Reproducibility of a semiautomatic lobar lung tissue assignment technique on noncontrast CT scans: a study on swine animal model.

BACKGROUND: To evaluate the reproducibility of a vessel-specific minimum cost path (MCP) technique used for lobar segmentation on noncontrast computed tomography (CT). METHODS: Sixteen Yorkshire swine (49.9 ± 4.7 kg, mean ± standard deviation) underwent a total of 46 noncontrast helical CT scans from November 2020 to May 2022 using a 320-slice scanner. A semiautomatic algorithm was employed by three readers to segment the lung tissue and pulmonary arterial tree. The centerline of the arterial tree was extracted and partitioned into six subtrees for lobar assignment. The MCP technique was implemented to assign lobar territories by assigning lung tissue voxels to the nearest arterial tree segment. MCP-derived lobar mass and volume were then compared between two acquisitions, using linear regression, root mean square error (RMSE), and paired sample t-tests. An interobserver and intraobserver analysis of the lobar measurements was also performed. RESULTS: The average whole lung mass and volume was 663.7 ± 103.7 g and 1,444.22 ± 309.1 mL, respectively. The lobar mass measurements from the initial (MLobe1) and subsequent (MLobe2) acquisitions were correlated by MLobe1 = 0.99 MLobe2 + 1.76 (r = 0.99, p = 0.120, RMSE = 7.99 g). The lobar volume measurements from the initial (VLobe1) and subsequent (VLobe2) acquisitions were correlated by VLobe1 = 0.98VLobe2 + 2.66 (r = 0.99, p = 0.160, RSME = 15.26 mL). CONCLUSIONS: The lobar mass and volume measurements showed excellent reproducibility through a vessel-specific assignment technique. This technique may serve for automated lung lobar segmentation, facilitating clinical regional pulmonary analysis. RELEVANCE STATEMENT: Assessment of lobar mass or volume in the lung lobes using noncontrast CT may allow for efficient region-specific treatment strategies for diseases such as pulmonary embolism and chronic thromboembolic pulmonary hypertension. KEY POINTS: • Lobar segmentation is essential for precise disease assessment and treatment planning. • Current methods for segmentation using fissure lines are problematic. • The minimum-cost-path technique here is proposed and a swine model showed excellent reproducibility for lobar mass measurements. • Interobserver agreement was excellent, with intraclass correlation coefficients greater than 0.90.

Experiences with intestinal failure from an intestinal rehabilitation unit in a country without home parenteral nutrition.

OBJECTIVE: For the first time, we reported experiences with an intestinal rehabilitation unit (IRU) from a country without home parenteral nutrition (HPN). METHODS: We included patients with a diagnosis of intestinal failure (IF) since the establishment of our IRU from February 2018 to February 2020. We further report on our protocols for management of enterocutaneous fistulas (ECFs), short-bowel syndrome (SBS), chronic intestinal pseudo-obstruction and motility disorders. RESULTS: Among a total of 349 patients who have been admitted, 100 patients had IF and were included . Mean (SD) age of patients was 46.3 ± 16.1 years. Most common cause of IF was ECFs (32%), SBS (24%), and SBS + fistula (22%). Most common causes of SBS were mesenteric ischemia (63.3%) and repeated surgery (22.4%). Median (interquartile range [IQR]) duration of parenteral nutrition (PN) for patients was 32 (18-60) days. The most common reconstructive surgery performed was resection and anastomosis (75.4%), followed by serial transverse enteroplasty procedure (10.5%) and closure of ostoma (7%). Patients were hospitalized for a median (IQR) of 33 (17.5-61) days. Most common complications were sepsis (45%), catheter infections (43%), and catheter thrombosis (20%), respectively. At the final follow-up, 61% stopped receiving PN, 23% became candidates for transplantation, and 16% died. CONCLUSION: Considering that most countries lack facilities for HPN, by establishing IRUs using specific treatment protocols and autologous gastrointestinal reconstruction techniques will provide a means to manage patients with IF, thus decreasing death rates and number of patients who require intestinal transplantations due to IF.

Primary renal non-Hodgkin's lymphoma: A narrative review of literature.

