Metastasizing "benign" cutaneous fibrous histiocytoma: a clinicopathologic analysis of 16 cases.

Cutaneous fibrous histiocytoma (FH) is considered a benign tumor; however, certain types of FH have been shown to have a tendency for local recurrence, and there are rare reported cases of metastasis. In this study, 16 cases of morphologically benign FH with locoregional or distant metastasis were identified in consult files. Pathologic features of primary, recurrent, and metastatic tumors, as well as clinical outcome, were evaluated. Nine were male and 7 were female patients; mean age was 42 years (range, 3 to 68 y). Primary tumors arose on the leg in 5 patients, buttock in 1, trunk in 3, shoulder in 3, neck in 2, and finger in 1. The primary site in 1 case was unknown. Fifteen primary tumors available for review involved the dermis; 6 extended into the superficial subcutis. Tumor size ranged from 1 to 5 cm (median 3.2 cm). Histologically, primary tumors showed characteristic features of FH, being composed in most cases of a polymorphous population of bland spindle and histiocytoid cells in a mixed storiform and fascicular growth pattern with admixed foam cells, multinucleate cells, and inflammatory cells in varying proportions. Histologic variants included 11 cellular (2 with mixed atypical and cellular features), 2 aneurysmal, 1 atypical, and 1 epithelioid type. All tumors showed entrapment of hyalinized collagen bundles. Mitotic activity ranged from <1 to 13/10 HPF. Focal necrosis was seen in 1 primary tumor. Ten patients had local tumor recurrence; 4 patients had multiple local recurrences. Time to first recurrence ranged from 6 weeks to 13 years. The local recurrences of 1 tumor showed increased cytologic atypia, but recurrences were otherwise morphologically similar to primary tumors. Metastases occurred 0 to 180 months after diagnosis (median 17 mo) and involved the lungs (12 patients), lymph nodes (8), soft tissues (6), and liver (1). Five patients developed multiple satellite nodules in the region of the primary tumor. Metastases were morphologically similar to the primary tumors. So far, 6 patients died of disease, with a median time to death of 64 months (range, 10 to 168 mo). Four patients are alive with metastatic disease. Two patients are disease free at last follow-up, and 1 patient died of unrelated disease. Metastasis of morphologically benign cutaneous FH is an extremely rare but clinically aggressive event. Primary tumors tend to be large and cellular, but aggressive behavior cannot be predicted on morphologic grounds alone; however, early or frequent local recurrence may warrant closer clinical follow-up.

Does delayed reexcision affect outcome after unplanned excision for soft tissue sarcoma?

BACKGROUND: Unplanned excision of a soft tissue sarcoma generally requires reexcision to achieve an adequate surgical margin. Many surgeons assume delay of definitive surgery adversely affects patient survival and local recurrence. However, no clear evidence of this assumption can be found in the literature. QUESTIONS/PURPOSES: We asked whether delay in reexcision affects patient survival and local recurrence in reexcision after unplanned excision for soft tissue sarcoma. PATIENTS AND METHODS: We retrospectively reviewed 104 patients who underwent definitive surgery after unplanned excision of a localized soft tissue sarcoma. The average age of the patients was 44 years (range, 5-81 years). The most common diagnoses were malignant fibrous histiocytoma (36) and synovial sarcoma (22). Locations of the tumors were the lower extremity (62), upper extremity (32), and trunk (10). The median interval to definitive surgery was 32 days (interquartile range, 22-50 days). The minimum followup was 0.2 years (median, 4.7 years; range, 0.2-16.7 years). RESULTS: The 5-year disease-specific survival was 88% and 5-year local control rate was 74%. We found no difference in disease-specific survival or local recurrence according to the time until definitive surgery. Higher histologic grade and larger tumor size independently predicted disease-specific survival whereas a positive margin at reexcision and larger tumor size independently predicted local control. CONCLUSIONS: The data suggest any influence of delayed definitive surgery is likely to be of minor clinical importance. LEVEL OF EVIDENCE: Level III, therapeutic study. See the Guidelines for Authors for a complete description of levels of evidence.

Why do patients with low-grade soft tissue sarcoma die?

