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Query: UMLS:C0027627 (metastases)
 103,950 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A retrospective study of 57 patients with atypical fibroxanthoma of the skin is presented. The light microscopy is described and the differential diagnosis is discussed. Most of the atypical fibroxanthomas (32 out of 57 cases) were originally diagnosed as soft tissue sarcomas, e.g. fibrosarcoma, dermatofibrosarcoma, neurofibrosarcoma, myosarcoma and unspecified sarcoma. The tumour occurred chiefly in middle-aged and elderly patients; three patients were 15 years old or younger. The median age was 73 years in patients in whom the tumour occurred in the head and neck, and 34 years in patients in whom the tumour developed on the extremities and trunk. The sex ratio (male to female) was almost equal. Follow-up information about 43 patients was available. The follow-up period ranged from 1 year to 25 years with a median of 9 years. Eight patients died from intercurrent disease; all the other 35 patients are alive and well. The clinical course was benign in all but one patient in whom a recurrence developed and metastases to the regional lymph nodes appeared 7 years after the initial excision. The tumour in this case did not differ histologically from the other atypical fibroxanthomas with respect to cellularity, cellular and nuclear atypia or mitotic activity. It is suggested that the recurrence per se might be of prognostic importance.
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PMID:Atypical fibroxanthoma of the skin. A clinico-pathological study of 57 cases. 125 44

In the absence of systematic immunohistochemistry investigations, only 25 cases (out of 69) clearly diagnosed as neurosarcoma due to the association with Von Recklinghausen disease (ie, neurofibromatosis type 1) and treated at the Institut Gustave Roussy were included in the present study. Neurosarcoma consists of a neurocristopathy whose cells migrate to several parts of the body in order to constitute neuroglia, Schwann cells, pigmented and endocrinal tissues. From 1967 to 1990, 25 cases of such neurosarcomas associated with a neurofibromatosis type 1 were seen at our institute. Three different histological terms exist for this tumour: malignant schwannoma, neurosarcoma, and more recently, malignant peripheral nerve sheath tumours (MPNST). The median age (23 years) of the patients with neurofibromatosis type 1 is lower than that of patient with isolated neurosarcoma. Their sex ratio is 2/1. Primary tumour surgical exeresis was performed in all cases, with poor results in 7. Post-operative radiation therapy was not systematically used in this series. It was administered only in cases with incomplete surgical exeresis or when a local recurrence occurred. Adjuvant CYVADIC (doxorubicin, procarbazine, cyclophosphamide, vincristine) chemotherapy was administered in 5 cases, of in cases of relapse (8). All cases but 2 (the most recent ones) relapsed within 1 to 226 months (median 7 months). In addition, 13 patients developed metastases. Overall, the 2-year and 4-year survival rates were 41% and 18%, respectively. Our observations confirm the very poor prognosis for the association of neurofibromatosis type 1 and neurosarcoma. This finding should lead to systematically associate radical surgical exeresis, post-operative irradiation and adjuvant chemotherapy in the treatment of these patients.
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PMID:[Neurosarcoma associated with Von Recklinghausen disease: apropos of 25 cases observed at the Gustave Roussy Institute from 1967 to 1990]. 152 Sep 49

A retrospective review of all malignant hand tumors seen at the University of Michigan from 1950 to 1987 demonstrated six biopsy-proven cases of neurofibrosarcoma involving the upper extremity. There were four male and two female patients; average age at presentation was 37 years and ages ranged from 15 to 63 years. All patients had a history of von Recklinghausen's disease. Three patients were seen initially with painful enlarging masses, one with a painful mass with a neurological deficit, and two with painless enlarging masses. Three patients had radical excisions requiring upper extremity or forequarter amputation, and three patients received limited or no surgical treatment. Five patients died of metastases an average of 3 years after diagnosis. One patient remains alive 18 years after upper extremity amputation despite an early local recurrence. Early diagnosis and radical surgical excision offer the best chance of long-term survival from this highly lethal cancer.
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PMID:Neurofibrosarcomas of the upper extremity. 194 Jan 67

