A case of intraductal papillary neoplasm of the bile duct suspected to be of peribiliary glands origin.

Peribiliary glands are complex lobular structures containing mucus and serous glands, distributed along the extrahepatic and intrahepatic bile ducts. In this report, we describe a case of intraductal papillary neoplasm of the bile duct suspected to be of peribiliary glands origin. The patient was an 80-year-old man who was referred to our hospital for a hepatic mass. On further examination, a 38 × 34 mm cystic lesion with papillary growth was found in S1/4. Because the lesion was extensively bordered by both hepatic ducts and the connection was unclear, it was difficult to determine the extent of hepatic resection. To confirm the location, a peroral cholangioscopy was performed. The connection with the cyst was detected in the right hepatic duct and a villous tumor mucosa protruded through the conduit lumen. Since we found that the lesion communicated with the right hepatic duct, a right hepatectomy was subsequently performed. The postoperative pathological diagnosis was an intraductal papillary neoplasm of the blie duct with associated invasive carcinoma. The postoperative course was good, and the patient experienced no recurrence.

Updated techniques and evidence for endoscopic ultrasound-guided tissue acquisition from solid pancreatic lesions.

Endoscopic ultrasound-guided tissue acquisition (EUS-TA), including fine-needle aspiration (EUS-FNA) and fine-needle biopsy (EUS-FNB), has revolutionized specimen collection from intra-abdominal organs, especially the pancreas. Advances in personalized medicine and more precise treatment have increased demands to collect specimens with higher cell counts, while preserving tissue structure, leading to the development of EUS-FNB needles. EUS-FNB has generally replaced EUS-FNA as the procedure of choice for EUS-TA of pancreatic cancer. Various techniques have been tested for their ability to enhance the diagnostic performance of EUS-TA, including multiple methods of sampling at the time of puncture, on-site specimen evaluation, and specimen processing. In addition, advances in next-generation sequencing have made comprehensive genomic profiling of EUS-TA samples feasible in routine clinical practice. The present review describes updates in EUS-TA sampling techniques of pancreatic lesions, as well as methods for their evaluation.

Rare case of mantle cell lymphoma of the lacrimal sac

ABSTRACT Mantle cell lymphoma of the ocular and periorbital regions is extremely rare but should be considered in the differential diagnosis of lesions affecting the periorbital tissues. In this study, we present a rare case of mantle cell lymphoma of the lacrimal sac in a 65-year-old male presenting with a mass in the lacrimal sac region and epiphora. After clinical examinations and imaging studies, the mucocele was misdiagnosed. Considering the unexpected findings during external dacryocystorhinostomy, a frozen biopsy was performed, which confirmed the diagnosis of lymphoma.

Successful embolization with subsequent pancreaticoduodenectomy for intraductal papillary mucinous neoplasm hemorrhage: a case report and review of literature.

Although various complications associated with intraductal papillary mucinous neoplasms have been reported, including acute pancreatitis, duct perforation, and fistula formation, spontaneous bleeding, especially life-threatening bleeding, is infrequent. In this case, emergency pancreatic resection might be one of the therapeutic options, which is associated with poor postoperative outcomes. An 87-year-old woman presented to our hospital with severe anemia (hemoglobin, 4.5 g/dl). Contrast-enhanced computed tomography revealed a large cystic lesion in the pancreatic head measuring 15 cm, with some solid components and an adjacent hematoma, suggestive of intra-cystic hemorrhage of the intraductal papillary mucinous neoplasm. The patient was hemodynamically unstable and had hypotension. After transcatheter arterial embolization, the patient became hemodynamically stable. Subsequently, an elective pylorus-preserving pancreaticoduodenectomy was successfully performed. Preoperative embolization was effective for subsequent elective pancreaticoduodenectomy in patients with severe intraductal papillary mucinous neoplasm bleeding.

Case Series of Endoscopic Transoral Excision of Huge Parapharyngeal Tumors: Pushing the Boundaries.

