Adult-Type Granulosa Cell Tumor with Similar Clinical Findings Seen during Ovarian Cystectomy Performed at the Same Time as Laparoscopic Ovarian Drilling for Polycystic Ovarian Syndrome: An Extremely Rare Case

Polycystic ovary syndrome (PCOS) is a major cause of anovulatory infertility. Laparoscopic ovarian drilling (LOD) is a treatment for PCOS that allows the laparoscopic identification of other intra-abdominal lesions and the provision of diagnostic treatment. This study reports a case of PCOS with an ovarian mass in which LOD was aggressively used and a granulosa cell tumor (GCT) was found. A 34-year-old woman with secondary amenorrhea and irregular menstrual cycles presented to the emergency department with abdominal pain of unknown etiology. Imaging studies revealed a 6-cm left ovarian mass with an internal appearance suggestive of a hemorrhage. The patient’s secondary amenorrhea was subsequently diagnosed as PCOS, and LOD was performed to preserve her fertility. Simultaneously, a cystectomy was performed to evaluate the tumor in the left ovary;the diagnosis was adult-type GCT. Although concomitant GCT and PCOS are extremely rare, the two conditions have similar clinical manifestations. In women of reproductive age, the impact of surgery on future fertility should be considered, and the initial surgical technique should be chosen carefully.

Clinicopathological and molecular insights into odontogenic tumors associated with syndromes:A comprehensive review

The association between genetic syndromes and odontogenic tumors encompasses several entities,reflecting the intricate interplay between genetic factors and the development of these lesions.The present study aimed to comprehensively investigate the associations between genetic syndromes and odontogenic tumors.We delineated the diverse spectrum of syndromic connections,including key syndromes such as Gardner syndrome,Gorlin syndrome,Schimmelpenning syndrome,and others.Our findings underscore the clinical significance of recognizing odontogenic tumors associated with genetic syndromes as diagnostic indicators for early intervention.We advocate for multidisciplinary collaboration among clinicians,geneticists,and researchers to deepen our understanding of the underlying mechanisms driving these syndromic associations.In light of this,our study contributes to the growing body of knowledge in dentistry and medical genetics,offering insights that may inform clinical practice and enhance patient care for individuals affected by genetic syndromes and odontogenic tumors.

Effects of increased human tumor necrosis factor-like molecule 1A expression in peripheral blood of children with acute Guillain-Barre syndrome on interferon-gamma secretion

BACKGROUND： Human tumor necrosis factor-like molecule 1A （hTL1A） is a strong T helper cell type 1 （Thl） co-stimulator. Guillain-Barre syndrome （GBS） is an autoimmune disorder of the nervous system, which is mediated by Thl cells. OBJECTIVE： To determine hTL1A expression in peripheral blood T lymphocytes of acute GBS children and the effects of hTL1A on secretion of interferon-γ. DESIGN, TIME AND SETTING： A randomized, controlled, neuroimmunological in vitro study was performed at the Central Laboratory of First Hospital of Jilin University, China from November 2005 to November 2007. MATERIALS： Venous blood samples were obtained from 6 healthy donors, aged 6-12 years （all routine blood examination items were normal）, and 6 additional children with acute GBS, aged 6-12 years. The GBS children fell ill within 1 week and were not treated with hormones or immunoglobulin Purified recombinant human soluble tumor necrosis factor-like molecule 1A （rhsTL1A, 1 mg/mL, relative molecular mass 22 000, 6× His tag, soluble form） was supplied by the Central Laboratory of First Hospital of Jilin University, China. METHODS： Peripheral blood mononuclear cells were isolated from healthy donors using the standard Ficoll gradient centrifugation and were incubated in 96-well culture plates. The cells were assigned to the following groups： control （2 μg/mL phytohemagglutinin）, 2μg/mL phytohemagglutinin ＋ 25, 100 and 400 ng/mL rhsTL1A. T cell proliferation was quantified using the tritiated thymidine （3H-TdR） method. Serum interferon-γ levels in acute GBS children were detected by enzyme-linked immunosorbent assay （ELISA）. The ratio of hTL1A-positive T cells to CD3-positive T cells in peripheral blood of acute GBS children was determined using flow cytometry. Following in vitro pre-activation of peripheral blood mononuclear cells by 2 μg/mL phytohemagglutinin, the peripheral blood mononuclear cells were treated with 400 ng/mL exogenous rhsTLIA. Finally, peripheral blood mononuclear cell-secreted interferon-γlevels were measured by ELISA. MAIN OUTCOME MEASURES： The following parameters were measured： rhsTLIA stimulation index to stimulate proliferation of T cells; the serum interferon-γ levels in acute GBS children; the ratio of hTL1A-positive cells to CD3-positive cells; the levels of interferon-γ secreted by peripheral blood mononuclear cells in acute GBS children, as well as rhsTL1A-stimulated interferon-γ levels. RESULTS： T cell proliferation assay revealed that the stimulation index in each rhsTL1A group was greater than the control group. The stimulation index of the 400 ng/mL rhsTL1A group was the greatest. Serum interferon-γ levels in acute GBS children were significantly greater than the control group （P 〈 0.05）. The ratio of hTLIA＋ CD3＋ T cells to CD3＋ T cells in acute GBS children was significantly greater than the control group （P 〈 0.01 ）. Phytohemagglutinin stimulated peripheral blood mononuclear cells to a greater extent than 400 ng/mL rhsTL1A in the acute GBS group, and the secreted interferon-γ levels were significantly increased （P 〈 0.05）. CONCLUSION： In T cells pre-activated with 2 μg/mL phytohemagglutinin, proliferation was effectively increased with 400 ng/mL rhsTL1A treatment. Expression of hTLIA was increased in activated T cells from peripheral blood of acute GBS children, followed by increased interferon-γ secretion. These mechanisms are considered to be part of the pathological process that induces the secretion of inflammatory cytokines in GBS syndrome.

