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10.1245/ASO.2005.09.002
Annals of Surgical Oncology 12:1090-1101 (2005)
© 2005 Society of Surgical Oncology
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Original Article

Malignant Vascular Tumors: Clinical Presentation, Surgical Therapy, and Long-Term Prognosis

Christine Leowardi, MD1, Ulf Hinz, MSc2, Yura Hormann1,5, Moritz N. Wente, MD1, Gunhild Mechtersheimer, MD3, Frank Willeke, MD4, Dittmar Böckler, MD5, Helmut Friess, MD1, Jens-Rainer Allenberg, MD5, Christian Herfarth, MD1, Markus W. Büchler, MD1 and Matthias H. M. Schwarzbach, MD1,5

1 Department of Surgery, University of Heidelberg, Im Neuenheimer Feld 110, 69120 Heidelberg, Germany
2 Unit for Documentation and Statistics, Department of Surgery, University of Heidelberg, Im Neuenheimer Feld 110, 69120 Heidelberg, Germany
3 Institute of Pathology, University of Heidelberg, Im Neuenheimer Feld 220/221, 69120 Heidelberg, Germany
4 Department of Surgery and Vascular Surgery, Mannheim University Clinic, University of Heidelberg, Theodor-Kutzer Ufer 1-3, 68135 Mannheim, Germany
5 Department of Vascular and Endovascular Surgery, University of Heidelberg, Im Neuenheimer Feld 110, 69120 Heidelberg, Germany

Correspondence: Address correspondence and reprint requests to: Matthias H. M. Schwarzbach, MD, Department of Surgery and Vascular Surgery, Mannheim University Clinic, University of Heidelberg, Theodor-Kutzer Ufer 1-3, 68135 Mannheim, Germany; E-mail: matthias. schwarzbach{at}chir.ma.uni-heidelberg.de.


   ABSTRACT
TOP
 ABSTRACT
INTRODUCTION
 PATIENTS AND METHODS
 RESULTS
 DISCUSSION
 REFERENCES
 
Background: The aim of this study was to analyze the presentation of, surgery for, and prognosis of malignant vascular tumors (MVTs).

Methods: This was an observational single-center study. Patients who underwent operation for MVTs between 1988 and 2004 were included. Data were gathered prospectively in a computerized registry.

Results: Of 568 adult patients with soft tissue malignancies, 43 (7.6%) were treated for MVTs. Twenty-four men and 19 women (median age, 55.3 years) were referred for 30 primary tumors and 13 recurrences. Symptoms were observed in 90.7% of the cases (swelling [37.2%], pain [34.9%], extrusion [11.6%], hemorrhage [7%], weight loss [4.7%], loss of energy [4.7%], impaired function [4.7%], and others [30.2%]). Tumors were located in the extremities (n = 16), trunk (n = 3), abdomen (n = 15), retroperitoneum (n = 7), and thyroid gland (n = 2). Twenty-two (51.2%) angiosarcomas, nine (20.9%) malignant hemangiopericytomas, eight (18.6%) malignant epithelioid hemangioendotheliomas, and four (9.3%) lymphangiosarcomas were seen. The median overall survival after surgery was 21.4 months, with 2-, 5-, and 10-year overall survival rates of 41.5%, 38.3%, and 18.8%, respectively. MVTs of the extremities and trunk and localized disease indicated a better prognosis than abdominal or retroperitoneal MVTs (univariate and multivariate analyses: P = .0122 and P = .0287) and metastasized stages (univariate and multivariate analyses: P = .0187 and P = .0287).

Conclusions: A considerable number of patients with soft tissue malignancies undergo surgery for MVT. Various symptoms and a multilocular occurrence are typical. The course of MVTs is aggressive. Tumor site and stage are important prognostic factors. Surgery is potentially curative, especially for localized disease of the extremities and trunk.

