Paraneoplastic and Other Autoimmune Neurologic Disorders

A paraneoplastic disorder arises from remote effects of malignancy, rather than direct tumor invasion. Paraneoplastic neurologic disorders occur because of the vigorous immune responses directed against antigens expressed in tumors. The vigor of the immune response usually ensures that neoplasm is confined to the primary organ and regional lymph nodes. However, in the clinical setting of paraneoplastic disease, the immune response may attack any part of the nervous system (brain, spinal cord, nerve, neuromuscular junction, or muscle), and antibodies are either directed at intracellular neuronal proteins or against neuronal cell surface or synaptic proteins. Treatment is often aimed at the cancer and suppression of the abnormal immune response.

Colon Cancer: An Unusual Presentation with a Paraneoplastic Syndrome

Dermatomyositis (DM) is a rheumatological disorder characterized by proximal myositis and distinctive dermatological manifestations. It can be an isolated clinical syndrome or, in rarer cases, can be the initial presentation for an underlying malignancy as a part of a paraneoplastic syndrome. In this case report, we describe a case of a 51-year-old lady who presented with proximal myopathy, typical DM skin rash, dysphagia, and markedly elevated creatine kinase. She was diagnosed with a seronegative DM and her malignancy screening revealed a mass in the ascending colon. During her hospital course, she also developed microangiopathic hemolytic anemia, another paraneoplastic disorder typically associated with late stages of malignancy, manifested as hemolytic anemia, thrombocytopenia, and low fibrinogen. The patient received intravenous corticosteroids and underwent tumor resection with following resolution of her both rheumatological and hematological manifestation. Unfortunately, due to her general poor health, she developed sepsis and died in the hospital.

Spinal Tumor Resection with Intraoperative Computed Tomography Navigation in Tumor-Induced Osteomalacia: A Case Report

This is the first report using intraoperative CT navigation for Tumor-induced Osteomalacia (TIO) lesions of the spine. TIO is a rare paraneoplastic disorder caused by tumors secret Fibroblast growth factor 23 secreted by tumor tissue. Surgical resection of the main tumor is the only definitive treatment. However, the tumor is usually small, and it hard to find it, and it tends to be seen when the recurrence is caused by the difficulty of whole tumor resection. A 57-year-old woman presented with a rare case of TIO located in T10 vertebra body. Using the intraoperative CT navigation, we resected the tumor in the T10 vertebral body very effective for accurate localization of the tumor and helpful for guidance of resection area and confirmation for excision of tumor. As a treatment for TIO, we report a tumor resection with intraoperative computed tomography navigation that made it possible to resect tumor in T10 vertebrae precisely and safely, which is small and difficult and dangerous access.

Utility of 18F-AlF-NOTA-octreotide PET/CT in the localization of Tumor-Induced Osteomalacia

Purpose Tumor-induced osteomalacia (TIO) is a paraneoplastic disorder, usually caused by benign mesenchymal tumors that produce high levels of the hormone fibroblast-growth-factor 23 (FGF23). The only curative therapy of the disease is resection of the causative tumors. This research was conducted to evaluate the efficacy of 18F-AlF-NOTA-octreotide ( 18F-OC) PET/CT in detecting TIO and its impact on patient management. Methods Retrospective analysis of 17 patients with hypophosphatemic osteomalacia suspected of TIO was performed. 18F-OC PET/CT study was performed in all 17 patients to localize the tumor. 68Ga-DOTATATE PET/CT was performed in 4 out of 17 patients. 18F-OC and 68Ga-DOTATATE PET/CT studies were performed within 1 week of each other. Both studies were interpreted blindly without the knowledge of other imaging findings. The image findings were compared with the results of histopathological examinations and clinical follow-ups. Results 18F-OC PET/CT scans were positive in 14 patients. Moreover, 4 out of 14 patients were performed with both 18F-OC and 68Ga-DOTATATE PET/CT. Both studies were able to localize the tumor in all the 4 patients. In total, 14 patients had surgery to remove the lesions. Postsurgical pathological examination confirmed causative tumors in these patients whose symptoms diminished promptly. The serum phosphate levels became normal confirming the diagnosis of TIO. 18F-OC PET/CT sensitivity, specificity and accuracy were 87.5%, 100% and 88.2% respectively. 18F-OC PET/CT findings affected patient management in 88.2% of cases. Conclusion 18F-OC PET/CT scan is useful in the detection of tumors causing TIO. Further studies with larger patient population are needed to validate the result.

