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Background: Undifferentiated pleomorphic sarcoma, previously called malignant fibrous histiocytoma, is a type of malignant mesenchymal tumor (sarcoma) of soft tissue and sometimes bone. It is uncommon in the oral cavity and very sporadic in the maxillary sinus. Microscopic diagnosis of this malignancy in the maxillary sinus can be very challenging, because there is a range of features that may overlap with other benign and malignant tumors. Case presentation: In this paper, we report a case of undifferentiated pleomorphic sarcoma in the maxillary sinus of a 61-year-old Iranian man who was referred to the maxillofacial surgery ward due to pain and swelling of the upper jaw and visual problems in the right eye. In the initial incisional biopsy, peripheral giant cell granuloma was diagnosed in the hospital service. Yet, on request of the surgeon, during reviewing the slides in the oral pathology service of the School of Dentistry, and using an immunohistochemical method, undifferentiated pleomorphic sarcoma was diagnosed. In this paper, a case of undifferentiated pleomorphic sarcoma in the maxillary sinus is reported, with an emphasis on the management of its problems and diagnostic errors. Conclusion: This study reviews the challenges and histopathological diagnostic errors of this uncommon tumor in this rare location. This lesion may be similar to other malignant tumors, and its correct diagnosis requires a detailed and complete examination. [ABSTRACT FROM AUTHOR]

Undifferentiated pleomorphic sarcoma (UPS) is a rare type of tumor, and UPS originating in the adrenal gland is even rarer. Up to now, there have been no reports in English literature of UPS originating from the adrenal gland. This case report presents a 44-year-old female patient with UPS of the adrenal gland, who has shown no signs of recurrence or metastasis half a year after undergoing resection of a left adrenal tumor. A retrospective analysis of the patient's diagnosis and treatment process is conducted, with the aim of providing a reference for the diagnosis and treatment of adrenal UPS. [ABSTRACT FROM AUTHOR]

Undifferentiated pleomorphic sarcoma (UPS) is an aggressive soft tissue sarcoma with a poor prognosis. The patients are usually found to have metastasis when the primary tumor is diagnosed. Eccrine syringofibroadenoma (ESFA) is a rare cutaneous adnexal lesion of eccrine duct origin. There are five subtypes, one of which is reactive ESFA, known to occur in reaction to an inflammatory or neoplastic process. In this article, we report a case of the co-existence of both UPS and ESFA in a 70-year-old male patient, presenting with a painless, erythematous, irregular surface nodule with a peripherally extended brownish hyperkeratotic plaque on the right palm. The histologic findings revealed an ill-defined dermal tumor of atypical epithelioid and spindle-shaped cells with large pleomorphic hyperchromatic nuclei and abundant eosinophilic cytoplasm. Some of those cells were multinucleated giant cells in the stroma with vascular proliferation and mixed inflammatory cell infiltrate. The tumor cells, which were only positive for vimentin, supported the diagnosis of undifferentiated pleomorphic sarcoma (UPS). Meanwhile, the overlying epidermis demonstrated hyperkeratosis, papillated epidermal hyperplasia, and proliferation of anastomosing slender cords and strands of cuboid cells within loose fibrovascular stroma. These findings are the characteristics of eccrine syringofibroadenoma (ESFA). We describe here a patient in whom reactive ESFA occurred on and surrounded the UPS tumor. [ABSTRACT FROM AUTHOR]

Malignant fibrous histiocytoma (MFH), while rare, is the most prevalent soft tissue sarcoma (STS) found in extremities. Its prognosis hinges on factors such as tumor size, invasion depth, and differentiation degree. For MFH and all STSs, wide surgical resection is the gold standard treatment method. In this study, a 48-year-old woman, initially diagnosed with lipoma and with a mass excised from her right forearm, later exhibited recurring MFH masses in the same location. Superficial tissue ultrasonography identified malignant soft tissue tumors in the lesion area. Positron emission tomography was used to stage the tumor preoperatively, revealing fluorodeoxyglucose uptake in the repeating lesions. Right forearm magnetic resonance imaging confirmed the malignant lesions. Post imaging, the patient underwent wide surgical excision, eliminating the fascia. Regular follow-up revealed no recurrence or metastasis at 6 months and 1 year. Excisions frequently occur without radiological imaging, based only on a preliminary lipoma or sebaceous cyst diagnosis. However, excisions should follow radiological imaging, particularly when soft tissue tumors beneath the skin appear benign. [ABSTRACT FROM AUTHOR]

