Myxofibrosarcoma

Last updated
Myxofibrosarcoma
Specialty Dermatology, Dermatopathology, Pathology, Surgical oncology, Oncology
TypesEpithelioid myxofibrosarcoma
CausesUnknown
Prognosis Guarded

Myxofibrosarcoma (MFS), although a rare type of tumor, is one of the most common soft tissue sarcomas, i.e. cancerous tumors, that develop in the soft tissues of elderly individuals. [1] Initially considered to be a type of histiocytoma termed fibrous histiocytoma or myxoid variant of malignant fibrous histiocytoma, [2] Angervall et al. termed this tumor myxofibrosarcoma in 1977. [3] In 2020, the World Health Organization reclassified MFS as a separate and distinct tumor [4] in the category of malignant fibroblastic and myofibroblastic tumors. [5]

Contents

MFS tumors are often treated by surgical resection. However, these tumors have high recurrence rates at the sites of their resections. [6] Local recurrences followed by surgical resections may be repeated multiple times but during these cycles MFS tumors often progress from a lower grade to a higher more aggressive grade, metastasize, and become life-threatening. [4] An uncommon variant of the MFS tumors termed epithelioid myxofibrosarcoma is even more likely to follow an aggressive, recurrent, metastasizing, and life-threatening course than the more common form of the MFS tumors. [6]

Presentation

MFS usually afflicts individuals in their fifth to seventh decades of life although uncommon cases occur in adults outside of this age range [7] In one large study, MFS was diagnosed in individuals 21 to 96 years old (median age, 66 years old). [4] Most studies have diagnosed MFS slightly more often in men than women [7] but one large study conducted in France found it to be 50% more common in men. [8] Individuals with MFS present with a tumor located in an extremity (77% of cases, usually in the lower extremity), trunk (12% of cases), and head and neck areas (3% of cases). [7] Rarely, these tumors have presented in the breast, heart, paratesticular region (i.e. area inside the scrotum including the epididymis the spermatic cord along with its coverings), [8] eye, [9] bone, liver, or multiple sites concurrently. [10] Primary tumors presenting in the abdominal cavity, retroperitoneum, or pelvis have been diagnosed as MFS [6] but larger studies indicate that these tumors are far more likely to be dedifferentiated liposarcomas. [6] [11] MFS tumors usually develop as painless, slowly enlarging masses in a muscle, skin (usually below the fascia i.e. a sheet of connective tissue, primarily collagen, running beneath the skin), or one of the non-cutaneous areas described above. [7] In one study of 69 FBS cases 36 were <5 cm, 23 were between 5 and 10 cm, and 19 were > 10 cm in diameter with the largest tumor being 27 cm. [12] MFS tumors often infiltrate along vascular and fascial planes, are incompletely removed at surgery, and consequently recur at the surgical site. [13] Recurrences of the MFS at the site of surgery have developed in 16% to 57% of patients with a significant proportion (25%–52%) recurring multiple times. [7] In one study, recurrences developed between 2 and 82 months (median 53 months) following primary surgery and metastatic disease developed in 23% of patients within 2 to 77 months (median 10 months) following primary surgery. [4] Recurrent tumors tend to be more aggressive and have a much greater tendency to metastasize than primary MFS tumors. [4] In one study, metastatic disease was detected in 23% of patients and occurred at a median of 10 months (range, 2–77 months) after resection of the primary tumor. In a review of multiple studies, the risk of developing metastases for lower grade MFS (defined in the following section) was <5% and for higher grade tumors was 25–30%. [11] MFS metastasize most commonly to the lungs, bone, and lymph-nodes. [4]

Individuals with the epithelioid variant of FBS generally present with a tumor in the limbs; the tumors tend to be somewhat larger, more aggressive, and more likely to metastasize than the tumors in non-variant cases. [7] [14] At least 50% of patients with this variant have developed metastases. [6] [11]

