Common Pathological Diagnosis Research Articles

Oculoplastic Surgeries in Patients Older than 90 Years of Age.

To report the epidemiology, indications and surgical outcomes of oculoplastic surgeries in older adults (≥ 90 years old). A retrospective study was conducted reviewing the medical charts of 114 patients aged 90 years old and older who underwent oculoplastic procedures from 2010 to 2020. Data retrieved from the medical records included: past medical and ocular history, indication for surgery, type of surgery, intra and post-operative complications, pathological analysis for removed tissues, and surgical outcome in the last follow-up. One hundred and twenty eight surgeries were performed on 114 patients (male: female = 1:1). The mean age was 92.95 years old (± 3.12 SD). Six patients (5.2%) were older than 100 years old. The most common indication for surgery was lower lid malpositioning (32%). Mass/lesion excision was performed in 34 procedures (25%). Forty-three biopsies were analyzed and basal cell carcinoma was found to be the most common pathological diagnosis (32%). Hypertension was the most common associated systemic comorbidity (79 patients, 69%). 80% of the surgeries were performed under local anesthesia. Surgical revision was required in seven patients (5.2%). One patient had suffered from a minor stroke one day after the surgical pocedure. The same patient had orbital-skin fistulas after orbital exenteration. In our experience, oculoplastic surgeries among the elderly population are safe without significant complications and can usually be performed under local anesthesia. Advanced age should not prevent surgery, especially if the procedure may improve vision and quality of life.

Surgical Resection of Intraocular Tumors (Partial Transscleral Sclerouvectomy Combined With Mircoinvasive Vitrectomy and Reconstruction of the Eyeball) in Asian Patients: Twenty-Five Years Results.

ObjectiveTo describe the outcome of intraocular tumor resection by partial transscleral sclerouvectomy (PTSU) combined with micro-invasive vitrectomy and reconstruction of the eyeball (MVRE) in Asian patients.Design, Methods and ParticipantsThis retrospective, interventional cohort study included 366 patients who underwent PTSU combined with MVRE for intraocular tumors both in adult and pediatric age groups. The medical records of these patients were reviewed for clinical, operative, and histopathological features.Main Outcome MeasuresGlobe salvage, best corrected visual acuity (BCVA), surgical side effects, tumor control, and tumor-related metastasis and death.ResultsThe mean follow-up duration was 87 months (median, 66; range, 1-303 months). Among the 366 patients, the mean age was 8.5 years (median, 7; range, 1-19 years) in the 37 pediatric patients, and was 43 years (median, 42; range, 20-51) in 329 adult patients. The tumor mainly involved the ciliary body (n=136; 37.2%) and choroid (n=86; 23.5%). The common pathologic diagnosis of the 366 patients was as follows. In the pediatric age group, histopathologic examination revealed positive tumor margins in 37 patients mainly including ciliary body medulloepithelioma (8/37), ciliary body melanocytoma (13/37) and uveal melanoma (5/37). In the adult group, the pathological diagnosis mainly included melanoma (195/329), RPE adenoma (21/329), amelanotic melanoma (13/329), ciliary body adenoma of nonpigmented epithelium (19/329), schwannoma/neurilemmoma (11/329), melanocytoma (24/329), and leiomyoma (9/329). The globe salvage rate was 81.1% in the pediatric age groups (<20 years), and 93.6% in the adult group (≥20 years), respectively. Of the 338 salvaged eyes, final BCVA was 20/20 to 20/40 in 16 (4.7%), 20/40 to 20/80 in 58 (17.2%), 20/80 to 20/200 in 160 (47.3%), and ≤ 20/200 in 104 (30.8%). Early side effects included corneal edema in 28 (7.7%) patients, hyphema in 46 (12.6%), and vitreous hemorrhage in 76 (21%) patients. Postoperative side effects included proliferative vitreoretinopathy (PVR) in 67 (18.3%), late cataract in 42 (11.5%), and glaucoma in 18 (5%) patients. Local tumor recurrence was detected in 20 patients (5.5%) at a mean interval of 23.6 months, including melanoma (n=19) and medulloepithelioma (n=1). Enucleation was necessary in 28 (7.7%) cases owing to recurrence in 15 (53.6%), eye prophylaxis with high-grade malignancy in 5 (17.8%), and blind painful eye in 8 (28.6%) cases. Kaplan-Meier estimated for 5, 10-year metastasis rate and metastasis-related death rate (95%CI) in 213 UM patients were 3.2% (1.4%-7.0%), 6.9% (3.8%-12.3%); and 3.5% (1.6%-7.6%), 7.6% (4.2%-13.5%), respectively.ConclusionsAs a surgically challenging procedure, PTSU combined with MVRE offers several theoretical advantages over enucleation and radiotherapy. It can achieve control of most intraocular tumors, preserve useful vision, and maintain a cosmetically normal eye.

