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Undifferentiated pleomorphic sarcoma or, as formerly called, malignant fibrous histiocytoma is a type of sarcoma which originates from fibroblast and histiocytic cells. It is the most common type of sarcoma among all soft tissue sarcomas in adults. Its most common site is the lower limb, followed by the upper limb and the retroperitoneum. It is rarely encountered on chest wall. In the differential diagnosis of masses on chest wall, it is important to consider undifferentiated pleomorphic sarcoma in surgical planning. In this article, we report a male case with a giant undifferentiated pleomorphic sarcoma located above the right scapula.

BACKGROUND Malignant fibrous histiocytoma (MFH) is one of the most common soft tissue sarcomas among adults. It is characterized by large size, high grade, and biological aggressiveness. There are many reports of MFH after local stimulation, such as bone fracture, implants, and chronic osteomyelitis. In this paper, we report a patient who developed MFH 6 years after amputation, suggesting that wound healing and mechanical force play a role in the local stimulation of this disease. CASE SUMMARY A 66-year-old man complained of persistent pain in his residual mid-thigh. He had undergone amputation surgery due to a traffic accident 6 years prior. Physical examination showed tenderness but no abnormalities in appearance. X-ray radiographs and magnetic resonance imaging supported the diagnosis of a tumor, and a biopsy confirmed that the lesion was MFH. The patient received neoadjuvant chemotherapy and left hip disarticulation. During the 6-mo follow-up, there were no symptoms of recurrence. CONCLUSION Postsurgery MFH has been reported before, and many studies have attributed it to the biological effects of implants. Our case report shows that this disease can develop without an implant and thus highlights the importance of local stimulation. The wound-healing process and mechanical force can both promote this tumor, but whether they directly cause MFH needs further investigation.

Pleomorphic sarcoma of the larynx is a rare variant of laryngeal cancer. We present the case of a 59-year-old male patient who has been smoking for 40 years. He presented with signs and symptoms of an obstructive glottic mass. The diagnostic workup pointed to a malignant pathology; the histopathology report confirmed the diagnosis of glottic undifferentiated pleomorphic sarcoma (malignant fibrous histiocytoma).

Highlights • Metastatic undifferentiated pleomorphic sarcoma (Malignant Fibrous Histiocytoma) is a rare entity in the visceral organs. • We report a case of a metastatic primary cardiac undifferentiated pleomorphic sarcoma which presented with a recurrent small bowel intussusception in a young man. • Laparotomy by a small midline incision performed on the same day identified an intussusception of a 15-cm section of small intestine caused by a 4-cm intraluminal metastasis from undifferentiated pleomorphic sarcoma. • Metastasis intussusception in the small intestine is a very rare condition and they are part of differential diagnosis in patient with a history of tumor who present with intussusception., Introduction Undifferentiated metastatic pleomorphic sarcoma (Malignant Fibrous Histiocytoma) is a rare entity in the small intestine, especially when the primary tumor is of cardiac origin. Case Report We report a case of metastatic intestinal undifferentiated pleomorphic sarcoma in a young patient with a history of primary cardiac tumor in the left atrium and recurrent small bowel intussusception. He was admitted for abdominal pain and constipation. A segmental resection of the small intestine was performed with side-to-side entero-enteroic anastomosis. Discussion Intussusception of the small bowel is rare in adults and it represents about 1–3% of intestinal obstructions. It mainly affects the fifth decade with a male/female ratio of 1:5 More than 60% of patients with intussusception have a tumor with 50% being malignant. This type of intussusception can be diagnosed on the CT abdominalscan. Radiological features include a typical “target” sign with overdistention of the proximal intestine and air-fluid levels, but the diagnosis of certainty is made by exploratory laparotomy. Conclusion The metastatic tumors that cause intussusception represent a rare clinical condition in adult patients, but much more common than primary ones. Metastasis to the small intestine are part of differential diagnosis in patient with a history of tumor who present with intussusception.

Undifferentiated pleomorphic sarcoma (UPS) is a high-grade neoplasm that is usually located in the extremities and retroperitoneum. In the past, UPS was considered the most common soft tissue sarcoma in adults; due to improvements in diagnostic techniques, most cases have been reclassified as other lineage-specific tumors. Gnathic bones are rarely affected, and the clinicopathological characteristics of this neoplasm when diagnosed in the jaw remain to be better described. In this report, we present a rare case of mandibular UPS affecting an 88-year-old female who demonstrated a painful swelling on the right side of the mandible that was accompanied by a pathological fracture. Microscopic examination revealed a pleomorphic spindle-cell neoplasm with mitotic figures and necrosis. The patient underwent surgery and adjuvant radiotherapy but experienced metastasis after 12 months of follow-up and died. Diagnosis of UPS is challenging, and oral pathologists must be aware of this entity when dealing with aggressive undifferentiated neoplasms.

