Complete Tumor Resection Research Articles

Significance of fusion status, Oberlin risk factors, local and maintenance treatment in pediatric and adolescent patients with metastatic rhabdomyosarcoma: Data of the European Soft Tissue Sarcoma Registry SoTiSaR.

Outcome of primary metastatic rhabdomyosarcoma (RMS) is poor. Certain risk factors as fusion status, Oberlin score, and local treatment of primary tumor are known to influence prognosis. Patients with metastatic RMS were treated according to Cooperative Weichteilsarkom Studiengruppe (CWS) guidance with chemotherapy (CHT), radiotherapy (RT) excluding total lung irradiation (TLI), complete resection of the primary tumor, and metastasectomy if possible. Kaplan-Meier estimators and Cox proportional hazard models were used to examine event-free survival (EFS) and overall survival (OS) involving also landmark analyses. In the European Soft Tissue Sarcoma Registry SoTiSaR (2009-2018), 211 patients were analyzed. Many patients had fusion-positive alveolar RMS (n=83; 39%). Median age was 9.4years [0.1-19.7years]. Treatment primarily consisted of CHT with CEVAIE (carboplatin, epirubicine, vincristine, actinomycin-D, ifosfamide, etoposide: 86%, other regimens: 14%), RT (71%), resection of primary tumor (37%), metastasectomy (19%), and lymph node sampling/dissection (21%). Maintenance treatment (MT) (oral trofosfamide, idarubicin, etoposide) was added in 74% of patients. Oberlin factors, fusion status, and MT were predictive for EFS and OS. MT with O-TIE was not improving outcome when adjusting for the immortal time bias. Local treatment of the primary tumor and radical irradiation (except TLI) improved EFS, not OS, when adjusting for the Oberlin score. Patients with fusion-negative alveolar RMS (n=9) had an excellent outcome with a 5-year EFS and OS of 100%, compared to patients with embryonal RMS (49%/62%), PAX7- (22%/47%) and PAX3/FOXO1-positive ARMS (10/13%), respectively (p<.001). Prognosis of metastatic RMS primarily depends on fusion status and Oberlin score. Fusion status needs to be considered in future trials to optimize treatment outcome. The role of radical irradiation needs further investigation.

Pediatric retroperitoneal non-organ-originated malignancies: An analysis based on SEER database.

Retroperitoneal non-organ-originated malignancies are rare pediatric tumors with challenging diagnosis and treatment. The present study aimed to analyze the clinicopathological characteristics, treatment, and prognosis of retroperitoneal non-organ-originated malignancies. In the study, we included the pathological diagnosis of pediatric retroperitoneal non-organ-originated malignant tumors between 2000 to 2019 through the updated Surveillance, Epidemiology, and End Results database. We use the Kaplan-Meier survival curve to calculate the overall survival (OS) and cancer-specific survival (CSS). The risk of all-cause death and disease-specific death were analyzed using Cox proportional hazard regression model and Fine-and-Grey competitive hazard model, respectively. In the study, a total of 443 pediatric retroperitoneal non-organ-originated malignancies were included. Of them, only 22.3% of patients had no metastatic disease, 42.9% had distant metastasis and 34.8% had locally advanced diseases. The primary pathological tumor was neuroblastoma followed by germ cell tumor. The overall 10-year OS and CSS were 70.7% and 73.1%, respectively, and the 10-year OS and CSS of metastatic diseases were 54.4% and 56.6%, respectively. Older children, worse tumor stage at diagnosis, incomplete resection, and prolonged time from diagnosis to treatment were significantly associated with worse survival outcomes. Radiotherapy and chemotherapy did not significantly improve the prognosis of patients without complete tumor resection. The study indicated that most pediatric retroperitoneal non-organ-originated malignancies diagnosed with metastatic diseases have plagued treatment. Radiotherapy and chemotherapy are the main treatment methods for children unable to undergo complete surgical treatment. However, these treatments do not reach the same therapeutic effect as complete tumor resection after early diagnosis. Hence, early diagnosis and surgery for complete tumor resection are of utmost importance.