Clinicopathologic Features of Mitochondrial Nephropathy

IntroductionThe clinicopathologic characteristics of nephropathy associated with mitochondrial disease (MD) remain unknown. We retrospectively analyzed a cohort of patients with proteinuria, decreased glomerular filtration rate, or Fanconi syndrome who had a genetic mutation confirmed as the cause of MD, defined as mitochondrial nephropathy.MethodsThis nationwide survey included 757 nephrology sections throughout Japan, and consequently, data on 81 cases of mitochondrial nephropathy were collected.ResultsThe most common renal manifestation observed during the disease course was proteinuria. Hearing loss was the most common comorbidity; a renal-limited phenotype was observed only in mitochondrial DNA (mtDNA) point mutation and COQ8B mutation cases. We found a median time delay of 6.0 years from onset of renal manifestations to diagnosis. Focal segmental glomerular sclerosis (FSGS) was the most common pathologic diagnosis. We then focused on 63 cases with the m.3243A>G mutation. The rate of cases with diabetes was significantly higher among adult-onset cases than among childhood-onset cases. Pathologic diagnoses were more variable in adult-onset cases, including diabetic nephropathy, nephrosclerosis, tubulointerstitial nephropathy, and minor glomerular abnormalities. During the median observation period of 11.0 years from the first onset of renal manifestations in patients with m.3243A>G, renal replacement therapy (RRT) was initiated in 50.8% of patients. Death occurred in 25.4% of the patients during the median observation period of 12.0 years. The median estimated glomerular filtration rate (eGFR) decline was 5.4 ml/min per 1.73 m2/yr in the cases, especially 8.3 ml/min per 1.73 m2/yr in FSGS cases, with m.3243A>G.ConclusionHere, we described the clinicopathologic features and prognosis of mitochondrial nephropathy using large-scale data.

Histopathological analysis of juvenile patients with melanocytic lesions of the conjunctiva

Objective: This study aimed to examine the frequency of atypical features in conjunctival nevi in pediatric patients referred to the McGill University Health Center-McGill University Ocular Pathology and Translational Research Laboratory and to emphasize the importance of histopathological analysis to rule out malignant lesions, such as melanoma.Methods: Forty-four pediatric patients younger than 20 years of age previously diagnosed with melanocytic lesions of the conjunctiva were included in this study, and the database was analyzed for a 10-year period (2006–2015). Clinical information such as age, gender, location, type, size, and agreement between clinical and pathological diagnosis was also recorded and presented as means and percentages.Results: The mean age was 11.3 years, of which only 9.1% were older than 18 years. Gender predilection was found toward males (52.3%). An agreement was noted between clinical and pathological diagnosis in 77.2% of cases; only 22.72% showed atypia upon histopathological examination. The most common pathological diagnosis was compound nevus with different characteristics. The atypical lesions included conjunctival melanoma, junctional nevi with atypia, compound nevi with atypia, and cystic compound nevi. Moreover, 70% of the patients with atypical lesions were males and 60% of the specimens were from the right eye.Conclusion: An impressive number of 30% of all patients had some type of atypia after histopathological analysis. Therefore, based on these results, it is of extreme importance that all melanocytic lesions are sent for histopathological analysis, so an accurate diagnosis can be established.