Renal involvement by Non-Hodgkin's lymphoma (NHL) is very rare, and involvement of the kidney as the primary site of NHL (PRNHL) is much more uncommon. Gold standard for the diagnosis of PRNHL is histology and imaging modalities although helpful are not specific. Nephrectomy has been mostly recommended for low grade lymphomas, and for high grade PRNHLs, chemotherapy without nephrectomy has been recommended as the treatment of choice. This tumor is aggressive with poor prognosis. This poor prognosis is partly because of delayed diagnosis and partly because of unnecessary surgeries, so it should be kept in mind, especially in bilateral renal tumors with unusual imaging characteristics, to take a tissue biopsy before nephrectomy. In this review, we will discuss all the detailed aspects of clinical, pathologic, and imaging characteristics of 83 cases of PRNHL reported in the last 20 years in the English literature so far. For this purpose, all the published cases of the primary non-Hodgkin's lymphoma of kidney were reviewed via a search in PubMed, Scopus, and Google Scholar, (1999-2019), using the keywords of "Primary renal lymphoma" and "Non-Hodgkin's lymphoma and kidney," "renal Non-Hodgkin's lymphoma," "renal lymphoma," and "lymphoma and kidney." There were 83 cases in the published English literature which were reviewed for this article. There was some missing information in some cases which has been recorded as "not reported."

Gastrointestinal Stromal Tumor of the Ampulla of Vater: A Narrative Review.

BACKGROUND: Gastrointestinal stromal tumor (GIST) of the ampulla of Vater is a rare occurrence. To the best of our knowledge, there has been no published review on this rare tumor in the English literature so far. SUMMARY: In this review, we will discuss all the reported details of the published cases, including demography, clinical presentation, imaging, gross pathology and histopathology, immunohistochemical findings, treatment modalities, and outcome of cases with the diagnosis GIST from the ampulla of Vater in the last 20 years. KEY MESSAGE: Twenty-five cases of GIST in the ampulla of Vater have been reported in the last 20 years in the English literature. GIST in the ampulla of Vater are usually small tumors (<5 cm) in middle-age patients. The majority of the patients present with lower GI bleeding and abdominal pain. Imaging findings are not characteristic, and most of the patients without biopsy and with no histologic diagnosis were operated with the primary impression of adenocarcinoma, neuroendocrine tumor, and GIST. Perioperative tissue biopsy has been accurate in <70% of the cases. The majority of the reported cases of GISTs in the ampulla of Vater have been low risk with spindle-cell morphology, low mitotic figures, and minimal atypia; reactive for C-KIT and DOG-1; and nonreactive for SMA, desmin, and S100. In the majority of the cases, duodenectomy with or without Whipple's operation has been performed, and most of the cases showed good prognosis.

P16 and P53 Expression in Esophageal Squamous Cell Carcinoma: A Brief Report From The Experience of South of Iran, and Review of the Literature.

BACKGROUND: Iran is one of the high-risk countries for esophageal squamous cell carcinoma (ESCC). Human papillomavirus (HPV) has been reported as one of the etiologic, pathogenetic, and prognostic factors in this tumor, especially in high-risk geographic areas. Previous reports from our geographic area, that is, the South of Iran failed to show any evidence of HPV in the cases of ESCC by molecular methods. OBJECTIVES: In this study, we evaluated P16 and P53 immunohistochemistry (IHC) expression in the cases of esophageal ESCC from Fars province in the South of Iran to find the presence of any correlation between clinicopathologic findings with P16 and P53 expression by IHC as etiologic and prognostic biomarkers. We also tried to compare the results from other geographic areas of Iran and the world. RESULTS: P16 and P53 expression were found in 42.9% and 66.12% of ESCCs, respectively. No statistically significant correlation was found between clinicopathologic findings and P16 pr P53 expression. CONCLUSION: Although P16 and P53 expression in ESCC in the South of Iran is significant, there is no statistically significant correlation between clinicopathologic findings and outcome in ESCC and expression of these 2 proteins to be considered as biomarkers. Results from other geographic areas of Iran and the world are also very controversial and inconsistent.

Hepatocellular Malignant Neoplasm, Not Otherwise Specified: A New Name in Liver Tumors: A Brief Narrative Review of Published Cases.

BACKGROUND: Hepatocellular malignant neoplasm, not otherwise specified (HEM, NOS), is a rare liver tumor that is most commonly seen in adolescents. To the best of our knowledge, there has been no published review on this rare tumor in the English literature so far. SUMMARY: In this review, we will discuss all reported details, including demographic findings, clinical presentations, molecular histogenesis, imaging, gross pathology and histopathology, immunohistochemical findings, treatment modalities, and outcome, of the published cases of HEM, NOS, in the liver. KEY MESSAGE: Twenty-two cases of HEM, NOS, have been reported in the last 10 years in the English literature. This tumor produces a large liver mass and is characteristically seen in adolescents presenting with right upper quadrant pain. Histologically, HEM, NOS, is a nonbiliary hepatocytic tumor with a biphasic pattern, that is, a mixture of 2 components of hepatocellular carcinoma and hepatoblastoma (HBL). There is no characteristic immunohistochemical feature for this tumor. Imaging findings are also not specific. Treatment of this tumor needs extensive surgery or even liver transplantation. Most of the cases in the literature were treated with the primary diagnosis of HBL, so pathologists, onco-surgeons, and oncologists should get familiar with this tumor to provide better treatment options. More case series with more follow-ups are necessary for the definite determination of the outcome of this tumor as a unique entity.

