Low Malignant Potential Research Articles (Page 10)

Salivary gland-like breast tumors: A review of diagnostic features and prognosis

Pediatric urothelial bladder neoplasm

These cases highlight the importance of monitoring choroidal nevi with benign imaging characteristics and the potential to quantify horizontal growth using optical coherence tomography (OCT), in the absence of color fundus photography. This study aimed to present reports of two patients with pigmented choroidal tumors with low malignant potential based on their multimodal imaging features at the time of referral, but access to prior OCT imaging confirmed horizontal growth consistent with melanoma. Two patients with pigmented, dome-shaped, subfoveal tumors were referred. Both tumors had basal diameters greater than 5 mm but no other risk factor for growth at the time of referral. Screening OCT scans had been taken of each patient's macula more than 5 years before referral, but color fundus photography was not available for either. Repeat OCT scanning at the time of referral showed horizontal growth of the tumors consistent with melanoma. As per the "To Find Small Ocular Melanoma-Do Imaging" risk factor assessment, the 5-year risk of growth of both tumors would be estimated at 11% at the time of referral, and in the absence of the documented horizontal growth on OCT scanning, the patients would have been monitored for growth. After discussion of the risks and benefits, both patients elected for their tumors to be managed as choroidal melanomas and underwent ruthenium plaque brachytherapy. Horizontal growth of choroidal tumors can be established using sequential OCT scans in the absence of color fundus photography. Access to prior imaging can expedite the diagnosis of choroidal melanoma, potentially allowing patients to be treated earlier.

Uterine tumors resembling ovarian sex cord tumors (UTROSCTs) are extremely rare, occurring in less than 1% of uterine stromal tumors, and they are considered to have a low malignant potential. Due to the small number of cases, no standard treatment has been defined. A 77-year-old woman with postmenopausal bleeding was admitted to our department. Imaging studies revealed a substantial mass around 30 mm in size on the anterior uterine wall. A total hysterectomy and bilateral salpingo-oophorectomy were performed for further diagnosis and treatment. The tumor revealed histopathological findings of a sex cord-like growth pattern in the form of fascicles, cords, or small nests. Immunohistochemical findings revealed that the tumor cells were positively reactive to alpha-SMA, calretinin, CD99, estrogen receptor, and progesterone receptor, collectively diagnosed as UTROSCT. No recurrence was observed over 12 months after treatment. We experienced the treatment of UTROSCT, an extremely rare tumor that occurs in elderly women. Although most cases of UTROSCT have a benign clinical course, several cases of recurrence and metastasis have been reported. It should be followed up for a long term after treatment.

4151 Background: One impact of medical imaging technology has been an approximately 3-fold increase in the incidental detection of pancreatic cysts during routine clinical examinations. To reduce burden on the healthcare system and patients, clinicians desire accurate classification of pancreatic cysts into benign non-mucinous or potentially malignant, mucinous populations. Using EUS-FNA, fluid from these cysts can provide molecular biomarkers of predictive value. However, current in vitro diagnostics lack the desired sensitivity and specificity for clinicians to accurately stratify patients for risk of pre-malignant cancers. This situation can be improved by introducing new biomarkers and novel assay platforms. An enzymatic biomarker, pepsin C, has shown high accuracy for diagnosing mucinous cysts. The use of enzymatic activity assays is applicable to clinical workflows without disruption of current standards of care. Methods: A pepsin C activity assay using a magnetic bead-based platform was developed, with both fluorescent and surface-enhanced Raman spectroscopy (SERS) readouts. The assay platform utilizes selective peptide substrates, and a dimeric Rhodamine-6G-based dye, which allows ultrasensitive detection and significantly decreases the sample volume requirement for analysis, down to 1 µL of cyst fluid. The dye-labeled substrate is immobilized on magnetic beads and reacted with enzyme-containing samples to produce a quantitative assay signal that is standardized and expressed in true enzyme activity units. Results: While both readouts were quantitative and produced linear standard curves, SERS-based analysis was more robust against established biological matrix effects than fluorescence. Nevertheless, both assay modes successfully differentiated mucinous and non-mucinous cysts in a retrospective cohort of 69 cyst fluid samples. Compared with the standard of care CEA assay, this activity-based assay displays much improved sensitivity in diagnosing mucinous pancreatic cysts (Table). Conclusions: This pepsin c activity assay differentiates between mucinous and non-mucinous cysts better than the CEA assay and provides a quantifiable standardized readout. This work establishes a path to a true rule-out assay enabling clinicians to better stratify patients into low risk vs. potential malignancy thus impacting treatment and monitoring plans. [Table: see text]