Is it a new phenotype? Right temporal variant frontotemporal dementia

AbstractBackgroundFrontotemporal dementia (FTD) is heterogeneous and presents with behavioral or language symptoms with frontal and/or temporal atrophy. A variant presenting with anomia and word comprehension deficits, is termed semantic dementia (SD) and is associated with predominant left anterior temporal atrophy. Although the clinical presentation is different on the right side, no unique syndrome to identify right temporal variant FTD (rtvFTD) has been established.MethodsWe retrospectively collected 70 rtvFTD patients from our cohort based on their initial MRI and identified the clinical characteristics based on the case notes and compared them with those reported in Alzheimer’s disease (n=70), behavioral FTD (n=70), and SD (n=70). In a subset of cases we reported the genetic (n=6) and pathological (n=5) features along with a review of cases in the literature (n=49).ResultsBased on our single‐center study, rtvFTD presented a unique combination of cognitive (face recognition and memory impairments), behavioral/psychiatric (disinhibition, apathy, depression, and bizarre preoccupations), and language problems (word‐finding difficulties and anomia). It's genetic/pathological background was heterogeneous, in contrast to SD. The most common genetic mutation was MAPT, followed by GRN and TARDBP. The most common pathological diagnoses were FTLD‐TDP type C and FTLD‐tau, followed by FTLD‐TDP type A and B. Moreover, pyramidal degeneration was observed as a co‐pathology.ConclusionThe definition of a syndrome associated with right temporal atrophy could promote its recognition as a variant of FTD, which is crucial for individual patient management. A multicenter study will shed light on the many unanswered questions about this equivocal syndrome.

A Multi-Institutional Retrospective Analysis of T-Cell Lymphomas with Central Nervous System Relapse

A Multi-Institutional Retrospective Analysis of T-Cell Lymphomas with Central Nervous System Relapse

Justifying Bilateral Salpingo-Oophorectomy at Hysterectomy: A Large Retrospective Study

<h3>Study Objective</h3> Evaluate proportion of patients with justified bilateral salpingo-oophorectomy (BSO) at hysterectomy based on pathologic diagnosis and determine rate and predictors of avoidable BSO based on preoperative considerations, surgical characteristics and pathologic diagnosis. <h3>Design</h3> Multicenter retrospective analysis. <h3>Setting</h3> 7 Ontario, Canada hospitals (4 academic, 3 community). <h3>Patients or Participants</h3> Patients with concomitant BSO at hysterectomy. <h3>Interventions</h3> All hysterectomies with concomitant BSO from July 2016 to December 2019. Cases by gynecologic oncologists were excluded. <h3>Measurements and Main Results</h3> Demographics, surgical factors, surgeon characteristics and pathologic diagnoses were recorded. BSO was considered justified if pathologic diagnosis was endometriosis or any (pre)malignant diagnosis except for gestational trophoblastic neoplasia or cervical cancer/dysplasia. Criteria for avoidable BSO included: age ˂51 years, preoperative diagnosis of cervical dysplasia or benign diagnosis other than gender dysphoria, risk reduction or premenstrual dysphoric disorder, absence of intraoperative endometriosis and adhesions, and unjustified final pathology. Those with avoidable BSO were compared to patients with at least one criterion for BSO. Multivariate analyses identified factors most strongly associated with avoidable BSO. During the study period, 4191 hysterectomies were completed with 1422 (33.9%) having concomitant BSO. Final pathologic diagnosis justified BSO in 72.8% (1035/1422). The most common pathologic diagnoses were endometrial cancer (439/1422, 30.6%), endometrial hyperplasia (275/1422, 19.3%) and endometriosis (200/1422, 14.1%). The remaining cases were unjustified, with 42/1422 (3%) BSOs deemed avoidable. Compared to cases with at least one criterion for BSO, avoidable BSOs were more frequently completed by generalists (OR 2.01, 95% CI 1.04 to 4.08, p=0.044) and for preoperative diagnoses of abnormal uterine bleeding (OR 3.75, 95% CI 1.96 to 7.43, p<0.001) and/or fibroids (OR 3.26, 95% CI 1.70 to 6.23, p<0.001). <h3>Conclusion</h3> Final pathologic diagnosis justified most BSOs at hysterectomy. Ovarian preservation may have been possible in 3% of women, underscoring the need to standardize practice with respect to BSO among gynecologic surgeons.