Undifferentiated pleomorphic sarcoma (UPS) was previously known as malignant fibrous histiocytoma (MFH). This sarcoma occurs preferentially in the extremities and retroperitoneal space; primary pulmonary UPS/MFH is rare. We report a 52-year-old woman referred to our hospital with dyspnea and severe cough. Chest computed tomography (CT) revealed a pulmonary mass in the left upper lobe and pleural effusion. Cytology of the effusion showed no malignancy; however, the tumor increased rapidly in size, and the patient’s respiratory symptoms worsened. The tumor occupied almost all of the left upper lobe and involved the adjacent pericardium. She underwent left upper lobectomy with pericardial resection and reconstruction. Postoperative pathology of the resected specimen showed undifferentiated pulmonary sarcoma, pT4N0M1a stage IV A, and genetic analyses revealed the v-Ki-ras2 Kirsten rat sarcoma viral oncogene homolog (KRAS) mutation. The patient’s dyspnea recurred 1 month postoperatively, and CT showed marked pleural effusion. An 18F-fluorodeoxyglucose positron emission tomography demonstrated abnormal diffuse accumulation of 18F-fluorodeoxyglucose in the left pleural cavity. We initiated five cycles of chemotherapy with doxorubicin and ifosfamide, and the patient has been well without recurrence for 24 months after multidisciplinary treatment with surgery followed by systemic combination chemotherapy. We successfully treated our patient with primary pulmonary UPS/MFH using a multidisciplinary approach, even though this sarcoma carries a poor prognosis and is insensitive to both chemotherapy and radiotherapy.

A heterogeneous solid mass in the spermatic cord was detected in Magnetic Resonance imaging in a 70-year-old patient who presented with a palpable mass in the left inguinal canal. Pathology result after radical orchiectomy was reported as Malignant Fibrous Histiocytoma (MFH). MFH is mostly seen in the extremities and retroperitoneum, rarely found in the spermatic cord. Its etiopathogenesis has not been fully elucidated, and radical orchiectomy is performed in the treatment. There is no proven treatment method for adjuvant therapy. We aimed to present this rare case in the spermatic cord with the aim of contributing to the literature and defining the treatment plan., Sol inguinal kanalda ele gelen kitle şikayeti ile başvuran 70 yaşındaki hastada Manyetik Rezonans görüntülemede spermatik kordda heterojen solit kitle tespit edildi. Radikal orşiektomi sonrası patoloji sonucu Malign Fibröz Histiyositoma (MFH) olarak raporlandı. MFH daha çok ekstremite ve retroperitonda görülmekte olup spermatik kordda nadiren saptanmaktadır. Etyopatogenezi tam olarak aydınlatılamamış olup tedavide radikal orşiektomi yapılmaktadır. Adjuvan tedavi için ise kanıtlanmış bir tedavi yöntemi bulunmamaktadır. Spermatik kordda nadir görülen bu olguyu literatüre katkısı olması ve tedavi planının tanımlanabilmesi amacı ile sunmayı amaçladık.

Cardiac tumors are generally benign; however, they can rarely be malignant. Malignant fibrous histiocytoma is a type of sarcoma. Cardiac malignant fibrous histiocytomas are clinically and histologically confused with atrial myxomas. In this case report, we present a patient who was administered to the hospital with non-specific complaints, determined to have an intracardiac mass and subsequently diagnosed with malignant fibrous histiocytoma, which shortly recurred in the form of intracardiac masses after postoperative chemotherapy.

This report describes a patient who presented with a large thyroid nodule and compressive symptoms. Immunohistochemical staining showed diffuse marked reactivity for vimentin and focal reactivity for CD68 and Ki‐67 that is compatible with primary undifferentiated pleomorphic sarcoma of the thyroid. This report emphasizes and discusses extremely rare thyroid cancer type., Primary thyroid undifferentiated pleomorphic sarcoma is fast‐growing neck tumor in the elderly patients clinically. Although all routine tests before thyroid radiofrequency ablation (RFA) show benign thyroid nodule, we chose surgery instead of RFA.

Highlights • Sickle cell disease pathophysiology. • Natural progression of a pre-existing bone infarct. • Malignant transformation of a pre-existing bone infarct. • A rare case describing malignant transformation of a pre-existing bone infarct in a known sickle cell anemia patient into malignancy in specific undifferentiated pleomorphic sarcoma., Introduction Avascular necrosis of the bone is a very common finding in sickle cell disease (SCD). The malignant transformation of a pre-existing bone infarct is extremely rare, only few cases has been reported related to different etiologies one of which is SCD. Presentation of case 40 years old male, known to have SCD, he presented as a case of avascular necrosis of the left proximal femur. Upon further investigations the lesion has transformed into undifferentiated pleomorphic sarcoma also known as Malignant Fibrous Histiocytoma (MFH). Discussion The exact mechanism of the malignant transformation of bone infarcts is not fully understood and yet to be investigated. Few cases were reported in literature of similar malignant transformation of a pre-exciting bone infarct and only one was linked to SCD. Conclusion In reporting this case we hope that further cases worldwide will be reported. A high index of suspicion should be present when encountering bone infarct lesion with an unusual course.

We present a very rare case of a laparoscopically-assisted repair of a small bowel perforation secondary to multiple metastases of undifferentiated pleomorphic sarcoma from the posterior mediastinum. A 46-year-old man presented with middle to upper abdominal pain during chemotherapy for lung metastases from undifferentiated pleomorphic sarcoma. Computed tomography revealed intra-abdominal free air, and emergency laparoscopy was performed. Consequently, a perforation was detected in the jejunum, and partial jejunal resection was performed by mini-laparotomy. Pathological examination demonstrated an ulcerated tumor with perforation and four additional tumors in the resected jejunum. Pathological examination also revealed undifferentiated pleomorphic sarcoma in all five tumors. To our knowledge, our case is the first report of a laparoscopically-assisted repair of a small bowel perforation secondary to metastasis of undifferentiated pleomorphic sarcoma. Although the perforation site was unclear preoperatively, laparoscopic observation readily identified the lesion in this patient. Therefore, minimally invasive surgery could be performed successfully with mini-laparotomy following laparoscopic observation. Laparoscopic techniques for the small intestine are viable options, even in acute and uncommon situations, and small bowel perforation secondary to metastasis should be considered in patients with undifferentiated pleomorphic sarcoma and acute abdomen.