INTRODUCTION: The patterns of failure and mechanisms of sarcoma-specific death are poorly characterized among the minority of patients with low-grade soft tissue sarcoma (STS) who succumb to disease. METHODS: Between 1982 and 2006, 2,041 patients aged >or=16 years with low-grade STS of all sites were treated with curative intent and prospectively followed at a single institution. RESULTS: Among this cohort, 181 (9%) died from disease (DOD). Overall, 105 patients (58%) died from locally recurrent disease (DOLR), and 59 (32%) died from distant disease (DODR). In 17 patients (9%), the mechanism of sarcoma-related death could not be verified. DOD occurred at a median of 62 months, while median disease-specific survival for the entire cohort was not reached. Median follow-up was 66 months (range 2-431 months). On multivariate analysis, DOD was associated with site, size, and less than R0 resection. For DOLR, site, size, positive margins, liposarcoma histology, and local recurrence (by definition) were significant factors. For DODR, site, histology, and positive margins were not significant factors, while size and local recurrence were. Of DOLR, 80% were retroperitoneal, 68% were liposarcoma, and only 2% were extremity. Conversely, of DODR, extremity (47%) and trunk (18%) were the most common sites, but histology was more variable [liposarcoma 35%, malignant fibrous histiocytoma (MFH) 20%, fibrosarcoma 12%, extraskeletal myxoid chondrosarcoma 10%]. High-grade recurrence rates were comparable between DOLR (27%) and DODR (25%). CONCLUSION: Among patients with low-grade STS, DOD occurs in approximately 9% of patients. Nonextremity site, larger size, and less than R0 resection are the most important risk factors for DOD, and distinct patterns of recurrence and death are predicted by primary tumor site.

Salvage surgical resection after high-dose ifosfamide (HDIF) based regimens in advanced soft tissue sarcoma (ASTS): a potential positive selection bias--a study of the Spanish group for research on sarcomas (GEIS).

PURPOSE: To assess the impact of different factors on response rate (RR), time to tumor progression (TTP), and overall survival time (OS) in patients with locally advanced or metastatic soft tissue sarcoma (ASTS), included in three protocols with high-dose ifosfamide (HDIF). PATIENTS AND METHODS: One hundred fifty six ASTS patients included in three consecutive phase II trials with HDIF (>10 g/m(2)), alone or in combination with doxorubicin (DX), were analyzed. Cofactors were institution, trial, gender, age, performance status, histologic type, grade of malignancy, prior radiotherapy, presence of locoregional disease, metastatic site, salvage surgery, number of organs involved, and disease-free interval. RESULTS: By multivariate analysis performance status >0 and lack of salvage surgery correlated with a poorer survival. A good-risk and a poor-risk group were identified, with median survival time (OS) of 29, 5, and 10 months, respectively (P = 0.00001). The 1-, 2-, and 3-year OS for 83 good-risk patients (either with PS = 0 or receiving salvage surgery) was 83, 44, and 29%, respectively, those figures being 37, 7, and 3% for 73 poor-risk patients. CONCLUSION: The design of randomized trials in ASTS including HDIF should consider those prognostic factors as stratification variables.

Intra-abdominal metastases from soft tissue sarcoma.

OBJECTIVE: To define the clinical features and prognosis of patients with abdominal metastasis from primary soft tissue sarcoma (STS) at other sites. METHODS: All patients with abdominal metastasis from STS were identified from the Royal Marsden Hospital Sarcoma Unit prospective database from January 1990 to July 2001. RESULTS: Nineteen patients developed abdominal metastasis out of a cohort of 2127 patients (0.9%) evaluated during the study interval. The median age was 49 (19-71) years. The median time to abdominal metastasis from diagnosis of the primary was 27 (8-91) months. The presenting complaints were incomplete intestinal obstruction (n = 5), abdominal pain (n = 4), mass (n = 2), gastrointestinal bleed (n = 2), urinary obstruction (n = 2), anorexia (n = 1), and abdominal distension (n = 1). Emergency laparotomy was done for perforative peritonitis (n = 2), intussusception (n = 2), and bleed in spleen (n = 1). Two patients were asymptomatic. The common histologies were myxoid liposarcoma (n = 6) and leiomyosarcoma (n = 4). The median follow-up of survivors post metastasis was 12 months. Abdominal metastatectomy was performed in 16 patients, 3 of these patients had abdominal recurrences. The 1- and 2-year overall disease specific survival for the 19 patients was 66% (SE = 11%) and 43% (SE = 13%) with a median survival of 13 months (95% CI = 11.8-14.7). Metastasectomy was associated with slight improved median post-metastasis survival (33 months vs. 8 months for unresected patients). CONCLUSIONS: Although abdominal metastasis is rare, vigilance is warranted. Symptomatic patients should be examined and investigated thoroughly for metastases. Surgery is the treatment of choice for patients with an acute presentation; however, survival is dismal.