Clinical Outcomes of Orbital Exenteration in Korean Patients with Orbital Malignancies

Purpose: We report the clinical outcomes of Korean patients who were diagnosed with orbital malignancies and underwent orbital exenteration.Methods: We retrospectively reviewed the tumor origins, histopathological diagnoses, local/regional recurrences, distant metastases, surgical margin clearances, overall and event-free survivals, and adjuvant chemotherapy or radiation therapy statuses of 14 patients who underwent orbital exenteration in our center from February 2009 to March 2020.Results: We enrolled seven men and seven women of mean age at the time of exenteration of 68 years (range, 37 to 80 years). The mean follow-up period was 44.6 months (range, 10 to 133 months). Most tumors had arisen in the eyelid (seven cases, 50.0%). The most common pathological diagnosis was malignant melanoma (five cases, 35.7%). We observed no local or regional recurrence after exenteration, but distant metastases developed in seven cases, of which four were malignant melanomas (80% of all melanomas). Positive surgical margins were observed in six cases (42.9%). The distant metastasis rate was 42.9%; the overall survival rate was 60%. The 1-year overall survival rate was 100%, the 2-year survival rate was 81.8%, and the 5-year survival rate was 56.1%. The 1-year event-free survival (EFS) rate was 100%, the 2-year EFS rate was 72.7%, and the 5-year EFS rate was 49.9%. Nine patients received adjuvant radiation or chemotherapy and six patients received combined chemoradiation.Conclusions: Patients underwent orbital exenteration to treat orbital malignancies and received postoperative chemotherapy and/or radiation exhibited differences in clinical outcomes and survival rates depending on the tumor type.

Safety of endoscopic endonasal biopsy for the pituitary stalk-hypothalamic lesions.

The indications for and the optimal biopsy approach in pituitary stalk-hypothalamic (PsH) lesions are controversial. Biopsies through an endoscopic endonasal approach (EEA) for PsH lesions have often been considered to cause the infundibulo-tuberal syndrome. The purpose of this study was to analyze the surgical and endocrinological safety of EEA biopsies for PsH lesions. A total of 39 consecutive patients who underwent an EEA biopsy between June 2011 and August 2020 in a single institute were retrospectively analyzed. The ophthalmological and endocrinological outcomes were assessed before and after surgery. PsH lesions were confirmed to be diverse pathological diagnoses, ranging from lymphocytic hypophysitis to diffuse midline glioma, and the most common pathologic diagnosis was a germinoma (18 patients, 46.2%). No patients developed visual deterioration after the biopsy. In patients without preoperative panhypopituitarism, 13 out of 28 patients (46.4%) developed new anterior pituitary hormonal deficiencies after the biopsy. When the tissue was collected from the stalk, the endocrinological deterioration rate was 100% (6 of 6 patients), while the rate was 31.8% (7 of 22 patients) when tissue could be harvested from an extra-stalk lesion. The rate of newly developed permanent diabetes insipidus after surgery was 40.9% (9 of 22 patients). The median surgery time was 125min, and there was no postoperative CSF leakage or infections noted. An EEA biopsy for PsH lesions is a safe and efficient surgical method unless the tissue is collected from the stalk.

The Outcome of Surgical Excision of Giant Supratentorial Brain Tumors in Pediatrics: A Study of Consecutive 70 Cases at our Institute

Abstract Background: Surgery of giant supratentorial pediatric brain tumors (GSPBT) is challenging. Diagnosis may be misled by the inconspicuous symptoms and signs. Surgery is difficult due to the propensity for severe hemorrhage which is risky in this age group. Aim of Study: We discuss the surgical management of GSPBT with emphasis on how to reach maximal safe resection and different surgical strategies to improve the outcome. Patients and Methods: All Patients with GSPBT under-going elective surgery were included of age 3 months to 12 years. Giant tumors were defined by being larger than 5cm. General anesthesia was performed by neuroanesthesiologists. Various tools and instruments were used such as Cavitating ultrasonic aspirator, microscope and endoscope. Blood trans-fusion was performed as early as possible to replace losses. Extent of removal was assessed from CT or MRI scans. Results: This study included 70 children operated for GSPBT. There were 47 males (67.1%) and 23 females (32.9%). The age ranged from 3 to 144 months with a mean age of 56.3 months. The commonest presenting symptoms were those of raised intracranial pressure (ICP) in 61 cases (87.1%). Gross total resection was achieved in 57 patients (81.4%), debulking up to 80% in 3 patients (4.3%), debulking up to 50% in 1 patient (1.3%) and partial resection less than 50% in 9 patients (12.9%). The amount of blood transfusion ranged from 5 to 300ml/kg with a mean amount of 39.4ml/kg. PNET was the most common pathological diagnosis. Two cases required interruption of the procedure due to massive bleeding causing hypotension (2.9%). There were no complications due to massive blood transfusion. There were no cases of mortality. Conclusion: GSPBT can be totally resected with a good outcome. This needs a team approach including a good an-esthesia team and proper postoperative care. Surgical tech-niques including sub-pial technique, endo scope- assisted microsurgery, and the CUSA may help accomplish this target.

