Refractory systemic capillary leak syndrome treated with bevacizumab: a case report.

Systemic capillary leak syndrome (SCLS) is a syndrome caused by many reasons and without a definitive mechanism. The main diagnostic criteria of SCLS are hemoconcentration, hypoalbuminemia, and hypotension. Though most SCLS improved spontaneously within a few days, it can be life-threatening without effective treatments. In previous literature, vascular endothelial growth factor (VEGF) inhibitor had shown its potential to be an effective treatment, but the treatment outcomes were inconsistent. This article was about a 58-year-old female suffering from refractory systemic capillary leak syndrome after bone marrow transplantation and being treated with bevacizumab, a VEGF inhibitor. In comparison with other successfully treated cases, this patient received four cycles of bevacizumab treatment without symptomatic improvement and eventually died in the intensive care unit. Further studies are needed to further confirm the role of bevacizumab in the management of SCLS.

A noninterventional observational registry of patients with multiple myeloma treated with lenalidomide in Taiwan.

BACKGROUND/PURPOSE: The incidence of multiple myeloma in Asia has risen in the past 30 years. Lenalidomide, an IMiD immunomodulatory agent, has improved the overall survival in patients with relapsed/refractory multiple myeloma (RRMM) when used with dexamethasone versus dexamethasone alone. This observational registry (T-CC-MM-009; NCT01752075) assessed the safety and efficacy of lenalidomide plus dexamethasone in a large Chinese population of patients with RRMM. METHODS: This registry followed the first 100 patients treated with lenalidomide plus dexamethasone in Taiwan. Patients were ≥18 years old and had ≥1 prior treatment. The recommended starting dose for the first four 28-day cycles was 25 mg lenalidomide on days 1-21 and 40 mg dexamethasone on days 1-4, 9-12, and 17-20. Thereafter, dexamethasone was given on days 1-4 only. The primary objective was safety; secondary objectives were efficacy, lenalidomide dosage, and reasons for discontinuation. RESULTS: The median duration of treatment was 34.6 weeks, and 75.5% completed ≥3 cycles. Most patients (82.7%) experienced ≥1 treatment-related adverse event; the most commonly reported were neutropenia (23.5%), thrombocytopenia (19.4%), anemia (16.3%), fatigue (16.3%), and hypoesthesia (15.3%). Bleeding events (25.5% of patients) were mostly grade 1/2 (80%). Three patients (3%) had venous thromboembolic events. Two invasive second primary malignancies were reported; however, time to onset was <1 year, suggesting they may not be related to lenalidomide. The overall response rate was 34.7%; median time to disease progression was 20.5 months. CONCLUSION: These data confirm the safety and efficacy of lenalidomide plus dexamethasone for patients with RRMM in Taiwan.

A phase I clinical study of immunotherapy for advanced colorectal cancers using carcinoembryonic antigen-pulsed dendritic cells mixed with tetanus toxoid and subsequent IL-2 treatment.

BACKGROUND: To better evaluate and improve the efficacy of dendritic cell (DC)-based cancer immunotherapy, we conducted a clinical study of patients with advanced colorectal cancer using carcinoembryonic antigen (CEA)-pulsed DCs mixed with tetanus toxoid and subsequent interleukin-2 treatment. The tetanus toxoid in the vaccine preparation serves as an adjuvant and provides a non-tumor specific immune response to enhance vaccine efficacy. The aims of this study were to (1) evaluate the toxicity of this treatment, (2) observe the clinical responses of vaccinated patients, and (3) investigate the immune responses of patients against CEA before and after treatment. METHODS: Twelve patients were recruited and treated in this phase I clinical study. These patients all had metastatic colorectal cancer and failed standard chemotherapy. We first subcutaneously immunized patients with metastatic colorectal cancer with 1 × 10(6) CEA-pulsed DCs mixed with tetanus toxoid as an adjuvant. Patients received 3 successive injections with 1 × 10(6) CEA-pulsed DCs alone. Low-dose interleukin-2 was administered subcutaneously following the final DC vaccination to boost the growth of T cells. Patients were evaluated for adverse event and clinical status. Blood samples collected before, during, and after treatment were analyzed for T cell proliferation responses against CEA. RESULTS: No severe treatment-related side effects or toxicity was observed in patients who received the regular 4 DC vaccine injections. Two patients had stable disease and 10 patients showed disease progression. A statistically significant increase in proliferation against CEA by T cells collected after vaccination was observed in 2 of 9 patients. CONCLUSIONS: The results of this study indicate that it is feasible and safe to treat colorectal cancer patients using this protocol. An increase in the anti-CEA immune response and a clinical benefit was observed in a small fraction of patients. This treatment protocol should be further evaluated in additional colorectal cancer patients with modifications to enhance T cell responses. TRIAL REGISTRATION: ClinicalTrials.gov (identifier NCT00154713 ), September 8, 2005.