A nitroreductase-responsive nanoprobe with homogeneous composition and high loading for preoperative non-invasive tumor imaging and intraoperative guidance

Tumor microenvironment (TME)-activatable probes have been proven to effectively increase signal-to-background ratios (SBRs) and improve the success rate of complete tumor resection. However, many fluorescence probes have to be loaded into a nanocarrier for tumor targeted delivery, which consequently encounters poor drug loading, heterogeneous composition and non-encapsulated drug aggregates occurred during nanoformulation fabrications. Herein, a nitroreductase (NTR)-activated “OFF-ON” near-infrared fluorescence nanoprobe, named NanoBodipy, was synthesized by the spontaneous self-assembling of NTR-responsive dye-polyethylene glycol (PEG) amphiphilic polymer in water. The NTR-responsive dye acted as the hydrophobic segment in the amphiphilic polymer, yielding a homogeneous composition and a high loading of 12.2 wt% (according to calculation) in the synthesized NanoBodipy. The synthesized NanoBodipy can efficiently accumulate in tumors via the enhanced permeability and retention (EPR) effect, enabling non-invasive tumor-targeted fluorescence imaging and guiding complete tumor resection. Once the synthesized NanoBodipy entered the tumor cells, they dissociated and were activated by overexpressed NTR. With the real-time fluorescence guide of NanoBodipy, complete tumor resection surgery was performed successfully.

FRI371 Serum Androstenedione As An Additive Biomarker In Ectopic Cushing’s Syndrome Evaluation

Abstract Disclosure: S. Zhang: None. J.C. Lo: None. M.G. Jaffe: None. H. Arzumanyan: None. Background: Ectopic Cushing’s syndrome (ECS) results from glucocorticoid excess secondary to ectopic ACTH or CRH producing tumors. Although ECS identification can be challenging when presentation lacks classic Cushing’s features, ectopic ACTH secretion can result in a key constellation of findings that facilitate early clinical recognition. We describe a case of ECS from an ACTH-producing carcinoid tumor of the lung presenting with resistant hypertension, severe hypokalemia, and muscle weakness. In addition to significant hypercortisolism, laboratory findings were notable for high androstenedione levels without concomitant increase in DHEAS and testosterone levels. Case: A 59-year-old female athlete with history of well-controlled hypertension developed difficult to control hypertension, requiring escalation to triple antihypertensive therapy within 3 months. During this time, she had multiple emergency department visits for progressive muscle weakness, hypertension, and persistent hypokalemia despite potassium supplementation. She was hospitalized for further evaluation. Physical examination was notable for bruising, muscle wasting, thin extremities, facial fullness, and disproportionate abdominal adiposity. Workup for Cushing’s syndrome demonstrated ACTH-dependent hypercortisolism and markedly elevated androstenedione 398 ng/dL (normal 20-75 ng/dL) despite normal DHEAS 48 mcg/dL (normal &amp;lt;430 mcg/dL) and total testosterone 11 ng/dL (normal &amp;lt;45 ng/dL) levels. A chest CT identified a 3.8 cm right lower lobe lung mass and transbronchial biopsy confirmed atypical carcinoid tumor. She underwent a right middle and lower lobe bilobectomy with complete tumor resection. Cortisol, androstenedione, and potassium levels rapidly normalized postoperatively, and blood pressure returned to baseline, well-controlled on monotherapy. The patient only required 3 days of hydrocortisone therapy for transient steroid withdrawal symptoms. Conclusions: Our case illustrates a difficult to diagnose uncommon disease presenting with resistant hypertension, hypokalemia and marked androstenedione elevation despite normal DHEAS and testosterone levels. This steroidogenic profile has been reported in other ECS cases and attributed to differential effects of ectopic ACTH production on adrenal androgen biosynthesis (1). Although ECS is rare and the primary lesion can be difficult to identify, disproportionate elevation in androstenedione may prompt recognition of ECS and support early detection and management. Reference: (1) Barbetta L, Dall’Asta C, Re T, Colombo P, Travaglini P, Ambrosi B. Androgen secretion in ectopic ACTH syndrome and in Cushing’s disease: modifications before and after surgery. Horm Metab Res Horm Stoffwechselforschung Horm Metab. 2001;33(10):596-601 Presentation: Friday, June 16, 2023

Mixed reality in primary retroperitoneal tumour surgery: Evaluation of preoperative and intraoperative application value.

