A quaint masquerader of breast cancer: Paraneoplastic cerebellar degeneration
Correspondence Address: Source of Support: None, Conflict of Interest: None DOI: 10.4103/0028-3886.263186
Source of Support: None, Conflict of Interest: None
Paraneoplastic cerebellar degeneration (PCD) is an extremely rare but devastatingly debilitating condition most commonly associated with small cell lung cancer, as well as breast and gynecologic malignancy. Breast cancer masquerading as paraneoplastic syndrome is an even rarer entity.
This study reports the case of a 55-year old female patient who presented with symptoms of unsteadiness of gait, slurring of speech, and bone pain of a 6-month duration. Clinical examination revealed dysarthria and positive cerebellar signs with normal vestibular, sensory, and motor examinations.
She was evaluated with magnetic resonance imaging (MRI) of the brain.
Sagittal T2-weighted image shows atrophy of cerebellar vermis with enlargement of the fourth ventricle [Figure 1].
Axial T2-weighted images showed symmetrical severe atrophy of the cerebellum and cerebellar peduncles causing enlargement of the fourth ventricle and widening of cerebellar fissures [Figure 2].
Coronal T2-weighted images showed symmetrical severe atrophy of the cerebellum causing enlargement of the fourth ventricle and widening of cerebellar fissures and cerebrospinal fluid (CSF) spaces around the cerebellum [Figure 3]. However, the CSF study, including the cytological evaluation, was normal.
Her detailed systemic clinical examination revealed a lump in the left breast. Mammography showed a lobulated, high-density mass inthe left breast of size 1.2 × 1 cm. Subsequently, an ultrasound-guided breast biopsy was performed and results of the histopathological examination revealed an invasive ductal carcinoma. Immunohistochemistry was positive for estrogen and progesterone receptors, and negative for the human epidermal growth factor receptor 2 (HER-2) status.
The metastatic workup with a CT and bone scan revealed extensive bone metastases. She was started on endocrinal treatment with letrozole 2.5 mg daily, along with monthly zoledronic acid and oral calcium supplementation.
The cerebellar symptoms, including dizziness and unsteadiness of gait, showed a marked improvement at a 12-month follow-up visit.
Pananeoplastic cerebellar degeneration (PCD) antedates the diagnosis of breast malignancy in 50% of cases. However, a vast majority of patients have an advanced and a metastatic disease.
Retrospectively, our patient had the classical presentation with predominant cerebellar symptoms and the imaging features aided us in establishing the diagnosis, though anti-Yo antibodies could not be demonstrated. The detailed examination and evaluation revealed that she had metastatic stage 4 disease at presentation.
Treatment options include both anti-tumor and immune therapy including steroids, immunoglobulin, and plasmapheresis. Though marked improvement is noted in a few patients, the outcome is dismal in the majority of patients. However, our patient responded well to hormonal treatment and showed improvement.
[Table 1] shows details of various cases of PCD in breast cancer reported in literature.
Hence, this case draws our attention to the important fact that physicians need to consider paraneoplastic syndrome as an important differential diagnosis in patients presenting with unexplained neurologic symptoms. It also highlights that inspite of recent advances in screening and an early detection of breast cancer, in developing countries, a vast majority of patients still present with advanced or metastatic disease. This emphasizes the need for improvement in our awareness and diagnostic strategies.
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There are no conflicts of interest.
[Figure 1], [Figure 2], [Figure 3]