Tumor Rupture Research Articles

Surgical and Oncological Outcomes after Neoadjuvant Therapy for Non-Metastatic Gastric GISTs.

Surgical resection with negative margins of non-metastatic gastric GISTs is considered the main therapeutic option in GISTs treatment. Neoadjuvant therapy with imatinib is associated with higher response rates in advanced GISTs. We reported 34 patients with non-metastatic gastric GISTs who underwent partial gastrectomy at the Oncology Center, Mansoura University, Egypt, after receiving a daily dose of 400mg of imatinib as a neoadjuvant treatment in the period between October 2012 and January 2021. Twenty-two cases underwent open partial gastrectomy, and twelve cases had a laparoscopic partial gastrectomy. The median tumor size at diagnosis was 13.5cm (range 9-26cm) and the duration of neoadjuvant therapy was 10.91months (range 4-12months). Thirty-three patients had a partial response, while one patient showed progression of the disease on neoadjuvant treatment. Adjuvant therapy was conducted in 29 (85.3%) cases. Complications of neoadjuvant treatment were reported in seven cases in the form of gastritis, bleeding per rectum, fatigue, thrombocytopenia, neutropenia, and edema lower limbs. The disease-free survival (DFS) in this study was 34.53months, and the overall survival (OS) was 37months. Recurrence developed in two cases, gastric and peritoneal recurrence (25 and 48months from the initial diagnosis, respectively). We haveconcluded thatneoadjuvant treatment with imatinib for non-metastatic gastric GISTs is a safe and effective method for tumor downsizing and devitalization to allow minimally invasive and/or organ sparing surgery. Moreover, it decreases the risk of intraoperative tumor rupture and relapse, thus improving the oncological outcome of such tumors.

Elevated preoperative controlling nutritional status (CONUT) scores as a predictor of postoperative recurrence in gastrointestinal stromal tumors.

The controlling nutritional status (CONUT) score is associated with the postoperative outcomes in various types of tumors, and its prognostic role in gastrointestinal stromal tumors (GISTs) needs to be clarified. Patients with completely resected primary GISTs in the absence of imatinib adjuvant therapy were included. Recurrence-free survival(RFS) was estimated with the Kaplan-Meier method and compared using log-rank test. Prognostic factors were compared using Cox proportional hazards model. A total of 455 patients were included. The median follow-up time was 132.0 months (range: 7.0-253.0). Recurrence/metastasis developed in 92 (20.2%) patients. Patients were assigned to three groups: 219 (48.1%) were in normal nutrition group (CONUT = 0-1), 196 (43.1%) were in mild malnutrition group (CONUT = 2-4) and 40 (8.8%) were in moderate-severe malnutrition group (CONUT ≥ 5). Nongastric primary tumor site, large tumor size, high mitotic index, tumor rupture and high CONUT score were independent prognostic factors for shorter RFS using multivariate analysis (p < 0.05). Elevated preoperative CONUT score was a predictor of recurrence for patients with resected GIST. The clinical application of the CONUT score is simple and feasible, and might contribute to the individualized treatment of GIST patients.

Large sized gastrointestinal stromal tumors

Background: large gastrointestinal stromal tumors (GISTs) represent a surgical challenge for complete en block resection, which is the mainstay strategy for management. This study was done to determine long term oncological outcomes following resection of GISTs &gt;10 cm. Material and methods: This retrospective study included 52 patients who underwent surgical resection for GIST &gt;10 cm. Our main outcome is to determine the overall survival along with the predictors of post-operative recurrence after large GISTs resection. Results: The stomach was the most commonly affected organ (55.8%). Mass size was larger than 20 cm in 36.5% of our patients and the rest of them ranged between 10 – 20 cm. Anatomical/formal resection was performed in 63.5%of patients, and the remaining ones received a limited resection. After follow-up (median 57 months), disease-free and overall survival had mean values of 80.97 and 100.56 months, respectively. Recurrence was encountered in 36.5%, while the total mortality rate was 26.9%. Multivariate analysis revealed that tumor rupture and CD 34 positive tumors were strong predictors of recurrence. Conclusion: surgical resection of GISTs is the mainstay therapy, but it is associated with relatively high rate of recurrence even with adjuvant tyrosine kinase inhibitor therapy (TKIs).

Risk Factors and Prognostic Analysis of Gastrointestinal Stromal Tumor Recurrence-Metastasis.

