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

No abstract available.
Bile Duct Neoplasms/complications/*diagnosis ; *Bile Ducts, Intrahepatic/chemistry/pathology/radiography ; Biopsy ; Cholangiocarcinoma/complications/*diagnosis ; Diagnosis, Differential ; Fever/diagnosis/*etiology ; Humans ; Immunohistochemistry ; Leukocytosis/*diagnosis/etiology ; *Liver/chemistry/pathology/radiography ; Liver Abscess, Pyogenic/*diagnosis ; Magnetic Resonance Imaging ; Male ; Middle Aged ; Paraneoplastic Syndromes/*diagnosis/etiology ; Predictive Value of Tests ; Tomography, Spiral Computed ; Tumor Markers, Biological/analysis

We report the rare case of a patient with advanced renal cell carcinoma (RCC) who initially presented to the hospital with symptoms of cardiac failure. Preoperative cardiac studies did not reveal any underlying ischemia. After resection of a large 14-cm left renal tumor, cardiac function was noted to improve dramatically. We discuss this case of concomitant RCC and nonischemic cardiomyopathy.
Asian Continental Ancestry Group ; Carcinoma, Renal Cell/*diagnosis/surgery ; Cardiomyopathies/*diagnosis ; Female ; Heart Failure/*etiology ; Humans ; Middle Aged ; Paraneoplastic Syndromes/complications ; Thalassemia/complications

Dermatomyositis is an idiopathic inflammatory myopathy with typical cutaneous manifestations. It has been proposed that dermatomyositis may be caused by autoimmune responses to viral infections. Previous studies have shown an association between dermatomyositis and malignant tumors such as ovarian cancer, lung cancer, and colorectal cancer. However, a chronic hepatitis B virus (HBV) infection associated with dermatomyositis and hepatocellular carcinoma (HCC) has been very rarely reported. Here, we report a rare case of dermatomyositis coinciding with HBV-associated HCC. A 55-year-old male was confirmed to have HCC and dermatomyositis based on proximal muscle weakness, typical skin manifestations, elevated muscle enzyme levels, and muscle biopsy findings. This case suggests that HCC and/or a chronic HBV infection may be factors in the pathogenesis of dermatomyositis through a paraneoplastic mechanism.
Antiviral Agents/therapeutic use ; Biopsy ; Carcinoma, Hepatocellular/diagnosis/*virology ; Dermatomyositis/diagnosis/drug therapy/*virology ; Disease Progression ; Fatal Outcome ; Glucocorticoids/therapeutic use ; Hepatitis B, Chronic/*complications/diagnosis/drug therapy ; Humans ; Liver Neoplasms/diagnosis/*virology ; Male ; Middle Aged ; Paraneoplastic Syndromes/diagnosis/drug therapy/*virology ; Risk Factors ; Time Factors ; Tomography, X-Ray Computed ; Treatment Outcome

No abstract available.
Acanthosis Nigricans/diagnosis/*etiology ; Biopsy ; Gastrointestinal Stromal Tumors/chemistry/*complications/pathology ; Humans ; Immunohistochemistry ; Laparoscopy ; Male ; Middle Aged ; Paraneoplastic Syndromes/diagnosis/*etiology ; Peritoneal Neoplasms/chemistry/*complications/pathology ; Proto-Oncogene Proteins c-kit/analysis ; Tomography, X-Ray Computed ; Tumor Markers, Biological/analysis

OBJECTIVETo study the clinicopathologic features of follicular dendritic cell sarcoma (FDCS) and its differential diagnosis.

METHODSTen cases of FDCS were studied by light microscopy, immunohistochemistry and in-situ hybridization. The clinical features and follow-up information were analyzed.