Pathology

The microscopic histopathology of hematoxylin and eosin stained FBS tumors varies. Lower-grade MFS tissues consist of scattered large, variability-sized and spindle-shaped-to-variably-shaped tumor cells with darkly stained nuclei. Overall, lower-grade tumors contain relatively few cells within a distinctive myxoid (i.e. more blue or purple compared to normal connective tissue because of excessive uptake of the hematoxylin stain) connective tissue background that contains curvilinear, thin-walled blood vessels. [4] Higher grade FBS tumors consist of relatively large sheets of these spindle-shaped/vatiably-shaped cells in a similar myxoid background containing thin-walled curvilinear blood vessels. [6] [4] Pseudo-lipoblasts (i.e. multivacuolated cells resembling lipoblasts but having vacuoles filled with mucin rather than lipids) are apparent in both lower grade and higher grade tumors. [4] Epithelioid FBS tumors are highly cellular lesions consisting of diffuse proliferations of extremely large, polygonal-shaped epithelioid cells set in a myxoid connective tissue background similar to that seen in the other types of FBS. [15] Epithelioid myxofibrosarcomas appear to behave more aggressively than myxofibrosarcomas dominated by spindle-shaped/variably-shaped cells. [7] While the cells in most types of tumors express specific marker proteins that help in diagnosing them, the tumor cells in FBS [4] and its epithelioid variant [14] have not yet been found to express marker proteins that are sufficiently specific to support either diagnosis.

Chromosome and gene abnormalities

Most cases of MFS have tumor cells that contain complex chromosome and/or gene abnormalities [7] including ring chromosomes (i.e. chromosome whose ends are fused together to form a ring), double minutes (i.e. small fragments of extrachromosomal DNA), chromosomes with deletions of part of their genetic material, and chromosome translocations (i.e. abnormal switches in genetic material between different chromosomes). There were no apparent differences in these abnormalities between lower grade and higher grade tumors but their numbers were higher and more prevalent in recurrent tumors. [16] These forms of chromosome/gene cytogenetic alterations are commonly found in various tumor types regardless of their grade or severity. [7] Tumor cell mutations or deletions in the NF1 gene occur ~10% of MFS cases while mutations in the CDKN2A/CDKN2B and amplifications in the CDK6, CCND1, and MDM2 genes occur in rare MFS cases. [1] While the cells in many tumor types express specific chromosome/gene abnormalities that help in determining their diagnoses, the cited chromosome and gene abnormalities discovered in TBS tumor cells have not yet been found specific enough to be of help in diagnosing MFS. [4] The chromosome/gene abnormalities have not yet been defined in the epithelioid variant of MFS.

Diagnosis

The diagnosis of MBS is heavily dependent on the presentation and histopathology of its tumors. [7] Of particular importance, the presence of pseudo-lipoblasts in a myxoid sarcoma-like background is an extremely strong indicator that the tumor is a MFS. [6] and tumors with a myxofibrosarcoma-like histopathology that initiate in the retroperitoneum, abdominal cavity, or pelvis are nearly always dedifferentiated liposarcomas. [6] [11] Magnetic resonance imaging (MRI) has been helpful in diagnosing MBS. On T2-weighted MRI, 81% of MFS tumors give a tail sign, i.e. a multidirectional signal spreading away from the main mass along a facial plane (i.e. a line or band of connective tissue). Among all myxoid-predominant tissue lesions, this MRI method diagnoses MBS with a specificity of 79% to 90%. This MRI finding is also extremely valuable for gauging the extent and depth of surgery needed to completely remove MBS tumors. [7]

Treatment and prognosis

The recommended treatment for individuals presenting with localized MFS tumors is radical surgical resection. [13] The resection should include a 2 cm margin of soft tissue surrounding the tumor, with planned resection of the entire area as defined by MRI-detected increased signals on T2-weighted images. This is done to ensure that all tumor tissue is removed in order to avoid the high risks of local recurrences and worsening prognoses. [7] Historically, about 10% of patients treated with radical surgery developed recurrences at the surgical site and a significant number of these patients developed metastatic disease. Consequently, adjuvant radiotherapy has been used to help reduce these recurrences and metastatic transformations. For patients with a limb tumor that cannot be resected (less than 5% of all cases)), limb amputation is the treatment of choice. [7] Recurrent and metastatic MFS first-line treatment has employed two chemotherapy drugs, anthracycline and Ifosfamide, while second-line treatment has employed two other chemotherapy drugs, gemcitabine and paclitaxel. [17] Triple chemotherapy drug treatment (adriamycin, ifosfamide, and dacarbazine) has also been used to treat MFS. [18] However, there are no randomized clinical trials evaluating the efficacy of these or other chemotherapy drugs in the treatment of inoperable or metastatic MFS and none of the uncontrolled studies have demonstrated a benefit in improving the overall survival in MFS. [7] Current drug therapies that are or may soon be tried in treating MFS include angiogenesis inhibitors and immunotherapeutics such as Bevacizumab and Nivolumab. [17]