Expression profiling using tissue microarray in 211 malignant fibrous histiocytomas confirms the prognostic value of Ki-67.

The tissue microarray technology is a high-throughput technique that allows studies of multiple markers in large tumor materials. We performed immunohistochemical profiling using tissue microarray and immunostaining for Ki-67, p53, bcl-2, CD44, cyclin A and Pgp in a series of 211 malignant fibrous histiocytomas (MFHs) with correlation to prognosis. Tissue from 50 local recurrences and 20 metastases was available for comparison with the primary tumors. In univariate analysis, Ki-67 was the only immunohistochemical marker significantly correlated with metastasis with a hazard ratio of 1.9. Multivariate analysis, with tumor size, depth, necrosis, vascular invasion, mitotic rate and Ki-67 expression, revealed an independent prognostic value of tumor size and Ki-67. Local recurrences did not differ from the corresponding primary tumors, whereas metastases showed a trend for upregulation of cyclin A and Pgp. In this large series of MFHs, a tumor size greater than 8 cm and a Ki-67 index of more than 20% were strong and independent prognostic factors for metastasis. In contrast, p53, bcl-2, CD44, cyclin A and Pgp, which have previously been suggested as prognostic factors in soft tissue sarcomas, did not show such correlations. Hence, we suggest that proliferation, as measured by Ki-67 index, should be considered as a prognostic marker in clinical management of pleomorphic soft tissue sarcomas.

Upper extremity limb salvage with microvascular reconstruction in patients with advanced sarcoma.

Limb salvage is a viable alternative to amputation in many cases of advanced sarcoma. The authors examined their experience with microvascular reconstruction of upper extremity defects after sarcoma resection, focusing on oncologic and functional outcomes. A retrospective analysis yielded 17 patients who underwent 18 free flap procedures and met the inclusion criteria. Most patients (71 percent, n = 12) had recurrent sarcoma at presentation to the authors' institution. Malignant fibrous histiocytoma was the most common pathologic subtype (n = 6). High-grade tumors were present in 94 percent of patients (n = 16). The free flap survival rate was 100 percent. The rectus abdominis flap was the most common free flap used (39 percent; n = 7). Local recurrence occurred in nine flaps (50 percent), and five patients ultimately required amputations. Six patients (35 percent) had distant recurrence. The mean Enneking score for limb function was 73 percent of the maximum (21.9 of 30). The 5-year disease-specific survival rate was 61.3 percent. In select patients with advanced upper extremity sarcoma undergoing limb salvage, microvascular flap reconstruction can provide reliable, safe coverage with reasonable preservation of function.

Soft tissue sarcoma of the upper extremity.

Soft tissue sarcomas of the upper extremities are rare and hand surgeons typically encounter only one or two undiagnosed soft tissue sarcomas during their careers. It is incumbent on the physician to review repeatedly the characteristics of these tumors and remain suspicious, because these lesions typically are misdiagnosed and treatment is delayed. The most common soft tissue sarcomas of the upper extremity are the epithelioid sarcoma, synovial cell sarcoma, and malignant fibrous histiocytoma. Limb salvage surgery is the treatment of choice for soft tissue sarcomas to preserve upper extremity function. Following wide tumor resection, adjuvant therapies such as chemotherapy, external beam radiation therapy, and brachytherapy may lessen local recurrence rates, but their effect on overall survival remains unclear.

[Locally recurrent malignant fibrous histiocytoma of the kidney and spermatic cord]. / Das lokal rezidivierende maligne fibröse Histiozytom der Niere oder des Samenstrangs.

