Primitive myxoid mesenchymal tumor of infancy, an enigmatic entity: A case report
Primitive myxoid mesenchymal tumor of infancy (PMMTI) is a rare soft tissue neoplasm of intermediate malignant potential occurring predominantly in infants and younger children. It can show aggressive local infiltration of surrounding structures and frequent relapses but rare metastasis. Morphologic...
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| Format: | Article |
| Language: | English |
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Wolters Kluwer Medknow Publications
2024-12-01
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| Series: | Indian Journal of Pathology and Microbiology |
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| Online Access: | https://journals.lww.com/10.4103/ijpm.ijpm_471_23 |
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| author | Malini Goswami |
| author_facet | Malini Goswami |
| author_sort | Malini Goswami |
| collection | DOAJ |
| description | Primitive myxoid mesenchymal tumor of infancy (PMMTI) is a rare soft tissue neoplasm of intermediate malignant potential occurring predominantly in infants and younger children. It can show aggressive local infiltration of surrounding structures and frequent relapses but rare metastasis. Morphologically, it is characterized by a proliferation of immature mesenchymal cells in a myxoid background with mild cytologic atypia and branching vessels. The tumor cells demonstrate immunoreactivity with vimentin and BCOR, however, are negative for more specific lineage markers. Its molecular hallmark is BCOR-internal tandem duplication. Previously classified as infantile fibrosarcoma/undifferentiated sarcoma, the association with BCOR-ITD has led to its reclassification as part of a distinct entity—”Sarcoma with BCOR Genetic Alterations”—in the 2020 WHO Classification of Soft Tissue Tumours. Here is a report of a case of a 2-year-old boy with a recurrent scalp mass identified as a primitive myxoid mesenchymal tumor of infancy. |
| format | Article |
| id | doaj-art-c39be88cea2c48cd95fda44cefb6cde6 |
| institution | Kabale University |
| issn | 0377-4929 0974-5130 |
| language | English |
| publishDate | 2024-12-01 |
| publisher | Wolters Kluwer Medknow Publications |
| record_format | Article |
| series | Indian Journal of Pathology and Microbiology |
| spelling | doaj-art-c39be88cea2c48cd95fda44cefb6cde62025-01-10T10:28:42ZengWolters Kluwer Medknow PublicationsIndian Journal of Pathology and Microbiology0377-49290974-51302024-12-0167493293510.4103/ijpm.ijpm_471_23Primitive myxoid mesenchymal tumor of infancy, an enigmatic entity: A case reportMalini GoswamiPrimitive myxoid mesenchymal tumor of infancy (PMMTI) is a rare soft tissue neoplasm of intermediate malignant potential occurring predominantly in infants and younger children. It can show aggressive local infiltration of surrounding structures and frequent relapses but rare metastasis. Morphologically, it is characterized by a proliferation of immature mesenchymal cells in a myxoid background with mild cytologic atypia and branching vessels. The tumor cells demonstrate immunoreactivity with vimentin and BCOR, however, are negative for more specific lineage markers. Its molecular hallmark is BCOR-internal tandem duplication. Previously classified as infantile fibrosarcoma/undifferentiated sarcoma, the association with BCOR-ITD has led to its reclassification as part of a distinct entity—”Sarcoma with BCOR Genetic Alterations”—in the 2020 WHO Classification of Soft Tissue Tumours. Here is a report of a case of a 2-year-old boy with a recurrent scalp mass identified as a primitive myxoid mesenchymal tumor of infancy.https://journals.lww.com/10.4103/ijpm.ijpm_471_23mesenchymalmyxoidprimitivetumor |
| spellingShingle | Malini Goswami Primitive myxoid mesenchymal tumor of infancy, an enigmatic entity: A case report Indian Journal of Pathology and Microbiology mesenchymal myxoid primitive tumor |
| title | Primitive myxoid mesenchymal tumor of infancy, an enigmatic entity: A case report |
| title_full | Primitive myxoid mesenchymal tumor of infancy, an enigmatic entity: A case report |
| title_fullStr | Primitive myxoid mesenchymal tumor of infancy, an enigmatic entity: A case report |
| title_full_unstemmed | Primitive myxoid mesenchymal tumor of infancy, an enigmatic entity: A case report |
| title_short | Primitive myxoid mesenchymal tumor of infancy, an enigmatic entity: A case report |
| title_sort | primitive myxoid mesenchymal tumor of infancy an enigmatic entity a case report |
| topic | mesenchymal myxoid primitive tumor |
| url | https://journals.lww.com/10.4103/ijpm.ijpm_471_23 |
| work_keys_str_mv | AT malinigoswami primitivemyxoidmesenchymaltumorofinfancyanenigmaticentityacasereport |