Key Words: Malignant vascular tumors • Survival • Prognostic factors • Univariate analysis • Multivariate analysis


   INTRODUCTION
TOP
 ABSTRACT
 INTRODUCTION
PATIENTS AND METHODS
 RESULTS
 DISCUSSION
 REFERENCES
 
Malignant vascular tumors (MVTs) arise from blood or lymphatic vessels. They collectively represent one of the rarest forms of soft tissue malignancies, comprising <1% of all sarcomas.1 They can occur at any location in the body and are most frequently very aggressive tumors with a poor prognosis.2–4 The expected number of and, thus, the clinical relevance for patients treated for soft tissue malignancies at a specialized surgical unit is not known. The clinical symptoms and sites of occurrence vary.2–4 Because of the scarce amount of clinical data about the presentation of MVT, early diagnosis is difficult, and tumors are, in most cases, discovered in advanced stages. Presently, surgery is the mainstay of treatment.5 Apart from case reports and a few retrospective observational studies, little has been reported about surgical treatment options and surgical treatment results.2,5,6 Moreover, the value of adjuvant therapy modalities such as chemotherapy or radiotherapy is still unclear,3,5 and only a few prognostic factors have been identified to date.2,5,6

The entity of MVT consists of different histopathologic subtypes. Angiosarcomas (AS) represent one of four major histological subtypes, along with lymphangiosarcomas (LAS), malignant hemangiopericytomas (MHP), and malignant epithelioid hemangioendotheliomas (EHE).7 AS, LAS, and EHEs are of endothelial cell origin, whereas MHP originate from the Zimmermann pericytes of the blood vessel wall.8 The term angiosarcoma is commonly used for all sarcomas showing endothelial differentiation, whether they originate in the vascular or lymphatic endothelium. AS have a predilection for cutaneous sites, especially in the head and neck region of elderly male patients,9 and metastasize early via either the blood or the lymphatic system.10 Chronic lymphedema and irradiation have been described as etiological factors in the pathogenesis of AS.7 The radiation-induced AS occur in the radiation field years after the radiotherapy. LAS are found in Stewart-Treves syndrome, in the upper extremity in postmastectomy patients, or are associated with lymphedema in other sites of the body.11 Malignant EHE show significant atypia and mitotic activity and can behave as aggressively as AS.7 They seem to occur more often in women and can be located in different tissues, including lung, liver, and soft tissues.7 MHP are rare tumors and can behave like high-grade sarcomas. Abdominal MHP, in particular, seem to take highly aggressive courses.12

Because of the limited data about MVT, we report the findings of an observational study performed at a single institution. The analysis of the clinical data focused on clinical presentation, tumor control, and prognosis in this rare but highly aggressive tumor entity.


   PATIENTS AND METHODS
TOP
 ABSTRACT
 INTRODUCTION
 PATIENTS AND METHODS
RESULTS
 DISCUSSION
 REFERENCES
 
Adult patients diagnosed with MVT and treated at the Department of Surgery at the University of Heidelberg from 1988 to January 2004 were included in the analysis. Of 568 patients with soft tissue sarcomas who underwent operation in the observation period, 43 patients (7.6%) received surgical treatment for MVT.

Of these 43 patients, 24 (55.8%) were male, and 19 (44.2%) were female. The median age was 55.3 years, with a range of 25 to 77 years. Histopathologically, 22 AS, 4 LAS, 9 MHP, and 8 EHE were diagnosed. In total, 23 patients were referred for a primary tumor, 7 patients were referred with a primary tumor and metastasis, 5 patients were referred with local recurrence, 4 patients were referred with local recurrence and metastasis, and 4 patients were referred with metastasis. Two of the 30 patients with primary tumors had previously undergone operations at other institutions (able 1). Surgery was the only treatment in 23 patients. Twelve patients received surgical therapy in combination with radiotherapy. Of these, four patients received intraoperative radiotherapy combined with postoperative boost radiotherapy, and eight patients received postoperative external radiotherapy alone. Seven patients were subjected to additive chemotherapy regimens with diverse chemotherapeutic agents (epirubicin, ifosfamide, doxorubicin, cisplatin, carboplatin, vincristine, and etoposide) according to the carboplatin, epirubicin, vincristine, ifosfamide, and etoposide (CEVAIE), vincristine, actinomycin D, ifosfamide, doxorubicin (VAIA); cisplatin, ifosfamide, doxorubucin, vincristine (PIAV); or cyclophosphamide, vincristine, doxorubicin, dacarbazine (CYVADIC) protocol. One patient received treatment with radiotherapy and chemotherapy (Table 1Go).