The Change of Interleukin-6 and Tumor Necrosis Factor in Patients with Obstructive Sleep Apnea Syndrome

The levels of lipopolysaccharide (LPS) induced interleukin 6 (IL 6) and tumor necrosis factor α (TNF α) expression in culture of peripheral blood mononuclear cells (PBMC) and the plasma levels of IL 6 and TNF α in the patients with obstructive sleep apnea syndrome (OSAS) were measured and the relationship between OSAS and IL 6 or TNF α expression studied. Both IL 6 and TNF α were detected by using ELISA in 22 patients with OSAS and 16 normal controls. The levels of LPS induced IL 6 (787.82±151.97 pg/ml) and TNF α (4165.45±1501.43 pg/ml) expression in the supernatant of the culture of PBMC and plasma level of IL 6 (50.67±4.70 pg/ml) and TNF α (299.09±43.57 pg/ml) in the patients with OSAS were significantly higher than those in the normal controls (in the supernatant of the culture of PBMC: 562.69±197.54 pg/ml and 1596.25±403.08 pg/ml respectively; in the plasma: 12.69±2.75 pg/ml and 101.88±21.27 pg/ml respectively). There were significantly positive correlation between the levels of IL 6 and TNF α and the percentage of time of apnea and hyponea, as well as the percentage of time spending at SaO 2 below 90 % in the total sleep time. It was concluded that LPS induced IL 6 and TNF α levels as well as plasma IL 6 and TNF α levels in the patients with OSAS were up regulated, which may be associated with the pathogenesis of OSAS.

Tumor lysis syndrome after transarterial chemoembolization of hepatocellular carcinoma: Case reports and literature review

Tumor lysis syndrome (TLS) is a potentially lethal complication in cancer therapy. It may occur in highly sensitive tumors, especially in childhood cancer and leukemia, whereas, it is rare in the treatment of solid tumors in adults. TLS results from a sudden and rapid release of nuclear and cytoplasmic degradation products of malignant cells. The release of these can lead to severe alterations in the metabolic prof ile. Here, we present two cases of large hepatocellular carcinoma (HCC) treated by transarterial chemoembolization (TACE) that resulted in TLS. Although TLS rarely happens in the treatment of adult hepatic tumor, only a few cases have been reported. We should keep in mind that all patients with HCC, particularly those with large and rapidly growing tumors, must be closely watched for evidence of TLS after TACE.