Undifferentiated pleomorphic sarcoma (UPS) formerly known as malignant fibrous histiocytoma is a high-grade aggressive soft-tissue sarcoma. UPS is a soft-tissue sarcoma without a definable line of differentiation. Men between the ages of 50 and 70 are frequently affected by it. In adults, the lower extremity and retroperitoneum are the most affected sites, while the paranasal sinus is affected in younger individuals. A 16-year-old male patient reported with swelling in his left posterior maxilla for 2 months. The swelling began quickly and gradually expanded to its current size. On examination, an ill-defined, deep-seated exophytic, ovoid growth was seen on the left maxillary alveolus. Histopathological findings indicated UPS. UPS is a rare malignancy that typically originates in the body’s soft tissues. The treatment for UPS is determined on the site of the cancer, but it typically includes surgery and radiation.

Undifferentiated pleomorphic sarcoma (UPS) is a rare type of tumor, and UPS originating in the adrenal gland is even rarer. Up to now, there have been no reports in English literature of UPS originating from the adrenal gland. This case report presents a 44-year-old female patient with UPS of the adrenal gland, who has shown no signs of recurrence or metastasis half a year after undergoing resection of a left adrenal tumor. A retrospective analysis of the patient’s diagnosis and treatment process is conducted, with the aim of providing a reference for the diagnosis and treatment of adrenal UPS.

Primary malignant fibrous histiocytoma (MFH) is a malignant tumor of mesenchymal origin that rarely occurs in the urinary tract, particularly in the urinary bladder. Unlike urothelial carcinoma, which accounts for most bladder cancers, it occurs in the submucosal portion of the bladder wall and consists of the lamina propria, muscularis propria, and adventitia. It is presumed to originate from poorly differentiated pluripotent mesenchymal cells in which fibroblasts and histiocytes are partially differentiated. Radiologically, it is known as the “non-papillary tumor” and is commonly diagnosed as a large mass without necrosis, which shows invasion beyond the muscularis propia. Although the prognosis of this rare malignancy depends on pathological parameters, it generally has a poor prognosis with high local tumor recurrence. Here, we present a case of primary MFH in the urinary bladder with clinical symptoms of lower abdominal pain without gross hematuria that recurred rapidly and showed an aggressive disease course.

Malignant fibrous histiocytoma (MFH), now termed undifferentiated pleomorphic sarcoma (UPS), is a high-grade tumour, mostly affecting the extremities and retroperitoneum. It is a rare entity in the mandible. After a literature search in the PubMed database, only 15 cases have been reported, indicating how uncommon it is in the mandible. In this instance, we document an unusual occurrence of MFH, affecting the mandible in a 37-year-old male patient. Histopathology of the biopsy specimen along with the strong positivity to immunohistochemical markers such as Vimentin and CD68 confirmed the diagnosis of MFH. It is a rare entity presenting diagnostic and therapeutic challenges requiring a multidisciplinary approach for optimal management and treatment. [ABSTRACT FROM AUTHOR]

Undifferentiated pleomorphic sarcoma (UPS) formerly known as malignant fibrous histiocytoma is a high-grade aggressive soft-tissue sarcoma. UPS is a soft-tissue sarcoma without a definable line of differentiation. Men between the ages of 50 and 70 are frequently affected by it. In adults, the lower extremity and retroperitoneum are the most affected sites, while the paranasal sinus is affected in younger individuals. A 16-year-old male patient reported with swelling in his left posterior maxilla for 2 months. The swelling began quickly and gradually expanded to its current size. On examination, an ill-defined, deepseated exophytic, ovoid growth was seen on the left maxillary alveolus. Histopathological findings indicated UPS. UPS is a rare malignancy that typically originates in the body’s soft tissues. The treatment for UPS is determined on the site of the cancer, but it typically includes surgery and radiation. [ABSTRACT FROM AUTHOR]