In one review of 109 individuals with MFS: overall survival for the entire group was 80% at 3 years and 76% at 5 years; local recurrence-free survival was 95% at 3 years and 88% at 5 years; median survival following local recurrence was 68 months; distant metastasis-free survival was 78% at 3 and 77% at 5 years; and 18 of 25 patients (72%) died of metastatic disease during a median follow-up time of 42 months for the overall review period of study. [4] In three large studies, overall 5 year disease-specific survival times (i.e. the percentage of patients surviving 5 years excluding death due to any other causes than MFS from this count) were 51%, 73%, and 96%. [7]

Related Research Articles

<span class="mw-page-title-main">Sarcoma</span> Medical condition

A sarcoma is a malignant tumor, a type of cancer that arises from cells of mesenchymal origin. Connective tissue is a broad term that includes bone, cartilage, fat, vascular, or other structural tissues, and sarcomas can arise in any of these types of tissues. As a result, there are many subtypes of sarcoma, which are classified based on the specific tissue and type of cell from which the tumor originates. Sarcomas are primary connective tissue tumors, meaning that they arise in connective tissues. This is in contrast to secondary connective tissue tumors, which occur when a cancer from elsewhere in the body spreads to the connective tissue. Sarcomas are one of five different types of cancer, classified by the cell type from which they originate. The word sarcoma is derived from the Greek σάρκωμα sarkōma 'fleshy excrescence or substance', itself from σάρξsarx meaning 'flesh'.

<span class="mw-page-title-main">Dermatofibrosarcoma protuberans</span> Medical condition

Dermatofibrosarcoma protuberans (DFSP) is a rare locally aggressive malignant cutaneous soft-tissue sarcoma. DFSP develops in the connective tissue cells in the middle layer of the skin (dermis). Estimates of the overall occurrence of DFSP in the United States are 0.8 to 4.5 cases per million persons per year. In the United States, DFSP accounts for between 1 and 6 percent of all soft-tissue sarcomas and 18 percent of all cutaneous soft-tissue sarcomas. In the Surveillance, Epidemiology and End Results (SEER) tumor registry from 1992 through 2004, DFSP was second only to Kaposi sarcoma.

<span class="mw-page-title-main">Liposarcoma</span> Medical condition

Liposarcomas are the most common subtype of soft tissue sarcomas, accounting for at least 20% of all sarcomas in adults. Soft tissue sarcomas are rare neoplasms with over 150 different histological subtypes or forms. Liposarcomas arise from the precursor lipoblasts of the adipocytes in adipose tissues. Adipose tissues are distributed throughout the body, including such sites as the deep and more superficial layers of subcutaneous tissues as well as in less surgically accessible sites like the retroperitoneum and visceral fat inside the abdominal cavity.

<span class="mw-page-title-main">Undifferentiated pleomorphic sarcoma</span> Medical condition

Undifferentiated pleomorphic sarcoma (UPS), also termed pleomorphic myofibrosarcoma, high-grade myofibroblastic sarcoma, and high-grade myofibrosarcoma, is characterized by the World Health Organization (WHO), 2020, as a rare, poorly differentiated neoplasm, i.e. an abnormal growth of cells that have an unclear identity and/or cell of origin. WHO classified it as one of the undifferentiated/unclassified sarcomas in the category of tumors of uncertain differentiation. Sarcomas are cancers known or thought to derive from mesenchymal stem cells that typically develop in bone, muscle, fat, blood vessels, lymphatic vessels, tendons, and ligaments. More than 70 sarcoma subtypes have been described. The UPS subtype of these sarcomas consists of tumor cells that are poorly differentiated and may appear as spindle-shaped cells, histiocytes, and giant cells. UPS is considered a diagnosis that defies formal sub-classification after thorough histologic, immunohistochemical, and ultrastructural examinations fail to identify the type of cells involved.