A Case of Tumor-Induced Osteomalacia: Finding the Culprit Acetabular Tumor and Successful Resection with a Novel Hip Joint-Preserving Surgery

Introduction: Tumor-induced osteomalacia (TIO) is a rare paraneoplastic disorder caused by tumors that produce fibroblast growth factor 23 (FGF23) resulting in phosphate wasting and inadequate bone mineralization. Complete resection of the tumor can be curative. However, these tumors are typically difficult to find anatomically due to small size and location. Case Report: We present the case of a patient who presented for evaluation of recurrent fractures and hypophosphatemia in the setting of elevated FGF23 suggestive of TIO. 68Gallium-DOTATATE revealed multiple somatostatin avid lesions in several ribs, left acetabulum, sacrum, right tibia, and feet, some of which appeared with fracture on computed tomography scan, initially concerning for metastatic disease. However, the lesion in acetabulum was considered the culprit tumor given its remarkably higher maximum standard uptake values. Complete surgical removal of the FGF23-secreting tumor led to cure of this disease. Conclusion: This case report highlights the challenges with functional imaging differentiating fractures from the culprit lesion and reports on a novel surgical technique that allowed for surgical cure while preserving the hip joint. Keywords: Hypophosphatemia, osteomalacia, tumor, functional imaging, surgical technique.

Pulmonary Large Cell Neuroendocrine Carcinoma Associated With Lambert-Eaton Syndrome

Lambert-Eaton syndrome is a rare paraneoplastic disorder of the neuromuscular junction, characterized by impaired release of acetylcholine, which causes proximal muscle weakness, depressed tendon reflexes, and autonomic changes. Most cases of Lambert-Eaton syndrome present in small-cell lung carcinoma, and only a few cases have been reported in other lung subtypes. Herein, we report a case of 69 years old male patient with Lambert-Eaton syndrome as a rare association with a pulmonary large-cell neuroendocrine carcinoma, which presented 5 months before neoplasm diagnosis. A lobectomy was auspiciously performed. A review of the literature is also presented.

Tripe Palms: A Rare Cutaneous Paraneoplastic Disorder

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P.029 Facial onset sensorimotor neuronopathy syndrome (FOSMN) associated with Non-Hodgkin Lymphoma (NHL)

Background: FOSMN is a recently describe neurological syndrome, characterizes by slow onset of facial sensory abnormalities and motor deficits. The initial description showed a very uniform clinical presentation. Since the initial description there are clinical cases describe in literature with subtle phenotype variations. Methods: We describe a clinical case associated with NHL. We will report clinical data, laboratory and neurophysiological findings. Results: Patient initiated with left perioral and mental sensory symptoms on her left side. It spread up to include left V2 area and spread to the right side. After 2 years she developed sensory symptoms on her right hand. Progressed to weakness and atrophy on the right upper limb. Also developed dysarthria, dysphonia, dysphagia, as well as photophobia, anisocoria and double vision. Had thorough work-up and everything unrevealing. Except for Spep that showed increased free kappa. Bone marrow biopsy showed evidence of a clonal cell expansion consistent with indolent lymphoma Conclusions: This case provides evidence of FOSMN associated with NHL. To our knowledge this is a first case describe with NHL. There had been reports with motor neuro diseases phenotype with lymphoma that may represent a paraneoplastic disorder. Our patient expands the clinical presentation. This finding should not lessen the diagnosis of FOSMN.