Intraductal papillary mucinous neoplasm of the pancreas: Cytomorphology, imaging, molecular profile, and prognosis.

BACKGROUND: Intraductal papillary mucinous neoplasm (IPMN) constitutes up to 20% of all pancreatic resections, and has been increasing in recent years. Histomorphological findings of IPMN are well established; however, there are not many published papers regarding the cytological findings of IPMN on fine needle aspiration (FNA) specimens. We review the cytomorphological features, molecular profile, imaging findings, and prognosis of IPMN. METHODS: The English literature was thoroughly searched with key phrases containing IPMN. OBSERVATIONS: IPMN is a rare entity, affecting men and women equally and is usually diagnosed at the age of 60-70 years. The characteristic imaging features include a cystic lesion with associated dilatation of the main or branch pancreatic duct, and atrophy of surrounding pancreatic parenchyma. Cytomorphological features of IPMN include papillary fragments of mucinous epithelium in a background of abundant thick extracellular mucin, a hallmark feature. IPMNs should be evaluated for high-grade dysplasia, which manifests with nuclear atypia, nuclear moulding, prominent nucleoli, nuclear irregularity, and cellular crowding. Molecular profiling of IPMN along with carcinoembryonic antigen and amylase levels is useful in predicting malignancy or high-grade dysplasia arising in IPMN. Overall, the prognosis of IPMN is excellent except in those cases with high-grade dysplasia and malignant transformation. Postoperative surveillance is required for resected IPMNs. CONCLUSION: IPMN requires a multidisciplinary approach for management. Cytomorphological findings of IPMN on FNA, in conjunction with tumour markers in pancreatic fluid cytology and imaging findings, are of paramount importance in clinical decision-making for IPMN.

Novel mutations in the PHKB gene in an iranian girl with severe liver involvement and glycogen storage disease type IX: a case report and review of literature.

BACKGROUND: Glycogen storage disease (GSD) type IXb is one of the rare variants of GSDs. It is a genetically heterogeneous metabolic disorder due to deficient hepatic phosphorylase kinase activity. Diagnosis of GSD can be difficult because of overlapping manifestations. Mutation analysis of the genes related to each type of GSD is supposed to be problem-solving, however, the presence of novel mutations can be confusing. In this case report, we will describe our experience with a young girl with the diagnosis of GSD and two novel mutations related to GSD type IXb. CASE PRESENTATION: A 3-year- old girl presented with short stature, hepatomegaly, and liver cirrhosis. No specific diagnosis was made based on laboratory data, so liver biopsy and targeted-gene sequencing (TGS) were performed to find out the specific molecular basis of her disease. It was confirmed that the patient carries two novel variants in the PHKB gene. The variant in the PHKB gene was classified as pathogenic. CONCLUSIONS: This is the first reported case of a dual molecular mutation of glycogen storage disease type IXb in the same patient. Two novel variants in PHKB were identified and one of them was a pathogenic split-site mutation. In conclusion, for the first time, identification of the novel variants in this patient expands the molecular and the phenotype basis of dual variants in GSD-IXb.

Novel PRKAG2 variant presenting as liver cirrhosis: report of a family with 2 cases and review of literature.

BACKGROUND: Mutations in the PRKAG2 gene encoding the 5' Adenosine Monophosphate-Activated Protein Kinase (AMPK), specifically in its γ2 regulatory subunit, lead to Glycogen storage disease of heart, fetal congenital disorder (PRKAG2 syndrome). These mutations are rare, and their functional roles have not been fully elucidated. PRKAG2 syndrome is autosomal dominant disorder inherited with full penetrance. It is characterized by the accumulation of glycogen in the heart tissue, which is clinically manifested as hypertrophic cardiomyopathy. There is little knowledge about the characteristics of this disease. This study reports a genetic defect in an Iranian family with liver problems using targeted-gene sequencing. CASE PRESENTATION: A 4-year-old girl presented with short stature, hepatomegaly, and liver cirrhosis. As there was no specific diagnosis made based on the laboratory data and liver biopsy results, targeted-gene sequencing (TGS) was performed to detect the molecular basis of the disease. It was confirmed that this patient carried a novel heterozygous variant in the PRKAG2 gene. The echocardiography was a normal. CONCLUSION: A novel heterozygous variant c.592A > T (p.Met198Leu) expands the mutational spectrum of the PRKAG2 gene in this family. Also, liver damage in patients with PRKAG2 syndrome has never been reported, which deserves further discussion.