Undifferentiated pleomorphic sarcoma of the breast with neoplastic fever: case report and genomic characterization

Abstract

Purpose Primary breast sarcomas are extraordinary rare, in particular undifferentiated pleomorphic sarcoma (UPS). UPS with neoplastic fever (UPS-NF) of the breast has not been reported yet. Here, we present an extended UPS-NF of the breast including its comprehensive molecular workup. Methods A 58-year-old female presented with general malaise, fever spikes, weight loss, and a massively swollen left breast. C-reactive protein and blood leucocytes were significantly increased. However, repeated blood cultures and smears were all sterile. Histopathology of the abscess-forming tumor revealed an undifferentiated malignancy with numerous of tumor giant cells as well as spindle-shaped cells with nuclear pleomorphism and hyperchromasia. Immunohistochemistry demonstrated partial, patchy desmin staining and weak heterogonous neuron-specific enolase immunoreactivity of tumor cells, but a focal staining for Melan-A. Results Neither common melanoma driver mutations nor an ultraviolet mutational signature was detected by whole genome sequencing. Using FISH and RT-PCR we also excluded translocations characteristic for clear cell sarcoma. Thus, the diagnosis of inflammatory UPS-NF of the breast was considered highly probable. Despite a complete mastectomy, the tumor recurred after only three months. This recurrence was treated with a combination of ipilimumab and nivolumab based on the primary tumor’s TPS score for PD-L1 of 30%. After an initial response, however, the tumor was progressive again. Conclusion We describe here the first case of UPS-NF of the breast, which shows great clinical and histopathologic resemblances to previously reported UPS-NF of other anatomic localizations.

Invasion of undifferentiated pleomorphic sarcoma (UPS) in breast tissue; a case report study

“…The diagnosis of inflammatory UPS with neoplastic fever in the breast was considered highly probable. Despite a complete mastectomy, the tumor recurred after only 3 months [1] . One of the main differences between this study and ours was the emergence of malignant tissue from the breast, but just as in our study and the study performed by Yamazaki et al, the patient eventually died.…”

“…Undifferentiated pleomorphic sarcoma (UPS) can occur in various locations of the body, including the chest wall and breast tissue [1] . Breast sarcomas account for less than 1 % of all breast tumors, with UPS being one of the most common types.…”

“…The presenting symptoms of UPS in the breast are similar to those of other breast tumors, such as a palpable mass, breast pain, nipple discharge, and skin changes. However, UPS tends to grow faster and be more aggressive than other breast tumors, which may result in a worse prognosis [1] .…”

Malignant Phyllodes Tumor of the Breast with Anemia and Unexplained Fever: A Case Report and Literature Review

“…This is more consistent with the procalcitonin concentration in our patient. Tumor fever presenting with only elevated in ammatory markers and no elevated infectious markers would have been rare 19 . Rapidly growing PT of the breast invading the skin has been reported with anemia 20 .…”

The devastating impact of unresectable infectious undifferentiated pleomorphic sarcoma in the gluteal region: A case report

“…UPS accounts for a significant portion of adult soft tissue sarcomas, representing approximately 20 % of all malignant mesenchymal tumors [ 3 ]. UPS of the buttock is a relatively rare presentation, with limited literature available on its clinical characteristics and management strategies [ 4 ].…”