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)

The symptoms and therapy of a multicentric angiomyolipoma of the kidney, adrenal gland, paraaortic lymph nodes and renal fat tissue in a 9-year-old boy with tuberous sclerosis are reported. Angiomyolipomas are benign mesenchymal tumours that often occur together with tuberous sclerosis. Renal cell carcinoma in an angiomyolipoma is rare. Multicentricity and lymph node involvement is not a sign of malignancy or metastatic disease. Radiological characteristics in CT and plain X-ray may help in the diagnosis. Characteristically, the renal lesions are asymptomatic. Patients with incidental symptom-free angiomyolipoma should be followed. In other patients with pain in the loin, or when a solid tumour cannot be confidently excluded, conservative surgery or nephrectomy should be performed.
 ...
PMID:[Multilocular, giant angiomyolipoma of the kidney, adrenal gland and para-aortic lymph nodes. Case report of a 9-year-old boy with tuberous sclerosis]. 797 36

Primary meningeal hemangiopericytomas (formerly referred to as angioblastic meningiomas) are by most authors no longer considered to be actual meningiomas but rather thought to be intracranial hemangiopericytomas. Their biological behavior is usually malignant, with recurrences and metastases, often at intervals of years. Both intracranial and extracranial hemangiopericytomas may, however rarely, be accompanied by paraneoplastic hypoglycemias. Our own characteristic observations are based on a female patient aged 67 at the time of her death, in whom a meningeal tumor was resected first at the age of 41. Later recurrencies were removed at the age of 52 and 58 respectively. Three years prior to her death liver metastases had developed followed by increasingly frequent attacks of early morning hypoglycemia with blood sugar levels ranging between 1.4-2.3 mmol/l. Specific examinations revealed low endocrine production of insulin and a distinctly decreased insulin-like growth factor (IGF) I of 25 ng/mb (normal 120-130) and a normal value of total IGF II of 724 ng/ml (normal 400-900), though with a big macromolecular share. The observed paraneoplastic hypoglycemia is probably brought about by coincidence of blocked hepatic glucose production, suppressed lipolysis and increased peripheral glucose uptake. Autopsy revealed a third intracranial recurrence of meningeal hemangiopericytoma and a large metastatic liver. No other sites of metastases were found. Histologic, immunohistologic and electron microscopic findings showed the characteristics features of a hemangiopericytoma. Light microscopic pictures of the primary tumor, recurrences and metastases were identical. Additional autopsy findings were a papillary carcinoma of the right kidney, an angiomyolipoma of the left kidney and a thecoma of the left ovary.
 ...
PMID:[Recurrent and metastasizing hemangiopericytoma of the meninges with paraneoplastic hypoglycemia]. 812 96

A 40-year-old man was admitted to our hospital with a fat-density extrarenal tumor and intrarenal nodules which were detected by ultrasonography (US) and CT scan obtained the general health check. After his admission, US, CT, MRI and angiography were performed. As the final diagnostic procedure, we performed a US-guided percutaneous needle biopsy of the extrarenal tumor, because this tumor was difficult to distinguish from liposarcoma and angiomyolipoma (AML). The pathological study revealed a well-differentiated liposarcoma, and the extrarenal tumor and the left kidney were removed en bloc. Pathologically, the tumor not only invaded into the renal parenchyma directly but also have metastasized independently to the intrarenal region. Postoperatively, he received a tumor dose of irradiation of 50 Gy. Liposarcoma arising in the perirenal space should be considered in the differential diagnosis of exophytic AML. If radiological studies can not confirm the appropriate diagnosis, we consider that biopsy of the tumor should be selected as an alternative diagnostic approach. And, we think that this is the first case report dealing with intrarenal metastases of liposarcoma.
 ...
PMID:[Retroperitoneal liposarcoma with intrarenal metastases which was difficult to distinguish from angiomyolipoma. A case report]. 830 25