OBJECTIVE: In this study, 26 patients with locally recurrent malignant fibrous histiocytoma of the kidney and spermatic cord after initial R0 resection were reviewed with regard to therapeutic options and prognosis. PATIENTS AND METHODS: Based on a literature query in the PubMed database, we identified 24 cases with locally recurrent malignant fibrous histiocytoma of the kidney and spermatic cord after initial R0 resection. Two of our own patients were included and afterwards the entire patient group was analyzed with regard to the time period of the first local recurrence and the overall survival rate. RESULTS: The average patient age was 58 years; in 17 cases (65%) the left side was affected. After primary therapy the 5-year survival rate was 25%; adjuvant therapy did not achieve any significant improvement in survival time ( p=0.259). The local recurrence was on average diagnosed after 13 months (with a median of 12 months). The prognosis of malignant fibrous histiocytoma after detection of the local recurrence was extraordinary poor, only 4 of 26 patients survived for longer than 3 years. The 1-, 2- and 5-year survival rates then were 34%, 28%, and 14%, respectively. The mean survival time was 31 months with a median survival of 9 months. Patients with a locally recurrent malignant fibrous histiocytoma of the spermatic cord showed a significant better survival prognosis than patients with a local recurrence of a renal malignant fibrous histiocytoma ( p=0.04). CONCLUSION: Malignant fibrous histiocytoma of the genitourinary tract are rare tumors with a high rate of local recurrence. If there are no distant metastases a R0 resection can result in a curative objective. Nevertheless, the prognosis of this disease is poor. Even early detection of local therapy failure and promptly initiated aggressive salvage therapy may offer the chance of long-term survival only in selected cases. Lifelong follow-up is necessary for patients with a malignant fibrous histiocytoma of the kidney or spermatic cord.

Head and neck soft tissue sarcomas: a multivariate analysis of outcomes.

BACKGROUND: Soft tissue sarcomas of the head and neck region represent a rare group of tumors of which a limited number of published individual- and institution-based experiences exist. METHODS: We performed an analysis of head and neck sarcoma patients identified from our institution between 1973 and 1999. Exclusion criteria included pediatric rhabdomyosarcomas, sarcomas of the neuromeningeal axis or non-head and neck primary disease sites, and bone sarcomas. All cases underwent pathologic re-review before statistical analysis. RESULTS: After pathologic review, 111 head and neck sarcoma patients remained (mean age, 47 +/- 20 years). The median duration of follow-up was 51 months; the actuarial 5-year relapse-free, disease-specific, and overall survivals were 55%, 52%, and 44%, respectively. Forty-six percent remained free of recurrence at the most recent follow-up, and the most common site of recurrence was local followed by distant sites. By multivariate analysis, size and grade significantly influenced relapse-free, disease-specific, and overall survivals, whereas margin status additionally influenced relapse-free survival. Subset analysis of the fibrosarcoma/malignant fibrous histiocytoma and desmoid/dermatofibrosarcoma protuberans histologies was undertaken. CONCLUSIONS: Size >5 cm and high-grade histology are considered poor prognostic indicators. Patients with either of these characteristics should be considered for adjuvant trials.

Prognostic factors in soft tissue leiomyosarcoma of the extremities: a retrospective analysis of 42 cases.

AIMS: Soft tissue leiomyosarcomas (LMS) are rare tumours which are associated with a poor prognosis. The goals of the present study were to describe the clinico-pathological and prognostic features of 42 patients affected by soft tissue LMS of the extremities. METHODS: The following clinical and pathological parameters were analysed: age, sex, site, size, depth, previous surgical procedures, stage, histological type, nuclear atypia, grade, mitotic activity, necrosis, surgical margins, therapy. Disease-free survival rates were calculated. RESULTS: The overall 2-year and 5-year disease free survival rates were 42.3% and 32.6%, respectively. By univariate analysis, tumour size (< or =10 cm vs. >10 cm; p = 0.01), average mitotic rate (< or =19/10 HPF vs. >19/10 HPF; p = 0.05), type of excision (wide vs. marginal or intralesional; p = 0.001) and adjuvant radiotherapy (none vs. brachytherapy+external beam radiotherapy vs. external beam radiotherapy; p = 0.02) were significantly correlated with disease progression. By multivariate analysis, the only factor that was found to be an independent predictor of disease relapse was type of excision (p = 0.001). CONCLUSIONS: Large tumour size and high mitotic rate resulted adverse prognostic factors. Adjuvant radiation therapy, in combination with wide surgical excision, allowed the best chance of cure.

