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PubMed2004

Myxoinflammatory fibroblastic sarcoma with complex supernumerary ring chromosomes composed of chromosome 3 segments.

Myxoinflammatory fibroblastic sarcoma is a rare, recently described, and distinctive low-grade tumor of soft tissue. To our knowledge, there is only one previous report on the cytogenetics of this tumor. That case showed complex structural abnormalities, including a reciprocal translocation between chromosomes 1 and 10 [t(1;10)(p22;q24)] with loss of chromosomes 3 and 13. We describe here a second case showing supernumerary ring chromosomes, and a derivative chromosome 13, with additional material on the short arm. We conclude that the presence of chromosomal abnormalities supports the neoplastic nature of this tumor and aids in its diagnosis. Furthermore, we also postulate that the finding of ring chromosomes, which have been identified in other low-grade soft-tissue tumors, may have important prognostic implications regarding the aggressiveness of this neoplasm.

Chromosome Aberrations↗
PubMed2004

Low-grade myxofibrosarcoma: a clinicopathologic analysis of 49 cases treated at a single institution with simultaneous assessment of the efficacy of 3-tier and 4-tier grading systems.

Grading of myxofibrosarcoma (MFS) is contentious and based on a variety of factors, such as the percentage of myxoid or solid areas, tumor necrosis, mitotic counts, and so on. These factors are often used in combination for different grading schemes, which have not been uniformly evaluated by consistent criteria for patients treated and prospectively followed up at a single institution. Because only a subset of low-grade (LG) MFS will progress to high grade and metastasize after relentless local recurrences, we analyze various histologic parameters and grading methods to identify prognostic predictors of the LGMFS. Forty-nine cases were classified as LGMFS after review, by using > or =30% of myxoid component, but < or =20% of solid areas and only focal, < or =10%, of tumor necrosis as cutoffs, as modified from a 2-tier system that is used in our hospital. These cases were also graded in parallel by French Federation of Cancer Centers Sarcoma Group (FNCLCC) 3-tier and 4-tier grading schemes. The study cohort consisted of 26 men and 23 women, with a median age of 60.5 years. Nineteen cases were superficial, and 30 were deep seated, with the most common site being the lower limb (57%), followed by the upper limb (31%), trunk (8%), and head and neck (4%). The primary tumors ranged from 1.5 to 24 cm in size. Solid areas (5% to 20%) were seen in 23 cases, tumor necrosis (5% to 10%) was observed in 4 cases, and a predominant myxoid area (> or =75%) was noted in 22 cases. Mitotic activity ranged from 0 to 16 (median, 2) per 10 HPF. Comparing FNCLCC 3-tier versus 4-tier grading, respectively, 26 versus 10 tumors were classified as grade I, and 23 versus 39, as grade II, with 16 cases (33%) graded discordantly by 2 schemes. A median follow-up of 55 months in 49 patients (range, 9 to 171 months) revealed local recurrence occurring in 28 patients (57%), 15 and 7 of which developed multiple local recurrences and distant metastases, respectively. There was only 1 case with pulmonary metastasis without a prior local recurrence. Currently, 33 patients are alive with no evidence of disease, 4 are alive with disease, 9 are dead of disease, and 3 are dead of unknown causes. The 5-year recurrence-free survival, metastasis-free survival (MeFS), and disease-specific mortality (DSM) rates were 41%, 90%, and 4.4%, respectively. Size of larger than 5 cm (P = 0.032), tumor necrosis (P = 0.033), and < 75% of myxoid area (P = 0.042) were significant risk factors for DSM; the former two (P = 0.011 for size larger than 5 cm, P = 0.038 for necrosis) were also significantly related to MeFS. Both FNCLCC 3-tier and 4-tier schemes failed to show a significantly better outcome in grade I LGMFS than grade II lesions with respect to all 3 endpoints. In conclusion, our data statistically validated the previous impression that even the blandest LGMFS still carries a recurrent potential that cannot be foreseen by either different grading schemes or other clinicopathologic parameters. However, DSM rate is significantly related to tumor necrosis, large size, and decreased myxoid area. Tumors having necrosis or exceeding 5 cm are at significant risk of metastatic relapse.

