Publication: Paraneoplastic cerebellar degeneration: initial presentation of occult breast cancer
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2020-10-22
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Aim/Introduction: Paraneoplastic cerebellar degeneration
(PCD) is a rare type of paraneoplastic syndrome
characterized by the progressive onset of cerebellar
dysfunction as a result of immune-mediated response
against neoplasm rather than metastasis. We present a case
of occult breast cancer who had PCD at initial presentation
with diffuse cerebellar uptake depicted on the FDG PET/
CT scan. Six months after the treatment, a follow-up FDG
PET/CT scan revealed complete resolution of the cerebellar
hypermetabolism. Materials and Methods: FDG PET/CT was
performed to A 64-year-old female patient presented with
complaints of progressive dysarthria, ataxia, nystagmus,
headache and gait disturbance. Results: FDG PET/CT
showed diffuse and intensely increased uptake in both
cerebellar hemispheres and vermis (A). FDG PET/CT images
also depicted moderate hypermetabolic lymph node in the
right axillary region; however, no hypermetabolic foci was
detected in breast tissues (A). Histopathology of the right
axillary lymph node revealed metastasis of breast cancer.
Based on these findings, the diagnosis of paraneoplastic
cerebellar degeneration associated with occult breast
cancer was made and treatment with doxorubicindocetaxel-trastuzumab-pertuzumab was initiated. After
four cycles of chemotherapy, follow-up FDG PET/CT showed
complete regression of cerebellar hypermetabolism (B).
Conclusion: PCD is a rare central nervous system disorder
that is triggered by an immune system response to a
neoplasm. PCD affects 1-3 % of all cancer patients, and it
occurs typically in patients with cancer of the ovary, uterus,
breast cancer, small-cell carcinoma of the lung and Hodgkin
lymphoma. Patients typically develop subacute cerebellar
ataxia, dysarthria, diplopia, and vertigo, which often precede
the detection of the primary tumor. PCD is characterized
by extensive loss of Purkinje cells and the presence of
highly specific antineuronal antibodies in the serum and
cerebrospinal fluid. Proteins expressed by tumors induce
the production of onconeural antibodies leading to a T-cell
mediated destruction of Purkinje cells in the cerebellum.
The treatment of the underlying malignancyis considered
the optimal treatment for PCD. FDG PET/CT is crucial in the
early detection of the unknown primary tumors. Most of
the recently published studies emphasizing the potential of
FDG PET/CT in the assessment of the functional neurologic
consequences of the disease in the brain as well as changes
that follow after therapeutic interventions. To the best of
our knowledge, this is the first case in the literature that
uses FDG PET / CT to assess the current state of occult breast cancer with PCD and subsequently following up the
disease
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FİLİZOĞLU N., KESİM S., ŞAHİN C. Ö., ÖNEŞ T., ÖZGÜVEN S., ERDİL T. Y., \"Paraneoplastic Cerebellar Degeneration: Initial Presentation of Occult Breast Cancer\", European Association of Nuclear Medicine October 22 – 30, 2020 Virtual, 22 - 30 Ekim 2020