Primary malignant tumors of the aorta: clinical presentation, treatment, and course of different entities.

OBJECTIVE: The objective of this study was to analyze possible correlations between the clinical presentation and the course of patients with different types of primary malignant aortic tumors. METHODS: A single academic center's experience was reviewed retrospectively. RESULTS: Four patients with primary malignant tumors of the aorta were treated in an 11-year period. Three different histologic entities were found: malignant fibrous histiocytoma, epitheloid angiosarcoma, and unclassified sarcoma. Two female patients presenting with clinical symptoms of vasculitis proved to have epitheloid aortic sarcoma. Both developed diffuse metastasis to bone and skin with initial lymphatic disease in the groin. The other patients developed local recurrence and pulmonary metastasis. Survival of the 4 patients was 11, 20, and 51 months, 1 patient with metastatic disease is still alive 6 months after surgery. CONCLUSION: Different types of malignant aortic tumors seem to have different clinical presentation and course.

Surgical management of malignant soft tissue tumours in patients aged 65 years or older.

OBJECTIVE: With the aim to determine the most effective treatment for primary malignant musculoskeletal tumours in patients aged 65 years or older, we reviewed cases of low- and high-grade neoplasms, surgical margins, surgical methods, and the prognoses of elderly and aged patients at our institution. METHODS: Records of 25 patients aged 65 years or older who had malignant soft tissue tumours from December 1986 to February 1997 were reviewed. Low- and high-grade neoplasms accounted for 8 and 17 patients, respectively. 11 patients were aged 65 to 69 years, while 14 were 70 years or older. Surgical margins were wide in 19 cases, marginal in 4, and intralesional in 2. Reconstruction was done using 6 musculocutaneous flaps and/or 4 vessel grafts. As adjuvant therapy, radiotherapy was used in 5 cases and chemotherapy in 3. There was no recurrence in patients with wide surgical margins (determined on the basis of gross inspection of the excised tumour and the cut surface); but there was recurrence in 4 patients with marginal margins, and one patient with intralesional margin. Two patients with intralesional, 4 with marginal, and 2 with wide margins, died from recurrence at the primary site and metastasis, or from metastasis without recurrence at the primary site. RESULTS: Follow-up periods ranged from 4 months to 180 months (mean, 91.6 months). The overall 5-year survival rate was 79.6%; for low- and high-grade neoplasms, the figures were 100% and 69.7%, respectively; for those aged 65 to 69 years and in their 70's or older, the figures were 90.9% and 70.1%, respectively. CONCLUSION: For geriatric patients, wide surgical margins are required to manage both low- and high-grade neoplasms, in order to avoid multiple surgeries.

Malignant fibrous histiocytoma and other fibrohistiocytic tumors in pediatric patients: the St. Jude Children's Research Hospital experience.

BACKGROUND: Malignant fibrous histiocytoma (MFH) is a controversial entity. In the current study, the authors reviewed their institutional experience with these tumors to characterize their clinical features in pediatric patients and assess the impact of surgical resection on outcome. METHODS: The records of the 28 patients who were diagnosed with MFH or MFH variants of soft tissue between January 1971 and December 2000 were reviewed and the tumors were reclassified according to the World Health Organization guidelines. RESULTS: Seventeen patients had MFH; 10 patients had angiomatoid fibrous histiocytoma (FH), and 1 patient had a plexiform fibrohistiocytic tumor. The median age of patients at the time of diagnosis was 7.3 years. The most common primary tumor site was the extremity (n = 14). Metastatic disease (to the lung) was present in only three patients, each of whom had MFH. Of the 17 MFH tumors, 13 were high grade, 8 were invasive, and 6 measured > 5 cm. All angiomatoid FH tumors and the plexiform fibrohistiocytic tumor were noninvasive, and 10 measured < or = 5 cm. Surgical treatment was comprised of wide local excision with clear margins (n = 18), amputation (n = 3), excision with positive or indeterminate surgical margins (n = 4), partial resection (n = 2), or biopsy only (n = 1). Primary reexcision was performed for 21 patients. The 5-year survival and event-free survival (EFS) estimates for patients with MFH were 76.5% +/- 11.2% and 70.6% +/- 12.1%, respectively; the 5-year survival and EFS estimates were 100% +/- 0% for patients with angiomatoid FH or plexiform fibrohistiocytic tumor. Compared with partial resection or excision, wide local excision or amputation was found to have a positive impact on the probability of EFS in patients with localized disease (P = 0.008). All four patients with metastatic or unresectable MFH had died by the time of last follow-up. CONCLUSIONS: MFH should be distinguished from angiomatoid FH and plexiform fibrohistiocytic tumors, both of which are less aggressive. Wide local excision is the treatment of choice, regardless of the histology or grade of the tumor. Patients with metastatic or unresectable MFH appear to have a poor outcome and would benefit from more effective therapies.