Lymphangiomyomatosis (LAM) is a rare disease that does not generally affect the female genital tract. We report two cases of uterine involvement by LAM in young women with tuberous sclerosis and renal angiomyolipomas. In both, the uterine lesions were grossly inapparent and were discovered during microscopic examination of hysterectomy specimens removed during surgical treatment for a primary ovarian adenocarcinoma with peritoneal and lymph node metastases in one case and a retroperitoneal lymphangiomyoma in the other. In one case, an area of uterine LAM with atypical features was interpreted as focal sarcomatous transformation. This patient also had pelvic and paraaortic lymph node involvement by typical lymphangiomyomas, a small uterine angiomyoma, and an occult primary endometrial adenocarcinoma. Immunostains for HMB-45 were strongly positive in the uterine LAM in both cases, the retroperitoneal and lymph node lymphangiomyomatous lesions, the uterine angiomyoma, and a resected renal angiomyolipoma. Although LAM is a rare uterine lesion, it must be distinguished from a variety of uterine smooth-muscle tumors.
 ...
PMID:Lymphangiomyomatosis of the uterus associated with tuberous sclerosis and malignant neoplasia of the female genital tract: a report of two cases. 859 38

The perirenal space may be involved by disease processes that arise within or outside the perirenal space. Key anatomic details that dictate the features of perirenal processes include the renal capsule, the perirenal septa, the renal fascia, and the conic shape and inferomedial orientation of the perirenal space. Superiorly, the perirenal space is open to the bare area of the liver. The perirenal spaces communicate with one another at the level of the lower lumbar vertebrae. The hallmark of perirenal infection is localized or diffuse gas. Chronic urinoma appears as an encapsulated cystic mass, often aligned parallel with the perirenal space. Fat within an apparent spontaneous hematoma of the perirenal space suggests angiomyolipoma. Renal cell carcinoma, lymphoma, and melanoma are the most common causes of discrete solid masses in the perirenal space; metastases occur due to the characteristic lymphatic and vascular supply of the space. Amyloidosis and fibrosis create a nonspecific rind of soft tissue around the kidneys. Diaphragmatic pseudotumor produces a linear "lesion" in the perirenal space.
 ...
PMID:The perirenal space: relationship of pathologic processes to normal retroperitoneal anatomy. 883 75

In detection and work-up of a renal carcinoma, imaging plays the most critical role. Differentiation of renal cell carcinoma from angiomyolipoma and complex cysts is highly accurate, but differentiation from benign oncocytoma is not. The most important discoveries regarding local staging are presence of venous tumor thrombus and its cephalad extension, followed by regional lymph node metastases. Biopsy of a renal mass is indicated when a concomitant primary malignancy is present elsewhere, but only if treatment will be influenced by findings. Preoperative embolization is reserved for the most difficult cases, and palliative embolization is useful in selected cases. Helical computed tomography is the most sensitive method for detecting small lung metastases.
 ...
PMID:Imaging work-up: is it renal carcinoma and is it operable? 894 17

The authors update the current status of diagnostic and staging work-up and therapy of renal cell carcinoma (RCC). They first point out that the disease is increasingly discovered incidentally (about 30% of cases) when symptoms are absent. This, on average, has not led to a clear variation in stage distribution at first observation. It is not rare, however, to find very small lesions, for which differential diagnosis and particular therapeutic strategy are needed, because in some instances small lesions can give distant metastases. Hematuria remains the onset symptom in about 60% of cases where in about 20% of cases systemic symptoms or paraneoplastic syndromes are present. Distant metastases at presentation are still not rare, being observed in about 6-15% of patients. A review of diagnostic tools is then made, concluding that CT scan should be considered the most sensitive examination. The differential diagnosis with oncocytoma, angiomyolipoma and the so-called 'pseudo-tumors' is discussed in detail because these are the most frequently observed renal lesions out of RCC. Special attention is reserved for the diagnostic problems of renal cysts, suggesting that the Bosniak classification should be generally followed, and to the indications for fine-needle aspiration and biopsy which should be performed only in very selected cases. Minimal requirements for staging are indicated after a survey of the most common diagnostic methods. In treatment issues, the efficacy of lymph node dissection and adrenalectomy are discussed, concluding that the present body of data is still unable to clearly indicate that there is an absolute indication for extensive lymph node dissection whereas in selected cases partial nephrectomy may be a valid therapeutic option. Results of immunotherapy, cytotoxic therapy and their association are finally summarized as well as future prospects.
 ...
PMID:Renal cell carcinoma--diagnosis and treatment: state of the art. 907 86