Chemotherapy-induced hepatitis B reactivation in lymphoma patients with resolved HBV infection: a prospective study.

UNLABELLED: Fatal hepatitis B virus (HBV) reactivation in lymphoma patients with "resolved" HBV infection (hepatitis B surface antigen [HBsAg] negative and hepatitis B core antibody [anti-HBc] positive) can occur, but the true incidence and severity remain unclear. From June 2009 to December 2011, 150 newly diagnosed lymphoma patients with resolved HBV infection who were to receive rituximab-CHOP (cyclophosphamide, doxorubicin, vincristine, prednisolone)-based chemotherapy were prospectively followed. HBV DNA was checked at baseline, at the start of each cycle of chemotherapy, and every 4 weeks for 1 year after completion of rituximab-CHOP chemotherapy. Patients with documented HBV reactivation were treated with entecavir at a dosage of 0.5 mg/day for 48 weeks. HBV reactivation was defined as a greater than 10-fold increase in HBV DNA, compared with previous nadir levels, and hepatitis flare was defined as a greater than 3-fold increase in alanine aminotransferase (ALT) that exceeded 100 IU/L. Incidence of HBV reactivation and HBV-related hepatitis flares was 10.4 and 6.4 per 100 person-year, respectively. Severe HBV-related hepatitis (ALT >10-fold of upper limit of normal) occurred in 4 patients, despite entecavir treatment. Patients with hepatitis flare exhibited significantly higher incidence of reappearance of HBsAg after HBV reactivation (100% vs. 28.5%; P=0.003). CONCLUSION: In lymphoma patients with resolved HBV infections, chemotherapy-induced HBV reactivation is not uncommon, but can be managed with regular monitoring of HBV DNA and prompt antiviral therapy. Serological breakthrough (i.e., reappearance of HBsAg) is the most important predictor of HBV-related hepatitis flare. (Hepatology 2014;59:2092-2100).

The treatment outcome of multiple myeloma patients ineligible for hematopoietic transplantation--a single institutional experience in Taiwan.

Multiple myeloma (MM) is characterized by the neoplastic proliferation of monoclonal plasma cells in the bone marrow and results in complications. In Taiwan, melphalan and several novel agents are used to treat myeloma patients who are not candidate for hematopoietic stem cell transplant (HSCT). This retrospective study aimed to evaluate the characteristics, treatment outcome, and prognostic factors of MM patients who were ineligible for HSCT at our institution from October 2000 until November 2012. A total of 101 MM patients were reviewed. The median age was 71.0 years, and median overall survival (OS) was 22.0 months. Most of patients were diagnosed as IgG-type myeloma (55.4 %). The initial presentations included anemia (89.1 %), skeletal events (49.5 %), severe renal insufficiency (30.7 %), and hypercalcemia (28.7 %). With regard to the frontline therapy, thalidomide/steroid was the most common. Infection was the leading cause of death and adverse effects. Treatment with bortezomib, almost in the second- or third-line setting, was associated with longer median OS (35.5 months) and the median time to progression (TTP) (6.0 months). Bortezomib treatment, chemotherapy, International Staging System (ISS) stage I, and better performance status significantly correlated with survival benefit. In the bortezomib-treated subgroup, better treatment response caused excellent median OS (67.7 months) and also significantly delayed TTP. Therefore, this current analysis concluded a median OS of 22 months in myeloma patients ineligible for HSCT at our institution during the past 10 years. The use of bortezomib with better treatment response also achieved significantly better median OS and TTP.

Long-term follow-up of cancer and catastrophic diseases in hematopoietic stem cell donors: a comprehensive matched cohort study.

