Low Malignant Potential Research Articles

Melanoma Diagnosis in the Mihm Era-And Beyond.

During the illustrious career of Martin C. Mihm Jr., MD, the diagnosis of melanoma underwent significant changes, to which he made many contributions. In early descriptions, melanomas were fungating tumor masses that were obviously malignant, and highly lethal. In seminal work by Dr. Mihm and his mentor, Wallace H. Clark, Jr., MD, the early phases of development of these neoplasms were recognized and distinguished from the more advanced disease. It was generally believed that the early stage of melanoma, termed radial growth phase (RGP) and characterized by absence of vertical growth phase (VGP) and by favorable microstaging attributes could be recognized, excised, and cured, thus preventing the development of advanced disease. However, strenuous efforts in this direction over several decades have resulted in little or no change in mortality, leading to the recognition that many of these neoplasms, at least, may not be true biological malignancies, and to the conclusion that overdiagnosis commonly occurs in this disease, which is defined as representing diagnosis as melanoma of a neoplasm that would not have had the capacity to cause death or symptoms in the lifetime of the host. Although there may be other subsets of neoplasms in this category, an important category of overdiagnosis in melanomas is concentrated in T1a melanomas that lack VGP. If these neoplasms can be recognized with sensitive and specific criteria, which may already be available, changes in terminology may be appropriate, recognizing that some of them may have low malignant potential, whereas others may have no capacity at all for metastasis and may not warrant the use of the term "melanoma."

Predicting Malignancy in Adnexal Tumors With FAPI PET/CT and FDG PET/CT: A Case Report on a Borderline Ovarian Tumor.

A 54-year-old woman presented with a 55 × 64-mm tumor in the ovary with high [18F]FDG uptake on [18F]FDG PET/CT highly suggestive of ovarian cancer. Prior to surgery, the patient underwent [68Ga]-Ga- fibroblast activation protein inhibitor (FAPI)-46 PET/CT, which revealed low [68Ga]-Ga-FAPI-46 tumor uptake. Histopathology revealed a borderline ovarian tumor, which has low malignant potential and a 10-year survival rate greater than 93%. High [18F]FDG uptake is considered a fair predictor of malignancy in adnexal tumors. The present case demonstrates the potential superiority of [68Ga]-Ga-FAPI-46 PET/CT over [18F]-FDG PET/CT in differentiating malignant adnexal tumors from borderline ovarian tumors.

Sialadenopapillary Ductal Tumors: Unifying the Spectrum of Sialadenoma Papilliferum-like Tumors With Low Malignant Potential.

Sialadenoma papilliferum is a tumor characterized by surface papillary projections and glandular/microcystic proliferation at the lesion base. Cases in which surface involvement is absent have been termed "sialadenoma papilliferum-like intraductal papillary tumor." Similar tumors that are present in the mandible have been termed "tubulopapillary hidradenoma-like tumor of the mandible." While previously considered benign, these tumors demonstrate variable clinical behavior and likely exist on a spectrum, rather than as discrete entities. In this study, we present a detailed clinicopathologic and molecular analysis of these lesions and propose a unifying diagnostic term: sialadenopapillary ductal tumor (SDT). Twenty-two cases with similar histologic features were reviewed, with special attention being paid to the clinicopathologic features. Immunohistochemistry for BRAF V600E and molecular testing were performed where material was available. The cases had varying diagnoses, ranging from benign to malignant. Six cases involved bone, 1 of which metastasized to a local lymph node. Of the 20 cases tested for BRAF V600E by immunohistochemistry, 18 were positive. Molecular testing was performed in 5 cases, where BRAF, PTPN11, and PIK3CA mutations were identified, predominantly members of the RAS-RAF-MEK-ERK pathway. In addition, 1 case was reclassified as an intraductal carcinoma after the identification of an NCOA4::RET gene fusion. Tumors on the SDT spectrum all share morphologic and molecular commonalities with unreliable distinguishing features. These tumors demonstrate the potential for aggressive local growth and regional metastasis. We propose a unifying diagnostic term for these lesions to reflect their common morphologic and molecular features and, most importantly, low malignant potential.

Long-term recurrence risk, metastatic potential and length of cystoscopic surveillance of low-grade non-muscle invasive bladder cancer.