Pediatric Aggressive Benign Mandibular Tumors: Clinical Features and Management.

Aggressive benign mandibular tumors are uncommon in the pediatric population, and there is few publishing in the literature specifically dealing with them. Aggressive tumors can be defined based on known biologic behavior and/or histologic type and/or clinical characteristics. To review the clinical features and management of lower jaw pediatric aggressive benign tumor. Medical records review of pediatric patients presented with aggressive benign mandibular tumors to the Maxillofacial and Plastic Surgery Department, University of Alexandria, Egypt between 2011 and 2019. Fifty-eight patients were included in this study, aged between 2 and 16 years (average = 11.8). Ameloblastoma was the commonest pathological diagnosis (n = 18) followed by central giant cell granuloma (n = 11) and juvenile ossifying fibroma (n = 10). Patients with central giant cell granuloma were treated by en-block resection (n = 4) or curettage after interferon alfa injection (n = 7). All other benign tumors were treated by en-block resection. The length of follow-up ranged from 1 to 8 years. Successful reconstruction was accomplished in 45 patients (88.2%). Aggressive lesion should be treated in an aggressive manner and immediate reconstruction is advocated. However, pharmacotherapy combined with enucleation is a more conservative approach for management of aggressive central giant cell tumors.

Investigation of the safety and usefulness of a renal biopsy for older elderly patients ≥75 years old

We herein report the safety and usefulness of a kidney biopsy in older elderly patients (≥75 years old). A retrospective observational study was conducted in older elderly patients who had received a renal biopsy at the Department of Nephrology, Shimane University Hospital, from January 1, 2008, to December 31, 2018. The native renal biopsy results of 52 later-stage elderly patients were analyzed (29 men and 23 women). The most common indication for a renal biopsy was nephrotic syndrome (n = 22), followed by rapidly progressive glomerulonephritis (n = 12) and asymptomatic urinary abnormalities (n = 12). The most common pathological diagnosis was membranous nephropathy (n = 12), followed by ANCA-associated nephritis (n= 8), minimal change nephrotic syndrome (6 case), membranoproliferative glomerulonephritis (n = 5), IgA nephropathy (n = 4), and diabetic nephropathy (n = 3). The concordance rate between the clinical and pathological diagnoses was 53.8%. The only complication of the renal biopsy was hemorrhaging requiring blood transfusion (1 patient; 1.9%). The hemoglobin level decreased by 0.5±0.05 d/dL after the biopsy. The rate of serious complications associated with a renal biopsy was comparable to that in previous reports in younger patient. Renal biopsies can therefore be safely performed even in older elderly patients. The concordance rate of the clinical and pathological diagnoses was about 50%. Therefore, renal biopsies should be performed in older elderly patients when necessary.

Primary membranous glomerulonephritis with negative serum PLA2R in haemophilia A successfully managed with rituximab \u2013 case report and review of the literature

BackgroundHepatitis C virus (HCV) and human immunodeficiency virus (HIV) cause a wide range of glomerular pathologies. In people with haemophilia, transfusion-associated infections with these viruses are common and definitive pathological diagnosis in this population is complicated by the difficulty of safely obtaining a renal biopsy. Membranous nephropathy (MN) is a common cause of adult onset nephrotic syndrome occurring in both primary and secondary forms. Primary MN is associated with podocyte autoantibodies, predominantly against phospholipase A2 receptor (PLA2R). Secondary disease is often associated with viral infection; however, infrequently with HIV or HCV. Distinguishing these entities from each other and other viral glomerular disease is vital as treatment strategies are disparate.Case presentationWe present the case of a 48-year-old man with moderate haemophilia A and well-controlled transfusion-associated HCV and HIV coinfection who presented with sudden onset nephrotic range proteinuria. Renal biopsy demonstrated grade two membranous nephropathy with associated negative serum PLA2R testing. Light and electron microscopic appearances were indeterminant of a primary or secondary cause. Given his extremely stable co-morbidities, treatment with rituximab and subsequent angiotensin receptor blockade was initiated for suspected primary MN and the patient had sustained resolution in proteinuria over the following 18 months. Subsequent testing demonstrated PLA2R positive glomerular immunohistochemistry despite multiple negative serum results.ConclusionsPursuing histological diagnosis is important in complex cases of MN as the treatment strategies between primary and secondary vary significantly. Serum PLA2R testing alone may be insufficient in the presence of multiple potential causes of secondary MN.

