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Background: Low-grade fibromyxoid sarcoma (LGFMS) is a rare soft-tissue tumor that usually occurs in the proximal extremities and trunk. The purpose of this systematic review was to assess patient demographics, treatment approach, and outcomes for patients with LGFMS of the distal upper extremity. Methods: We performed a literature review of case reports and patient reports of LGFMS using Medical Literature Analysis and Retrieval System Online (MEDLINE) and PubMed. Inclusion criteria included case reports and case series of patients with LGFMS of the distal upper extremity. Animal studies, comments, and non-English publications were excluded. Data on available patient demographics, treatment regimen, recurrence, and metastasis were collected. Results: A total of 365 publications were reviewed. After applying the inclusion and exclusion criteria, 19 articles were included. There were 24 unique patients with LGFMS of the distal upper extremity. The median age was 32 years, and most patients were treated with a surgical excision. Of the 15 reported outcomes, 4 patients had local recurrences, and no patient had metastases or died due to the tumor. Conclusions: Although LGFMS of the distal upper extremity is less common, it is critical for surgeons to consider it as a differential. The current approach is a wide excision with negative margins, and chemotherapy or radiotherapy may not be necessary. We found decreased proportions of local recurrence and metastases in the distal upper extremity compared with other anatomical regions. We recommend that surgeons follow up with their patients indefinitely given the slow mitotic rate of LGFMS. [ABSTRACT FROM AUTHOR]

Low Grade Fibromyxoid Sarcoma (LGFMS), a rare entity characterized by bland histologic features, typically affects deep soft tissues of the trunk and lower extremities. Rare cases have been reported arising from the viscera and few demonstrating morphology of high-grade dedifferentiation. Here we report a 39-year-old Chinese woman presenting with primary lung LGFMS, which metastasized to the pancreas five years after diagnosis and then relapsed ten years later as a mediastinum mass. Microscopically, the lung and pancreatic lumps shared similar classical features of LGFMS, composed of bland spindle-shaped cells with low mitotic activity. However, the mediastinal mass had dedifferentiated morphology of dense sheets of round and epithelioid cells with high degree of nuclear pleomorphism and brisk mitosis. Molecular studies showed both classical and dedifferentiated areas had FUS::CREB3L2 rearrangement. However, the mediastinal dedifferentiated area presented with extra H193Y mutation of the TP53. Moreover, the mediastinal tumor displayed a strong and diffuse pattern of p53 expression immunohistochemically, but the primary lung and secondary pancreatic masses did not. Thus, we diagnosed the mediastinal mass as dedifferentiated LGFMS and proposed that TP53 mutation was probably the driver gene alteration in the process, which, to the best of our knowledge, has not been reported in the existing literature. [ABSTRACT FROM AUTHOR]

Abstract Low‐grade fibromyxoid sarcoma (LGFMS) is a rare mesenchymal tumor that primarily arises in the limbs and trunk of young adults, and rarely in the thoracic cavity. An 84‐year‐old Japanese woman presented with a right intrathoracic mass which was 8 cm in size. CT‐guided needle biopsy did not provide a definitive diagnosis. Perioperatively, a mass was found in the right lower lobe of the lung and was suspected to have invaded the chest wall at the sixth–eighth ribs. A right lower lobectomy and combined chest wall resection were performed. Microscopic examination revealed that the tumor was a low‐grade spindle cell tumor originating from the pleura demonstrating focal invasion of the lung. The tumor exhibited positivity for MUC4, and FUS gene translocation was confirmed through fluorescence in situ hybridization. Unfortunately, 10 months postoperatively, tumor recurrence was noted as peritoneal dissemination, and the patient passed away 13 months postoperatively. Although LGFMS may be diagnosed histologically as a low‐grade tumor by needle biopsy, in this case, it was highly malignant. Postoperative long‐term regular medical follow‐up is recommended considering the highly malignant nature of the tumor and the high risk of local recurrence and pulmonary metastasis.

Abstract Background Low-grade fibromyxoid sarcoma (LGFMS) is an uncommon neoplasm generally affecting muscle tissue. It presents rarely in abdominal viscera and even more rarely occurs in the pancreas. All types of pancreatic sarcomas are uncommon, and LGFMS is a rarer still. We present the case of an LGFMS in the pancreas. Because of its rarity, there are no guidelines for appropriate treatment or summations of the natural course of this illness. Case presentation We present the case of a 49-year-old female who presented with epigastric pain. She had a prior history of three episodes of acute pancreatitis many years earlier. A CT revealed a pancreatic body mass, which was biopsied. Pathology returned LGFMS. The patient underwent a distal pancreatectomy and splenectomy. She did well after the case and did not require further intervention. Conclusion Though it is exceedingly rare, cases of pancreatic LGFMS should be reported in order to guide clinical decisions. LGFMS has been shown to have high malignant potential in other tissues, and there is no reason to think pancreatic masses will be different. By building a body of evidence about these rare tumors, patient care will benefit.