Patients with Ta low-grade (LG) non-muscle invasive bladder cancer (NMIBC) rarely develop metastases or die from it. Long-term data are scant and length of follow-up poorly defined. This retrospective study included 521 patients diagnosed with primary TaLG (n=491) or papillary urothelial neoplasm of low malignant potential (PUNLMP, n=30) from 1989-2019 at an Academic center. Patient data was acquired using patient records chart review and a bladder cancer informatics registry at the center. Risk of recurrence, progression in stage, to muscle invasion, metastases and death due to BC were analyzed. RNAseq assessed the transcriptomic profiles of four TaLG that metastasized. Interobserver variability in pathological grading (WHO 2004/2022 and 1973, n=80) was blindly assessed by three expert pathologists. Median follow-up was 9.6 (95%CI: 8.6-10.2) years. Among 521 patients (73% men, median age 67.0 years), 350 recurred, 57 progressed in stage, 20 developed metastases, and 15 died from BC (median of 9.6 years after diagnosis). Cancer-specific survival probabilities were 0.99, 0.98 and 0.96 at 5-, 10- and 15- years, respectively. Fifty patients who were recurrence-free for the first 5 years developed late recurrences and 2 of them died of BC. Metastatic TaLG had more adverse transcriptomic findings in keeping with higher grade tumors despite phenotypically similar to indolent tumors. Grading concordance for the 2004/2022 system and WHO 1973 was 0.78 (95%CI: 0.65-0.90) and 0.41 (95%CI: 0.32-0.50), respectively. This study with long-term data challenges the assumption that primary TaLG NMIBC nearly never progresses to lethal disease if followed long enough. However, the risk of BC-related mortality is extremely low in patients who are recurrence-free for the first 5 years. Minimizing variability in pathological grading remains an unmet need.

Clinical and Pathological Characteristics of Bladder Cancer in Patients Aged 18–45 Undergoing Transurethral Resection of Bladder Tumor

Background and Objectives: Bladder cancer in patients under 45 is poorly characterized and rarely described, with variabilities in clinical outcomes and tumor properties. Our study aimed to elucidate the clinical and pathological features and outcomes of bladder cancer in this younger demographic to better inform management strategies. Materials and Methods: We conducted a retrospective analysis at the Urology Department of “Pius Brînzeu” County Emergency Clinical Hospital in Timișoara, Romania, on 60 patients aged 18–45 who underwent transurethral resection of bladder tumor (TURBT) during a 9-year period. Results: The cohort had a mean age of 38.5 ± 5.6 years with a male predominance (70%). Most tumors were non-muscle-invasive (NMIBC; 80%), with 16.7% being papillary urothelial neoplasms of low malignant potential (PUNLMP), 50% stage pTa, and 30% stage pT1. High-grade tumors were present in 43.3% of the patients. Recurrence occurred in 40% of the patients, while progression was observed in 16.7%. The 3-year overall survival rate was 93.3%, and the progression-free survival rate was 83.3%. Patients with high-grade tumors had a significantly higher recurrence rate (61.5% vs. 23.5%, p = 0.003) and lower survival rates compared to those with low-grade tumors. Conclusions: Young patients predominantly present with low-to-intermediate-stage tumors, yet a significant portion exhibit high-grade tumors associated with poorer outcomes. These findings suggest that while bladder cancer in younger patients tends to be less invasive, aggressive follow-up and treatment are crucial in those with high-grade tumors.

Papillary Urothelial Neoplasia with Low Malignant Potential (PUNLMP) in a 9-year-old Child: A Case Report and Review of Literature

Papillary Urothelial Neoplasia with Low Malignant Potential (PUNLMP) in a 9-year-old Child: A Case Report and Review of Literature

Renal Oncocytoma: A Systematic Review of Its Metastatic Features.