Primary UPS of the lung, so‐called MFH, is a rare aggressive neoplasm and known to be a high risk of recurrence and metastasis. Surgical resection without residual tumor is the main option of treatment and the best chance for long‐term survival.

Background: Sarcomas comprise a heterogenous group of malignant tumours of mesenchymal origin and can arise in the skin. Definitive management of skin sarcoma usually entails surgical resection with wide margins, often requiring reconstruction. The incidence and demographics of these neoplasms in Australia are poorly understood. Method: Incidence, gender and age distribution data for skin sarcomas for the period 1982–2009 were obtained from the Australian Cancer Database (ACD). Morphology and topographical region coding via the International Classification of Diseases for Oncology third edition (ICD-O-3)1 were used to identify the data.Results: A total of 5453 cases of skin sarcoma in the Australian population were identified over the 28-year period. Anatomically, 1610 cases (29%) occurred on the limbs, 1416 (26%) on the head and neck area, 957 (18%) on the trunk and 1470 (27%) had an unspecified skin origin. Overall incidence was 2.09 per 100,000 population. Males were more commonly affected (70%), most commonly in the 30–49 years and 70+ years age groups. The most common pathological subtypes were fibromatous sarcoma (including dermatofibrosarcoma protuberans), Kaposi’s sarcoma and pleomorphic dermal sarcoma.Conclusion: The Australian Cancer Database data used to describe the pattern and epidemiological trends for skin sarcoma in Australia demonstrated variation from international trends and highlight the need for further research into the aetiology of these tumours.

Highlights • Think of undifferentiated pleoimorphic sarcoma as a differential in older adults with a history of radiation therapy. • It is important to discover the type and grade of the tumor. • Patients should be thoroughly worked up for the best possible outcome., Introduction An 85 year old male presented to his General Practitioner with a vague history of a lump on his back that was mobile and non tender. He had a previous history of advanced prostate cancer and radiotherapy treatment. Presentation We present an unexpected diagnosis of malignant fibrous histiocytoma of a subcutaneous lesion removed from the patient’s back. Discussion Excisions of subcutaneous lesions along with cutaneous lesions form the majority of cases on a usual minor operations list. All lesions are sent for histopathological analysis and most are confirmed benign sebaceous cysts or lipomas. However the entity of malignant fibrous histiocytoma, now referred to as undifferentiated pleomorphic sarcoma (UPS) should be kept in mind. Patients will usually present late with advanced local disease or metastasis, usually to lungs and lymph nodes. Treatment is surgical with wide local excision and neoadjuvant/adjuvant radiotherapy has been advocated as well. Conclusion Undifferentiated pleomorphic sarcoma should be a differential in subcutaneous lesions in older adults with a previous history of malignancy, radiation therapy and a mass in the subcutaneous tissue of the back.

Introduction and importance Undifferentiated pleomorphic sarcoma (UPS) is one of the most common sarcomas affecting elderly patients, majority of UPS usually in the extremities, trunk, and retroperitoneum. However, its rarely observed in the digestive system. There are minimal data published regarding this topic. Case presentation Here we present a 58 years old female patient with a rare case of the pleomorphic sarcoma of retro-gastric mass measures about (24.7 × 23.5 × 17.4 cm) who underwent exploration laparotomy founded a freely and mobile retro-gastric mass measuring 30 × 20 cm with a cystic and solid component. The mass was freely and mobile located with no true gastric relation is the first one to be reported. Clinical discussion The undifferentiated pleomorphic sarcomas or MFH has been regarded as the most common soft tissue sarcoma for many years (Kabashima et al., 2017). The incidence increases with age, with an inclusive incidence of about 1–2 cases per 100,000 patients per year, predominately 1.2:1 for males. Undifferentiated pleomorphic sarcoma of the stomach is rare and counted to have a worse prognosis when compared to the other types of pleomorphic malignant fibrous histiocytoma. Conclusion We present a rare case of high-grade undifferentiated pleomorphic sarcoma of the retro-gastric mass with no invasion to the surrounding structures, managed with surgical resection of the tumor., Highlights • A rare case of the of retro-gastric sarcoma with no true direct relation to the stomach, the first case reported • Undifferentiated pleomorphic sarcoma is usually reported in the extremities, trunk, and retroperitoneum and observed in the digestive system. • The potential of adjuvant radiotherapy for USP showed no sufficient data was supporting such treatment

We report a case of a 56-year-old man with persistent right upper lobe cavitary mass. A chest computed tomography scan showed about 4-cm-sized mass with internal low attenuation and peripheral enhancement in right upper lobe apical segment. The lesion size increased over 1 month. Right upper lobectomy was performed with the intention to treat the lesion. Pathological examination showed primary pulmonary undifferentiated pleomorphic sarcoma. We describe this rare lung disease to remind that primary pulmonary undifferentiated pleomorphic sarcoma could be the differential diagnosis of pulmonary cavitary mass lesions.

