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Radiology > MUSCULOSKELETAL
Malignant Fibrous Histiocytoma, Soft Tissue
Article Last Updated: Mar 26, 2008
AUTHOR AND EDITOR INFORMATION
Section 1 of 12  
Author: Doris Yip, MD, Staff Physician, Department of Radiology, University of Chicago Hospitals
Doris Yip is a member of the following medical societies: American College of Radiology
Coauthor(s):
Gregory Scott Stacy, MD, Assistant Professor, Department of Radiology, University of Chicago Hospitals
Editors: Michael A Bruno, MD, Associate Professor, Departments of Radiology and Medicine, Pennsylvania State University College of Medicine; Director, Radiology Quality Management Services, Milton S Hershey Medical Center, Pennsylvania State University College of Medicine; Bernard D Coombs, MB, ChB, PhD, Consulting Staff, Department of Specialist Rehabilitation Services, Hutt Valley District Health Board, New Zealand; Murali Sundaram, MBBS, FRCR, FACR, Consulting Staff, Department of Diagnostic Radiology, The Cleveland Clinic Foundation; Robert M Krasny, MD, Consulting Staff, Department of Radiology, The Angeles Clinic and Research Institute; Felix S Chew, MD, MBA, EdM, Professor, Department of Radiology, Vice Chairman for Radiology Informatics, Section Head of Musculoskeletal Radiology, University of Washington
Author and Editor Disclosure
Synonyms and related keywords:
malignant fibrous xanthoma, fibroxanthoma, MFH
Background
Malignant fibrous histiocytoma (MFH), described by O'Brien and Stout in 1964, is the most common soft-tissue sarcoma of late adult life.1
Related eMedicine topics:
Fibrous Histiocytoma
Multinucleate Cell Angiohistiocytoma
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Malignant Fibrous Histiocytoma (MFH)
Pathophysiology
Uncertain histogenesis and numerous subtypes make MFH a rather controversial entity. Researchers have postulated both histiocytic and primitive mesenchymal cell theories of origin. In general, the tumor contains both fibroblastlike and histiocytelike cells in varying proportions, with spindled and rounded cells exhibiting a storiform arrangement. Five histologic subtypes have been described: (1) storiform/pleomorphic (most common), (2) myxoid, (3) giant cell, (4) inflammatory (usually retroperitoneal), and (5) angiomatoid (often located more superficially than other varieties).2, 3, 4
Frequency
United States
MFH accounts for 20-24% of soft-tissue sarcomas, making it the most common soft-tissue sarcoma occurring in late adult life.
Mortality/Morbidity
The clinical stage of the tumor, which is defined by tumor grade, size, and presence of distant metastases, is the most important prognostic factor. Histologic subtype and method of surgical treatment are also important prognostic factors. The anatomic site and depth of the primary tumor may also be of prognostic importance, but this is controversial.5
- Patients with low-grade, intermediate-grade, and high-grade tumors have 10-year survival rates of 90%, 60%, and 20%, respectively. Patients with tumors smaller than 5 cm at presentation have survival rates of 79-82%. Patients with tumors of 5-10 cm have survival rates of 62-68%, and those with tumors larger than 10 cm have survival rates of 41-51%.
- Distant metastasis most commonly occurs to the lung (90%), bone (8%), and liver (1%). The rate of metastasis varies with the histologic subtype from 23% (myxoid) to 50% (giant cell). Positive microscopic margins are associated with decreased disease-free survival, while resection with negative microscopic margins decreases the incidence of local recurrence; however, these factors do not have a direct impact on outcome.
- The overall survival rate of patients with MFH ranges from 36-58% at 5 years; however, patients with retroperitoneal tumors have an overall 5-year survival rate of 15-20%.
Race
MFH occurs more commonly in white patients than in patients of African or Asian descent.
Sex
The male-to-female ratio is approximately 2:1.
Age
The tumor occurs with a peak incidence in the fifth and sixth decades, but an age range of 10-90 years is reported. Although the tumor is rare in children, the angiomatoid subtype is the most frequently occurring variety in patients younger than 20 years.