<span class="mw-page-title-main">Mesoblastic nephroma</span> Medical condition

Congenital mesoblastic nephroma, while rare, is the most common kidney neoplasm diagnosed in the first three months of life and accounts for 3-5% of all childhood renal neoplasms. This neoplasm is generally non-aggressive and amenable to surgical removal. However, a readily identifiable subset of these kidney tumors has a more malignant potential and is capable of causing life-threatening metastases. Congenital mesoblastic nephroma was first named as such in 1967 but was recognized decades before this as fetal renal hamartoma or leiomyomatous renal hamartoma.

The Ewing family of tumors (EFTs) is a group of small cell sarcomas including Ewing sarcoma of the bone, extra osseous Ewing tumors, and primitive neuroectodermal tumors. They are rare cancers, usually diagnosed in peoples' twenties. The sarcoma of bone is the most common of the variants. All forms are predisposed to metastasis and have had historically high rates of mortality. The family of tumors shares a common translocation mutation of the EWS gene on chromosome 22 to an ETS-type gene, most commonly the FLI1 gene. EFTs are highly malignant, with 5-year survival for patients with metastatic disease at 20%. The current standard of care includes resection, radiation, and chemotherapy.

<span class="mw-page-title-main">Epithelioid sarcoma</span> Medical condition

Epithelioid sarcoma is a rare soft tissue sarcoma arising from mesenchymal tissue and characterized by epithelioid-like features. It accounts for less than 1% of all soft tissue sarcomas. It was first definitively characterized by F.M. Enzinger in 1970. It commonly presents itself in the distal limbs of young adults as a small, soft mass or a cluster of bumps. A proximal version has also been described, frequently occurring in the upper extremities. Less commonly, cases are reported in the pelvis, vulva, penis, and spine.

Fibrous hamartoma of infancy (FHI) is a rare, typically painless, benign tumor that develops in the subcutaneous tissues of the axilla, arms, external genitalia, or, less commonly, various other areas. It is diagnosed in children who are usually less than 2 years old or, in up to 20% of cases, develops in utero and is diagnosed in an infant at birth.

Extraskeletal myxoid chondrosarcoma (EMC) is a rare low-grade malignant mesenchymal neoplasm of the soft tissues, that differs from other sarcomas by unique histology and characteristic chromosomal translocations. There is an uncertain differentiation and neuroendocrine differentiation is even possible.

Sharon Ann Whelan Weiss is an American pathologist who is best known for her contribution to the subspecialty of soft tissue pathology. She is the main author of Soft Tissue Tumors, one of the most widely used textbooks in the field of sarcoma and soft tissue pathology. She is also well known for her seminal descriptions of multiple soft tissue tumors, such as epithelioid hemangioendothelioma and pleomorphic hyalinizing angiectatic tumor of soft parts among others. She has also mentored and trained other well-known soft tissue pathologists.

<span class="mw-page-title-main">Low-grade fibromyxoid sarcoma</span> Medical condition

Low-grade fibromyxoid sarcoma (LGFMS) is a rare type of low-grade sarcoma first described by H. L. Evans in 1987. LGFMS are soft tissue tumors of the mesenchyme-derived connective tissues; on microscopic examination, they are found to be composed of spindle-shaped cells that resemble fibroblasts. These fibroblastic, spindle-shaped cells are neoplastic cells that in most cases of LGFMS express fusion genes, i.e. genes composed of parts of two different genes that form as a result of mutations. The World Health Organization (2020) classified LGFMS as a specific type of tumor in the category of malignant fibroblastic and myofibroblastic tumors.

<span class="mw-page-title-main">Inflammatory myofibroblastic tumour</span> Medical condition

Inflammatory myofibroblastic tumor (IMT) is a rare neoplasm of the mesodermal cells that form the connective tissues which support virtually all of the organs and tissues of the body. IMT was formerly termed inflammatory pseudotumor. Currently, however, inflammatory pseudotumor designates a large and heterogeneous group of soft tissue tumors that includes inflammatory myofibroblastic tumor, plasma cell granuloma, xanthomatous pseudotumor, solitary mast cell granuloma, inflammatory fibrosarcoma, pseudosarcomatous myofibroblastic proliferation, myofibroblastoma, inflammatory myofibrohistiocytic proliferation, and other tumors that develop from connective tissue cells. Inflammatory pseudotumour is a generic term applied to various neoplastic and non-neoplastic tissue lesions which share a common microscopic appearance consisting of spindle cells and a prominent presence of the white blood cells that populate chronic or, less commonly, acute inflamed tissues.