Hepatic epithelioid angiomyolipomas (HEAMLs) are rare and usually arise in case reports, and thus, we aimed to study the clinicopathological features, treatment and prognosis of these lesions. We identified patients from institutional database with HEAMLs and retrospectively collected clinical data. Of 12 patients, 10 were females, and the median age was 49 years. A liver mass was found on chance upon routine health screening in nine patients. Imaging features included clear border (9/12), internal heterogeneity (8/12), arterial enhancement (12/12), fat (5/12), intra-tumour vessel (3/12), draining hepatic vein (2/12) and pseudocapsule (1/12). The preoperative diagnoses contained HEAML (n=5), hepatocellular carcinoma (n=6), and hepatic cystadenocarcinoma (n=1). All cases had received surgery and obtained a negative margin. All specimens showed positive findings for HMB-45 and Melan A. No tumour recurrence or mortality was described with a mean follow-up time of 23.5months. HEAML is a low potential malignancy tumour that frequently appears in middle-aged females. It has nonspecific symptoms and may present special imaging features including intra-tumour vessel, early draining hepatic vein and lack of a pseudo capsule. The lesion is confirmed by pathological and immunohistochemical findings. Surgery and subsequent long-term follow-up are the most appropriate management approaches.

BackgroundMediastinal teratomas are rare tumors that frequently occur in the anterior mediastinum. The majority of these tumors are benign and slow growing. Due to their low malignant potential, the treatment for these tumors is surgical resection. More recently, the surgical management has shifted from invasive approaches such as a sternotomy to minimally invasive ones such as robotic-assisted thoracoscopic resections utilizing lung isolation ventilation. We present a rare case of a locally advanced mediastinal teratoma requiring resection, which was initially attempted thoracoscopically using double lung ventilation.Case presentationA 43 year-old female was found to have an anterior mediastinal mass during work-up for an intermittent cough in 2009. Chest imaging and biopsy at the time showed evidence of a cystic teratoma without concerning features. She underwent imaging surveillance until 2018, when repeat chest imaging showed increasing growth and worrisome radiologic features concerning for malignant degeneration. She underwent an elective robotic-assisted thoracoscopic resection utilizing double lung ventilation, but due to extensive involvement of the right lung, pericardium, superior vena cava, and right phrenic nerve the patient had to be repositioned and started on single lung ventilation mid-procedure to facilitate a safe and complete resection.ConclusionsAnterior mediastinal teratomas can be successfully removed by robotic-assisted thoracoscopic resections utilizing single lung ventilation. Though robotic-assisted thoracoscopic resection utilizing double lung ventilation can be effective in performing certain procedures such as lung wedge resections, thymectomy, pleural biopsies and minimally invasive cardiac procedures, it is limited in removing locally advanced mediastinal tumors.

MDM2 analysis in the management of benign lipomas versus atypical lipomatous tumors/well-differentiated liposarcomas: A useful prognostication tool?

BackgroundPrimary adenoid cystic carcinoma (ACC) of breast is rarely seen clinically. It is a special subtype of triple-negative breast cancer characterized by low expression of Ki-67, low malignant potential, slow progression and favorable prognosis. To date, treatment for this disease is controversial and no consensus is reached. We analyzed clinical manifestations and pathological characteristics of seven primary breast ACC cases and reported in combination with literature review to promote understanding, diagnosis and treatment of this disease.Case presentationWe collected seven breast ACC cases pathologically diagnosed and treated in Department of breast surgery of the First Affiliated Hospital of China Medical University from January 2015 to December 2018. We organized and summarized the clinical, imaging, pathological and prognostic information and performed statistical analysis. The median age was 60 years (ranging from 54 to 64 years). Tumors of all patients were detected by immunohistochemistry. Molecular types were mostly triple negative (4/7), and Ki-67 expression was low (5/7). Lymph node metastases were absent in all patients received axillary lymph node surgery. Median follow-up time was 39 months (ranging from 25 to 68 months). There was no occurrence of relapse, distant metastasis or death.ConclusionBreast ACC is accompanied with favorable diagnosis, which is different from typical triple-negative breast cancer. Accurate diagnosis of ACC is particularly important.