To evaluate the feasibility and application value of mixed reality technology (MR) in Primary retroperitoneal tumour (PRT) surgery. From 276 patients who underwent PRT resection at the First Affiliated Hospital of Xi'an Jiaotong University, we screened 46 patients who underwent MR-assisted retroperitoneal tumour resection and 46 patients who underwent tumour resection without MR assistance. The intraoperative and postoperative recovery of the patients in both groups were compared, and the reliability and validity of the application of MR were further examined using the Likert scale. There was a significant difference in the mean intraoperative bleeding volume between the two groups, but it was reduced in the MR group. The resultsof the Likert scale showed higher scores in the MR group than non-MR group. MR can be used to assist PRT resection and has great potential to improve the rate of complete retroperitoneal tumour resection.

Critical elements of pediatrics sacrococcygeal germ cell tumor surgery

Sacrococcygeal teratoma is the most common extragonadal germ cell tumor in the pediatric population, and accounts for approximately 70% of all teratomas in childhood.1,2 They present in two distinct phases, with most cases seen in neonates with large predominately exophytic tumors, often detected in utero on prenatal sonography or at birth. A smaller cohort presents in older infants and children with primarily hidden tumors in the pelvis which have a much higher rate of malignancy. The primary surgical objective is complete tumor resection without compromise to critical structures or function. Herein we outline the critical elements of tumor resection and management of sacrococcygeal germ cell tumors with a focus on the technical aspects of this tumor across a range of presentations.

Anatomy-Specific Classification Model Using Label-Free FLIm to Aid Intraoperative Surgical Guidance of Head and Neck Cancer.

Intraoperative identification of head and neck cancer tissue is essential to achieve complete tumor resection and mitigate tumor recurrence. Mesoscopic fluorescence lifetime imaging (FLIm) of intrinsic tissue fluorophores emission has demonstrated the potential to demarcate the extent of the tumor in patients undergoing surgical procedures of the oral cavity and the oropharynx. Here, we report FLIm-based classification methods using standard machine learning models that account for the diverse anatomical and biochemical composition across the head and neck anatomy to improve tumor region identification. Three anatomy-specific binary classification models were developed (i.e., "base of tongue," "palatine tonsil," and "oral tongue"). FLIm data from patients (N = 85) undergoing upper aerodigestive oncologic surgery were used to train and validate the classification models using a leave-one-patient-out cross-validation method. These models were evaluated for two classification tasks: (1) to discriminate between healthy and cancer tissue, and (2) to apply the binary classification model trained on healthy and cancer to discriminate dysplasia through transfer learning. This approach achieved superior classification performance compared to models that are anatomy-agnostic; specifically, a ROC-AUC of 0.94 was for the first task and 0.92 for the second. Furthermore, the model demonstrated detection of dysplasia, highlighting the generalization of the FLIm-based classifier. Current findings demonstrate that a classifier that accounts for tumor location can improve the ability to accurately identify surgical margins and underscore FLIm's potential as a tool for surgical guidance in head and neck cancer patients, including those subjects of robotic surgery.

Pediatric Spinal Giant Cell-rich Osteosarcoma: Case Report and Brief Literature Review.

Osteosarcoma, the most common primary malignant bone tumor in childhood, very rarely occurs in the spine. Criteria of complete tumor resection/stable reconstruction of osteosarcoma and the latest protocol of neoadjuvant chemotherapy of the spine have not been reported because of its rarity, technical difficulties, and its continued severe surgical risk. A 11-year-old female complained of back pain for several months and walking disability. The workup discovered a large destructive bone lesion in the thoracic 12th (Th12) with vertebral body collapse and subluxation, large amount of associated anteroposterior soft tissue components, and narrowing of the spinal canal. Histology at the 1st decompression and emergent instrumentation surgery revealed giant cell-rich osteosarcoma. Following the 1st surgery, we performed three cycles of neoadjuvant chemotherapy based on the osteosarcoma 95J (NECO95J) protocol and evaluated efficacy of chemotherapy on the Th12 tumor. The tumor was isolated only to Th12 spine following chemotherapy. Therefore, following vascular embolization of the Th12 tumor, we performed surgical resection by single posterior approach that included total en bloc spondylectomy (TES). She recovered well postoperatively, without motor or sensory deficit and no back pain. Six cycles of postoperative neoadjuvant chemotherapy were administered after the 2nd surgery and TES. The patient was disease-free at the 8-months clinical and radiological follow-up and showed no neurological involvement at 8-months. We reported a case of pediatric spinal osteosarcoma, the surgical technique of complete tumor resection, and stable reconstruction of spinal osteosarcoma. We also discussed the recent neoadjuvant chemotherapy protocol for osteosarcoma.

