Pleomorphic Dermal Sarcoma.

Pleomorphic dermal sarcoma (PDS) is a rare cutaneous/subcutaneous neoplasm of purported mesenchymal differentiation that exists along a clinicopathologic spectrum with atypical fibroxanthoma (AFX). While PDS and AFX share histopathologic and immunohistochemical features, PDS exhibits deeper tissue invasion and has a higher rate of metastasis and local recurrence than AFX. Given its aggressive clinical course, early recognition and clinical management of PDS are essential for optimizing patient outcomes. This review aims to provide a brief overview of the clinicopathologic and molecular features, prognosis, and treatment of PDS.

Undifferentiated pleomorphic sarcoma of the spleen: a case report and literature review.

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 of the palate: a case report.

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.

Peritoneal sarcomatosis due to undifferentiated pleomorphic sarcoma: A case report and review of the literature.

Undifferentiated pleomorphic sarcoma (UPS), which was previously known as malignant fibrous histiocytoma (MFH), rarely presents in the abdomen, and sarcomatosis due to UPS has not yet been reported in the literature. Here, we present a 62-year-old man who had abdominal sarcomatosis due to UPS with a poor prognosis.

Undifferentiated pleomorphic sarcoma of the floor of mouth: A rare case.

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.

Undifferentiated Pleomorphic Sarcoma of a Chronic Burn Scar of the Knee With Lymph Node Metastasis.

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.

Malignant fibrous histiocytoma of the axilla with breast cancer: A case report.

BACKGROUND: Multiple primary malignant neoplasms refer to multiple tumors with different origins. They may be synchronous or metachronous. The incidence is 0.73%- 11.7%. Synchronous cases of breast cancer with sarcoma are rare. CASE SUMMARY: Here, we report a 78-year-old female patient admitted to hospital after accidental discovery of a left axillary mass. Preoperative examination revealed a breast mass. Pathology showed left breast cancer and left axillary sarcoma. The patient underwent surgery, endocrine therapy and radiotherapy. She has been followed up for 1 year, and no local recurrence or distant metastasis was observed. CONCLUSION: Attention should be paid to multiple primary malignant neoplasms, not limited to the current diagnosis and analysis, avoiding missed diagnosis and misdiagnosis.

Use of Ultrasound for Evaluation of a Large Undifferentiated Pleomorphic Sarcoma in the Lower Extremity of an Elderly Male.

Soft tissue sarcomas arise infrequently and make up less than 1% of all cancers. An undifferentiated pleomorphic sarcoma (UPS), formerly known as a malignant fibrous histiocytoma, is a malignant subtype of soft tissue sarcoma identified by a lack of specific immunohistochemical markers for lineage differentiation. These tumors are aggressive and enlarge rapidly with an increased risk of metastasis, thus prompt diagnosis, treatment, and post-resection surveillance are imperative. This case report demonstrates an incidental finding of a large undifferentiated pleomorphic sarcoma of the posterior thigh in an 86-year-old male initially evaluated with a bedside ultrasound.

Histopathological Features of Myxoid Pleomorphic Liposarcoma in an African Pygmy Hedgehog (Atelerix Albiventris).

Myxoid pleomorphic liposarcoma is characterized by pathological features of both pleomorphic liposarcoma and myxoid liposarcoma, as the name suggests. In this case, a myxoid pleomorphic liposarcoma was observed in a 5-year-old male African pygmy hedgehog. It consisted of ~60% of the myxoid substance area with proliferating round cells and ~30% of pleomorphic neoplastic cells. The subject presented with extrapulmonary metastasis, but a good prognosis during 6 months of follow-up, which is similar to the characteristics of myxoid liposarcoma. The histopathological features of myxoid pleomorphic liposarcoma may reflect the features of either myxoid liposarcoma or pleomorphic liposarcoma depending on the proportion of each histopathological feature. The proportion of the pleomorphic area and the myxoid area may offer information on the prognosis and metastasis of myxoid pleomorphic liposarcoma, which will be helpful for setting up a treatment plan. Thus, analyzing the proportion of pleomorphic area and myxoid area could be suggested as one of the ways to predict clinical outcomes. In addition to the fact that this is the first case of a myxoid pleomorphic liposarcoma in hedgehogs, this case is meaningful, considering the unique histopathological characteristics and rare incidence of myxoid pleomorphic liposarcoma that could be important in humans as well.

