Pancreatic Head Research Articles

An innovative surgical approach for solid pseudopapillary neoplasm with duodenal invasion in a pediatric patient: a case report

BackgroundPediatric pancreatic tumors, especially with duodenal invasion, are exceptionally rare and a strategy for their treatment has not been established. A pancreaticoduodenectomy is often the desired treatment, but may be over-invasive for solid pseudopapillary neoplasm (SPN). This study reports an innovative surgical approach for SPN with duodenal invasion using pancreatic enucleation and endoscopically guided partial duodenectomy.Case presentationAn 11-year-old girl complained of malaise and presented with severe anemia; imaging revealed a tumor of undetermined origin, involving the pancreatic head and descending duodenum. Intraoperative findings showed tumor adherence to the pancreatic head and endoscopy revealed invasion of the duodenum. The tumor was enucleated from the pancreatic head, and partial duodenectomy was performed under endoscopically guided direct visualization. Pathology confirmed SPN with duodenal invasion, and no residual tumor. Although a Grade B pancreatic fistula occurred postoperatively, it was managed conservatively. At the 15-month follow-up, no signs of tumor recurrence, duodenal stenosis, or pancreatic dysfunction were evident.ConclusionsGiven the good prognosis of SPN, we believe that enucleation from the pancreatic head combined with an endoscopically guided partial duodenectomy could be a useful and less invasive alternative to pancreaticoduodenectomy for cases with duodenal invasion.

Disseminated Cryptococcus over pancreas, lung, and brain: a case report

BackgroundCryptococcus is an opportunistic infection acquired through inhalation from the environment, primarily affecting individuals with compromised immune systems. It typically leads to pneumonia upon passing through lung tissue. The infection can disseminate to various organs via the bloodstream, resulting in meningitis or encephalitis in the central nervous system. Disseminated Cryptococcus has been reported to involve the skin, liver, eyes, lymph nodes, bone marrow, spleen, kidneys, and intestines, significantly increasing morbidity and mortality. However, pancreatic involvement in Cryptococcus is relatively rare, and a few case reports have highlighted severe organ damage and high mortality rates.Case presentationIn this case report, we present the case of a 36-year-old Asian man who presented with a 2-week history of headaches and blurred vision in his right eye. Brain magnetic resonance imaging revealed multiple brain masses, along with a mass in the lower left lung field and a tumor in the pancreatic tail, as detected by chest computed tomography. Endoscopic ultrasound-guided fine needle biopsy and computed tomography-guided lung biopsy confirmed the diagnosis of disseminated cryptococcal infection involving the pancreas, lung, and brain. The patient’s clinical condition improved following antifungal therapy. Additionally, we identified anti-granulocyte–macrophage colony-stimulating factor antibody as a risk factor for disseminated cryptococcal infection in this patient.ConclusionDisseminated cryptococcosis can be a potentially lethal condition, as highlighted by previous literature. However, early diagnosis using contrast-enhanced harmonic endoscopic ultrasound and endoscopic ultrasound-guided biopsies, as well as prompt treatment as demonstrated in our case, can improve outcomes and prevent mortality.

An aggressive gastric CIC-DUX4 sarcoma surgically resected with multivisceral organs: a case report

BackgroundCapicua transcriptional repressor-double homeobox 4 sarcoma (CDS) is a rare and aggressive malignant soft tissue tumor that typically arises within the soft tissues. We report an exceptionally rare case of a gastric CDS successfully resected despite its extensive invasion into surrounding organs.Case presentationA 48-year-old male presented with a progressively enlarging abdominal mass. Upper gastrointestinal endoscopy revealed a large ulcerative tumor on the posterior gastric wall. Biopsy results initially suggested a neuroendocrine cell carcinoma. Contrast-enhanced computed tomography showed a 20 cm tumor protruding from the posterior stomach wall, directly invading the pancreas and colon. We performed a multivisceral resection (stomach, pancreatic tail, spleen, and transverse colon) achieving an R0 resection. Pathological examination of the permanent specimen revealed small round cells with high nuclear-to-cytoplasmic ratios. Immunohistochemical staining confirmed the diagnosis of CDS. The patient recovered well and was discharged on postoperative day 33.ConclusionsThis case report describes the first detailed account of a surgically resected aggressive CDS originating from the stomach.