Two case reports of hepatic angiomyolipoma, both originating in the caudate lobe, are reported with a review of the literature. The liver is the second most common site of angiomyolipoma, an uncommon benign tumor of mixed mesenchymal origin. It is commonly diagnosed following abdominal pain or as an asymptomatic mass discovered on abdominal ultrasound or computed tomography scan. Of 74 cases reported, the lesions ranged from 0.3 to 36 cm in diameter and are noted between the first and eighth decade, with predominant female predilection. The right lobe is the most common site, with lesions arising in the caudate lobe comprising only five cases. The natural history of the hepatic lesion is unknown. Malignant invasion or metastatic disease has not been documented. Hepatic and renal angiomyolipoma can occur concurrently (13 of 60 cases), although the majority are not biopsy proven. Multicentric hepatic disease occurs. The correlation between tuberous sclerosis and hepatic angiomyolipoma is not confirmed histologically and occurs rarely. These lesions have a characteristic radiographic appearance due to high fat content. Histologically, angiomyolipoma are characterized by an admixture of adipose tissue, blood vessels, and smooth muscle cells. These lesions cannot reliably be differentiated from a malignant lesion based on clinical history, radiologic examination, and/or pathologic interpretation. If clinical suspicion for malignancy is low, then careful observation with serial radiologic follow-up is performed. The treatment for a symptomatic or suspicious lesion is resection, if feasible. Liver transplantation may be considered for large or centrally located lesions not amenable to resection.
 ...
PMID:Hepatic angiomyolipoma: two case reports of caudate-based lesions and review of the literature. 937 58

Primary renal angiosarcoma is very rare. To our knowledge, only 15 cases have been reported to date. A 77-year-old Japanese man with a unilateral kidney presented with massive hematuria followed by renal failure. A renal tumor was suspected and a left nephrectomy was performed. The histopathological diagnosis was angiosarcoma of the kidney. A hemorrhagic tumor measuring 10 x 5 cm and clotted blood was found in the medullary area. The atypical tumor cells had a sinusoidal and solid appearance, and showed immunohistochemically positive reactions for some of the endothelial markers. The patient died about 21 months after the nephrectomy and the autopsy revealed massive metastases to the liver and retroperitoneum. One of the differential diagnoses of the case was angiomyolipoma, because the tumor cells were relatively bland in their histological appearance with entrapped fat cells in the pelvic area. Fifteen case reports with titles that included the term 'hemangiosarcoma/angiosarcoma', 'hemangioendothelioma/endothelioma' or 'vascular sarcoma' of the kidney were reviewed and compared to the present case.
 ...
PMID:Primary renal angiosarcoma: a case report and review of the literature. 941 38

Renal epithelial tumors (carcinoma and oncocytoma) have been reported with higher a frequency than expected in patients with the tuberous sclerosis complex. However, the recent identification of a monotypic, epithelioid variant of angiomyolipoma, closely simulating renal cell carcinoma, has cast doubt on the real frequency of carcinoma. Immunohistochemical analysis with a panel of antibodies, including melanogenesis marker HMB45, can discriminate between carcinoma and carcinoma-like angiomyolipoma. We studied five tumors previously reported as carcinoma and found that only one of them showed an immunohistochemical phenotype indicative of an epithelial tumor (Ker+, HMB45-). Three tumors exhibited a phenotype compatible with the monotypic epithelioid variant of angiomyolipoma (HMB45+, Ker-), and two of the three patients died of metastatic disease. The last patient had unusual clinical features, and the tumor was positive both for HMB45 and keratin. It is concluded that (1) renal cell carcinoma is less common in tuberous sclerosis complex than previously believed, (2) some cases called renal cell carcinoma probably represent a monotypic, epithelioid variant of angiomyolipoma, and (3) epithelioid angiomyolipoma is a potentially malignant tumor with invasion and metastases. These findings indicate that all reported renal carcinomas in tuberous sclerosis complex, therefore, must be reevaluated.
 ...
PMID:Apparent renal cell carcinomas in tuberous sclerosis are heterogeneous: the identification of malignant epithelioid angiomyolipoma. 1007 31


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