Introduction As a result of the increased use of radiotherapy (RT) and improved long-term overall survival of patients with cancers of the head and neck (HN), the frequency of radiation-induced sarcomas of the head and neck (RISHN) may be increasing. The main objective of this systematic review was to determine the existing evidence on the frequency, treatment, and outcome of RISHN. Methods Using PRISMA guidelines we conducted a systematic review of the literature published from 2000 to 2020. Results Our review includes data of 560 patients from 64 articles. The total frequency of RISHNs among the reviewed series was 0.15%. The most frequent location of the primary tumor treated by RT was the nasopharynx. The mean RT dose used was 62 Gy, mean latency interval between irradiation and occurrence of RISHN was 11.1 years, and the most common RISHN location was the sinonasal region. Osteosarcoma was the principal histology, followed by fibrosarcoma. Surgery was the most frequently applied treatment modality. Of all patients with RISHN, 40.7% died of this disease after a mean interval of 13.9 months. Conclusions Notwithstanding the increased use of RT, the number of reported RISHNs has not increased substantially during the past two decades. Surgery with wide margins forms the best therapeutic option for these cases, but the outcome remains poor.

Introduction As a result of the increased use of radiotherapy (RT) and improved long-term overall survival of patients with cancers of the head and neck (HN), the frequency of radiation-induced sarcomas of the head and neck (RISHN) may be increasing. The main objective of this systematic review was to determine the existing evidence on the frequency, treatment, and outcome of RISHN. Methods Using PRISMA guidelines we conducted a systematic review of the literature published from 2000 to 2020. Results Our review includes data of 560 patients from 64 articles. The total frequency of RISHNs among the reviewed series was 0.15%. The most frequent location of the primary tumor treated by RT was the nasopharynx. The mean RT dose used was 62 Gy, mean latency interval between irradiation and occurrence of RISHN was 11.1 years, and the most common RISHN location was the sinonasal region. Osteosarcoma was the principal histology, followed by fibrosarcoma. Surgery was the most frequently applied treatment modality. Of all patients with RISHN, 40.7% died of this disease after a mean interval of 13.9 months. Conclusions Notwithstanding the increased use of RT, the number of reported RISHNs has not increased substantially during the past two decades. Surgery with wide margins forms the best therapeutic option for these cases, but the outcome remains poor.

Osteopetrosis comprises a rare, heterogeneous group of heritable conditions that are characterized by a defect in bone resorption by osteoclasts. We report the case of a 53-year-old woman with previously undiagnosed osteopetrosis who presented with a pathologic proximal humeral fracture secondary to pleomorphic sarcoma, which is previously undescribed in the English literature. Management of the primary lesion necessitated ablative surgery, but the malignancy nonetheless was associated with rapidly progressive metastatic disease.

Primary malignant fibrous histiocytoma (MFH) of the chest wall is extremely rare and is characterized by aggressive features, including a high incidence of local recurrence and distant metastasis. Surgical resection of the chest wall is the primary modality of management. However, surgical treatment is not generally recommended in patients with evidence of distant metastasis. Here, we present a case of chest wall MFH along with a schwannoma mimicking distant metastasis in the right upper arm. The patient was treated by radical en bloc resection and survived for more than 9 years without recurrence.

We are going to present a case of malignant fibrous histiocytoma in the right atrium, which is a very rare entity. The patient had a right atrial mass, which prolapsed through the tricuspid valve into the right ventricle, causing functional tricuspid valve stenosis. The tumor was completely resected and the patient had an uneventful postoperative period. Histopathological examination reported malignant fibrous histiocytoma. The patient presented to the emergency department five weeks after discharge with dyspnea and palpitation. Echocardiography and magnetic resonance imaging revealed recurrent right atrial tumor mass. His clinical status has worsened, with syncope and acute renal failure. On the repeated echocardiography, suspected tumor recurrence was observed in left atrium, which probably caused systemic embolization. Considering the aggressive nature of the tumor and systemic involvement, our Heart Council decided to provide palliative treatment by nonsurgical management. His status deteriorated for the next few days and the patient succumbed to a cardiac arrest on the 4th day.

Gastrointestinal tract involvement in undifferentiated pleomorphic sarcoma (UPS) is extremely rare. To the authors’ knowledge, only 21 cases of primary small bowel UPS have been reported in the literature available in English. Reported CT findings in primary small bowel UPS have been nonspecific, and MRI findings have been reported in only one case. The present article describes a case involving a 72-year-old male with histologically confirmed primary UPS arising from the ileum, focusing on both CT and magnetic resonance enterography findings. On CT, primary small bowel UPS was noted as a heterogeneously enhanced small bowel mass without obstruction. Magnetic resonance enterography revealed heterogeneous intermediate T1 and T2 signal intensity, with hemorrhagic or necrotic foci within the mass and heterogeneous enhancement. The differential diagnosis included malignant gastrointestinal tumor; however, the prognosis of UPS is worse, with higher incidences of extra-abdominal metastasis.

Malignant fibrous histiocytoma (MFH) is one of the most common types of soft tissue sarcomas in adults. Distant metastases are developed in 30–40% of patients with MFH, with the most common site being the lung. However, metachronous MFH has not been reported previously in literature. This report describes a case of a 30-year-old man, who had two metachronous thigh tumors, both of which were confirmed to be MFH on histopathology and immunohistochemistry evaluations. A contemporary multidisciplinary approach to therapy including surgery, radiation and chemotherapy was advocated. Two primary sites of MFH raised the possibility of a genetic abnormality that could predispose such a patient to develop multiple primary sites of the same tumor.

Malignant fibrous histiocytoma (MFH) is one of the most common types of soft tissue sarcomas in adults. Distant metastases are developed in 30–40% of patients with MFH, with the most common site being the lung. However, metachronous MFH has not been reported previously in literature. This report describes a case of a 30-year-old man, who had two metachronous thigh tumors, both of which were confirmed to be MFH on histopathology and immunohistochemistry evaluations. A contemporary multidisciplinary approach to therapy including surgery, radiation and chemotherapy was advocated. Two primary sites of MFH raised the possibility of a genetic abnormality that could predispose such a patient to develop multiple primary sites of the same tumor.