Study of variations in the origin and branching of the human dorsal pancreatic artery

In surgical practice, the human pancreas is conventionally divided into two major anatomical and surgical segments: the right (cephalo-cervical) and the left (corpore-caudal). Both of these segments are abundantly supplied with blood. The artery-first approach is widely used in pancreatic resections to avoid venous stasis and achieve reduced blood loss. The dorsal pancreatic artery participates in the blood supply of both segments with its branches, which makes its anatomical study relevant and promising for clinical medicine. The aim was to describe the main branches of the dorsal pancreatic artery and to determine the frequency of their occurrence in the studied cases. Materials and Methods. The work was performed on archival fixed material (n = 60) of duodenum-pancreas-spleen organ complexes with extra-organ vessels taken from the collection of the Department of Anatomy, RyazSMU. All organ complexes belonged to mature age people without pronounced pathology of the organs of the investigated area. The division by sex was not performed. Results. The dorsal pancreatic artery was identified in all cases studied. Most often (n = 40; 67%) it originated from the branches of the ventral trunk, less often (n = 18; 30%) it was a branch of the superior mesenteric artery and in two cases (3%). Despite the place of origin, its trunk diameter averaged 1.8 mm (min 1.6; max 2.0). The branches of this artery were most often directed towards the gland body (85% of cases), less often - towards the head (15%). Conclusion. The dorsal pancreatic artery is the main artery feeding the body and tail of the human pancreas. In the present study, its branches were more frequently recorded towards the body of the gland, despite the origin of the vessel. Despite the accumulated anatomical and clinical knowledge about the peculiarities of the origin and branching of the human dorsal pancreatic artery, the understanding of the principles of the direction of its branches remains open to researchers.

Comparative evaluation of hepatosteatosis in patients with type 2 diabetes mellitus using non-contrast abdominal CT and laboratory findings

Aim: This study aims to underscore the significance of employing multiple parameters from non-contrast abdominal CT scans for the assessment of hepatosteatosis in patients with Type 2 Diabetes Mellitus. Methods: Non-enhanced Computed Tomography of the diabetic subjects were analyzed. Control subjects were selected from non-diabetic patients who had undergone abdominal tomography within the same period. The craniocaudal length of the liver and liver, spleen, pancreas densities, and epicardial adipose tissue were measured. Additionally, patient demographics and laboratory values were retrospectively obtained. Results: The craniocaudal length of the liver was significantly greater in the diabetes mellitus group compared to the control group (168.3 ± 17.2 mm vs 152.3 ± 14.8 mm, p < 0.001). Hepatosteatosis was observed in 22 individuals with diabetes mellitus, whereas only one participant in the control group had this condition (p < 0.001). The diabetes mellitus group exhibited significantly lower median liver density (p < 0.001), liver-spleen density ratio (p = 0.004), pancreatic head density (p = 0.001), and pancreatic body density (p = 0.013). Additionally, the average thickness of epicardial adipose tissue was markedly higher in the diabetes mellitus group compared to the control group (8.1 ± 1.9 mm vs 4.9 ± 1.1 mm, p < 0.001). Conclusions: These data indicate an association between hepatosteatosis and increased epicardial adipose tissue thickness, liver and pancreatic densities in individuals with diabetes mellitus. These findings suggest that non-contrast abdominal CT findings such as epicardial adipose tissue thickness and relevant laboratory tests may aid in evaluating metabolic disorders and fat accumulation in diabetic patients.

Increased Proportion of the Squamous Cell Carcinoma Components Is Associated with Aggressive Behavior and a Worse Prognosis in Resected Pancreatic Adenosquamous Carcinoma.

Pancreatic adenosquamous carcinoma (PASC) is a subtype of pancreatic cancer with a poorer prognosis than pancreatic ductal carcinoma (PDAC). The pathogenesis of this histological subtype has not been fully explained due to its rarity. Of the 245 patients who underwent pancreatic resection for pancreatic cancer, six (2.3%) were diagnosed with PASC. They were retrospectively allocated to Group A (≥ 50% adenocarcinoma components) or Group S (≥ 50% squamous cell carcinoma components). The six patients with PASC were all males between the ages of 63 and 77years, with tumors of 12 to 52mm in diameter. Tumors were located in the pancreatic head (n = 2) and the pancreatic tail (n = 4). Relative to Group A, all three patients in Group S had larger tumors diameters, ≥ 40mm with invasion to other organs. Cancer-specific survival of Group S was worse than that of the PDAC group (median survival, 1.5years vs. 4.1years). All patients in Group A were alive at the end of follow-up. Recurrence-free survival of Group S was inferior to that of the PDAC group (median survival, 0.2years vs. 1.8years; Group A, not defined). Immunohistochemistry revealed the MIB-1 positivity rate in squamous cell carcinoma regions was 1.8 times higher than that in adenocarcinoma regions in the same specimens. In PASC patients, an increased proportion of squamous cell carcinoma components was associated with aggressive behavior and a worse prognosis. This was due to the high MIB-1 positivity rate of squamous cell carcinoma components.