Objective Gastrointestinal stromal tumors (GISTs) are potential malignancies that occur in the digestive tract. This study aimed to investigate the risk factors and prognosis of recurrence and metastasis of gastrointestinal stromal tumor (GIST). Methods From January 2018 to December 2019, 422 patients with GIST who received surgery in the First Affiliated Hospital of Wenzhou Medical University were enrolled. Their clinical data were retrospectively analyzed, and their follow-ups were continued until March 31, 2022. Subsequently, univariate and multivariate Cox analyses, survival curves, and nomograms were adopted to explore the relationship between clinicopathological characteristics and recurrence or metastasis in patients with GIST. Results Univariate and multivariate Cox analysis exhibited that the prognosis of patients was affected by tumor rupture (P = 0.040), tumor location (P < 0.001), tumor diameter (P = 0.016), mitotic figures (P < 0.001), and risk grade (P < 0.009). The above variables were selected to create the nomogram for 3-year disease-free survival (DFS). The 3-year the ROC (receiver operating characteristic) curves of the nomogram were (0.878 95% confidence interval [CI]: 0.871–0.939). Conclusion Collectively, risk factors affecting postoperative recurrence or metastasis of GIST consist of primary site of tumors, tumor rupture, tumor diameter >10 cm, high-risk tumor classification, and mitotic figures ≥10 per 50 HPFs. And the application of nomogram may help physicians provide individualized diagnosis and treatment for patients with GISTs following surgical resection.

Application of modified Gibson combined with modified ilioinguinal approach in treatment of Enneking Ⅱ+ Ⅲ pelvic malignant tumors with three-dimensional printed hemipelvic prosthesis replacement

To explore the surgical skills of modified Gibson combined with modified ilioinguinal (MGMII) approach in the treatment of Enneking Ⅱ+Ⅲ pelvic malignant tumors in the three-dimensional (3D) printed customized integrated hemipelvic prosthesis, and to evaluate the convenience and accuracy of the surgical approach and the short-term effectiveness. Between January 2017 and March 2019, 7 patients with Enneking Ⅱ+Ⅲ pelvic malignant tumors were treated with tumor resection and 3D printed hemipelvic prosthesis replacement via MGMII approach. There were 6 males and 1 female. The age ranged from 23 to 68 years, with an average of 43.7 years. There was 1 chondrosarcoma, 1 Ewing's sarcoma, 1 osteosarcoma, 1 malignant Schwannoma, 2 metastatic renal clear cell carcinoma, and 1 metastatic hepatocellular carcinoma. The Enneking stage of 4 cases of primary malignant tumor was stage ⅡB. The disease duration was 6-12 months, with an average of 9.5 months. The preoperative Harris hip score (HHS) was 82.1±1.4 and the Musculoskeletal Tumor Society (MSTS) score was 21.4±1.1. The tumor size by imaging examination was 5.1-9.1 cm, with an average of 6.9 cm. The operation time, intraoperative blood loss, postoperative blood transfusion volume, and postoperative complications were recorded. Postoperative pathological examination confirmed tumor residue according to R classification criteria. The lower limb length, acetabular height, acetabular eccentricity, abduction angle, and anteversion angle were measured and the bone integration was observed by imaging review. Bilateral abductor muscle strengths were measured, and joint function was evaluated by MSTS score and HHS score. All operations were successfully completed. The operation time was 210-360 minutes (mean, 280.0 minutes); the intraoperative blood loss was 1 300-2 500 mL (mean, 1 785.7 mL); the postoperative blood transfusion volume was 0-11 U (mean, 6.1 U). Postoperative pathological examination confirmed R0 resection assisted by osteotomy guide plate. All incisions healed by first intention. All patients were followed up 30-48 months (mean, 41.3 months). At last follow-up, the imaging review showed the good osseointegration in all 7 cases. There was no significant difference in the lower limb length, acetabular height, acetabular eccentricity, abduction angle, and anteversion angle between the affected side and the healthy side ( P>0.05), all of which met the requirements of anatomical reconstruction. At 3 months after operation, the ratios of muscle strength between the affected side and the healthy side was 68.29%±7.41% at 3 months and 89.86%±2.79% at 12 months, showing a significant difference between the two time points ( t=8.242, P=0.000). At last follow-up, the MSTS score and HHS score were 27.3±0.8 and 96.6±1.4, respectively, which significantly improved when compared with those before operation ( P<0.05). None of the patients had assisted walking at last follow-up. There was no recurrence, death, or complications such as deep infection, dislocation of the prosthesis, or fracture of the prosthesis or screw. MGMII approach can expose the posterior column of the acetabulum, especially the ischial tubercle, which is helpful to avoid tumor rupture during tumor resection and preserve the muscle functions such as gluteus medius and iliac muscle while ensuring the resection scope.

