eMedicine Specialties > Radiology > Musculoskeletal
Malignant Fibrous Histiocytoma, Soft Tissue
Updated: Sep 16, 2009
Introduction
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
In 2002, the World Health Organization (WHO) modified the nomenclature for soft tissue neoplasms, with the most significant changes being in the nomenclature for fibrous and lipomatous malignancies.2,3,4 Originally, MFH was defined as a neoplasm showing both fibroblastic and histiocytic differentiation; however, more recent evaluation has shown no evidence of true histiocytic differentiation. Furthermore, the morphologic pattern seen with pleomorphic MFH is shared by a variety of poorly differentiated malignant neoplasms. For these reasons, the WHO suggests new terminology for the various subtypes of MFH; this is described further in the Pathophysiology section, below4,5 In this article, however, we will continue to use the term "malignant fibrous histiocytoma".
Radiograph demonstrates a soft tissue mass posterior to the femoral diaphysis (same patient as Images 2-5 in Multimedia). Although not appreciated on the radiograph, a component of the mass extends inferiorly and contains a small cluster of calcifications (within circle).
Noninfused T1-weighted MRI reveals a low signal intensity mass posterior to the femoral diaphysis (same patient as Images 1, 3, 4, and 5 in Multimedia).
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 in Multimedia).
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 in Multimedia).
Angiogram reveals tumor hypervascularity (same patient as Images 1-4 in Multimedia).
Recent studies
Park et al studied CT and MR images of 13 cases of MFH of the head and neck with regard to the lesions' location and extent, size, margin, internal architecture, and pattern and degree of enhancement. They also studied the signal-intensity characteristics on MR images. All lesions were seen as well-defined or as ill-defined, aggressive masses (mean size, 4.9 cm). The tumors were located in the sinonasal cavity in 6 cases; soft tissue of the face and neck in 5 cases; oral cavity in 1 case; and orbital roof in 1 case. One lesion arose in the bones with background fibrous dysplasia; 12 lesions invaded the adjacent soft tissues; and bone destruction with 11 of the lesions. Attenuation, signal intensity, and enhancement pattern were nonspecific except in cases of myxoid MFH.6
Guo et al assessed 33 previously diagnosed MFH cases based on the WHO classification to identify whether some of the MFH diagnoses would have the diagnosis changed. Among the 33 cases, 17 cases (51.5%) of MFH had their diagnoses changed, including 5 leiomyosarcomas, 3 malignant peripheral nerve sheath tumors, 1 fibrosarcoma, 1 inflammatory myofibrosarcoma, 1 giant cell tumor, and 1 angiomatoid fibrous histiocytoma. The remaining 16 cases (48.5%) were reconfirmed as MFH/undifferentiated pleomorphic sarcoma (UPS). Only Vimentin was always expressed in MFH/UPS, while some of the tumors were positive for myogenic antigen and CD68.7
Pathophysiology
Uncertain histogenesis and numerous subtypes make malignant fibrous histiocytoma (MFH) a rather controversial entity. While original researchers postulated histiocytic theories of origin. more recent evaluation has shown no evidence of true histiocytic differentiation.4,5
In general, the tumor contains both fibroblastlike and histiocytelike cells in varying proportions, with spindled and rounded cells exhibiting a storiform arrangement. Several histologic subtypes have been described:
- Storiform/pleomorphic MFH (current WHO classification: undifferentiated high-grade pleomorphic sarcoma)
- Myxoid MFH (current WHO classification: myxofibrosarcoma)
- Giant cell MFH (current WHO classification: undifferentiated pleomorphic sarcoma with giant cells)
- Inflammatory MFH (current WHO classification: undifferertiated pleomorphic sarcoma with prominent inflammation)
Angiomatoid fibrous histiocytoma (formerly known as angiomatoid MFH) is now classified by the WHO as a tumor of uncertain differentiation; it generally affects children and young adults and shows indolent behavior with low metastatic potential.4,8,9,10,5
Frequency
United States
Malignant fibrous histiocytoma (MFH) accounts for 20-24% of soft-tissue sarcomas, making it the most common soft-tissue sarcoma occurring in late adult life. The storiform/pleomorphic subtype is the most common variety.
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.11
- 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
Malignant fibrous histiocytoma (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
Malignant fibrous histiocytoma (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.12
Presentation
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, malignant fibrous histiocytoma (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.13 Early and complete surgical removal using wide or radical resection is indicated because of the aggressive nature of the tumor.14,15
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.16,17,18
Limitations of Techniques
Although, typically, MRI is suited best for defining the anatomy of the tumor and its surrounding structures, the signal characteristics of malignant fibrous histiocytoma (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.
Differential Diagnoses
Liposarcoma, Soft Tissue
Synovial Sarcoma
Other Problems to Be Considered
Imaging findings of MFH are nonspecific. Other malignant tumors, such as pleomorphic liposarcoma, synovial sarcoma, leiomyosarcoma, rhabdomyosarcoma, and some benign tumors, may have an identical appearance. Biopsy is often necessary to make a diagnosis.
