# Undifferentiated High-Grade Pleomorphic Sarcoma of the Posterior Mandible: A Report of a Rare Case

**Authors:** Nivetha D., Christeffi Mabel, Abimathi R., Varshaa Ramesh

PMC · DOI: 10.7759/cureus.101947 · Cureus · 2026-01-20

## TL;DR

A rare case of a high-grade jaw tumor was initially mistaken for a benign lesion, highlighting the need for thorough testing and imaging for accurate diagnosis.

## Contribution

The paper presents a rare case emphasizing the diagnostic challenges of UPS mimicking benign lesions in the mandible.

## Key findings

- UPS can mimic benign lesions like CGCG on limited biopsies.
- Aggressive imaging features and extended immunohistochemistry are critical for accurate diagnosis.
- Adequate tissue sampling and multidisciplinary evaluation are essential for destructive mandibular lesions.

## Abstract

Undifferentiated pleomorphic sarcoma (UPS) is a high-grade mesenchymal malignancy that can closely resemble benign giant-cell-rich lesions such as central giant cell granuloma (CGCG), especially on limited biopsies. We report a case of a female patient with an enlarging mandibular swelling and an expansile osteolytic lesion that was initially diagnosed as CGCG based on an incisional biopsy showing multinucleated giant cells in a fibroblastic stroma. However, extended immunohistochemistry and the aggressive radiologic features supported a revised diagnosis of giant-cell-rich variant UPS. This case highlights the potential for diagnostic confusion on insufficient samples and emphasizes the importance of adequate tissue sampling, correlation with imaging, and comprehensive ancillary testing when evaluating destructive mandibular lesions.

## Linked entities

- **Diseases:** undifferentiated pleomorphic sarcoma (MONDO:0002142), central giant cell granuloma (MONDO:0006770)

## Full-text entities

- **Genes:** SATB2 (SATB homeobox 2) [NCBI Gene 23314] {aka C2DELq32q33, DEL2Q32Q33, GLSS}, MUC1 (mucin 1, cell surface associated) [NCBI Gene 4582] {aka ADMCKD, ADMCKD1, ADTKD2, CA 15-3, CD227, Ca15-3}, CMPK1 (cytidine/uridine monophosphate kinase 1) [NCBI Gene 51727] {aka CK, CMK, CMPK, UMK, UMP-CMPK, UMPK}, DES (desmin) [NCBI Gene 1674] {aka CDCD3, CSM1, CSM2, LGMD1D, LGMD1E, LGMD2R}, KIT (KIT proto-oncogene, receptor tyrosine kinase) [NCBI Gene 3815] {aka C-Kit, CD117, MASTC, PBT, SCFR}, MYOG (myogenin) [NCBI Gene 4656] {aka MYF4, bHLHc3, myf-4}, S100A1 (S100 calcium binding protein A1) [NCBI Gene 6271] {aka S100, S100-alpha, S100A}, TP63 (tumor protein p63) [NCBI Gene 8626] {aka AIS, B(p51A), B(p51B), EEC3, KET, LMS}, ALK (ALK receptor tyrosine kinase) [NCBI Gene 238] {aka ALK1, CD246, NBLST3}, TP53 (tumor protein p53) [NCBI Gene 7157] {aka BCC7, BMFS5, LFS1, P53, TRP53}, SMN1 (survival of motor neuron 1, telomeric) [NCBI Gene 6606] {aka BCD541, GEMIN1, SMA, SMA1, SMA2, SMA3}, CD34 (CD34 molecule) [NCBI Gene 947], F13A1 (coagulation factor XIII A chain) [NCBI Gene 2162] {aka F13A}, CD68 (CD68 molecule) [NCBI Gene 968] {aka GP110, LAMP4, SCARD1}, PGR (progesterone receptor) [NCBI Gene 5241] {aka NR3C3, PR}, VIM (vimentin) [NCBI Gene 7431], CETN1 (centrin 1) [NCBI Gene 1068] {aka CEN1, CETN}, ADA2 (adenosine deaminase 2) [NCBI Gene 51816] {aka ADGF, CECR1, IDGFL, PAN, SNEDS, VAIHS}
- **Diseases:** necrosis (MESH:D009336), erythema (MESH:D004890), Undifferentiated/unclassified sarcoma (MESH:C562442), radiation-associated sarcomas (MESH:D011832), paresthesia (MESH:D010292), mandibular swelling (MESH:D008338), undifferentiated round cell sarcoma (MESH:D018208), Ewing sarcoma (MESH:D012512), bone and soft tissue tumors (MESH:D012983), odontogenic cysts (MESH:D009807), weight loss (MESH:D015431), CGCG (MESH:D006101), metastasis (MESH:D009362), giant cell-rich lesions (MESH:D018286), STS (MESH:D012509), fever (MESH:D005334), osteolytic lesion (MESH:D030981), head and neck giant cell-containing lesions (MESH:D000077195), hemorrhage (MESH:D006470), lytic lesion (MESH:D009059), rhabdomyosarcoma (MESH:D012208), giant (MESH:D005870), mesenchymal malignancy (MESH:C535700), odontogenic sarcoma (MESH:D018126), Malignant Fibrous Histiocytomas (MESH:D051677), malignant neoplasms (MESH:D009369), mandibular lesions (MESH:D008336), UPS (MESH:D002277), swelling (MESH:D004487), metastatic disease (MESH:D000092182), head and neck cancers (MESH:D006258), osteosarcoma (MESH:D012516), jaw lesions (MESH:D007571), inflammatory (MESH:D007249), leiomyosarcoma (MESH:D007890), trauma (MESH:D014947), Pain (MESH:D010146)
- **Chemicals:** eosin (MESH:D004801), FDG (MESH:D019788), H&amp;E (MESH:D006371), alkylating chemotherapeutic agents (-), hematoxylin (MESH:D006416), doxorubicin (MESH:D004317)
- **Species:** Homo sapiens (human, species) [taxon 9606]

## Full text

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## Figures

6 figures with captions in the complete paper: https://tomesphere.com/paper/PMC12921370/full.md

## References

16 references — full list in the complete paper: https://tomesphere.com/paper/PMC12921370/full.md

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Source: https://tomesphere.com/paper/PMC12921370