We report a case of a 54-year-old female who presents with a gradually expanding mass at the right lateral malleolus. The diagnosis of undifferentiated pleomorphic sarcoma was made after a histopathological examination of the mass following a wide tumor excision. The defected soft tissue area was reconstructed using a local flap, reverse sural artery flap. Following the surgical management, multiple radiotherapy sessions were completed. The patient's follow-up result showed no signs of local recurrence or metastasis, and the wound was well-healed with no complications other than paresthesia in a small area at the posterolateral aspect of the ankle under the lateral malleolus. This case represents a rare form of malignant neoplasm and emphasizes the effectiveness and safety of the reverse sural artery flap reconstruction technique, especially in places where microsurgery is unavailable or when the patient's status does not allow for prolonged anesthesia. [ABSTRACT FROM AUTHOR]

The soft tissue lesions around the knee range from congenital, traumatic, and vascular to neoplastic in aetiology. The various imaging modalities employed for diagnostic work-up include plain radiographs, ultrasonography, Computed Tomography (CT) scan, and Magnetic Resonance Imaging (MRI). Based on the peculiar imaging findings, the differential diagnosis can be narrowed down; however, the final diagnosis relies on histopathological findings. Some of the highly vascular lesions are arteriovenous malformation, malignant fibrous histiocytoma, haemangiopericytoma, and synovial sarcoma. The abundant neovascularity within these lesions poses a major problem during surgery due to potential blood loss. Presurgical interventional management in the form of endovascular embolisation significantly reduces intraoperative blood loss. Here, four different cases, one of each type, presenting as highly vascular lesions around the knee joint, were successfully managed with endovascular embolisation.

Abstract Background Undifferentiated pleomorphic sarcoma is an uncommon sarcoma and its presence in the spleen is even rarer, with only a handful of cases reported in English literature. It is typically only diagnosed following histological analysis. Its rarity also means that there is little consensus over ideal management. Case presentation This report presents a case of a 40-year-old Caucasian male who was found to have a splenic mass after presenting with non-specific abdominal pain and generalized malaise. Numerous imaging modalities were used which demonstrated a large partially solid and partially cystic lesion in spleen with no evidence of metastasis. As core biopsies were undiagnostic, he was planned for a diagnostic and therapeutic splenectomy. However, despite magnetic resonance imaging 11 days prior to his operation showed no evidence of liver metastasis, a massive splenic tumour with hepatic metastases was identified intraoperatively. An open splenectomy, distal pancreatectomy and liver metastasectomy was hence carried out. Histological analysis confirmed liver metastasis secondary to a splenic undifferentiated pleomorphic sarcoma. The patient recovered well and was discharged home. He presented again three weeks after his operation with lower back pain, abdominal pain and fever. Computed tomography demonstrated extensive recurrent disease burden in the peritoneum and liver. The patient passed away a month after surgery. Conclusion Splenic undifferentiated pleomorphic sarcoma is a rare tumour which may pose a significant diagnostic challenge on both clinical and histopathological grounds. Following diagnosis and treatment, its aggressive nature often results in a poor prognosis. Current literature fails to delineate any superior management strategy to increase survival.

Histiocytic sarcoma is a malignant proliferation of cells that exhibit morphological and immunophenotypic features of mature histiocytes. Owing to its rarity, its clinical features and standard treatment have not yet been established. This report describes a case of histiocytic sarcoma of the palate that developed in a 76-year-old man, the first report of an intraoral histiocytic sarcoma. An extended resection was performed; however, establishing the excision line was extremely difficult because assessing the tumour boundary on imaging was challenging and the tumour underwent dynamic gross morphological changes following biopsy. Complete resection is required to obtain a favourable prognosis for high-grade tumours with indistinct borders. In this case, an intraoperative rapid examination with frozen section analysis was performed along the planned excision line to completely resect the tumours exhibiting such behaviour. At 28 months postoperatively, the patient demonstrated no recurrence or metastasis; however, he is under careful monitoring. [ABSTRACT FROM AUTHOR]

