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)

Lymphangioma cavernosum of the kidney is a benign tumor. Metastasis has not been found in any of the 20 cases reported to date. The sex ratio of patients is 1.5 to 1, favoring female subjects. Average patient age is 33.7 years. Treatment has consisted of nephrectomy in 18 patients, since the possibility of a malignant tumor could not be eliminated preoperatively.
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PMID:Renal lymphangioma. 87 89

Lymphangiomas are rare, benign tumors which may become opacified during lymphography and may be confused with malignant lymph node disease. A case is reported in which lymphography showed focal collections of contrast material retained for several days in dilated lymphatic spaces which initially simulated metastatic disease.
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PMID:Lymphographic Demonstration of a Retroperitoneal Lymphangioma. 114 50

Primary vascular tumors of lymph nodes other than Kaposi's sarcoma are very rare, as attested to by only a handful of case reports in the literature. Based on an analysis of 39 such cases, we could distinguish five major groups. Hemangiomas of capillary/cavernous, lobular capillary, and cellular types were composed of compact aggregates of blood-filled vessels, variable in size, that replaced the nodal architecture partly or almost completely; some appeared to have originated in the hilum or medulla. These hemangiomas either represented incidental findings in lymph nodes or were seen with solitary lymph node enlargement; the evolution was benign with no recurrence. A distinctive benign lesion occurring exclusively in inguinal lymph nodes, which we propose designating "angiomyomatous hamartoma," showed replacement of the nodal parenchyma by smooth muscle cells and fibrous tissue, in continuity with exuberant proliferation of muscular vessels in the hilum. Epithelioid vascular tumors, characterized by plump endothelial cells with dense eosinophilic cytoplasm and numerous vacuoles, exhibited a range of differentiation, from hemangioma with well-formed vascular channels (with or without tissue eosinophilia) to hemangioendotheliomas composed predominantly of cords and sheets of tumor cells lying in a hyaline-myxoid matrix. Epithelioid hemangioendothelioma was particularly likely to be mistaken for metastatic carcinoma, and local recurrence could occur. A variant, the spindle and epithelioid hemangioendothelioma, was characterized by the presence of an additional component of spindle cells. Another tumor we found, polymorphous hemangioendothelioma, is a previously uncharacterized borderline malignant vascular tumor exhibiting solid, primitive vascular and angiomatous patterns and relatively bland cytologic features. Lymphangiomas of lymph nodes usually showed simultaneous multifocal and extra-nodal involvement and were characterized by cystic endothelium-lined spaces filled predominantly with lymph fluid. It is important to recognize these primary vascular tumors of lymph nodes to avoid mistaking them for a variety of benign vasoproliferative lesions, Kaposi's sarcoma, angiosarcoma, and metastatic cancer.
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PMID:Primary vascular tumors of lymph nodes other than Kaposi's sarcoma. Analysis of 39 cases and delineation of two new entities. 831 16

Three unusual clinical and histopathological changes were found in a 59-year-old male patient with congenital lymphedema of the lower left leg. Firstly, a keratotic lymphangioma had developed in the course of only a few years and in the end involved the entire left leg up to the buttocks--far beyond the edematous area. Secondly, concealed beneath the lymphangiokeratoma, a solitary tumor almost the size of a table-tennis ball was found on the outer side of the lower left leg. Histopathologically, the tumor proved to be a malignant angiosarcoma, which had grown over a period of some months. It was characterized histopathologically by solid and angiomatous differentiation. Thirdly, the superficial and deep lymph vessels revealed remarkable atypia and papillary proliferations of endothelial cells. After amputation of the left leg at the thigh, with complete removal of the angiosarcoma, but leaving the pathologically altered vessels in the upper part of the left leg and the buttocks, no clinical signs of metastases of progression have been noted during the first year after operation.
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PMID:[Progressive lymphangiokeratoma and angiosarcoma (Stewart-Treves syndrome) in congenital lymphedema]. 337 86