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  		TABLE 1. Clinical and pathologic data of 43 patients with malignant vascular tumors
 
Definitions
Primary tumor was defined as the initially diagnosed mass, including previously biopsied or incompletely resected lesions, within 12 weeks after the initial diagnosis. Local recurrence was defined as the development of a mass of the same histological subtype at the same site after prior complete resection. Recurrence at another site was considered as a distant recurrence. Complete resection with negative margins was defined as the complete removal of all macroscopic disease with histologically negative margins; complete resection with positive margin was defined as complete removal of all macroscopic tumor with histologically positive margins. Incomplete resection was defined as incomplete removal of the tumor mass with macroscopic residual tumor and tumor exploration/biopsy when the tumor was considered to be inoperable. EHE were not included in the analysis of tumor grading because grading has not been established for this subtype of MVT.

Statistical Analysis
SAS software (release 8.02; SAS Institute, Inc., Cary, NC) was used for statistical analysis. Fisher’s exact test was applied to compare the histological and histopathologic factors. The Kruskal-Wallis test was performed to analyze tumor diameter and histological characteristics. The distribution of age at operation, operation time, blood loss, postoperative hospital stay, and follow-up time of patients alive were presented as median and interquartile range (IQR).

Overall survival from the date of operation was calculated by the Kaplan-Meier estimate.13 The log-rank test was performed to compare survival time distributions between curves. Patients alive were censored at the last follow-up. Multivariate hazard ratio (HR) estimates and corresponding 95% confidence intervals were computed by using Cox proportional hazards regression analysis.14 As potential risk factors, the variable localization, presentation status, histological characteristics, and surgery were included in the multivariate regression analysis. Follow-up investigations of all patients alive were performed in February 2004. Two-sided P values were always computed, and an effect was considered statistically significant at P ≤ .05.


   RESULTS
TOP
 ABSTRACT
 INTRODUCTION
 PATIENTS AND METHODS
 RESULTS
DISCUSSION
 REFERENCES
 
Predisposing Characteristics
Concerning etiology, three (75%) of four patients with LAS had disease diagnosed as Stewart-Treves syndrome. Two of them were patients who developed LAS 8 and 30 years after mastectomy. The third patient presented with a history of primary lymphedema of both legs for 40 years before he developed LAS in one leg. Predisposing factors were observed in three (14%) patients with AS. Among these, a thorotrast-induced angiosarcoma of the liver, one liver angiosarcoma in a patient with cirrhosis, and one angiosarcoma of the thigh 19 years after radiotherapy for a malignant melanoma were diagnosed. Risk factors in patients with EHE and MHP were not observed.

Clinical Symptoms and Site of Presentation
Of the 43 patients, 39 (90.7%) presented with clinical symptoms. Swelling, pain, and extrusion predominated. Other symptoms included hemorrhage, weight loss, loss of energy, and impaired function (Table 2Go). Only four patients did not show any symptoms. Tumors were mainly located in the visceral organs (39.5%) and in the extremities (37.2%), followed by the retroperitoneum (16.3%) and trunk (7.0%) (Table 3Go). As far as subtype and site of tumor growth are concerned, seven (32%) AS occurred in the extremities, six (27%) in the liver, three (14%) in the retroperitoneum, two (9%) in the trunk, two (9%) in the thyroid gland, one (4.5%) in the spleen, and one (4.5%) in the stomach. All LAS (n = 4) were found in the limbs (half of them in the lower extremity). MHP were located in the retroperitoneum (n = 4; 45%), extremities (n = 3; 33%), head (n = 1; 11%), and stomach (n = 1; 11%). Five (63%) EHE were diagnosed in the liver, two (25%) in the extremities, and one (12%) in the small bowel.