Epidemiology and Survival of Patients With Brainstem Gliomas: A Population-Based Study Using the SEER Database.

BackgroundBrainstem glioma is a primary glial tumor that arises from the midbrain, pons, and medulla. The objective of this study was to determine the population-based epidemiology, incidence, and outcomes of brainstem gliomas.MethodsThe data pertaining to patients with brainstem gliomas diagnosed between 2004 and 2016 were extracted from the SEER database. Descriptive analyses were conducted to evaluate the distribution and tumor-related characteristics of patients with brainstem gliomas. The possible prognostic indicators were analyzed by Kaplan-Meier curves and a Cox proportional hazards model.ResultsThe age-adjusted incidence rate was 0.311 cases per 100,000 person-years between 2004 and 2016. A total of 3387 cases of brainstem gliomas were included in our study. Most of the patients were white and diagnosed at 5-9 years of age. The most common diagnosis confirmed by histological review was ependymoma/anaplastic ependymoma. The median survival time was 24 months. Patients with tumors less than 3 cm in size had a better prognosis. Surgery was effective at improving overall survival. There was no evidence that radiotherapy and chemotherapy improved overall survival.ConclusionBrainstem gliomas can be diagnosed at any age. Ependymoma/anaplastic ependymoma is the most common pathological diagnosis. The prognosis is poor, and timely diagnosis and surgery are effective at improving the prognosis. We suggest that more attention should be given to the treatment of patients with brainstem gliomas.

MO142A SINGLE-CENTER CASE SERIES OF 150 PATIENTS WITH RENAL FANCONI SYNDROME

Abstract Background and Aims Renal Fanconi syndrome (RFS) is characterized by generalized dysfunctions of renal proximal tubular (PT) transport. The causes of FS can be inherited or acquired, the latter of which mostly includes drugs, heavy metals, monoclonal light chains (LC), and primary Sj gren’s syndrome (pSS). We intended to observe the clinico-pathological features of RFS with different etiologies. Method From January 2012 to September 2018, all the patients diagnosed as RFS in our hospital were enrolled. Their clinicopathological records were retrospectively reviewed. The diagnosis of RFS was defined as existence of ≥ 3 of the following five items alone or ≥ 2 items combined with an evidence of PT damages in kidney pathology: (I) normoglycemic glycosuria; (II) generalized aminoaciduria; (III) hypophosphatemia and hyperphosphaturia; (IV) hypouricemia and hyperuricosuria; (V) proximal RTA. Results: 1. Clinical characteristics We identified 150 RFS patients, with an average age of (45.9±14.2) years old and male: female ratio 1.3:1. They presented with different degrees of PT dysfunctions and the most common were hypophosphatemia (83.2%) and aminoaciduria (80.6%). Their mean eGFR levels were 76.3 (4.5-188.6) ml/min/1.73m2 with 73.5% had proteinuria. 2. Renal pathological features 47 RFS patients received kidney biopsy and the most common pathological diagnosis was interstitial nephritis. They all presented with different degrees of proximal tubule atrophy, defective brush border, interstitial edema and fibrosis. 3. Etiology-related clinicopathologic features The most common causes of our RFS were LC (14.0%), drugs (13.3%), and pSS (9.3%). Compared to pSS associated RFS, LC associated RFS patients showed a higher prevalence of bone involvement, more severe proteinuria and less severe hypokalemia (P &amp;lt; 0.05) despite similar eGFR levels. Specific renal pathological features were seen in different etiology groups, including crystalline formation and increased lysosomes in PT cells under electron microscope in LC associated RFS, and CD21 positive ectopic germinal center formation in renal interstitum in parts of the pSS associated RFS. Conclusion We identified 150 RFS with different etiologies and they showed etiology-specific patterns of PT dysfunctions and renal pathologic changes.

