Tumors Of Low Malignant Potential Research Articles

Clinicopathological characteristics and typing of multilocular cystic renal neoplasm of low malignant potential.

Up until now, no research has been reported on the association between the clinical growth rate of multilocular cystic renal neoplasm of low malignant potential (MCRNLMP) and computed tomography (CT) imaging characteristics. Our study sought to examine the correlation between them, with the objective of distinguishing unique features of MCRNLMP from renal cysts and exploring effective management strategies. To investigate optimal management strategies of MCRNLMP. We retrospectively collected and analyzed data from 1520 patients, comprising 1444 with renal cysts and 76 with MCRNLMP, who underwent renal cyst decompression, radical nephrectomy, or nephron-sparing surgery for renal cystic disease between January 2013 and December 2021 at our institution. Detection of MCRNLMP utilized the Bosniak classification for imaging and the 2016 World Health Organization criteria for clinical pathology. Our meticulous exploration has revealed compelling findings on the occurrence of MCRNLMP. Precisely, it comprises 1.48% of all cases involving simple renal cysts, 5.26% of those with complex renal cysts, and a noteworthy 12.11% of renal tumors coexisting with renal cysts, indicating a statistically significant difference (P = 0.001). Moreover, MCRNLMP constituted a significant 22.37% of the patient population whose cysts demonstrated a rapid growth rate of ≥ 2.0 cm/year, whereas it only represented 0.66% among those with a growth rate below 2.0 cm/year. Of the 76 MCRNLMP cases studied, none of the nine patients who underwent subsequent nephron-sparing surgery or radical nephrectomy following renal cyst decompression experienced recurrence or metastasis. In the remaining 67 patients, who were actively monitored over a 3-year postoperative period, only one showed suspicious recurrence on CT scans. MCRNLMP can be tentatively identified and categorized into three types based on CT scanning and growth rate indicators. In treating MCRNLMP, partial nephrectomy is preferred, while radical nephrectomy should be minimized. After surgery, active monitoring is advisable to prevent unnecessary nephrectomy.

Proton therapy in pediatric neuro-oncology. Potential advantages and the relationship between cost and results

The aim of study: to analyze the accumulated experience and try to identify those clinical situations in which the use of protons will be economically and clinically more effective than photon radiation therapy. Material and methods. The articles devoted to the study of the evaluation and comparison of the effectiveness of proton and photon radiation beams in the treatment of tumors of the central nervous system and published over the past 25 years were searched in the Medline, Embase and the Cochrane Library databases. Results. the analysis of available publications has shown that accelerated protons do not improve survival rates and disease-free rates in all forms of brain tumors compared with photon therapy. However, protons can significantly increase the level of dose distribution conformity and reduce the dose to critical structures (pituitary gland, cochlea, eye lenses, hypothalamus), thus reducing the risk of hearing and visual impairment as well as hormonal and cognitive disorders. All this is critically important for potentially curable malignant tumors, such as medulloblastoma and germinoma, for low malignant potential tumors (grade 1–2 glioma) or tumors with decreased metastatic potential, since proton therapy compared to photon therapy reduces the risk of late side effects that worsen the quality of life of cured children. Conclusion. central nervous system tumors are one of the most common solid malignant neoplasms in children. Radiation therapy (RT) is recognized as an important therapeutic component of treatment and is often used in strategies for multimodal therapy of tumors of the central nervous system in children. Proton radiation therapy is one of the attractive methods of radiotherapy with minimal dose distribution to normal tissues and a decrease in the absorbed dose. The precision of protons reduces the risk of long-term side effects associated with this type of treatment and the induction of secondary malignancies, which is of particular importance for the quality of life.

The Predictive Power of Bosniak 3 and 4 Cystic Renal Lesion Categorization Using Contrast-Enhanced Ultrasound.

Contrast-enhanced ultrasound (CEUS) is increasingly utilized for the noninvasive assessment of renal cystic lesions, using the Bosniak grading system. Bosniak 3-4 lesions require surgical referral, which allows correlation with the histopathological outcome. In this single-center, retrospective study we evaluated renal CEUS exams conducted with SonoVue® with a diagnosis of a Bosniak 3 or 4 lesion between 2019 and 2022. A total of 49 patients and 50 lesions met the inclusion criteria, 31 lesions had available histopathological results. Patient demographics, cyst morphology, and dominant imaging features were registered. The histopathological diagnosis was considered a reference standard. Positive predictive power (PPV) for neoplastic lesions was comparable in the Bosniak 3 and 4 categories (75 vs 93.3%, P = .33), while PPV for histopathologically malignant lesion was considerably higher in the latter group (25 vs 93.33%, P = .0002). None of the lesions which had vividly enhancing thin septa as their dominant CEUS feature were malignant. Oncocytoma, multilocular cystic renal neoplasm of low malignant potential, and cystic nephroma were the major benign entities among Bosniak 3 lesions. Localized cystic kidney disease and hemorrhagic cysts were found to be the primary mimickers leading to false positive imaging findings. CEUS has a high predictive power for malignancy in the Bosniak 4 category, which is not maintained in the Bosniak 3 group due to the large proportion of benign lesions. Adherence to rigorous rule-in criteria and active surveillance strategies need to be considered for equivocal CEUS Bosniak 3 lesions.