Perioperative medication therapy for Muslim patients in Germany undergoing oncological surgery: a retrospective study

PurposeEngagement of healthcare professionals with patients from diverse cultural and religious backgrounds is crucial in our multicultural society, where miscommunication and errors in medical history taking can lead to incorrect treatment. In particular, Muslim patients may present unique considerations due to their specific cultural and religious beliefs, which can significantly impact treatment outcomes. This study focuses on perioperative medication therapy for patients undergoing upper and lower gastrointestinal tract and pancreatic tumor surgery, specifically examining whether Islamic beliefs were duly considered in medication selection compared to a matching patient cohort.Materials and methodsData from January 2004 to July 2023 were analyzed. Muslim patients were identified using the onomastic method and matched with non-Muslim patients at a 1:3 ratio based on age, gender, and procedure. Analysis included examination of subcutaneous, oral, and intravenous medications, with attention to ingredients and compatibility with Islamic principles.ResultsAmong 5272 patients, only 5 met the study’s inclusion criteria as Muslim patients, undergoing procedures such as anterior rectum resection, gastrectomy, and pancreatic head resection. Their religious affiliations were not documented in the admission records. According to the matched-pair analysis, consistent treatment was performed regardless of religious beliefs. All patients received subcutaneous medication, primarily enoxaparin, instead of fondaparinux, an Islam-compliant alternative. Intravenous heparin was used once for short period. Contrary to Islamic dietary restrictions and the availability of alternatives, capsules containing animal-derived gelatin and other non-compliant medications were administered orally.ConclusionThis study underscores the importance of acknowledging Muslim patients’ cultural and religious backgrounds in the perioperative setting, as failure to do so may lead healthcare professionals to overlook their potential alternative medication needs, which are essential for providing tailored medical care in modern societies. Integration of diversity-related topics into medical curricula is essential for better preparing physicians for clinical practice and ensuring patient-centered care.

Elevated Urinary Levels of Fungal and Environmental Toxins in Patients with Pancreatic Ductal Adenocarcinoma

BackgroundRisk factors for pancreatic ductal adenocarcinoma (PDAC) include tobacco/alcohol abuse, genetic predisposition, insulin resistance, and pancreatic cysts. Despite these well-established risk factors and the screening of high-risk individuals, some people still develop PDAC. This study aims to explore a potential risk factor for PDAC by investigating the association between fungal toxins (FT) and environmental toxins (ET) and the disease. We predicted that individuals with PDAC would have higher levels of these toxins compared to healthy controls. The rationale behind this hypothesis is that exposure to FT and ET might contribute to the development of PDAC by elevating cancer risk.MethodsA pilot retrospective cohort study was conducted at Moffitt Cancer Center from 2022 to 2023. This study compared FT and ET levels, demographic data, and PDAC features between subjects with PDAC and healthy controls.ResultsForty subjects were enrolled in the study, comprising 20 with pancreatic ductal adenocarcinoma (PDAC) and 20 healthy controls. Baseline demographics were similar between the two groups. Among the PDAC subjects, the most common tumor location was the head of the pancreas (55%); 30% had locally advanced disease, 45% were borderline resectable, and 10% had metastatic disease.Compared to the controls, subjects with PDAC had significantly higher levels of fungal toxins (FTs) including ochratoxin, gliotoxin, and citrinin (p < 0.05). Additionally, PDAC patients had significantly elevated levels of environmental toxins (ETs) such as methyl tert-butyl ether (MTBE), xylene, styrene, acrylonitrile, perchlorate, diphenyl phosphate, bromopropane, organophosphates, acrolein, tiglylglycine, and diethylphosphate (p < 0.05).ConclusionOur study demonstrates that subjects with PDAC, without other risk factors, have higher FT and ET levels than controls. Further studies are needed to evaluate whether ET and FT exposure can be clinically utilized as a risk factor for PDAC development.

Afferent loop syndrome following pancreatic head cancer surgery treated with metal stent placement using a short-type single-balloon enteroscope.

Afferent loop syndrome following pancreatic head cancer surgery treated with metal stent placement using a short-type single-balloon enteroscope.

Anatomical variants of the jejunal veins and their technical implications in pancreaticoduodenectomy: a systematic review and meta-analysis.