Clinical and histological features in pediatric and adolescent/young adult patients with renal disease: a cross-sectional analysis of the Japan Renal Biopsy Registry (J-RBR).

Only a few studies have investigated epidemiological and clinicopathological information regarding pediatric and adolescent and young adult (AYA) patients with renal disease. The purpose of this study was to clarify the differences and relationship of clinicopathological findings between pediatric and AYA patients using the Japan Renal Biopsy Registry (J-RBR). This cross-sectional study analyzed data from patients registered in the J-RBR between 2007 and 2017. Clinicopathological findings at diagnosis were analyzed for 3,463 pediatric (age < 15years) and 6,532 AYA (age 15-30years) patients. Although chronic nephritic syndrome was the most common clinical diagnosis at age > 5years, nephrotic syndrome was the most frequent diagnosis at age < 4years. The most common pathological diagnosis as classified by pathogenesis in pediatric patients was primary glomerular disease (except IgA nephropathy), whereas IgA nephropathy was increased in AYA patients. Mesangial proliferative glomerulonephritis was the most common pathological diagnosis as classified by histopathology in both pediatric and AYA patients. Minor glomerular abnormalities were the most frequent histopathologic diagnoses of nephrotic syndrome in childhood, but their frequency decreased with age. To the best of our knowledge, this is the first report of clinicopathological features of pediatric and AYA patients in a large nationwide registry of renal biopsy. There were differences of clinical, pathological and histopathologic findings between pediatric and AYA patients.

Prospective Evaluation of Quality of Life and Functional Outcomes after Carbon Ion Radiotherapy for Inoperable Bone and Soft Tissue Sarcomas.

Simple SummaryQuality of life (QOL) and functional outcomes in patients with inoperable bone and soft tissue sarcoma treated with definitive carbon-ion radiotherapy (CIRT) were prospectively investigated. CIRT showed favorable clinical efficacy and safety, maintaining the physical component of QOL and functional outcomes, and improving the mental component of QOL. The physical component of QOL was positively correlated with functional outcomes. Poor performance status at diagnosis and female gender were independent predictors of the physical component of QOL and functional outcomes after CIRT.Carbon-ion radiotherapy (CIRT) represents a definitive treatment for inoperable bone and soft tissue sarcoma (BSTS). This prospective study analyzed 61 patients with inoperable BSTS who were treated with CIRT to evaluate QOL, functional outcomes, and predictive factors in patients with inoperable BSTS treated with definitive CIRT. The Musculoskeletal Tumor Society (MSTS) scoring system and the Short Form (SF)-8 questionnaire were completed before and at 1, 3, 6, 12, and 24 months after CIRT. The median follow-up period was 38 months. The main site of primary disease was the pelvis (70.5%), and the most common pathologic diagnosis was chordoma (45.9%). The 3-year overall survival and local control rates were 87.8% and 83.8%, respectively. The MSTS score and physical component score (PCS) of SF-8 did not change significantly between the baseline and subsequent values. The mental component score of SF-8 significantly improved after CIRT. Multivariate analysis showed that the normalized MSTS and normalized PCS of SF-8 at the final follow-up were significantly affected by performance status at diagnosis and sex. CIRT showed clinical efficacy, preserving the physical component of QOL and functional outcomes and improving the mental component of QOL, suggesting its potential value for the treatment of patients with inoperable BSTS.

Role of Hysterectomy in Gestational Trophoblastic Neoplasia.

Hysterectomy has a limited role in the management of gestational trophoblastic neoplasia because of the high effectiveness of chemotherapy and the young age of patients. In selected patients, it is believed to help in reducing the number of chemotherapy cycles, overcoming chemo-resistance, and treating acute haemorrhagic events. The present study aimed to evaluate the indications and outcomes of hysterectomy in patients with GTN at a tertiary care centre in India. Between 2012 and 2019, we identified all patients with GTN from the hospital database. Demographic, clinical, and follow-up details of patients who underwent hysterectomy were obtained from the electronic medical records. During the study period, 98 cases of GTN were treated at our centre of which 54% were low-risk and 46% were high-risk cases. Twenty-six patients (26%) underwent hysterectomy as part of their management for GTN. The patients belonging to the high-risk group had more hysterectomies (65%) with an odds ratio of 2.96. The common pathological diagnosis was choriocarcinoma in 44% and an invasive mole in 30% of patients. Bleeding, either intraperitoneal or vaginal, was the most common indication for hysterectomy (48%). The median number of chemotherapy cycles received was 5 in patients who had primary hysterectomy and 6 in patients who did not have hysterectomy. The majority of patients received EMACO (57.7%) chemotherapy. The mean duration of follow-up was 18months (range 1-67).After treatment, complete remission was achieved in 94 out of 98 (95.9%) and also in all patients (100%) who had undergone hysterectomy as adjuvant procedure. Three patients died during treatment (3.06%), all belonging to the high-risk group, and one patient had a recurrence (0.01%). In selected cases of GTN, hysterectomy may be an effective means to reduce or eliminate tumour bulk, to overcome chemoresistance and manage acute bleeding events.