Cystic Features in Renal Epithelial Neoplasms and Their Increasing Clinical and Pathologic Significance.

Most cystic renal tumors after resection (Boniak IIF to IV cysts) have an indolent course despite the significantly higher proportion of malignant [ie, renal cell carcinoma (RCC)] diagnosis. Most cystic renal tumors have clear cell histology that include cystic clear cell RCC and multilocular cystic renal neoplasm of low malignant potential (MCNLMP). There is growing evidence to suggest that MCNLMP, cystic clear cell RCC, and noncystic clear cell RCC form a cystic-to-solid biological spectrum with MCNLMP representing the most indolent form and with cystic clear cell RCC behaving better than noncystic (solid) clear cell RCC. Extensively (>75%) cystic clear cell RCC also has an excellent outcome similar to MCNLMP stressing the need to reevaluate the histologic criteria that separate these 2 cystic clear cell tumors. Other tumors with clear cells that can be extensively cystic such as the recently reclassified noncancerous clear cell papillary renal tumor and the newly described MED15::TFE3 RCC also have indolent course and may mimic MCNLMP. Cystic features occur also in renal tumors with nonclear cell histology including tumors capable of metastasis such as acquired cystic disease-associated, tubulocystic, fumarate hydratase-deficient, and eosinophilic solid and cystic RCCs. Cystic imaging presentation of some renal tumors such as papillary RCC can be attributed in part to pseudocystic necrosis and hemorrhage. It is important to know that tubulocystic RCC may have a lower Bosniak class presentation that overlaps with benign renal cysts (Bosniak I to IIF) that are managed conservatively. This review highlights the cystic renal tumors with clear cell and nonclear cell morphologies including some novel RCC subtypes that may have cystic features. The presence of cystic features and their extent may aid in the classification and prognostication of renal neoplasms underscoring its increasing importance in the pathologic diagnosis and reporting of renal neoplasia.

Alpha-methyl CoA racemase (AMACR) reactivity across the spectrum of clear cell renal cell neoplasms

a-Methylacyl coenzyme A racemase (AMACR) is traditionally considered to be a marker of papillary renal cell carcinoma. However, AMACR expression can be seen in other renal tumors. The aim of this study was to investigate AMACR immunoreactivity within the spectrum of clear cell renal cell neoplasms.Fifty-three clear cell renal epithelial tumors were used in assembling the following four cohorts: low grade (LG) clear cell renal cell carcinoma (CCRCC), high grade (HG) CCRCC, CCRCC with cystic changes, and multilocular cystic renal neoplasm of low malignant potential (MCRNLMP). Representative blocks were stained for AMACR, using two different clones (SP52 and OV-TL12/30).There were at least some AMACR immunoreactivity in 77.8 % and 68.9 % of CCRCCs (using SP52 and OV-TL12/30 clone, respectively). Moderate to strong positivity, or positivity in more than one third of the tumor (even weak in intensity) was detected in 46.7 % of CCRCCs using SP52 and in 48.9 % of CCRCC using OV-TL12/30 clone. The highest AMACR reactivity was observed in HG CCRCC (60 % by SP52 and 66.7 % by OV-TL12/30). Strong and diffuse AMACR positivity was detected in 8.9 % of all CCRCCs. AMACR immunoreactivity in MCRNLMP was 37.5 % (SP52 clone) and 25 % (OV-TL12/30 clone).We demonstrated relatively high expression rate of AMACR in CCRCC, while very variable in intensity and distribution. This finding may have diagnostic implications especially in limited samples (i.e., core biopsies), as AMACR positivity does not exclude the diagnosis of CCRCC.

Central versus distal pancreatectomy for low-grade and benign pancreatic neck-body tumours.