Acral myxoinflammatory fibroblastic sarcoma (AMSF), also termed myxoinflammatory fibroblastic sarcoma (MSF), is a rare, low-grade, soft tissue tumor that the World Health Organization (2020) classified as in the category of rarely metastasizing fibroblastic and myofibroblastic tumors. It is a locally aggressive neoplasm that often recurs at the site of its surgical removal. However, it usually grows slowly and in only 1–2% of cases spreads to distant tissues.

<span class="mw-page-title-main">Proliferative fasciitis and proliferative myositis</span> Medical condition

Proliferative fasciitis and proliferative myositis (PF/PM) are rare benign soft tissue lesions that increase in size over several weeks and often regress over the ensuing 1–3 months. The lesions in PF/PM are typically obvious tumors or swellings. Historically, many studies had grouped the two descriptive forms of PF/PM as similar disorders with the exception that proliferative fasciitis occurs in subcutaneous tissues while proliferative myositis occurs in muscle tissues. In 2020, the World Health Organization agreed with this view and defined these lesions as virtually identical disorders termed proliferative fasciitis/proliferative myositis or proliferative fasciitis and proliferative myositis. The Organization also classified them as one of the various forms of the fibroblastic and myofibroblastic tumors.

Lipofibromatosis-like neural tumor (LPF-NT) is an extremely rare soft tissue tumor first described by Agaram et al in 2016. As of mid-2021, at least 39 cases of LPF-NT have been reported in the literature. LPF-NT tumors have several features that resemble lipofibromatosis (LPF) tumors, malignant peripheral nerve sheath tumors, spindle cell sarcomas, low-grade neural tumors, peripheral nerve sheath tumors, and other less clearly defined tumors; Prior to the Agaram at al report, LPF-NTs were likely diagnosed as variants or atypical forms of these tumors. The analyses of Agaram at al and subsequent studies uncovered critical differences between LPF-NT and the other tumor forms which suggest that it is a distinct tumor entity differing not only from lipofibromatosis but also the other tumor forms.

Sclerosing epithelioid fibrosarcoma (SEF) is a very rare malignant tumor of soft tissues that on microscopic examination consists of small round or ovoid neoplastic epithelioid fibroblast-like cells, i.e. cells that have features resembling both epithelioid cells and fibroblasts. In 2020, the World Health Organization classified SEF as a distinct tumor type in the category of malignant fibroblastic and myofibroblastic tumors. However, current studies have reported that low-grade fibromyxoid sarcoma (LGFMS) has many clinically and pathologically important features characteristic of SEF; these studies suggest that LGSFMS may be an early form of, and over time progress to become, a SEF. Since the World Health Organization has classified LGFMS as one of the malignant fibroblastic and myofibroblastic tumors that is distinctly different than SEF, SEF and LGFMS are here regarded as different tumor forms.

The FET protein family consists of three similarly structured and functioning proteins. They and the genes in the FET gene family which encode them are: 1) the EWSR1 protein encoded by the EWSR1 gene located at band 12.2 of the long arm of chromosome 22; 2) the FUS protein encoded by the FUS gene located at band 16 on the short arm of chromosome 16; and 3) the TAF15 protein encoded by the TAF15 gene located at band 12 on the long arm of chromosome 7 The FET in this protein family's name derives from the first letters of FUS, EWSR1, and TAF15.

Angiofibroma of soft tissue (AFST), also termed angiofibroma, not otherwise specified, is a recently recognized and rare disorder that was classified in the category of benign fibroblastic and myofibroblastic tumors by the World Health Organization in 2020. An AFST tumor is a neoplasm that was first described by A. Mariño-Enríquez and C.D. Fletcher in 2012.

Dermatofibrosarcoma protuberans, fibrosarcomatous (DFSP-FS), also termed fibrosarcomatous dermatofibrosarcoma protuberans, is a rare type of tumor located in the dermis. DFSP-FS tumors have been viewed as: 1) a more aggressive form of the dermatofibrosarcoma protuberans (DFSP) tumors because they have areas that resemble and tend to behave like malignant fibrosarcomas or 2) as a distinctly different tumor than DFSP. DFSP-FS tumors are related to DFSP. For example, surgically removed DFSP tumors often recur with newly developed fibrobosarcoma-like areas. Nonetheless, the World Health Organization (WHO), 2020, classified DFSP and DFSP-FS as different tumors with DFSP being in the category of benign and DFSP-FS in the category of rarely metastasizing fibroblastic and myofibroblastic tumors. This article follows the WHO classification: the 5-15% of DFSP tumors that have any areas of fibrosarcomatous microscopic histopathology are here considered DFSP-FS rather than DFSP tumors.