Results of surgery for malignant fibrous histiocytomas of soft tissue.

BACKGROUND: To address the prognosis and the role of surgery in the management of patients with malignant fibrous histiocytoma (MFH), strict definition and accurate evaluation of local recurrence is mandated, together with adequate gross and microscopic evaluation of the achieved surgical margins. METHODS: One hundred and twenty-three patients with MFH were retrospectively analyzed regarding prognostic factors of the disease, which included the following: (1). primary or recurrent, (2). histological grade, (3). adjuvant chemotherapy, (4). size, (5). local recurrence after primary surgery done at the authors' institute, (6). adjuvant radiotherapy, and (7). surgical margin. Initially, all patients were metastasis-free (M0N0). RESULTS: Local recurrence after primary surgery done at the authors' institute was the most significant prognostic factor, where 20 of 123 patients developed local recurrence ( P < 0.0001). The cause of local recurrence were insufficient surgical margin in 16 patients, while in 4, local recurrences were related to lymph node metastasis, skip metastasis, and tumor embolism. The local recurrence rate for each surgical procedure was 75% for intralesional, 44.4% for marginal, 30.8% for inadequate wide, 12.3% for adequate wide, and 5% for curative procedures. In patients with a history of recurrent tumor or infiltrative pattern, local recurrence was not observed after a curative procedure, but occurred even after an adequate wide procedure. CONCLUSION: To improve the prognosis of MFH, surgical prevention of local recurrence is essential. An adequate wide procedure for primary tumors and a curative procedure for recurrent tumors and tumors with an infiltrative pattern provide safe surgical margins.

Effect of neuronol on lifespan and development of spontaneous tumors in SAMP-1 mice with genetically accelerated aging.

Treatment of female SAMP-1 mice with Neuronol (drug containing succinic acid) given with drinking water starting from the age of 2 months during the whole life prolonged the lifespan and markedly reduced mortality of animals aged 1.5-2 years. Neuronol inhibited the development of spontaneous tumors, primarily lymphomas, and significantly prolonged lifespan in mice with tumors. Long-term treatment with Neuronol had no pathological side effects. Our experiments demonstrated geroprotective and anticarcinogenic activity of Neuronol and safety of its long-term use.

[The role of polychemotherapy in the management of malignant bone tumors]. / Die Rolle der Polychemotherapie im multimodalen Behandlungskonzept der malignen Knochentumoren.

Osteosarcoma,chondrosarcoma and Ewing tumors are the most common primary malignant bone tumors. Due to early hematogenous dissemination, surgery alone is able to cure less than 20% of patients with osteosarcoma. In Ewing-tumors less than 10% of patients will be cured with local treatment alone. By the use of aggressive polychemotherapy, given both pre- and post-surgery, cure rates of more than 50% for Ewing-tumors and about 70% for osteosarcoma could be achieved.Thus,chemotherapy is an indispensable part of the multimodal treatment of these tumor types. Chondrosarcomas are usually more highly differentiated and therefore show a lower rate of metastasis. The use of chemotherapy is not established in these tumors.

Reassessment and clinicopathological prognostic factors of malignant fibrous histiocytoma of soft parts.