Hepatic lymphangioma is an extremely rare benign tumor, which usually occurs as part of a multiorgan lymphangiomatous involvement. We describe an adult patient with a huge hepatomegaly caused by a diffuse lymphangiomatous infiltration of the liver, which has never been reported before. Extrahepatic localizations could not be demonstrated. On ultrasonography and computed abdominal tomography, the liver lesions were not distinguishable from necrotic metastases. However, the endoscopic and histologic picture as well as the benign clinical evolution during a 2-yr follow-up allowed us to diagnose this rare benign disorder.
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PMID:Hepatic lymphangiomatosis. Report of a case and review of the literature. 388 71

Primary abnormalities of the peritoneum are rare in children. However, there is frequent secondary involvement of the peritoneal cavity and its specialized folds, the mesentery and omentum, in the presence of infectious, neoplastic, and traumatic conditions that originate at other sites in the abdomen or pelvis. Computed tomography (CT) is usually the modality of choice for evaluation of complex abdominal or pelvic pathologic conditions. Peritoneal cavity abnormalities include peritoneal fluid, pneumoperitoneum, and hemoperitoneum; peritoneal abscesses and peritonitis; metastases; and bladder or bowel rupture and solid organ injury. Mesenteric and omental abnormalities include an increase in or infiltration of mesenteric and omental fat; mesenteric lymphadenitis; mid-gut malrotation and bowel herniation; a variety of infections; metastases, lymphoma, and lymphangioma; and mesenteric injury. Knowledge of the spectrum of abnormalities that involve the mesentery, omentum, and peritoneal cavity and the characteristic CT appearances of these abnormalities is essential for improved diagnosis of these conditions.
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PMID:CT of the mesentery, omentum, and peritoneum in children. 789 16

Six childhood vascular tumors were designated as "malignant endovascular papillary angioendothelioma" by Dabska in 1969. Since then, a few reports of similar cases were published, often called "Dabska tumors." Twelve similar cases were identified in review of vascular tumors from the authors' institutions. There were five men and seven women, including seven adults. Patient ages ranged from 8 to 59 years (mean, 30 years). The tumors occurred in the dermis or subcutis of the buttocks or thigh (n = 6), thumb or hand (n = 3), abdomen (n = 2), and heel (n = 1). The tumor sizes ranged from 1 to more than 40 cm (mean, 7.0 cm). The unifying feature of all cases was distinctive intravascular growth of well-differentiated endothelial cells presenting as a matchstick columnar configuration, sometimes with a large production of matrix that was positive for collagen type IV. In half the cases, these intravascular proliferations had an associated actin-positive pericytic proliferation. There was minimal cytologic atypia and rare to absent mitotic activity. Two cases had an adjacent lymphangioma, and two additional cases had clusters of lymphatic vessels adjacent to the tumor. All but two of the cases showed varying degrees of stromal or intraluminal lymphocytes. Occasional epithelioid endothelial cells were seen, but no cases had features typical of epithelioid, spindle cell, or retiform hemangioendothelioma. Tumor cells were positive for vimentin, von Willebrand factor, CD31, and focally for CD34 and were negative for keratins, epithelial membrane antigen, S-100 protein, and desmin. Vascular endothelial cell growth factor receptor type 3, a recently introduced marker for lymphatic endothelia, was positive in all eight cases that were studied, supporting a lymphatic phenotype. Follow-up in 8 of the 12 cases showed no evidence of recurrences, metastases, or residual disease during follow-ups ranging from 1 to 17 years (mean, 9 years). Based on the proliferative borderline features and the lymphatic phenotype, we propose to designate these tumors as papillary intralymphatic angioendothelioma. Additional cases with extensive follow-up should be studied to rule out variants with malignant potential.
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PMID:Papillary intralymphatic angioendothelioma (PILA): a report of twelve cases of a distinctive vascular tumor with phenotypic features of lymphatic vessels. 1047 59

