Chronic inflammatory demyelinating polyneuropathy caused by hepatocellular carcinoma

Abstract

Paraneoplastic syndromes are rare abnormal endocrine or immune responses triggered by neoplasms. Chronic inflammatory demyelinating polyneuropathy (CIDP) is one such example. CIDP is an acquired, immune-mediated neuropathy affecting the peripheral nerves and nerve roots. It is associated with many types of cancers, especially haematological malignancies. We report the case of a man in his 60s who presented to the emergency department with acute symptoms of upper and lower extremity paresis and decreased sensation in the toes and tips of his fingers. Laboratory tests were normal. Electrodiagnostic studies showed diffuse motor and sensory dysfunction in all extremities; a diagnosis of CIDP was consequently made. Imaging studies showed a large left lobe liver mass. Subsequent biopsy revealed histopathological findings characteristic of hepatocellular carcinoma. After failure of medical treatment with intravenous immunoglobulin and corticosteroids, laparoscopic resection of the tumour was planned, performed and resulted in complete resolution of symptoms. At 18 months postoperatively, the patient was asymptomatic.

Keywords: liver disease; peripheral nerve disease; surgery.

Figure 1

(A) Non-contrast CT and (B) positron emission tomography scan showing a large heterogenous hypodense mass in the liver and central area of photopenia related to necrosis at the junction of segments II and IVa, extending to segments II and III.

Figure 2

Histology of the liver tumour (H&E stain). (A) Medium-power view, 20×: the tumour cells resemble normal hepatocytes. They have a polygonal cytoplasm. (B) High-power view, 40×: abundant cytoplasm with round to oval vesicular nuclei and nuclear invaginations.

Figure 3

Immunohistochemistry of tumour cells: (A) Hepar-1: positive; (B) glypican: patchy positivity; (C) CK8/18: positive; (D) CD34: highlights tumour vascularity; (E) AFP (Alpha Fetoprotein): negative; and (F) CK7: negative.