Lipofibromatosis-like neural tumor (LPF-NT) is an extremely rare soft tissue tumor first described by Agaram et al in 2016.[1] As of mid-2021, at least 39 cases of LPF-NT have been reported in the literature.[2][3][4][5] LPF-NT tumors have several features that resemble lipofibromatosis (LPF) tumors,[6]malignant peripheral nerve sheath tumors, spindle cell sarcomas,[3] low-grade neural tumors, peripheral nerve sheath tumors, and other less clearly defined tumors;[1] 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[4] 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.[7]
LPF-NTs are locally invasive, are commonly treated by surgical excision, and have a relatively high rate of local recurrence if their surgical excisions are incomplete.[8] They are generally considered to be benign, non-metastasizing (i.e. not spreading to other parts of the body) tumors.[6][8] However, one case of LFT-NT reported by Agaram et al was associated with metastasis, apparently as a result of the tumor's cells transformation into a malignant sarcoma. Further studies are needed to determine the frequency of such cases and the overall metastatic potential of LPF-NT.[1]
LPF-NTs were given the "neural tumor" terminology because in at least some cases: 1) their tumor cells express S100 and CD34 but not SOX10 proteins, a pattern that is often found in neural[7] and neuroectodermal tumor cells;[1] and 2) their histopathology consists of tumor cell infiltrations into adipose tissues in a pattern that is very similar to that found in some low grademalignant peripheral nerve sheath tumors.[1]
^Crumbach L, Descotes F, Bringuier PP, Poulalhon N, Balme B, Juliet T, Lopez J, Harou O (November 2020). "Lipofibromatosis-Like Neural Tumor: A Case Report and Review of the Literature". The American Journal of Dermatopathology. 42 (11): 881–884. doi:10.1097/DAD.0000000000001734. PMID32618702. S2CID220327572.
^ abPanse G, Reisenbichler E, Snuderl M, Wang WL, Laskin W, Jour G (February 2021). "LMNA-NTRK1 rearranged mesenchymal tumor (lipofibromatosis-like neural tumor) mimicking pigmented dermatofibrosarcoma protuberans". Journal of Cutaneous Pathology. 48 (2): 290–294. doi:10.1111/cup.13772. PMID32519338. S2CID219562066.
^Wang H, Chen L (December 2020). "[Clinicopathological and molecular genetic features of lipofibromatosis-like neural tumor]". Zhonghua Bing Li Xue Za Zhi = Chinese Journal of Pathology (in Chinese). 49 (12): 1272–1275. doi:10.3760/cma.j.cn112151-20200319-00231. PMID33287512.
^ abMalik F, Santiago T, Newman S, McCarville B, Pappo AS, Clay MR (June 2020). "An addition to the evolving spectrum of lipofibromatosis and lipofibromatosis-like neural tumor: Molecular findings in an unusual phenotype aid in accurate classification". Pathology, Research and Practice. 216 (6): 152942. doi:10.1016/j.prp.2020.152942. PMID32299759. S2CID215803522.
^ abLao IW, Sun M, Zhao M, Yu L, Wang J (August 2018). "Lipofibromatosis-like neural tumour: a clinicopathological study of ten additional cases of an emerging novel entity". Pathology. 50 (5): 519–523. doi:10.1016/j.pathol.2018.03.007. PMID29958731. S2CID49615346.