Central pancreatectomy (CP) offers parenchymal preservation compared to conventional distal pancreato-splenectomy for pancreatic neck and body tumours. However, it is associated with more morbidity. This study is aimed at evaluating the peri-operative and long-term functional outcomes, comparing central and distal pancreatectomies (DPs). Retrospective analysis of patients undergoing pancreatic resections for low-grade malignant or benign tumours in pancreatic neck and body was performed (from January 2007 to December 2022). Preoperative imaging was reviewed for all cases, and only patients with uninvolved pancreatic tail, whereby a CP was feasible, were included. Peri-operative outcomes and long-term functional outcomes were compared between CP and DP. One hundred twenty-two (5.2%) patients, amongst the total of 2304 pancreatic resections, underwent central or distal pancreatectomy for low-grade malignant or benign tumours. CP was feasible in 55 cases, of which 23 (42%) actually underwent CP and the remaining 32 (58%) underwent DP. CP group had a significantly longer operative time [370min (IQR 300-480) versus 300min (IQR 240-360); p = 0.002]; however, the major morbidity (43.5% versus 37.5%; p = 0.655) and median hospital stay (10 versus 11days; p = 0.312) were comparable. The long-term endocrine functional outcome was favourable for the CP group [endocrine insufficiency rate was 13.6% in central versus 42.8% in distal (p = 0.046)]. Central pancreatectomy offers better long-term endocrine function without any increased morbidity in low malignant potential or benign pancreatic tumours of neck and body region.

MED15::TFE3 fusion renal cell carcinoma with extensive cystic change: A clinicopathologic and molecular genetic study of 2 cases, with an emphasis on differential diagnosis.

TFE3-rearranged renal cell carcinomas (RCCs) harbor gene fusions between TFE3 and 1 of many partner genes. MED15::TFE3 fusion RCC is rare, often cystic, and easily misdiagnosed. This study aimed to characterize 2 cases of MED15::TFE3 fusion RCC with extensive cystic change using fluorescence in situ hybridization and targeted RNA sequencing. Both patients were young adult women aged 29 and 35 years. Radiologically, both presented with a cystic Bosniak category II renal lesion. The cysts measured 9.3cm and 4.8cm in greatest dimension. Both patients underwent cyst enucleation, and neither had tumor recurrence or metastasis at 26 and 6 months of follow-up, respectively. Microscopically, both tumors were entirely cystic, with thick, fibrous cystic walls lined by small clusters of cells with clear to eosinophilic cytoplasm and uniform, round nuclei with inconspicuous nucleoli. There were also small aggregations of similar clear cells within the cystic walls. Foci of basement membrane-like material depositions were noted in 1 case; calcifications were observed in both cases. Both cases demonstrated nuclear positivity for PAX8 and TFE3 and cytoplasmic staining for Melan-A; HMB45, CAIX, and CK7 were negative. Fluorescence in situ hybridization revealed that both tumors were positive for TFE3 rearrangements. RNA sequencing identified MED15::TFE3 gene fusions in both cases. The main differential diagnosis of MED15::TFE3 fusion RCC includes multilocular cystic renal neoplasm of low malignant potential and atypical renal cysts. Molecular confirmation of TFE3 fusion is essential for establishing the correct diagnosis.

P-02 A case of solitary fibrous tumor originating from the adrenal gland presenting with hypoglycemia