One of the most common causes of bleeding during pancreaticoduodenectomy (PD) is dissection of the pancreatic head from the superior mesenteric vein (SMV) and superior mesenteric artery (SMA). Knowledge of the anatomical variants of the veins draining the proximal jejunum may allow a better control of bleeding during detachment of the uncinate process and pancreatic head from the mesenteric pedicle and division of the mesopancreas. The aim of this systematic review and meta-analysis is to evaluate the anatomical variations of the first jejunal vein (FJV) and jejunal trunk (FJT). Fourteen studies (1,888 patients) were included. We performed a systematic review of the available Literature according to the PRISMA guidelines. The analysis has shown that the posterior course of the FJT and FJV represents the most frequent topographical location (PPE 79.6%) with the anterior jejunal trunk (JT) having a lower rate (PPE 20.4%). Few articles reported the variations with separate trunks for the first and second jejunal vein. A thorough preoperative radiological assessment of the anatomical variation of FJT and FJV may confer some advantage to establish the best therapeutic strategy and the best surgical approach in case of pancreatic head carcinoma, as it can allow a better estimate of the extent of the neoplasm and improve the accuracy of surgical dissection with potential for reduced bleeding.

Application of LEF-1 immunohistochemical staining in the diagnosis of solid pseudopapillary neoplasm of the pancreas

IntroductionSolid pseudopapillary neoplasm (SPN) is a tumor of young females with gain-of-function mutation in catenin beta 1 gene involved in Wnt signal transduction pathway. Beta-catenin immunohistochemistry (IHC) is used to diagnose SPN. Lymphoid enhancer-binding factor 1 (LEF-1) has been recognized in the transactivation of Wnt pathway. We aim to study LEF-1 IHC in SPN and other pancreatic tumors and compare it with beta-catenin IHC. MethodsWe retrieved cases of SPN, pancreatic neuroendocrine tumor (PanNET), serous cystadenoma (SCA), ductal adenocarcinoma (PDAC) and acinar cell carcinoma (ACC) from 2011 to 2023. Formalin-fixed, paraffin-embedded blocks with adequate tumor were cut and stained with beta-catenin (B-Catenin-1 clone) and LEF-1 (EP310 clone) IHC. Cases were reviewed by two pathologists independently. Nuclear staining with LEF-1 and beta-catenin was considered as positive. ResultsOur cohort consisted of 111 cases [SPN = 59 (42 resections, 11 FNA, 6 biopsies), PDAC = 24, PanNET = 22, SCA = 5, ACC = 1]. For SPN cases male to female ratio was1:8. Age ranged from 9 to 81 years (average: 32 years). Pancreatic tail was the most common location (47 %) followed by head (28 %), body (19 %) and neck (6 %). Tumor size ranged from 1.0 to 12.2 cm (average: 5 cm). Among the SPN cases 57/59 demonstrated strong nuclear LEF-1 staining. 2/49 cases were negative for LEF-1 (both pathologist in agreement). All SPN tumors demonstrated nuclear staining with beta-catenin. Among the non-SPN tumors, beta-catenin showed nuclear staining in 2/52 cases (2 PDAC). The remaining 50 cases were negative for nuclear beta-catenin and demonstrated variable staining pattern with interpretation variability between the two pathologists. The sensitivity and specificity for LEF-1 were 97 % and 100 %, respectively, while for beta-catenin, they were 100 % and 96 % respectively. ConclusionCrisp nuclear staining of LEF-1 without background staining makes diagnostic interpretation relatively easy and accurate compared to beta-catenin IHC. This is further helpful for small biopsy samples to help differentiate SPN from mimickers such as PanNET. None of the non-SPN cases displayed nuclear LEF-1 rendering it a valuable adjunct to beta-catenin in the diagnostic evaluation of SPN.

The efficacy of wrapping with polyglycolic acid mesh and fibrin glue in preventing clinically relevant pancreatic fistula after minimally invasive distal pancreatectomy (WRAP Study): study protocol for a multicenter randomized controlled trial in Japan