Malignant transformation of chronic burn scars are usually toward cutaneous lineages, including squamous cell carcinoma, basal cell carcinoma, and malignant melanoma. Sarcomas are less common. Undifferentiated pleomorphic sarcoma(UPS) is a subtype of soft tissue sarcoma with storiform-pleomorphic cells of uncertain origin, and has sparingly been reported to arise from burn scars. The majority are localized lesions probably due to the spatial distance of mesenchymal cells from the epithelium. The authors describe a rare case of UPS of the chronic burn scar of his knee with ipsilateral femoral and external iliac lymph node metastasis. [ABSTRACT FROM AUTHOR]

The soft tissue lesions around the knee range from congenital, traumatic, and vascular to neoplastic in aetiology. The various imaging modalities employed for diagnostic work-up include plain radiographs, ultrasonography, Computed Tomography (CT) scan, and Magnetic Resonance Imaging (MRI). Based on the peculiar imaging findings, the differential diagnosis can be narrowed down; however, the final diagnosis relies on histopathological findings. Some of the highly vascular lesions are arteriovenous malformation, malignant fibrous histiocytoma, haemangiopericytoma, and synovial sarcoma. The abundant neovascularity within these lesions poses a major problem during surgery due to potential blood loss. Presurgical interventional management in the form of endovascular embolisation significantly reduces intraoperative blood loss. Here, four different cases, one of each type, presenting as highly vascular lesions around the knee joint, were successfully managed with endovascular embolisation. [ABSTRACT FROM AUTHOR]

Background: Undifferentiated pleomorphic sarcoma is an uncommon sarcoma and its presence in the spleen is even rarer, with only a handful of cases reported in English literature. It is typically only diagnosed following histological analysis. Its rarity also means that there is little consensus over ideal management. Case presentation: This report presents a case of a 40-year-old Caucasian male who was found to have a splenic mass after presenting with non-specific abdominal pain and generalized malaise. Numerous imaging modalities were used which demonstrated a large partially solid and partially cystic lesion in spleen with no evidence of metastasis. As core biopsies were undiagnostic, he was planned for a diagnostic and therapeutic splenectomy. However, despite magnetic resonance imaging 11 days prior to his operation showed no evidence of liver metastasis, a massive splenic tumour with hepatic metastases was identified intraoperatively. An open splenectomy, distal pancreatectomy and liver metastasectomy was hence carried out. Histological analysis confirmed liver metastasis secondary to a splenic undifferentiated pleomorphic sarcoma. The patient recovered well and was discharged home. He presented again three weeks after his operation with lower back pain, abdominal pain and fever. Computed tomography demonstrated extensive recurrent disease burden in the peritoneum and liver. The patient passed away a month after surgery. Conclusion: Splenic undifferentiated pleomorphic sarcoma is a rare tumour which may pose a significant diagnostic challenge on both clinical and histopathological grounds. Following diagnosis and treatment, its aggressive nature often results in a poor prognosis. Current literature fails to delineate any superior management strategy to increase survival. [ABSTRACT FROM AUTHOR]

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.

Vulvar sarcomas are one of the rarer female genital tract malignancies, characterized by nonspecific signs and symptoms often leading to clinical misdiagnosis which increases the morbidity and mortality. It presents in 5th to 6th decade of life. Undifferentiated pleomorphic sarcomas (UPS) previously known as malignant fibrous histiocytoma (MFH) are the rarest of vulval sarcomas and hence least suspected initially. It is advised to keep vulvar sarcomas as a clinical differential in cases of non specific vulval swellings in order to establish an early accurate diagnosis to expedite its management. We present an unusual and rare case which was clinically misdiagnosed as a benign vulval swelling in a 26 year old married female who presented with painless vulval swelling for 1 year. It was provisionally diagnosed as malignant mesenchymal neoplasm on fine needle aspiration cytology which later was confirmed as UPS on after evaluating a judicious panel of immunohistochemistry markers where a myogenic, vascular and neurogenic origin was ruled out. Patient was treated with wide local excision and adjuvant platinum based chemotherapy which showed good response with no signs of reoccurrence or metastasis in a follow up period of 6 months.

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.

Undifferentiated pleomorphic sarcoma is a common soft tissue sarcoma. Unfortunately, any attempt to describe the line of differentiation fails. It represents a final common pathway in tumors that undergoes progression towards dedifferentiation. We report a man with an undifferentiated pleomorphic sarcoma presenting as an exophytic pedunculated tumor of the left scapula. Histopathology analysis revealed spindle-shaped cells with great pleomorphism and numerous mitoses. Immunohistochemistry showed diffuse expression of vimentin. Wide local excision was performed after an oncology consultation. After two-years of follow-up, the patient has shown no evidence of recurrence or metastases.

