Clinicopathological spectrum and treatment outcomes of cryofibrinogen-associated nephropathies

BackgroundOrbito-ocular tumors are rare, but they cause significant morbidity and mortality. There are reported variations in their pattern of presentation and frequency of occurrence.ObjectiveTo evaluate the occurrence, clinico-pathologic spectrum, and patterns of orbito-ocular neoplasms as well as the treatment and outcome profiles.MethodsA 5-year retrospective analysis of consecutive patients treated for orbito-ocular tumors. We evaluated patterns of occurrence, clinic-pathological concordance, and outcome of treatment. Associations were evaluated with chi square and confidence interval. Data analysis was performed using SPSS for windows version 23 and inferences were judged using the 95% level of significance.ResultsAmong 100 patients with orbito-ocular masses, 62 were histologically confirmed. The female to male ratio (F:M) was 1.0. Their ages ranged from 7 months to 93 years, mean = 33.4 ± 2.7 years. There was an age variation among tumor types. Patients with squamous cell carcinoma (SCC) had a mean age of 46.4 years, while for retinoblastoma the mean age was 3.09 years. All patients with retinoblastoma had proven macroscopic orbital extension. SCC was the most common tumor type (n = 19), however, among children, retinoblastoma (n = 11) was more common. Melanocytic nevus, sebaceous gland carcinoma, and adenocarcinoma of lacrimal gland (n = 6, n = 5, n = 5), respectively, among other tumors were treated. Concordance between clinical and histopathological diagnoses was obtained among 30 (48.3%) cases. 1-year and 3-year survival for retinoblastoma was 90% and 72.9%, respectively, and 78.9% and 68.4% for SCC.ConclusionSCC is currently the most common orbito-ocular tumor in our setting.

Primary intradural and dumbbell spinal tumours, are most commonly found in the thoracic region. The presentation, diagnosis, and management are at times challenging. The primary treatment of choice is surgical resection; however, this could be challenging in resource-limited settings, especially for intramedullary tumours. A retrospective study of patients with primary intradural and dumbbell spinal tumours. The age, sex, and clinical presentation, duration of symptoms, spinal region, anatomic classification, the extent of resection, histology, and outcome of these patients were assessed, analysed, and presented. There were 31 patients comprising of 17 females and 14 males, respectively. The age range was 11 months to 76 years (mean age of 39.3y). The thoracic spine was the most commonly affected (20 patients, 64.5%). The most common initial presenting complaints were: limb weakness/paralysis (87.5%) for the intramedullary group, axial pain (88.9%) for the intradural extramedullary group, and nonradicular pain (60%) for the dumbbell group. Primary intradural and dumbbell spinal tumours are mostly benign or low-grade tumours and are commonly found in the thoracic spine. They are individually unique with respect to their diverse clinical presentations and peculiar histologic types. These eventually determine their treatment outcomes and quality of life.

In high-income countries (HICs) 17-20% of colorectal cancer (CRC) patients have metastatic CRC (mCRC) at the time of diagnosis, of which 10-25% are or become resectable, and a further 4-11% of patients will develop metachronous metastases. The study aimed to establish the prevalence and pattern of metastatic CRC to document treatment outcomes in KwaZulu-Natal (KZN), and to compare results to international norms. The study population comprised patients with mCRC presenting between 2000 and 2019. Demographics, primary tumour site, spectrum of metastatic disease and resection rate were assessed. MCRC occurred in 33% of the CRC patient population. Eight hundred and thirty-six patients had metastatic disease, comprising Africans (325, 38.8%), Indians (312, 37.3%), Coloureds (37, 4.4%) and Whites (161, 19.2%). Six hundred and fifty-four patients (79%) had synchronous metastases and 182 patients had metachronous metastases (21%). Single organ metastases occurred in 596 patients (71.2%) (M1A) and multiple organ metastasis occurred in 240 patients (28.7%) (M1B). Metastases occurred in the liver (613), lung (240) and peritoneum (85). Fifty-two patients (6.2%) underwent resection of their metastases. The prevalence of stage IV CRC in our setting is at the upper limit of international norms. mCRC occurred in 33%, with similar proportions in all races. Resection rate for metastases is low.