BackgroundPostoperative pancreatic fistula (POPF) continues to be the most common complication after distal pancreatectomy (DP). Recent advancements in surgical techniques have established minimally invasive distal pancreatectomy (MIDP) as the standard treatment for various conditions, including pancreatic cancer. However, MIDP has not demonstrated a clear advantage over open DP in terms of POPF rates, indicating the need for additional strategies to prevent POPF in MIDP. This trial (WRAP study) aims to evaluate the efficacy of wrapping the pancreatic stump with polyglycolic acid (PGA) mesh and fibrin glue in preventing clinically relevant (CR-) POPF following MIDP.MethodsThis multicenter, randomized controlled trial will include patients scheduled for laparoscopic or robotic DP for tumors in the pancreatic body and/or tail. Eligible participants will be centrally randomized into either the control group (Group A) or the intervention group (Group B), where the pancreatic stump will be reinforced by PGA mesh and fibrin glue. In both groups, pancreatic transection will be performed using a bioabsorbable reinforcement-attached stapler. A total of 172 patients will be enrolled across 14 high-volume centers in Japan. The primary endpoint is the incidence of CR-POPF (International Study Group of Pancreatic Surgery grade B/C).DiscussionThe WRAP study will determine whether the reinforcement of the pancreatic stump with PGA mesh and fibrin glue, a technique whose utility has been previously debated, could become the best practice in the era of MIDP, thereby enhancing its safety.Trial registrationThis trial was registered with the Japan Registry of Clinical Trials on June 15, 2024 (jRCTs032240120).

A Curious Case of Pancreatoblastoma in an Older Age Woman

Abstract Introduction/Objective Pancreatoblastoma is a malignant epithelial neoplasm of the pancreas and displays multiple lines of differentiation with acinar differentiation as the predominant pattern. The squamoid nests are considered a defining component of pancreatoblastoma. Pancreatoblastoma is seen most often in children, however, rare cases in adults have been described in the literature. In children, most pancreatoblastomas are indolent and curable, however, the prognosis is worse in adults due to the presence of frequent metastasis. Methods/Case Report We report an interesting case of pancreatoblastoma in a 65-year-old woman with no pertinent medical history who presented with abdominal discomfort. MRI abdomen showed an 11.3 cm mass with central necrosis within the head of the pancreas. Fine-needle aspiration (FNA) of the mass showed monotonous cells with neuroendocrine differentiation and FNA was signed out as a low-grade neuroendocrine tumor (NET). The patient underwent a Whipple resection for a presumed NET. On gross examination, a 14 cm large solid, fleshy, lobulated, partially encapsulated mass was noted in the head of the pancreas. H&amp;E slides on low power showed the tumor composed of highly cellular lobules separated by fibrous bands. Within the lobules, the neoplastic cells predominantly had an acinar differentiation with focal trabecular or solid areas. Dispersed within the tumor were squamoid nests composed of plump to spindle-shaped cells with abundant eosinophilic cytoplasm arranged in whorls. Immunohistochemical stains demonstrated squamoid nests being positive for CK5/6 and had a nuclear-staining pattern with beta-catenin. The remaining tumor was positive for trypsin, chymotrypsin, BCL-10, synaptophysin (focal), chromogranin (focal), and INSM-1 (patchy). On MSK-Impact (next- generation sequencing), a mutation in CTNNB1 was detected. Results (if a Case Study enter NA) NA Conclusion In adults, it is important to consider pancreatoblastoma in the differential diagnoses of solid pancreatic tumors while being cautious not to confuse it with neuroendocrine tumor or acinar cell carcinoma. The presence of squamoid nests is critical for its diagnosis. Making a diagnosis of pancreatoblastoma through FNA or pancreatic biopsy can be exceptionally challenging in cases lacking squamoid nests.

Splenic Epidermoid Cyst arising within an Intrapancreatic Accessory Spleen Masquerading as a Pancreatic Mucinous Cystic Neoplasm-a diagnostic enigma