Atypical fibroxanthoma (AFX) is a rare cutaneous fibrohistiocytic tumor that typically arises on chronically sun-damaged skin, such as the head and neck, as a nondescript ulcerated papule, nodule, or tumor. The clinical prognosis is usually favorable and metastasis is rare. Pleomorphic dermal sarcoma (PDS), or undifferentiated pleomorphic sarcoma, is a recently introduced diagnostic moniker for AFX-like tumors with more aggressive clinical and histologic features such as necrosis and vascular invasion. The exact relationship between AFX and PDS has been debated. Diagnosis of these tumors is generally based on immunohistochemical staining to exclude other mimics. A wholly specific marker for this tumor does not exist, leading to diagnostic ambiguity in certain cases. Herein, we present a case of pleomorphic dermal sarcoma in a 53-year-old man with human immunodeficiency virus that displayed patchy S100 staining concerning for melanoma upon hospital pathology review. Next-generation sequencing analysis confirmed a mutation pattern consistent with published molecular signatures of AFX/PDS. In discussing this case, we review the current understanding of AFX/PDS and discuss diagnostic pitfalls, as well as emphasize on how next-generation sequencing techniques might improve accuracy in the diagnosis of tumors in the spectrum of AFX/PDS.

Abstract Background Undifferentiated pleomorphic sarcoma (UPS), also known as malignant fibrous histiocytoma (MFH), hardly originates from the colorectum. Case presentation We reported a 65-year-old female presented with UPS in the descending colon. Computed tomography (CT) revealed an irregularly thickened descending colon. On colonoscopy examination, an ulcerative tumour was identified. The patient received radical resection of the left colon and partial enterectomy. The resected tumor was ulcerative, 10 cm × 8 cm × 5 cm in size, and infiltrated the serosa layer. Postsurgical pathology showed that the tumor was high-graded UPS in the colon with large amounts of necrotic tissues. Conclusions UPS in the large intestine is a rare malignant tumor with a poor prognosis and unknown pathogenesis. The main treatment for UPS is early complete resection. Postsurgery adjuvant radiotherapy or chemotherapy can be attempted.

Undifferentiated pleomorphic sarcoma (UPS) previously called as malignant fibrous histiocytoma comprises a group of high-grade pleomorphic sarcomas that cannot be otherwise classified and considered as a diagnosis of exclusion. In the head neck region, UPS is extremely rare and accounts for 3% of all the undifferentiated pleomorphic sarcomas. Some of the reported sites include maxilla, mandible, buccal mucosa, temperomandibular fossa, tongue, gingiva, paranasal sinuses, salivary glands, and retro-orbital soft tissue. Undifferentiated pleomorphic sarcoma of the floor of the mouth is very rare. To our knowledge, only one case has been reported earlier. We report the second case of undifferentiated pleomorphic sarcoma of the floor of mouth. [ABSTRACT FROM AUTHOR]

Introduction: Undifferentiated sarcomas of the gastrointestinal tract are rare and have poor prognoses, especially those with lymph node metastases. There is no consensus on the treatment plan. While there are reports on undifferentiated pleomorphic sarcomas of the rectum, no reports on undifferentiated rectal spindle sarcomas with lymph node metastases have been presented previously., Case Presentation: We report a case of a 97-year-old woman referred to our hospital with anal pain. Imaging findings indicated multiple tumors in the rectum below the peritoneal reflection protruding from the anus and two enlarged pararectal lymph nodes. Laparoscopic abdominoperineal resection of the rectal sarcomas with lymph node metastasis was performed to alleviate the pain with uneventful postoperative courses. The immunostaining did not reveal a trend of tumor cell differentiation. The tumor was diagnosed as undifferentiated spindle cell sarcoma based on histopathological findings. Because of advanced age, the patient is followed up on an outpatient basis without additional postoperative treatment., Conclusion: The prognosis of undifferentiated sarcomas is poor. While radical resection is the primary treatment, the efficacy of preoperative radiation therapy, cytotoxic chemotherapy, and immune checkpoint inhibitors has been investigated recently. Accumulating cases of this disease is important to determine treatment plans, and this report is valuable in this regard., Competing Interests: The authors declare no conflict of interest for this article., (© 2025 The Author(s). Published by Japan Surgical Society.)