Endoscopic transoral excision of parapharyngeal space (PPS) tumors is often condemned for its many limitations. We revisit this approach and aim to introduce some updated perspectives following the advancement of endoscopic instrumentation, robotic transoral surgery, and radical tonsillectomy. We illustrate the techniques that were deployed for six patients with huge benign PPS tumors originating from the pre-styloid compartment at our center. Only patients who had a tumor size of five cm or larger were included in this study. We summarized our patients' outcomes, the important considerations, and the advantages and disadvantages of this approach. With accurate selection and surgical strategy, all our patients had good clinical outcomes. We demonstrated that even larger tumors can be safely removed endoscopically. Surely, this approach will gain traction, and better-designed studies should be conducted in the future to assess its credibility.

Cytologic diagnosis of papillary renal neoplasm with reverse polarity.

BACKGROUND: Papillary renal neoplasm with reverse polarity is a recently recognized low-grade neoplasm with a favorable prognosis. To date, its cytologic features have not been well documented. METHODS: Two patients with papillary renal neoplasm with reverse polarity sampled by fine needle aspiration and core needle biopsy are described, one of whom is under active surveillance without clinical progression and the other is alive and well 16 years after partial nephrectomy. RESULTS: The cytologic features included a mix of papillae and dispersed cells with abundant oncocytic cytoplasm and round, bland nuclei apically displaced away from the papillary core. Immunohistochemistry showed positive staining for GATA3 in both cases. Molecular studies on one of the cases showed a KRAS p.G12V mutation. CONCLUSIONS: The cytologic features of this distinctive, indolent neoplasm are important to recognize because patients with papillary renal neoplasm with reverse polarity may be excellent candidates for partial nephrectomy or even active surveillance.

Tailoring rectal cancer surgery: Surgical approaches and anatomical insights during deep pelvic dissection for optimal outcomes in low-lying rectal cancer.

This review article explores advanced surgical approaches and anatomical insights for tailoring rectal cancer surgery, particularly focusing on low-lying rectal cancer. With the evolution of imaging technologies, precise preoperative planning has become possible, enhancing the visualization of anatomy surrounding the rectum and enabling more accurate assessments of circumferential resection margin (CRM) involvement. The article emphasizes the dynamic nature of rectal cancer treatment, advocating for individualized surgical planning based on comprehensive preoperative imaging and intraoperative assessment. This approach aims to optimize patient care by integrating recent advancements and anatomical insights into clinical practice for the management of low-lying rectal cancer. Particularly, the article discusses the importance of customizing the excision of Denonvilliers' fascia (DVF) based on tumor characteristics for optimal oncologic and functional outcomes, especially for anteriorly located tumors. It highlights the need for precise dissection techniques to ensure a negative CRM while preserving critical anatomical structures. Additionally, the review addresses the surgical management of tumors at the anorectal ring and introduces the Gate Approach for deep anterolateral pelvic dissection. Special attention is given to tumors impacting the membranous and prostate urethra, emphasizing tailored surgical approaches to balance complete tumor resection with the preservation of urogenital functions.

Risk of alterations in neurodevelopment in infants and preschool children with cancer.

BACKGROUND: Some cancer survivors experience difficulties with concentration, attention, and memory; however, there are no studies on neurodevelopment in patients under 5 years of age who are undergoing cancer treatment. Our aim was to evaluate neurodevelopment in cancer patients under 5 years of age using the Early Development Instrument (EDI) test, considering factors such as nutritional status, type of cancer, and treatment effect. METHODS: A cross-sectional study was conducted from February 2018 to March 2019. Patients with cancer diagnoses outside the central nervous system in any phase of cancer treatment were included. RESULTS: A total of 45 patients were included. Regarding fine motor skills, 28% of patients with retinoblastoma and 23% of patients with leukemia or lymphoma had a risk of developmental delay compared to 0% of patients with solid tumors (p = 0.025). The final results showed that 19 (42.2%) patients had normal neurodevelopment (gray), 7 (15.5%) had a delay in neurodevelopment (light gray), and 19 (42.2%) had a risk of developmental delay (black). Regarding developmental delay, 52% of patients in the leukemia and lymphoma group, 71% in the retinoblastoma group, and 23% in the solid tumor group presented developmental delay (p = 0.06). CONCLUSIONS: The risk of delay and lag in neurodevelopment is common in cancer patients under 5 years of age undergoing treatment. However, more studies are required to evaluate the effect of treatment on this group of patients as it may be affected by various factors.