Ganglioneuromas in Childhood: Hacettepe Experience With 70 Cases

BackgroundGanglioneuromas (GNs) are rare benign peripheral neuroblastic tumors (PNTs). We shared our institutional experience with childhood GNs. MethodsRecords of the children with PNTs between January 1995 and December 2021 were reviewed, and cases with histopathological diagnoses of GN were identified. Clinical, laboratory, radiological, and histopathological findings, image-defined risk factors (IDRFs), procedures, and overall outcomes were recorded. ResultsOf 668 cases with PNTs, 70 (10.4%) had GNs. The median age was 7.4 years (range, 2.6–15.7 years) (girls/boys, 41/29). Common presenting complaints were abdominal pain and cough; 33/70 cases (47.1%) were diagnosed incidentally. Primary tumors were in the abdomen in 41/70, the thorax in 25/70, the neck in 3 cases, and the pelvis in one. The median tumor size was 6.5 cm (range, 1.4–17). Fifty cases (71.4%) were staged as INRG-L1; 20 cases with IDRFs (15 single, five >1) were staged as INRG-L2. Complete and partial tumor resections were performed in 58/70 and 6/70 cases, while 6 had no resection. The overall complication rate was 17.1% (11/64). At a median follow-up of 9 years, five were lost to follow-up; 65 were alive. One patient with gross residue underwent total resection due to progression 13 years after the surgery, and one in the unresected group was lost to follow-up. Ten other cases without a complete resection experienced no tumor progression. ConclusionsGanglioneuromas are benign PNTs, and most are free of IDRFs. Even without complete resection, long-term outcomes are excellent. Guidelines should be devised considering the high surgical complication rates and benign course of GNs. Level of EvidenceCase series, IV.

Deep learning-enabled realistic virtual histology with ultraviolet photoacoustic remote sensing microscopy

The goal of oncologic surgeries is complete tumor resection, yet positive margins are frequently found postoperatively using gold standard H&E-stained histology methods. Frozen section analysis is sometimes performed for rapid intraoperative margin evaluation, albeit with known inaccuracies. Here, we introduce a label-free histological imaging method based on an ultraviolet photoacoustic remote sensing and scattering microscope, combined with unsupervised deep learning using a cycle-consistent generative adversarial network for realistic virtual staining. Unstained tissues are scanned at rates of up to 7 mins/cm2, at resolution equivalent to 400x digital histopathology. Quantitative validation suggests strong concordance with conventional histology in benign and malignant prostate and breast tissues. In diagnostic utility studies we demonstrate a mean sensitivity and specificity of 0.96 and 0.91 in breast specimens, and respectively 0.87 and 0.94 in prostate specimens. We also find virtual stain quality is preferred (P = 0.03) compared to frozen section analysis in a blinded survey of pathologists.

Dural reconstruction following resection of ventral and lateral spinal cord meningiomas: Fenestrated Durotomy with Oversized Graft technique

BackgroundMeningiomas, although benign, often require complete resection due to their tendency for recurrence. However, dural reconstruction poses significant challenges, especially in the case of ventral meningiomas. While some reports have highlighted the usefulness of dural reconstruction using an artificial dura mater, no studies have yet confirmed dural canal enlargement through MRI post-surgically. This study aimed to assess the effectiveness of the Fenestrated Durotomy with Oversized Graft (FDOG) technique in cases of meningiomas and other intradural extramedullary tumors and evaluated dural canal dilation through MRI after using an artificial dura mater. MethodsThis retrospective case series included 8 patients who underwent combined resection of intradural extramedullary tumors and dural repair using the FDOG technique. An artificial dura mater larger than that resected was inserted into the dural defect resulting from complete tumor resection on the ventral or lateral side of the spinal cord. The new dura mater was secured with a single dorsal suture. The dural incision was closed using watertight sutures following standard procedure. Measurement of the extent of dural canal enlargement was achieved via pre- and postoperative MRI scans. ResultsNone of the patients required additional treatments or lumbar drainage. All achieved independent ambulation without complications, and imaging tests indicated satisfactory dural expansion without signs of cerebrospinal fluid leakage. ConclusionsThe proposed method for dural repair in cases involving large dural defects on the ventral or lateral side of the spinal cord was shown to be a straightforward and effective approach with minimal postoperative complications.