Low-grade myofibroblastic sarcoma (LGMS) is a subtype of the malignant sarcomas. As it is currently recognized, LGMS was first described as a rare, atypical myofibroblastic tumor by Mentzel et al. in 1998. Myofibroblastic sarcomas had been divided into low-grade myofibroblastic sarcomas, intermediate‐grade myofibroblasic sarcomas, i.e. IGMS, and high‐grade myofibroblasic sarcomas, i.e. HGMS based on their microscopic morphological, immunophenotypic, and malignancy features. LGMS and IGMS are now classified together by the World Health Organization (WHO), 2020, in the category of intermediate fibroblastic and myofibroblastic tumors. WHO, 2020, classifies HGMS as a soft tissue tumor in the category of tumors of uncertain differentiation. This article follows the WHO classification: here, LGMS includes IGMS but not HGMS which is a more aggressive and metastasizing tumor than LGMS and consists of cells of uncertain origin.

References

  1. 1 2 Martínez-Trufero J, Cruz Jurado J, Gómez-Mateo MC, Bernabeu D, Floría LJ, Lavernia J, Sebio A, García Del Muro X, Álvarez R, Correa R, Hernández-León CN, Marquina G, Hindi N, Redondo A, Martínez V, Asencio JM, Mata C, Valverde Morales CM, Martin-Broto J (September 2021). "Uncommon and peculiar soft tissue sarcomas: Multidisciplinary review and practical recommendations for diagnosis and treatment. Spanish group for Sarcoma research (GEIS - GROUP). Part I". Cancer Treatment Reviews. 99: 102259. doi:10.1016/j.ctrv.2021.102259. ISSN   0305-7372. PMID   34311246.
  2. ↑ Weiss SW, Enzinger FM (April 1977). "Myxoid variant of malignant fibrous histiocytoma". Cancer. 39 (4): 1672–85. doi:10.1002/1097-0142(197704)39:4<1672::aid-cncr2820390442>3.0.co;2-c. PMID   192434. S2CID   29351227.
  3. ↑ Angervall L, Kindblom LG, Merck C (March 1977). "Myxofibrosarcoma. A study of 30 cases". Acta Pathologica et Microbiologica Scandinavica, Section A. 85A (2): 127–40. doi:10.1111/j.1699-0463.1977.tb00410.x. PMID   15396.
  4. 1 2 3 4 5 6 7 8 9 10 11 12 Gilg MM, Sunitsch S, Leitner L, Bergovec M, Szkandera J, Leithner A, Liegl-Atzwanger B (October 2020). "Tumor-associated mortality and prognostic factors in myxofibrosarcoma - A retrospective review of 109 patients". Orthopaedics & Traumatology, Surgery & Research. 106 (6): 1059–1065. doi: 10.1016/j.otsr.2020.04.017 . PMID   32778437. S2CID   225432867.
  5. ↑ Sbaraglia M, Bellan E, Dei Tos AP (April 2021). "The 2020 WHO Classification of Soft Tissue Tumours: news and perspectives". Pathologica. 113 (2): 70–84. doi:10.32074/1591-951X-213. PMC   8167394 . PMID   33179614.
  6. 1 2 3 4 5 6 7 8 Hornick JL (December 2018). "Subclassification of pleomorphic sarcomas: How and why should we care?". Annals of Diagnostic Pathology. 37: 118–124. doi:10.1016/j.anndiagpath.2018.10.006. PMID   30340082. S2CID   53008610.
  7. 1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 Roland CL, Wang WL, Lazar AJ, Torres KE (October 2016). "Myxofibrosarcoma". Surgical Oncology Clinics of North America. 25 (4): 775–88. doi:10.1016/j.soc.2016.05.008. PMID   27591498.
  8. 1 2 Amadeo B, Penel N, Coindre JM, Ray-Coquard I, Ligier K, Delafosse P, Bouvier AM, Plouvier S, Gallet J, Lacourt A, Coureau G, Monnereau A, Mathoulin-Pélissier S, Desandes E (March 2020). "Incidence and time trends of sarcoma (2000-2013): results from the French network of cancer registries (FRANCIM)". BMC Cancer. 20 (1): 190. doi: 10.1186/s12885-020-6683-0 . PMC   7059296 . PMID   32138705.
