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What’s new in adipocytic neoplasia?

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Abstract

Adipocytic tumors are frequently encountered in routine practice, and while the vast majority represent commonly encountered tumor types (e.g., benign lipoma), the heterogeneity and rarity of other adipocytic neoplasms can pose diagnostic challenges. Atypical and malignant adipocytic tumors account for approximately 20% of all sarcomas. The 2013 World Health Organization (WHO) classification of soft tissue and bone tumors recognizes four major liposarcoma subtypes, characterized by distinct clinical behavior, distinctive morphologies, as well as unique genetic findings: atypical lipomatous tumor/well-differentiated liposarcoma, dedifferentiated liposarcoma, myxoid liposarcoma, and pleomorphic liposarcoma. Since the publication of the 2013 WHO classification of soft tissue and bone tumors, the most notable change in the category of adipocytic tumors has been made in the clinicopathologic and molecular characterization of the heterogeneous but distinct group of “atypical low-grade adipocytic neoplasms with spindle cell features,” for which the term atypical spindle cell/pleomorphic lipomatous tumor has been proposed. Another substantive change in the group of adipocytic tumors is the introduction of pleomorphic myxoid liposarcoma (myxoid pleomorphic liposarcoma) as an apparently novel subtype of aggressive liposarcoma, especially occurring in children and young adults with a predilection for the mediastinum. This review will further focus upon the diagnostic criteria of these novel emerging entities in the group of adipocytic tumors.

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References

  1. Fletcher CDM, Bridge JA, Hogendoorn PCW, Mertens F (2013) World Health Organization Classification of Tumours. Pathology and Genetics. In: Tumours of Soft Tissue and Bone. IARC, Lyon

    Google Scholar 

  2. Fletcher CDM (2014) The evolving classification of soft tissue tumours—an update based on the new 2013 WHO classification. Histopathology 64:2–11

    PubMed  Google Scholar 

  3. Dei Tos AP (2014) Liposarcomas: diagnostic pitfalls and new insights. Histopathology 64:38–52

    PubMed  Google Scholar 

  4. Coindre JM, Pédeutour F, Aurias A (2010) Well-differentiated and dedifferentiated liposarcomas. Virchows Arch 456:167–179

    CAS  PubMed  Google Scholar 

  5. Fletcher CDM, Unni KK, Mertens F (2002) World Health Organization Classification of Tumours. Pathology and Genetics. In: Tumours of Soft Tissue and Bone. IARC, Lyon

    Google Scholar 

  6. Marino-Enriquez A, Fletcher CDM, Dal Cin P, Hornick JL (2010) Dedifferentiated liposarcoma with “homologous” lipoblastic (pleomorphic liposarcoma-like) differentiation: clinicopathologic and molecular analysis of a series suggesting revised diagnostic criteria. Am J Surg Pathol 34:1122–1131

    PubMed  Google Scholar 

  7. Boland JM, Weiss SW, Oliveira AM, Erickson-Johnson ML, Folpe AL (2010) Liposarcomas with mixed well-differentiated and pleomorphic features: a clinicopathologic study of 12 cases. Am J Surg Pathol 34:837–843

    PubMed  Google Scholar 

  8. Dei Tos AP, Mentzel T, Newman PL, Fletcher CD (1994) Spindle cell liposarcoma, a hitherto unrecognized variant of liposarcoma. Analysis of six cases. Am J Surg Pathol 18:913–921

    CAS  PubMed  Google Scholar 

  9. Allen PW, Strungs I, MacCormac LB (1998) Atypical subcutaneous fatty tumors: a review of 37 referred cases. Pathology 30:123–135

    CAS  PubMed  Google Scholar 

  10. Cai YC, McMenamin ME, Rose G, Sandy CJ, Cree IA, Fletcher CD (2001) Primary liposarcoma of the orbit: a clinicopathologic study of seven cases. Ann Diagn Pathol 5:255–266

    CAS  PubMed  Google Scholar 

  11. Nascimento AF, McMenamin ME, Fletcher CD (2002) Liposarcomas/atypical lipomatous tumors of the oral cavity: a clinicopathologic study of 23 cases. Ann Diagn Pathol 6:83–93

    PubMed  Google Scholar 

  12. Mentzel T, Toenissen J, Rutten A, Schaller J (2005) Palmar atypical lipomatous tumor with spindle cell features (well-differentiated spindle cell liposarcoma): a rare neoplasm arising in an unusual anatomical location. Virchows Arch 446:300–304

    CAS  PubMed  Google Scholar 

  13. Nascimento AF, Fletcher CDM (2005) Spindle cell liposarcoma/atypical lipomatous tumor. A clinicopathologic study of 120 cases. Mod Pathol 8:70A