Primary sarcomas are the rarest among primary malignant splenic neoplasms. Undifferentiated pleomorphic sarcoma (UPS), described in the past as malignant fibrous histiocytoma (MFH) of spleen is an extremely rare soft tissue malignant neoplasm. Due to non-specific symptoms, delayed presentation and aggressive nature of the tumour, overall prognosis is poor. We report the management experience of a case of splenic UPS of a 52-year-old Sri Lankan female who presented with epigastric fullness and discomfort. Initial clinical and ultrasonographic impression was of a large complex cystic lesion probably arising from the spleen or distal pancreas. Computed tomography confirmed the lesion to origin from the spleen. Due to spiking temperature and rising inflammatory markers, clinical suspicion was made of a splenic abscess related to the complicated cyst. At the laparotomy, a large predominantly cystic mass with concealed leaks arising from the spleen was noted containing inflammatory material within cyst cavities. The mass was adherent to the stomach and diaphragm. The patient underwent splenectomy with an uncomplicated post-operative period. Histology confirmed the lesion to be a UPS of the spleen and the patient was scheduled for a staging whole-body computed tomography. Unfortunately, she defaulted follow-up after 4 weeks from surgery and got readmitted as an emergency after 3 months with abdominal pain, distention and constitutional symptoms complicated with acute kidney injury. Ultrasonography proved her to have massive early local tumour recurrence and she succumbed due to the illness. This is the first reported case of UPS of the spleen in Sri Lanka. [ABSTRACT FROM AUTHOR]

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).

We present the case of a 69-year-old non-smoker male who came to the hospital for dyspnea, thoracic pain, hemoptysis, loss of appetite and involuntary weight loss, evolving for two months. The thorax-CT scan revealed a giant lung tumor involving the right upper lobe and part of the middle lobe, with pleural invasion. The patient was known with an osteosarcoma (malignant fibrous histiocytoma-like; MFHlike) of the spine surgically removed 10 years ago and treated with chemotherapy. The initial biopsy of the tumor that invaded the anterior parietal pleura was not conclusive, this fact requiring a surgical intervention with both a diagnostic and therapeutic role. Thus, superior bilobectomy was performed, which was associated with a complete right mediastinal lymph node dissection. The postoperative course was favorable and uneventful. The histopathological findings and immunohistochemistry staining revealed a giant lung osteosarcoma metastasis, MFH-like subtype, and no metastasis in the mediastinal lymph nodes. The peculiarity of the case is represented by the evolution of a single osteosarcoma metastasis with very large dimensions after more than 10 years. [ABSTRACT FROM AUTHOR]

Background: Undifferentiated pleomorphic sarcoma (UPS), also known as malignant fibrous histiocytoma (MFH), hardly originates from the colorectum.Case Presentation: We reported a 65-year-old female presented with UPS in the descending colon. Computed tomography (CT) revealed an irregularly thickened descending colon. On colonoscopy examination, an ulcerative tumour was identified. The patient received radical resection of the left colon and partial enterectomy. The resected tumor was ulcerative, 10 cm × 8 cm × 5 cm in size, and infiltrated the serosa layer. Postsurgical pathology showed that the tumor was high-graded UPS in the colon with large amounts of necrotic tissues.Conclusions: UPS in the large intestine is a rare malignant tumor with a poor prognosis and unknown pathogenesis. The main treatment for UPS is early complete resection. Postsurgery adjuvant radiotherapy or chemotherapy can be attempted. [ABSTRACT FROM AUTHOR]

Abstract 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.

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.

Malignant fibrous histiocytoma (MFH) or undifferentiated pleomorphic sarcoma (UPS) is a sarcoma capable of invading adjacent structures. It is a mesenchymal neoplasia that predominates in men between the sixth and seventh decades of life. It is located mainly in the lower limbs and may affect the head and neck, trunk, and retroperitoneum, presenting a tendency to recurrence and local metastasis. This report aims to present a case of MFH in the ankle of a 49-year-old woman with an adjacent bone invasion, which evolved with transtibial amputation. Clinical, radiological, histopathological, and therapeutic aspects were addressed, highlighting the importance of early diagnosis.

