The patient was a 42-year-old woman with Bloom syndrome and a history of malignant lymphoma, who presented with left central facial nerve paralysis.
Examination revealed deafness, and obstruction of the left external auditory meatus. CT revealed a soft tissue shadow with destruction of the left apex in the petrous area, and cholesteatoma was suspected. It was Bloom syndrome and considered the merger of the malignant tumor and photographed PET-CT, and a tumor with the accumulation of FDG was indicated at parapharyngeal space. Since it was considered difficult to perform radical excision, a craniotomy operation was performed together with neuro surgeon team of our hospital. A frozen-section examination revealed the diagnosis of squamous cell carcinoma.
Bloom syndrome is a hereditary autosomal recessive disease characterized by a distinctive facial appearance, low body weight, immunodeficiency, primary amenorrhea, sunlight-hypersensitive erythema. In addition, it is known even that I have a high cancer-causing percentage to be associated with a high incidence of cancer.