Oncocytomas are referred to as benign kidney neoplasms. They primarily affect adults, with patients over 70 years old being the most affected. Renal oncocytomas (ROs) are frequently detected by excision, biopsy, or scan. Hematuria, flank pain, and a palpable mass are the traditional trio of symptoms. Oncocytomas appear as well-circumscribed, tanor mahogany-coloured masses with a central scar that is stellate. Histological features include well-circumscribed lesions, bland cytology, eosinophilic cytoplasm, regular nuclei with prominent central nucleoli, and nested architecture. ROs are rarely linked to an aggressive clinical course and have an excellent prognosis. There is proof that the disease can spread to the liver and bones.Some literature has also reported oncocytoma metastases to the lung and liver. This systematic review of the literature examines and evaluates the malignant potential of oncocytoma. The purpose of the study was to determine whether ROs can be diagnosed as a benign condition or if malignancy needs to be considered and investigated. Seventeen studies were analysed which had a total of 412 ROs. Four patients (one percent) died as a result of their illness. There was evidence of disease progression in every patient who passed away from their illness. Six patients (1.5%) experienced disease progression in total. Three hundred and seventeen patients (80%) underwent radical nephrectomy, while 81 patients (20%) underwent partial nephrectomy. Liver, bone, lung, lymphadenopathy, and local recurrence were among the metastasis sites. Perinephric fat invasion, renal sinus fat invasion, renal capsular invasion, and vascular invasion are characteristics of metastatic behavior that have been found. Despite this, the small number of patients who experienced disease progression and/or death as a result of ROs implies that aggressive malignant behavior is not always correlated with the presence of metastatic features or disease. Oncocytomas should be viewed as having a low potential for malignancy rather than as benign. Individuals who exhibit aggressive characteristics, such as vascular invasion and/or perinephric fat invasion, have an atypically good prognosis. Despite advancements in imaging and immunochemical techniques, it is indisputable that ROs, which were first classified as renal tumours in 1976, continue to pose a diagnostic challenge for multidisciplinary teams.There is considerable variation in practice across the globe due to difficulties in confirming ROs, especially when it comes to metastatic disease. There is even more variation in the management of follow-up care that follows. This will remain the MDT's current state until randomised controlled trials, long-term results, and a better comprehension of the behavior of this tumour are obtained.

8120 Effective Management of Neonatal Hypercalcemia in Congenital Mesoblastic Nephroma: A Case Report on the Use of Calcitonin

Abstract Disclosure: C.S. Galvez: None. K. Rifai: None. C. Moorthy: None. S. Chheda: None. M. Zerah: None. Introduction: Pediatric patients with hypercalcemia of malignancy are typically treated with hyperhydration, loop diuretics and bisphosphonates. Calcitonin, a peptide functionally antagonizes the effects of PTH. Calcitonin has limited pharmacokinetic data in neonates, especially those with Congenital Mesoblastic Nephroma (CMN), making treatment challenging with unclear safety and efficacy parameters. We present the fourth published case describing the use of calcitonin for likely PTH-rP-mediated severe hypercalcemia in a neonate with CMN. Clinical Case: A 15-day-old late preterm male, presented with lethargy, poor feeds, severe failure to thrive and a large abdominal mass. The mother is a G4P4, with good prenatal care. On admission, the patient was hypertensive (138/84 mmHg) and appeared dehydrated. Physical examination revealed a firm mass in the right abdomen crossing the midline. Serum calcium levels were 13.5 mg/dL (normal 9.0-10.9 mg/dL), with an ionized calcium 1.71 mmol/L (normal 1.15 and 1.30 mmol/L). Abdominal ultrasound showed a large, 9.1 x 8 cm dominant solid right renal mass, most likely CMN, with left-sided medullary nephrocalcinosis. Cardiac Echo and EKG were within normal limits. The patient received hydration, nicardipine (titrated to decrease blood pressure by 10-15 mmHg per day) and furosemide at 1 mg/kg/day IV q6h. Due to the persistence of hypercalcemia after 24 hours of treatment (14.7 mg/dL serum calcium with ionize of 1.81 mmol/L), the patient received one dose of calcitonin (4 units/kg subcutaneously), resulting in a decrease in serum calcium to 11.5 mg/dL in 4 hours. The patient underwent a successful radical right uretero-nephrectomy, with post-surgery improvement of neurological, cardiovascular and electrolyte status. Unfortunately, PTH-rP sample was insufficient. Pathology reported CMN of the cellular type. CMN is a rare tumor with low malignant potential, representing approximately 3% of all pediatric renal tumors. It is the most common renal neoplasm diagnosed in the first 6 months of life. The most common symptom is an abdominal mass (76%), with hypertension (19%). Hypercalcemia was reported in only 4% which can be secondary to paraneoplastic secretion of PTH or PTH-rP. Conclusion: This case represents the fourth instance of calcitonin use in a neonate with CMN to manage persistent hypercalcemia. Subcutaneous administration of calcitonin at 4 units/kg proved effective with a short onset of action (within 2 hours) and duration of 6 to 8 hours, making it a viable alternative to bisphosphonates (action around 48 hours and duration of 7 to 14 days). Calcitonin's advantages include less significant side effects and a faster resolution of complications like "Hungry bone Syndrome." The flexibility of repeated dosing every 6 hours, with the option to increase the dose, if necessary, adds to its clinical utility. Presentation: 6/3/2024

Clinical Insights Into Pediatric Solid Pseudopapillary Neoplasms of the Pancreas.