Case Report: Rare Acute Abdomen: Focal Nodular Hyperplasia With Spontaneous Rupture

Focal nodular hyperplasia (FNH) of the liver is a benign lesion characterized by hypertrophic nodules with central star-shaped fibrous scars. The etiology and pathogenesis of FNH are not completely understood. A 43-year-old man was hospitalized because of acute abdominal pain. Emergency computed tomography(CT) showed hepatic tumor rupture and bleeding. The patient’s condition improved following arteriographic embolization to stop bleeding. Laparotomy confirmed spontaneous rupture and hemorrhage of focal hyperplasia and the patient remains asymptomatic after an uneventful recovery. FNH with spontaneous rupture and bleeding is extremely rare. Currently, there is no unified management standard for FNH and most previous studies recommend observation and follow-up. We recommend consideration of surgical treatment of cases with spontaneous rupture and bleeding.

Combined and Modified Gibson and Ilioinguinal Approaches in Type II + III Internal Hemipelvectomy for Periacetabular Tumors.

BackgroundThe routine iliofemoral approach and its modifications in type II+III resection require extensive skin incision and massive periacetabular muscle detachment, leading to prolonged hospital stay, increased complication incidence, and impaired lower limb function. Under the management of an enhanced recovery after surgery (ERAS) protocol, a combined and modified Gibson and ilioinguinal (MGMII) approach was used to avoid unnecessary soft tissue trauma during tumor resection and therefore advantageous to patients’ return to normal life.MethodsTwenty-five patients with type II + III (including type II) periacetabular tumors who underwent reconstruction with 3D printed customized endoprostheses at our center between January 2017 and March 2019 were included in this study. There were 13 cases using MGMII approach and 12 cases using iliofemoral approach. The operation duration and blood loss were assessed by chart review. The surgical margin was evaluated by the histopathological studies. The reconstruction accuracy, the abductor muscle strength, the 1993 version of the Musculoskeletal Tumor Society (MSTS-93), the Harris Hip scores (HHS), and the limp score were evaluated. Complications were recorded after reviewing the patients’ records.ResultsThe operative duration and blood loss in MGMII group were shorter than those in the iliofemoral group, but the postoperative hemoglobin was slightly higher than that in the iliofemoral group. The MGMII group had stronger postoperative hip abductors, better functional restoration, and relatively fewer patients with higher limp scores. No complication was observed in the MGMII group. In the iliofemoral group, three patients encountered wound healing delay, and one patient suffered deep infection.ConclusionsThe MGMII approach can better expose the posterior column of the acetabulum, especially the ischial tuberosity, which is beneficial for avoiding tumor rupture during resection. The MGMII approach also helps to preserve residual muscle function, such as the origin of the gluteus medius, while ensuring the extent of resection.

Clinical outcomes and prognostic factors for patients with high-risk gastrointestinal stromal tumors treated with 3-year adjuvant imatinib.

Three years of adjuvant imatinib is the current standard for patients with high-risk gastrointestinal stromal tumors (GIST). We aimed to investigate the safety and efficacy profiles of 3-year imatinib, focusing on the prognostic value of various factors. In this registry-based study, 222 patients with high-risk GIST who underwent surgical resection followed by 3 years of adjuvant imatinib between 2010 and 2018 were included. The imatinib dose was reduced in 39 (17.6%), and 13 (5.9%) discontinued imatinib due to toxicity. With a median follow-up duration of 65.7months, 5-year recurrence-free survival (RFS) and overall survival (OS) were 73.2% and 93.9%, respectively. Tumor rupture, tumor size of >10cm, mitotic index of >10/50 high power fields (HPF) were independent factors for short RFS. Patient subgroups stratified by the risk factors showed distinct RFS (P < .001): patients without the above risk factors or those with only a tumor size of >10cm showed favorable RFS (5-year RFS 83.8% and 92.3%, respectively), whereas those with tumor rupture or those with tumor size of >10cm and mitotic index of >10/50 HPF showed prominently poor RFS (5-year RFS of 54.8% and 47.9%, respectively). Three years of adjuvant imatinib treatment was generally tolerable and effective, which were consistent with the clinical outcomes of previous reports. The presence of tumor rupture, large tumor and high mitotic count was independently associated with poor RFS. Based on these risk factors, different management strategies, such as different durations of adjuvant imatinib, deserve further investigation.