Hematopoietic stem cell (HSC) transplantation, using either bone marrow (BM) or peripheral blood stem cells (PBSC), is a well-established therapy for various hematologic and non-hematologic diseases. However, the long-term health outcomes after HSC donation remain a major concern for several potential donors. Thus, we aimed to conduct a matched cohort study of 5003 unrelated donors (1099 BM and 3904 PBSC) and randomly selected 50,030 matched controls based on age, sex, and resident area from the donor registry between 1998 and 2018. The medical insurance claims of all the participants were retrieved from the Taiwan National Health and Welfare Data Science Center after de-identification. Our findings revealed no differences in the incidence of cancer, death, and catastrophic diseases between HSC donors and matched healthy participants during long-term follow-up. Kaplan-Meier curves depicting the cumulative incidence of cancer and overall mortality throughout the follow-up period also demonstrated similar outcomes between donors and non-donors. In conclusion, our results indicate that HSC donation, whether through BM or PBSC, is safe and not associated with an increased risk of cancer, death, or catastrophic diseases. These findings provide valuable information for counseling potential HSC donors and for long-term management of HSC donor health.

Acute hemolytic anemia in glucose-6-phosphate dehydrogenase deficiency complicated by Ginkgo biloba.

We report on a patient with glucose-6-phosphate dehydrogenase (G6PD) deficiency who developed acute hemolytic anemia after having received an injection of Ginkgo biloba for dementia prophylaxis without medical advice. She suddenly developed general malaise, generalized yellowish skin color, and tea-colored urine. Intravenous fluid infusion and cessation of G. biloba quickly relieved her clinical symptoms. To the best of our knowledge, this is the first case report of G. biloba-induced acute hemolytic anemia in vivo.

Comparing miR-16 and miR-1228 as an optimal endogenous control for quantification of circulating microRNAs in colorectal cancer patients.

Objectives: Circulating microRNAs (miRNAs) have been discovered to play a novel role in intercellular communication and cancer biology. They are emerging candidates for noninvasive molecular biomarkers of cancer and other diseases. However, current translational researches have been limited by the lack of consensus on the optimal endogenous control of circulating miRNAs quantitation. In this study, we compared two promising miRNAs, miR-1228 and miR-16, as an endogenous control. The effects of normalizers on the relative quantification of circulating miR-31 in plasma samples of colorectal cancer (CRC) were also assessed. Materials and Methods: The cel-miR-39 was a spiked-in RNA used as an external control and added to plasma samples before RNA extraction. Quantitative real-time polymerase chain reaction technology was used to analyze the expression levels of circulating miRNAs in plasma samples of 4 healthy controls and 14 CRC patients. The expression stability of the candidate controls was compared by Ct analysis and NormFinder algorithms. Results: There was no significant difference in expression level of miR-16 and miR-1228 between healthy control group and before or after therapy of CRC patient groups. The expression of miR-1228 has smaller the range Ct values (28.25-25.64)compared with those of miR-16 (24.91-20.34). The stability value of miR-1228 (0.102) is lower than that of miR-16 (0.350). The expression of miR-1228 endogenous reference candidate has lower stability value and smaller the range Ct values compared with those in miR-16. According to the range Ct values and stability value, miR-1228 is better than miR-16 as endogenous control in CRC patients. There are significant differences in circulating miR-31 expression between healthy control and CRC patients when miR-1228 was used to standardize miR-31 expression. Conclusions: miR-1228 is recommended as a better endogenous control in quantification of circulating miRNAs in CRC patients.

Preventing chemotherapy-induced hepatitis B reactivation in breast cancer patients: a prospective comparison of prophylactic versus deferred preemptive lamivudine.

PURPOSE: Prophylactic lamivudine to prevent chemotherapy-induced hepatitis B virus (HBV) reactivation has been widely adopted in hematological cancer patients. We examined the deferred preemptive strategy, upon rising viremia, in breast cancer (BC) patients based on sensitive serum HBV DNA level monitoring in a non-randomized controlled study. PATIENTS AND METHODS: Baseline virological profiles before cytotoxic chemotherapy were retrospectively analyzed in historical BC and non-BC patients. A prospective cohort study, including 22 early BC patients (Group I) who were hepatitis B surface antigen (HBsAg)± and required adjuvant chemotherapy, were enrolled and had deferred preemptive use of lamivudine upon viremic surge. During the study period, another 23 BC patients, who did not participate in the above-mentioned study, received prophylactic use of lamivudine as routine practice (Group 2). Chemotherapy-induced hepatitis events and the lamivudine treatment course were compared. RESULTS: There was no significant difference in the incidence of hepatitis during chemotherapy between these two groups. Patients in Group I had statistically significant shorter duration of lamivudine use during chemotherapy. However, once lamivudine had been initiated, the treatment course is not significantly shorter than those patients given prophylactically. CONCLUSIONS: Deferred preemptive strategy is feasible to control HBV replication and prevent its reactivation in BC patients undergoing chemotherapy. However, it may not be superior to prophylactic strategy and clinically practical.

