Malignant fibrous histiocytoma (MFH) is primarily encountered in late adulthood and in the teenage population [1]. The clinical presentation depends on the site of its occurrence, and the upper and lower limbs are the predominant sites where this tumor arises [2]. Both distal and proximal femur, proximal tibia and the proximal humerus are the most common sites [1], 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 [1] [2] [3] [4] [5]. The main symptom of MFH regardless of the site, however, is localized pain and swelling, and patients often report a rapidly enlarging mass [1]. In 20% of cases with limb involvement, pathological fractures may occur [1]. Abdominal pain, low-grade fever, weight loss, anorexia, and malaise are the main symptoms of hepatic MFH, apart from local pain and swelling [3]. 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 [4]. Manifestations of pulmonary MFH include cough, hemoptysis, dyspnea and fever [5].

• Splenectomy

  Splenectomy was done. On histopathological examination, a diagnosis of malignant mesenchymal tumor, possibly storiform variant of malignant fibrous histiocytoma, was made. On immunohistochemistry, the tumor was positive for vimentin and CD68. [ncbi.nlm.nih.gov]

• Localized Pain

  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]

• 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]

• Swelling of the Scrotum

  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. [symptoma.com]

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 [1]. 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 [1]. 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 [2] [3], while multinodular appearance is very rare [3]. 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 [2] [3]. Bone scanning may also be useful in the setting of MFH [1]. To distinguish MFH from dozens of other sarcomas, a biopsy, and subsequent histopathological examination is mandatory, and five subtypes have been recognized [6]. 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 [1]. Furthermore, immunohistochemistry and electron microscopy (where available) should be used as adjunctive procedures [4] [6].

• 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]

• Ovarian Mass

  A 46-year-old woman presented with whole abdominal discomfort, and imaging revealed a 3-cm-sized ill-defined ovarian mass with extensive peritoneal carcinomatosis. [ncbi.nlm.nih.gov]

The first-line treatment is complete excision of the mass, but in some cases postoperative chemo- and/or radiotherapy is recommended. [ncbi.nlm.nih.gov]

Treatment The treatment strategy was decided by a multidisciplinary team who are experienced in surgical oncology, radiation oncology, radiology, and tumor pathology. [cancerjournal.net]

This article represents the largest study analyzing prognosis of this tumor in the head and neck to date. STUDY DESIGN: Retrospective analysis of SEER database. [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]

The objective of this study is to use the Surveillance, Epidemiology, and End Results (SEER) database to compare characteristics of this tumor based on location to better understand its prognosis in the head and neck region. [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]

A population-based epidemiologic and prognostic study of 137 patients. Cancer 1991;67:499–505. [link.springer.com]

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]

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]

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]

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]

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. [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]

1. 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.
2. 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.
3. 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.
4. 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.
5. 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.
6. 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.