Appendiceal tumors in patients undergoing primary surgery for mucinous ovarian tumors in a tertiary hospital, in Southern Thailand

ObjectiveTo evaluate the prevalence of appendiceal tumors in patients diagnosed with mucinous ovarian tumors and to determine factors associated with coexisting appendiceal tumors. Materials and methodsRetrospective review of all patients who were diagnosed with mucinous ovarian tumors and underwent an appendectomy during surgery between January 2002 and June 2017 was performed. Univariate and multivariate logistic regression analyses were used to identify risk factors for coexisting appendiceal tumors. ResultsA total of 303 patients with mucinous ovarian tumors who underwent appendectomy were identified, including 77 (25.4%) mucinous cystadenoma and 226 (74.6%) mucinous borderline tumor or carcinoma. Twenty-one (6.9%) had coexisting appendiceal tumors including 8 that were primary appendiceal mucinous adenocarcinomas, 6 low-grade appendiceal mucinous neoplasms, 6 secondary appendiceal metastasis from the ovary, and one hyperplastic polyp. None of mucinous cystadenoma had coexisting appendiceal tumors. Multivariate analysis revealed advanced age ≥50 years, previous rupture of ovarian tumors, abdominal extension of tumors, and grossly abnormal appendix were independent factors for coexisting appendiceal tumors. ConclusionPrevalence of coexisting appendiceal tumors in mucinous ovarian tumors was not uncommon. The risk factors were grossly abnormal appendix, abdominal extension of tumor, previous rupture of ovarian tumors, and advanced age.

The analysis of prognostic factors of primary small intestinal gastrointestinal stromal tumors with R0 resection: A single-center retrospective study.

Objective:We aim to assess factors that affect overall survival in patients with primary small intestinal gastrointestinal stromal tumors (GISTs) who had undergone R0 resection.Method:A retrospective analysis reviewed the data of 82 consecutive confirmed GIST patients at a single medical center in China from January 2012 to June 2020. The survival curve was estimated using the Kaplan–Meier method, and independent prognostic factors were confirmed using the Cox regression model.Results:A total of 82 patients were included in the study: 42 men and 40 women, the mean age was 59 years old (23–83 years old). Tumors were commonly found in the jejunum (46.3%), ileum (20.7%), and duodenum (32.9%). The median tumor size was 6.0 cm (range: 1.0–15.0 cm). The number of mitoses per one 50 high-power field was used to define the mitotic rates. In our present study, 56 patients presented a mitotic rate ≤5 (68.3%) and 26 patients showed a rate >5 (31.7%) at the time of diagnosis. All patients accepted tumor resection without lymph node resection. The positivity rate was 97.6% for CD117, 96.3% for delay of germination 1, 65.9% for CD34, 6.1% for S-100, and 59.8% for smooth muscle actin using immunohistochemistry. Tumor size, tumor rupture, Ki67 index, mitotic index, and postoperative imatinib were independent prognostic factors for small intestinal GISTs.Conclusions:In this study, larger tumor size, high Ki67 index, high mitotic index, the occurrence of tumor rupture, and use of imatinib were independent unfavorable prognostic indicators.

Adrenocortical Tumors and Pheochromocytoma/Paraganglioma Initially Mistaken as Neuroblastoma-Experiences From the GPOH-MET Registry.