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  		TABLE 2. Clinical symptoms of 43 patients with malignant vascular tumors
 

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  		TABLE 3. Tumor localization in 43 patients with malignant vascular tumors
 
Surgical Treatment
Among operations in the extremities, eight wide excisions, one compartment resection, three amputations, and four biopsies were performed (Table 4Go). Of the visceral operations, 94.1% were associated with organ resections (seven liver transplantations, three hemihepatectomies, two gastrectomies, one small-bowel resection, two thyroidectomies, and one splenectomy (Table 4Go). Retroperitoneal surgery in seven patients consisted of one multivisceral resection, two en-bloc excisions, and four exploratory procedures with biopsy (Table 4Go). MVT in the trunk were treated by wide excision or local en-bloc excision. Complete resection with curative intent was performed in 32 patients (74.4%). In 23 patients (53.5%), complete resection with microscopically negative margins could be achieved; in 9 patients (20.9%), tumors were completely resected with microscopically positive margins. In two patients (4.7%), only incomplete tumor resection was achieved. In nine patients (20.9%) with nonresectable tumors, exploratory or biopsy procedures were performed. Reasons for exploratory surgery without resection were liver metastasis in three cases, peritoneal metastasis in two cases, lymphangiosis blastomatosis in one case, and inoperability in five cases because of widespread tumor infiltration.


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  		TABLE 4. Surgical procedures according to tumor localization in 43 patients with malignant vascular tumors
 
Operative morbidity occurred in 17 (39.5%) of 43 patients. Wound-related complications and bleeding were the most common subtypes. Reoperation was necessary in 10 cases (23.3%). The 30-day mortality rate was 7% (Table 5Go). One patient died because of liver failure after transplantation, and two patients died of hemorrhage due to diffuse peritoneal angiosarcomatosis without the option of intervention.


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  		TABLE 5. Surgical morbidity and mortality in 43 patients with malignant vascular tumors
 
Histopathologic Findings
The median tumor diameter was 7.2 cm (IQR, 3.7–10.5 cm). The tumor diameter differed significantly among the four histological subtypes (P = .0424; Table 6Go). MHP and EHE were the largest tumors, with a median diameter of 9 and 10 cm, respectively. AS had a median diameter of 5.8 cm, and LAS had a median diameter of 4 cm. In all, 19 AS, 4 LAS, and 7 MHP were graded: 83.3% were graded as high, 13.4% as intermediate, and 3.3% as low. A difference in tumor grade did not reach statistical significance among the different histological subtypes (P = .0952; Table 6Go). However, rates of complete resections differed significantly among the four histological subtypes (P = .0106). The highest complete resection rates were achieved for EHE and AS (87.5% and 86.3%, respectively) compared with MHP and LAS (33.3% and 75%, respectively; Table 6Go). Metastases were detected in 17 (39.5%) of the 43 patients. In two, metastases were diagnosed during surgery; the others presented at first admission with metastases. The occurrence of metastasis did not correlate with histological subtype (P = .626).


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  		TABLE 6. Clinicopathologic factors and subtype of malignant vascular tumor in 43 patients
 