Ninety-two cases of intrathoracic neurogenic tumors operated on between 1950 and 1982 are reviewed. The benign forms (86 cases, 93.5%) included 23 ganglioneuromas, 50 neurilemmomas and 13 neurofibromas. Of the latter, 4 occurred in patients with Von Recklinghausen's disease. A double local recurrence was observed after the removal of a neurilemmoma. One of the patients with generalized neurofibromatosis died 5 months after operation from local sarcomatous degeneration and distant metastases. In this group of benign lesions, no other death was observed which could be attributed to the endothoracic neural tumor. The malignant forms (6 cases, 6.5%) included 4 ganglioneuroblastomas and 2 neurofibrosarcomas. The surgical excisions were described as radical in every case and all the patients were given radiotherapy postoperatively. One patient with ganglioneuroblastoma died from metastases 2 years later, and one with neurofibrosarcoma from local recurrences 7 months later. One patient with neurofibrosarcoma and 3 with ganglioneuroblastomas are alive and well 4, 5, 6 and 11 years, respectively, later. Surgical excision remains the best method of diagnosing and treating endothoracic tumors of neural origin. Of special interest are the "dumbbell" or hourglass tumors and lesions which occur in Von Recklinghausen's disease.
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PMID:Neurogenic intrathoracic tumors. A clinicopathological review of 92 cases. 242 96

Between 1979 and 1985, seven patients (five children and two adults) were treated for primary cardiac tumours other than benign atrial myxomas. There were five malignant neoplasms (two non-classifiable sarcomas, one haemangiosarcoma, one histiocytoma and one neurofibrosarcoma) and two benign tumours (fibromas). Echocardiography, cardiac catheterisation, computed tomography and magnetic resonance imaging provided diagnostic confirmation. The two patients with fibroma are alive and well 4 and 5 years after radical resection of the tumours from the interventricular septum. The patient with a neurofibrosarcoma underwent orthotopic cardiac transplantation and is well 5.5 years postoperatively with no evidence of residual disease or recurrence. One patient died awaiting a donor heart for transplantation. Another patient who was a candidate for heart and lung transplantation was found to have an unresectable tumour at the time of operation. One patient with sarcoma who underwent a successful emergency partial resection for relief of cardiac tamponade died 18 months later from widespread metastases. The seventh patient was inoperable due to multiple secondaries. It is concluded that radical resection of large, benign, cardiac tumours can give good results and that early cardiac transplantation probably offers the only hope for patients with malignant tumours of the heart.
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PMID:Primary cardiac tumours--is there a place for cardiac transplantation? 263 39

From 1964 to 1978, 313 patients, 192 males and 121 females, with soft tissue sarcoma underwent surgery in our hospital. All patients have been followed up for over 5 years. One hundred ninety patients (60.7%) had recurrence of the tumor after previous surgery. The tumor was located in the head in 55 cases, in the trunk in 136, and in the extremities in 122 cases. Regional lymph node metastases occurred in 23 patients (7.4%). Fibrosarcoma, neurofibrosarcoma, and rhabdomyosarcoma were the types most commonly encountered in this series. The 5-year survival rate, according to the extent of surgical resection for 117 patients with local resection, was 47.9%; for 75 patients with wide local resection, 62.7%; for 43 patients with amputation, 18.6%; and for 78 patients with local resection combined with irradiation, 48.7%. For the whole series, the 5- and 10-year survival rates were 47.6% and 35.8%, respectively. The 5-year survival rates of stages I, II, III, and IV (TNM classification) were 60%, 46.9%, 40%, and 22.2%, respectively.
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PMID:Surgical management of soft tissue sarcomas, with an analysis of 313 cases. 335 24

