Malignant fibrous histiocytoma is a rare undifferentiated pleomorphic type of sarcoma, most commonly arising in the lower and upper extremities and the abdomen. Imaging studies and biopsy with histopathological examination of the tumor are required to confirm the diagnosis.
Malignant fibrous histiocytoma (MFH) is primarily encountered in late adulthood and in the teenage population . The clinical presentation depends on the site of its occurrence, and the upper and lower limbs are the predominant sites where this tumor arises . Both distal and proximal femur, proximal tibia and the proximal humerus are the most common sites , but the abdomen, retroperitoneum, the breast, the lungs, the head and neck and scrotum have all been described in literature as potential locations as well     . The main symptom of MFH regardless of the site, however, is localized pain and swelling, and patients often report a rapidly enlarging mass . In 20% of cases with limb involvement, pathological fractures may occur . Abdominal pain, low-grade fever, weight loss, anorexia, and malaise are the main symptoms of hepatic MFH, apart from local pain and swelling . Scrotal tumors present as gradual and painless (or sometimes painful) swelling of the scrotum, similar to breast MFH, in which a solitary painless mass is palpated on the breast (more commonly found on the left), with a mean diameter of 7.9 cm . Manifestations of pulmonary MFH include cough, hemoptysis, dyspnea and fever .
Entire Body System
The tumour was well localised within the spleen and the patient remains alive and well 34 months after splenectomy without any adjuvant therapy. [ncbi.nlm.nih.gov]
- Inguinal Mass
An 80-year old male presented with a 2-week history of a right inguinal mass, with mild pain in the same region. Ultrasonography and computed tomography showed a mass arising from the right spermatic cord. [ncbi.nlm.nih.gov]
However, deep venous thrombosis developed, resulting in left foot ischemia and toe gangrene, necessitating left below-the-knee amputation. Disseminated metastasis occurred 3 months after tumor resection. [ncbi.nlm.nih.gov]
A 66-year-old man with a history of asymptomatic bilateral swelling of the axillary lymph nodes was admitted with hoarseness and pain of the right knee. Computed tomography showed a large pseudoaneurysm deriving from descending aorta. [ncbi.nlm.nih.gov]
Laryngoscopy detected two grossly different tumors in the hypopharynx: a malignant fibrous histiocytoma (MFH) in the postcricoid area and a squamous cell carcinoma (SCC) in the posterior pharyngeal wall. [ncbi.nlm.nih.gov]
- Abdominal Pain
We herein report a rare case of primary splenic MFH in a 30-year-old Indian male who presented with abdominal pain with a history of recurrent hydatid cyst of liver and spleen. [ncbi.nlm.nih.gov]
Abdominal pain, low-grade fever, weight loss, anorexia, and malaise are the main symptoms of hepatic MFH, apart from local pain and swelling. [symptoma.com]
A 22-year-old man underwent an FDG PET/CT scan to evaluate possible malignancy due to melena and fever for 1 month. The images demonstrated intense FDG activity with an SUVmax of 24.6 in a large mass in the fundus of the stomach. [ncbi.nlm.nih.gov]
The patient presented with a 1-year history of a muffled voice and mild odynophagia. [ncbi.nlm.nih.gov]
Liver, Gall & Pancreas
- Knee Pain
The patient underwent a third operation, with extensive curettage and total knee arthroplasty 38 years after the initial surgery, because of progressive knee pain. Postoperative histopathology study showed high-grade malignant fibrous histiocytoma. [ncbi.nlm.nih.gov]
pain experienced in last month 0.8 1.2 3.2 1.5 0.8 1.8 0.9 0.4 0.5 Hypertension (Self-reported) 6.8 18.8 37.6 3.6 3.6 6.1 5.9 -0.7 -3.8 Gastro-oesophageal reflux (gord) / gastric reflux (Self-reported) 0.5 0.6 1.6 0.5 -1.0 -0.2 -1.3 -0.3 0.8 Illnesses [twas-hub.org]
- Thigh Pain
The patient remained well until 4 months later, when left thigh pain returned with fever and leukocytosis (white blood cell count as high as 20.7 10(9)/L, 80% neutrophils). No source of infection was localized. [ncbi.nlm.nih.gov]
Face, Head & Neck
The diagnostic workup has to start with a detailed patient history that will note the onset and course of symptoms, as well as preexisting events that may have increased the risk for MFH, including radiation, surgery, fractures, osteonecrosis, Paget's disease or fibrous dysplasia . A thorough physical examination should follow, during which detection of a palpable mass will support the need for imaging studies to determine its additional features. In the setting of skeletal lesions, an aggressive soft tissue mass with peripheral calcifications (and sometimes cortical erosion) can be observed by plain radiography . Ultrasonography may be useful for initial evaluation of a mass in the abdomen and the breast, and a solitary heterogenous low-density mass is usually seen  , while multinodular appearance is very rare . Computed tomography (CT) and magnetic resonance imaging (MRI), however, are superior methods compared to radiography and ultrasonography, as they can depict the extension of the lesion into adjacent tissues and aid in staging of the tumor  . Bone scanning may also be useful in the setting of MFH . To distinguish MFH from dozens of other sarcomas, a biopsy, and subsequent histopathological examination is mandatory, and five subtypes have been recognized . The mixture of spindle and polygonal or rounded cells arranged in a storiform pattern, presence of multinucleated giant cells, nuclear pleomorphism, abundant mitotic activity and marked cellularity are main features of the storiform-pleomorphic subtype, encompassing 70% of all cases, while myxoid, giant cell, inflammatory and angiomatoid are other subtypes of MFH . Furthermore, immunohistochemistry and electron microscopy (where available) should be used as adjunctive procedures  .
