Objective: To evaluate the clinical characteristics, treatment, and prognosis of patients with paraneoplastic neurological syndrome (PNS) associated with lymphoma. Methods: Between January 2012 and May 2021, the clinical data of 11 patients with lymphoma complicated with PNS treated at Peking Union Medical College Hospital were retrospectively reviewed. Results: Among the 11 patients (8 male and 3 female) , the median onset age was 61 (range, 33-78) years. The symptoms of PNS preceded lymphoma in 10 patients. The median time from the onset of PNS to the diagnosis of lymphoma was 4 months. Of the 11 patients, one had Hodgkin's lymphoma, 8 had B-cell non-Hodgkin's lymphoma, and 2 had peripheral T-cell lymphoma. Seven patients were evaluated for onconeural antibody, of whom 2 were positive (1 for anti-Ma2 antibody and 1 for anti-Yo antibody) . Of the 11 patients, the PNS symptoms of 3 patients were located in the central nervous system, 4 were located in the peripheral nervous system, and 3 were located in the muscle. Eight of the 11 patients were treated with glucocorticoid-based immunosuppressive therapy before the diagnosis of lymphoma. Patients with central nervous system involvement and dermatomyositis responded well to glucocorticoid, whereas patients with peripheral neuropathy did not significantly benefit. All 11 patients were treated with chemotherapy after the diagnosis of lymphoma. The efficacy of chemotherapy was assessed in 9 patients, 7 cases achieved complete remission, 1 case was evaluated as stable disease, and 1 case was evaluated as disease progression. The PNS symptoms of the patients who achieved complete response were almost completely recovered. The median follow-up time was 42 (range, 4-95) months. At the end of the follow-up period, 6 of the 11 patients survived, 3 were lost to follow-up, and 2 died. The median overall survival of the whole group was not reached. Conclusions: PNS can involve various parts of the nervous system and can be associated with different types of lymphoma. Through early diagnosis and treatment, the PNS symptoms could improve in most patients who achieve complete remission of lymphoma.
Adult ; Aged ; Antibodies, Neoplasm ; Autoantibodies ; Female ; Glucocorticoids ; Humans ; Lymphoma/diagnosis* ; Male ; Middle Aged ; Paraneoplastic Syndromes, Nervous System/complications* ; Retrospective Studies

Adult ; Aged ; Female ; Humans ; Male ; Middle Aged ; Motor Neuron Disease ; diagnosis ; Paraneoplastic Syndromes, Nervous System ; diagnosis

POEMS syndrome is a rare paraneoplastic syndrome, which includes polyneuropathy, organomegaly, endocrinopathy, M-protein, and skin changes due to plasma cell (PC) neoplasm. Diagnosis of this disease is challenging because of its rarity and complex clinical manifestations. We attempted to identify the key clinical features and characteristic bone marrow (BM) findings of POEMS syndrome, by reviewing the medical records and BM analyses of 24 Korean patients. Frequent clinical manifestations included polyneuropathy (100%), monoclonal gammopathy (100%), organomegaly (92%), extravascular volume overload (79%), and endocrinopathy (63%). The BM analyses revealed mild PC hyperplasia (median PCs: 5.5%) and frequent megakaryocytic hyperplasia (88%), megakaryocyte clusters (88%), and hyperlobation (100%). Flow cytometry of BM aspirates using CD138/CD38/CD45/CD19/CD56 showed normal (67%, 4/6) or neoplastic PC immunophenotypes (33%, 2/6). A diagnosis of POEMS syndrome must be considered when a patient suspected of having PC dyscrasia shows the above clinical presentation and BM findings.
Bone Marrow ; Diagnosis ; Flow Cytometry ; Humans ; Hyperplasia ; Medical Records ; Megakaryocytes ; Paraneoplastic Syndromes ; Paraproteinemias ; Plasma Cells ; POEMS Syndrome ; Polyneuropathies ; Skin