In children and adolescents, neuroblastoma (NBL), pheochromocytoma (PCC), and adrenocortical tumors (ACT) can arise from the adrenal gland. It may be difficult to distinguish between these three entities including associated extra-adrenal tumors (paraganglioma, PGL). Precise discrimination, however, is of crucial importance for management. Biopsy in ACT or PCC is potentially harmful and should be avoided whenever possible. We herein report data on 10 children and adolescents with ACT and five with PCC/PGL, previously mistaken as NBL. Two patients with adrenocortical carcinoma died due to disease progression. Two (2/9, missing data in one patient) patients with a final diagnosis of ACT clearly presented with obvious clinical signs and symptoms of steroid hormone excess, while seven patients did not. Blood analyses indicated increased levels of steroid hormones in one additional patient; however, urinary steroid metabolome analysis was not performed in any patient. Two (2/10) patients underwent tumor biopsy, and in two others tumor rupture occurred intraoperatively. In 6/10 patients, ACT diagnosis was only established by a reference pediatric pathology laboratory. Four (4/5) patients with a final diagnosis of PCC/PGL presented with clinical signs and symptoms of catecholamine excess. Urine tests indicated possible catecholamine excess in two patients, while no testing was carried out in three patients. Measurements of plasma metanephrines were not performed in any patient. None of the five patients with PCC/PGL received adrenergic blockers before surgery. In four patients, PCC/PGL diagnosis was established by a local pathologist, and in one patient diagnosis was revised to PGL by a pediatric reference pathologist. Genetic testing, performed in three out of five patients with PCC/PGL, indicated pathogenic variants of PCC/PGL susceptibility genes. The differential diagnosis of adrenal neoplasias and associated extra-adrenal tumors in children and adolescents may be challenging, necessitating interdisciplinary and multidisciplinary efforts. In ambiguous and/or hormonally inactive cases through comprehensive biochemical testing, microscopical complete tumor resection by an experienced surgeon is vital to preventing poor outcome in children and adolescents with ACT and/or PCC/PGL. Finally, specimens need to be assessed by an experienced pediatric pathologist to establish diagnosis.

Laparoscopic En Bloc Pelvic Resection with Rectosigmoid Resection and Anastomosis for Stage IIB Ovarian Cancer: Hudson's Procedure Revisited

To demonstrate a systematic approach to the laparoscopic en bloc pelvic resection with rectosigmoid resection and anastomosis as part of ovarian cancer treatment in a tertiary gynecologic surgery referral center. This video illustrates an en bloc pelvic resection performed par laparoscopy in 10 steps. A 56-year-old patient with an advanced high-grade serous ovarian cancer extending into the rectum was amenable to primary debulking surgery in accordance with the French guidelines [1]. In diagnostic laparoscopy, a bilateral adnexectomy was performed, and the pelvic carcinomatosis was considered primarily resectable. Histopathology of the subsequent en bloc resection was consistent with stage IIB high-grade serous ovarian cancer with an indication for adjuvant chemotherapy. The Hudson's procedure revisited consists of a radical monobloc excision by way of a completely extraperitoneal dissection and total mobilization of the rectum. In this case, owing to rectal invasion, we achieved a laparoscopic radical resection including rectosigmoidectomy and primary anastomosis without the need for a defunctioning stoma [2]. Traditionally, an en bloc pelvic resection with rectosigmoid resection and anastomosis was performed by laparotomy. The feasibility of performing laparoscopic optimal cytoreductive surgery in selected patients with advanced ovarian cancer was recently demonstrated without compromising survival in case of low residual disease. The prognosis depends rather on the resectability than on the operative access. However, the radicality and completeness of the cytoreduction, as well as the potential risk of tumor seeding, remain controversially discussed. Here, we demonstrate the minimally invasive approach following the same operative strategy as in open surgery. In this way, the radicality of the "en bloc resection" entailing avoidance of tumor rupture, less bleeding, and less urethral injury is combined with the benefits of a minimally invasive access. In expert hands, this procedure can be performed laparoscopically for other pelvic malignancies with peritoneal carcinomatosis.

Mature mediastinal teratoma with tumor rupture into airway

A previously healthy 16-year-old girl presented with hemoptysis in the setting of a persistent cough for two months. Imaging identified a large mediastinal mass with cystic and solid components, calcifications and fat suggestive of a germ cell tumor. There was associated right middle lobe consolidation with an air-fluid level concerning for a necrotizing pneumonia, right upper lobe bronchiectasis, hilar lymphadenopathy, and right upper lobe and right pleural-based nodules concerning for disseminated disease. Histologic analysis of a percutaneous biopsy revealed skeletal muscle, fibroadipose tissue, skin, and keratinous debris consistent with a mature germ cell tumor. Rigid and flexible bronchoscopy were performed and identified inspissated, purulent, and fibrinous material in the right upper and middle lobe bronchi. Microbial cultures grew Staphylococcus aureus. After 3 weeks of antibiotics, complete surgical resection was performed. No malignant components were identified. Her post-operative course was uncomplicated, and she remains without evidence of disease 6 months after resection. While mediastinal teratomas are common, rupture is uncommon with fewer than 60 reported cases in the literature. Rupture can be associated with inflammation and/or infection, resulting in diagnostic and treatment challenges.