Abstract Introduction Solitary fibrous tumor (SFT) is a rare mesenchymal neoplasm usually located in the pleura and associated with NAB2-STAT6 gene fusion. According to the World Health Organization (WHO) classification, although most SFTs are considered well benign, some SFTs have cytological atypia, hypercellularity, tumor necrosis, Ki-67 greater than 5%, high mitotic rate (>4 per high-power field (HPF)) and/or the presence of infiltrative surgical margins are considered malignant. SFT with severe hypoglycemia is called Doege-Potter syndrome which occurs with ectopic secretion of a prohormone of insulin-like growth factor 2 (IGF-2) from tumor tissue. We present a case who applied with severe hypoglycemia and was diagnosed with a solitary fibrous tumor thought to originate from the adrenal gland located in the right retroperitoneal region. Clinical Case A 61-year-old female patient was admitted to the hospital after hypoglycemia was detected in her application due to clouding of consciousness. While the patient's glucose level was 23 mg/dL, insulin:0.4 µIU/mL, C-peptide:<0.01 ng/mL, serum cortisole: 24,20 (µg/mL), growth hormone (GH): 13,20 ng/ mL and insulin-like growth factor I (IGF-1) 38 ng/mL were measured. Insulinoma was excluded and because of IGF-2 level could not be measured in our hospital, low serum insulin and IGF-1 levels suggested high serum IGF-2 levels. HbA1c, adrenal gland, and pituitary function tests were normal (Table 1). A mass lesion of approximately 145×133 mm in size, with heterogeneous contrast, containing cystic areas, was seen, which was thought to originate from the right adrenal gland in the upper abdomen tomography taken due to the presence of a palpable abdominal mass on physical examination (Figure 1). Urine catecholamines were found to be normal, and the plasma aldosterone concentration (PAC)/plasma renin activity (PRA) ratio was 2.56. The patient was operated under intravenous infusion of 10% dextrose due to persistent hypoglycemia. The immunohistochemical evaluation showed CD34, SOX-1 and STAT6 positivity and negativity for S-100, HMB45, straight muscle actin, desmin, caldesmon and melan A, thus confirming the diagnosis of a solitary fibrous tumor. After the operation, the patient's hypoglycemia disappeared. Since there are cases in the literature in which Doege-Potter syndrome was diagnosed without measuring IGF-2, we think that our patient had IGF-2-related hypoglycemia. Conclusion The development of hypoglycemia in Doege-Potter syndrome has been associated with the secretion of large amounts of IGF-2 by tumor tissue, resulting in decreased IGF-1 and Insulin-like growth factor-binding protein 3 (IGFBP-3) concentrations. In these patients, the tumor originates from mesenchymal tumors of low malignant potential. In our case, the Ki-67 proliferation index was around 2%, it was considered benign, and the patient's symptoms completely regressed after resection.Figure 1.CT images of the patientA mass in the right retroperitoneal area, associated with the adrenal gland and containing areas of necrosis, is seen on non-contrast (a) and contrast-enhanced (b) CT scans. Table 1.Laboratory workup

Urachal mucinous cystic tumor of low malignant potential: a report of a rare case with literature review

Neoplasms originating from the urachus are exceptionally uncommon, comprising only 0.17% of all bladder neoplasms. Among these, the mucinous cystic tumor of low malignant potential (MCTLMP) is particularly rare, with only 25 documented cases in medical literature. Despite their rarity, it is essential to identify MCTLMPs given the possibility of severe complications. Fortunately, surgical removal offers promising cure rates. In this report, A 40-year-old female patient presented to the hospital with abdominal pain. Subsequently, a CT scan showed a 9-cm cystic mass pressing the bladder. The lesion was surgically excised and histopathological examination was performed. Grossly, the specimen consisted of a cyst with a smooth external surface and mucinous content. Microscopically, the cyst was lined by columnar mucinous epithelial cells that included basally-located hyperchromatic nuclei and scattered Goblet cells consistent with an intestinal phenotype. The epithelial cells show mild atypia with focal papillary formations and flattening due to compression. Immunohistochemically, the epithelial tumor cells expressed cytokeratin 20, CDX2, beta-catenin (membranous); and were negative for cytokeratin 7. The location, histopathological, and immunohistochemical findings were consistent with a diagnosis of MCTLMP. In this report, we present an instance of urachal MCTLMP, which represents less than 30 cases documented in the medical literature. This case study marks the importance of early identification of MCTLMP due to their potential for severe complications, despite the tumor’s low malignant potential and to exclude more aggressive tumors with areas of intraepithelial or invasive carcinoma.

Endometriosis and epithelial ovarian cancer: a two-sample Mendelian randomization analysis

Endometriosis, a prevalent condition, has long been recognized as a chronic and debilitating ailment affecting an estimated 1790 million women worldwide. Observational studies have established a correlation between endometriosis and ovarian cancer. Thus, we endeavored to employ Two-Sample Mendelian Randomization, utilizing summary statistics from a Genome-Wide Association Study of endometriosis and epithelial ovarian cancer, with genetic markers serving as proxies for epithelial ovarian cancer. The analysis revealed a significant correlation between these entities, with an odds ratio (OR) of 1.23 (95% CI 1.11–1.36). Upon histotype-specific examination, robust evidence emerged for an association of endometriosis with the risk of endometrioid carcinoma (OR 1.49, 95% CI 1.24–1.81), clear cell carcinoma (OR 2.56, 95% CI 1.75–3.73), and low malignant potential tumors (OR 1.28, 95% CI 1.08–1.53). These findings provide a theoretical framework for prospective investigations aimed at enhancing the potential therapeutic efficacy of managing endometriosis in averting the onset and progression of ovarian cancer.