Solid pseudopapillary neoplasms(SPNs) of the pancreas are rare tumors with low malignant potential that usually occur in young girls and women. This study investigated the treatment experiences and outcomes after surgery for pediatric SPNs in our institutions as pediatric case series ofsolid SPNs are few, and long-term follow-up is also limited. We retrospectively reviewed the outcomes of nine patients diagnosed with SPNs who underwent surgery in our three hospitals (The Jikei University Hospital, The Jikei University Kashiwa Hospital, and Kawaguchi Municipal Medical Center)between 2001 and 2023. All nine patients who underwent surgery were girls. Their ages ranged from 8 to 15 years (median: 10years). The location of the tumor was at the pancreatic head, body, and tail in five, one, and three patients, respectively. Enucleation, pancreaticoduodenectomy, and laparoscopic distal pancreatectomy (LDP) were performed in four, two, and three patients, respectively. Regarding postoperative complications, a pancreatic fistula was detected in six patients, with three and three patients having grades A and B fistulas, respectively; two patients required percutaneous drainage, and one patient required endoscopic ultrasonography (EUS)-guided transgastric drainage. The follow-up period rangedfrom six to 261 months (median: 97 months). At the final follow-up, all nine patients were alive without recurrence. SPNs of the pancreas are incidentally diagnosed by diagnostic workups following trauma in children more frequently compared to adults. Additionally, the tumor resection by minimally invasive approaches, such as enucleation, or laparoscopic procedures results in a good prognosis in some cases.

Aggressiveness evaluation of borderline serous ovarian tumors by analysis of Psammoma bodies present in cancer tissues using micro-FTIR spectroscopy

Borderline ovarian tumor is a type of tumor with generally low malignant potential. However, these tumors pose diagnostic challenges with benign and malignant epithelial ovarian tumors because the clinical symptoms are similar and investigation procedures for specific diagnosis are still debated. In addition, a small number of borderlines transform into high-grade serous ovarian carcinoma with a poor prognosis. Therefore, tools improving a better characterization of high-risk subtypes of borderline tumors to enable understanding of possible unfavorable evolution are essential for patients’ management. Psammoma bodies (PBs) are microcalcifications found both in serous epithelial ovarian cancer and serous borderline tumors with possible correlation with disease progressionIn this work, the chemical composition of PBs found in the tissues of borderline, high-grade and low-grade ovarian tumors was evaluated using micro-FTIR spectroscopy. Applying principal component analysis to spectral data, it was observed that among the borderline tumors analyzed (1-bl,2-bland3-bl), the PBs of3-blshowed a different chemical content from that of the PBs of the high-grade and low-grade tumors, while the PBs of1-bland2-blappeared to have similar chemical content to the PBs of high- and low-grade tumors. The discriminating wavenumbers were found to be those related to carbonate CO32− (1454, 1413 cm−1and 872 cm−1) and phosphate PO43−(1018 cm−1and 960 cm−1) present in microcalcifications. A higher ratio between peak intensities at 1413 cm−1and 1018 cm−1(I1413/I1018) was observed in the PBs of3-blcompared with those of high- and low-grade ovarian carcinoma patients, which correlates with higher CO32− content. On the other hand, the PBs of1-bland2-blshowed a CO32−level close to that of the PBs of high- and low-grade patients. Several studies on microcalcifications in breast carcinoma have reported that increased carbonate content is related to decreased tumor aggressiveness. Therefore, case bl-3 appeared to be the less aggressive. The results obtained from FTIR analysis were in agreement with histopathological tumor classification and molecular analysis for BRAFV600E. The FTIR technique could become a reliable tool for identifying borderline low- and high-risk tumors.