BackgroundThe WHO-fifth edition classified the sinonasal respiratory epithelial malignancies (SREMs) based on their histomorphological, immunohistochemical, and molecular features, due to their diverse manifestations.AimsWe aimed to ambispectively reclassify the SREMs at a North Indian tertiary care center, to understand the clinico-pathological spectrum of the WHO-defined entities and their prognostic implications.Material and MethodsHistopathologically proven, 162 patients with SREMs from 8 years (2016-2023) were retrieved, and re-examined for histomorphology, and relevant immunohistochemistry was performed to reclassify them. The clinical details and the follow-up data were also retrieved.ResultsKeratinizing squamous cell carcinoma (KSCC) was the most common sinonasal carcinoma (n = 76; 46.9%), followed by non-keratinizing squamous cell carcinoma (NKSCC) (n = 29; 17.9%), sinonasal undifferentiated carcinoma (SNUC) (n = 23; 14.2%), sinonasal adenocarcinoma (n = 22; 13.6%), SWI/SNF-deficient carcinoma (n = 6; 3.7%), lymphoepithelial carcinoma (n = 2; 1.2%), NUT carcinoma (n = 2; 1.2%), and teratocarcinosarcoma (n = 2; 1.2%). The median age of presentation of SREMs was 54 (IQR 45-62) years, with a male:female ratio of 2.4:1. Sinonasal adenocarcinoma showed a significantly lower median age of presentation (43.5 years) (P-value = .036). SWI/SNF-deficient carcinoma, NUT carcinoma, and teratocarcinosarcoma showed dismal prognosis irrespective of treatment. The median overall survivals of KSCC, NKSCC, SNUC, and sinonasal adenocarcinoma were 31, 63, 17, and 53 months, respectively, and showed a significant difference (P -value = .047).ConclusionReclassification of the SREMs according to the WHO-fifth edition elucidated their clinico-pathological spectrum and prognosis. This study highlights the relevance of the accurate classification of these entities for accurate treatment options and prognostication.

Clinicopathological spectrum and treatment outcome of idiopathic steroid-resistant nephrotic syndrome in children at a tertiary care center

Introduction:Disease spectrum of upper tract transitional cell carcinoma (TCC) in Indian patients is not known. Herein, we present data on clinical presentation, pathological characteristics, and the outcome of treatment of upper tract TCC.Materials and Methods:Clinicopathological data of patients who were diagnosed for upper tract TCC between January 2000 and January 2010 were collected from the hospital information system and case records. Preoperative diagnosis was based on contrast-enhanced computerized tomography of the whole abdomen and urine cytology. Cross tab and logistic regression analysis was done on the effect of various clinicopathological characteristics on the outcome and cancer-specific and recurrence survival were derived.Results:There were total 40 patients, 35 (87.5%) of them were male. The mean age was 62.7 ± 7.9 years. The most common symptom was gross hematuria present in 30 (75%). Mean tumor size was 2.8 ± 1.2 cm. Median duration of follow up was 36 (12 to 100) months. Laparoscopic nephroureterectomy was done in 27 patients along with bladder cuff excision and seven patients underwent open surgery. Thirty two (88.8%) patients had invasive T stage and high-grade lesions were seen in 24 (66.6%). Lymphovascular invasion was found only in one case and necrosis in 30 (83.3%). Necrosis was found to be the poor prognostic factor. Five-year recurrence-free and cancer-specific survivals were 36.33% and 26%, respectively.Conclusion:Patients with upper tract TCC present very late with a high-stage disease and a very low 5-year cancer-specific and recurrence-free survivals.

Background and Aim: Rapidly progressive glomerulonephritis (RPGN) is characterized by a rapid decline in the renal function and urinary abnormalities. There is limited information on epidemiological factors and clinical and histopathological patterns of RPGN from developing countries. Therefore, the objective of this study was to identify the etiology, clinical features, histopathological patterns, and treatment outcomes of patients with clinically suspected RPGN. Methods: This retrospective study was conducted in the Pediatric Nephrology Department of Bangabandhu Sheikh Mujib Medical University from January 2014 to January 2019. Patients with clinically suspected RPGN that underwent renal biopsy were enrolled in this study. Results: Thirty-five patients were recruited in this study. Macroscopic hematuria, edema, hypertension, uremia, and oliguria were common clinical presentations. Diffuse proliferative GN (28.5%) and crescentic GN (22.8%) were the most common histological diagnoses in this study. Immune mediated GN (62%) followed by idiopathic GN (25%) were found to be the most frequent cause of crescentic GN. Renal replacement therapy was required in 45% of the cases and 11.4% of the patients developed end-stage renal disease.Conclusion: Renal histology is an integral part of the investigation of patients with suspected RPGN for both diagnostic and prognostic purposes. Diffuse proliferative GN was the most common histopathological diagnosis in patients with clinical RPGN in our population. Preservation of renal function depends on early intervention and detection of RPGN in pediatric patients.Keywords: Nephritis; Glomerulonephritis; ESRD; End-Stage Kidney Disease; Child.