Clinical Outcome
The median follow-up was 56 months for surviving patients (IQR, 1–140 months). No patient was lost to follow-up. The median overall survival was 21.4 months (IQR, 4.2–85.1 months), with a 2-, 5-, and 10-year survival rate of 41.5%, 38.3%, and 18.8%, respectively (Figure 1Go). At last follow-up, 28 patients had died of disease (65.1%). Localization of the tumor was the strongest prognostic factor in a univariate analysis that showed a benefit in median overall survival for tumors located in the extremities and trunk, with a 2- and 5-year survival rate of 67.1% and 60.4%, respectively, compared with intra-abdominal and retroperitoneal tumors, with a 2- and 5-year survival rate of 22.6% and 17%, respectively (P = .0122; Table 7Go and Figure 2Go). This significant difference could be confirmed by multivariate analysis (P = .0287), with an increased risk of death for patients with intra-abdominal and retroperitoneal tumors (HR, 2.385; Table 7Go). The second significant prognostic factor in a univariate analysis was metastatic status at presentation, which showed a benefit in median overall survival for patients without metastases at presentation (2- and 5-year rates of 62.6% and 57.4%, respectively) compared with patients with metastases at presentation (2-and 5-year rates of 22.1% and 14.7%, respectively; P = .0187; Table 7Go and Figure 3Go). This significant result could also be confirmed by multivariate analysis (P = .0441) and revealed an increased risk of death for patients with metastases at presentation (HR, 2.442; Table 7Go). Completeness of resection with negative margins was associated with a trend toward improved overall survival compared with complete resection with positive margins and incomplete resection. This difference, however, did not reach statistical significance in univariate and multivariate analyses (P = .387 and P = .293, respectively; Table 7Go). There was no difference in survival in univariate and multivariate analyses among the diverse histological subtypes (P = .569 for the univariate analysis and P = .868 for the comparison between AS and LAS/EHE in the multivariate analysis, P = .315 for the comparison between MHP and LAS/EHE in the multivariate analysis, respectively; Table 7Go and Figure 4Go). Taking the two significant prognostic factors together, patients with tumors of the extremities and trunk without metastasis at presentation had a significantly better prognosis than those with abdominal or retroperitoneal tumors with or without metastases at presentation and had a better prognosis than those with extremity and trunk tumors with metastases at presentation (P = .0135; Figure 5Go).


Figure 1
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  		FIG. 1. Survival distribution function of all patients included in this study. The median follow-up time was 56 months (interquartile range, 1–140 months).

 

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  		TABLE 7. Results from univariate and multivariate analyses of survival in 43 patients with malignant vascular tumors (likelihood ratio test of the final model: P = .0059)
 

Figure 2
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  		FIG. 2. Five-year survival distribution function associated with tumor location (tumors of extremities and trunk vs. visceral and retroperitoneal tumors). The median follow-up was 56 months (interquartile range, 1–140 months).

 

Figure 3
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  		FIG. 3. Five-year survival distribution function associated with metastasis status at presentation. The median follow-up was 56 months (interquartile range, 1–140 months).

 

Figure 4
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  		FIG. 4. Five-year survival distribution function associated with tumor histology (angiosarcomas [AS], lymphangiosarcomas [LAS], malignant hemangiopericytomas [MHP], and epithelioid hemangioendotheliomas [EHE]). The median follow-up was 56 months (interquartile range, 1–140 months). Heidelberg Sarcoma Registry, 1988 to 2004.

 

Figure 5
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  		FIG. 5. Five-year survival distribution function associated with tumor location and metastatic status at presentation (interquartile range, 1–140 months). Extr., extremity; Visc., visceral; Retrop., retroperitoneal.

 
Patients with gross residual tumor (locally or distant) survived for a median of 8 months. Within this group, there are three long-term survivors. In one woman, biopsy was performed for a soft tissue lesion of the left thigh which had metastasized to the lung when she was 31 years old. After histological diagnosis of AS, chemotherapy (six cycles of doxorubicin, holoxane, and ifosfamide) and radiotherapy (50 Gy) were administered. Presenting with a complete remission, the patient is still alive 8.5 years after the initial diagnosis of a metastasized AS. A male patient underwent surgical exploration and biopsy of a retroperitoneal and gluteal MHP at the age of 35 years. Bone metastases were diagnosed 4 months after surgery. Subsequently, chemotherapy (ifosfamide and doxorubicin) was given, but the disease progressed. A partial response was seen after the chemotherapy regimen was changed to the CEVAIE protocol. Nine months later, disease had progressed further, and the patient was again treated by CEVAIE chemotherapy, resulting in a survival time of almost 5 years (the patient died 59 months after diagnosis of the disease). Another male patient survived 180 months after the diagnosis of an unresectable mandibular MHP at the age of 50 years. The type of chemotherapy given was not documented, and, thus, the information was not available for investigation.