Malignant fibrous histiocytoma is a type of soft tissue sarcoma that originates from mesenchymal tissues and that mostly affects extremities. Abdominal malignant fibrous histiocytoma usually has a poor prognosis and is rarely seen in the rectum. A 52-year-old female patient presented with rectal bleeding, tenesmus and anemia. She was operated for an obstructive rectal mass and postoperative pathology was reported as malignant fibrous histiocytoma. Herein, we aimed to report a case of malignant fibrous histiocytoma, a rare cause of rectal mass, who was treated with abdominoperineal resection + end colostomy.

An undifferentiated pleomorphic sarcoma (UPS), also known as a malignant fibrous histiocytoma in the past, commonly involves the soft tissue of the extremities and the retroperitoneum. However, a primary UPS of the colon mesentery is very rare. A 69-year-old male patient visited our outpatient department for treatment of an enlarged, palpable mass in the right lower quadrant (RLQ). Computed tomography showed a 15-cm multilobulated, heterogeneous, enhanced mass in the RLQ, which we suspected originated from the colon. He underwent a right hemicolectomy, and the pathologic result was a colon mesenteric UPS. We report a rare case of a primary UPS of the colon mesentery and discuss the characteristics of this neoplasm in reference to the literature.

Undifferentiated pleomorphic sarcoma (UPS) previously described as malignant fibrous histiocytoma (MFH) is a common soft tissue sarcoma, but relatively rare in oral cavity. In this article we report a case of 23-year-old male patient diagnosed UPS in left side of maxilla anterior region which had a deceptive clinical and orthopantomogram findings. Patient had diffuse swelling left upper side of the face with periapical radiolucency involving 21, 22, 23 regions and decreased response to electric pulp testing in those teeth. Patient underwent surgical removal of the entire lesion with wide marginal clearance followed by radiation and chemotherapy. This case report highlights the importance of tissues to be sent for histopathological examination for definitive diagnosis.

Fibrous histiocytoma (FH) commonly occurs in the superficial layers of the skin. Orbit and limbus are documented ophthalmic sites of involvement but isolated corneal FH has never been reported in literature. We present the first case of FH exclusively involving the cornea where a 10-year-old male child presented with a 3-month history of a painless growth on the superior cornea of the right eye with deterioration of vision. Tumor excision with therapeutic penetrating keratoplasty was done and the histopathological examination confirmed the diagnosis. There was no recurrence and the corneal graft was clear at 1 year.

INTRODUCTION: As a result of the increased use of radiotherapy (RT) and improved long-term overall survival of patients with cancers of the head and neck (HN), the frequency of radiation-induced sarcomas of the head and neck (RISHN) may be increasing. The main objective of this systematic review was to determine the existing evidence on the frequency, treatment, and outcome of RISHN. METHODS: Using PRISMA guidelines we conducted a systematic review of the literature published from 2000 to 2020. RESULTS: Our review includes data of 560 patients from 64 articles. The total frequency of RISHNs among the reviewed series was 0.15%. The most frequent location of the primary tumor treated by RT was the nasopharynx. The mean RT dose used was 62 Gy, mean latency interval between irradiation and occurrence of RISHN was 11.1 years, and the most common RISHN location was the sinonasal region. Osteosarcoma was the principal histology, followed by fibrosarcoma. Surgery was the most frequently applied treatment modality. Of all patients with RISHN, 40.7% died of this disease after a mean interval of 13.9 months. CONCLUSIONS: Notwithstanding the increased use of RT, the number of reported RISHNs has not increased substantially during the past two decades. Surgery with wide margins forms the best therapeutic option for these cases, but the outcome remains poor. ispartof: ADVANCES IN THERAPY vol:38 issue:1 pages:90-108 ispartof: location:United States status: published

Malignant fibrous histiocytoma (MFH) is a rare tumor included in the group of soft tissue sarcomas. Approximately 1-3% of these tumors affect the head and neck, with about 100 cases reported to date. A case affecting the retromolar trigone and involving the postero-lateral portion of the left hemi-tongue is reported. Etiological and pathological aspects are discussed. Diagnosis, treatment and prognosis of this neoplasm are reviewed., Le fibro-histiocytome malin est une tumeur rare faisant partie du groupe des sarcomes des tissus mous. Environ 1-3% de ce type de tumeur est localisé dans la région de la tête et cou, dont 100 cas ont été rapportés à ce jour. Le cas rapporté affecte le trigone rétromolaire envahissant la portion postéro-latérale de l’hémi-langue. Les aspects étiologiques et pathologiques sont discutés. Le diagnostic, le traitement et le pronostic sont passés en revue.

We report the clinical observation of acute myocardial infarction development associated with the compression of the left coronary artery trunk and discuss etiological role of primary and secondary cardiac tumors in the development of acute coronary syndrome.

We are going to present a case of malignant fibrous histiocytoma in the right atrium, which is a very rare entity. The patient had a right atrial mass, which prolapsed through the tricuspid valve into the right ventricle, causing functional tricuspid valve stenosis. The tumor was completely resected and the patient had an uneventful postoperative period. Histopathological examination reported malignant fibrous histiocytoma. The patient presented to the emergency department five weeks after discharge with dyspnea and palpitation. Echocardiography and magnetic resonance imaging revealed recurrent right atrial tumor mass. His clinical status has worsened, with syncope and acute renal failure. On the repeated echocardiography, suspected tumor recurrence was observed in left atrium, which probably caused systemic embolization. Considering the aggressive nature of the tumor and systemic involvement, our Heart Council decided to provide palliative treatment by nonsurgical management. His status deteriorated for the next few days and the patient succumbed to a cardiac arrest on the 4th day.

