22

Osteoclast-like giant cells (GC) may dominate the histologic pattern not only in conventional giant-cell tumor (GCT)--originating as a radiologically pure lytic, possibly trabeculated lesion especially within the epiphyses of long tubular bones (LB) and pelvic bones of adults--but also in many tumor-like lesions as well as in various benign and malignant bone tumors which may simulate each other. Although the mononuclear cells as well as the amount of collagene fibres don't differ significantly, these lesions can be distinguished by substantial differences concerning their site, radiomorphology and the patients age. The unique lesion which can be recognized by histology only is chondroblastoma--centered in epiphyseal regions as GCT, mostly in the 2nd decade--by its typical mononuclear cells independently of the typical chondroid matrix and calcifications. The brown tumor of hyperparathyreoidism is associated with elevated serum Ca and parathormon, which is not altered in the histologically identical giant cell granuloma. In contrast to GCT aneurysmal bone cyst prefers the metaphyseal area of LB and the posterior parts of vertebras in the 2nd decade. Metaphyseal fibrous defects occurring during growth period leave the epiphyses unaffected and display typical x-rays. Villondoular synovitis sometimes can produce osteolytic defects. GC-rich malignant tumors which present with clear cut atypia except some cases of GC-variants of osteosarcoma, are: Giant-cell rich osteosarcoma, the rare malignant GCT, giant-cell ("osteoclastic") sarcoma, MFH and GC-rich metastases of carcinomas. All of them occur in middle aged and older patients except osteosarcoma and don't affect the epiphyses primarily except malignant GCT. To avoid confusion in GC-lesions it is conditio sine qua non to take into account for diagnosis not only histology but especially radiomorphology as well as site of the lesion and patients age.
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PMID:[Differential diagnosis of giant cell tumor of bone]. 1009 27

The animal and human studies presented, at first glance, present a confusing and conflicting story. In regards to the animal studies, much of this confusion can be traced to the use of a variety of different models, none of which truly reproduces the human situation. Nonetheless, there is much to be gleaned from these efforts. The authors present conceptualization of the port wound tumor dilemma follows. In order for wound tumors to develop, viable tumor cells must be liberated from the primary tumor and find transport to a wound. Rarely, patients with colon tumors will present with or will develop widespread intraabdominal carcinomatosis. These tumors have the ability to spontaneously shed considerable numbers of viable cells which have the ability to implant on uninjured peritoneal surfaces. Unfortunately, the surgeon has little chance for success in these patients with either open or minimally invasive methods. Fortunately, most colon adenocarcinomas do not spread in this manner. Differences in the clinical behavior and manifestations of colon tumors most likely reflect the genetic makeup of individual tumors. Colonic neoplasm's ability to invade and metastasize varies considerably from tumor to tumor. Thankfully, as mentioned, the vast majority of colon tumors are not prone to cause carcinomatosis. Despite this fact, the human data available suggests that tumor cells can be found in the peritoneal cavity using sophisticated methods in about half of the patients after colectomy. If this is the case, then why aren't more wound tumors seen? Logic dictates that there must be a critical number of free intraabdominal cells above which successful wound seeding is likely. It makes sense that traumatization of the tumor will result in increased numbers of liberated cells. Therefore, surgical approach and technique should impact considerably on outcome. For the majority of colon tumors, if the lesion is assiduously avoided during mobilization and resection, it is unlikely that enough tumor cells will be shed to result in port site tumors. The recent interim results of the Cleveland Clinic's and the Barcelona randomized trials certainly support such a hypothesis. With over 300 patients enrolled (combined series) and with an average follow up of over 2 years, in neither trial has a port site tumor been noted. Similarly, with an average follow up of just under 3 years, Franklin et al noted that there were no port site tumors in their prospective trial of 191 consecutive laparoscopic colectomies for cancer. In the clinical setting, experience and surgical expertise seem to be the best predictor of outcome, in regards to wound tumors. The few animal studies that allow assessment of the impact of technique (i.e. those that utilize an intraabdominal solid tumor model which allows tumor excision) indeed support this hypothesis. In these studies poor technique resulted in significantly more wound tumors. Furthermore, it has been shown that for laparoscopic procedures, there is a definite learning period during which the incidence of wound tumors is considerably higher than that of open resection. With experience the laparoscopic incidence falls to that of open resection. Furthermore, a number of recent studies suggest that is possible to lower the incidence of wound tumors via peritoneal and wound irrigation with a variety of agents. These animal study results are in keeping with the recent clinical results. Both would suggest that given proper and adequate training and with sufficient attention being paid to avoid tumor handling that the incidence of wound tumors will be as low as that following open colectomy. How large a part, if any, does the CO2 pneumoperitoneum play in the port wound tumor story? Certainly, the results of the bulk of the animal studies performed, to date, have suggested that the CO2 pneumoperitoneum plays a critical role in the development of port wound tumors. With few exceptions, these studies have utilized tumor cel
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PMID:Review of investigations regarding the etiology of port site tumor recurrence. 1019 87

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."
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PMID:Myxoid solitary fibrous tumor: a study of seven cases with emphasis on differential diagnosis. 1034 83

Malignant sweat gland tumors are rare tumors of the extremity. Their insidious growth patterns and often confusing pathological characteristics can cause confusion with more common benign tumors. However, these tumors cannot be neglected because they do have a propensity to metastasize. Presented is a 56-year-old woman with a malignant clear-cell hidradenoma of the foot actually presenting as a benign lesion.
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PMID:Malignant clear-cell hidradenoma of the toe. 1049 Jan 89


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