Trastuzumab deruxtecan treatment for HER2-low metastatic scrotal extramammary Paget’s disease with bone marrow involvement: a case report - Summary - MDSpire
To report a case of metastatic scrotal extramammary Paget’s disease (EMPD) involving bone marrow and evaluate the efficacy of trastuzumab deruxtecan (T-DXd) in treatment.
Approach:
Case Presentation: A 68-year-old man with a history of EMPD developed metastatic disease with bone marrow involvement, presenting with scrotal lesions, systemic symptoms, and a history of prior treatment.
Diagnostic Assessment: Biopsies confirmed stage IV EMPD. Imaging showed multiple osteolytic lesions and lymphadenopathy. HER2 expression was assessed as 2+.
Therapeutic Intervention: The patient received T-DXd, with disease stability observed during treatment despite significant tumor burden.
Key Findings:
The patient exhibited aggressive biological behavior of EMPD with bone marrow metastasis and systemic symptoms.
Histopathological assessment confirmed HER2 expression at 2+.
Treatment with T-DXd resulted in stable disease despite extensive metastasis.
Interpretation:
This case highlights the potential for targeted therapies like T-DXd to control disease in tumors with low HER2 expression.
Limitations:
The patient declined repeat bone marrow biopsy, limiting further immunohistochemical evaluation and understanding of disease progression.
Lack of comprehensive diagnostic procedures, such as cystoscopy and gastrointestinal investigations.
Conclusion:
Further research is needed to define optimal treatment strategies for metastatic EMPD.