Liver Resection for Primary Hepatic Angiosarcoma: Bicentric Analysis of a Challenging Entity.

Primary hepatic angiosarcoma (PHA) is a rare malignant tumor of the liver, and data on patient outcome after surgical treatment are scarce. The aim of this study was to evaluate postoperative morbidity and overall survival (OS) of patients who underwent hepatectomy for PHA. This is a bicentric retrospective analysis of all consecutive patients who underwent liver resection in curative intent for PHA between 2012 and 2019 at the University Hospital of Muenster and the University Hospital of Bern. Nine patients (five female, four male) were included from both centers. Median age was 72 years (44–82). Most lesions (77.8%) were larger than 5 cm, and mean size of the biggest lesion was 9.4 ± 4.5 cm. Major hepatectomy was performed in four (44.4%), and radical resection (R0) was achieved in six (66.7%) patients. Postoperative complication rate was 88.8%, including 44.4% higher than 3a in the Clavien–Dindo classification. OS survival rates at 1, 2, and 3 years were 44.4%, 22.2%, and 12.5%, respectively, and median OS was 5 months. OS was significantly better after radical resection (R0: 15 months vs. R1: 0 months, p = 0.04), whereas presentation with tumor rupture at diagnosis was associated with the worst OS (0 months vs. 15 months, p = 0.005). Disease recurrence occurred in three patients (33.3%) between three and seven months after surgery. Radical resection remains the only potentially curative treatment option for PHA. However, postoperative morbidity is high, and the overall prognosis remains poor. Multimodal therapy options and management strategies are urgently needed and could improve the prognosis of patients suffering from PHA in the future.

Surgical resection could provide better outcomes for patients with hepatocellular carcinoma and tumor rupture

We investigated the outcomes of patients with ruptured hepatocellular carcinoma (HCC) and identified the optimal treatment modality for such patients. We retrospectively enrolled 91 patients with treatment-naive HCC and tumor rupture at diagnosis, including 38 patients who underwent surgical resection (SR) alone, 28 patients who were treated with transarterial chemoembolization (TACE) only, 20 patients who had a sequential combination therapy of TACE and SR, and 5 patients who received best supportive care. After a median follow-up of 13.1 months, 54 patients died. The cumulative 5 years overall survival (OS) rates were 55.1% and 0% in the SR group and non-SR group, respectively (p < 0.001). Non-SR therapy was associated with poorer OS according to a multivariate analysis with a hazard ratio of 6.649 (95% confidence interval 3.581–12.344, p < 0.001). Moreover, whether patients received TACE or not did not impact the OS in both the SR group and the non-SR group. In conclusion, for patients with HCC and tumor rupture at the time of diagnosis, SR could lead to better prognoses than non-surgery treatment modalities. Moreover, a sequential combination of TACE and SR had similar clinical outcomes when compared to SR alone.

Haemorrhagic rupture of hepatocellular carcinoma: Management of a case in a resource-constrained hospital in TOGO.

IntroductionHepatocellular carcinoma (HCC) is the major malignant tumour of the liver. Its rupture is a serious and fatal complication. Its diagnosis and management are a major challenge in sub-Saharan Africa. The peri-hepatic packing: the only alternative for the management of hepatic tumour ruptures in resource-constrained hospitals.Case reportWe are reporting the case of a 45 years old man, carrier of viral hepatitis B and poorly followed up, who presented a sudden picture of hemodynamic instability. A laparotomy allowed finding a hepatic tumour rupture. He benefited from a damage control by peri hepatic packing.ConclusionPeri hepatic packing is a major solution in the management of ruptured liver tumour in Africa.

