Rare Fibroblastic and Myofibroblastic Tumors

2017 ◽  
Author(s):  
Vasilena Zheleva ◽  
Yu Liang ◽  
Laleh Melstrom
Keyword(s):  
Young Adults ◽  
Local Recurrence ◽  
Soft Tissues ◽  
Distant Metastases ◽  
Low Grade ◽  
Linear Arrangement ◽  
Myxoid Stroma ◽  
High Local Recurrence Rate ◽  
Inflammatory Myofibroblastic Tumors ◽  
Hands And Feet

This review discusses the clinical presentation, histopathologic characteristics, and diagnostic and treatment algorithms for several distinct fibroblastic and myofibroblastic tumor subtypes. Low-grade fibromyxoid sarcomas typically arise in the deep soft tissues of the proximal extremities or trunk in young adults. They tend to have a high local recurrence rate, although some may have distant metastases. Histologically, they consist of bland fibroblasts with a whorled or linear arrangement alternating with hypocellular myxoid stroma. Sclerosing epithelioid fibrosarcomas typically arise in the deep soft tissues of the lower extremities of middle-aged adults. They tend to be slow growing but clinically aggressive, with a high propensity for local recurrence and distant metastases. Classic histologic features include the presence of small, uniform, ovoid epithelioid cells with sparse cytoplasm, arranged in cords within a sclerotic fibrous stroma. Myxoinflammatory fibroblastic sarcomas mainly affect the hands and feet. They are prone to local recurrence, with a very low likelihood of distant metastasis. Histologically, they have nodular architecture and highly cellular areas with an abundance of inflammatory cells, among which characteristic large Reed-Sternberg–like and lipoblast-like cells and hypocellular areas rich in myxoid stroma can be seen. Inflammatory myofibroblastic tumors typically occur in children and young adults, with common anatomic locations being the omentum, mesentery, retroperitoneum, and lung. Histologically, they are composed of myofibroblastic spindle cells accompanied by a lymphoplasmacytic inflammatory infiltrate. Surgical resection is the mainstay of treatment for all of the above tumor types. Radiation therapy may be used as adjuvant therapy for tumors with a high risk of local recurrence, in the setting of positive margins, or for unresectable primary or metastatic disease. There is no definite role for standard chemotherapy, but in certain cases, targeted therapy may be of use, such as ALK inhibitors in ALK-positive inflammatory myofibroblastic tumors.  Key words: inflammatory myofibroblastic sarcoma, low-grade fibromyxoid sarcoma, myxoinflammatory fibroblastic sarcoma, sclerosing epithelioid fibrosarcoma

Rare Fibroblastic and Myofibroblastic Tumors

2017 ◽  
Author(s):  
Vasilena Zheleva ◽  
Yu Liang ◽  
Laleh Melstrom
Keyword(s):  
Young Adults ◽  
Local Recurrence ◽  
Soft Tissues ◽  
Distant Metastases ◽  
Low Grade ◽  
Linear Arrangement ◽  
Myxoid Stroma ◽  
High Local Recurrence Rate ◽  
Inflammatory Myofibroblastic Tumors ◽  
Hands And Feet

This review discusses the clinical presentation, histopathologic characteristics, and diagnostic and treatment algorithms for several distinct fibroblastic and myofibroblastic tumor subtypes. Low-grade fibromyxoid sarcomas typically arise in the deep soft tissues of the proximal extremities or trunk in young adults. They tend to have a high local recurrence rate, although some may have distant metastases. Histologically, they consist of bland fibroblasts with a whorled or linear arrangement alternating with hypocellular myxoid stroma. Sclerosing epithelioid fibrosarcomas typically arise in the deep soft tissues of the lower extremities of middle-aged adults. They tend to be slow growing but clinically aggressive, with a high propensity for local recurrence and distant metastases. Classic histologic features include the presence of small, uniform, ovoid epithelioid cells with sparse cytoplasm, arranged in cords within a sclerotic fibrous stroma. Myxoinflammatory fibroblastic sarcomas mainly affect the hands and feet. They are prone to local recurrence, with a very low likelihood of distant metastasis. Histologically, they have nodular architecture and highly cellular areas with an abundance of inflammatory cells, among which characteristic large Reed-Sternberg–like and lipoblast-like cells and hypocellular areas rich in myxoid stroma can be seen. Inflammatory myofibroblastic tumors typically occur in children and young adults, with common anatomic locations being the omentum, mesentery, retroperitoneum, and lung. Histologically, they are composed of myofibroblastic spindle cells accompanied by a lymphoplasmacytic inflammatory infiltrate. Surgical resection is the mainstay of treatment for all of the above tumor types. Radiation therapy may be used as adjuvant therapy for tumors with a high risk of local recurrence, in the setting of positive margins, or for unresectable primary or metastatic disease. There is no definite role for standard chemotherapy, but in certain cases, targeted therapy may be of use, such as ALK inhibitors in ALK-positive inflammatory myofibroblastic tumors.  This review contains 16 figures, 1 table and 30 references Key words: inflammatory myofibroblastic sarcoma, low-grade fibromyxoid sarcoma, myxoinflammatory fibroblastic sarcoma, sclerosing epithelioid fibrosarcoma