EML4-ALK rearrangement in primary malignant fibrous histiocytoma of the lung treated with alectinib: A case report.

Primary malignant fibrous histiocytoma of the lung (PMFHL) is extremely rare. It is more common in the right lung and has no specific symptoms. Lymph node metastasis is rare, but hematogenous metastasis is more common. The common metastatic sites are the brain and bone. In this study, a 59-year-old male patient was diagnosed with PMFHL with brain metastasis due to persistent cough and blood in the sputum for the past week. Genetic testing revealed EML4-ALK gene rearrangement (fusion). We first used alectinib in a patient with advanced PMFHL with EML4-ALK gene rearrangement (fusion) accompanied by brain metastasis. The treatment was effective and successfully delayed the development of the disease. Satisfactory results were observed, with an overall survival time of 19 months. Therefore, genetic testing in PMFHL and the choice of treatment plan are important. Local treatment methods, including surgery and radiotherapy, are important when the disease is less advanced. Multidisciplinary discussion is recommended for the best prognosis.

Surgical management of primary undifferentiated pleomorphic sarcoma of the rectum: a case report and review of the literature.

BACKGROUND: Undifferentiated pleomorphic sarcoma (UPS) is a malignant soft tissue tumor that has been reclassified from malignant fibrous histiocytoma with the development of the pathological diagnosis. It principally occurs in the extremities but rarely occurs in the rectum. We herein report a rare case of UPS arising in the rectum. CASE PRESENTATION: A 85-year-old woman was referred to our hospital with a complaint of anal pain, which had persisted for several months. Computed tomography (CT) showed a 53 × 58 × 75 mm mass on the left side of the rectum. Colonoscopy revealed a submucosal elevation in the rectum without any exposure of the tumor to the surface. Contrast-enhanced CT and magnetic resonance imaging revealed an 80-mm mass that originated in the rectal muscular propria, and we suspected a gastrointestinal stromal tumor. No lymph node metastasis or distant metastasis was observed. We performed a laparoscopic Hartmann's operation. Intraoperatively, severe adhesion around the tumor caused tumor injury and right ureteral dissection. Thus, laparoscopic right ureteral anastomosis and ureteral stenting were additionally performed. The operation time was 6 h and 3 min, and the estimated blood loss was small. The patient was discharged without complications 25 days after surgery. A pathological examination showed that the tumor was composed of highly heterogeneous cells with no specific differentiation traits, leading to a diagnosis of UPS. Contrast-enhanced CT performed 2 months after surgery showed bilateral pelvic lymph node enlargement, which indicated recurrence. Considering the patient's age, we performed radiotherapy (50 Gy/25 Fr targeting the pelvic region). At present, 16 months have passed since the completion of radiotherapy. Contrast-enhanced CT shows that the recurrent lymph nodes have disappeared, and no new distant metastasis has been observed. CONCLUSIONS: We reported a case of UPS arising in the rectum. The surgical procedure and indication of preoperative therapy should be carefully selected because complete removal of the tumor is desirable in UPS.

Management of Myxofibrosarcoma and Undifferentiated Pleomorphic Sarcoma.

Undifferentiated pleomorphic sarcoma (UPS) and myxofibrosarcoma (MFS) are genomically complex tumors commonly diagnosed in the extremities or trunk of elderly patients. They likely represent a spectrum of disease differentiated by myxoid stroma and curvilinear vessels observed in MFS but not in UPS. Limb-sparing surgery is the standard of care although the infiltrative nature of MFS mandates wider resection margins than are necessary for UPS. UPS are conversely associated with high risks of distal recurrence, often prompting recommendations for adjuvant chemotherapy. In both histologies, anthracycline-based therapies or gemcitabine and docetaxel are used to manage advanced disease; immunotherapy may be of benefit in a subset of patients.