Abstract Introduction/Objective Accessory spleens are congenital embryological aberrations usually found within the splenic hilum with no clinical significance. An IntraPancreatic Accessory Spleen (IPAS) is an uncommon benign pancreatic lesion encountered clinically. A splenic epidermoid cyst arising within an IPAS is exceedingly rare and is discussed below. Methods/Case Report A 68-year-old male presented with intermittent post-prandial epigastric pain radiating to the left flank for &amp;gt;1.5 years. Radiological imaging identified a 5.2 cm calcified cyst in the pancreatic tail with a differential of mucinous pancreatic neoplasm/ pancreatic pseudocyst. Additionally, a 2.8cm lobulated solid hypoechoic mass was seen at the porta hepatis with a differential of enlarged lymph node/benign neoplasm. Laboratory biochemical tests were within normal limits except for mildly elevated ferritin. Fine needle aspiration biopsy was indeterminate. The patient elected to proceed with surgical resection. A laparoscopic distal pancreatectomy with splenectomy and excision of the hilar mass was performed. The hilar mass was analyzed by hematopathology and revealed a hyaline- vascular variant of Castleman disease. On gross examination of the distal pancreatic/splenectomy specimen, a well- demarcated cystic lesion with brownish fluid was noted within the pancreatic tail. Microscopic examination revealed a nonpathological pancreas separated by a fibrous capsule with a large cyst arising within an intrapancreatic accessory spleen. The cyst was lined with multilayered non-keratinized CK5/6, and p63 positive squamous epithelium with no lymphocytic infiltrates and absent hair/ dermal appendages confirming a splenic epidermoid cyst. Post-operative follow-up at 6 weeks was uneventful. Results (if a Case Study enter NA) NA Conclusion An epidermoid cyst arising within an intrapancreatic accessory spleen (ECIAS) is extremely rare thus precluding accurate preoperative diagnosis as this entity shares similar radiological imaging characteristics as other cystic lesions such as mucinous pancreatic cysts. It is important for anatomic pathologists to recognize and consider the intrapancreatic compartment as a possible location for accessory spleens. Accurate preoperative diagnosis can prevent unnecessary surveillance and/or extensive surgical resection.

Duodenal Adenocarcinoma Arising from Brunner’s Glands, A Rare Case Report

Abstract Introduction/Objective Malignancies arising from the Brunner’s glands in duodenum are exceedingly rare with very few cases reported in the literature. Here, we reported a case of duodenal adenocarcinoma of Brunner’s gland origin that obstructed duodenum and extended to involve the pancreatic head. Methods/Case Report A 48-year-old woman came to the Emergency Department of our hospital with epigastric and right upper quadrant abdominal pain. CT imaging demonstrated a heterogeneously enhancing 8.4 x 5.4 x 6 cm mass involving the duodenal lumen, wall, and pancreatic head. An endoscopic ultrasound showed a large fungating, infiltrative, and ulcerative mass in the second portion of the duodenum distal to the ampulla but not involving it. The pancreatic and common bile ducts were uninvolved. Her endoscopic ultrasound biopsy was positive for adenocarcinoma. CA19-9 levels were normal. The patient received chemotherapy with subsequent Whipple procedure. Gross examination revealed an exophytic, tan-white mass with the epicenter in the duodenal wall obstructing the duodenal lumen and extending to involve the pancreatic head inferiorly. The mass was predominantly solid with a few small mucus cysts. Light microscopy demonstrated moderately differentiated adenocarcinoma with prominent necrosis and inflammation. There are intestinal-type, gastric-type and indeterminate types of epithelia in the carcinoma. There are areas in the duodenal wall where a continuous transition from benign Brunner’s glands to dysplastic glands and then to adenocarcinoma is present. Neither adenoma in the overlying duodenal mucosa nor abnormalities in the pancreatic parenchyma is observed. Immunohistochemical stains showed that the neoplastic cells are positive for CAM 5.2, AE1/AE3, and CK7, while only patchy positivity with CK20 and CDX2. Taken together, a diagnosis of duodenal adenocarcinoma arising from Brunner’s glands was rendered. Results (if a Case Study enter NA) N/A Conclusion More cases are needed in order to establish standard treatment guidelines and determine prognostic factors.

The umblicus says it all

Abstract Introduction/Objective Umbilical nodules can be divided into benign and malignant. Malignant lesions of the umbilicus are very rare. They can arise from primary tumors or secondary to abdominal and pelvic malignancies. Literature review suggests that these umbilical lesions can present as primary tumor in 38% of the cases, endometriosis in 32%, and metastatic lesions in 30%. Umbilical metastasis is one of the main characteristic signs of extensive neoplastic disease and is known as Sister Mary Joseph’s nodule (SMJN). It is a very rare finding and the incidence of SMJN is 1- 3% in people diagnosed with intra-abdominal or pelvic malignancies. Methods/Case Report A 31-year-old male with no past medical history presented to the emergency room with 3 months history of umbilical nodule and 60 pounds weight loss. He experienced early satiety, abdominal pain, nausea and generalized fatigue. CT abdomen showed hepatomegaly with numerous hypoattenuating lesions throughout the liver with peripheral enhancement, and hypoattenuating mass within the pancreatic tail and body measuring approximately 2.1 x 5.8 cm that appears contiguous with hilar splenic lesion. CT chest showed multiple pulmonary metastases with prominent precarinal and subcarinal lymph nodes. Serologic tests revealed elevated Alpha fetoprotein, ALP, ALT, and CA19-9. Patient underwent umbilical nodule core biopsy and right liver core biopsy. Microscopic evaluation of the specimens revealed pancreatic acinic cell carcinoma with two different morphological variants. Umbilical nodule with glandular pattern and liver lesion with trabecular pattern. Immunohistochemical stains were performed with tumor cells positive for CK 7, Trypsin and BCL-10 and negative for Glypican 3, CK 20, AFP and CD56. The prognosis is poor, with an average survival time of about 19 months. After the diagnosis patient underwent chemotherapy. Results (if a Case Study enter NA) NA Conclusion Umbilical nodules can be diagnosed with careful clinical and histopathological evaluation. The prognosis of patients presenting with SMJN is generally poor as it is a sign of advanced malignancy. Sometimes it may be the first and only sign of an internal neoplasm. Therefore, this is an important differential diagnosis to consider.