Rapid enlargement of pulmonary benign metastasizing leiomyoma with fluid-containing cystic change: a case report

BackgroundPulmonary benign metastasizing leiomyoma (PBML) is a rare disease that can occur in women with a history of uterine leiomyoma. Despite its benign histological features, like a malignancy, leiomyomas can on rare occasion spread to the lung. Typically, PBML presents with asymptomatic multiple solid lung nodules with slow tumor progression, following hysterectomy. Here, we present an atypical case with rapid enlargement of PBML with fluid-containing cystic change.Case presentationWe experienced a case of a 49-year-old woman with bilateral lung nodules following hysterectomy. Two nodules in the right lung had cystic change with fluid in the tumors. Hormone therapy was initiated after surgical biopsy of the left lung confirmed a diagnosis of PBML. However, the cystic component of right upper lobe lesion enlarged rapidly over the following 7 months, and, considering the risk of malignant transformation or tumor rupture, right upper lobectomy was performed. Pathologically, the fluid-containing tumor was diagnosed as PBML.ConclusionGiven the risk of rapid progression, we should carefully consider the surgical indications of fluid-containing PBML.

Wilms\u2019 tumor in low- and middle-income countries: survey of current practices, challenges, and priorities

PurposeTo identify the current practices and priorities in Wilms’ tumor management for surgeons in low- and middle-income countries (LMICs).MethodsOne hundred thirty-seven pediatric surgeons from 44 countries completed surveys on Wilms’ tumor surgical strategy in LMIC. This survey was distributed through the Global Initiative for Children’s Surgery, Pan-African Pediatric Surgical Association, and Latin American Pediatric Surgical Oncology Group.ResultsNinety-two respondents (67.2%) participated from 19 lower middle-income countries (43.2%). Twenty-one respondents (15.3%) participated from nine lower income countries (20.5%). Nineteen respondents (13.9%) participated from 13 upper middle-income countries (29.5%). Most providers do not obtain biopsy for suspected Wilms’ tumor (79%). Delayed resection after preoperative chemotherapy is the preferred approach (70%), which providers chose due to protocol (45%), to decrease tumor rupture (22%), and to decrease complications (8%). The providers’ goal was to prevent tumor spillage and upstaging (46%) or to prevent bleeding, complication, or other organ resections (21%). Most surgeons believed that upfront resection increased the risk of tumor spillage (72%).ConclusionProviders in LMICs prefer delayed resection after preoperative chemotherapy to reduce the incidence of tumor spillage and upstaging of Wilms’ tumor. An evidence-based guideline tailored to the LMIC context can be developed from these findings.

Osteosarcoma/Ewing Sarcoma

Osteosarcoma/Ewing Sarcoma

Spontaneous rupture of an undifferentiated carcinoma with osteoclast-like giant cells of the pancreas presenting as intra-abdominal bleeding: a case report

BackgroundUndifferentiated carcinoma is a very rare histologic subtype, representing only 0.8% to 5.7% of all pancreatic exocrine neoplasms. Additionally, spontaneous abdominal hemorrhage is a particularly rare, life-threatening cause.Case presentationA 68-year-old man was taken by ambulance to our hospital because of sudden-onset abdominal pain. Contrast-enhanced abdominal computed tomography revealed a huge mass measuring 99 × 70 mm in the pancreatic tail with enhanced rim staining in the peripheral area. Imaging also showed extravasation and fluid collection beside the tumor. Hence, spontaneous rupture of the pancreatic tumor and intra-abdominal bleeding were diagnosed. Emergency laparotomy was performed because of acute abdominal pain with peritoneal signs. With an intraoperative diagnosis of rupture of the pancreatic tumor, distal pancreatectomy was successfully performed. Histologically, hematoxylin and eosin staining showed round to spindle-shaped, highly pleomorphic mononuclear cells and multinucleated giant cells as well as a component of ductal adenocarcinoma. Immunohistochemical staining showed that the tumor cells were negative for AE1/AE3, whereas the non-neoplastic osteoclast-like giant cells were positive for CD68. Taken together, these results led to a diagnosis of undifferentiated carcinoma with osteoclast-like giant cells. The patient’s postoperative course was uneventful.ConclusionWe experienced an extremely rare case of spontaneous rupture of an undifferentiated carcinoma with osteoclast-like giant cells presenting as intra-abdominal bleeding. Obtaining a correct preoperative diagnosis is quite difficult at the first evaluation. Undifferentiated carcinoma should be considered as a differential diagnosis in the case with spontaneous rupture of a pancreatic tumor.