INTRODUCCIÓN: En algunos pacientes supervivientes de cáncer se presentan dificultades de concentración, atención y memoria, sin embargo no hay estudios en relación al neurodesarrollo en pacientes menores de 5 años que se encuentran en tratamiento oncológico. Por lo que el objetivo fue valorar el neurodesarrollo en pacientes con cáncer durante el tratamiento oncológico mediante la prueba EDI tomando en cuenta diversos factores como su estado nutricional, tipo de cancer, y el efecto del tratamiento. MÉTODOS: Se realizó un estudio transversal, de febrero de 2018 a marzo de 2019. Se incluyeron pacientes mayores de 1 año y menores de 5 años con diagnóstico de cáncer fuera del sistema nervioso central, en tratamiento oncológico. RESULTADOS: Se incluyeron 45 pacientes. En el área motor fina el 28% de los pacientes con retinoblastoma y 23% con leucemias y linfomas se encontraron en rojo (retraso) en comparación con 0% de los pacientes con tumores sólidos (p = 0.025). En el resultado global se encontró que 19 (42.2%) pacientes tuvieron neurodesarrollo normal (gris), 7 (15.5%) rezago en el neurodesarrollo (gris claro) y 19 (42.2%) con riesgo de retraso en el desarrollo (negro). De los pacientes que presentaron riesgo de retraso el 52% fueron del grupo de leucemias y linfomas, el 71% en el grupo de retinoblastoma y el 23% del grupo de tumores sólidos (p = 0.06). CONCLUSIONES: La presencia de riesgo de retraso y rezago en el neurodesarrollo es frecuente en menores de 5 años con diagnóstico de cáncer. Se requieren más estudios, para evaluar el efecto del tratamiento en este grupo de pacientes, ya que pueden influir diversos factores.

Impact of gated FDG PET/CT on the staging of patients with suspected or proven newly diagnosed advanced epithelial ovarian, fallopian tube, and primary peritoneal cancer: results from a non-randomized, phase II clinical trial.

OBJECTIVE: Imaging for staging ovarian cancer is important to determine the extent of disease. The primary objective of this study was to compare gated 18F-fluorodeoxyglucose positron emission tomography coupled with computed tomography (FDG PET/CT) and standard CT scan with intravenous contrast to diagnose thoracic involvement in patients with advanced ovarian cancer prior to treatment. The secondary objective was to estimate changes in the International Federation of Gynecology and Obstetrics (FIGO) stage and clinical management resulting from gated PET/CT. METHODS: The IMAGE trial is a non-randomized phase II clinical trial comparing standard CT scanning with gated PET/CT in diagnosing thoracic involvement in a non-selected group of patients with suspected ovarian cancer on a contrast CT scan. Three sets of PET images were obtained comprising an ungated 2 min whole body image, a static 7.5 min image of the upper abdomen and thorax, and a gated end-expiratory image over the upper abdomen and thorax. Images were evaluated for specificity, sensitivity, diagnostic accuracy, and the proportion of patients with changes in FIGO stage and subsequent clinical management was compared between imaging techniques. RESULTS: A total of 84 patients were enrolled based on a standard CT scan, 67 of whom were eligible for gated PET/CT scans. Diagnostic accuracy with gated PET/CT was more than 80% for lesions in lung, liver, extra-abdominal sites, and pleura, but less than 50% for extra-abdominal lymph nodes. Compared with CT scan at baseline, 46% of patients who had 7.5 min gated PET/CT had disease upstaged from stage III to IV, and 8% had disease downstaged from stage IV to III. However, this led to a change of management in only 5% of patients. CONCLUSIONS: Gated PET/CT enables upstaging; however, in our institution it altered clinical management only in a minority of patients. TRIAL REGISTRATION NUMBER: NCT02258165.

Risk factors and incidence of oral tumors: Findings from a longitudinal population-based study.

OBJECTIVE: This study investigated risk factors contributed to benign and malignant oral tumors using longitudinal cohort big data. MATERIALS AND METHODS: We included individuals aged ≥40 years who participated in the National Health Examination in South Korea between 2003 and 2004. National Health Insurance claims data after 16 years were used to determine the incidence of oral tumors and the related risk factors. Hazard ratios were calculated using the Cox proportional hazard regression. RESULTS: A total of 5,992,671 participants were included. The incidence of oral cancer was significantly higher in men and increased with age, whereas that of benign tumors was unaffected by sex and decreased with age. Periodontal disease was associated with the incidence of oral cancer but not benign tumors. Soft tissue diseases were associated with both benign and malignant tumors. Various systemic diseases influence the development of oral tumors. Light alcohol consumption reduced the incidence of oral tumors, whereas heavy alcohol consumption increased the incidence of malignant tumors only. Smoking increased the incidence of benign but not malignant tumors. CONCLUSION: Recognized risk factors such as sex, age, comorbidities, and dental diseases were associated with oral tumors. Alcohol consumption and smoking were not significantly associated with malignant tumors.