   DISCUSSION
TOP
 ABSTRACT
 INTRODUCTION
 PATIENTS AND METHODS
 RESULTS
 DISCUSSION
REFERENCES
 
MVT of the blood and lymphatic vessels have not been very well evaluated. Only a few clinical series are available (Table 8Go), and it is difficult to predict how many MVT patients will be treated at a specialized surgical unit.2,3,5,6,15–18 Our analysis showed that a considerable percentage (7.8%) of patients who underwent operation for malignant mesenchymal tumors at our institution had disease diagnosed as MVT. As proposed before, the different histological subtypes of MVT were included in this analysis.2,7,15 Specifically, the subtypes included AS, LAS, MHP, and malignant EHE.7 AS occurred with a predilection for the liver and lower extremities; however, diverse localizations are known (gastrointestinal organs, secretory glands, and deep or superficial soft tissues).16–22 All LAS in our series were diagnosed in the extremities reflecting the etiology of LAS, namely, Stewart-Treves syndrome in patients with chronic lymphedema.11,23 There are only a few reports of MHP locations, which include retroperitoneal, pelvic, intracranial, pulmonary, or hepatic sites.24–28 We found MHP in the extremities, stomach, retroperitoneum, chest, and head. In contrast to MHP, malignant EHE were observed to develop organotropically in the liver; this has been described previously.29,30 The present analysis revealed that MHP and EHE were usually much larger than AS and LAS. This may reflect a predisposition of each subtype to a certain region.


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  		TABLE 8. Review of the literature: malignant vascular tumors and clinical outcome
 
In this series, we found that >90% of the patients with MVT presented with clinical symptoms at the time of diagnosis. The most common symptoms were pain, swelling, and extrusion, followed by diverse other signs of the disease (Table 2Go). Most of the symptoms observed were related to the expanding tumor growth of MVT. Organotropic MVT, conversely, can mimic benign disease (e.g., goiter in this series) or metastatic disease (e.g., suspected liver or spleen metastasis), thus leading to an unexpected diagnosis at histopathologic examination. Moreover, MVT are diagnosed after emergency procedures, e.g., for abdominal or gastrointestinal bleeding.31,32 In this series, one patient presented with abdominal bleeding due to spontaneous rupture of an AS of the liver, and another two patients presented with intestinal hemorrhage. The heterogeneity of symptoms and tumor occurrence makes early diagnosis difficult.6 Furthermore, oncological treatment standards are impeded by MVT that either mimic benign disease or require emergency operations.

Inherent to diagnostic modalities in MVT, as in soft tissue tumors, is the difficulty in noninvasively characterizing whether a lesion is malignant. Neither radiological tomography (computed tomography and magnetic resonance imaging) nor angiographic examinations can be applied to determine whether a lesion is benign or malignant.33 Positron emission tomography can be used to characterize functional activity in MVT, as has been shown for various other types of soft tissue sarcomas.34,35

In this analysis, we observed that patients with MVT require a wide range of different surgical procedures, depending on the tumor site. The predominant procedures in the extremities and trunk are wide excision, compartment resection, or amputation. Specific organ operations (thyroidectomy, gastrectomy, splenectomy, liver resection, and liver transplantation) are required for organotropic disease. Malignant EHE of the liver is an accepted indication for liver transplantation, whereas the results of such a procedure in AS are discouraging.36 In our series, EHE was the most common clinical setting for liver transplantation. A complete tumor resection was achieved in most of our patients (74.4%), whereas a considerable percentage of patients (20.9%) were found to have unresectable disease during the exploratory procedure. AS and EHE were resectable in most cases. However, most MHP were diagnosed as being unresectable during surgery, and the LAS specimen, although completely excised, often showed positive resection margins. Metastatic disease and widespread tumor infiltration were the reasons for inoperability. Operative morbidity was acceptable: infection-related complications and bleeding were the most common causes. The 30-day mortality for MVT surgery was high when patients who died because of diffuse hemorrhage from peritoneal angiosarcomatosis were included. Besides surgery, radiotherapy has been used as adjuvant treatment and as prevention of tumor progression, and remission has even been documented.4,37 Some reports claim that surgery combined with radiotherapy may be the best treatment currently available for patients with AS.3,5,37–39 We, like others, have observed long-term survivors among patients receiving chemotherapy in advanced tumor stages.40