Clinical outcomes of bulky parotid gland cancers: need for self-examination and screening program for early diagnosis of parotid tumors

BackgroundEarly detection and diagnosis of parotid gland cancer (PGC) are essential to improve clinical outcomes, because Tumor-Node-Metastasis stage at diagnosis is a very strong indicator of prognosis in PGC. Nevertheless, some patients still present with large parotid mass, maybe due to the unawareness or ignorance of their disease. In this study, we aimed to present the clinical outcomes of bulky PGC (defined by a 4 cm cutoff point for T3–4 versus T1–2 tumors), to emphasize the necessity of a self-examination tool for parotid gland tumor.MethodsWe retrospectively reviewed 60 consecutive cases with bulky (equal to and greater than 4 cm in the longest diameter, determined radiologically) malignant tumors arising from the parotid gland from 1995 to 2016. The clinical and pathological factors were analyzed to identify risk factors for poor outcomes using Cox proportional hazard models. In addition, we designed a self-examination tool for parotid gland tumors, similar to breast self-examination for breast cancer detection.ResultsPatients with bulky parotid cancer showed 48.9% 5-year and 24.5% 10-year overall survival rates and a 47.9% risk of high-grade malignancy. The common pathological diagnoses were carcinoma ex pleomorphic adenoma (18.3%), adenocarcinoma (16.7%), mucoepidermoid carcinoma (16.7%), salivary duct carcinoma (16.7%), and adenoid cystic carcinoma (11.7%). Survival analyses revealed that tumor size (hazard ratio, HR = 1.262 upon increase of 1 cm, 95% confidence interval, 95%CI 1.059–1.502), lymph node metastasis (HR = 2.999, 95%CI 1.048–8.583), and high tumor grade (HR = 4.148, 95%CI 1.215–14.154) were independent prognostic factors in multivariable analysis. Functional preservation of the facial nerve was possible only in less than half of patients.ConclusionIn bulky PGC, lymph node metastasis at diagnosis and high tumor grade indicated poor survival outcomes, and functional outcomes of the facial nerve were suboptimal. Thus, a public effort seems to be necessary to decrease these patients with bulky PGC, and to increase patients’ self-awareness of their disease. As a way of early detection, we proposed a parotid self-examination tool to detect parotid gland tumors at an early stage, which is similar to breast self-examination.

Retrospective study of Lymphadenopathy by FNAC in National Institute of Health Islamabad-Pakistan

The objective of the study was to review the pathology of lymph node disorders in adults with peripheral lymphadenopathy. A five year (1998 - 2003) retrospective study of lymph node Fine Needle Aspiration Cytology (FNAC) performed at Histopathlogy department of National Institute of Health Islamabad. Females constitute the major group with 130 cases (64%) in our study. Fifty four percent of the cases were below 20 years of age. Benign lesions were found in 91% of the patients, the majority of which were tuberculous lymphadenitis (52%) followed by nonspecific reactive hyperplasia (39%). Frequency of metastatic carcinoma was 9 (4%), malignant lymphoma 2 (1.0%) and insufficient aspirate in 10 (5%) cases. The commonest site of lymphadenopathy was cervical (71%) followed by submandibular (11%), inguinal 8% and axillary 7%. Reactive hyperplasia was more frequent in female (62%) than male patients (38%), similarly tuberculous lymphadenitis was comparatively more frequent in female (64%) than male patients (36%) in our study. Tuberculous lymphadenitis was the most common pathological diagnosis in young females (15 - 25 years). It is concluded that the pattern of disease is similar to that of other countries of the region.