Background: Low-grade fibromyxoid sarcoma (LGFMS) is an uncommon neoplasm generally affecting muscle tissue. It presents rarely in abdominal viscera and even more rarely occurs in the pancreas. All types of pancreatic sarcomas are uncommon, and LGFMS is a rarer still. We present the case of an LGFMS in the pancreas. Because of its rarity, there are no guidelines for appropriate treatment or summations of the natural course of this illness. Case presentation: We present the case of a 49-year-old female who presented with epigastric pain. She had a prior history of three episodes of acute pancreatitis many years earlier. A CT revealed a pancreatic body mass, which was biopsied. Pathology returned LGFMS. The patient underwent a distal pancreatectomy and splenectomy. She did well after the case and did not require further intervention. Conclusion: Though it is exceedingly rare, cases of pancreatic LGFMS should be reported in order to guide clinical decisions. LGFMS has been shown to have high malignant potential in other tissues, and there is no reason to think pancreatic masses will be different. By building a body of evidence about these rare tumors, patient care will benefit. [ABSTRACT FROM AUTHOR]

An 18-year-old boy presented with a giant midline mass with 9 years of evolution. The tumor was excised, and reconstruction made with a customized sternum implant and a free latissimus dorsi muscle flap with skin graft. Histological analysis was compatible with low-grade fibromyxoid sarcoma (LGFMS). [ABSTRACT FROM AUTHOR]

A low-grade fibromyxoid sarcoma (LGFMS) is an extremely rare tumor first described by Evans in 1987. LGFMS is a type of soft tissue sarcoma characterized by a deceptively benign histological appearance but completely malignant behavior. LGFMS is usually seen in the deep soft tissues of the extremities and trunk. We have examined many reviews, case reports and case series previously published in PubMed and Google Scholar. To date, only five cases have been reported in the maxilla. LGFMS generally affects young adults, but it can also be seen in children and older adults. A radical surgical approach is the most recommended treatment option. LGFMS has a very low mitotic activity; therefore, it is considered that neither chemotherapy nor radiotherapy has a significant effect on long-term LGFMS prognosis. However, to date, there has been no study suggesting any protocol for the follow-up of patients with LGFMS. In this report, we present a case with LGFMS located in the maxillary sinus, in which despite radiotherapy following extensive surgical excision, the tumor recurred in a short period of three months and reached its former size. [ABSTRACT FROM AUTHOR]

Low-grade fibromyxoid sarcoma is a rare tumor that manifests as a deep soft tissue mass characterized by a benign histology, but with potentially aggressive clinical behavior and a high rate of recurrence; primary intracranial sarcomas are even rarer. We present a case of primary intracranial low-grade fibromyxoid sarcoma, emphasizing its clinical, radiologic, and histologic features. A 39-year-old woman presented with a recent history of headache and seizures. A right parietal mass was detected on computed tomography and magnetic resonance imaging, and she was subsequently operated on to remove the intracranial mass. Histologic examination of the resected tumor revealed mildly atypical fibroblastic cells embedded within a myxoid matrix. The diagnosis was confirmed by electron microscopy and cytogenetic analyses. This report describes electron microscopic evaluation of intracranial low-grade fibromyxoid sarcoma, which has an extremely rare occurrence. [ABSTRACT FROM AUTHOR]

Background: Low-grade fibromyxoid sarcoma (LGFMS) is a rare sarcoma subtype with a generally indolent pattern of clinical behaviour, but treatments for advanced disease are limited. Patients and Methods: A retrospective search of a prospectively maintained institutional database identified 102 patients treated from December 1994 to August 2018. We evaluated the outcome of patients and the efficacy and safety of non-surgical therapies in LGFMS. Results: Ninety-four out of 102 (92.2%) underwent primary resection, seven (6.9%) were treated with systemic therapy and one (1.0%) is currently being treated with pre-operative radiotherapy. The RECIST 1.1 response rate to first-line chemotherapy was 0%, and median progression-free survival was 1.84 months (95% confidence intervaI=0.10-3.6 months). Conclusion: Conventional systemic therapy has limited efficacy in advanced LGFMS. [ABSTRACT FROM AUTHOR]

Abstract Background Low-grade fibromyxoid sarcoma (LGFMS) is a rare soft tissue tumor that has a tendency to grow in the deep soft tissue of the trunk and extremities. Despite its benign appearance, the tumor has a high recurrence rate and metastatic potential. LGFMS in the perineal space is rare, and only a few cases have been reported. We present the first case of LGFMS to be located at the external anal sphincter. Case presentation A 27-year-old male patient admitted to our Surgical Department with perianal pain and swollen for a year. The digital rectal examination revealed a perianal mass. Oral metronidazole and analgesia were prescribed on suspicion of perianal abscess failed to alleviate the symptom; hence, the patient was scheduled for surgery. Intraoperative diagnosis revealed an encapsulated tumor in the external anal sphincter that extended from the perianal region orally to the pararectal space. The results of immunohistochemistry (MUC4 staining) and FUS gene rearrangement by fluorescence in situ hybridization confirmed the diagnosis of LGFMS. Conclusions This case is unique in terms of the location of the rare soft tissue tumor. Although LGFMS is considered low grade, its unpredictable behavior necessitates a long-term follow-up.