Low-Grade Fibromyxoid Sarcoma: A Brief Review

2006 ◽  
Vol 130 (9) ◽  
pp. 1358-1360 ◽  
Author(s):  
Stephen E. Vernon ◽  
Pablo A. Bejarano
Keyword(s):  
Spindle Cell ◽  
Fusion Gene ◽  
Cytogenetic Abnormality ◽  
Low Grade ◽  
Deep Soft Tissue ◽  
Balanced Translocation ◽  
Linear Arrangement ◽  
Myxoid Stroma ◽  
Fibromyxoid Sarcoma ◽  
The Common

Abstract Low-grade fibromyxoid sarcomas are uncommon deep soft tissue neoplasms first described by Evans in 1987. They exhibit a deceptively benign appearance, with a whorled or linear arrangement of spindle-shaped cells showing few to absent mitoses. A characteristic, but not specific, feature is the presence of areas of myxoid stroma. Recurrences are common, and late metastases have been recorded. A closely related but morphologically distinct tumor, the so-called hyalinizing spindle cell tumor with giant rosettes, has also been described; both neoplasms share the same cytogenetic abnormality, a balanced translocation resulting in a FUS/CREB3L2 fusion gene. Because of similar clinical behavior and the common cytogenetic abnormality, some authors prefer to consider both lesions as a single entity within the spectrum of low-grade sarcomas.

scholarly journals CYTO HISTOLOGIC CORRELATION OF A LOW GRADE FIBROMYXOID SARCOMAAT AN UNUSUAL LOCATION – A CASE REPORT

2021 ◽  
pp. 1-3
Author(s):  
Pooja Jain ◽  
Annu Nanda ◽  
Deepak Kumar Singh ◽  
Udita Singhal
Keyword(s):  
Soft Tissues ◽  
Correct Diagnosis ◽  
Subcutaneous Fat ◽  
Needle Aspiration ◽  
Low Grade ◽  
Difficult Case ◽  
Nuclear Pleomorphism ◽  
Rare Tumour ◽  
Spindle Cells ◽  
High Local Recurrence Rate

BACKGROUND Low grade fibromyxoid sarcoma (LGFMS) is a rare tumour of the deep soft tissues seen in young adults. The histologic features of LGFMS are well established, however the cytologic findings are scantily defined and the distinction from other benign and malignant myxoid soft tissue tumours is difficult. CASE DETAILS A 29 year old male presented with a superficial swelling in the left areolar region for two months. USG revealed a well-defined 2 by 2 cm hyperechoic lesion extending upto the subcutaneous fat. FNA yielded mucoid material and smears showed spindle cells having round to elongated nuclei with mild anisokaryosis, bland chromatin and wispy cytoplasm in a myxoid background. No significant nuclear pleomorphism or mitoses were noted. A diagnosis of myxoid mesenchymal lesion was made. The excised tumour was well circumscribed with grey white cut surface showing myxoid areas. Microscopically, the tumour displayed moderate cellularity with storiform, intersecting and parallel bundles of spindle cells showing mild nuclear pleomorphism. The myxoid areas showed cells in a haphazard fashion floating in abundant mucoid matrix associated with a capillary network. The mitotic index was low. IHC showed tumor cells positive for vimentin and negative for S-100 & SMA. CONCLUSION LGFMS is a rare low-grade malignancy with a high local recurrence rate and late metastasis. The diagnosis of LGFMS can be difficult to render from fine needle aspiration cytology (FNAC) alone because of morphological overlap with other spindle cell and myxoid lesions. Cytohistological correlation and IHC is helpful in arriving at a correct diagnosis.