Benign urinary bladder masses: rare entities

BackgroundThis study aimed to present the characteristics and outcomes of benign urinary bladder masses, as well as the characteristics of the patients diagnosed with such lesions.MethodsA single-center, cross-sectional, retrospective study was conducted. The study involved patients who underwent transurethral resection of the primary bladder tumor over a four-year period (May 2017–2021) and were subsequently diagnosed with a benign bladder lesion.ResultsOut of 478 patients who underwent transurethral resection of the primary bladder tumor, 26 (5.4%) were diagnosed with a benign bladder lesion. The most common benign bladder lesion was urothelial papilloma (50%, 13 patients). The majority of patients with urothelial papilloma were men (76.9%) and had a history of smoking (61.5%). The mean age was 62 years. Most were diagnosed accidentally (69.2%), while others presented with hematuria (23.1%) or dysuria (7.7%). Most urothelial papillomas had a macroscopic polypoid appearance (61.5%), and most of them were solitary (84.6%), with a mean size amounting to 1 cm. Only one patient experienced a recurrence and developed papillary urothelial neoplasm of low malignant potential (PUNLMP). Cystitis cystica/glandularis and polypoid cystitis were the second most commonly diagnosed benign bladder lesion, each identified in 3 patients. Singular cases of leiomyoma, inflammatory myofibroblastic tumor, chondroma, paraganglioma, villous adenoma, eosinophilic cystitis (pseudotumor), and ectopic prostatic tissue are described.ConclusionBenign bladder lesions constitute a group of various rare entities that can clinically and radiologically mimic urothelial carcinoma, but mostly show a good prognosis and a low incidence of recurrence.

Algorithm for the provision of comprehensive psychotherapeutic support to women experiencing neuropsychiatric symptoms during rehabilitation following the treatment of malignant neoplasms of the reproductive system

Background. Radical treatment of malignant neoplasms (MNs) of female reproductive system often has serious consequences. Chronic pain syndrome, distress, anxiety and depression, decreased self-esteem, sexual dysfunction and social maladjustment reduce quality of life (QoL) and require psychotherapeutic support.Objective: to create an algorithm for the provision of comprehensive psychotherapeutic support during rehabilitation following the treatment of reproductive system MNs and to evaluate its effect on QoL indicators in women with neuropsychiatric disorders.Material and methods. The algorithm for the provision of comprehensive psychotherapeutic support included clinical interview, cognitive behavioral therapy, visualization, audio therapy, art therapy, group and individual psychotherapy, family psychotherapy, image therapy. The QoL indicators were determined by questionnaires using the Functional Assessment of Cancer Therapy – General (FACT-G) with nosology-specific extensions (subscales of physical well-being, social/family well-being, emotional wellbeing and functional well-being), Hospital Anxiety and Depression Scale (HADS), Kupperman–Uvarova Modified Menopausal Index (MMI). The follow-up period was 1 year after radical surgical treatment of reproductive system MNs in different localizations. The main group received the algorithm for the provision of comprehensive psychotherapeutic support as part of active medical rehabilitation, the comparison group received basic rehabilitation.Results. The study included 47 women with vulvar cancer (VC): active rehabilitation group (VC-1) – 24 patients and basic rehabilitation group (VC-2) – 23 patients; 61 women with endometrial cancer (EC): active rehabilitation group (EC-1) – 29 patients and basic rehabilitation group (EC-2) – 32 patients; 103 women with cervical cancer (CC): active rehabilitation group (CC-1) – 51 patients and basic rehabilitation group (CC-2) – 52 patients; 62 women with stages I–II ovarian cancer (OC) and low malignant potential tumor (LMPT): active rehabilitation group (LMPT-1) – 29 patients and basic rehabilitation group (LMPT-2) – 33 patients. Differences in physical, social/family and functional well-being (FACT-G) were significant from the 6th month and in emotional well-being – by the end of follow-up. Neurovegetative and psycho-emotional disorders, as assessed by Kupperman–Uvarova MMI, showed significant positive dynamics in women who received the algorithm for the provision of comprehensive psychotherapeutic support, in contrast to the comparison group, where these indicators remained without significant dynamics or worsened by the end of the follow-up period. The HADS scores during psychotherapeutic support decreased to normal values at the 6–12th months of follow-up, while those who received the basic rehabilitation continued to have subclinical anxiety and depression.Conclusion. The developed algorithm for psychotherapeutic support as part of active medical rehabilitation of women who underwent radical treatment for reproductive system MNs (VC, EC, CC, OC, and LMPT) has demonstrated its effectiveness, justifuing its implementation in clinical practice.

Bladder Neoplasia in Pediatric Patients-A Single-Center Experience Including a Case Series.