OBJECTIVE: To analyze the clinical characteristics, prognosis and parallel clinical course of ovarian cancer (OC) and dermatomyositis (DM). METHODS: The medical records of 23 consecutive patients who were diagnosed with OC and DM and were treated at Peking Union Medical College Hospital (PUMCH) between 2002 and 2017 were reviewed. Propensity score matching method was used to match control group (OC patients without DM) at a ratio of 1:5. The correlation between OC and DM was measured using the Pearson correlation scatter plot and Pearson's r. Kaplan-Meier survival analysis and Cox proportional hazard regression analysis were performed to evaluate the prognostic factors. RESULTS: After matching, 23 patients who have the concurrence of OC and DM (DM group) and 115 patients diagnosed with OC alone (No DM group) were included. The 5-year overall survival rates (71.6% vs. 51.8%, p=0.020) and 5-year progression-free survival (30.5% vs. 0%, p=0.018) were poorer in DM group. Correlation between serum cancer antigen 125 (CA 125) and creatine kinase (CK) level was observed in 12 patients. The time between OC and DM diagnosis is significant through univariable analysis (p=0.021) but not in multivariable analysis in patients who have the concurrence of OC and DM. CONCLUSION: The concurrence of OC and DM as a paraneoplastic syndrome is rare and has a poor prognosis. The risk for patients diagnosed with DM is highest within 3 years before or after OC diagnosis. A correlation and a parallel clinical course exist between these 2 diseases.
Beijing ; Creatine Kinase ; Dermatomyositis ; Diagnosis ; Disease-Free Survival ; Humans ; Medical Records ; Methods ; Ovarian Neoplasms ; Paraneoplastic Syndromes ; Prognosis ; Propensity Score ; Survival Rate ; Treatment Outcome

Tumor-induced osteomalacia (TIO), also known as oncogenic osteomalacia, is a rare paraneoplastic syndrome characterized by hypophosphatemia resulting from decreased tubular phosphate reabsorption, with a low or inappropriately normal level of active vitamin D. The culprit tumors of TIO could produce fibroblast growth factor 23 which plays a role in regulating renal Pi handling and 25-hydroxyvitamin D 1α-hydroxylase activity. Chronic hypophosphatemia could eventually lead to inadequate bone mineralization, presenting as osteomalacia. The diagnosis should be considered when patients manifest as hypophosphatemia and osteomalacia, or rickets and needs to be differentiated from other disorders of phosphate metabolism, such as the inhereditary diseases like X-linked hypophosphataemic rickets, autosomal dominant hypophosphataemic rickets, autosomal recessive hypophosphataemic rickets and acquired diseases like vitamin D deficiency. Localization of responsible tumors could be rather difficult since the vast majority are very small and could be everywhere in the body. A combination of thorough physical examination, laboratory tests and imaging techniques should be applied and sometimes a venous sampling may come into handy. The technology of somatostatin-receptor functional scintigraphy markedly facilitates the localization of TIO tumor. Patients undergoing complete removal of the causative neoplasm generally have favorable prognoses while a few have been reported to suffer from recurrence and metastasis. For those undetectable or unresectable cases, phosphate supplements and active vitamin D should be administrated and curative intended radiotherapy or ablation is optional.
Calcification, Physiologic ; Diagnosis ; Fibroblast Growth Factors ; Humans ; Hypophosphatemia ; Metabolism ; Neoplasm Metastasis ; Osteomalacia ; Paraneoplastic Syndromes ; Physical Examination ; Prognosis ; Radionuclide Imaging ; Radiotherapy ; Recurrence ; Rickets ; Vitamin D ; Vitamin D Deficiency

Syndrome of inappropriate antidiuretic hormone secretion (SIADH) has various causes including central nervous system disorders, pulmonary and endocrine diseases, paraneoplastic syndromes, and use of certain drugs. SIADH induced by chemotherapy with irinotecan-cisplatin is not a common complication. Here, we review a case of SIADH after treatment with irinotecan-cisplatin. A 45-year-old woman received adjuvant chemotherapy (paclitaxel-carboplatin) for ovarian clear cell carcinoma, but the cancer recurred within 9 months of chemotherapy. Subsequently, a second line of combination chemotherapy containing irinotecan-cisplatin was initiated. However, 5 days after chemotherapy administration, her general condition began to deteriorate; her hematological tests revealed hyponatremia. Therefore, it is imperative to consider the possibility of SIADH in patients being treated with irinotecan-cisplatin–based chemotherapy. Proper monitoring of serum sodium levels and assessment of clinical symptoms should be performed in such patients for early diagnosis and prompt management.
Central Nervous System Diseases ; Chemotherapy, Adjuvant ; Cisplatin ; Drug Therapy ; Drug Therapy, Combination ; Early Diagnosis ; Endocrine System Diseases ; Female ; Hematologic Tests ; Humans ; Hyponatremia ; Inappropriate ADH Syndrome ; Middle Aged ; Paraneoplastic Syndromes ; Sodium