Anatomy
MFH occurs most commonly in the extremities (70-75%, with lower extremities accounting for 59% of cases), followed by the retroperitoneum. Tumors typically arise in deep fascia or skeletal muscle. MFH has been reported to occur in the lung, kidney, bladder, scrotum, vas deferens, heart, aorta, stomach, small intestine, orbit, CNS, paraspinal area, dura mater, facial sinuses, nasal cavity, oral cavity, nasopharynx, and soft tissues of the neck.6
Clinical Details
The most common clinical presentation is an enlarging painless soft-tissue mass in the thigh, typically 5-10 cm in diameter. Two thirds of tumors are intramuscular. Rare signs and symptoms include episodic hypoglycemia and rapid tumor enlargement during pregnancy. Additionally, MFH has been associated with hematopoietic diseases such as non-Hodgkin lymphoma, Hodgkin lymphoma, multiple myeloma, and malignant histiocytosis. Retroperitoneal MFH usually presents with constitutional symptoms, including fever, malaise, and weight loss. The tumor is often larger than 10 cm in diameter at presentation and may cause displacement of the bowel, kidney, ureter, and/or bladder. MFH may also occur secondary to radiation exposure and shrapnel injury and may be seen adjacent to metallic fixation devices, including total joint prostheses.7 Early and complete surgical removal using wide or radical resection is indicated because of the aggressive nature of the tumor.8, 9
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Preferred Examination
As with other soft-tissue tumors, MRI is the imaging method of choice because of its ability to provide superior contrast between tumor and muscle, excellent definition of surrounding anatomy, and ease of imaging in multiple planes. Axial CT may be obtained in lieu of MRI if the patient is claustrophobic or if metal implants (eg, pacemakers, aneurysm clips) render the patient unsuitable for MRI. CT is also useful for evaluation of calcifications.10, 11, 12
Limitations of Techniques
Although, typically, MRI is suited best for defining the anatomy of the tumor and its surrounding structures, the signal characteristics of MFH are not specific, and the true histologic nature of the tumor or other soft-tissue masses often cannot be ascertained by imaging alone, with few exceptions (eg, lipoma). Furthermore, patients with cardiac pacemakers and aneurysm clips may not be able to undergo examination with MRI. In these patients, CT can provide adequate information regarding the location and gross extent of the mass, although the contrast between tumor and muscle is often less than that seen with MRI. However, no single imaging technique can provide a specific histologic diagnosis of MFH, and biopsy is usually necessary.
Liposarcoma, Soft Tissue
Synovial Sarcoma
Other Problems to Be Considered
Imaging findings of MFH are nonspecific. Other malignant tumors, such as liposarcoma (specifically, the round cell, pleomorphic, and undifferentiated subtypes), synovial sarcoma, leiomyosarcoma, rhabdomyosarcoma, and some benign tumors, may have an identical appearance. Biopsy is often necessary to make a diagnosis.
Findings
- Radiographs may reveal a nonspecific soft-tissue mass, often greater than 5 cm in diameter.
- Deep intramuscular tumors often lie adjacent to the diaphysis of a long bone.
- Secondary osseous involvement, including periosteal reaction, cortical erosion, and pathologic fracture, is uncommon but suggestive of MFH.
- Calcification or ossification can be detected in 5-20% of patients.
- Calcifications within the tumor may be punctate, curvilinear, and/or poorly defined (see Image 1).
- Heterotopic bone formation may be present in the periphery of the mass.
Findings
- CT typically reveals a nonspecific, large, lobulated, soft tissue mass of predominantly muscle density, with nodular and peripheral enhancement of solid portions (see Image 6).
- Central areas of low attenuation may be present, corresponding to myxoid regions, old hemorrhage, or necrosis (see Image 8).
- Fat attenuation is not observed in the tumors; this fact can be useful in distinguishing MFH from some well-differentiated liposarcomas.
- CT may be used to evaluate potential internal matrix and/or cortical erosion.
- Retroperitoneal tumors manifest as heterogeneous masses with areas of hemorrhage and/or necrosis and occasionally focal or diffuse coarse calcifications (approximately 10%). The tumors may invade the abdominal musculature but do not invade the renal veins or inferior vena cava.