This series presents one of the longest follow-up periods of surgically treated MVT patients reported to date (Table 8Go). With a median follow-up of 56 months, the observation time of this analysis clearly exceeds the average of 35 months (reviewed in the literature3,5,6,9,15,16,42,43 and in Table 8Go). In our series, we observed a median overall survival time of 21 months. In a series of 44 patients, including AS, LAS, and EHE (skin and soft tissue), a median overall survival of 20 months was found.6 Another analysis showed a median overall survival of 28.4 months in a cohort of 88 patients with AS of the scalp.9 These results reflect the poor prognosis and aggressive course of MVT (Table 8Go). The 2-, 5-, and 10-year survival rates in this analysis were 41.5%, 38.3%, and 18.1%, respectively. In a study of 67 patients with AS, an overall 5-year survival of 35% was reported.3 Morgan et al.16 investigated 47 patients with cutaneous AS, outlining the poor prognosis and demonstrating a 5-year survival rate of 34%.

Visceral and retroperitoneal MVT were associated with significantly reduced 2- and 5-year survival rates compared with tumors of the extremities and trunk. This finding has not been shown previously for MVT. Data sustaining this result were reported by Meis-Kindblom and Kindblom,15 who found that retroperitoneal AS, compared with other locations, was associated with a worse prognosis. Additionally, Auguste et al.41 and Bardaxogou et al.12 found that intra-abdominal MHP take more aggressive courses than MHP of other locations. Furthermore, Mark et al.3 reported that AS of the extremities have a better prognosis than those at other sites. Delayed clinical presentation and diagnosis of visceral or retroperitoneal MVT may explain the aggravated prognostic outcome. In our study, localized tumor growth without metastatic disease was associated with improved 2- and 5-year survival rates compared with metastasized disease at initial presentation. Although this result might be expected, only two studies have addressed this clinically relevant issue. Karpeh et al.6 and Morgan et al.16 showed that patients with metastatic disease have a lower survival rate than patients with localized disease. Although MVT have a poor prognosis, the most encouraging results have been reported for patients after complete resection of localized disease.6 In this analysis, complete resection presented a trend toward an improved clinical outcome, although statistical significance was not reached. Tumor size, reported by Maddox and Evans2 and Holden et al.4 to be a prognostic factor, was not corroborated as such in our patients. Like tumor size, histological subtyping showed no association with clinical outcome in our analysis. The fact that histology was shown to have no influence on survival is consistent with two other larger studies of MVT.2,6 These data show that clinical analysis, as performed in this series, can include the different subtypes of MVT.

MVT represent a group of tumors including AS, LAS, MHP, and malignant EHE. These rare malignancies present with various clinical symptoms and a ubiquitous occurrence. Prognosis correlates with tumor location and metastatic disease at initial presentation. The histopathologic subtype did not correlate with prognosis in this analysis. Surgical resection of localized disease is a potentially curative treatment, especially in patients with MVT in the extremities and trunk. Metastatic disease is the major cause for poor clinical outcome and underlines the need for improved adjuvant therapy modalities.

Received for publication September 8, 2004. Accepted for publication July 30, 2005.


   REFERENCES
TOP
 ABSTRACT
 INTRODUCTION
 PATIENTS AND METHODS
 RESULTS
 DISCUSSION
 REFERENCES
 

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