P15.05.B THE IMPACT OF IOMRI ON GLIOBLASTOMA RESECTION AND CLINICAL OUTCOMES IN A STATE-OF-THE-ART NEURO-ONCOLOGICAL SETUP

Abstract BACKGROUND Intraoperative magnetic resonance imaging (ioMRI) is applied to improve the extent of resection in patients suffering from primary brain tumors, especially glioblastoma (GBM). While some older randomized trials reported data on safety and feasibility, ioMRI’s actual impact in a modern neurosurgical setting has not been sufficiently investigated. We therefore aimed to evaluate the effect of ioMRI on tumor resection and progression-free survival (PFS) in GBM patients in a modern high-volume center. MATERIAL AND METHODS Patients undergoing ioMRI for resection of supratentorial GBM were prospectively included between March 2018 and June 2020. In all cases, ioMRI was performed after complete macroscopic tumor resection. The extent of resection (EOR) was determined by volumetric analysis. Gross total resection (GTR) was defined as EOR ≥95%. Furthermore, progression-free survival (PFS) was analyzed through univariate and multivariate Cox proportional regression analysis. RESULTS Overall, 172 patients were enrolled in this study. The mean EOR increased from 93·9% on ioMRI to 98·3% (p&amp;lt;0·0001) on postoperative MRI, with residual tumor volume decreasing from 1·3 ± 4·2 cm3 to 0·6 ± 2·5 cm3(p=0·0037). The GTR rate increased from 78·5% to 93·0% (p=0·0002). Logistic regression revealed recurrent GBM (odds ratio [OR] =3·047, 95% confidence interval [CI] 1·165-7·974, p=0·023) and tumor volume less than 15 cm3 (OR=3·031, 95% CI 1·062-8·651, p=0·038) as independent risk factors leading to STR. Tumor resection performed additionally after ioMRI lead to equally long PFS compared to patients with complete tumor removal achieved before ioMRI (HR=0·898, 95%CI 0·543-1·483, p=0·67). CONCLUSION ioMRI serves as a valuable tool in a modern neurosurgical setting, severely decreasing residual tumor volume. ioMRI considerably helps to achieve comparable PFS even in cases, where an unexpected tumor residual is found on ioMRI after initial resection.

Advancements in Intraoperative Near-Infrared Fluorescence Imaging for Accurate Tumor Resection: A Promising Technique for Improved Surgical Outcomes and Patient Survival.

Clear surgical margins for solid tumor resection are essential for preventing cancer recurrence and improving overall patient survival. Complete resection of tumors is often limited by a surgeon's ability to accurately locate malignant tissues and differentiate them from healthy tissue. Therefore, techniques or imaging modalities are required that would ease the identification and resection of tumors by real-time intraoperative visualization of tumors. Although conventional imaging techniques such as positron emission tomography (PET), computed tomography (CT), magnetic resonance imaging (MRI), or radiography play an essential role in preoperative diagnostics, these cannot be utilized in intraoperative tumor detection due to their large size, high cost, long imaging time, and lack of cancer specificity. The inception of several imaging techniques has paved the way to intraoperative tumor margin detection with a high degree of sensitivity and specificity. Particularly, molecular imaging using near-infrared fluorescence (NIRF) based nanoprobes provides superior imaging quality due to high signal-to-noise ratio, deep penetration to tissues, and low autofluorescence, enabling accurate tumor resection and improved survival rates. In this review, we discuss the recent developments in imaging technologies, specifically focusing on NIRF nanoprobes that aid in highly specific intraoperative surgeries with real-time recognition of tumor margins.

Cross-species evaluation of fibroblast activation protein alpha as potential imaging target for soft tissue sarcoma: a comparative immunohistochemical study in humans, dogs, and cats.