The purpose of this study was to analyse the sonographic findings of focal splenic lesions with an attempt to differentiate benign lesions from malignancies. The sonographic findings of 53 cases of verified focal splenic lesions, excluding post-traumatic haematomas and phleboliths, were retrospectively analysed. Of the 53 cases, 30 cases (57%) were benign and 23 cases (43%) were malignant. The malignancies included lymphoma (n=13), metastases (n=10); while the benign lesions included cysts (n=17), infarcts (n=3), and abscesses (n=5), and one case each of haemangioma, hamartoma, spontaneous rupture, tuberculosis, and lymphangioma. Significant differences were noted between the benign and malignant groups with respect to the presence of solitary lesions (p<0.0001), anechoic mass (p<0. 0001), lesions with highly echogenic foci due to gas or calcification (p = 0.0303), hyperechoic/mixed echoic lesions (p<0. 0001), presence of extrasplenic abdominal masses (p<0.0001), and nodules with the target sign (p<0.0001). Solitary lesions, anechoic mass, and lesions with highly echogenic foci due to gas or calcification each had a positive predictive value of 85%, 100%, and 100%, respectively, for the lesions to be benign. The multifocal/diffuse lesions, presence of extrasplenic abdominal masses, hyperechoic/mixed echoic lesions, and nodules with the target sign each had a positive predictive value of 70%, 100%, 70%, and 100%, respectively, for the lesions to be malignant. In summary, focal lesions with anechoic pattern or echogenic foci due to gas or calcification are suggestive signs of benign process. The sonographic observations of multifocal or diffuse solid lesions, especially those associated with target sign or extrasplenic abdominal masses are suggestive of malignancy.
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PMID:Ultrasonographic findings and differentiation of benign and malignant focal splenic lesions. 1090 77

Primary angiosarcoma of the spleen is a rare neoplasm that has not been well characterized. We describe the clinical, morphologic, and immunophenotypic findings of 28 cases of primary splenic angiosarcoma, including one case that shares features of lymphangioma/lymphangiosarcoma. The patients included 16 men and 12 women, aged 29 to 85 years, with a mean of 59 years and median of 63 years. The majority of patients (75%) complained of abdominal pain, and 25% presented with splenic rupture. The most common physical finding was splenomegaly (71%). Seventeen of 21 patients were reported to have anemia. Macroscopic examination showed splenomegaly in 85% cases. Sectioning revealed discrete lesions in 88% of cases, ranging from well-circumscribed firm nodules to poorly delineated foci of necrosis and hemorrhage associated with cystic spaces. Microscopically, the tumors were heterogenous; however, all cases demonstrated at least a focal vasoformative component lined by atypical endothelial cells. Solid sarcomatous, papillary, and epithelioid growth patterns were observed. The solid sarcomatous component resembled fibrosarcoma in two cases and malignant fibroushistiocytoma in one case. Hemorrhage, necrosis, hemosiderin, extramedullary hematopoiesis, and intracytoplasmic hyaline globules were frequently identified. A panel of immunohistochemical studies revealed that the majority of tumors were immunoreactive for at least two markers of vascular differentiation (CD34, FVIIIRAg, VEGFR3, and CD31) and at least one marker of histiocytic differentiation (CD68 and/or lysozyme). Metastases developed in 100% of patients during the course of their disease. Twenty-six patients died of disease despite aggressive therapy, whereas only two patients are alive at last follow-up, one with disease at 8 years and the other without disease at 10 years. In conclusion, primary splenic angiosarcoma is an extremely aggressive neoplasm that is almost universally fatal. The majority of splenic angiosarcomas coexpress histiocytic and endothelial markers by immunohistochemical analysis, which suggest that some tumors may originate from splenic lining cells.
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PMID:Splenic angiosarcoma: a clinicopathologic and immunophenotypic study of 28 cases. 1100 38

Although orbital cellulitis is the most common cause of acute-onset proptosis with inflammatory signs in a child, the clinician should always be alert to the possibility of rhabdomyosarcoma. We describe an unusual presentation of acute-onset nonaxial proptosis of the left orbit without sinus disease or systemic toxicity in a 6-year-old boy. Our clinical differential diagnosis included orbital cellulitis, metastatic disease, capillary haemangioma, lymphangioma with cyst, ruptured dermoid cyst, and orbital rhabdomyosarcoma. Only after orbital biopsy and subsequent microbiologic confirmation were obtained was a diagnosis of chronic orbital abscess tenable. Features in our patient included paucity of symptoms and signs of inflammation. This case illustrates the difficulty in differentiating a chronic orbital infection from orbital rhabdomyosarcoma on the basis of clinical, laboratory, and orbital imaging findings. Possible causes of this unusual presentation are discussed.
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PMID:Orbital abscess masquerading as a rhabdomyosarcoma. 1104 Apr 85


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