Tumor lysis syndrome: A clinical review

Tumor lysis syndrome is an oncometabolic emergency resulting from rapid cell death. Tumor lysis syndrome can occur as a consequence of tumor targeted therapy or spontaneously. Clinicians should stratify every hospitalized cancer patient and especially those receiving chemotherapy for the risk of tumor lysis syndrome. Several aspects of prevention include adequate hydration, use of uric acid lowering therapies, use of phosphate binders and minimization of potassium intake. Patients at high risk for the development of tumor lysis syndrome should be monitored in the intensive care unit. Established tumor lysis syndrome should be treated in the intensive care unit by aggressive hydration, possible use of loop diuretics, possible use of phosphate binders,use of uric acid lowering agents and dialysis in refractory cases.

Gynecological tumors in patients with Peutz-Jeghers syndrome (PJS)

Peutz-Jeghers syndrome (PJS) is an autosomal dominant disorder characterized by the development of hamartomatous polyposis in the gastrointestinal tract and melanin-pigmented macules on the skin mucosa. The responsible gene is a tumor suppressor, STK11/LKB1, on chromosome 19p13.3. PJS complicates with benign and malignant tumors in various organs. In gynecology, there has been a particular focus on complications of PJS with sex cord tumor with annular tubules (SCTAT) and minimal deviation adenocarcinoma (MDA), which are rare diseases. Approximately 36% of patients with SCTAT are complicated with PJS and these patients are characterized by multifocal, bilateral, small and benign lesions that develop into tumors with mucinous to serous ratios of 8:1. In addition, 10% of cases of MDA are complicated with PJS and mutation of STK11, the gene responsible for PJS, has a major effect on onset and prognosis. The disease concept of lobular endocervical glandular hyper-plasia (LEGH) has recently been proposed and LEGH is thought to be a potential premalignant lesion of MDA, however, the relationship between PJS and LEGH remains unclear. Several case reports of PJS patients complicated with gynecological tumors have been published and further studies are needed to determine the underlying

Primary Sjgren's syndrome related optic neuritis

AIM:To determine the clinical features,diagnosis and treatment of the primary Sjogren syndrome(SS)related optic neuritis.METHODS:The clinical data of 8 patients(12 eyes)with primary SS related optic neuritis were analyzed retrospectively.RESULTS:Eight of 128 consecutive patients with optic neuritis resulted from varied causes fulfilled the diagnostic criteria for the primary SS.They presented initially with the signs and symptoms of non-specific optic neuritis,and 5 patients presenting without dryness showed a chronic inflammation of submandibular gland or parotid gland,and lymphocyte infiltration was demonstrated by labial gland biopsy in 2 patients.There were serum positive titers for anti-Sjogren syndrome A(SSA)in 7 patients and anti-Sjogren syndrome B(SSB)in 8 patients.Anti-aquaporin-4(AQP4)antibody was negative in all the 8 patients.Both glucocorticoids and immunosuppressive agent were administered,and visual acuity elevated in 8 eyes(66.7%),3 patients(37.5%)recurred in the follow-up.CONCLUSION:Primary SS related optic neuritis is less common and easily misdiagnosed.The conventional therapies for optic neuritis could not control the recurrence.

Sorafenib induced tumor lysis syndrome in an advanced hepatocellular carcinoma patient

A 55-year-old male patient with hepatitis B-related liver cirrhosis was found to have advanced hepatocellular carcinoma.His AFP was initially 9828μg/L and rapidly dropped to 5597μg/L in ten days after oral sorafenib treatment.However,he developed acute renal failure, hyperkalemia,and hyperuricemia 30 d after receiving the sorafenib treatment.Tumor lysis syndrome was suspected and intensive hemodialysis was performed. Despite intensive hemodialysis and other supportive therapy,he developed multiple organ failure(liver, renal,and respiratory failure)and metabolic acidosis. The patient expired 13 d after admission.

DETERMINATION OF URINE TUMOR NECROSIS FACTOR, IL-6, IL-8 AND SERUM IL-6 IN PATIENTS WITH HEMORRHAGIC FEVERS WITH RENAL SYNDROME