Complete surgical tumor resection is paramount in the management of soft tissue sarcoma (STS) in humans, dogs, and cats alike. Near-infrared targeted tracers for fluorescence-guided surgery (FGS) could facilitate intraoperative visualization of the tumor and improve resection accuracy. Target identification is complicated in STS due to the rarity and heterogeneity of the disease. This study aims to validate the expression of fibroblast activation protein alpha (FAP) in selected human, canine, and feline STS subtypes to assess the value of FAP as a target for FGS and to validate companion animals as a translational model. Formalin-fixed and paraffin-embedded tissue samples from 53 canine STSs (perivascular wall tumor (PWT), canine fibrosarcoma (cFS), and STS not further specified (NOS)), 24 feline fibrosarcomas, and 39 human STSs (myxofibrosarcoma, undifferentiated pleomorphic sarcoma, dermatofibrosarcoma protuberans, and malignant peripheral nerve sheath tumor) as well as six canine and seven feline healthy controls and 10 inflamed tissue samples were immunohistochemically stained for their FAP expression. FAP labeling in tumor, peritumoral, healthy skin, and inflamed tissue samples was quantified using a visually assessed semiquantitative expression score and digital image analysis. Target selection criteria (TASC) scoring was subsequently performed as previously described. Eighty-five percent (85%) of human (33/39), 76% of canine (40/53), and 92% of feline (22/24) STSs showed FAP positivity in over 10% of the tumor cells. A high expression was determined in 53% canine (28/53), 67% feline (16/24), and 44% human STSs (17/39). The average FAP-labeled area of canine, feline, and human STSs was 31%, 33%, and 42%, respectively (p > 0.8990). The FAP-positive tumor area was larger in STS compared to healthy and peritumoral tissue samples (p < 0.0001). TASC scores were above 18 for all feline and human STS subtypes and canine PWTs but not for canine STS NOS and cFS. This study represents the first cross-species target evaluation of FAP for STS. Our results demonstrate that FAP expression is increased in various STS subtypes compared to non-cancerous tissues across species, thereby validating dogs and cats as suitable animal models. Based on a TASC score, FAP could be considered a target for FGS.

Seizure outcomes after resection of primary brain tumors in pediatric patients: a systematic review and meta-analysis.

Primary brain neoplasms are the most common solid tumors in pediatric patients and seizures are a common presenting symptom. Surgical intervention improves oncologic outcomes and seizure burden. A better understanding of factors that influence seizure outcomes in the surgical management of primary brain tumors of childhood can guide treatment approach thereby improving patient quality of life. We performed a systematic analysis using articles queried from PubMed, EMBASE, and Cochrane published from January 1990 to August 2022 to determine predictors of seizure outcomes in pediatric patients undergoing resection of primary brain tumors. We identified 24 retrospective cohort studies, one prospective cohort study, and one mixed retrospective and prospective study for the systematic analysis. A total of 831 pediatric patients were available for analysis. 668 (80.4%) patients achieved seizure freedom after surgery. Complete tumor resection increased the likelihood of a seizure-free (Engel I) outcome compared to subtotal resection (OR 7.1, 95% CI 2.3-21.9). Rates of Engel I seizure outcomes did not significantly differ based on factors such as age at seizure onset, duration of epilepsy, gender, tumor laterality, or age at surgery, but trended towards significance for improved outcomes in temporal lobe tumors. Primary brain tumors in the pediatric population are commonly associated with seizures. Resection of these lesions reduces seizure burden and is associated with high rates of seizure freedom. Complete resection, compared to subtotal resection, significantly increases the likelihood of seizure-free outcomes.

PTCH1 and CTNNB1 emerge as pivotal predictors of resistance to neoadjuvant chemotherapy in ER+/HER2- breast cancer.

Endeavors in the molecular characterization of breast cancer opened the doors to endocrine therapies in ER+/HER2- breast cancer, increasing response rates substantially. Despite that, taxane-based neoadjuvant chemotherapy is still a cornerstone for achieving breast-conserving surgery and complete tumor resection in locally advanced cancers with high recurrence risk. Nonetheless, the rate of chemoresistance is high, and deselecting patients who will not benefit from chemotherapy is a significant task to prevent futile toxicities. Several multigene assays are being used to guide decisions on chemotherapy. However, their development as prognostic assays but not predictive assays limits predictive strength, leading to discordant results. Moreover, high costs impediment their use in developing countries. For global health equity, robust predictors that can be cost-effectively incorporated into routine clinical management are essential. In this study, we comprehensively analyzed 5 GEO datasets, 2 validation sets, and The Cancer Genome Atlas breast cancer data to identify predictors of resistance to taxane-based neoadjuvant therapy in ER+/HER2- breast cancer using efficient bioinformatics algorithms. Gene expression and gene set enrichment analysis of 5 GEO datasets revealed the upregulation of 63 genes and the enrichment of CTNNB1-related oncogenic signatures in non-responsive patients. We validated the upregulation and predictive strength of 18 genes associated with resistance in the validation cohort, all exhibiting higher predictive powers for residual disease and higher specificities for ER+/HER2- breast cancers compared to one of the benchmark multi-gene assays. Cox Proportional Hazards Regression in three different treatment arms (neoadjuvant chemotherapy, endocrine therapy, and no systemic treatment) in a second comprehensive validation cohort strengthened the significance of PTCH1 and CTNNB1 as key predictors, with hazard ratios over 1.5, and 1.6 respectively in the univariate and multivariate models. Our results strongly suggest that PTCH1 and CTNNB1 can be used as robust and cost-effective predictors in developing countries to guide decisions on chemotherapy in ER +/HER2- breast cancer patients with a high risk of recurrence. The dual function of PTCH1 as a multidrug efflux pump and a hedgehog receptor, and the active involvement of CTNNB1 in breast cancer strongly indicate that PTCH1 and CTNNB1 can be potential drug targets to overcome chemoresistance in ER +/HER2- breast cancer patients.

