Primary primitive neuroectodermal tumor of the breast: a rare case presentation

Area of Science:

• Oncology
• Pathology
• Medical Imaging

Background:

• Primitive neuroectodermal tumors (PNETs) are rare in adults, with most reported cases occurring in the chest wall or other visceral sites.
• Primary PNET of the breast is exceptionally uncommon, presenting diagnostic challenges.
• This case highlights the potential for PNET to arise in the breast, mimicking other malignancies.

Purpose of the Study:

• To report a rare case of primary primitive neuroectodermal tumor (PNET) of the breast in an adult Indian woman.
• To discuss the diagnostic and therapeutic challenges associated with this rare entity.
• To contribute to the limited literature on breast PNET.

Main Methods:

• Initial diagnosis of malignant phyllodes tumor followed by recurrence.
• Incisional biopsy and histopathological examination revealing PNET.
• Immunohistochemical analysis positive for CD99, vimentin, and neuron-specific enolase (NSE).
• Treatment involved lumpectomy, incision and drainage, mastectomy, chemotherapy, and radiotherapy.

Main Results:

• The patient initially presented with a malignant phyllodes tumor, which recurred within two months.
• Histopathology and immunohistochemistry confirmed the diagnosis of PNET.
• Following mastectomy, chemotherapy, and radiotherapy, the patient achieved stable disease without distant metastasis at 18 months post-surgery.

Conclusions:

• Primitive neuroectodermal tumors (PNETs) can rarely occur primarily in the breast in adults.
• Accurate diagnosis requires thorough histopathological and immunohistochemical evaluation.
• Multimodal treatment approaches may lead to stable disease in rare cases of breast PNET.