Objective To explore the roles of cytokines in the pathogenesis of hemorrhagic fever with renal syndrome(HFRS). Methods Double-antibody sandwich ELISA was used to determine serum interleukin (IL)-6, urine tumor necrosis factor (TNF), IL-6 and IL-8 levels in 56 patients with HFRS. Results Serum IL-6, urine TNF, IL-6 and IL-8 concentrations in HFRS patients were significantly higher than those in control group, respectively (P<0.001). The concentrations increased at fever stage, then continued to increase during hypotension stage and peaked at oliguria stage. The concentrations of serum IL-6, urine TNF, IL-6 and IL-8 increased in accord with the severity of the disease and differed greatly among different types of the disease. Serum IL-6 had remarkable relationships with serum specific antibodies. It was positively related to serum β_2-microglobulin (β_2-MG), blood ureanitrogen (BUN) and creatinine (Cr). Significant positive relationships were also found both between urine IL-6 and TNF, and between IL-6 and IL-8 (r=0.5768, P<0.05; r=0.3760, P<0.01). Conclusion TNF, IL-6 and IL-8 activated during the course of the disease. IL-6 is associated with the immunopathological lesions caused by the hyperfunction of humoral immune response. IL-6, IL-8 and TNF are involved in the renal immune impairment. Determining them might, in certain extent, be used in predicting the prognosis and outcome of patients with HFRS.

Postoperative secondary aggravation of obstructive sleep apneahypopnea syndrome and hypoxemia with bilateral carotid body tumor:A case report

BACKGROUND Carotid body tumor(CBT)is a chemoreceptor tumor located in the carotid body,accounting for approximately 0.22%of head and neck tumors.Surgery is the main treatment method for the disease.CASE SUMMARY We reviewed the diagnosis and treatment of one patient who had postoperative secondary aggravation of obstructive sleep apnea–hypopnea syndrome(OSAHS)and hypoxia after surgical resection of bilateral CBTs.This patient was admitted,and relevant laboratory and imaging examinations,and polysomnography(PSG)were performed.After the definitive diagnosis,continuous positive airway pressure(CPAP)treatment was given,which achieved good efficacy.CONCLUSION This case suggested that aggravation of OSAHS and hypoxemia is possibly caused by the postoperative complications after bilateral CBTs,and diagnosis by PSG and CPAP treatment are helpful for this patient.

Meigs’syndrome caused by granulosa cell tumor accompanied with intrathoracic lesions:A case report

BACKGROUND Meigs’syndrome is regarded as a benign ovarian tumor accompanied by pleural effusion and ascites,both of which resolve after removal of the tumor.Patients often seek treatment in the Department of Respiratory and Critical Care Medicine or other internal medicine departments due to symptoms caused by ascites or hydrothorax.Here,we report a rare case of Meigs'syndrome caused by granulosa cell tumor accompanied with intrathoracic lesions.CASE SUMMARY A 52-year-old women was admitted to the Department of Respiratory and Critical Care Medicine due to coughing and expectoration accompanied with shortness of breath.Chest X-ray and chest computed tomography showed a modest volume of pleural fluid with pleural thickening in the right lung.The carbohydrate antigen 125(CA125)concentration was 150.8 U/mL(normal,0-35 U/mL)and no tumor cells were observed in pleural fluid.Nodules and a neoplasm with a fish meat-like appearance in the parietal pleura and nodules with a‘string of beads’-like appearance in the diaphragm were found by thoracoscopic examination.Furthermore,pelvic magnetic resonance revealed a pelvic mass measuring about 11.6 cm×10.0 cm×12.4 cm with heterogeneous signal intensity and multiple hypointense separations.Total abdominal hysterectomy,bilateral adnexectomy,and separation of pelvic adhesion were performed under general anesthesia.The pathology results showed granulosa cell tumor.At the 2-mo follow-up after the surgery,the hydrothorax subsided,and the CA125 level returned to normal.CONCLUSION For postmenopausal women with unexplained hydrothorax and elevated CA125,in addition to being suspected of having gynecological malignancy,Meigs’syndrome should be considered.