Findings
- MRI typically reveals an intramuscular mass with heterogeneous signal intensity on all pulse sequences.
- As with other soft-tissue neoplasms, the signal intensity pattern is nonspecific, usually low to intermediate on T1-weighted images (see Image 2, Image 13, Image 16) and intermediate to high on T2-weighted images (see Image 7, Image 9, Image 15, Image 18).
- Regions of prominent fibrous tissue (high collagen content) may demonstrate low signal intensity on both T1-weighted and T2-weighted images.
- Calcification may present as foci of low signal on both T1-weighted and T2-weighted sequences.
- Subacute hemorrhage should be considered when regions of high signal are noted on both T1-weighted and T2-weighted images.
- Areas of necrosis demonstrate a signal pattern similar to that of fluid.
- As with CT, solid components of MFH typically reveal nodular and peripheral enhancement (see Image 3, Image 14, Image 17).
- Tumor margins appear relatively well defined on MRI. A low signal intensity margin may be observed, representing a pseudocapsule.
Gadolinium-based contrast agents (gadopentetate dimeglumine [Magnevist], gadobenate dimeglumine [MultiHance], gadodiamide [Omniscan], gadoversetamide [OptiMARK], gadoteridol [ProHance]) have recently been linked to the development of nephrogenic systemic fibrosis (NSF) or nephrogenic fibrosing dermopathy (NFD). For more information, see the eMedicine topic Nephrogenic Fibrosing Dermopathy. The disease has occurred in patients with moderate to end-stage renal disease after being given a gadolinium-based contrast agent to enhance MRI or MRA scans.
As of late December 2006, the FDA had received reports of 90 such cases of NSF/NFD . Worldwide, over 200 cases have been reported, according to the FDA. NSF/NFD is a debilitating and sometimes fatal disease. Characteristics include red or dark patches on the skin; burning, itching, swelling, hardening, and tightening of the skin; yellow spots on the whites of the eyes; joint stiffness with trouble moving or straightening the arms, hands, legs, or feet; pain deep in the hip bones or ribs; and muscle weakness. For more information, see the FDA Public Health Advisory or Medscape.
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False Positives/Negatives
The radiologist should keep in mind that the diagnosis of MFH is made using histopathology, not imaging; however, MRI remains invaluable for delineating tumor extent.
Findings
Sonography typically reveals a well-defined heterogeneous mass that contains hyperechoic areas of cellularity and hypoechoic regions of necrosis (see Image 11, Image 12). The appearance of tumors on ultrasound is nonspecific; however, sonography may be used to evaluate tumor volume. Retroperitoneal tumors tend to appear as hypoechoic solid masses with scattered regions of heterogeneity.
Findings
Technetium-99m bone scintigraphy often shows increased uptake by tumor, regardless of the presence of calcium within the tumor or invasion of adjacent bone (see Image 4, Image 19, Image 20). Hypervascular lesions show increased radionuclide uptake on both dynamic and blood pool images. The mildly increased uptake observed on static images is also probably related to hypervascularity and may be more prominent with internal calcification. Bone scans usually are not ordered to evaluate the primary tumor but may be obtained if osseous metastases are suggested. Gallium-67 scans may also demonstrate increased activity (see Image 10).
Findings
Angiographic findings are nonspecific. The tumor may be hypovascular or, more commonly, hypervascular with early venous return (see Image 5). Similarly, retroperitoneal tumors may be either hypovascular or hypervascular, with blood supply from the lumbar, celiac, iliac, renal, renal capsular, and/or inferior adrenal arteries.
Early and complete surgical removal using wide or radical resection is indicated because of the aggressive nature of the tumor.13 Follow-up studies to exclude tumor recurrence usually are performed using MRI.
Medical/Legal Pitfalls
- Radiologists should keep in mind that although a large deep soft-tissue mass without visible fat in an extremity of an otherwise healthy older patient is statistically likely to represent MFH, the actual diagnosis can be made only via biopsy. Many malignant and several benign soft-tissue tumors may have imaging appearances identical to that of MFH.