Chondrosarcoma of the hands and feet

2021 ◽  
Vol 103-B (3) ◽  
pp. 562-568
Author(s):  
Gilber Kask ◽  
Minna K. Laitinen ◽  
Jonathan Stevenson ◽  
Scott Evans ◽  
Lee M. Jeys ◽  
...  
Keyword(s):  
Local Recurrence ◽  
Surgical Management ◽  
Histological Grade ◽  
Anatomical Location ◽  
Appendicular Skeleton ◽  
Low Grade ◽  
Disease Specific Survival ◽  
Metatarsal Bones ◽  
Hands And Feet ◽  
Disease Specific

Aims Although chondrosarcomas (CSs) display true malignant features, including local recurrence (LR) and metastases, their behaviour in the hands and feet is thought to differ from that in other parts of the axial and appendicular skeleton by having a lower metastatic potential. The purpose of this study was to investigate the disease-specific and surgical factors that affect the local and systemic prognosis of CS of the hands and feet. Methods A multicentre retrospective study was carried out at two tertiary sarcoma centres. A database search identified all patients with a CS treated between January 1995 and January 2018. There were 810 CSs of which 76 (9.4%) were located in the fingers, toes, metacarpals, and metatarsal bones. Results The median age of the study population was 55 years (36 to 68) with a median follow-up of 52 months (22 to 87) months. Overall, 70% of the tumours were in the hand (n = 54) and 30% in the foot (n = 22). Predictors for LR were margin (p = 0.011), anatomical location (p = 0.017), and method of surgical management (p = 0.003). Anatomical location (p = 0.026), histological grade between 1 and 3 (p = 0.004) or 2 and 3 (p = 0.016), and surgical management (p = 0.001) were significant factors for LR-free survival. Disease-specific survival was affected by histological grade (p < 0.001), but not by LR (p = 0.397). Conclusion Intralesional curettage of a low-grade CS is associated with an increased risk of LR, but LR does not affect disease-specific survival. Therefore, for low-grade CSs of the hands and feet, surgical management should aim to preserve function. In grade 2 CS, our study did not show any decreased disease-specific survival after recurrence; however, we suggest a more aggressive surgical approach to these tumours to prevent local recurrence, especially in the metacarpal and metatarsal bones. In high-grade tumours, the incidence of progressive disease is high and, therefore, the treatment of the primary tumour should be aggressive where possible, and patients observed closely for the development of metastatic disease. Cite this article: Bone Joint J 2021;103-B(3):562–568.

scholarly journals Mitotically Active Plexiform Fibrohistiocytic Tumor

2013 ◽  
Vol 2013 ◽  
pp. 1-3 ◽  
Author(s):  
Ebru Zemheri ◽  
Şeyma Özkanlı ◽  
Serkan Şenol ◽  
Filiz Ozen ◽  
Cigdem Ulukaya Durakbaşa ◽  
...  
Keyword(s):  
Young Adults ◽  
Local Recurrence ◽  
Soft Tissues ◽  
Upper Extremities ◽  
Mesenchymal Neoplasm ◽  
Systemic Metastasis ◽  
Children And Young Adults ◽  
Plexiform Fibrohistiocytic Tumor ◽  
Slow Growing

Plexiform fibrohistiocytic tumor is an intermediate malignant tumor situated in superficial soft tissues. It affects children and young adults. The tumor is most commonly located on upper extremities, whereas involvement of back region is rare. Mitotic activity is generally low (~3/10 HPF). It is rare, but it can exhibit aggressive behavior, so total excision with clear surgical margins and long-term followup is necessary to detect local recurrence and metastases. We report a child with a solid mass on back region which was found to be a mitotically active plexiform fibrohistiocytic tumor (6/10 HPF) after excision. Plexiform fibrohistiocytic tumor (PFT) is a mesenchymal neoplasm of children, adolescents, and young adults. It is characterized by fibrohistiocytic cytomorphology and multinodular growth pattern. Clinically it is usually a slow-growing mass of upper extremities with frequent local recurrence and rare regional lymphatic and systemic metastasis (Fletcher et al. (2002), Enzinger and Zhang (1988), Remstein et al. (1999)).

scholarly journals Surgical treatment and long-term outcomes of low-grade myofibroblastic sarcoma: a single-center case series of 15 patients

2021 ◽  
Vol 19 (1) ◽  
Author(s):  
Jong-Ho Kim ◽  
Woosuk Choi ◽  
Hwan Seong Cho ◽  
Kyu Sang Lee ◽  
Joseph Kyu-hyung Park ◽  
...  
Keyword(s):  
Surgical Treatment ◽  
Local Recurrence ◽  
Univariate Analysis ◽  
Case Series ◽  
Distant Metastases ◽  
Resection Margins ◽  
Low Grade ◽  
Long Term Outcomes ◽  
Myofibroblastic Sarcoma