Adult↗
PubMed2005

Stacking of a dermal regeneration template for reconstruction of a soft-tissue defect after tumor excision from the palm of the hand: a case report.

Excision of tumors from the hand often leaves tissue defects with exposed tendons or neurovascular structures that require coverage. Various types of free and pedicled grafts have been described for use in these situations. We present a patient who had a tumor excision in the hand followed by wound coverage with a stacked dermal regeneration template. A 50-year-old man presented with a mass over the palm of the hand. He had an incisional biopsy procedure, the results of which suggested malignancy. He then had wide excision with planned temporary skin coverage with a dermal regeneration template. The dermal template incorporated nicely. With adequate skin coverage the palmar defect still was substantial in terms of depth. This was raised with layering or stacking of the template followed by a split-thickness skin graft. Excellent wound healing and coverage of the defect ultimately were achieved. Additionally the patient went on to obtain full range of motion. Stacking of a dermal regeneration template coupled with split-thickness skin grafting was used to fill a soft-tissue defect over the median and nerve and flexor tendons after wide tumor excision.

Diagnosis, Differential↗
PubMed2003

Inflammatory myofibroblastic tumor of the left ventricle in an older adult.

We report an unusual study of a 72-year-old woman who presented with a symptomatic space-occupying lesion in the left ventricle. The lesion was surgically excised with a good long-term result. It was histologicaly diagnosed as an inflammatory myofibroblastic tumor. Also known as an inflammatory pseudotumor, these tumors affecting the heart are extremely rare and occur mainly in children and adolescents. We review the literature and highlight the unique nature of the lesion.

Aged↗
PubMed1983

Primary pulmonary neoplasms of childhood: a review.

Primary tumors of the lung rarely occur in children. However, 230 well-documented cases, including the 2 presented in this review, have been identified in the English-language literature. One hundred fifty-one tumors in these reports were classified as malignant lesions and 79 as benign neoplasms. Bronchial "adenomas" constituted the largest group; most of these lesions were of the carcinoid variety, and 8% were definitely malignant. Forty-seven cases of bronchogenic carcinoma were reported in children under 16 years of age, although there were few squamous cell tumors (12%). Fifty-six percent of the benign tumors were classified as inflammatory pseudotumor. Most of the children in this collective series were seen with symptoms related to bronchial irritation or obstruction, such as cough, hemoptysis, atelectasis, or pneumonitis. Respiratory distress was an unusual symptom that was often associated with large tumors seen in the neonatal period. Approximately 20% of the children were totally symptomatic. The limited survival data that are available indicate that leiomyosarcoma and mucoepidermoid carcinoma have a more favorable prognosis in children than in adults. Survival with bronchial carcinoid tumors (90%), bronchogenic carcinoma (30%), and pulmonary blastoma (45%) appears to parallel that for adults. The experience with pulmonary rhabdomyosarcoma, as described in this review, emphasizes the importance of early diagnosis and the use of combined modes of therapy in the approach to these malignancies. Despite the rarity of primary pulmonary neoplasms in children, this diagnosis should be considered in young patients with solitary pulmonary masses or persistent, atypical pulmonary symptoms. It is hoped that early diagnosis will result in an improved prognosis and prevent life-threatening complications.

Adenoma↗
PubMed1987

Malignant clinical behavior of cardiac myxomas and "myxoid imitators".

The clinical experience with cardiac myxomas and "myxoid imitators" at the Humana Heart Institute International is reviewed and contrasted with a comprehensive review of the world literature. Twenty-two patients with primary cardiac tumors have been treated at our institution. Three (13.6%) of them had myxoid imitators, and 19 had classic myxomas. One of the 19 patients with a classic myxoma had recurrence on two occasions (recurrence rate, 5.3%). The cases of 57 patients from the world literature are reviewed. Recurrence and/or metastases (including formation of mycotic aneurysm) occurred in approximately 50% of these patients. The overall mortality in these patients was 47%. This review provides documentation of the malignant clinical behavior of cardiac myxomas and myxoid imitators. Neither local recurrence nor regrowth of the tumor in a new location, whether intracardiac or extracardiac, can be predicted from the microscopic appearance of the primary tumor. Resection of the myxoma with a wide margin is the recommended surgical procedure but does not ensure a cure. Therefore, long-term follow-up is mandatory.