RESULTSAmongst the 10 cases of FDCS studied, the male-to-female ratio was 1:1. The mean age of the patients was 42 years. Six of them were located in cervical and peritoneal lymph nodes and four in extranodal sites (including tonsil, pelvic cavity, tail of pancreas and spleen). Histologically, the tumor cells had whorled, storiform or diffuse growth patterns. They were spindle in shape and contained syncytial eosinophilic cytoplasm, with round or oval nuclei, vesicular chromatin, distinct nucleoli and a variable number of mitotic figures. Multinucleated tumor giant cells and intranuclear pseudoinclusions were occasionally seen. There was a sprinkling of small lymphocytes and neutrophils within the tumor as well as in the perivascular region. Immunohistochemical study showed that the tumor cells were diffusely or focally positive for CD21, CD23, CD35 and D2-40, but negative for LCA, CD20, CD3, CD1a, HMB45 and CK. Some of them showed EMA, CD68 and S-100 reactivity. In-situ hybridization for Epstein-Barr virus-encoded RNA (EBER) showed positive signals in only one case (which was diagnosed as inflammatory pseudotumor-like FDCS). Of the 7 patients with follow-up information available (duration: 2 months to 39 months; mean: 14 months), 2 cases with paraneoplastic pemphigus died of pulmonary infection at 5 and 7 months respectively. The remaining 5 patients were alive and disease-free after surgical excision (+/- chemotherapy and radiotherapy).

CONCLUSIONSFDCS is a rare low to intermediate-grade malignant tumor. Appropriate application of FDC markers, such as CD21, CD35 and D2-40, would be helpful for arriving at a correct diagnosis. Most cases are associated with good prognosis after surgical treatment, with or without chemotherapy and radiotherapy. Patients with paraneoplastic pemphigus carry a less favorable prognosis.

Adult ; Antibodies, Monoclonal, Murine-Derived ; metabolism ; Dendritic Cell Sarcoma, Follicular ; complications ; metabolism ; pathology ; surgery ; Dendritic Cell Sarcoma, Interdigitating ; pathology ; Diagnosis, Differential ; Female ; Follow-Up Studies ; Humans ; Lymph Node Excision ; Lymph Nodes ; pathology ; surgery ; Male ; Meningioma ; pathology ; Middle Aged ; Nasopharyngeal Neoplasms ; pathology ; Paraneoplastic Syndromes ; complications ; Pemphigus ; complications ; Receptors, Complement 3b ; metabolism ; Receptors, Complement 3d ; metabolism ; Receptors, IgE ; metabolism ; Tonsillar Neoplasms ; metabolism ; pathology ; surgery ; Young Adult

OBJECTIVETo investigate the clinical characteristics, surgical treatment and prognosis analysis of localized retroperitoneal Castleman disease (CD), and to improve the level of diagnosis and treatment of retroperitoneal Castleman disease with paraneoplastic pemphigus (PNP).

METHODSThe clinical data of retroperitoneal CD with PNP from January 1993 to May 2009 were compared with CD without PNP retrospectively, including clinical features, tumor site, lab examination, surgical treatment, pathologic subtype and results of surgery.

RESULTS(1) Retroperitoneal Castleman disease more likely originated in para-kidney and iliac fossa with middle age of 36 years old, especially in left retroperitoneum. Of the 20 cases, 18 tumors (90%) were hyaline vascular variants and 2 were mixed variants. There was no statistical difference in gender, age, tumor site and pathological subtype between two groups. (2) Retroperitoneal CD with PNP more likely complicated with bronchiolitis obliterans (BO) and high level of serum CEA/CA242. (3) Retroperitoneal Castleman tumors had clear margin, intact envelop and were easily resectable, however the biological behavior of CD with PNP might tend malignant changing, invade adjacent blood vessel or seed locally, and eventually relapse after operation. (4) The 5-year survival rate of retroperitoneal CD with PNP was 42.8%, significantly lower than those without PNP. The average survival time was 30 months. Bronchiolitis obliterans and radical resection were the key effect in prognosis of retroperitoneal CD.

CONCLUSIONSRetroperitoneal CD with PNP has distinctive clinical features. Early diagnosis, prompt removal of tumor and termination secretion of causative antibody are critical to the management of this disease.

Adolescent ; Adult ; Castleman Disease ; complications ; diagnosis ; therapy ; Female ; Follow-Up Studies ; Humans ; Male ; Middle Aged ; Paraneoplastic Syndromes ; complications ; Pemphigus ; complications ; Prognosis ; Retroperitoneal Space ; Retrospective Studies ; Young Adult