Abstract Background Low-grade myofibroblastic sarcoma (LGMS) is a poorly studied, rare, soft tissue sarcoma. LGMS is characterized by a low malignancy potential, tendency for local recurrence, and low likelihood of distant metastases. However, no studies have reported on the surgical treatment method and its long-term outcomes. Methods We included all patients treated for LGMS at our institution between March 2010 and March 2021. Medical charts were retrospectively reviewed to collect demographic information, as well as information about the clinical course, tumor characteristics, and outcomes. Statistical analysis was performed to identify the factors associated with the recurrence rate. Results Fifteen patients who underwent surgical treatment were enrolled in this study. There were seven cases in the upper extremities, four in the trunk area, three in the lower extremities, and one in the head and neck area. There were no metastatic cases and two cases of local recurrence. Conclusions The incidence of LGMS in the extremities or trunk may be higher than expected based on the current literature. Univariate analysis showed that local tissue invasion and surgical method could be associated with local recurrence. Although further large studies are needed to establish risk factors of local recurrence or extent of resection margins, based on our study, wide local excision under the proper diagnosis is the most important treatment.

Myxofibrosarcoma and Undifferentiated Pleomorphic Sarcoma

2017 ◽  
Author(s):  
Ann Y Lee
Keyword(s):  
Local Recurrence ◽  
Malignant Fibrous Histiocytoma ◽  
Fibrous Histiocytoma ◽  
Distant Metastases ◽  
Metastatic Potential ◽  
Low Grade ◽  
High Grade ◽  
Recurrence Rates ◽  
Undifferentiated Pleomorphic Sarcoma ◽  
Pleomorphic Sarcoma

Both myxofibrosarcoma and undifferentiated pleomorphic sarcoma (UPS) are sarcomas that primarily occur in the extremities of older patients. UPS (previously known as malignant fibrous histiocytoma) is now considered a diagnosis of exclusion, representing a group of high-grade, heterogeneous tumors with no clear line differentiation and characterized by high rates of distant metastases. Myxofibrosarcoma (formerly myxoid variant of malignant fibrous histiocytoma) is a distinct histologic entity that is largely distinguished from UPS by its myxoid stroma and curvilinear vascular pattern. Myxofibrosarcoma tends to have a locally infiltrative behavior that can often be seen as T2 hyperintense “tails” on magnetic resonance imaging. Local recurrence rates can be as high as 61% and are independent of grade. Unlike other sarcomas, it is recommended that tumors be excised with at least a 2 cm margin around both clinically and radiographically evident tumor. Low-grade tumors do not tend to metastasize but can recur as high-grade tumors with increased metastatic potential. Surgery is the mainstay of treatment for localized disease. For high-grade tumors, this may require complex plastics and vascular reconstruction. Selecting cases for multimodality therapy, including radiation and chemotherapy, should be done on a case-by-case basis after review by a multidisciplinary sarcoma team with particular attention to a patient’s risk of local recurrence and distant metastases. Further studies into histology-specific therapeutic targets will help inform future clinical trials. This review contains 4 figures, 4 tables and 54 references Key words: chemotherapy, distant metastases, extremity sarcoma, local recurrence, malignant fibrous histiocytoma, myxofibrosarcoma, radiation, sarcoma, surgical margins, T2 hyperintense tails, undifferentiated pleomorphic sarcoma

A Report of a Rare Case of Myxoinflammatory Fibroblastic Sarcoma

2010 ◽  
Vol 100 (6) ◽  
pp. 497-501 ◽  
Author(s):  
Antonio Córdoba-Fernández ◽  
José María Juárez-Jiménez ◽  
José Mazuecos-Blanca ◽  
Matilde Illanes-Moreno
Keyword(s):  
Soft Tissue ◽  
Rare Case ◽  
Soft Tissues ◽  
Low Grade ◽  
Rare Neoplasm ◽  
Plantar Ulcer ◽  
Subcutaneous Tissues ◽  
Hands And Feet ◽  
Malignant Soft Tissue ◽  
Myxoinflammatory Fibroblastic Sarcoma

Myxoinflammatory fibroblastic sarcoma of the soft tissues is a rare low-grade tumor of uncertain origin that most often occurs on the extremities of adults. The tumor predominantly involves the subcutaneous tissues of the hands and feet. Despite being a rare neoplasm, owing to its varied histologic appearance, myxoinflammatory fibroblastic sarcoma should be differentiated from various benign and malignant soft-tissue lesions. Myxoinflammatory fibroblastic sarcoma has been well described in pathology journals but not in the surgical literature. We report a case of myxoinflammatory fibroblastic sarcoma in a 19-year-old man with a plantar ulcer lesion in his left foot. To our knowledge, this is the first reported case in the literature involving the epidermis. (J Am Podiatr Med Assoc 100(6): 497–501, 2010)

