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)

Leiomyosarcoma of the inferior vena cava (IVC) is a rare malignant tumor originating in the smooth muscle of the media. Although rare, it is the most common malignancy in the IVC. One hundred and six cases have been reported thus far in the world literature, usually as isolated case reports. Clinical, radiologic, and therapeutic management and follow-up, including 7 additional cases, have been reviewed and summarized. Clinical manifestations are dependent upon the location of the tumor. The main symptom was a palpable mass for a tumor in segment I, abdominal pain for segment II, the presence of Budd-Chiari syndrome for segment III. Segment II was the most frequent site of leiomyosarcoma of the IVC, alone (n = 41) or with other segments (n = 39). Before laparotomy, clinical recognition was difficult or impossible. Recently, however, newer imaging modalities including ultrasound and CT scan have permitted earlier diagnosis. Metastases, when diagnosed, were either present at diagnosis (n = 20) or appeared as the disease progressed (n = 18). Metastatic disease frequently involved the liver, lung, lymph nodes, or bone. The small number of patients alive without metastases (16/113) must be analyzed all the more carefully because these patients were followed for less than 2 years. When prolonged follow-up is possible, the number of patients alive without neoplastic disease is significantly reduced. We found the prognosis of patients with LMS of the IVC to be poor. Diagnosis was made at autopsy for 27 patients. Among the 86 patients with follow-up information, 59 died within a mean of 16 months, and 26 were alive 25 months after the diagnosis. The main prognostic factor is topography, particularly the highest level of extension of the tumor. The upper-segment tumors have the poorest prognosis. The best therapeutic management is difficult to recommend because most of the cases in the literature did not include a sufficient follow-up. Given the very small number of patients completely free of neoplastic disease after sufficient follow-up, it seems unlikely that leiomyosarcoma of the IVC can now be cured. Patients who received a combination of surgery, radiotherapy and chemotherapy remained free of disease for longer periods. The unanswered question is: what is the best timing for each of these treatments? We recommend diagnosis of leiomyosarcoma of the IVC through biopsy guided by ultrasonography or computed tomographic scan. Therapeutic management should include large doses of chemotherapy preoperatively with or without radiotherapy to reduce tumor size.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Leiomyosarcoma of the inferior vena cava. Experience with 7 patients and literature review. 192 4

Two cases of leiomyosarcoma of retrohepatic inferior vena cava are reported. The two patients are women and are currently alive with metastases one, seven years, and the other one year postoperatively. Leiomyosarcoma of inferior vena cava s a rare tumor which has an unexplained predilection for women. Clinical symptoms are non specific and depend mostly on the segment of the vessel involved. Preoperative diagnosis is based on ultrasonography, computed tomography and cavography. Although the prognosis is poor due to local recurrence and metastasis, we recommend an aggressive approach by radical tumor resection and caval reconstruction.
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PMID:[Leiomyosarcoma of the inferior vena cava: literature review and surgical treatment. Apropos of 2 retrohepatic localizations]. 206 76

A teenage girl is described who had an osteogenic sarcoma of the tibia at 11 years of age, a pulmonary metastasis at 13 years, and an intestinal leiomyosarcoma at 15 years. She remains well at the age of 18 years. Leiomyosarcoma is extremely rare in children either as a primary or secondary tumor.
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PMID:Intestinal leiomyosarcoma following treatment of osteosarcoma in a teenage girl. 264 4

Leiomyosarcoma has been rarely encountered in the orbit. Leiomyosarcoma may arise as a primary tumor in the orbit, metastasize from distant sites to the orbit, extend into the orbit from the paranasal sinuses, or appear after radiation therapy to the orbit. The 43-year-old female patient described in this paper underwent excision of a leiomyosarcoma in the subcutaneous tissue of the buttock. Seven years later, a leiomyosarcoma in the medial orbit was removed. The clinical course of this patient's disease was similar to that of other subcutaneous leiomyosarcomas documented in the literature. Gynecological history was remarkable for a leiomyoma of the uterus, an interesting association found in at least one other case in the literature. This was the third documented case of metastatic leiomyosarcoma in the orbit.
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PMID:Leiomyosarcoma of the orbit. A case report and review of the literature. 315 73