A 3D-printed patient-specific modular implants for pelvic reconstruction of bone tumors involving the sacroiliac joint.

Background: Current reconstruction methods of the pelvic ring after extensive resection of tumors involving the sacroiliac joint have a high incidence of failure. We aimed to study the effect of 3D-printed patient-specific implant reconstruction to show that this method is stable and has a low risk of failure. Methods: Between February 2017 and November 2021, six patients with bone tumors involving the sacroiliac joint (Enneking I + IV) who received 3D-printed patient-specific implants for pelvic reconstructive surgery were retrospectively analyzed. Two female and four male patients with a mean age of 41.83years (range 25-65years) were included. Two were osteosarcomas, two chondrosarcomas, one malignant fibrous histiocytoma, and one giant cell tumor of bone. For each patient, preoperative osteotomy guides were designed to ensure accurate tumor resection and individualized prostheses were designed to ensure a perfect fit of the bone defect. General, oncologic, and functional outcomes, implant status, and complications were retrospectively analyzed. The Visual Analog Scale (VAS) was used to assess pain and the Musculoskeletal Tumor Society (MSTS) score was used to assess hip function. Osseointegration was assessed by CT. Results: According to the preoperative design, complete resection of the entire tumor and reconstruction with a custom 3D-printed sacroiliac joint implant was completed without perioperative severe complications or deaths. Relatively satisfactory surgical margins were achieved. The mean operative time and intraoperative blood loss were 495min (420-600min) and 2533.33mL (range, 1,200-3,500mL), respectively. The mean follow-up was 49.83months (range, 18-75months). At the last follow-up, all four patients were disease-free, and the two patients who developed lung metastases were alive with tumors. All patients could walk unassisted. The mean VAS was 1.33 (range, 0-2). The mean MSTS score was 25.33 (range, 24-27). CT showed complete osseointegration of the implant to the ilium and sacrum. Conclusion: The 3D-printed custom prosthesis can effectively reconstruct pelvic stability after total sacroiliac joint resection with satisfactory clinical results.

Hepatic adenoma in a 7-year-old girl: a case report and literature review

BackgroundHepatocellular adenomas (HCAs) are rare benign tumors of the liver that occur predominantly in women taking oral contraceptives. In children, HCAs comprise < 5% of hepatic tumors. We report a case of HCAs in a 7-year-old girl with estrogen and glucose imbalance.Case presentationA 7-year-old girl was presented to our hospital with bilateral breast enlargement for 2 months, polydipsia, polyuria, polyphagia, hyperglycemia, and significant weight gain. Computed tomography (CT) showed a 7.2 cm×6.9 cm×5.3 cm round-shaped mass in the left inner lobe of the liver, ovarian ultrasound showed multiple follicles in the ovaries bilaterally, and cranial magnetic resonance imaging (MRI) showed an enlarged superior pituitary. Hematological and biochemical results were as follows: fasting glucose was 19.7 mmol/L, estradiol was 122.9 pmol/L, follicle-stimulating hormone 10.81 IU/L, luteinizing hormone 10.99 IU/L, insulin-like growth factor 1,513 ng/mL, glutamine aminotransferase 86 U/L, and alkaline phosphatase 362 U/L. Thyroid functions, methemoglobin, fetal protein, carcinoembryonic antigen, and chorionic gonadotropin were normal. The patient had a complete surgical resection of the liver tumor, and the postoperative histopathological diagnosis was HCAs. After the surgery, insulin was injected and the glucose levels were stable. During the 36-month follow-up period, neither tumor recurrence nor significant abnormalities were detected using color Doppler ultrasound of the liver. The child’s precocious puberty is currently under control.ConclusionsHCAs are particularly rare in children with liver tumors, and risk factors for the development of HCAs in children include sex hormone imbalance, obesity, Fanconi anemia (FA), glycogen storage diseases (GSDs) type I, III, and IV, galactosemia, immunodeficiency, congenital portosystemic shunts (CPSS), cardiac hepatopathy status-post Fontan procedure, Hurler syndrome, familial adenomatous polyposis, germline HNF1A mutations, and maturity-onset diabetes of the young type 3. Most HCAs are detected during a physical examination without clinical symptoms, and some patients may present with symptoms such as abdominal pain, abdominal distension, and abdominal masse. Serum liver function tests can show increased alkaline phosphatase (ALP) and γ- glutamyl transferase (GT), whereas α-Fetoprofein (AFP) levels are normal. The definitive diagnosis relies mainly on histopathological examination. Because HCAs can rupture and bleed and become malignant. Early surgical treatment is recommended after detection.

