Humans ; Soft Tissue Neoplasms ; Mesenchymoma/diagnostic imaging* ; Paraneoplastic Syndromes/pathology*

OBJECTIVE: Critically ill patients with solid tumors complicated with paraneoplastic pemphigus are usually treated in intensive care units (ICU) for perioperative management after surgical treatment. In this study, the clinical characteristics and predictors of long-term prognosis of these critically ill patients were analyzed. METHODS: the clinical and laboratory data of 63 patients with solid tumors complicated with paraneoplastic pemphigus admitted to ICU from 2005 to 2020 were retrospectively analyzed, and the survival status of the patients were followed up. RESULTS: Among the 63 patients, 79.4% had Castleman disease as the primary tumor, and 20.6% with other pathological types; 69.8% had severe-extensive skin lesions, and 30.2% had other skin lesions; the patients with bronchiolitis obliterans accounted for 44.4%, and 55.6% were not merged. Postoperative fungal infection occurred in 23.8% of the patients, and 76.2% without fungal infection. The median follow-up time was 95 months, and 25 patients died during the study period. The 1-year, 3-year and 5-year survival rates were 74.6% (95%CI 63.8%-85.4%), 67.4% (95%CI 55.6%-79.2%) and 55.1% (95%CI 47.9%-62.3%), respectively. The log-rank univariate analysis showed that the patients had age>40 years (P=0.042), preoperative weight loss>5 kg (P=0.002), preoperative albumin < 30 g/L (P < 0.001), paraneoplastic pemphigus complicated with bronchiolitis obliterans (P=0.002), and perioperative fungal infection (P < 0.001) had increased mortality. Cox univariate analysis showed that preoperative weight loss >5 kg (P=0.005), preoperative albumin < 30 g/L (P < 0.001), paraneoplastic pemphigus complicated with bronchiolitis obliterans (P=0.009), preoperative bacterial pulmonary infection (P=0.007), prolonged surgical time (P=0.048), postoperative oxygenation index (P=0.012) and low albumin (P=0.010) and hemoglobin concentration (P=0.035) in ICU, acute physiology and chronic health evaluation (APACHE Ⅱ) score (P=0.001); sequential organ failure assessment (SOFA) score (P=0.010), and postoperative fungal infection (P < 0.001) were risk factors for long-term survival. Cox regression model for multivariate analysis showed that preoperative weight loss > 5 kg (HR 4.44; 95%CI 1.47-13.38; P=0.008), and preoperative albumin < 30 g/L (HR 4.38; 95%CI 1.72-11.12; P=0.002), bronchiolitis obliterans (HR 2.69; 95%CI 1.12-6.50; P=0.027), and postoperative fungal infection (HR 4.85; 95%CI 2.01-11.72; P < 0.001) were independent risk factors for postoperative mortality. CONCLUSION The 5-year survival rate of critically ill patients undergoing surgery for paraneoplastic pemphigus combined with solid tumors is approximately 55.1%, with preoperative weight loss > 5 kg, albumin < 30 g/L, bronchiolitis obliterans and postoperative fungal infection were associated with an increased risk of near- and long-term postoperative mortality.
Adult ; Albumins/therapeutic use* ; Bronchiolitis Obliterans/pathology* ; Critical Illness ; Hemoglobins ; Humans ; Neoplasms/complications* ; Paraneoplastic Syndromes/pathology* ; Pemphigus/drug therapy* ; Retrospective Studies ; Weight Loss

Pure red cell aplasia (PRCA) and hypogammaglobulinemia are paraneoplastic syndromes that are rarer than myasthenia gravis in patients with thymoma. Good syndrome coexisting with PRCA is an extremely rare pathology. We report the case of a 50-year-old man with thymoma and PRCA associated with Good syndrome who achieved complete PRCA remission after thymectomy and postoperative immunosuppressive therapy, and provide a review of the pertinent literature.
Agammaglobulinemia ; Humans ; Middle Aged ; Myasthenia Gravis ; Paraneoplastic Syndromes ; Pathology ; Red-Cell Aplasia, Pure* ; Thymectomy ; Thymoma