Postauricular Myofibroma of the Facial Canal: A Case Report.

Myofibromatosis is a proliferative disorder characterized by the development of soft tissue neoplasms referred to as myofibromas that predominantly occur in infants and young children. These lesions have been described using varied terminologies since they were first reported in 1951. However, these neoplasms are histopathologically characterized by benign fibroblast and myofibroblast proliferation containing a biphasic presentation of spindle-shaped cells surrounding a central zone of less differentiated cells arranged in a hemangiopericytoma-like pattern in all cases. Usually, myofibromatosis occurs in children aged <2 years and is observed at birth in nearly 60% of cases. We report a rare case of myofibroma in an 8-year-old boy who presented with an approximately 6-month history of a left postauricular mass. Histopathological evaluation of the resected mass confirmed the diagnosis.

Imaging approach for jaw and maxillofacial bone tumors with updates from the 2022 World Health Organization classification.

Jaw and maxillofacial bone lesions encompass a wide variety of both neoplastic and non-neoplastic pathologies. These lesions can arise from various tissues, including bone, cartilage, and soft tissue, each presenting distinct challenges in diagnosis and treatment. While some pathologies exhibit characteristic imaging features that aid in diagnosis, many others are nonspecific. This overlap often necessitates a multimodal imaging approach, combining techniques such as radiographs, computed tomography, and magnetic resonance imaging to achieve a diagnosis or narrow the diagnostic considerations. This article provides a comprehensive review of the imaging approach to jaw and maxillofacial bone tumors, including updates on the 2022 World Health Organization classification of these tumors. The relevant anatomy of the jaw and dental structures that is important for accurate imaging interpretation is discussed.

Leiomyoma: An Exceptional Benign Tumor of the Kidney.

Renal leiomyoma is a benign finding in kidney pathology. It has been documented in various organs; renal location is less frequent and has been rarely documented in the literature. We present here the case of a renal leiomyoma revealed by an abdominal mass and flank pain. The diagnosis of certainty is histological, generally on surgical specimens. Due to the challenges associated with clinically diagnosing this tumor, a high level of suspicion is warranted when a patient presents with sizable and clearly defined renal lesions.

An Inflammatory Myofibroblastic Tumour Presenting with Limited Mouth-Opening, Hypoesthaesia of The Left Chin and Infraorbital Area, Intermittent Left Eye Ptosis and Converging Strabismus.

An inflammatory myofibroblastic tumour (IMT) is a rare neoplasm of mesenchymal origin, defined by myofibroblastic spindle cells accompanied by inflammatory cells, lymphocytes and eosinophils. Its symptomatology depends on the involved site and tends to mimic a malignant tumour clinically and radiologically. The head and neck region accounts for 5% of all IMTs. Here, we report a case of a 35-year-old woman, with no medical history, who presented with a mouth-opening limitation of 8 mm evolving for three years and occurring six months after of a wisdom tooth extraction. She also experienced a recent occurrence of left eye ptosis and a converging strabismus. On examination, the patient had a body temperature at 37°C, with hypoesthaesia of the left chin and infraorbital area, without any other abnormality. Laboratory examinations did not reveal a biological inflammatory syndrome or rhabdomyolysis. The infectious investigations were all negative, as well as the immunological tests, in particular negative for anti-AChR and anti-MuSK antibodies. On the facial computed tomography (CT) scan, we noted an active reshuffle in the left mandible ascending branch with a thickening of the ipsilateral pterygoid muscles and the left temporal meningeal tissue. After corticosteroid therapy 0.7 mg/kg/j, we obtained an improvement in the patient's mouth-opening, thus a biopsy of the lesion was performed under local anaesthesia, revealing IMT. The patient continued the corticosteroids therapy with a progressive tapering resulting in a marked clinical improvement of the mouth-opening limitation and her ptosis. LEARNING POINTS: An inflammatory myofibroblastic tumour (IMT) is a challenging disease.Given the variable clinical and radiological presentation of the disease, it is of paramount importance to know it, to be swiftly recognised so diagnosis can be promptly made.The adapted treatment should be immediately started to prevent possible life-threatening outcomes.