Background Low-grade fibromyxoid sarcoma (LGFMS) and sclerosing epithelioid fibrosarcoma (SEF) are two rare but aggressive soft tissue sarcomas that can be difficult to distinguish due to histopathological similarities. The present study examines the diagnostic capacities of mucin-4 (MUC4), a transmembrane mucin, in identifying different types of sarcomas and broadens its evaluation to include a wide range of sarcomas. Methods Immunohistochemical (IHC) examination of tissue samples from various sarcomas was performed using a mouse anti-MUC4 monoclonal antibody. IHC was conducted on 4-mm thick formalin-fixed paraffin-embedded tissue sections after pressure cooker antigen retrieval with a mouse anti-MUC4 monoclonal antibody. Results MUC4 was shown to be highly expressed in SEF (n=13) and LGFMS (n=10), while focal positivity in synovial sarcoma (n=1). Other sarcomas, such as malignant peripheral nerve sheath tumors, fibrosarcoma, leiomyosarcoma, liposarcoma, and myxofibrosarcoma, exhibited no expression (n=0). These findings are consistent with previous research and support MUC4 specificity as a SEF and LGFMS marker. This study provides information on the diagnostic efficacy of MUC4, particularly in the context of certain subtypes. It not only helps our understanding of these unique instances, but it also provides context for histopathological and IHC findings in soft tissue sarcoma. Furthermore, this study investigates the influence of age and gender on MUC4 expression in a range of sarcomas, which was typically understudied in the literature and found no relation with expression of MUC4. Conclusion In conclusion, this study adds to our understanding of soft tissue sarcomas by emphasizing the crucial role of MUC4 in certain sarcoma subtypes while acknowledging the complex variety of the sarcoma landscape. Further research is needed to understand the molecular mechanism that governs marker expression patterns, as well as the therapeutic implications., Competing Interests: The authors have declared that no competing interests exist., (Copyright © 2023, Hassan et al.)

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Low-grade fibromyxoid sarcoma (LGFMS) are rare soft-tissue sarcomas that usually come to attention as slow growing masses. Males and females are affected approximately equally and typically arising in the soft tissue of the proximal extremities. Primary occurrence within the head and neck is exceedingly rare. We report a case arising from the hard palate of a 40-year-old male and according to our knowledge this is the first ever case of LGFMS involving the palate to be reported.

Low-grade fibromyxoid sarcoma (LGFMS) is a rare sarcoma typically presenting as a deep soft tissue mass in the lower extremities or trunk. Only four cases of primary intracranial LGFMS have been reported in the literature, but none with molecular/cytogenetic data.We report here a new bona fide case confirmed by the characteristic FUS gene rearrangement, as shown by fluorescence in situ hybridization analysis, in addition to typical histopathological findings. This would be the first genetically confirmed report of the extremely rare occurrence of intracranial LGFMS. [ABSTRACT FROM AUTHOR]

Low-grade fibromyxoid sarcoma (LGFMS) are rare soft-tissue sarcomas that usually come to attention as slow growing masses. Males and females are affected approximately equally and typically arising in the soft tissue of the proximal extremities. Primary occurrence within the head and neck is exceedingly rare. We report a case arising from the hard palate of a 40-year-old male and according to our knowledge this is the first ever case of LGFMS involving the palate to be reported. [ABSTRACT FROM AUTHOR]

Background: Low-grade fibromyxoid sarcoma (LGFMS) is a rare soft tissue tumor that has a tendency to grow in the deep soft tissue of the trunk and extremities. Despite its benign appearance, the tumor has a high recurrence rate and metastatic potential. LGFMS in the perineal space is rare, and only a few cases have been reported. We present the first case of LGFMS to be located at the external anal sphincter., Case Presentation: A 27-year-old male patient admitted to our Surgical Department with perianal pain and swollen for a year. The digital rectal examination revealed a perianal mass. Oral metronidazole and analgesia were prescribed on suspicion of perianal abscess failed to alleviate the symptom; hence, the patient was scheduled for surgery. Intraoperative diagnosis revealed an encapsulated tumor in the external anal sphincter that extended from the perianal region orally to the pararectal space. The results of immunohistochemistry (MUC4 staining) and FUS gene rearrangement by fluorescence in situ hybridization confirmed the diagnosis of LGFMS., Conclusions: This case is unique in terms of the location of the rare soft tissue tumor. Although LGFMS is considered low grade, its unpredictable behavior necessitates a long-term follow-up.