Objective: Bladder lesions like urothelial carcinoma are rare in the first two decades of life. A biopsy of the bladder or urinary cytological examination is seldom required. Gross painless hematuria is the most relevant clinical syndrome. Methods: A retrospective analysis of surgical pathology records collected between 1984 and 2014 at our institution was performed in a search for cases of urothelial neoplasms originating within the urinary bladder in pediatric patients. Diagnoses were confirmed based on pathologic examination using the 2004 World Health Organization (WHO) classification system. We selected keywords such as bladder neoplasia, bladder lesion, urothelial neoplasia, rhabdomyosarcoma, and children. In addition, we describe clinical presentation and diagnostic procedures as well as treatment and follow-up of two patients. A review of the literature was performed to analyze recommendations concerning diagnostic staging, treatment, and follow-up examinations as well as surveillance of urothelial tumors in the pediatric population. Results: Screening the pathology database of the Institute of Medical Genetics and Pathology of the University Hospital Basel between 1988 and 2014 yielded 287 samples involving the urinary bladder, 110 autopsies, 135 biopsies, and 42 cytology specimens. Of these, most samples originated from malformations and inflammation. Only five were tumors: two were urothelial tumors and three were rhabdomyosarcomas. The majority of specimens comprised resections of the diverticula or distal ureter. Our case reports include two patients with a urothelial tumor. Among the urothelial tumors, one was a papillary urothelial neoplasm of low malignant potential (PUNLMP). Painless hematuria was the directing clinical symptom. The tumor was investigated by FISH, and a 9p21 deletion was found. The second tumor-like lesion was a fibroepithelial polyp arising from the bladder neck. Conclusions: Bladder tumors in children are rare and mostly consist of urothelial and mesenchymal neoplasms. Rhabdomyosarcoma is the most common malignant bladder tumor in childhood. Similar to adult urothelial neoplasms, the loss of 9p21 is also implicated in urothelial neoplasms in childhood. Despite an increasing number of case reports and small series published within the last 2 decades, general treatment protocols including recommendations for staging, tumor markers, and follow-up examinations are still not yet available for this tumor entity in the pediatric population.

Multilocular Cystic Renal Neoplasm of Low Malignant Potential in a Patient with Chronic Pyelonephritis and End-Stage Renal Disease: A Rare Case Report

AbstractMultilocular cystic renal neoplasm of low malignant potential (MCNLMP) is a rare subtype of clear cell carcinoma having an excellent prognosis. These tumors exhibit distinct morphology and comprises of multiple cysts separated by thin septa lined by clear cells with no expansile growth. These cysts, though rare in a setting of chronic pyelonephritis and end-stage renal disease, are usually diagnosed on radiography using the Bosniak classification. But in certain situations where cysts are not visible radiologically, a final diagnosis is made with the help of histopathology and immunohistochemistry only. We, hereby, report such a rare incidental case of MCNLMP diagnosed on histopathology in a nephrectomy specimen of an elderly male with chronic pyelonephritis and end-stage renal disease.

Recurrence of composite hemangioendothelioma of the kidney after surgical resection

Composite hemangioendothelioma is an extremely rare form of kidney tumor. The tumor mainly occurs in the extremities, head and neck; internal organs involvement is rarely reported. Patient, 61-year-old male, was admitted to the urology department for a left kidney tumor, which was found accidentally during an ultrasound examination. Magnetic resonance imaging showed an irregularly shaped tumor measuring 5.0 × 6.0 × 4.0 cm and located in the lower pole of the left kidney. The tumor was surgically removed with resection of the capsule of the kidney lower pole. Immunohistochemical study revealed diffuse bright expression of CD31 (clone JC70A), CD34 (clone QBEnd 10), ERG (clone ER111), FLI-1 (clone MRQ-1) in tumor cells. The index of proliferative activity Ki-67 (clone SP6) was 40 %. The morphological picture and immunophenotype of the tumor correspond to composite hemangioendothelioma of the retroperitoneal space. Magnetic resonance imaging of the retroperitoneal space on follow-up visit in 9 months visualized a tumor of the left kidney measuring 8.3 × 8.4 × 7.8 cm. Radical nephrectomy was performed. Pathology examination showed that tumor tissue was mainly represented by solid fields of the spindle cell component. In samples of the border between the tumor and fatty pararenal tissue, tumor invasion was observed up to the adjacent striated muscles, tumor growth into the tissue of the kidney gate was also found. Taking into account the morphological picture and the earlier immunohistochemical study, the removed tumor corresponds to composite hemangioendothelioma.Composite hemangioendothelioma is a tumor of low malignant potential. It is extremely rare for this tumor to affect the kidney. At the same time, in the described case, the tumor was initially located in the retroperitoneal space, with involvement of the kidney capsule, and was assessed as a benign lesion. After 9 months, there was a recurrence of the tumor localized in the kidney with damage to the elements of the renal sinus, retroperitoneal tissue, and lumbar muscles. In this case, the tumor has significant malignant potential.