Unraveling the Molecular Landscape of Uterine Tumor Resembling Ovarian Sex Cord Tumor: Insights From A Clinicopathological, Morphologic, Immunohistochemical, and Molecular Analysis of 35 Cases

Uterine tumor resembling ovarian sex cord tumor (UTROSCT) is a rare tumor of uncertain lineage and low malignant potential. Most tumors behave in a benign manner, but a subset of UTROSCT exhibit an aggressive clinical course with recurrences and metastases. The recurrent molecular alterations in UTROSCT mostly represent gene fusions involving NCOA1-3. We performed a comprehensive clinicopathological, morphologic, immunohistochemical, and molecular analysis on a cohort of 35 UTROSCT. The tumors exhibited various architectural patterns (diffuse, corded/trabecular, tubular, sertoliform, fascicular, whorled, nested, microfollicular, and pseudoglandular), often in combination. The immunohistochemical analysis confirmed the polyphenotypic immunoprofile, often with coexpression of sex cord–stromal, smooth muscle, and epithelial markers, as well as hormone receptors. Next-generation sequencing RNA analysis revealed recurrent NCOA1-3 gene fusions in 22/32 analyzed cases (69%), including ESR1::NCOA3 (11/22), GREB1::NCOA2 (7/22), ESR1::NCOA2 (3/22), and GREB1::NCOA1 (1/22). Tumor mutation burden was low in all cases. The fusion-positive cases exhibited statistically significant association with whorled architecture, conversely necrosis was associated with fusion-negative status. We did not find a significant relationship between any architectural pattern and GREB1 alterations, but the NCOA2-altered tumors were associated with pseudoglandular architecture. The GREB1-altered cases occurred in older patients and tended to be more often intramural masses compared with ESR1-altered cases. On the contrary, the ESR1-altered cases presented more often like submucosal or polypoid tumors. Two tumors exhibited aggressive behavior with recurrent disease. Both of these cases harbored a GREB1::NCOA2 fusion. Unsupervised hierarchical cluster analysis of our cohort revealed 2 main clusters. The tumors with GREB1 or NCOA2 fusion cluster together, suggesting that there are underlying molecular differences between these cases and cases with ESR1::NCOA3 fusion or without fusion. Our findings contribute to the growing knowledge about a rare neoplasm with currently uncertain biological behavior.

Tumor in the liver: Six inflammatory pseudotumor patient

Inflammatory pseudotumor (IPT) is a rare liver disease confused with liver tumors. It is a disease that should be known in the differential diagnosis for clinicians as the correct diagnosis of IPT will prevent unnecessary surgery. Demographic datas, diagnoses and imaging modalities of six patients with radiologically and/or histopathologically diagnosed hepatic IPT between 2016 and 2023 were retrospectively analyzed. Four out of six patients were female and median age was 57.5 (47-66). C-reactive protein was higher in four patients, and carbohydrate antigen 19.9 level was higher in one patient. We used magnetic resonance imaging (MRI) for diagnosis in five patients. Only in one patient computed tomography was enough for diagnosis. Tumor locations were segment 5 for two patients, segment 7-8 in two patients, segment 7 in one patient, and 8 in one patient. Liver biopsy was performed in five patients because it could not be distinguished from malignancy by imaging methods. Histopathological results of all these biopsies defined as IPT. Initial tumor median size was 31 (17-55) mm. Two patients were operated on. The first one underwent right hepatectomy due to a 2-fold increase in size within 11 months. The second one had a mass indistinguishable from hepatic adenoma by MRI and underwent nonanatomic resection. In one patient, IPT disappeared completely in the 18th month of follow-up period while it regressed in size in two patients. Two of our patients had a history of recurrent endoscopic retrograde cholangiopancreatography, which we noticed incidentally before IPT was diagnosed. IPTs are liver masses with low malignant potential and may shrink spontaneously during follow-up. We suspected that biliary tract interventions may be the cause of IPT.

A Case of Malignant Transformation of an Orbital Epidermoid Cyst to Cystic Squamous Cell Carcinoma.

Squamous cell carcinoma of the orbit is uncommon as there is no squamous epithelium in the orbit. Thus, mechanistically squamous cell carcinoma of the orbit most commonly arises from a cutaneous lesion. Although orbital epidermoid cysts are thought to have very low malignant potential, these lesions possess squamous epithelium and theoretically can undergo malignant transformation. Here we present the case of a 63-year-old woman who presented with a 3-month history of diplopia and forehead tenderness with an orbital extraconal lesion on MRI consistent with a ruptured epidermoid cyst. Six months following resection, she suddenly experienced new-onset left upper eyelid ptosis, recurrent diplopia, and left orbital pain. MRI revealed a recurrence of the left orbital mass. Left anterior orbitotomy and biopsy revealed cystic squamous cell carcinoma. This case appears to demonstrate a very rare malignant transformation of an epidermoid cyst to cystic squamous cell carcinoma.