A case of pseudomyogenic hemangioendothelioma misdiagnosed as low-grade malignant fibrous histiocytoma and review of literature. / è¯¯è¯Šä¸ºä½Žåº¦æ¶æ€§çº¤ç»´ç»„ç»‡ç»†èƒžç˜¤çš„å‡è‚Œæºæ€§è¡€ç®¡å† çš®ç˜¤1ä¾‹å¹¶æ–‡çŒ®å¤ä¹ .

Pseudomyogenic hemangioendothelioma (PHE) is a rare angiogenic tumor. Histologically, the morphological characteristics of neoplastic vessels and endothelial differentiation are not obvious, and it is easy to be confused with epithelioid sarcoma, epithelioid hemangioendothelioma and myogenic tumor. PHE usually occurs in arms and legs in young people and has a significant male predominance. The tumor has a predilection for the distal extremities and its typical manifestation is multiple center invasion of a single limb, which can involve all layers of skin and subcutaneous tissues,and is often accompanied by abvious pain. Histologically, PHE is characterized by infiltrative growth of tumor. Most tumor lesions are composed of sheets and loose fascicles of plump spindle or epithelioid cells within a background of variably prominent inflammatory infiltration, which was commonly composed of neutrophils. Some cells may resemble rhabdomyoblasts, and nuclear atypia and mitosis were rare. The tumor cells generally expressed positive cytokeratin (CK), ETS-related gene (ERG), Friend leukemia virus integration 1 (FLI1) and integrase interactor 1(INI1). In some cases, the tumor cells expressed CD31. A case of a young woman was reported in this paper, who presented with a subcutaneous mass with severe pain and was chronologically misdiagnosed with herpes zoster, low-grade malignant fibrous histiocytoma and epithelioid hemangioendothelioma. In this study, the clinical and pathological features, differential diagnosis and the latest progress in therapy of PHE were analyzed based on relevant literature.

Undifferentiated high-grade pleomorphic sarcoma of the colon: a rare case report and literature review.

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.

Descriptive epidemiology of soft tissue and bone sarcomas in Lebanon.

OBJECTIVES: Most epidemiologic studies on soft tissue sarcomas (STS) and bone sarcomas (BS) are performed in western countries, with few in the Middle East and North Africa region. We describe the epidemiology of sarcomas in Lebanon using the medical records database at the American University of Beirut Medical Center (AUBMC). METHODS: This single-center retrospective cohort study included patients with sarcomas registered in the database between 2015 and 2019. Their charts were reviewed for baseline characteristics, tumor biology and location, treatment modalities, recurrence, metastasis, and death. RESULTS: The cohort included 234 patients with STS and 99 patients with BS. Most tumors were <10 cm in size. The most common subtypes were liposarcoma for STS and osteosarcoma for BS. The most common location of STS was the thigh. The most frequent sites of STS metastasis were the lungs. Histological subtype, smoking status, and tumor size and grade were significant for progression-free survival (PFS) in patients with STS. By multivariable analysis, smoking was significantly associated with poorer PFS in STS. For BS, only tumor grade was significant for PFS. CONCLUSION: The epidemiology of sarcomas at AUBMC is similar to that previously reported. Smoking history was associated with poorer survival in patients with STS.

Pleomorphic Dermal Sarcoma: A Clinical and Histopathologic Emulator of Atypical Fibroxanthoma, but Different Biologic Behavior.