Myxofibrosarcoma and Undifferentiated Pleomorphic Sarcoma

2017 ◽  
Author(s):  
Ann Y Lee
Keyword(s):  
Local Recurrence ◽  
Malignant Fibrous Histiocytoma ◽  
Fibrous Histiocytoma ◽  
Distant Metastases ◽  
Metastatic Potential ◽  
Low Grade ◽  
High Grade ◽  
Recurrence Rates ◽  
Undifferentiated Pleomorphic Sarcoma ◽  
Pleomorphic Sarcoma

Both myxofibrosarcoma and undifferentiated pleomorphic sarcoma (UPS) are sarcomas that primarily occur in the extremities of older patients. UPS (previously known as malignant fibrous histiocytoma) is now considered a diagnosis of exclusion, representing a group of high-grade, heterogeneous tumors with no clear line differentiation and characterized by high rates of distant metastases. Myxofibrosarcoma (formerly myxoid variant of malignant fibrous histiocytoma) is a distinct histologic entity that is largely distinguished from UPS by its myxoid stroma and curvilinear vascular pattern. Myxofibrosarcoma tends to have a locally infiltrative behavior that can often be seen as T2 hyperintense “tails” on magnetic resonance imaging. Local recurrence rates can be as high as 61% and are independent of grade. Unlike other sarcomas, it is recommended that tumors be excised with at least a 2 cm margin around both clinically and radiographically evident tumor. Low-grade tumors do not tend to metastasize but can recur as high-grade tumors with increased metastatic potential. Surgery is the mainstay of treatment for localized disease. For high-grade tumors, this may require complex plastics and vascular reconstruction. Selecting cases for multimodality therapy, including radiation and chemotherapy, should be done on a case-by-case basis after review by a multidisciplinary sarcoma team with particular attention to a patient’s risk of local recurrence and distant metastases. Further studies into histology-specific therapeutic targets will help inform future clinical trials. This review contains 4 figures, 4 tables and 54 references Key words: chemotherapy, distant metastases, extremity sarcoma, local recurrence, malignant fibrous histiocytoma, myxofibrosarcoma, radiation, sarcoma, surgical margins, T2 hyperintense tails, undifferentiated pleomorphic sarcoma

scholarly journals Review of thirty patients with bone chondroblastoma

2005 ◽  
Vol 62 (10) ◽  
pp. 705-713 ◽  
Author(s):  
Slobodan Slavkovic ◽  
Slavko Tomic ◽  
Nemanja Slavkovic ◽  
Milan Apostolovic
Keyword(s):  
Local Recurrence ◽  
Malignant Transformation ◽  
Soft Tissues ◽  
Diagnostic Procedures ◽  
Proximal Part ◽  
Medical Documentation ◽  
Radiographic Findings ◽  
High Local Recurrence Rate ◽  
Physical Examinations ◽  
Rare Lesion

Background/Aim. To analyse the results of the treatment of the patients with the diagnosis of chondroblastoma, to confirm the possible malignancy and to recommend the best and the safest method of the treatment. Methods. We reviewed the cases of 30 patients with chondroblastoma who were treated between 1975 and 2004. Data were obtained using complete medical documentation, physical examinations, radiographic findings, and the available additional diagnostic procedures. Results. We found that the proximal part of the tibia, proximal part of the humerus, and distal part of the femur were the most common sites of the tumor in 63% of the cases. The higher prevalence of chondroblastoma in male patients was found, especially in the second decade of life. The patients were treated with different surgical procedures after histologically confirmed chondroblastoma. In 1 of the patients, radiation therapy was performed because the lesion recurred, after which the malignant transformation of chondroblastoma occurred. We found two more malignant chondroblastomas, one of which had been diagnosed as a primary tumor. Seven patients had a local recurrence, one of them had a second recurrence and the malignant transformation of chondroblastoma. The only solution was a below knee amputation. Conclusion. Chondroblastoma of bone is a rare lesion with the high local recurrence rate. We emphasized the need for an adequate and rapid diagnosis, including histological verification. The treatment was strictly surgical. The basic goal of the treatment was to avoid tumor penetration into articular cavity and/or local soft tissues. Malignant chondroblastoma of bone should be treated with radical surgical resection, avoiding any adjuvant therapy.

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