Tumor-related cytokine release syndrome in a treatment-naïve patient with lung adenocarcinoma:A case report

BACKGROUND Cytokine release syndrome(CRS)is defined as systemic inflammation that usually occurs following chimeric antigen receptor T-cell therapy administration;however,it has not been reported in patients with untreated non-small cell lung cancer to date.CASE SUMMARY A 44-year-old nonsmoking woman presented to the hospital due to fever,palpitation,nausea,and cough for 1 mo and was diagnosed with stage cT3N3M0(IIIc)adenocarcinoma of the lung.Auxiliary examinations revealed elevated cytokine[tumor necrosis factor-α,interleukin(IL)-1β,and IL-6]and inflammatory factor levels,which decreased after treatment with corticosteroids and immunoglobulin and when tumor growth was controlled following chemotherapy,radiotherapy,and antiangiogenesis therapy.However,tumor recurrence was observed.After administration of nivolumab as third-line treatment,the patient’s condition was transiently controlled;however,CRS-like symptoms suddenly emerged,which led to a resurgence of cytokines and inflammatory factors and rapid death.CONCLUSION CRS can develop in treatment-naïve lung cancer patients.Patients with tumorrelated CRS may be at risk of CRS recurrence,aggravation,and onset of immune checkpoint inhibitor-related adverse events.

Predictive factors associated with carcinoid syndrome in patients with gastrointestinal neuroendocrine tumors

AIM To discover unknown factors associated with carcinoid syndrome(CS) with the goal of earlier diagnosis of CS.METHODS In this retrospective case-control study using United States administrative claims, patients(≥ 18 years) newly-diagnosed with gastrointestinal neuroendocrine tumors(GI NETs) without CS(controls) were exactly matched to patients with CS(cases) based on NET diagnosis date at a 3-to-1 ratio. Study index date was first CS diagnosis(controls: same distance from NET diagnosis as cases). The most observed conditions, excluding CS-associated symptoms/diagnoses, during the year before index date were assessed. Forwardstepwise logistic regression models were used to derive predictors, and were validation within another claims database. RESULTS In the development database, 1004 patients with GI NETs were identified; 251(25%) had CS and 753(75%) were controls. In the validation database, 724 patients with GI NETs were identified; 181(25%) had CS and 543(75%) were controls. A total of 33 common diagnoses(excluding conditions already known to be associated with CS) in the development database were entered in forward step-wise logistic regression models. In the final, validated logistic regression model, three factors prior to CS diagnosis were found consistently associated with higher risks for CS, including liver disorder [odds ratio(95%CI): 3.38(2.07-5.51)], enlargement of lymph nodes [2.13(1.10-4.11)], and abdominal mass [3.79(1.87-7.69)].CONCLUSION GI NET patients with CS were 2-4 times as likely to have preexisting diagnoses(i.e., liver disorder, enlarged lymph nodes, abdominal mass) than non-CS patients.

Relationship of Tumor Necrosis Factor-α and Nitrogen Oxide with Treatment of Frequent Relapse Nephrotic Syndrome by Shenkangling(肾康灵)Granule in Children

Objective: To observe the relationship of tumor necrosis factor-o (TNF-a) and nitrogen oxide (NO) with the treatment of frequent relapse nephrotic syndrome (FRNS) and to explore the patho-genesis of FRNS and the therapeutic mechanism of Shenkangling (肾康灵,SKL) Granule in children. Methods: Sixty children suffering from FRNS were randomly divided into the treated group and control group, 30 in each, and the other 30 healthy children were taken as healthy group. The patients were treated with prednisone for a long-term course, and those with no effect or partial effect shown were treated with additional Tripterygium or Cytoxan in the control group, while in the treated group patients were treated with prednisone and additional SKL. The two groups were compared as to their changes of TNF-a, NO before and after treatment, and the relapses after treatment. Results: The levels of TNF-a and NO in the sick children before treatment were markedly higher than those after treatment and normal group (P< 0. 01). The positive correlation between TNF-o of FRNS cases and relapse risk displayed more significance than that between the relapse of FRNS and NO. The difference between treated group and control group was significant (P<0. 01). Conclusion: TNF-a can be regarded as the monitoring parameter of the active phase in FRNS, and the higher the level, the more possible the relapse would occur. SKL could markedly reduce the relapse rate of FRNS in children.

Carcinoid tumor of the common bile duct: A rare complication of von Hippel-Lindau syndrome

Von HippeI-Lindau syndrome （VHL） is a rare autosomal-dominant, inherited familial cancer syndrome. Hemangioblastomas, pheochromocytomas and renal carcinoma are the frequent reported VHL tumors. Neuroendocrine tumors have also been described, mostly in the pancreas and rarely in the biliary trees. We report the second case of bile duct carcinoid in a 31-year-old VHL woman. She underwent right adrenalectomy for a pheochromocytoma in the past. She also had a positive family history of phenotypic expression of VHL syndrome. The patient presented with biliary colic. Endoscopic retrograde cholangio-pancreatography showed intraluminal bile duct mass. Surgical exploration identified a beige nodular lesion that was a carcinoid tumor on histology. This new association should be clarified by further genetic investigations.