Evaluating the benefit of adjuvant radiotherapy after extensive lymph node dissection for gastric cancer: a single-institute retrospective study.

OBJECTIVE: This study aimed to evaluate whether adjuvant radiotherapy (RT) can improve the treatment outcome of patients with locally advanced gastric cancer who underwent extensive lymph node dissection (ELND). MATERIALS AND METHODS: This retrospective study included patients with gastric cancer pathological stages IIA-IIIC at Taipei Tzu Chi Hospital between 2008 and 2015. Patients (a) aged >80 years, (b) with distant metastasis at diagnosis, (c) with coexisting malignancies, (d) who did not complete the prescribed RT course, and (e) who died 1 month after surgery were excluded. Among 420 patients diagnosed with gastric cancer, 98 were included. RESULTS: The median follow-up was 24.5 months. Of 39 patients who underwent adjuvant RT, 38 also received adjuvant chemotherapy (CT). Of 59 patients who did not receive adjuvant RT, only 34 received adjuvant CT. ELND was performed in 67.3% of the patients. The 5-year overall survival (OS) rate was 40%. In the univariate analyses, adjuvant CT regimen, 5-fluorouracil + leucovorin, was associated with worst outcome, while TS-1 was associated with better survival outcome (P = 0.018). The number of involved lymph nodes was strongly related to the OS and disease-free survival (DFS) (P < 0.001). We tried using different numbers of involved lymph nodes as a cutoff point and found that adjuvant RT significantly improved both OS and DFS in patients whose involved lymph nodes were ≥4 (OS, P = 0.017; DFS, P = 0.015). In multivariate analyses, better DFS was associated with negative surgical margin (P = 0.04), earlier disease stage (P = 0.001), adjuvant radiotherapy (P = 0.045), and adjuvant CT regimen TS-1 (P = 0.001). CONCLUSION: Adjuvant RT could improve DFS of patients with locally advanced gastric cancer with or without ELND. When the number of involved lymph nodes is ≥4, adjuvant RT is strongly suggested.

The clinicopathological analysis of 303 cases with malignant lymphoma classified according to the World Health Organization classification system in a single institute of Taiwan.

Several reports have shown a different distribution of malignant lymphoma (ML) in Asian and Western populations. The purpose of our survey was to elucidate whether there are substantial differences in the frequencies of subtypes of ML between different geographical areas. All entities diagnosed as ML between June 1995 and December 2007 were selected according to the 2008 World Health Organization (WHO) classification and searched for clinical outcomes. The cases were retrieved and reviewed by a panel of clinical haematologists and haematopathologists. A total of 303 patients with ML were identified for retrospective analysis. Of the 303 patients with ML, 278 patients (91.7%) had non-Hodgkin's lymphoma (NHL), and 25 (9.2%) had Hodgkin's lymphoma. Of the 278 patients with NHL, 223 (73.6%) had lymphoma of B-cell lineage, and 55 (18.1%) had lymphoma of T-cell lineage. One hundred and thirty-seven patients were diagnosed with diffuse large B-cell lymphoma, which was the most common B-cell lineage subtype and accounted for 45.2% of patients with NHL. Peripheral T-cell lymphomas were the most frequent subset of the T-cell neoplasms, comprising 10.6% of ML. Extranodal involvement was found in 125 (44.9%) of the 278 patients with NHL, and the lymph node was the site of primary involvement in 153 patients (55.1%). Fifty-nine (47.2%) of the 125 patients with extranodal presentation had gastrointestinal tract involvement. Outcome was worse in patients with extranodal NHL than in those with nodal NHL through the entire follow-up period; the difference in survival rates was significant. Our findings clarify the applicability and prognostic relevance of the WHO classification system and provide further information about the incidence of various lymphoma subtypes in Taiwan. Primary extranodal NHL was associated with a worse prognosis and distinct characteristics compared with nodal NHL. The outcome of different types of extranodal NHL should be investigated further.