An autopsy case of von Recklinghausen's disease (vRD) associated with malignant pheochromocytoma is reported. The patient is a 36-year-old Japanese male and diagnosed as vRD both clinically and pathologically. He died from right adrenal tumor with wide spread metastases to lungs and bone marrow. The tumors presented satisfactory histological features in favor of pheochromocytoma and neurosecretory granules were demonstrated in both primary and metastatic lesions ultrastructurally. Statistical study of 182, 673 autopsy cases from Annuals of Japanese Autopsy Cases was also done in order to investigate the relationship between vRD and associating tumors including benign and malignant pheochromocytoma. Cases with vRD showed significantly higher incidences of malignant Schwannoma, neurofibrosarcoma, intracranial glioma, and pheochromocytoma compared to that of non-vRD cases. Other malignancies revealed rather smaller incidences than non-vRD cases. These neurogenic tumors are to be principal life threatening problems in patients with vRD. Rare incidence of malignant pheochromocytoma in vRD is to become from low incidence of pheochromocytoma, though significantly greater than that of non-vRD cases.
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PMID:Von Recklinghausen's disease (neurofibromatosis) associated with malignant pheochromocytoma. 643 30

Case histories of 10 patients with neurofibrosarcoma, including 14 (70%) with neurofibromatosis, evaluated over 10 years were reviewed to determine the incidence of local and systemic recurrence and the most effective means of therapy for this rare neoplasm. Initial therapy resulted in complete local disease control in only 11 (55%) patients. Local excision, or local excision plus radiation or chemotherapy resulted in local recurrence in 8 of 12 patients. Radical surgery alone, or radical surgery combined with radiation and chemotherapy resulted in local recurrence in 1 of 6. Even with complete local disease control, 7 of 16 (44%) patients died of metastases. Both A.J.C. Clinical Stage II and III patients had a similar poor prognosis. Associated neurofibromatosis did not worsen prognosis. These data suggest that both moderate and high-grade primary neurofibrosarcoma are highly malignant neoplasms and should be treated by radical resection. Preoperative intraarterial Adriamycin and radiation--found to be successful for other highly malignant sarcomas--may be of benefit. Since distant disease occurs despite local control, postoperative adjuvant chemotherapy trials are warranted.
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PMID:Neurofibrosarcoma. 676 81

A neurofibroma, a fibroma, a primary neurofibrosarcoma, and four neurofibrosarcoma metastases from a woman with hereditary neurofibromatosis who was heterozygous (GdB/GdA-) for the X-linked enzyme glucose-6-phosphate dehydrogenase were studied to determine the number of cells from which the tumors developed. Both enzyme types were observed in the benign tumors in proportions similar to those present in seven different normal tissues studied. These findings indicated that the benign tumors arose from many cells. In marked contrast, only type A activity was detected in the primary neurofibrosarcoma and in all of the metastases. Two or more steps probably were involved in the development of neurofibrosarcoma in this patient: the inherited genetic mutation producing neurofibromatosis and a rare event or combination of events that permitted a single cell to undergo malignant proliferation.
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PMID:Probable clonal origin of neurofibrosarcoma in a patient with hereditary neurofibromatosis. 681 62

A known risk of radiation therapy is the induction of secondary neoplasms, most commonly osteosarcoma and fibrosarcoma. A recent addition to the list of postirradiation neoplasms is neurofibrosarcoma, a Schwann cell or fibroblastic malignancy arising in peripheral nerves, often associated with von Recklinghausen's disease. In a clinicopathologic review of 109 patients with neurofibrosarcoma seen at the Mayo Clinic from 1912 to 1981, the tumors in 12 cases were found to originate in areas that had previously been irradiated for benign or malignant disease. Seven of the 12 patients demonstrated stigmata of von Recklinghausen's disease. The mean latency period between irradiation and clinical presentation of the sarcoma was 15.6 years (range, 5-26 years). Eight patients experienced at least one recurrence; metastases were present in two, and nine patients died of their disease. The mean interval between initial diagnosis and death was 3.4 years. In summary, neurofibrosarcoma may arise secondary to radiation; animal studies on the effects of radiation on peripheral nerves support this concept. It is the authors' opinion that patients with von Recklinghausen's disease should not be unnecessarily irradiated, and that those who do receive radiation therapy should be carefully observed for the development of secondary neurofibrosarcoma.
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PMID:Postirradiation neurofibrosarcoma. 682 67


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