Inflammatory pseudotumor of the spinal nerve complicated by paraneoplastic pemphigus

Abstract

We describe finding an inflammatory pseudotumor (IPT) in the thoracic spinal nerve, a previously unreported location, in combination with paraneoplastic pemphigus (PNP). This 48-year-old woman presented with a 1-month history of progressive dyspnea and pleuritic pain in the right lower chest region, as well as a 1-month history of thoracic back pain. Physical examination revealed multiple oral ulcers, multiple blisters, and erythematous papules on the skin of the trunk. These lesions were diagnosed as pemphigus vulgaris based on the results of biopsy examination. All laboratory tests, plain chest radiographs, and plain spine radiographs showed normal findings. Thoracic magnetic resonance (MR) imaging studies showed an extradural paraspinal ellipsoidal mass exhibiting regular boundaries; and contrast enhancement was homogeneous in the right costodiaphragmatic recess at the T-12 level and extended into the spinal foramen (Fig. 1 left). Because of the patient’s progressive symptoms, a costotransversectomy was performed via a paramedian incision. Intraoperatively, we observed a well-circumscribed 5 3 3 3 4‐cm tumor originating from the proximal part of T-12 spinal nerve, which was not grossly infiltrated; the lesion bulged into the right thoracic cavity. The final histological diagnosis was an IPT (Fig. 1 right). The patient’s chest pain progressively resolved after surgery, but dyspnea persisted and progressive pulmonary compromise developed. She was transferred to the department of internal medicine, and a repeated biopsy sample of an oral ulcer was obtained. Direct tissue immunofluorescence demonstrated intracellular immunoglobulin G (IgG) and complement C3 deposition within the epidermis (Fig. 2). The final diagnosis was PNP combined with fibrosa obliterans, which was caused by the underling IPT. During the following months, the progressive dyspnea improved slightly after intravenous Ig therapy, as did the skin and mucosal lesions. Three months after the operation, however, the patient was readmitted to the intensive care unit for progressive dyspnea, and she died of pulmonary failure. In the present case the IPT’s origin from the spinal nerve in the paravertebral region was unique.4 In such cases involving an IPT diagnosis is seldom confirmed preoperatively, and the treatment of choice is resection. 2