  9. ↑ Pujari A, Ali MJ, Honavar SG, Mittal R, Naik M (2014). "Orbital myxofibrosarcoma: a clinicopathologic correlation of an extremely rare tumor". Ophthalmic Plastic and Reconstructive Surgery. 30 (5): e111–3. doi:10.1097/IOP.0b013e3182a230cc. PMID   24833459.
  10. ↑ Sanfilippo R, Miceli R, Grosso F, Fiore M, Puma E, Pennacchioli E, Barisella M, Sangalli C, Mariani L, Casali PG, Gronchi A (March 2011). "Myxofibrosarcoma: prognostic factors and survival in a series of patients treated at a single institution". Annals of Surgical Oncology. 18 (3): 720–5. doi:10.1245/s10434-010-1341-4. PMID   20878245. S2CID   9711352.
  11. 1 2 3 4 Sioletic S, Dal Cin P, Fletcher CD, Hornick JL (January 2013). "Well-differentiated and dedifferentiated liposarcomas with prominent myxoid stroma: analysis of 56 cases". Histopathology. 62 (2): 287–93. doi:10.1111/j.1365-2559.2012.04348.x. PMID   23020289. S2CID   25393792.
  12. ↑ Scoccianti G, Ranucci V, Frenos F, Greto D, Beltrami G, Capanna R, Franchi A (July 2016). "Soft tissue myxofibrosarcoma: A clinico-pathological analysis of a series of 75 patients with emphasis on the epithelioid variant". Journal of Surgical Oncology. 114 (1): 50–5. doi:10.1002/jso.24250. PMID   27076198. S2CID   13082675.
  13. 1 2 Widemann BC, Italiano A (January 2018). "Biology and Management of Undifferentiated Pleomorphic Sarcoma, Myxofibrosarcoma, and Malignant Peripheral Nerve Sheath Tumors: State of the Art and Perspectives". Journal of Clinical Oncology. 36 (2): 160–167. doi:10.1200/JCO.2017.75.3467. PMC   5759316 . PMID   29220302.
  14. 1 2 Dey B, Srinivas BH, Badhe B, Nachiappa Ganesh R, Gochhait D, Toi PC, Jinkala S (December 2020). "Malignant Epithelioid Soft Tissue Tumours- A Pathologist's Perspective With Review of Literature". Cureus. 12 (12): e12263. doi: 10.7759/cureus.12263 . PMC   7834554 . PMID   33520482.
  15. ↑ Wakely PE (2021). "Cytopathology of myxofibrosarcoma: a study of 66 cases and literature review". Journal of the American Society of Cytopathology. 10 (3): 300–309. doi:10.1016/j.jasc.2020.09.004. PMID   33041221. S2CID   222299645.
  16. ↑ Willems SM, Debiec-Rychter M, Szuhai K, Hogendoorn PC, Sciot R (March 2006). "Local recurrence of myxofibrosarcoma is associated with increase in tumour grade and cytogenetic aberrations, suggesting a multistep tumour progression model". Modern Pathology. 19 (3): 407–16. doi: 10.1038/modpathol.3800550 . PMID   16415793.
  17. 1 2 Song L, Pan D, Zhou R (November 2020). "Combination nivolumab and bevacizumab for metastatic myxofibrosarcoma: A case report and review of the literature". Molecular and Clinical Oncology. 13 (5): 54. doi:10.3892/mco.2020.2124. PMC   7468213 . PMID   32905214.
  18. ↑ Look Hong NJ, Hornicek FJ, Raskin KA, Yoon SS, Szymonifka J, Yeap B, Chen YL, DeLaney TF, Nielsen GP, Mullen JT (January 2013). "Prognostic factors and outcomes of patients with myxofibrosarcoma". Annals of Surgical Oncology. 20 (1): 80–6. doi:10.1245/s10434-012-2572-3. PMC   3837421 . PMID   22890594.
This page is based on this Wikipedia article
Text is available under the CC BY-SA 4.0 license; additional terms may apply.
Images, videos and audio are available under their respective licenses.