An unusual radiologic pattern of cryptogenic organizing pneumonia: diffuse pulmonary nodules in a leukemia patient.

The radiological appearance of diffuse discrete pulmonary nodules associated with cryptogenic organizing pneumonia (COP) has been rarely described. We describe a case of COP in 49-year-old woman with acute myeloid leukemia who developed diffuse pulmonary nodules during the second course of induction chemotherapy. The clinical status of the patient and imaging findings suggested the presence of a pulmonary metastasis or infectious disease. A video-assisted thoracoscopic lung biopsy resulted in the unexpected diagnosis of COP as an isolated entity. Steroid therapy led to dramatic improvement of the clinical symptoms and the pulmonary lesions.

Spontaneous Splenic Rupture as a Rare Initial Presentation in an Acute Lymphoblastic Leukemia Patient.

A spontaneous rupture of the spleen is a rare but critical diagnosis of an acute abdomen, which may accompany unspecific symptoms mimicking acute pancreatitis, rupture of aortic aneurism, or acute coronary syndrome, delaying diagnosis and treatment. In patients that have experienced a severe spleen rupture, hypovolemic shock may cause catastrophic clinical outcomes. Therefore, early diagnosis is very important in order for physicians to declare the etiology for prevention and timely correction of the shock status. Several causes of spontaneous splenic rupture have been reported, including infection, vasculitis, pancreatitis, or hematological malignancies. Acute lymphoblastic leukemia (ALL) remains a rare but important cause of non-traumatic splenic rupture that physicians are required to assess for. Here, we describe a case presenting an acute abdomen due to spontaneous spleen rupture as the first manifestation. The purpose of this case report was to highlight the importance of considering spontaneous ruptures of the spleen as a rare but critical differential diagnosis of an acute abdomen, especially in patients with acute lymphoblastic leukemia.

High efficacy of erlotinib in Taiwanese NSCLC patients in an expanded access program study previously treated with chemotherapy.

PURPOSE: Erlotinib is the first epidermal growth factor receptor-tyrosine kinase inhibitor (EGFR-TKI) which has demonstrated a survival benefit in non-small-cell lung cancer (NSCLC) patients. An open label phase II study was conducted in Taiwanese patients with NSCLC to evaluate its efficacy. METHODS: Patients with proven stage IIIB/IV NSCLC who had received at least one line of standard chemotherapy or radiotherapy were enrolled into this study. All patients were given oral erlotinib, 150mg/day till disease progression. RESULTS: From May 2005 to July 2006, 300 patients were entered from 14 hospitals in Taiwan. This analysis was based on 299 patients who received at least one dose of erlotinib. The best response rates were a 29% partial response and 44% stable disease in 273 patients who had response data available. Non-smoking (p=0.033), adenocarcinoma/BAC (p=0.0027), female (p=0.0013), aged less than 65 years (p=0.0115), stage IV (p=0.0492), patients with skin rash (p=0.0216), and a higher grade of skin rash (p=0.003) were significantly correlated with response to treatment. Skin rash was a common adverse event (any grade: 84%, Gr 3-4: 16%). The median time to disease progression was 5.6 months. Cox regression model for progression free survival showed patients most at risk of early progression were males of low performance status having squamous cell carcinoma. CONCLUSIONS: This was the largest multicenter prospective clinical study of NSCLC in Taiwan. The results demonstrated the excellent response rates, time-to-progression and overall survival of erlotinib in a large population of Taiwanese NSCLC patients who had been previously treated with chemotherapy or radiotherapy.

Oxidative stress and erythropoietin response in altitude exposure.