- If the radiologist is asked to perform a biopsy on a potentially malignant soft-tissue mass, the orthopedic surgeon resecting the mass must be consulted first. With certain tumors, the biopsy tract must be removed with the mass; a presurgical image-guided biopsy performed without appropriate orthopedic consultation may result in more extensive surgery (including amputation) than would have been necessary otherwise.
See also the Medscape topic Medical Malpractice and Legal Issues.
| Media file 1:
Radiograph demonstrates a soft tissue mass posterior to the femoral diaphysis (same patient as Images 2-5). Although not appreciated on the radiograph, a component of the mass extends inferiorly and contains a small cluster of calcifications (within circle). |
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| Media file 2:
Noninfused T1-weighted MRI reveals a low signal intensity mass posterior to the femoral diaphysis (same patient as Images 1, 3, 4, and 5). |
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| Media file 3:
T1-weighted MRI obtained following intravenous gadolinium administration reveals inhomogeneous enhancement of the soft tissue mass (same patient as Images 1, 2, 4, and 5). |
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| Media file 4:
Spot image of a technetium-99m bone scintigram (delayed phase) reveals increased radiotracer uptake in the soft tissue tumor (same patient as Images 1, 2, 3, and 5). |
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| Media file 5:
Angiogram reveals tumor hypervascularity (same patient as Images 1-4). |
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| Media file 6:
TI-weighted image obtained following intravenous contrast administration reveals a soft tissue mass within the posteromedial right thigh (same patient as Image 7). Mild enhancement is noted. The mass is relatively isointense. |
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| Media file 7:
T2-weighted MRI reveals a hyperintense mass within the posteromedial right thigh (same patient as Image 6). The mass proved to be a malignant fibrous histiocytoma. |
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| Media file 8:
CT reveals a mass within the lateral musculature of the left thigh (same patient as Images 9 and 10). The malignant fibrous histiocytoma is only slightly hypointense to muscle and conceivably could be overlooked. |
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| Media file 9:
T2-weighted MRI demonstrates the tumor much more conspicuously (same patient as Images 8 and 10). |
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| Media file 10:
Gallium-67 scintigram reveals increased radiotracer activity in the left thigh (same patient as Images 8 and 9). |
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| Media file 11:
Ultrasound image demonstrates a predominantly hypoechoic mass in the thigh representing a malignant fibrous histiocytoma. Regions of hyperechogenicity likely correspond to cellular components of the tumor. |
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| Media file 12:
Ultrasound image demonstrates another example of a predominantly hypoechoic mass within the patient's thigh, proven to be a malignant fibrous histiocytoma (same patient as Images 13-15). |
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| Media file 13:
Preinfusion T1-weighted MRI reveals a large mass in the anterior thigh (same patient as Images 12, 14, and 15). Although slightly heterogeneous, the tumor is predominantly isointense to muscle. |
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| Media file 14:
T1-weighted MRI obtained following intravenous administration of gadolinium reveals circumferential enhancement of the tumor (same patient as Images 12, 13, and 15). |
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| Media file 15:
T2-weighted MRI reveals heterogeneous signal of the malignant fibrous histiocytoma (same patient as Images 12-14). Central low signal intensity may be the result of old blood products. |
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| Media file 16:
Preinfusion T1-weighted MRI of a patient with a medial left thigh mass proven at biopsy to represent a malignant fibrous histiocytoma (same patient as Images 17-20). Although slightly heterogeneous, the mass is predominantly isointense to muscle. |
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| Media file 17:
T1-weighted MRI obtained following gadolinium administration reveals heterogeneous enhancement of the malignant fibrous histiocytoma (same patient as Images 16 and 18-20). |
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| Media file 18:
Malignant fibrous histiocytoma. T2-weighted MRI reveals predominantly increased signal intensity in the tumor mass (same patient as Images 16, 17, 19, and 20). |
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| Media file 19:
Technetium-99m bone scintigram (delayed phase) reveals increased radiotracer uptake in the left thigh (same patient as Images 16-18 and 20). |
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| Media file 20:
Spot images from technetium-99m scintigram (blood pool phase) reveal increased radiotracer uptake in the left thigh (same patient as Images 16-19). |
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Malignant Fibrous Histiocytoma, Soft Tissue excerpt Article Last Updated: Mar 26, 2008
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