    Google Scholar 

  14. Marino-Enriquez A, Nascimento AF, Ligon AH, Liang C, Fletcher CDM (2017) Atypical spindle cell lipomatous tumor: clnicopathologic characterization of 232 cases demonstrating a morphologic spectrum. Am J Surg Pathol 41(2):234–244

    PubMed  Google Scholar 

  15. Creytens D, Mentzel T, Ferdinande L, Lecoutere E, van Gorp J, Atanesyan L, de Groot K, Savola S, Van Roy N, Van Dorpe J, Flucke U (2017) “Atypical” pleomorphic lipomatous tumor: a clinicopathologic, immunohistochemical and molecular study of 21 cases, emphasizing its relationship to atypical spindle cell lipomatous tumor and suggesting a morphologic spectrum (‘atypical spindle cell/pleomorphic lipomatous tumor’). Am J Surg Pathol 41(11):1443–1455

    PubMed  Google Scholar 

  16. Alaggio R, Coffin CM, Weiss SW, Bridge JA, Issakov J, Oliveira AM, Folpe AL (2009) Liposarcomas in young patients: a study of 82 cases occurring in patients younger than 22 years of age. Am J Surg Pathol 33(5):645–658

    PubMed  Google Scholar 

  17. Mentzel T, Palmedo G, Kuhnen C (2010) Well-differentiated spindle cell liposarcoma (‘atypical spindle cell lipomatous tumor’) does not belong to the spectrum of atypical lipomatous tumor but has a close relationship to spindle cell lipoma: clinicopathologic, immunohistochemical analysis of six cases. Mod Pathol 23(5):729–736

    CAS  PubMed  Google Scholar 

  18. Deyrup AT, Chibon F, Guillou L, Lagarde P, Coindre JM, Weiss SW (2013) Fibrosarcoma-like lipomatous neoplasm: a reappraisal of so-called spindle cell liposarcoma defining a unique lipomatous tumor unrelated to other liposarcomas. Am J Surg Pathol 37(9):1373–1378

    PubMed  Google Scholar 

  19. Creytens D, van Gorp J, Savola S, Ferdinande L, Mentzel T, Libbrecht L (2014) Atypical spindle cell lipoma: a clinicopathologic, immunohistochemical, and molecular study emphasizing its relationship to classical spindle cell lipoma. Virchows Arch 465(1):97–108

    CAS  PubMed  Google Scholar 

  20. Bahadir B, Behzatoglu K, Hacihasanoglu E, Koca SB, Sigirci BB, Tokat F (2018) Atypical spindle cell/pleomorphic lipomatous tumor: a clinicopathologic, immunohistochemical, and molecular study of 20 cases. Pathol Int 68(10):550–556

    CAS  PubMed  Google Scholar 

  21. McCarthy AJ, Chetty R (2018) Tumours of fat are no longer a simple diagnosis: an overview of fatty tumours with a spindle cell component. J Clin Pathol 71(6):483–492

    CAS  PubMed  Google Scholar 

  22. Creytens D, Mentzel T, Ferdinande L, van Gorp J, Van Dorpe J, Flucke U (2018) Atypical multivacuolated lipoblasts and atypical mitoses are not compatible with the diagnosis of spindle cell/pleomorphic lipoma. Hum Pathol 74:188–189

    PubMed  Google Scholar 

  23. Creytens D, Mentzel T, Ferdinande L, van Gorp J, Van Dorpe J, Flucke U (2018) Atypical mitoses are present in otherwise classical pleomorphic lipomas—reply. Hum Pathol 81:300–302

    PubMed  Google Scholar 

  24. Creytens D, Mentzel T, Ferdinande L, van Gorp J, Van Dorpe J, Flucke U (2019) “Fat-rich” (spindle cell-poor) variants of atypical spindle cell lipomatous tumors show similar morphologic, immunohistochemical and molecular features as “dysplastic lipomas”: are they related lesions? Comment on Michal et al (2018). Am J Surg Pathol 43(2):288–289

    PubMed  Google Scholar 

  25. Creytens D, Mentzel T, Ferdinande L, van Gorp J, Van Dorpe J, Flucke U (2019) “Fat-rich” (spindle cell-poor) variant of atypical spindle cell/pleomorphic lipomatous tumor: striking mimic of “classical” atypical lipomatous tumor/well-differentiated liposarcoma. Int J Surg Pathol Epub ahead of print:106689691984656