Although neutrophilia can manifest from various causes, it is important to be able to distinguish chronic neutrophilic leukemia (CNL) from neutrophilic leukemoid reactions (NLR). In this paper, we describe four cases of leukocytosis with neutrophilia, including one case of CNL with a T618I mutation in colony stimulating factor 3 receptor (CSF3R) and three cases of NLR associated with malignancy or sepsis, which were initially suspected as CNL. Of the three NLR cases, one was associated with ovarian cancer, one with monoclonal gammopathy of undetermined significance and one with multiple myeloma with sepsis. This study demonstrated that confirming the clonality of myeloid cells with CSF3R T618I could contribute to making an accurate differential diagnosis between CNL and NLR in patients with solid cancers or plasma cell neoplasms caused by paraneoplastic syndromes and/or infection.
Colony-Stimulating Factors ; Diagnosis, Differential ; Humans ; Leukemia, Neutrophilic, Chronic* ; Leukemoid Reaction* ; Leukocytosis ; Monoclonal Gammopathy of Undetermined Significance ; Multiple Myeloma ; Myeloid Cells ; Neoplasms, Plasma Cell ; Neutrophils* ; Ovarian Neoplasms ; Paraneoplastic Syndromes ; Sepsis

Inflammatory myositis is a heterogeneous group of rare diseases characterized by inflammation of the skeletal muscle. The association between cancer and inflammatory myositis is well established, with most cancer-associated myopathies diagnosed within 2 years of initial diagnosis. However, despite this strong association, inflammatory myositis as a paraneoplastic syndrome of urethral cancer has not been reported in Korea. Furthermore, compartment syndrome in the context of inflammatory myositis is extremely rare. A 69 year-old woman presented with paresthesia and painful swelling of the right lower extremity 2 months after diagnosis with urethral cancer, which was treated by chemoradiotherapy. Painful numbness was particularly severe when extending the knee, leading to a preliminary diagnosis of myositis with compartment syndrome, which was confirmed by magnetic resonance imaging. After fascia resection, the patient was treated with high dose steroid and immunoglobulin therapy. Here, we report a case of compartment syndrome followed by inflammatory myositis in a patient with urethral cancer.
Chemoradiotherapy ; Compartment Syndromes* ; Diagnosis ; Fascia ; Female ; Humans ; Hypesthesia ; Immunization, Passive ; Inflammation ; Knee ; Korea ; Lower Extremity ; Magnetic Resonance Imaging ; Muscle, Skeletal ; Muscular Diseases ; Myositis* ; Paraneoplastic Syndromes ; Paresthesia ; Rare Diseases ; Urethral Neoplasms*

Aged ; Colonic Neoplasms ; diagnosis ; Humans ; Male ; Paraneoplastic Syndromes ; diagnosis ; Pemphigus ; diagnosis

Polyarthritis is a common manifestation of rheumatologic disorders; however, paraneoplastic arthropathies also arise as polyarthritis or polymyalgia, particularly in patients with myelomas, lymphomas, acute leukemia, and solid tumors. Because paraneoplastic syndromes, in some instances, might be manifested before a cancer diagnosis, they are difficult to diagnose and are often misdiagnosed. We experienced a 63-year-old female patient who had nonspecific arthritis on both hands and feet accompanied by fever. She had been diagnosed as rheumatoid arthritis and treated with prednisolone and disease modifying anti-rheumatic drugs (DMARDs) including methotrexate and anti-tumor necrosis factor agents. Her arthritis did not respond with anti-rheumatic treatment and diffuse large B-cell lymphoma was diagnosed by bone marrow biopsy. After 6 cycles of chemotherapy, her arthritis was improved as well as underlying lymphoma.
Antirheumatic Agents ; Arthritis* ; Arthritis, Rheumatoid ; B-Lymphocytes* ; Biopsy ; Bone Marrow* ; Diagnosis ; Drug Therapy ; Female ; Fever ; Foot ; Hand ; Humans ; Leukemia ; Lymphoma ; Lymphoma, B-Cell* ; Lymphoma, Large B-Cell, Diffuse ; Methotrexate ; Middle Aged ; Necrosis ; Paraneoplastic Syndromes ; Prednisolone