- Mediastinal Mass
We may encounter with the major airway obstruction in the endoluminal or extraluminal lung and mediastinal masses or those with both components together. [ncbi.nlm.nih.gov]
- Left Pleural Effusion
During the immediate postoperative evolution, the patient presented left pleural effusion for 2 weeks. The patient was referred to Oncology Service for complementary treatment. [ncbi.nlm.nih.gov]
After treatment of childhood ALL, reported cases of secondary MFH is extremely rare in the literature. Herein we present a case of MFH/UPS that developed as a secondary cancer 6 years after the end of ALL treatment. [ncbi.nlm.nih.gov]
PURPOSE: To review the experience in transarterial neoadjuvant chemotherapy of malignant fibrous histiocytoma (MFH) in soft tissue and to analyze the factors related to prognosis of MFH in soft tissue. [ncbi.nlm.nih.gov]
Although its etiology is not clearly known, radiotherapy, chemical agents, previous history of surgery, trauma and fracture, and Hodgkin lymphoma have been blamed. [ncbi.nlm.nih.gov]
The etiology of this tumor is unknown, but seems to be multifactorial. [jdrntruhs.org]
[…] histiocytoma Hereditary bone dysplasia with malignant change Atlas_Id 10056 Note DMS-MFH is an hereditary bone dysplasia / cancer syndrome Inheritance autosomal dominant; rare hereditary cancer syndrome with only four families identified worldwide; etiology [atlasgeneticsoncology.org]
(Etiology) The exact cause and mechanism of formation of Pleomorphic Malignant Fibrous Histiocytoma of Heart is unknown. However, extra-cardiac tumors have shown genetic involvement. [dovemed.com]
Etiology The site of origin is thought to be a histiocyte or fibroblast, it is most likely a primitive mesenchymal cell with divergent differentiation that expresses fibroblastic, histiocytic, and myofibroblastic phenotypes. [eyewiki.aao.org]
PATIENTS AND METHODS: Subjects with a diagnosis of DFSP, MFH, KS, or SC between 1990 and 2006 were identified in the Surveillance, Epidemiology, and End Results Program database. [ncbi.nlm.nih.gov]
It is relatively uncommon in the head and neck (about 5 percent of the total), but when it does occur, it is usually in soft tissue or, rarely, in the mandible, said Erich Sturgis, MD, MPH, Associate Professor of Head and Neck Surgery and Epidemiology [enttoday.org]
Medical Center, Guangzhou Medical University, Guangzhou, China 3 Institute/Department of Dermatology, No.1 Hospital, Anhui Medical University, Hefei, China 4 Division of Genetics and Molecular Medicine, King’s College London, London, UK 5 Department of Epidemiology [ard.bmj.com]
High expression levels of AMPD2 have been shown to correlate with poor patient outcome and a proliferative tumor phenotype in UPS.  Epidemiology [ edit ] UPS is regarded as the most common soft-tissue sarcoma of late adult life. [en.wikipedia.org]
Pathophysiology MFH has variable pathophysiology and many subtypes. [enttoday.org]
TLR2 and TLR4, which are expressed on the cell surface, are the only TLRs known to be responsive to microbial ligands. 36 TLR4 and TLR2 pathways are the key pathways in sepsis pathophysiology. 37 Unlike TLR4 and TLR3, TLR2 is associated with deleterious [nature.com]
Cancer care at Mayo Clinic's campus in Arizona Mayo Clinic experts in Arizona discuss the new facility, which allows them to create a seamless experience for patients by integrating all cancer services from prevention to survivorship. [medprofvideos.mayoclinic.org]
By retaining the phosphatase complex PP2A into the cytoplasm, prevents the dephosphorylation of the AP-1 subunit JUN which is required for proper activation of the transcription factor (PubMed:28609714). [ghr.nlm.nih.gov]
Protein coding - A0A1B0GSG4 - 5' truncation in transcript evidence prevents annotation of the start of the CDS. CDS 5' incomplete Transcript Support Level 1, when transcripts are supported by at least one non-suspect mRNA. [asia.ensembl.org]
Prevention Because the causes of MFHs are not known, there is no known prevention. Special concerns Repeat surgery may be necessary for MFHs because these tumors sometimes redevelop. Careful monitoring by the medical team will be required. [encyclopedia.com]
- Pobirci DD, Bogdan F, Pobirci O, Petcu CA, Roşca E. Study of malignant fibrous histiocytoma: clinical, statistic and histopatological interrelation. Rom J Morphol Embryol. 2011;52(1):385-388.
- Guo J, Zhou S, Rao NP, Pez GH. Pleomorphic malignant fibrous histiocytoma/undifferentiated high-grade pleomorphic sarcoma of the scrotum in a patient presenting as fournier gangrene: a case report. Appl Immunohistochem Mol Morphol. 2010;18(5):473-478.
- Li YR, Akbari E, Tretiakova MS, et al. Primary hepatic malignant fibrous histiocytoma: clinicopathologic characteristics and prognostic value of ezrin expression. Am J Surg Pathol. 2008;32(8):1144-1158.
- Qiu S-Q, Wei X-L, Huang W-H, et al. Diagnostic and therapeutic strategy and the most efficient prognostic factors of breast malignant fibrous histiocytoma. Scientific Reports. 2013;3:2529.
- Huang C, Xin L, Cui Y, Qi D. Primary malignant fibrous histiocytoma of the lung: a report of 20 cases [Article in Chinese]. Zhongguo Fei Ai Za Zhi. 2011;14(5):414-417.
- Matushansky I, Charytonowicz E, Mills J, Siddiqi S, Hricik T, Cordon-Cardo C. MFH classification: differentiating undifferentiated pleomorphic sarcoma in the 21st Century. Expert Rev Anticancer Ther. 2009;9(8):1135-1144.