  26. Michal M, Agaimy A, Contreras AL, Svajdler M, Kazakov DV, Steiner P, Grossmann P, Martinek P, Hadravsky L, Michalova K, Svajdler P, Szep Z, Michal M, Fetsch JF (2018) Dysplastic lipoma: a distinctive atypical lipomatous neoplasm with anisocytosis, focal nuclear atypia, p53 overexpression, and a lack of MDM2 gene amplification by FISH: a report of 66 cases demonstrating occasional multifocality and a rare association with retinoblastoma. Am J Surg Pathol 4211:1530–1540

    Google Scholar 

  27. Agaimy A (2017) Anisometric cell lipoma: insight from a case series and review of the literature on adipocytic neoplasms in survivors of retinoblastoma suggest a role for RB1 loss and possible relationship to fat-predominant (“fat-only”) spindle cell lipoma. Ann Diagn Pathol 29:52–56

    PubMed  Google Scholar 

  28. Evans HL (2015) Subcutaneous minimally atypical lipomatous tumors with variable fat cell size—a study of 13 cases. Mod Pathol 28(suppl2):17A

    Google Scholar 

  29. Thway K (2019) Well-differentiated liposarcoma and dedifferentiated liposarcoma: an updated review. Sem Diagn Pathol 36:112–121

    Google Scholar 

  30. Creytens D, Ferdinande L, van Gorp J, Van Dorpe J, Flucke U (2019) Atypical spindle cell lipomatous tumor with benign heterologous (metaplastic) cartilaginous differentiation. Int J Surg Pathol 27:521–523

    PubMed  Google Scholar 

  31. Italiano A, Chambonniere ML, Attias R, Chibon F, Coindre JM, Pedeutour F (2008) Monosomy 7 and absence of 12q amplification in two cases of spindle cell liposarcomas. Cancer Genet Cytogenet 184(2):99–104

    CAS  PubMed  Google Scholar 

  32. Schaefer IM, Hornick JL (2018) Diagnostic immunohistochemistry for soft tissue and bone tumors: an update. Adv Anat Pathol 25:400–412

    CAS  PubMed  PubMed Central  Google Scholar 

  33. Schaefer IM, Fletcher CDM (2018) Recent advances in the diagnosis of soft tissue tumours. Pathology 50(1):37–48

    PubMed  Google Scholar 

  34. Coffin CM, Alaggio R (2012) Adipose and myxoid tumors of childhood and adolescence. Pediatr Dev Pathol 15(1 Suppl):239–254

    PubMed  Google Scholar 

  35. Boland JM, Colby TV, Folpe AP (2012) Liposarcomas of the mediastinum and thorax: a clinicopathologic and molecular cytogenetic study of 24 cases, emphasizing unusual and diverse histologic features. Am J Surg Pathol 36(9):1395–1403

    PubMed  Google Scholar 

  36. Creytens D, van Gorp J, Ferdinande L, Van Roy N, Libbrecht L (2014) Array-based comparative genomic hybridization analysis of a pleomorphic myxoid liposarcoma. J Clin Pathol 67(9):834–835

    PubMed  Google Scholar 

  37. Anderson WJ, Jo VY (2019) Pleomorphic liposarcoma: updates and current differential diagnosis. Semin Diagn Pathol 36:122–128

    PubMed  Google Scholar 

  38. Putra J, Al-Ibraheemi A (2019) Adipocytic tumors in children: a contemporary review. Semin Diagn Pathol 36:95–104

    PubMed  Google Scholar 

  39. Creytens D (2019) A contemporary review of myxoid adipocytic tumors. Semin Diagn Pathol 36:129–141

    PubMed  Google Scholar 

  40. Sinclair TJ, Thorson CM, Alvarez E, Tan S, Spunt SL, Chao SD (2017) Pleomorphic myxoid liposarcoma in an adolescent with li-Fraumeni syndrome. Pediatr Surg Int 33(5):631–635

    PubMed  Google Scholar 

  41. Hofvander J, Jo VY, Ghanei I, Gisselsson D, Martensson E, Mertens F (2016) Comprehensive genetic analysis of a paediatric pleomorphic myxoid liposarcoma reveals near-haploidization and loss of the RB1 gene. Histopathology 69(1):141–147

    PubMed  Google Scholar 

  42. Demicco EG (2019) Molecular updates in adipocytic neoplasms. Sem Diagn Pathol 36:85–94

    Google Scholar 

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  1. Department of Pathology, Ghent University and Ghent University Hospital, C. Heymanslaan 10,, B-9000, Ghent, Belgium

    David Creytens

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  1. David Creytens
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Correspondence to David Creytens.

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Creytens, D. What’s new in adipocytic neoplasia?. Virchows Arch 476, 29–39 (2020). https://doi.org/10.1007/s00428-019-02652-3

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