ICU Management of Post-Craniotomy Patients with Schwannoma Vestibular Tumor Removal

Background: Vestibular schwannoma (VS) is a relatively common tumor that arises from the vestibulocochlear nerve (CN VIII) and represents 80% of cerebellopontine angle (CPA) masses. CPA tumors are mostly benign, slow growing with low malignant potential (~1%). VS have rarely been reported, and there is a lack of in-depth discussion on the experience of management of massive acoustic neuromas in ICU. It represents the case in which the patient presented with obstructive hydrocephalus and progressive neurological deficits. The purpose of this case report is to understand the management of post-operative patients with VS with several comorbidities in the ICU.Case: We present a 53 years old woman with a giant VS and obstructive hydrocephalus. An imaging findings revealed a brain tumor in the CPA region and obstructive hydrocephalus. Consequently, she relieved her hydrocephalus with a ventriculoperitoneal shunt (VP shunt). After 1.5 years, her mental condition deteriorated, and her left limb muscle strength gradually decreased. Under a joint consultation with Department of Neurosurgery and Anesthesiology, she underwent tumor removal. Upon discharge, the previously observed neurological deficits, which were reversible had been successfully resolved. The neuroimaging confirmed the complete tumor removal, while the neuropathologic examination revealed a VS.Discussion: If untreated, an acoustic neuroma can grow large enough to cause pressure on the brain stem. The tumor can block the flow of cerebrospinal fluid (CSF) between the brain and the spinal cord, causing a buildup of the fluid in the brain. Because the skull is a closed structure, excess fluid in the brain (hydrocephalus) can press against the brain, causing unsteady movement and lack of coordination (ataxia), headaches and confusion. Patients with brainstem compression had significantly longer mean LOS than patients without. Also, the dissection of the facial nerve from the tumor in order to preserve it can sometimes cause swelling, which can result in weakness or paralysis (complete or partial loss of muscle function). This is usually temporary but can take weeks to months to recover. After the operation, the patient was treated in the ICU, a ventilator was installed,insulin was given to regulate blood sugar and anti-hypertension medication for maintaining blood pressure. During treatment in the ICU, adequate fluids and nutrition are provided. Monitoring is carried out on cardiovascular function, hemodynamics and respiration by monitoring blood pressure, electrocardiogram (ECG), oxygen saturation. The patient was moved to high care unit (HCU) after being treated for 3 days in the ICU.Conclusion: Postoperative therapy is more focused on supportive therapy, including maintaining the airway, regulating blood sugar, blood pressure and providing mechanical ventilation to maintain adequate oxygenation.

Low-Grade Urothelial Neoplasia

This chapter reviews the clinical, histopathologic, and cytologic features of low-grade urothelial neoplasia, including urothelial papilloma, papillary urothelial neoplasm of low malignant potential, and low-grade papillary urothelial carcinomas. An overview of various surgical pathology and cytology classification systems, diagnostic criteria, and common molecular alterations will be reviewed.

Fibrosarcomatous Transformation in Dermatofibrosarcoma Protuberans of the Male Breast and its Association with Magnetic Resonance Imaging and Immunohistopathologic Features.

Dermatofibrosarcoma Protuberans (DFSP) is a rare soft tissue sarcoma, accounting for approximately 1% of all tumors; however, DFSP of the breast is extremely rare. Moreover, DFSP generally has a low malignant potential and is characterized by a high rate of local recurrence along with a small but definite risk of metastasis. The risk of metastasis is higher in fibrosarcomatous transformation in DFSP than in ordinary DFSP. We have, herein, reported a case of a 61-year-old male patient with fibrosarcomatous transformation in DFSP. Preoperative Dynamic Contrastenhanced Magnetic Resonance Imaging (DCE-MRI) of the breast revealed an oval-shaped mass with heterogeneous internal enhancement, a large vessel embedded within, and a washout curve pattern on kinetic curve analysis. The mass exhibited a hyperintense signal on Diffusion-weighted Imaging (DWI), with a low apparent diffusion coefficient value. Histologically, the bland spindle tumor cells were arranged in a storiform pattern. Areas with the highest histological grade demonstrated increased cellularity, cytological atypia, and mitotic activity. Immunohistochemically, Ki-67 and p53 were highly expressed. Recognizing the risk and accurately diagnosing fibrosarcomatous transformation in male breast DFSP are critical for improving prognosis and establishing appropriate treatment and follow-up plans. This emphasizes the significance of combining immunohistopathological features with DCE-MRI and DWI to assist clinicians in the early and accurate diagnosis of sarcomas arising from male breast DFSP.