Primary tumors of the central nervous system are relatively uncommon, comprising only 1%-2% of all neoplasms. However, they constitute the second most common type of malignancy in children (after leukemia) and the leading cause of cancer-related morbidity and mortality in children and young adults worldwide. Globally, there is substantial variability with nearly five-fold difference in incidence between various parts of the world. Brain tumors are quite heterogeneous with regard to histology, biological behavior, and prognosis mandating multidisciplinary therapeutic decision-making. This retrospective audit of all consecutive patients registered in a single calendar year (2013) in the neuro-oncology disease management group at Tata Memorial Centre is reflective of the ground reality and fair representation of outcomes in routine neuro-oncologic practice.

Objective:Although till date no management protocol for esthesioneuroblastoma (ENB) has been standardized due to tumor rarity, still multimodality approach shows better treatment outcomes as compared to surgery alone. The objective of this study was to analyze the clinicopathological spectrum of ENB and to correlate treatment response with tumor staging, histopathological grading, and various treatment modalities.Materials and Methods:Twenty-one consecutive patients with biopsy-proven ENB were studied and evaluated for response to treatment in the form of complete tumor resolution. Results were analyzed and correlated with stage and grade of tumor and form of therapy received.Results:There was male preponderance (3.2:1) with age ranging between 7 and 63 years (median of 25 years). Survival rates significantly dropped with increasing tumor stage (63.6% in stages A and B vs. 30% in stages C and D) and grade (100% in Grades 1 and 2 vs. 31.25% in Grades 3 and 4). The recurrence rate was 80% in surgery alone group, which came down to 43.7% if surgery was supplemented with other modalities. In cases where multimodality treatment plan was used, endoscopic procedures fared equally as open surgical procedures.Conclusion:Hyam's grade and Kadish stage are important prognostic indicators of treatment outcome, with survival rates dropping with increasing tumor stage and grade. Multimodality treatment protocols have improved the disease outcome, making endoscopic surgery equivalent to radical surgeries regarding result outcomes and giving other advantages such as better cosmesis, less treatment-related morbidities, decreased hospital stay, and better cost-effectiveness.

To study the clinico-pathological characteristics of primary ovarian malignant mixed mullerian tumor (OMMMT) and assess the prognostic factors associated with treatment outcome and survival. The pathology database was searched for primary ovarian carcinosarcoma diagnosed and/or managed at our institute from period of January 2004 to July 2010. The histological sections were reviewed, with emphasis on type and grade of epithelial and sarcomatous components. The medical records were retrospectively analyzed for clinical details and follow up. A total of 27 cases of primary ovarian carcinosarcoma were identified. The median age at diagnosis was 51 years. Fourteen patients had advanced stage (stage III and IV) at presentation. Cytoreductive surgery was done in 18 cases, and 7 had received upfront chemotherapy. Histologically, 10 cases had epithelial predominance (> 50% epithelial component) and 11 had sarcoma predominance. The most frequent epithelial component was endometroid type, and most common sarcoma component was rhabdomyosarcomatous. Hyaline droplets within sarcomatous stroma were seen prominently in 15 cases. Three cases showed germ cell /yolk sac-like areas. Eighteen cases had follow up with a median of 15 months (4-40 months). The recurrence-free survival in advanced stage and sarcoma predominant was 10.5 months in comparison to 13 months in early stage and epithelial predominant OMMMT. Primary ovarian carcinosarcoma is a rare biphasic malignancy with variable proportions of epithelial and spindle elements. Presence of hyaline droplets within spindle sarcoma in a biopsy from ovarian mass should alert the pathologists regarding MMMT. Advanced stage, suboptimal cytoreduction, and sarcoma predominant tumors are likely to have a worse outcome in ovarian MMMT.