Recently, the category of malignant fibrous histiocytoma (MFH) has been under discussion and new entities resembling MFH have appeared. To clarify the recent situation regarding MFH, we reassessed previously diagnosed MFH cases in accordance with the most up-to-date diagnostic criteria, which included allied tumors. We carefully reassessed 428 cases that had been diagnosed in our institute during the past 28 years. Moreover, we searched for clinicopathological prognostic factors among the cases that were finally diagnosed as MFH. Among the 428 cases, 138 cases had their diagnoses changed. The revised cases included 78 leiomyosarcomas (57%; ordinary leiomyosarcoma, 45 cases; pleomorphic leiomyosarcoma, 23 cases; myxoid leiomyosarcoma, 10 cases), 12 liposarcomas (9%; pleomorphic liposarcoma, 11 cases; dedifferentiated liposarcoma, one case), seven dermatofibrosarcoma protuberans (5%), six unclassified sarcomas (4%), five primary or metastatic carcinomas (4%), four low-grade fibromyxoid sarcomas (3%), four inflammatory myofibroblastic tumors (3%), three rhabdomyosarcomas (2%), three malignant peripheral nerve sheath tumors (2%), three acral myxoinflammatory fibroblastic sarcomas (2%) and two atypical fibroxanthomas (1.5%). Among the 1974 soft tissue sarcomas registered in our institute, MFH (428 cases) had been the most common sarcoma, followed by liposarcoma, leiomyosarcoma and rhabdomyosarcoma. However, after reassessment, leiomyosarcoma proved to be the most common soft tissue sarcoma (322 cases), followed by 290 MFH, 273 liposarcomas and 202 rhabdomyosarcomas. Among these 290 cases finally diagnosed as MFH, survival data were available in 189 cases. Tumor location in the abdominal cavity, the retroperitoneum or the head and neck (P = 0.0024), tumor size of 5 cm or more (P < 0.0001), deep tumor location (P < 0.0001), high histological grade (grade 3) based on the French Federation of Cancer Centers' grading system (P = 0.0007), and high stage (stage III or IV) based on the American Joint Committee on Cancer (AJCC) staging system (P < 0.0001) were significantly worse prognostic factors by univariate analysis. In multivariate analysis, deep tumor location and high AJCC stage were independent adverse prognostic factors. We conclude that leiomyosarcoma is the most important differential diagnosis for MFH, especially pleomorphic leiomyosarcoma from storiform-pleomorphic type and myxoid leiomyosarcoma from myxoid type. Tumor depth and AJCC stage are the most important predictive prognostic factors in MFH.

Malignant fibrous histiocytoma of the nasal cavity and paranasal sinuses: review of the last 30 years.

The clinical and pathologic features of four patients with malignant fibrous histiocytoma of the maxillary sinus were studied. All patients were male, with an age range of 43-71 years at the time of diagnosis. The main symptoms were pain in the facial region, swelling of the cheek and nasal bleeding. Pathologically, three patients were subclassified with the striform-pleomorphic type of malignant fibrous histiocytoma and one with the myxoid type. All patients were operated on and received various combinations of pre- and postoperative irradiation and intra-arterial chemotherapy given via the temporal artery. In the patient with the best prognosis, tumor extension within the maxillary sinus was minimal. Two patients developed local recurrences and died. None of the patients developed locoregional lymph node or systemic metastases.

Expression of minichromosome maintenance-2 in human malignant fibrous histiocytomas: Correlations with Ki-67 and P53 expression, and apoptosis.

This study examined the clinicopathological significance of minichromosome maintenance-2 (MCM2) expression in 38 human malignant fibrous histiocytomas (MFHs) and 36 benign fibrohistiocytic tumors (BFHTs) immunohistochemically, and in 9 human sarcoma or carcinoma cell lines, as well as 7 surgical specimens by Western blotting. MCM2 was detected in all the cell lines and surgical specimens as a single band at 120 kDa, while P53 expression was variable. Nuclear expression of MCM2 was noted in tumor but not mitotic cells of all the MFHs and 26 (72.2%) of the BFHTs, the labeling indices (LIs) being 62.0% in the 28 ordinary types, 38.5% in the 10 myxoid types, and 11.2% in the BFHTs with significant difference. Moreover, the LI was significantly higher for MCM2 than that for Ki-67 in the MFHs of both types (p<0.05). No correlation was noted between the MCM2-LI and P53 expression or apoptotic indices, which were significantly higher in the MFHs than BFHTs (p<0.01). These results indicate that MCM2 would correlate with cell proliferation rather than apoptosis in MFHs, and the expression is ubiquitous in proliferating cells, regardless of the expression of P53. Thus, MCM2 might be a reliable marker of proliferating cells in human MFH.