Head-to-head comparison of contrast-enhanced CT, dual-layer spectral-detector CT, and Gd-EOB-DTPA-enhanced MR in detecting neuroendocrine tumor liver metastases.

PURPOSE: To explore the optimal of kiloelectron voltage (keV) of virtual monoenergetic imaging (VMI) of dual-layer spectral-detector CT (DLCT) in detecting neuroendocrine tumor liver metastases (NETLM) and to investigate diagnostic performance of polyenergetic images (PEI), DLCT, and Gd-EOB-DTPA-enhanced MR. METHODS: Seventy-two patients with suspected NETLM who underwent DLCT and Gd-EOB-DTPA-enhanced MR were retrospectively enrolled. Tumor signal-to-noise ratio (SNR) and contrast-to-noise ratio (CNR) were compared between PEI and VMI at 40-140 keV. Two radiologists read the CT examinations with and without VMI separately in consensus. Two other radiologists read the Gd-EOB-DTPA-enhanced MR in consensus. The diagnostic performance was evaluated. Reference standard was histopathology, follow-up, and interpretation of all available imaging. RESULTS: The highest SNR and CNR were observed at VMI40keV, significantly higher than PEI in the arterial and venous phases (all P<0.01). A total of 477 lesions were identified (396 metastases, 81 benign lesions). Per-lesion AUC was 0.86, 0.91, and 0.97 (PEI, DLCT, and Gd-EOB-DTPA-enhanced MR, respectively). Sensitivity of PEI, DLCT, and Gd-EOB-DTPA-enhanced MRI were 0.76, 0.86, and 0.95, respectively. DLCT significantly improved sensitivity compared to PEI. MR had significantly higher sensitivity than DLCT and PEI. Subgroup analysis demonstrated that the difference in diagnostic performance was concentrated on lesions < 10 mm. CONCLUSION: The image quality of VMI40keV is higher than that of PEI. DLCT with VMI40keV provides better diagnostic sensitivity for NETLM detection than PEI. Gd-EOB-DTPA-enhanced MR yielded the best diagnostic performance for NETLM detection.

Evaluating the feasibility of repeat sentinel lymph node biopsy in ipsilateral breast tumor recurrence: Technical considerations and oncologic outcomes.

INTRODUCTION: Ipsilateral breast tumor recurrence (IBTR) remains a concern despite standard treatments. Advances in early detection have shifted surgical paradigms towards less invasive approaches. While repeat sentinel lymph node biopsy (rSLNB) emerges as a viable option according to the 2023 National Comprehensive Cancer Network (NCCN) guidelines, its efficacy remains uncertain. This study aimed to assess lymphatic drainage patterns in IBTR and evaluate the feasibility of rSLNB, along with analyzing oncologic outcomes. METHODS: A retrospective analysis involving 78 patients with IBTR who had prior breast-conserving surgery (BCS) with sentinel lymph node biopsy (SLNB) and adjuvant whole breast irradiation (WBI) at Samsung Medical Center was conducted. Data on patient characteristics, lymphatic mapping techniques, and oncologic outcomes were collected and analyzed. RESULTS: Among 78 patients with IBTR, 82.1 % underwent successful rSLNB, predominantly detecting lymphatic drainage to the ipsilateral axilla (80.8 %). The initial tumor location correlated significantly with failed lymphatic mapping (p = 0.019). A third event occurred in 28.8 % of invasive IBTR cases, notably associated with postmenopausal status, higher T stages, and HR(-)/HER2(-) subtype (p < 0.001). The risk of a third event increased by over 50 % within a 2-year interval post-IBTR. CONCLUSION: rSLNB in patients with IBTR, particularly for tumors initially located outside the upper-outer quadrant, demonstrated technical feasibility. The combined use of blue dye with lymphoscintigraphy may enhance rSLNB success rates. Active surveillance, especially for triple negative IBTR cases, may be important due to their aggressive nature and rapid progression potential within a short interval post-IBTR.