Standard pancreatic resections (SPRs) might have long-term deleterious effects on pancreatic function, without added oncological advantage in low malignant potential (LMP) or benign neoplasms. This study aimed to evaluate outcomes following organ-preserving pancreatic resections (OPPARs) and SPRs. Post hoc analysis of patients undergoing OPPAR or SPR for benign or LMP pancreatic tumors from January 2011 to January 2020 at Tata Memorial Hospital, Mumbai. Thirty-six and 114 patients were identified in OPPAR and SPR groups respectively. The overall morbidity (58.3% vs 43.9%, p-0.129) was comparable. Major morbidity (41.7% vs 21.9%, p-0.020), post-operative pancreatic fistula (POPF) (63.9% vs 35.1%, p-0.002), and clinically relevant POPF (41.7% vs 20.2%, p-0.010) were significantly higher with OPPAR. Post-operative endocrine insufficiency (14.9% vs 11.1%, p-0.567), exocrine insufficiency (19.3% vs 0%, p-0.004), and requirement of long-term pancreatic enzyme replacement (17.5% vs 0%, p-0.007) were higher in SPRs. Comparing left-sided and right-sided resections in the entire cohort, incidence of endocrine insufficiency was 17.1% vs 11.2% (p-0.299) and that of exocrine insufficiency was 8.6% vs 20% (p-0.048) respectively. OPPAR is associated with high post-operative major morbidity and pancreatic fistula rate but offers long-term benefit due to better preservation of pancreatic function than SPR. The incidence of exocrine insufficiency is higher in right sided as compared to left-sided pancreatic resections.

Introduction: Solid pseudopapillary neoplasms (SPN) and pancreatic neuroendocrine tumors (NET) are rare diseases that are generally incidental findings in imaging tests. SPN is a low-grade tumor with good prognosis that more commonly affects young female patients. Pancreatic NET has a significant variability in outcomes, with low malignant potential in non-progressing tumors that are welldifferentiated. Both tumors may appear very similar in imaging tests and immunohistochemical (IHC) evaluation, which makes the differential diagnosis challenging, especially in small lesions. Case report: The authors present the case of a 34-year-old male with a medical history of a primary mediastinal germ cell tumor. The patient had no symptoms. Follow-up abdominal CT scan evidenced a single, well-delimited nodular lesion in the pancreatic neck, measuring 17mm in diameter. The patient was submitted to an endoscopic ultrasound-guided fine needle aspiration biopsy (EUS-FNA) and after IHC analysis, there was a diagnostic suspicion of low-grade pancreatic NET. Central pancreatectomy (CP) was performed and complete lesion analysis evidenced a pancreatic SPN. Conclusion: The SPN can mimic low-grade nonfunctioning pancreatic NETs in imaging tests and EUSFNA IHC evaluation.

Objective: to compare short-term and long-term postoperative complications between patients who have undergone central pancreatectomy (CP) and distal pancreatectomy (DP).Materials and methods. This retrospective study included patients who had CP for benign pancreatic tumors and tumors of low malignant potential (cases) and patients who had DP for similar reasons (controls). The controls were randomly selected and matched cases for tumor size, presence of diabetes mellitus (Dm), and ASA physical status. we evaluated the incidence of grade ≥III complications (Clavien–Dindo classification), clinically significant pancreatic fistulas, Dm, and impaired exocrine pancreatic function in the late postoperative period.Results. There were 25 cases and 25 controls. Both groups were matched for the main clinical characteristics. Surgeries were significantly longer in the CP groups compared to the DP group (230 min vs 180 min, р < 0.0001). There was no difference in the overall incidence of postoperative complications (9 (36 %) vs 14 (56 %), р = 0.26); there was a trend towards a higher incidence of postoperative complications in the CP group. Two patients after CP (8 %) required repeated surgeries. none of the study participants died. Clinically significant (B and C) pancreatic fistulas were registered in 8 (32 %) and 11 (44 %) patients, respectively (p = 0.56). Two patients in the DP group (8 %) developed impairments of exocrine pancreatic function that required pharmacotherapy. none of the patients developed Dm postoperatively.Conclusion. Despite the fact that CP and DP outcomes were comparable in terms of the main parameters evaluated, severe post-CP complications indicate that there is a need for careful selection of patients for such interventions and further accumulation of experience. Our findings can be used in the subsequent analysis of the experience of different clinics.