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References
O'Brien JE, Stout A P. Malignant fibrous xanthomas. Cancer (Philad.). 1964;17:1445–1455.
Al-Agha OM, Igbokwe AA. Malignant fibrous histiocytoma: between the past and the present. Arch Pathol Lab Med. Jun 2008;132(6):1030-5. [Medline].
Nakayama R, Nemoto T, Takahashi H, Ohta T, Kawai A, Seki K, et al. Gene expression analysis of soft tissue sarcomas: characterization and reclassification of malignant fibrous histiocytoma. Mod Pathol. Jul 2007;20(7):749-59. [Medline].
Murphey MD. World Health Organization classification of bone and soft tissue tumors: modifications and implications for radiologists. Semin Musculoskelet Radiol. Sep 2007;11(3):201-14. [Medline].
So-called fibrohistiocytic tumours. In: Fletcher CDM, Unni KK, Mertens F. World Health Organization Classification of Tumours. Pathology and Genetics of Tumours of Soft Tissue and Bone. Lyon: IARC Press; 2002:120-125.
Park SW, Kim HJ, Lee JH, Ko YH. Malignant fibrous histiocytoma of the head and neck: CT and MR imaging findings. AJNR Am J Neuroradiol. Jan 2009;30(1):71-6. [Medline].
Guo H, Xiong Y, Nong L, Zhang S, Li T. [Reassessment of the pathological diagnosis in 33 cases of malignant fibrous histiocytoma]. Beijing Da Xue Xue Bao. Aug 18 2008;40(4):374-9. [Medline].
Gibbs JF, Huang PP, Lee RJ, et al. Malignant fibrous histiocytoma: an institutional review. Cancer Invest. 2001;19(1):23-7. [Medline].
Kearney MM, Soule EH, Ivins JC. Malignant fibrous histiocytoma: a retrospective study of 167 cases. Cancer. Jan 1 1980;45(1):167-78. [Medline].
Ros PR, Viamonte M Jr, Rywlin AM. Malignant fibrous histiocytoma: mesenchymal tumor of ubiquitous origin. AJR Am J Roentgenol. Apr 1984;142(4):753-9. [Medline].
Pezzi CM, Rawlings MS Jr, Esgro JJ, et al. Prognostic factors in 227 patients with malignant fibrous histiocytoma. Cancer. Apr 15 1992;69(8):2098-103. [Medline].
Sabesan T, Xuexi W, Yongfa Q, Pingzhang T, Ilankovan V. Malignant fibrous histiocytoma: outcome of tumours in the head and neck compared with those in the trunk and extremities. Br J Oral Maxillofac Surg. Jun 2006;44(3):209-12. Epub 2005 Jul 18. [Medline].
Makimoto Y, Yamamoto S, Takano H, Motoori K, Ueda T, Kazama T, et al. Imaging findings of radiation-induced sarcoma of the head and neck. Br J Radiol. Oct 2007;80(958):790-7. [Medline].
Weiss SW, Enzinger FM. Malignant fibrous histiocytoma: an analysis of 200 cases. Cancer. Jun 1978;41(6):2250-66. [Medline].
Thway K. Angiomatoid fibrous histiocytoma: a review with recent genetic findings. Arch Pathol Lab Med. Feb 2008;132(2):273-7. [Medline].
Kransdorf MJ, Murphey MD. Imaging of Soft Tissue Tumors. Philadelphia: WB Saunders Co;1997:192-209.
Resnick D. Diagnosis of Bone and Joint Disorders. 3rd ed. Philadelphia: WB Saunders Co;1995:3964-5, 4524-9.
Nascimento AF, Raut CP. Diagnosis and management of pleomorphic sarcomas (so-called "MFH") in adults. J Surg Oncol. Mar 15 2008;97(4):330-9. [Medline].
Issakov J, Kollender Y, Soyfer V, Bickels J, Flusser G, Meller I, et al. A single-team experience of limb sparing approach in adults with high-grade malignant fibrous histiocytoma. Oncol Rep. Oct 2005;14(4):1071-6. [Medline].
Further Reading
Related eMedicine topic
Fibrous Histiocytoma
Clinical studies
Gemcitabine and Docetaxel With Or Without Bevacizumab in Leiomyosarcoma, Malignant Fibrous Histiocytoma and Angiosarcoma
Ipilimumab in Young Patients With Advanced or Refractory Solid Tumors
Sunitinib in Treating Patients With Metastatic, Locally Advanced, or Locally Recurrent Sarcomas
Observation, Radiation Therapy, Combination Chemotherapy, and/or Surgery in Treating Young Patients With Soft Tissue Sarcoma
Monoclonal Antibody IMC-A12 and Doxorubicin in Treating Patients With Unresectable, Locally Advanced, or Metastatic Soft Tissue Sarcoma
Keywords
malignant fibrous histiocytoma, fibrous histiocytoma, malignant fibrous xanthoma, fibroxanthoma, MFH, undifferenatiated pleomorphic sarcoma