Surgical approach for complete resection of giant retroperitoneal liposarcoma with diaphragmatic hernia via ninth rib thoracotomy.

Resection of a giant retroperitoneal liposarcoma is difficult and technically demanding, especially for large retroperitoneal tumors accompanied by a diaphragmatic hernia. Technically, the open abdominal approach can be time-consuming and difficult to perform, with possible intraoperative complications and other factors bringing psychological and physical difficulties to the patient. This study reports a safe and feasible approach for the complete resection of a large retroperitoneal tumor complicated by a diaphragmatic hernia. A 58-year-old male patient with persistent upper abdominal pain and distension was treated at a local hospital on 4 July 2022. Computed tomography showed a mixed-density mass on the right retroperitoneum, and liposarcoma was considered. On 6 July 2022, the patient was transferred to our hospital for further treatment. Computed tomography showed a mass with low-density fatty shadow in the right adrenal region. The boundary with the right adrenal gland was unclear. The mass was 102mm × 74mm, and the right lobe of the liver was compressed. Insufficiency of the right middle lobe of the liver was seen due to a right diaphragmatic hernia and left mediastinal deviation. We considered the traditional approach for tumor resection via laparotomy, but we opted to perform a comprehensive evaluation first. The tumor was close to the back of the right kidney and liver, causing the diaphragm to rise because of its proximity to these organs. Exposing the tumor through laparotomy would be difficult, making it challenging to remove. The patient had a diaphragmatic hernia and moderate pulmonary dysfunction; therefore, we decided to enter the abdomen through a thoracotomy of the ninth rib. Using our technique, the tumor was easily visualized and completely removed in approximately 30min. The intraoperative blood loss was 100ml, and no postoperative bleeding, pneumothorax, intestinal fistula, infection, or other complications occurred. The transthoracic approach may be a safer and more feasible resection method than the traditional open approach for patients with giant retroperitoneal liposarcoma with a diaphragmatic hernia.

Shaving Papillary Thyroid Carcinoma Involving Functioning Recurrent Laryngeal Nerve: Safety of Incomplete Tumor Resection and Nerve Sparing.

Whether to sacrifice or spare the recurrent laryngeal nerve (RLN) when papillary thyroid carcinoma (PTC) involves a functioning RLN remains controversial. Oncological outcomes after shaving PTC with gross remnant on the RLN have been rarely reported. The objective of this study was to evaluate the oncological outcomes of patients who underwent shaving of a PTC from the RLN, leaving a gross residual tumor with the intent of vocal function preservation. A retrospective, cohort study was conducted in 47 patients who were determined to have PTC invasion of the RLN via intraoperative inspection and underwent tumor shaving with macroscopic remnant (R2 resection) less than 1 cm in length and 4 mm in thickness. Median follow-up period was 93 (range, 60-215) months. The primary endpoint was the recurrence-free survival and the progression-free survival. Secondary endpoints were biochemical outcomes (serum thyroglobulin) and vocal cord function. Of the 47 patients, five (10.6%) patients showed recurrence (central neck, 3; lateral neck, 2) without death or distant metastasis. The RLN was resected along with the tumor in one (2.1%) patient who presented with progression of the residual tumor. Postoperative temporary vocal cord paralysis occurred in six (12.8%) patients without permanent cases. The final nonstimulated serum thyroglobulin was 0.7 ± 1.8 ng/ml. Shaving a tumor from a RLN with gross residual disease may be considered an alternative strategy to preserve vocal function when complete tumor resection with nerve preservation is impossible in patients with PTC invading a functioning RLN.