We present a case of spontaneous undifferentiated/unclassified sarcoma, of a pleomorphic subtype formerly known as malignant fibrous histiocytoma (UPS/MFH), arising from the pancreas of a laboratory rat. The mass was excised after laparotomy from a 6‐month‐old female laboratory Wistar rat. It presented a giant multilobulated mass of irregular shape, which had arisen from the pancreas and occupied almost the entire peritoneal cavity. Histologically the tumor was characterized by a highly variable morphological pattern, with frequent transitions from storiform to pleomorphic areas. An extensive immunohistochemical examination revealed no specific lines of differentiation. Immunohistochemical positivity was observed only to MIB‐1 (high Ki‐67 proliferation index), vimentin and CD68 antibodies. The diagnosis was compatible with UPS/MFH. To the best of our knowledge, the present case is the first report of a spontaneous primary UPS/MFH arising from the pancreas of a laboratory rat.

Melanoma-associated antigen 3 (MAGE-A3) expression is generally restricted to the placenta and germline cells of the testis, but it may also be expressed in sarcoma and other cancers and is associated with poor prognosis. Immunotherapy approaches targeting MAGE-A3 in other cancers have shown mixed results in the clinic, however, use of cancer testis antigens such as MAGE-A3 may have therapeutic value in the treatment of soft tissue sarcomas. Based on the recent success of anti-programmed death-1 (PD-1) therapy in undifferentiated pleomorphic sarcoma, we hypothesize that MAGE-A3-based immunotherapies may also provide benefits in this sarcoma type. We analyzed MAGE-A3 expression of sarcoma subtypes available in the Cancer Genome Atlas and Cancer Cell Line Encyclopedia and show that undifferentiated pleomorphic sarcoma/myxofibrosarcoma (UPS/MFS) expresses this potential target gene. We have identified high protein expression by tissue microarray of 106 UPS cores. We also found that high MAGE-A3 mRNA and protein expression is associated with worse overall survival in UPS/MFS. Furthermore, our results show no human leukocyte antigen (HLA) expression loss and relatively high lymphocyte infiltration by lymphocyte specific protein tyrosine kinase (LCK) marker expression. Based on these results, we propose targeting MAGE-A3 in UPS/MFS by immunotherapy techniques.

Highlights • There is no reported evidence in the literature of malignant fibrous histiocytoma having metastasized in pancreas and stomach. • The incidence of Malignant fibrous histiocytoma (MFH) in the mandible is not frequent and incidence risk is only 3% of all MFHs in bones. • Metastasis in extrapulmonary locations are not frequent., Background Malignant fibrous histiocytoma is a sarcoma of uncertain origin that can be found both in soft tissues and in bones. It is currently called undifferentiated pleomorphic sarcoma not otherwise specified and it represents a final common pathway in several tumors that are subject to the progress of dedifferentiation. Local recurrence of the tumor in the same location where it was originated occurs in 20–30% of the total number of soft tissue sarcomas. It is less frequent in limbs and more likely to recur in retroperitoneal sarcomas and in head and neck. Most tumors grow during the first two years after treatment. Most sarcoma metastases, especially those in the head and neck (including malignant fibrous histiocytoma) present as a lung disease (90%). Extrapulmonary diseases are not frequent and can occur in lymph nodes (10%), bones (8%) and in the liver (1%). Case report 61 years old woman with history of malignant fibrous histiocytoma in the right portion of the mandible, resected four years ago. Subsequent checkups did not show any disease. The patient reports a constant abdominal pain not diagnosed, related to a pancreatic nodule. Conclusion Malignant fibrous histiocytoma is a potentially curable disease. The most important part of the treatment is complete surgical removal, usually followed by adjuvant radiation therapy. Chemotherapy is a treatment usually used only on those patients with higher recurrence risk or on those showingrecurrence or who have already showed recurrence.

Malignant soft-tissue tumors which were designated as malignant fibrous histiocytoma are regrouped by the WHO (in 2002) under the new entity termed as “undifferentiated pleomorphic sarcoma.”1 It accounts for less than 5% of all adult soft-tissue sarcomas. Here, we report the lesion in a 70-year-old man who presented with high-grade undifferentiated pleomorphic sarcoma in the lower extremity.

ΔΕΝ ΔΙΑΤΙΘΕΤΑΙ ΠΕΡΙΛΗΨΗ, In this report, a case of malignant fibrous histiocytoma in a budgerigar (Melopsittacus undulatus) is described. At necropsy, a tumorous subcutaneous mass was noted to be located under the right wing at the level of humerus in a budgerigar. On microscopic examination, the tumor was composed of pleomorphic cells having mostly round to oval large nucleus. Presence of occasional bands composed of fibroblastoid cells were noted. Occasional giant cells and mitotic figures were also observed. Based on the microscopic findings, a diagnosis of malignant fibrous histiocytoma was made. The present case in an exotic bird was found to be interesting, since malignant fibrous histiocytomas are rare tumors of domestic animals and mostly seen in dogs.

With the improvement in computer graphics and sensors, technologies like virtual reality (VR) and augmented reality (AR) have created new possibilities for developing diagnostic and surgical techniques in the field of surgery. VR and AR are the latest technological modalities that have been integrated into clinical practice and medical education, and are rapidly emerging as powerful tools in the field of maxillofacial surgery. In this report, we describe a case of total maxillectomy and orbital floor reconstruction in a patient with malignant fibrous histiocytoma of the maxilla, with preoperative planning via VR simulation and AR-guided surgery. Future developments in VR and AR technologies will increase their utility and effectiveness in the field of surgery.