Malignant Peripheral Nerve Sheath Tumor of the Pancreas with Molecular Confirmation: A Case Report with Comprehensive Histopathologic and Molecular Characterization

Abstract Introduction/Objective Malignant peripheral nerve sheath tumors (MPNSTs) are rare soft tissue sarcomas arising from a peripheral nerve or exhibiting nerve sheath differentiation. They are often associated with neurofibromatosis type 1, and most often arise in the trunk, extremities, or head and neck region. In patients without neurofibromatosis type 1, sporadic de novo or radiotherapy-associated MPNSTs can pose a diagnostic challenge. MPNSTs arising from visceral organs are exceedingly rare. Here we report a case of MPNST arising in the pancreas. Methods/Case Report The patient is a 75-year-old female, MUTYH mutation carrier, with prior history of breast and bladder cancers, each treated with respective local excision and radiation therapy. Magnetic resonance imaging (MRI) performed for pancreatic cancer screening showed a 4.7 cm heterogeneous mass in the pancreatic tail suggestive of pancreatic neoplasm. Fine needle aspiration cytology was non-diagnostic. However, due to the radiologic concern for malignancy, the patient proceeded with surgical resection by distal pancreatectomy with splenectomy. Macroscopically, a 6.5 cm, tan-white, partially necrotic mass was identified in the pancreatic tail. Microscopic examination showed a spindle cell neoplasm arranged in a vague fascicular fashion with moderate nuclear pleomorphism, geographic necrosis, and frequent mitotic figures (15 per 10 high power fields). Fifteen lymph nodes were negative for metastasis. The tumor was confined to the pancreas grossly and microscopically. Immunohistochemical stains showed the neoplastic spindle cells to be positive for TLE1 (diffuse) and S100 (patchy), while negative for SOX10, CD117, DOG1, desmin, actin, MyoD1, ERG, STAT6, and ALK1. In addition, H3K27me3 showed loss of nuclear expression. Next generation sequencing (NGS) revealed gene mutations in NF1, TP53, PIK3CA, and PTEN, and copy number losses involving CDKN2A and CDKN2B. Genome-wide DNA methylation analysis was performed which revealed copy number losses of CDKN2A/B and gains of PTCH1 and MDM2. However, the Sarcoma DNA Methylation Classifier did not provide any diagnostic matches. Overall, the morphologic features, loss of H3K27me3 expression, presence of NF1 and TP53 mutations, and copy number losses in CDKN2A/B supported a diagnosis of high-grade MPNST. The patient has been followed clinically without adjuvant therapy. Results (if a Case Study enter NA) N/A Conclusion We report an extremely rare case of MPNST of the pancreas with molecular confirmation.

Evaluation of vascular invasion in pancreatic head tumors and its impact on radical surgical treatment