Hemangiopericytoma of the lacrimal sac: A rare case report and review of literature

A primary hemangiopericytoma (HPC) of the lacrimal sac in a 40-year-old patient is reported. HPCs are rare soft tissue tumors derived from the pericapillary pericytes with low malignant potential. Only 10 cases of lacrimal sac HPCs have been previously reported. Here, the authors report an additional case that presented clinically with epiphora and painless mass with no recurrence during 36 months of follow-up. The treatment of choice for this neoplasm with frequently unpredictable behavior is total excision with surrounding tissue and long-term follow-up.

Study of non-invasive follicular thyroid neoplasm: A borderline entity

ABSTRACT Background: Non-invasive follicular thyroid neoplasm with papillary-like nuclear features (NIFTP) is a newly added entity in the fourth edition of the WHO Classification of Tumors of Endocrine Organs (2017). It is regarded as a neoplasm with very low malignant potential, thus affecting further treatment of the patients. Aims: To study the recently diagnosed entity, NIFTP, as not many cases have been reported of the same. Materials and Methods: A retrospective analysis of all thyroid neoplasms received in the Department of Pathology of our institute over two years was done. Out of the 188 cases that we reported, 38 were of non-invasive follicular neoplasms. Results: Thirty-two patients were female, whereas six patients were male. Thirty-five patients presented with solitary thyroid nodule and the others had an ill-defined thyroid swelling. Thirty-four patients had normal thyroid profile while two patients each presented with hypothyroidism and hyperthyroidism. Conclusion: It is crucial to carefully study the histopathological features of solitary thyroid swellings and correlate with clinical and radiological findings. The recently added diagnostic category of NIFTP helps clinicians prevent over-treatment of a group of low-risk thyroid tumors.

Pediatric Solid Pseudopapillary Neoplasm of the Pancreas.

Solid pseudopapillary neoplasm (SPN) of the pancreas in children is a rare tumor with low malignant potential. Some tumors, however, behave aggressively. There is very little literature on managing these variants, especially in children. We share our experience of managing large and recurrent SPN and explore the clinicopathological findings correlating to the risk of recurrence. This is a retrospective study of children treated for SPN between 2012 and 2022 at a tertiary care center in India. The clinicopathological features and management strategies in these children were evaluated. Sixteen children with SPN were treated during this period (88% of girls). The median age of presentation was 12 years (interquartile range [IQR]: 9-14). All children presented with abdominal pain. Computed tomography gave a definitive diagnosis in 81% of cases. The tumor predominantly involved the head of the pancreas (n = 9, 56%). Eight of nine children classified as high-grade (HG) malignant had a benign course. One child had a recurrence of the tumor 4 years after the initial resection and further recurrence on chemotherapy. She required radiation therapy in addition to reoperation following which she was disease free for 77 months. The overall median follow-up was 46 months (IQR: 18-72 months). Complete resection of the tumor provides a cure in most patients with SPN. Recurrent tumors require a multi-modality approach. Long-term survival is good. There is a need for clear definitions of the components within the WHO criteria for HG malignancy.

Inflammatory rhabdomyoblastic tumor, pheochromocytoma, and pulmonary hamartoma in a patient with neurofibromatosis type 1: a case report

BackgroundInflammatory rhabdomyoblastic tumors are relatively recently recognized soft tissue tumors with a low malignant potential. Here, we present a case of concurrent inflammatory rhabdomyoblastic tumor (IRMT), adrenal pheochromocytoma, and pulmonary hamartoma in a patient with neurofibromatosis type 1 (NF1). To our knowledge, this is the first time that this constellation of tumors has been described in the literature.Case presentationA female patient in her late 20s with known NF1 was diagnosed with an inflammatory rhabdomyoblastic tumor, pheochromocytoma, and pulmonary hamartoma in a short succession. IRMT was found to harbor a near-haploid genome and displayed a typical immunohistochemical profile as well as a focal aberrant p53 expression pattern.ConclusionsThis case report strengthens the theory that defects in the tumor suppressor NF1 play a central role in the pathogenesis of inflammatory rhabdomyoblastic tumors and that IRMT may be part of the spectrum of neurofibromatosis type 1 related tumors.