Paraneoplastic pemphigus is a rare autoimmune bullous dermatosis, which is caused by potential neoplasm, especially the Castleman's disease. Castleman's disease associated with paraneoplastic pemphigus is misdiagnosed frequently and easily in clinical practices. Furthermore, it is reported that the mortality rate for this disease is very high. Bronchiolitis obliterans is the most common complication and the most important cause of death. There was a female patient presenting recalcitrant mucocutaneous erosions, ulcers and scattered erythemas in the body. The patient was diagnosed and treated for pemphigus vulgaris with little success in Xiangya Hospital, Central South University in January 2015. Further investigations confirmed the diagnosis of paraneoplastic pemphigus with retroperitoneal tumor. Subsequently, the patient was treated with tumor resection in combination with intravenous immunoglobulin and corticosteroids. The pathology revealed that it was the Castleman's disease. Her mucocutaneous performance recovered obviously and the bronchiolitis obliteran did not appear in the follow-up. Castleman's disease associated with paraneoplastic pemphigus should be considered when mucosal and skin lesions showing no improvement under corticosteroids. Early and complete removal of the tumor together with immunotherapy could be beneficial to the patient's prognosis.
Adrenal Cortex Hormones ; therapeutic use ; Castleman Disease ; complications ; therapy ; Female ; Humans ; Immunoglobulins, Intravenous ; therapeutic use ; Paraneoplastic Syndromes ; complications ; therapy ; Pemphigus ; complications ; therapy ; Retroperitoneal Space ; pathology

Encephalitis ; diagnosis ; pathology ; Humans ; Lymphoma ; diagnosis ; pathology ; Male ; Middle Aged ; Paraneoplastic Syndromes ; diagnosis ; pathology

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 a 59-year-old patient with malignant acanthosis nigricans associated with metastasis of endometrial carcinoma. The patient presented papillomatosis lesions that appeared to be benign on multiple skins of body folds, particularly on lips. The lesions in lips and axilla had histological characteristic appearances of acanthosis nigricans, while the masses in abdomen and pelvis were metastasis endometrial adenocarcinoma. The article highlights the importance of biopsy and histopathological diagnosis in presumed benign lesions and the role of doctors in screening for body internal tumors.
Abdominal Neoplasms ; secondary ; Acanthosis Nigricans ; pathology ; Adenocarcinoma ; secondary ; Endometrial Neoplasms ; pathology ; Female ; Follow-Up Studies ; Humans ; Lip Diseases ; pathology ; Lymphatic Metastasis ; pathology ; Middle Aged ; Paraneoplastic Syndromes ; pathology ; Pelvic Neoplasms ; secondary

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

No abstract available.
Adenocarcinoma/*secondary/surgery ; Chemotherapy, Adjuvant ; Colectomy ; Glomerulonephritis, Membranoproliferative/diagnosis/*etiology/therapy ; Hepatectomy ; Humans ; Liver Neoplasms/*secondary/surgery ; Male ; Middle Aged ; Paraneoplastic Syndromes/diagnosis/*etiology/therapy ; Renal Dialysis ; Renal Insufficiency/etiology ; Sigmoid Neoplasms/*pathology/surgery ; Treatment Outcome

Antineoplastic Agents ; adverse effects ; Child ; Child, Preschool ; Hematopoietic Stem Cell Transplantation ; adverse effects ; Humans ; Kidney Diseases ; etiology ; pathology ; therapy ; Kidney Neoplasms ; etiology ; pathology ; therapy ; Neoplasms ; complications ; therapy ; Paraneoplastic Syndromes ; etiology ; pathology ; therapy ; Radiotherapy ; adverse effects ; Risk Factors ; Tumor Lysis Syndrome ; etiology ; pathology ; therapy