A retrospective study on expression and clinical significance of PHH3, Ki67 and P53 in bladder exophytic papillary urothelial neoplasms.

Exophytic papillary urothelial neoplasms (EPUN) are difficult to diagnose pathologically and are well-known for their heterogeneous prognoses. Thus, searching for an objective and accurate diagnostic marker is of great clinical value in improving the outcomes of EPUN patients. PHH3 was reported to be expressed explicitly in the mitotic phase of the cell cycle, and recent studies have shown that PHH3 expression was associated with the differential diagnosis and prognosis of many tumors. However, its significance in EPUN remains unclear. This study aimed to determine the expression of PHH3 in different EPUN, compare its expression with cell-cycle related proteins Ki67 and P53, and analyze its significance in the differential diagnosis and prognostic value for high-grade papillary urothelial carcinoma (HGPUC), low-grade papillary urothelial carcinoma (LGPUC), papillary urothelial neoplasm of low malignant potential (PUNLMP) and urothelial papilloma (UP). We retrospectively analyzed the pathological diagnosis and clinical features of 26 HGPUC cases, 43 LGPUC cases, 21 PUNLMP cases and 11 UP cases. PHH3, Ki67 and P53 were detected by immunohistochemistry in 101 EPUN cases samples. The cut-off values of PHH3 mitosis count (PHMC), HE mitosis count (HEMC), Ki67 and P53 in the different EPUN were determined using the ROC curve. The distribution of counts in each group and its relationship with clinical parameters and prognosis of EPUN patients were also analyzed. The determination coefficient (R2=0.9980) of PHMC were more potent than those of HEMC (R2=0.9734) in the EPUN mitotic counts microscopically by both pathologists. Of the 101 EPUN cases investigated, significant positive linear correlations were found between PHMC and HEMC, PHMC and Ki67, and HEMC and Ki67 (P<0.0001). In HGPUC, LGPUC, PUNLMP and UP, a decreasing trend was observed in the median and range of PHMC/10HPFs, HEMC/10HPFs, Ki67 (%) and P53 (%). PHMC, HEMC, Ki67 and P53 were associated with different clinical parameters of EPUN. PHMC, HEMC, Ki67 and P53 were found to exhibit substantial diagnostic values among different EPUN and tumor recurrence. Based on the ROC curve, when PHMC was >48.5/10HPFs, a diagnosis of HGPUC was more likely, and when PHMC was >13.5/10HPFs, LGPUC was more likely. In addition, when PHMC was >5.5/10HPFs, the possibility of non-infiltrating LGPUC was greater. Kaplan-Meier survival curve analysis showed that the median recurrence-free survival (RFS) for cases with PHMC > 13.5/10HPFs and HEMC > 14.5/10HPFs were 52.5 and 48 months, respectively, and their respective hazard ratio was significantly higher (Log-rank P<0.05). PHH3 exhibited high specificity and sensitivity in diagnosing EPUN. Combined with HEMC, Ki67 and P53, it can assist in the differential diagnosis of EPUN and estimate its clinical progression with high predictive value to a certain extent.

Clinicopathologic Features of Noninvasive Inverted Urothelial Papillary Tumor.

Clinicopathologic features and recurrence rates of inverted noninvasive urothelial papillary tumors have been poorly characterized to date with few larger studies evaluating long-term outcomes. The spectrum of histomorphology, clinical features, and prognosis of inverted lesions of the urinary bladder are retrospectively reviewed. Archived paraffin-embedded urothelial tumor samples from patients diagnosed with inverted urothelial papillary lesions between January 2005 and June 2020 were collated. A matched control population of patients with exophytic papillary lesions of the urothelium diagnosed during the same time period was randomly selected. The conventional clinicopathologic features of inverted urothelial papillary tumor were evaluated retrospectively and patient demographics, tumor characteristics, recurrence, and survival information were recorded. Lower recurrence rates were observed for inverted papillary urothelial neoplasm of low malignant potential (IPUNLMP) relative to papillary urothelial neoplasms of low malignant potential and for low-grade papillary urothelial carcinoma with an inverted growth pattern (LG-PUCI) relative to low-grade papillary urothelial carcinomas. No recurrence was found among the inverted urothelial papilloma cases. The 2- and 5-year disease-free survival rates were 100.0% and 85.2% for IPUNLMP patients; 94.4% and 80.4% for papillary urothelial neoplasms of low malignant potential; 89.5% and 82.0% for LG-PUCI; 73.7% and 54.6% for low-grade papillary urothelial carcinoma; 40.0% and 20.0% for high-grade papillary urothelial carcinoma with an inverted growth pattern patients and 26.7% and 26.7% for high-grade papillary urothelial carcinoma. Multivariate Cox regression analysis of IPUNLMP and LG-PUCI/high-grade papillary urothelial carcinoma with an inverted growth pattern indicated that tumor number (hazard ratio=4.356; 95% CI: 1.145-16.570; P =0.031) was a powerful prognostic factor for disease-free survival. Noninvasive, papillary urothelial lesions of the bladder tend to have lower recurrence and a better outcome if an inverted growth pattern is shown.