Case of pseudo-Meigs' syndrome caused by gastric cancer-related metastatic ovarian tumor with prolonged survival

A 48-year-old woman presented with bilateral enlarged ovaries, ascites, bilateral pleural effusion, and advanced gastric cancer. Pleural fluid cytology did not reveal malignant cells. Oophorectomy, performed as a palliative procedure, was followed by rapid resolution of the pleural effusion and ascites. The patient was diagnosed with pseudo-Meigs&rsquo; syndrome, and underwent chemotherapy followed by partial gastrectomy. At the last follow-up, 84 mo following oophorectomy, she was alive, and free of disease recurrence, despite not receiving any further treatment. Pseudo-Meigs&rsquo; syndrome should be considered in patients with bilateral ovarian tumors, ascites and pleural effusion, and treatment such as oophorectomy may result in symptomatic improvement and better prognosis in similar patients.

Uncharted Territory: Frequent Relapsing, Steroid Sensitive Secondary Minimal Change Nephrotic Syndrome Cause by Solid Tumor of the Gastro-Esophageal Junction —(Case Presentation and Review of the Literature)

We reported a biopsy proved case of minimal change nephrotic syndrome in a 72-year-old patient. The minimal change nephrotic syndrome has been steroid sensitive, but the patient had 7 relapses over a span of 5 years. Each time the dose of steroid is tapered, a relapse of the nephrotic syndrome occurred. Eventually, the patient was complaining of dysphagia and difficulty swallowing. Hospital work-up with barium swallow, endoscopy, and CT of the chest, abdomen and pelvis, revealed a focal stenotic lesion with mild to moderate esophageal dysmotility 7/15/2022. A diagnosis of an ulcerating lesion with biopsy confirmed a neuro-endocrine carcinoma of the gastro-esophageal junction was entertained. The CT of the chest/abdomen/pelvis, 7/19/2022, has shown, an esophageal mass of 5.1 × 5.6 × 7 cm of the gastro-esophageal junction with ulceration. No evidence of spread beyond the esophagus and stomach. The histology revealed a poorly differentiated neuroendocrine tumor of the gastro-esophageal junction. The patient underwent several rounds of chemotherapy, radiation, and surgery culminating in tumor control. His nephrotic syndrome was resolved after the tumor has been controlled by surgery and chemotherapy.

Tumor Susceptibility in Proteus Syndrome: a Case Report

PROTEUS syndrome is characterized by patchy and progressive overgrowth affecting multiple tissues, including bone, soft tissue, and skin, along with susceptibility to the development oftumors. It was originally described by Cohen and Hayden in 19791 and named by Wiedmann in 1983.2 The cause of Proteus syndrome is proposed to be a somatic mutation that is lethal in non-mosaic state; cells derived from the mutated cell line carrying this mutation result in anomalies in multiple tissues.3 The clinical manifestations are variable,

Factors influencing Frey syndrome after parotidectomy with acellular dermal matrix

BACKGROUND Frey syndrome,also known as ototemporal nerve syndrome or gustatory sweating syndrome,is one of the most common complications of parotid gland surgery.This condition is characterized by abnormal sensations in the facial skin accompanied by episodes of flushing and sweating triggered by cognitive processes,visual stimuli,or eating.AIM To investigate the preventive effect of acellular dermal matrix(ADM)on Frey syndrome after parotid tumor resection and analyzed the effects of Frey syndrome across various surgical methods and other factors involved in parotid tumor resection.METHODS Retrospective data from 82 patients were analyzed to assess the correlation between sex,age,resection sample size,operation time,operation mode,ADM usage,and occurrence of postoperative Frey syndrome.RESULTS Among the 82 patients,the incidence of Frey syndrome was 56.1%.There were no significant differences in sex,age,or operation time between the two groups(P>0.05).However,there was a significant difference between ADM implantation and occurrence of Frey syndrome(P<0.05).ADM application could reduce the variation in the incidence of Frey syndrome across different operation modes.CONCLUSION ADM can effectively prevent Frey syndrome and delay its onset.