Pleomorphic hyalinizing angiectatic tumor (PHAT) is a rare soft tissue tumor of uncertain histogenesis that was initially described in 1996. The tumor has a strong predilection for the subcutaneous soft tissue of the distal extremities with handful number of cases reported in other locations. Here, we report a case of PHAT involving the breast soft tissue of a 61-year-old man, who presented with a painless left breast mass slowly growing over the period of 4 years. Core needle biopsy was performed to rule out malignancy. The biopsy showed several ectatic hyalinized blood vessels, surrounded by mitotically inactive spindled to pleomorphic cells embedded in a fibromyxoid stroma. The tumor had strong immunohistochemical expression of CD34, CD99 and Bcl2, while being negative for S100, cytokeratin, actin and desmin. The features were consistent with PHAT. This was followed by a wide local excision with no documented recurrence or metastasis 36 months after surgery. This case is reported to increase the awareness of this entity to be considered in the differential diagnosis of pleomorphic and spindle cell tumors of the breast seen on core biopsies to avoid misdiagnosis and unnecessary radical surgical procedures.

Key Clinical Message Undifferentiated high‐grade pleomorphic sarcoma is a slow‐growing tumor rarely localized in the head and neck region. The treatment of UHPS should be based on large surgical resections in free margins associated with neck dissection. Postoperative radiotherapy improves local control of the disease and the prognosis quod vitam.

Background Malignant fibrous histiocytoma has been uncommonly described in dogs. Several extranasal neoplasias have been reported to result hypertensive epistaxis. There are, however, no published case reports of extranasal malignant fibrous histiocytoma with concurrent hypertension and epistaxis in dogs. Case presentation A 10-year-old dog presented with a spontaneous massive epistaxis persisting for 5 days. The dog exhibited unstable hypertension, which was considered as a cause of epistaxis. The complete blood count, prothrombin time, and activated partial thromboplastin time were within the reference limits, and other systemic examination showed no abnormalities except for a splenic mass occupying more than one third of the abdomen. Histologic examination of the resected spleen revealed the characteristic features of a malignant fibrous histiocytoma. One week after splenectomy, the hypertension and epistaxis resolved clinically and did not recur on the 5-month follow-up. Conclusions The dog’s blood pressure and epistaxis normalized after malignant fibrous histiocytoma resection suggesting that hypertensive epistaxis may be a rare manifestation of canine malignant fibrous histiocytoma.

A3, generated as a monoclonal antibody against rat malignant fibrous histiocytoma (MFH)-derived cloned cells, recognizes somatic stem cells (bone-marrow/hair follicle stem cells). We investigated the distribution of cells immunoreactive to A3 in the developing rat intestine (particularly, the colon), focusing on the ontogenic kinetics of A3-positive cells. In the rat intestine, A3 labeled spindle-shaped stromal cells localized in the submucosa and labeled endothelial cells of capillaries in the lamina propria forming villi in the early development stage. With development progression, A3-positive cells were exclusively localized around the crypts of the colon. Double immunofluorescence revealed that A3-positive cells around the crypts reacted to vimentin (for mesenchymal cells) and Thy-1 (for mesenchymal stromal cells) but not to α-SMA (for mesenchymal myofibroblastic cells) or CD34 (for hematopoietic stem cells), indicating that A3-positive cells around the crypts may have characteristics of immature mesenchymal cells. In addition, A3 labeled a few epithelial cells at the base of colon crypts. Furthermore, immunoelectron microscopy revealed that A3-positive cells lay inside myofibroblasts adjacent to the epithelium of the crypts. A3-positive cells were regarded as a new type of immature mesenchymal cells around the crypts. Collectively, A3-positive cells might take part in the stem cell niche in the colon, which is formed through epithelial-mesenchymal interaction.

Guanghui Ji,1 Liu Hong,2 Ping Yang3 1Department of Traditional Chinese Medicine, 2Department of Pathology, 3Department of Oncology, Navy General Hospital, Beijing, People’s Republic of China Abstract: Malignant fibrous histiocytoma (MFH) is the most common soft-tissue sarcoma in late adult life. Unfortunately, advanced MFH has a poor prognosis due to a lack of effective drugs. We present here a case of advanced MFH with partial response to apatinib, a new potent oral small-molecule tyrosine kinase inhibitor targeting the intracellular domain of vascular endothelial growth factor receptor 2 (VEGFR-2). To the best of our knowledge, this is the first case report using apatinib for MFH. Quantitative polymerase chain reaction analysis revealed high expression of VEGFR-2 mRNA, suggesting that apatinib leads to clinical response by inhibiting VEGFR-2 tyrosine kinase activity and the crucial role of VEGFR-2 for MFH. Apatinib could be a new option for the treatment of MFH. Further studies are needed to optimize the treatment. Keywords: malignant fibrous histiocytoma, apatinib, chemotherapy, angiogenesis inhibitor, targeted therapy

Malignant fibrous histiocytoma (MFH) is the most common soft-tissue sarcoma in late adult life. Unfortunately, advanced MFH has a poor prognosis due to a lack of effective drugs. We present here a case of advanced MFH with partial response to apatinib, a new potent oral small-molecule tyrosine kinase inhibitor targeting the intracellular domain of vascular endothelial growth factor receptor 2 (VEGFR-2). To the best of our knowledge, this is the first case report using apatinib for MFH. Quantitative polymerase chain reaction analysis revealed high expression of VEGFR-2 mRNA, suggesting that apatinib leads to clinical response by inhibiting VEGFR-2 tyrosine kinase activity and the crucial role of VEGFR-2 for MFH. Apatinib could be a new option for the treatment of MFH. Further studies are needed to optimize the treatment.