Fibroma↗
PubMed1985

Primary cardiac tumors: experience with 30 consecutive patients since the introduction of two-dimensional echocardiography.

Experience with 30 consecutive patients who had a total of 32 primary cardiac tumors and who underwent two-dimensional echocardiographic examinations between January 1977 and June 1983 was reviewed. Most of the tumors were atrial myxomas (20 left and 4 right), and 30 were identified on echocardiography. Twenty-five patients, including 21 of 22 with atrial myxoma, underwent surgical resection on the basis of the echocardiographic examination, without preoperative angiocardiography. When the morphologic characteristics of the left atrial myxomas were studied statistically in relation to clinical abnormalities, large tumor size was most closely related to the number and type of associated clinical and laboratory abnormalities. The single exception was embolization, which correlated with echocardiographic tumor consistency. Since the introduction of two-dimensional echocardiography, the yearly incidence of cardiac tumor diagnosis at this clinic has increased several fold and the incidence of unexpected intraoperative diagnosis has been very low (one case). Echocardiography is the method of choice for clinical diagnosis. It has replaced angiocardiography for routine preoperative assessment, permits early diagnosis of cardiac neoplasms and provides insight into the pathophysiology of primary cardiac tumors.

Adolescent↗
PubMed2003

Acral myxoinflammatory fibroblastic sarcoma in FNAB samples: can we distinguish it from other myxoid lesions?

We analysed cytomorphological characteristics of three fine needle aspiration biopsy (FNAB) samples of acral myxoinflammatory fibroblastic sarcoma (AMIFS) as well as the features of a number of other benign and malignant myxoid lesions. The analysis showed that FNAB samples from two cases of AMIFS had similar cytomorphology, containing all the characteristic features described in surgical biopsies: myxoid material, spindle cells with bipolar cytoplasmic extensions, epithelioid cells with globules of extra-cellular material, ganglion-like and lipoblast-like giant cells. Only the inflammatory component was scarce. The third sample did not contain features typical of AMIFS. Samples from other myxoid tumours resembled AMIFS to some extent, however, none of them contained all three tumour components characteristic of AMIFS. Cytomorphology of AMIFS may be characteristic enough to enable a definitive diagnosis from FNAB, provided all the distinctive features are sampled.

Adult↗
PubMed1999

Malignant myxoid endobronchial tumour: a report of two cases with a unique histological pattern.

AIMS: To present two cases of malignant endobronchial myxoid tumours with a highly distinctive sarcomatoid pattern not previously described at this site, and discuss their histogenesis in relation to previously documented endobronchial neoplasms. METHODS AND RESULTS: Both tumours presented in young adult females and were purely sarcomatoid with interweaving cords of small uniform, rounded or slightly elongated cells lying within a myxoid stroma. The stroma was alcian blue positive, but sensitive to hyaluronidase in both cases. The tumour cells contained a small volume of periodic acid-Schiff-positive eosinophilic cytoplasm and stained positively for vimentin only, but there also was a prominent background population of CD68-positive dendritic cells. Ultrastructural studies showed that the tumour cells contained an excess of rough endoplasmic reticulum, with some of the cisternae appearing dilated, and scalloping of the cell surfaces, although no intracisternal tubules were identified. CONCLUSIONS: Although the histological pattern was most reminiscent of extraskeletal myxoid chondrosarcoma, the sensitivity of the stroma to pretreatment with hyaluronidase precluded the diagnosis. However, there were similarities with the sarcomatoid component of malignant salivary gland-type mixed tumours of the lung and this tumour possibly represents a variant of a bronchial gland tumour. Despite this uncertainty over origin, this pattern should be recognized as part of the differential diagnosis of myxoid tumours in the lung, as an apparently indolent type of malignant endobronchial neoplasm.

Adult↗
PubMed2003

Progression of a myxoid pleomorphic fibroma to myxofibrosarcoma.