Cystic Clear Cell Renal Cell Carcinoma: A Morphological and Molecular Reappraisal.

A wide variety of renal neoplasms can have cystic areas. These can occur for different reasons: some tumors have an intrinsic cystic architecture, while others exhibit pseudocystic degeneration of necrotic foci or they have cystically dilated renal tubules constrained by stromal neoplastic cells. Clear cell renal cell carcinoma (CCRCC), either solid or cystic, is the most frequent type of renal cancer. While pseudocysts are found in high-grade aggressive CCRCC, cystic growth is associated with low-grade indolent cases. The latter also form through a cyst-dependent molecular pathway, and they are more frequent in patients suffering from VHL disease. The differential diagnosis of multilocular cystic renal neoplasm of low malignant potential and clear cell papillary renal cell tumor can be especially hard and requires a focused macroscopical and microscopical pathological analysis. As every class of renal tumor includes cystic forms, knowledge of the criteria required for a differential diagnosis is mandatory.

CT and MRI findings of primary hepatic giant cell tumor of soft tissue: A case report

AbstractPrimary giant cell tumor of soft tissue (GCT-ST) is a low-grade malignant tumor and uncommon cancer originating from soft tissue. Primary hepatic GCT is an extremely rare finding. Only a handful of cases have been reported in the literature. Although GCT-ST has a relatively low malignant potential tumor in the view of histology, some patients who suffered from this tumor have poor prognosis after surgery because of the aggressiveness. Thus, the early diagnosis is important, and a preoperative imaging can supply the evidence of differential diagnosis to evaluate tumor malignancy. In this study, we systematically present a rare case of 56-year-old women with chills, fever, jaundice and anorexia for 3 days. CT showed a huge and multinodular confluent lesion in the left lobe of the liver, and it had a calcification component. This tumor is characterized by fibrous components and calcification, which showed iso-intense with peripheral hypo-intense on T1WI, and mainly inhomogeneous hypo-intense on fat-suppressed T2WI. Moreover, it was mainly hypointense and peripheral linear hyperintense on DWI images with a b-value of 800 (b800) and iso-hyperintense on ADC.

International Society of Urological Pathology (ISUP) Consensus Conference on Current Issues in Bladder Cancer. Working Group 1: Comparison of Bladder Cancer Grading System Performance.

Grade is a key prognostic factor in determining progression in nonmuscle invasive papillary urothelial carcinomas. The 2 most common grading methods in use worldwide are the World Health Organization (WHO) 2004 and 1973 schemes. The International Society of Urological Pathology (ISUP) organized the 2022 consensus conference in Basel, Switzerland on current issues in bladder cancer and tasked working group 1 to make recommendations for future iterations of bladder cancer grading. For this purpose, the ISUP developed in collaboration with the European Association of Urology a 10-question survey for their memberships to understand the current use of grading schemes by pathologists and urologists and to ascertain the areas of potential improvements. An additional survey was circulated to the ISUP membership for their opinion on interobserver variability in grading, reporting of urine cytology, and challenges encountered in grade assignment. Comprehensive literature reviews were performed on bladder cancer grading prognosis and interobserver variability along with The Paris System for urine cytology. There are notable differences in practice patterns between North American and European pathologists in terms of used grading scheme and diagnosis of papillary urothelial neoplasm of low malignant potential. Areas of common ground include difficulty in grade assignment, a desire to improve grading criteria, and a move towards subclassifying high-grade urothelial carcinomas. The surveys and in-person voting demonstrated a strong preference to refine current grading into a 3-tier scheme with the division of WHO 2004 high grade into clinically relevant categories. More variable opinions were voiced regarding the use of papillary urothelial carcinoma with low malignant potential.