Background: Fibrous histiocytoma (FH), a mesenchymal tumour, usually have an orbital presentation. Rarely, FH in both benign and malignant forms have been reported at limbus and conjunctiva. Present narrative review was conducted to determine demographic profile, clinical presentation and management options for this rare tumour. Methods: PubMed database was searched to identify articles presenting with fibrous histiocytoma of conjunctiva, cornea and limbus. Data was tabulated for age of presentation, sex, eye involved, area of involvement, if tumor was benign or malignant, management and recurrences. Results: Total of 35 articles were selected, which included 42 cases, of which 27 were benign and 15 malignant. 25 cases showed presence of limbal FH, 12 conjunctival, 4 caruncular and 1 corneal FH. Mean age of presentation was 30.9 years with male preponderance (females-10, males-17) in benign FH while malignant FH presented with mean age of 44.5 years and sex distribution of 7 females and 8 males. 4 cases of benign FH showed recurrence, which required re-excision and immunotherapy, while recurrence in malignant FH was 60%, where 4 needed exenteration, 3 enucleation and 1 expired due to metastasis. Conclusion: FH is one of the rare ocular tumours of eye presenting usually at the limbus and conjunctiva and rarely cornea. Histopathological examination along with immunostaining has a definite role in the diagnosis. Surgical excision with tumour free margins is the corner stone of treatment. Recurrences in benign varieties need re-excision or immunotherapy, while malignant cases need aggressive surgical options like exenteration or enucleation. [ABSTRACT FROM AUTHOR]

Abstract Background Malignant fibrous neoplasms (MFN) of long bones are rare lesions. Moreover, the prognostic determinants of MFN of long bones have not been reported. This study aimed to present epidemiological data and analyse the prognostic factors for survival in patients with MFN. Materials and methods The Surveillance, Epidemiology, and End Results (SEER) programme database was used to screen patients with malignant fibrous neoplasms (MFN) of long bones from 1973 to 2015, with attention to fibrosarcoma, fibromyxosarcoma, periosteal fibrosarcoma and malignant fibrous histiocytoma. The prognostic values of overall survival (OS) and cancer-specific survival (CSS) were assessed using the Cox proportional hazards regression model with univariate and multivariate analyses. The Kaplan–Meier method was used to obtain OS and CSS curves. Results A total of 237 cases were selected from the SEER database. Malignant fibrous histiocytoma was the most common form of lesion in long bones. Multivariate analysis revealed that independent predictors of OS included age, stage, tumour size and surgery. Age, stage, tumour size and surgery were also independent predictors of CSS. Additionally, the most significant prognostic factor was whether metastasis had occurred at the time of initial diagnosis. Conclusion Among patients with MFN of long bones, age (> 60 years), tumour size (> 10 cm), distant stage, and non-surgical treatment are factors for poor survival.

Abstract 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.

Abstract 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.

The natural history of malignant fibrous histiocytoma (MFH) is still heavily debated. It's anonymous natural history is precisely denoted by its new adapted terminology ‘undifferentiated pleomorphic sarcoma (UPS)’. Thus to diagnose a lesion as UPS, it is vital to correlate its histopathological features along with its immunohistochemical (IHC) expressions to confirm the tumor cell lineage. MFH is extremely rare in mandible with the literature search in PubMed database revealing only 13 case reports of MFH involving the mandible. Among these, only 7 case reports provide IHC details of the case. Without IHC data it is not possible to determine the accuracy of the diagnosis in the remaining 6 cases. Here we report an additional 8th case of MFH involving the ramus and angle of the mandible. Histopathology revealed proliferating malignant spindle cells interspersed with histiocyte-like cells. The tumor cells were strongly positive for vimentin and CD68 and were negative for S-100, epithelial membrane antigen (EMA) and cytokeratin (CK). The diagnosis was made by correlating the histopathological findings with the IHC profile. The report also provides the data (clinical, radiographic, histopathological, immunohistochemical features and treatment details) extracted from the 7 confirmed MFH case reports involving the mandible.

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.