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

  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]

• 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]

• 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]

• Gangrene

  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]

• Hoarseness

  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]

• Pharyngitis

  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]

• 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]

• Melena

  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]

• Odynophagia

  The patient presented with a 1-year history of a muffled voice and mild odynophagia. [ncbi.nlm.nih.gov]

• Jaundice

  Major clinical presentation (78.4%) was abdominal pain or discomfort, accompanied with some other non-specific symptoms such as malaise, anorexia, weight loss, jaundice and fever, and small cases (14.9%) were asymptomatic. [ncbi.nlm.nih.gov]

• 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]

• Facial Swelling

  Occasionally a similar presentation may be due to a malignant tumor, and oral clinicians must always bear this possibility in mind with facial swellings that do not respond to conventional therapy Malignant fibrous histiocytoma is a high-grade aggressive [ncbi.nlm.nih.gov]

• Dysuria

  A 43-year-old man presented with dysuria for 12 months. Serum prostate-specific antigen level was normal. T2-weighted MR images showed a well-circumscribed tumor with inhomogeneous intensity in the prostate. [ncbi.nlm.nih.gov]

• 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]

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.