A 75-year-old man presented with a protuberant stable lesion of many years on his left second toe. Histology on an incisional biopsy showed myxoid pleomorphic fibroma. Over the subsequent 21 months, the lesion enlarged and became exophytic and irregular. Repeat biopsy showed features consistent with a grade III myxofibrosarcoma. Treatment involved amputation of his toe. Six months later he remains well, with no evidence of local recurrence or systemic spread.

Aged↗
PubMed2001

Low-grade pulmonary myxoid sarcoma of uncertain histogenesis.

The present report describes an unusual case of low-grade pulmonary sarcoma with extensive myxoid change in a 60-year-old man. During 30 months, the tumor enlarged gradually and thereafter rapidly to 9 cm. Preoperative biopsy and cytology gave negative results. The resected mass, located at the periphery of the right upper lobe, was well circumscribed and showed gelatinous without necrosis or hemorrhage. Histologically, the tumor was composed of slightly atypical, spindle-shaped or stellate cells, which were loosely distributed within a prominent myxoid stroma. Epithelial differentiation was not seen. Lacunar structures were occasionally evident, but no cartilaginous matrix was seen. Mitotic figures were infrequent. Immunohistochemistry failed to clarify the nature of the neoplastic cells except vimentin positivity. Histochemically, the myxoid ground substance was composed of hyaluronic acid and acid mucopolysaccharide. Electron microscopy revealed no specific differentiation other than aggregates of filaments, which were seen in a number of neoplastic cells. Flow cytometric analysis of the neoplastic cells revealed a diploid pattern. These findings indicated that the neoplasm was a low-grade myxoid sarcoma; however, a definite diagnosis could not be made. The tumor might have been a variant of extraskeletal myxoid chondrosarcoma, especially considering the histochemical results.

Biomarkers, Tumor↗
PubMed1979

[Active and passive vascular involvement in tumours and tumour-like processes in the extremities (author's transl)].

Angiography for tumours of the extremities may demonstrate active or passive involvement of vessels by the tumour. This technique has been used for a considerable time for the diagnosis and differentiation of bone tumours. Angiography is particularly suitable for distinguishing between benign and malignant tumours of soft tissues. Non-neoplastic space-occupying lesions which can be elucidated by angiography include aneurysms, haematomas, inflammatory processes and abscesses.

Angiography↗
PubMed1988

Primary cardiac tumors--clinical experiences and late results in 48 patients.

Between 1971 and 1987 48 patients (35 female and 13 male) underwent operations at the Department of Cardiovascular Surgery, Justus-Liebig-University Giessen, for primary cardiac tumors. The age of the patients ranged from 21 to 71 years. Clinical symptoms were congestive heart failure, cardiac murmurs and findings similar to mitral stenosis, chest pain, arrhythmias, arterial embolism, myocardial infarction and pericarditis. 39 of the tumors were benign and 9 were malignant. Diagnosis was confirmed by echocardiography and/or cineangiocardiography. The left atrium was most commonly involved, followed by the right atrium. Surgery was performed in all cases using cardiopulmonary bypass. Benign tumors were totally removed, including attachment to the atrial septum or the free wall. In one case the tumor resection was carried out by autotransplantation of the heart. During the perioperative period we lost one of the 39 patients with benign tumors due to low cardiac output. Observing a mean follow up period of 48 months, no recurrence of tumors was noted and all patients are doing well now. The malignant neoplasms could not be removed completely, and in 4 cases only a reduction of the tumor mass was possible. Three of the 9 patients died during the postoperative period. Only one patient survived longer than 48 months. Whereas surgical therapy of malignant cardiac tumors is still a matter of discussion based upon these discouraging results, benign or semimalignant cardiac neoplasms have a very favourable prognosis when the surgical intervention can be performed prior to the occurrence of severe complications, such as congestive heart failure and arterial embolism.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗
PubMedDate not supplied

[Maffucci's syndrome (dyschondroplasia combined with hemangioma)].

Dyschondroplasia as congenital, non inherited mesodermal dysplasia is known as most seldom disease in the classical sense with hemangioma as Maffuccis Syndrome in literature. Two cases not previously described are presented and discussed with reference to world literature. In the foreground we see distortion of the chondroma deformity, less often is the development of malignity as a result of the hemangioma.

Adolescent↗