Leiomyosarcoma (LMS) of dermal and subcutaneous tissues is an uncommon neoplasm. In order to analyze the specialized pathologic features of this tumor, we undertook a histological, ultrastructural, and immunohistochemical study of 9 superficial LMS, including 7 dermal lesions and 2 subcutaneous neoplasms. These were compared with 12 examples of "deep" extracutaneous LMS. Metastases to the skin from two of the latter neoplasms were also examined. Immunohistochemistry was found to be a useful diagnostic adjunct to light microscopic and ultrastructural studies in that all LMS coexpressed vimentin and desmin, regardless of site, and 90% also expressed muscle-specific actin. Variable expression of cathepsin B and myelin basic protein was noted in 8 and 10 tumors, respectively, whereas none contained cytokeratin. Weak cytoplasmic positivity for epithelial membrane antigen was seen in 1 dermal and 3 extracutaneous LMS. Of 7 dermal LMS, 4 contained S-100 protein, whereas this determinant was found in only 1 of 12 extracutaneous tumors. Conversely, Leu 7 reactivity was present in 7 of 12 extracutaneous LMS, but only 2 of 9 superficial lesions. Review of clinical features confirmed that subcutaneous LMS is capable of aggressive behaviour, whereas dermal LMS was more likely to behave in an indolent fashion. However, one example of dermal LMS exhibited aggressive local recurrences and distant metastasis, ultimately leading to the death of the patient. Therefore, careful clinical followup is indicated in all cases.
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PMID:Primary cutaneous leiomyosarcoma. A histological and immunohistochemical study of 9 cases, with ultrastructural correlation. 329 54

Leiomyosarcoma of the oral region has rarely been reported. A review of the literature yield only 24 cases. The histopathology with special reference to ultrastructural findings and immunohistochemical examination of an additional case of leiomyosarcoma of mandibular gingiva has been presented. The manifestation and symptoms, treatment and prognosis, and the origin of this tumor are discussed. Because of the high rate of local recurrence and metastases, extensive surgical excision and long-term follow-up are mandatory.
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PMID:Leiomyosarcoma of gingiva: report of a case and review of the literature. 330 28

Leiomyosarcoma originating in the anal canal internal sphincter is an extremely rare malignant tumor of often late diagnosis, since usually asymptomatic, and with malignancy criteria that are sometimes difficult to define. These tumors affect men and women with equal frequency, usually in the 6 th decade. Two cases are reported in patients aged 67 and 65 respectively in whom the tumor was revealed by a painful perianal mass shown on rectoscopy to be due to a submucous tumor projecting into anal canal, Prognosis and treatment of these tumors is discussed, diagnosis being confirmed only after excision biopsy. Tumoral extension was both local and regional by continuity but blood dissemination had occurred with metastases more frequently in liver (1 case) but also in lungs. Glandular extension was exceptional. Limited local exeresis of small tumors appears justified if sufficiently wide, since malignancy of leiomyosarcoma appears to remain circumscribed over long periods. However, this limited procedure runs the risk of local recurrence with the need for abdominoperineal amputation (the case in the 2 patients reported), although long-term results appear to be similar whether excision is or is not extensive, the abdominoperineal amputation failing to avoid the unfavorable course. Complementary radiotherapy or chemotherapy is ineffective, and prognosis is related more to the degree of tumoral differentiation than to the operative treatment itself.
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PMID:[Leiomyosarcoma of the anal canal]. 339 31

Leiomyosarcoma of the hand is a rare tumor. A case report of a leiomyosarcoma lying in the superficial tissues of the dorsal aspect of the right hand in a 64-year-old woman is given. Nine months after initial excision a local recurrence appeared and was treated by radical local excision and free skin grafting. The follow-up is now seven years. The patient is free of tumor and metastases. A review of the literature is given. The problems in therapy of leiomyosarcoma of skin and superficial tissues are discussed.
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PMID:[Subcutaneous leiomyosarcoma of the hand]. 380 45

Leiomyosarcoma of the inferior vena cava in a rare tumor which is predominantly seen in women. The symptomatology is non specific and depends mostly of the segment of the vessel which is involved. Radiologic approach for correct preoperative diagnosis is based on ultrasonography, computed tomography and inferior vena cavography. The best treatment is en bloc resection of the mass associated with pre and post-operative chemotherapy. Although the tumor is slow-growing, the prognosis is poor and the average survival is less than two years, due to local recurrence and metastases.
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PMID:[Leiomyosarcomas of the inferior vena cava]. 380 78

Leiomyosarcoma of the inferior vena cava is reported with increasing frequency but remains a rare lesion. In this article we report one case and review 69 cases. The disease affects mainly women approximately 50 years of age. The symptoms vary according to the location of the tumor; Budd-Chiari syndrome and leg edema occur in the more centrally located tumors and abdominal pain and swelling are seen in the more peripherally located ones. In 40% of the cases distant metastases are evident at the time of the patients' first presentation and are found most commonly in the liver and/or lungs. After resection the majority of the patients eventually die of metastatic disease with a mean survival of approximately 40 months.
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PMID:Leiomyosarcoma of the inferior vena cava. Case report and review of the literature. 395 63


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