PURPOSE: Oxidative stress and erythropoietin (EPO) levels are increased following high altitude exposure. We hypothesized that the altitude-oxidative stress and EPO response would be associated with the presence or absence of acute mountain sickness (AMS) in subjects exposed at high altitude. METHODS: The study enrolled 29 healthy volunteers exposed at altitudes without strenuous physical exercise. Oxidative stress was determined by the spectrophotometric measurement of the colour occurring during the reaction of malondialdehyde (MDA) with thiobarbituric acid (TBA) on blood samples. Ferritin and EPO were also measured simultaneously. RESULTS: During a rise in altitude at 2000 and 3000 m, there were no changes in plasma ferritin level in either of the 2 groups with or without AMS. In contrast, EPO increased at an altitude of 3000 m and after returning to sea level (28.2+/-2.7, 26.9+/-3.3 vs 12.2+/-1.4 and 17.1+/-1.6, P < 0.05, in group without AMS; 29.3+/-4.5, 22.8+/-2.7 vs 10.6+/-1.0 and 16.1+/-1.5, # P < 0.05, in group with AMS; compared with the baseline level and at the height of 2000 meters). At a height of 3000 m, plasma MDA level was elevated compared with that at the altitude of baseline and 2000 m in both groups of subjects with and without AMS (3.77+/-0.29 vs 1.14+/-0.17, and 1.64+/-0.22, P < 0.001, in subjects with AMS; 3.65+/-0.39 vs 1.71+/-0.21, and 1.73+/-0.21, P < 0.001, in subjects without AMS) . After returning to sea level, subjects without AMS had lower MDA oxidative stress compared with those with AMS (2.58+/-0.26 vs 3.51+/-0.24, P = 0.0223). Along with a rise in altitude, the oxidative stress in these both groups was not correlated with the changes in EPO (r2 = 0.0728, P = 0.1096). CONCLUSION: High altitude-induced oxidative stress, detected by MDA assay, is not different between the two groups of subjects with and without AMS. Upon return to sea level, subjects without AMS had lower MDA oxidative stress burden and higher EPO level than those with AMS. Whether the subjects with altitude illness had delayed recovery from oxidative stress merits further investigation.

Characteristics comparisons of bacteremia in allogeneic and autologous hematopoietic stem cell-transplant recipients with levofloxacin prophylaxis and influence on resistant bacteria emergence.

BACKGROUND: The aim of this study was to compare the risk factors and clinical outcomes of bacteremia in allogeneic and autologous hematopoietic stem cell transplant (allo-HSCT and auto-HSCT) recipients with levofloxacin prophylaxis during the early period after transplantation. METHODS: Characteristics of bacteremia within 45 days after transplantation between allo-HSCT and auto-HSCT recipients who received levofloxacin prophylaxis between January 2005 and December 2014 were retrospectively reviewed. RESULTS: Of 105 HSCT recipients included in this study, 55 (52.4%) received an allo-HSCT and 50 (47.6%) received an auto-HSCT. Twenty-five patients (23.8%) with HSCT developed 28 episodes of bacteremia. Of these 25 bacteremia patients, 15 received an allo-HSCT, while 10 received an auto-HSCT. The occurrence of Grade 3-4 graft-versus-host disease and longer engraftment duration were associated with bacteremia in allo- and auto-HSCT recipients (p = 0.001 and p = 0.002, respectively). Auto-HSCT recipients with bacteremia had a longer hospital stay after transplantation, while allo-HSCT recipients with bacteremia had an increased 45-day mortality rate as compared with those without bacteremia (p = 0.014 and p = 0.013, respectively). All 14 Gram-negative blood isolates in this study were resistant to fluoroquinolone. CONCLUSION: Levofloxacin prophylaxis in HSCT recipients is associated with the emergence of fluoroquinolone-resistant Gram-negative bacteria. The risk factors and clinical outcomes of bacteremia differ between allo- and auto-HSCT recipients, and these differences should be taken into account when designing strategies to prevent bacteremia.

Epithelial-Mesenchymal Transition with Malignant Transformation Leading Multiple Metastasis from Disseminated Peritoneal Leiomyomatosis.

Disseminated peritoneal leiomyomatosis (DPL) is a rare condition that is characterized by the presence of multiple subperitoneal or peritoneal smooth muscle nodules of varying sizes on the omentum and peritoneal surfaces, grossly mimicking disseminated carcinoma. DPL usually develops in premenopausal women with a benign course, and it is often found incidentally during abdominal surgery. Malignant transformation is a rare clinical course of DPL. Only a few studies have focused on DPL transformation into a leiomyosarcoma. Herein, we describe the case of a 61-year-old woman with a history of recurrent leiomyoma of the uterus who presented with intermittent progressive abdominal pain. The imaging study revealed a huge heterogeneous density mass in the pelvic region with pulmonary and hepatic metastases. Exploratory laparotomy and debulking surgery were performed, and showed the coexistence of DPL and leiomyosarcoma. She died approximately one month after the diagnosis because of rapid progression of pleural effusion due to malignancy. This case highlights the clinical features of DPL and its malignant transformation and metastasis so physicians can make an early diagnosis and provide timely management.