Objectives: Preoperative chemotherapy plays a key role in management of bone sarcomas. Postoperative evaluation of histological necrosis has been the gold standard method of assessing response to preoperative chemotherapy. This study was done to evaluate the efficacy of static and dynamic magnetic resonance imaging (MRI) for assessing response preoperatively. Materials and Methods: Our study included 14 patients (12 osteosarcomas and 2 malignant fibrous histiocytomas) with mean age of 21.8 years, treated with preoperative chemotherapy followed by surgery. They were evaluated with static and dynamic MRI twice, before starting chemotherapy and again prior to surgery. Change in tumor volume and slope of signal intensity - time curve were calculated and correlated with percentage of histological necrosis using Pearson correlation test. Results: The change in dynamic MRI slope was significant (P = 0.001). Also, ≥60% reduction in slope of the curve proved to be an indicator of good histological response [positive predictive value (PPV) =80%]. Change in tumor volume failed to show significant correlation (P = 0.071). Although it showed high negative predictive value (NPV = 85.7%), PPV was too low (PPV = 57.14%). Conclusions: Dynamic MRI correctly predicts histological necrosis after administration of preoperative chemotherapy to bone sarcomas. Hence, it can be used as a preoperative indicator of response to neoadjuvant chemotherapy. On the other hand, volumetric assessment by static MRI is not an effective predictor of histological necrosis. This study proves the superiority of dynamic contrast-enhanced study over volumetric study by MRI.

Resumo: Neoplasmas em suínos são raros. Esse trabalho descreve os neoplasmas encontrados em suínos na rotina diagnóstica de um laboratório de patologia veterinária localizado na Região Central do Rio Grande do Sul. Durante um período de 49 anos, 2.266 casos de várias afecções em suínos foram encontrados, dos quais 37 (1,6%) eram neoplasmas. Em ordem decrescente de frequência, os seguintes neoplasmas foram encontrados: Linfoma (11/37), nefroblastoma (11/37), melanoma (8/37) e papiloma (2/37). Adicionalmente, um caso de cada um dos seguintes tumores foi observado: Adenoma hepatocelular, carcinoma hepatocelular, colangiocarcinoma, histiocitoma fibroso maligno e sarcoma granulocítico. O aspecto macroscópico e histológico desses tumores é descrito e a sua epidemiologia é comparada com os dados disponíveis na literatura para neoplasia suína. Abstract: Neoplasms in swine are rare. This paper describes neoplasms found in swine in the diagnostic routine of a veterinary pathology laboratory in the Central Region of Rio Grande do Sul, Brazil during a 49-year period, during which 2,266 cases of the various affections in swine were diagnosed. Of those 37 cases (1.6%) were neoplasms. In decreasing order of prevalence, the following neoplasms were found: lymphoma (11 out of 37 cases), nephroblastoma (11/37), melanoma (8/37), and papilloma (2/37). Adenoma hepatocelular, carcinoma hepatocelular, cholangiocarcinoma, malignant fibrous histiocytoma, granulocytic sarcoma were each found in one case out of the 37 cases. The gross and histological aspects of these tumors are described and their epidemiology is compared with the data available in the literature for neoplasia in swine.

A 69-year-old male, treated for colonic carcinoma 15 years back, presented to our services with status epilepticus. He had complaints of headache and vomiting for 7 days prior to presentation. Computed tomography and magnetic resonance imaging of the brain revealed heterogeneous, lobulated and osteolytic tumor involving middle and posterior cranial fossa. Excision of the tumor was planned in two stages. Middle fossa component was removed through right temporobasal approach. Histopathology was suggestive of malignant fibrous histiocytoma. Patient succumbed to pneumonia and septicemia in the postoperative period. Pathology, clinical features and therapeutic challenges of this clinical entity have been briefly reviewed.

Undifferentiated pleomorphic sarcoma, known as malignant fibrous histiocytoma, is a malignant neoplasm that arises in both soft tissue and bones. In 2002, the World Health Organization declassified malignant fibrous histocytoma as a formal diagnostic entity and renamed it 'undifferentiated pleomorphic sarcoma not otherwise specified.' It most commonly occurs in the lower extremities and rarely metastasizes cutaneously. We report a case of cutaneous metastatic undifferentiated pleomorphic sarcoma of the buttocks occurring in a 73-year-old man diagnosed with mediastinal sarcoma 4 years previously. He first noticed the mass approximately 2 months previously. Histological findings with immunomarkers led to a final diagnosis of cutaneous metastatic sarcoma from mediastinal undifferentiated pleomorphic sarcoma.

Malignant fibrous histiocytoma which was once considered as a tumor of adults, is now being re-classified and subtyped either as a pleomorphic undifferentiated sarcoma or pleomorphic variant of other spindle cell tumors. Only less than 5% of these cases are reported in the head and neck, and its presentation intra-orally is still rarer. Herein, we report a case of gingival swelling in relation to the 16 region, which on microscopic examination showed a histomorphometric pattern of storiform pleomorphic-malignant fibrous histiocytoma. Immunohistochemical studies were done to exclude similar appearing spindle cell neoplasms. The tumor showed focal positivity for pan histiocytic marker, CD68. Based on the histopathological and immunohistochemical profiles, the tumor was diagnosed as undifferentiated pleomorphic sarcoma.