CEP89 (centrosomal protein 89) is required for ciliogenesis and mitochondrial metabolism, but its role in cancer has yet to be clarified. We report that CEP89 is overexpressed in ovarian cancer (OC) compared to normal ovaries. Likewise, its expression is higher in malignant ovarian tumors than in borderline ovarian tumors with low malignant potential. More than a quarter of patients with OC have copy number gains in the CEP89 gene, and patients with high expression have more than a year shorter overall survival compared to those with low expression. Moreover, we found that CEP89 can be considered as a prognostic marker for poor overall survival in patients with OC, after adjusting for tumor stage and residual tumor. Nine out of the top 10 protein interactors of CEP89 have the highest percentage of total copy number variation (CNV) events in OC among all other cancer types. Furthermore, CEP89 messenger RNA (mRNA) levels are higher in OC patients with disease recurrence compared to those with no recurrence. We also analyzed CEP89 levels in OC cell lines in terms of CNV, mRNA,and protein levels; and observed that the FUOV-1 cell line has the highest levels among cell lines that originated from primary sites. Our study suggests that CEP89 may be a valuable prognostic predictor for the overall survival of patients with OC, and it could also be a novel therapeutic target in this malignancy.

Sinonasal glomangiopericytoma is a tumor showing similarities to a glomus tumor but is less aggressive with a low malignant potential. The treatment of choice is complete excision followed by a regular long-term surveillance. The role of adjuvant chemotherapy and radiation remains undetermined. Our patient was a 45-year-old man who presented with nasal obstruction. Endoscopic excision showed sinonasal glomangiopericytoma. No adjuvant treatment was offered and patient was advised active surveillance. Patient was on a regular three-monthly follow-up. There was no evidence of disease recurrence after one year.

Objective To overview the updated Swedish National Guidelines on Urothelial Carcinoma 2021, with emphasis on non-muscle-invasive bladder cancer (NMIBC) and upper tract urothelial carcinoma (UTUC). Methods A narrative review of the updated version of the Swedish National Guidelines on Urothelial Carcinoma 2021 and highlighting new treatment recommendations, with comparison to the European Association of Urology (EAU) guidelines and current literature. Results For NMIBC the new EAU 2021 risk group stratification has been introduced for non-muscle invasive bladder cancer to predict risk of progression and the web-based application has been translated to Swedish (https://nmibc.net.). For patients with non-BCG -responsive disease treatment recommendations have been pinpointed, to guide patient counselling in this clinical situation. A new recommendation in the current version of the guidelines is the introduction of four courses of adjuvant platinum-based chemotherapy to patients with advanced disease in the nephroureterectomy specimen (pT2 or higher and/or N+). Patients with papillary urothelial neoplasms with low malignant potential (PUNLMP) can be discharged from follow-up already after 3 years based on a very low subsequent risk of further recurrences. Conclusions The current version of the Swedish national guidelines introduces a new risk-stratification model and follow-up recommendation for NMIBC and adjuvant chemotherapy after radical surgery for UTUC.

BackgroundMucinous tubular and spindle cell carcinoma (MTSCC) is a type of renal cell carcinoma (RCC) described as an entity with low-grade and low malignant potential. This report presents a high-grade transformation of MTSCC which is an uncommon finding in this subtype of RCC. Although most reported cases showed low malignant potential and good prognosis after surgical resection, a small subset of patients has been reported with aggressive phenotype and fatal outcome.Case presentationA case of a 23-year-old male is discussed with an incidental renal mass in the left kidney after a workup of bilateral flank pain. Morphologically, the tumor was composed of tubular structures lined by cuboidal cells mixed with spindle cells and sarcomatoid change was present in the tumor. Previously sarcomatoid change has been mostly reported along with conventional morphology in other common type of RCC with a worse prognosis.ConclusionsThe aim of this report is to draw the attention of pathologists and clinicians to the importance of high grade MTSCC owing to the unfavorable prognosis.