Objective. To evaluate the informativeness and sensitivity of radiological criteria for vascular invasion in pancreatic head tumors in comparison with intraoperative data and results of routine pathological examination. Materials and methods. The study included 98 patients with ductal adenocarcinoma of the pancreatic head with suspected venous invasion and spread of tumor growth along the vessels of the portomesenteric axis, who were operated on at the Department of Pancreatic and Bile Duct Surgery of the National Research Center of Surgery and Transplantation named after O. A. Shalimov in the period from 2019 to 2023. Group 1 included 51 (52.0%) patients who underwent radical surgery with resection of the superior mesenteric/hepatic portal vein and extended lymph and neurodissection, and group 2 included 47 (48.0%) patients who had positive vascular margins of pancreatic resection according to routine pathological examination. Diagnostic criteria for vascular alterations were developed. A comparative analysis of the informativeness of the developed criteria and the results of a routine pathological examination was performed. The edges of the pancreatic resection were evaluated, including the neck of the gland, common bile duct, duodenum/gastric duct, and the bed of the superior mesenteric/hepatic portal vein and superior mesenteric artery separately. R1 status was defined as the presence of tumor cells at a distance of less than 1 mm to the resection margin. Results. According to the criteria of the National Comprehensive Cancer Network (NCCN) 2023.2, 61 (62.2%) patients had a marginally resectable stage of ductal adenocarcinoma of the pancreatic head and tumor contact with the superior mesenteric/hepatic veins according to multislice computed tomography of the abdominal cavity. In group 1, there were 36 (70.6%) such patients, in group 2 – 25 (53.2%). According to the proposed radiologic criteria for vascular invasion, tumor contact with the superior mesenteric/hepatic portal veins was suspected in 76 (77.6%) patients, which is higher than in the standard approach. Determination of vascular alterations according to the results of our study is a more reliable method for diagnosing invasion of the superior mesenteric/hepatic portal vein (χ2 = 5.46, p = 0.019) and predicting the performance of radical surgery, including vascular resection. Conclusions. The proposed criteria for the radiological diagnosis of vascular invasion at the preoperative stage increased the sensitivity of this method from 62.2 to 77.6%. The most frequent radiological signs of vascular alteration are vascular wall irregularity (47.7%) and parapancreatic tissue density (62.5%). In the presence of any of the radiological signs of vascular alteration, resection of the affected area of the superior mesenteric/hepatic portal vein with extended lymph and neurodissection is indicated, which allows a statistically significant increase in the number of R0 resections.

Oncological Outcomes of Open Versus Minimally Invasive Surgery for Ductal Adenocarcinomas of Pancreatic Head: A Propensity Score Matching Analysis.

Minimally invasive pancreatic resections (MIPRs) have been shown to be safe and feasible, but there is still a lack of high-level evidence on oncological outcomes for cephalic pancreatic ductal adenocarcinoma (PDAC). The aim of this study was to compare the oncological outcomes of patients undergoing MIPR and open pancreatic resection (OPR) for pancreatic head cancer in a single high-volume center. Data from a prospectively collected database of patients who underwent radical-intent surgery for resectable and borderline resectable PDAC of the head at our institution between January 2013 and May 2023 were retrieved and analyzed, comparing the surgical and oncological outcomes of MIPR and OPR, using a propensity score matching analysis. In the study period, 220 patients were selected. After matching, a total of 81 MIPRs and 81 OPRs were compared. No difference was found regarding R0 rate (OPR 83.9% vs. MIPR 74.1%, p = 0.122). Median overall survival (24 and 31 months for the OPR and MIPR groups, respectively; log rank p = 0.665) and disease-free survival (12 and 21 months for the OPR and MIPR groups, respectively; log rank p = 0.118) did not differ between the groups. The MIPR group was associated with a greater number of harvested lymph nodes (22 vs. 16, p = 0.0008), longer operative time (565 vs. 420 min, p < 0.0001), and shorter length of stay (12 vs. 18 days; p = 0.0001). No differences between the groups were found regarding all other postoperative and pathological outcomes. Regarding oncological outcomes, MIPR appeared to be comparable to OPR for treating patients with PDAC of the head. Despite an increased operative time, MIPR was associated with a greater number of LNs harvested and a shorter length of stay.

Fine Needle Aspiration Cytology of Pancreatoblastoma in Adult/Adolescent Patients, One With Histology Mimicking NUT Carcinoma.

Pancreatoblastoma is a rare malignant neoplasm. Cytologic diagnosis is challenging due to the tumor's heterogeneity and requirement of the presence of squamoid nests. Commonly affects children, but the tumor rarely is seen in adult patients. We are reporting three cases from two patients. First patient was a 38-year-old male with a mass in the pancreatic body and numerous hepatic lesions. Fine-needle aspiration (FNA) of the pancreas showed a biphasic malignancy, predominantly composed of a primitive component with intermingled squamoid nests. Subsequent Liver FNA from the same patient showed a similar biphasic malignancy. NUT carcinoma was the top differential and was ruled out by molecular testing. Second patient was a 24-year-old female with a history of pancreatoblastoma related to Gardner's syndrome initially diagnosed in 2015 at age 17, status post distal pancreatectomy and chemotherapy. Celiac lymph node FNA in 2021 showed few cohesive clusters of atypical epithelioid cells, which were highlighted by beta-catenin. Lastly, the literature was reviewed; differential diagnosis and ancillary testing were discussed.