Leukocytoclastic vasculitis (LCV) is a histopathologic descriptor for a prevalent type of small-vessel vasculitis (SVV) that affects arterioles, capillaries, and postcapillary venules. Although the association between vasculitis and malignancy only accounts for <5% of vasculitis cases, it has been recognized as a true paraneoplastic syndrome in several studies. A 57-year-old Filipino male presented with erythematous, nonblanching macules on his lower extremities, which rapidly progressed to violaceous lesions on his trunk, buttocks, and lower extremities. He also reported significant weight loss, decreased appetite, and vomiting. A skin biopsy confirmed LCV. Initially treated for meningococcemia, his condition did not improve. Abdominal imaging revealed an enlarged heterogeneous liver with retroperitoneal lymphadenopathy and a parenchymal nodule. He was eventually diagnosed with vasculitis secondary to an underlying hepatic malignancy and expired later from multiorgan failure.

Dermatomyositis (DM) is a rare autoimmune myopathy characterized by progressive muscle weakness that typically affects the shoulder and hip girdle first. It is a multisystem disorder characterized by symmetric proximal, extensor, inflammatory myopathy, vascular involvement and a constellation of pathognomonic (Gottron papules, Gottron Sign, Heliotrope rash) and characteristic cutaneous eruptions, which frequently presents in the fifth and sixth decades. As adult DM presents as a paraneoplastic syndrome in up to 30% of cases, metastatic workups appropriate for age are warranted.

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

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

Germ cell tumor is the most prevalent ovarian tumor in young women between 10 and 30 years of age.[1] However, immature teratomas account for only 20% of the malignant ovarian tumors found in the adolescent age group.[2] More uncommon is the occurrence of immature teratoma causing anti‑N‑methyl‑D‑aspartate receptor (Anti‑NMDAR) encephalitis and Guillain‑Barré Syndrome (GBS). This is a case of a 15‑year‑old female, nulligravid, who initially presented sudden behavioral change and symmetrical weakness of both lower and upper extremities with concomitant seizure episodes and with palpable lower abdominal mass. The patient was diagnosed to have GBS and treated with intravenous immunoglobulin causing resolution of neurologic symptoms months after. During the management of GBS, the patient noted increasing abdominal girth. Tumor markers showed elevated alpha‑fetoprotein, CA125, and lactate dehydrogenase. An imaging study revealed predominantly solid ovarian mass hence malignancy was considered. Once the medical condition stabilized, the patient underwent fertility‑sparing surgery with the final histopathologic result of immature teratoma. The aim of this report is to present a case of anti‑NMDAR encephalitis and GBS as paraneoplastic manifestation of immature teratoma.
Encephalitis ; Paraneoplastic Syndromes

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

Orofacial granulomatosis is a rare granulomatous inflammatory disease, characterized by recurrent orofacial swelling. Infectious, genetic, and immunologic etiologies are suggested, but not fully understood. Herein, we report a case of synchronous orofacial granulomatosis with brain cavernous hemangioma in a 44-year-old female patient, which may be considered paraneoplastic syndrome.
Adult ; Brain ; Female ; Granulomatosis, Orofacial ; Hemangioma, Cavernous ; Hemangioma, Cavernous, Central Nervous System ; Humans ; Paraneoplastic Syndromes

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

No abstract available.
Chorea ; Paraneoplastic Syndromes ; Small Cell Lung Carcinoma