Description Pleomorphic dermal sarcoma (PDS) can clinically and histopathologically mimic atypical fibroxanthoma (AFX). However, it has a more aggressive clinical course with a higher recurrence rate and metastatic potential. This case presentation aims to report a rapidly-growing, exophytic, 4 cm tumor following a non-diagnostic shave biopsy 2 months prior and to highlight distinctive features between PDS and AFX needed to make the correct diagnosis. Like AFX, PDS occurs on the sun-damaged skin of the elderly, usually on the head and neck. Also, like AFX, PDS histopathologically consists of sheets or fascicles of epithelioid and/or spindle-shaped cells, often with multinucleation, pleomorphism, and numerous mitotic figures. Immunohistochemistry cannot distinguish PDS from AFX but is used to exclude other malignancies. PDS can be distinguished from AFX by size (PDS is usually >2.0 cm) and by the presence of more aggressive histopathologic features, such as subcutaneous involvement, perineural and/or lymphovascular invasion, and necrosis. PDS is a rare entity not well documented in the literature with confusing, misleading, and changing nomenclature. PDS is a diagnosis of exclusion made after complete excision of the tumor with the aid of histopathology and immunohistochemistry.

Malignant fibrous histiocytoma of the floor of mouth: a case report and review of the literature.

Malignant fibrous histiocytoma (MFH) is the most common soft tissue sarcoma in late adulthood and usually occurs in the limbs, trunk, and peritoneum. Less than 10% of MFH cases occur in the head and neck region. The clinical manifestations and pathological features of MFH are atypical, and it is difficult to make a clinical diagnosis. We describe a rare case of MFH of the floor of mouth and provide our diagnosis and treatment experiences. Through this review, we also evaluate the origin, World Health Organization (WHO) classification, clinical presentations, pathological features, treatment methods, and prognosis of MFH. MFH may originate from fibroblasts or primitive mesenchymal cells. MFH was defined as undifferentiated pleomorphic sarcoma in the 2002 WHO classification of bone and soft tissue tumors. The most common manifestation of MFH is a painless enlarging nodule, often without overlying epidermal ulcers. Jaw lesions are usually found after displays of swelling, pain, paresthesia, and loose teeth. MFH is composed of pleomorphic spindle cells, usually with hemorrhage, necrosis, and lymphocyte infiltration. The main treatment method is surgical resection. Moreover, radiotherapy and chemotherapy have certain auxiliary effects. The local recurrence and distant metastasis of MFH are common, and the prognosis is poor. Therefore, determining the histopathological features of MFH and conducting appropriate immunohistochemical examinations are crucial in establishing the correct diagnosis. In-depth study is required in order to have a better understanding of head and neck MFH.

A case of pseudomyogenic hemangioendothelioma misdiagnosed as low-grade malignant fibrous histiocytoma and review of literature / 中南大学学报(医学版)

Pseudomyogenic hemangioendothelioma (PHE) is a rare angiogenic tumor. Histologically, the morphological characteristics of neoplastic vessels and endothelial differentiation are not obvious, and it is easy to be confused with epithelioid sarcoma, epithelioid hemangioendothelioma and myogenic tumor. PHE usually occurs in arms and legs in young people and has a significant male predominance. The tumor has a predilection for the distal extremities and its typical manifestation is multiple center invasion of a single limb, which can involve all layers of skin and subcutaneous tissues,and is often accompanied by abvious pain. Histologically, PHE is characterized by infiltrative growth of tumor. Most tumor lesions are composed of sheets and loose fascicles of plump spindle or epithelioid cells within a background of variably prominent inflammatory infiltration, which was commonly composed of neutrophils. Some cells may resemble rhabdomyoblasts, and nuclear atypia and mitosis were rare. The tumor cells generally expressed positive cytokeratin (CK), ETS-related gene (ERG), Friend leukemia virus integration 1 (FLI1) and integrase interactor 1(INI1). In some cases, the tumor cells expressed CD31. A case of a young woman was reported in this paper, who presented with a subcutaneous mass with severe pain and was chronologically misdiagnosed with herpes zoster, low-grade malignant fibrous histiocytoma and epithelioid hemangioendothelioma. In this study, the clinical and pathological features, differential diagnosis and the latest progress in therapy of PHE were analyzed based on relevant literature.