Costello syndrome (CS) is a rare disorder affecting development and growth characterized by cancer predisposition and caused by mutations in HRAS proto-oncogene. Somatic HRAS mutations drive bladder carcinogenesis. The aim of this study was to analyze prevalence and histological characterization of bladder cancer (BC) in a cohort of patients with CS to help clinicians plan effective management strategies. This study included 13 patients above 10 years of age with molecular diagnosis of CS. Screening cystoscopies (31 total procedures) were performed to exclude BC. Any lesion was analyzed through cold-cup biopsy or trans-urethral resection of the bladder. According to histology, patients were followed-up with urinalysis and abdominal ultrasound yearly, and cystoscopies every 12-24months. During study enrollment, bladder lesions (often multifocal) were detected in 11/13 patients. Histological analysis documented premalignant lesions in 90% of cystoscopies performed, epithelial dysplasia in 71%, and papillary urothelial neoplasm of low-malignant potential in 19%. BC G1/low grade (Ta) were removed in 10%. Overall, 76% of patients showed a bladder lesion at first cystoscopy. The present findings document that individuals with CS aged 10 years and older have high prevalence of bladder lesions (premalignant/malignant), highlighting the importance of personalized screening protocols.

Simple SummaryMultilocular cystic renal neoplasm of low malignant potential (MCRNLMP) is a cystic renal neoplasm with an excellent prognosis. This neoplasm was previously named as “multilocular cystic renal cell carcinoma”, which is now considered obsolete. In 2016, the WHO distinguished this neoplasm of low malignant potential from cystic renal cell carcinomas, which have some overlapping morphologic features.Multilocular cystic renal neoplasm of low malignant potential (MCRNLMP) is a cystic renal tumor with indolent clinical behavior. In most of cases, it is an incidental finding during the examination of other health issues. The true incidence rate is estimated to be between 1.5% and 4% of all RCCs. These lesions are classified according to the Bosniak classification as Bosniak category III. There is a wide spectrum of diagnostic tools that can be utilized in the identification of this tumor, such as computed tomography (CT), magnetic resonance (MRI) or contrast-enhanced ultrasonography (CEUS). Management choices of these lesions range from conservative approaches, such as clinical follow-up, to surgery. Minimally invasive techniques (i.e., robotic surgery and laparoscopy) are preferred, with an emphasis on nephron sparing surgery, if clinically feasible.

Neuroendocrine tumors are a vast group of neoplasms. Insulinomas are the most frequent functioning pancreatic neuroendocrine tu-mors. Therapeutic gold standard is surgery without which most patients have a fatal outcome due to hypoglycemia. Herein we report a case of a 91-year-old female, with a 3 month history of asthenia and adynamia, which progressed to several neuroglycopenic events, leading to hospitalization. An abdominal CT-scan reported 1.8 x 1.4 cm pancreatic tumor. Labs reported proinsulin 40.5 pM/L. Laparos-copic enucleation was performed. 3 months later she presented a pancreatic pseudocyst treated successfully by endoscopy. Insulino-mas, are considered to have low-malignant potential, it is a rare endocrinal tumor and there is minimal mention about a consensus for optimal surgical treatment. Insulinoma remains an exclusion diagnosis after analyzing other differentials. Correct utilization of pre-operative imaging is critical in pre-surgical planning to choose the appropriate surgery. Keywords: Laparoscopy; Insulinoma; Whipple’s triad; Neuroendocrine tumor.

Neoplasms of the urachus are exceedingly rare, representing 0.17% of all bladder cancers. The mucinous cystic tumor of low malignant potential (MCTLMP) subtype is particularly rare with just 25 previous cases reported in the literature. Although rare, MCTLMPs are important to identify due to potential devastating complications and good cure rates with surgical removal. We present a 43 year old female with a nuanced constellation of comorbidities and confirmed MCTLMP following a workup for abdominal pain and irritative lower urinary tract symptoms. Notably, this tumor did not change in size over a 3-year course of serial imaging prior to surgical excision. This urachal MCTLMP represents roughly the 26th and one of the smallest of its subtype reported in the literature. This case illustrates the diagnosis and management of this rare urachal MCTLMP. Individual patient medical history, clinical considerations, and neoplasm characteristics are examined. Although rare, the potential for increased malignancy and potential complications necessitates surgical management and further investigation by the academic community.