Inflammatory myofibroblastic tumors (IMTs) are benign neoplasms that can occur at different anatomic sites with nonspecific clinical symptoms. A 48-yr-old woman presented with a 2-month history of a relapsed oral ulcer, progressive dyspnea, and a thoracic pain induced by breathing. A tumorous mass was noticed in the right costodiaphragmatic recess on chest computed tomography and magnetic resonance imaging, and the patient underwent a right costotransversectomy with excision of the tumor, which originated from the 12th intercostal nerve. Histology and immunohistochemistry showed that the tumor was an IMT of the intercostal nerve. The patient's postoperative course was not favorable; dyspnea persisted after surgery, and a progressive pulmonary compromise developed. The cause of the respiratory failure was found to be bronchiolitis obliterans, which in this case proved to be a fatal complication of paraneoplastic pemphigus associated with an IMT. This case of IMT of the spinal nerve in the paravertebral region is unique in terms of its location and presentation in combination with paraneoplastic pemphigus, which is rare. A brief review of the heterogeneous theories concerning the pathogenesis, clinicopathological features, and differential diagnosis of this disease entity is presented.
Diagnosis, Differential ; Fatal Outcome ; Female ; Granuloma, Plasma Cell/complications/*pathology ; Humans ; Intercostal Nerves/*pathology ; Lung Diseases/etiology/pathology ; Middle Aged ; Paraneoplastic Syndromes/etiology/*pathology ; Pemphigus/etiology/*pathology ; Peripheral Nervous System Neoplasms/complications/*pathology

BACKGROUND/AIMS: Patients with hepatocellular carcinoma (HCC) may manifest paraneoplastic syndromes such as hypercholesterolemia, hypoglycemia, hypercalcemia and erythrocytosis. This study was aimed at evaluating the incidence and clinical significance of paraneoplastic syndromes in Korean HCC patients. METHODS: The medical records of 165 HCC patients who were diagnosed and died in the Kyung Hee University Hospital, were reviewed retrospectively. The following variables were analyzed: age, gender, hepatitis markers, platelet, liver function test, alpha-fetoprotein (AFP), Child-Pugh score, tumor features, and the duration of their survival. RESULTS: In total, paraneoplastic syndromes were presented in 43.6% of the HCC patients during the course of their disease. Hypercholesterolemia was solely presented in 14.5%, hypoglycemia in 12.7% and hypercalcemia in 7.8%. The patients who presented with more than 2 syndromes were 8.5%. While 80% of erythrocytosis (4/5) and 51.6% of hypercholesterolemia (16/31) was presented at the time of HCC diagnosis, hypoglycemia and hypercalcemia mainly occurred as terminal events. The patients with paraneoplastic syndromes were younger and had higher rates of portal vein thrombosis, bi-lobar tumor involvement and tumor more of more than 10 cm in diameter, compared to those patients without them. The proportion of patients with a serum AFP more than 400 ng/mL tended to be higher in the patients with paraneoplastic syndromes. The HCC patients with paraneoplastic syndromes, except for erythrocytosis, had a shorter survival than those patients without them. CONCLUSIONS: Paraneoplastic syndromes are not infrequently presented in HCC patients, especially at an advanced stage, and the survival of these patients is relatively shorter.
Aged ; Carcinoma, Hepatocellular/*complications/mortality ; English Abstract ; Female ; Humans ; Liver Neoplasms/*complications/mortality ; Male ; Middle Aged ; Paraneoplastic Syndromes/complications/diagnosis

Granulocytosis occurs in 40% of patients with lung and gastrointestinal cancers, 20% of patients with breast cancer, 30% of patients with brain tumor and ovarian cancer and 10% of patients with renal cell carcinoma. Granulocytosis occurs because of production of G-CSF, GM-CSF and IL-6. Uterine cervical carcinoma with granulocytosis as a paraneoplastic syndrome, however, has been rarely reported. We recently witnessed a case of invasive squamous cell carcinoma of the uterine cervix with granulocytosis. Leukocytosis developed up to 69, 000/micro L, and then normalized after chemo-radiotherapy. There was no evidence of infection, tumor necrosis, glucocorticoid administration, or myeloproliferative disease by examination of a bone marrow aspirate when granulocytosis appeared. This phenomenon was probably associated with the secretion of hematopoietic growth factors such as G-CSF, GM-CSF and IL-6 by the tumor. We suggest that, like some other solid tumors, cervical cancer can present with granulocytosis as a paraneoplastic syndrome.
Uterine Neoplasms/complications/*diagnosis ; Uterine Cervical Neoplasms/complications/*diagnosis/physiopathology ; Paraneoplastic Syndromes/*etiology ; Middle Aged ; Leukocytosis/*etiology ; Humans ; Granulocytes/*pathology ; Female