Endometrioid adenocarcinoma with sacral metastasis.

Most bone tumors are metastatic. Breasts, lungs, kidneys, and thyroid are the primary sites most commonly involved in bone metastasis-type outcomes. This case study describes the involvement of a patient with a bone tumor located in the axial skeleton, initially in the sacral region. However, the primary site was undefined. Therefore, it was necessary to expand the investigation with immunohistochemistry, which demonstrated a metastatic tumor compatible with endometrioid adenocarcinoma. But even after examination, no active lesion was found in the endometrial region. The study was observational, descriptive, and aimed to discuss the importance of more specific investigative methods. In this context, immunohistochemistry stands out as an exquisite method capable of optimizing diagnosis, therapy, and consequently, prognosis.

Association between myeloid disorders and adult onset-inflammatory syndromes, successful treatment with JAK-inhibitors: Case series and literature review.

Approximately one-third of patients with myeloid disorders like myelodysplastic syndrome (MDS) and chronic myelomonocytic leukemia (CMML) exhibit inflammatory and autoimmune disorders (IADs). These IADs often include atypical and incomplete forms of common autoimmune conditions, and exhibit resistance to conventional immunosuppressive therapies. There is growing interest in molecular relationships between IADs and MDS/CMML to find potential targeted therapies. Recently, patients with somatic mutations in the UBA1 gene were identified as having VEXAS syndrome. Herein, we present a concise case-series illustrating concurrent elderly-onset inflammatory manifestations and myeloid disorders (MDS, CMML, and idiopathic cytopenia of undetermined significance). These patients manifested inflammatory or autoimmune symptoms, including erythema nodosum, Raynaud's phenomenon, Sjogren syndrome, and refractory pruritus, having onset after 60-years of age. The inflammatory manifestations were largely refractory to traditional immunosuppressive regimens. Remarkably, treatment with a JAK-1 inhibitor, upadacitinib, in two cases yielded marked resolution of inflammatory symptoms, facilitating the gradual tapering of corticosteroids, improvement of hemoglobin levels, and reduction in serum C-reactive protein levels. Upon loss of response to upadacitinib, JAK-2 inhibitor ruxolitinib provided clinical benefit in one of the cases, facilitating further tapering of glucocorticoids. This arena warrants further exploration through prospective studies of larger cohorts to delineate optimal management strategies.

Therapy-related myeloid neoplasms following curative treatment of acute promyelocytic leukemia: incidence, correlation with therapeutic regimen, and future directions.

All-trans retinoic acid (ATRA) and arsenic trioxide (ATO) have revolutionized the treatment of acute promyelocytic leukemia (APL), offering a cure rate of > 80%. Along with improved survival, the long-term consequences of anti-APL therapy are becoming increasingly apparent, including potential therapy-related myeloid neoplasms (t-MNs). T-MNs are well known to arise after cytotoxic chemotherapy, but the leukemogenic potential of regimens utilizing only ATRA/ATO is not well established. The objective of this study is to examine the incidence, long-term risk, and clinicopathologic features of t-MNs arising after anti-APL therapy and how they correlates with the therapeutic regimen employed. We retrospectively collected treated APL patients between 01/2001 and 02/2021, categorized them into ATRA/ATO + chemo and ATRA/ATO groups based on the regimen used, and evaluated for the development of t-MN. A total of 110 APL patients were identified, including 67 (61%) treated with ATRA/ATO + chemo and 43 (39%) treated with ATRA/ATO only. Overall, 4/110 (3.6%) patients developed t-MNs, with all four emerging in the ATRA/ATO + chemo group. Ultimately, the incidence of t-MN in ATRA/ATO + chemo group was significantly higher compared with ATRA/ATO only group(5.97% vs. 0.0%, respectively; p = 0.0289). Our data spanning over two decades suggests that conventional chemotherapy for APL is associated with a small but significant risk of t-MN, whereas ATR/ATO does not carry this risk. This takes on new significance, considering several recent and ongoing trials have shown that a chemotherapy-free approach might become feasible for all risk APL types in the near future. Consequently, the omission of leukemogenic and arguably unnecessary chemotherapy from APL regimens may reduce the incidence of t-MNs in long-term survivors without sacrificing their cure rates.