ObjectiveThis study aimed to evaluate the epidemiological and clinicopathological spectrum of ocular malignancies among patients presenting to a teaching hospital in Northern India.MethodsA total of 246 histopathologically diagnosed patients with ocular malignancies were included in the study. Tumor type and size, primary origin and location of tumor, clinical staging, radiological findings, histopathological type, and treatment outcomes were assessed.ResultsOverall, males over 55 years of age were most commonly affected and the majority of cases were primary ocular or adnexal malignancies (n = 226; 91.87%). The eyelids and periocular structures (n = 92; 37.40%) were the most commonly involved site, followed by the orbit (n = 72; 29.27%), ocular surface (n = 46; 18.70%) and intraocular region (n = 36; 14.63%). The majority of the patients (n = 68; 27.64%) were managed by primary surgical excision and reconstruction. However, 46 patients (18.70%) with advanced lesions underwent neoadjuvant chemotherapy followed by surgical excision and more extensive orbital lesions were treated by exenteration followed by adjuvant chemotherapy (n=48; 19.51%), while patients with metastatic tumor were given palliative chemotherapy/external beam radiation therapy (n= 46; 18.70%). Overall, 45.12% of patients were cured completely, 15.45% showed a partial response to the treatment, 13.04% had progressive disease and 16.67% demonstrated disease recurrence.ConclusionA clinicopathological analysis of ocular malignancies at a teaching hospital in Northern India indicated the preponderance of primary ocular malignancies, with eyelid sebaceous gland carcinomas being the most common pathological diagnosis. Most of our patients had advanced and extensive disease among them majority belonged to the rural background and poor socio-economic status.

Surgical therapy of mid-stage gastric cancer (GC) and other neoplastic conditions requiring gastric resection remains at the center of curative outcomes, while epidemiologic changes and multimodality treatment options have evolved rapidly. Putative quality metrics for gastrectomy such as R0 rate, total lymph node (LN) count or postoperative morbidity may depend partly on changing disease and treatment patterns, and deserve evaluation under various practice conditions. Data within a U.S.-based single surgical oncologist's practice over 15 years were prospectively recorded and retrospectively analyzed for clinicopathologic factors, operative treatment aspects and outcomes. Trends and spectrum changes over three time intervals were analyzed with contingency analysis and continuous data comparative statistics. Of 179 patients undergoing gastric resection, 119 were male and 60 female, with a median age of 63 years (range, 24-98 years). Resections included 56 total, 56 subtotal/distal, 30 proximal and 37 segmental gastrectomies. Diagnoses included 96 GCs, 31 gastroesophageal (GE) junction (GEJ) cancers, 21 GI stromal tumors (GISTs), and 31 other conditions. Significant trends from first towards last time interval were observed for resection type (16% to 32% proximal, 9% to 30% segmental, P=0.0003), curative intent (76% to 98%, P=0.002), diagnosis (5% to 42% GEJ cancer, P<0.0001) and preoperative therapy use (0% to 58%, P<0.0001), among others. Intraoperative aspects showed significantly reduced blood loss (median: 500 to 150 mL) and transfusion requirements (39% to 4%), and an increased use of minimally invasive techniques over time (all at P<0.001). Among patients undergoing curative intent GC resection with LN dissection, total LN counts remained steady (mean: 26), while the number of involved LNs decreased (9.0 to 3.7, P=0.0003) and the R0 resection rate increased from 74% to 85% (P=0.05). The number of specimens with >15 LNs examined increased from 69.0% to 92.5% (P=0.022). At the same time, spleen preservation rate (91% overall) and major morbidity (16%) remained unchanged throughout. Postoperative length of stay decreased from a median of 12 to 8 days (P<0.0001). This experience represents some variable practice patterns within a clinicopathologic spectrum of GE diseases. Postoperative or oncologic quality metrics have been sustained or did improve, which would support their utility for various practice settings; they compare favorably to other published U.S. experiences during the same time period.

Xanthogranulomatous pyelonephritis (XGP) is an uncommon and distinct type of chronic infective pyelonephritis causing destruction of the kidney, severely affecting the renal function. The perinephric adipose tissue and peritoneum is not uncommonly involved. The study was undertaken to decipher the clinicopathologic spectrum of XGP. Forty cases of XGP were diagnosed on histopathology over a period of 13 years (2005–2017). Relevant clinical details and radiological findings were recorded from the case files. Out of a total of 40 cases, 26 were female and 14 were male with a mean age of 39.5 ± 13.6 years. Flank pain was the most common presenting symptom. All the patients had unilateral disease and underwent nephrectomy for a nonfunctional kidney. Gross examination showed enlarged kidney with replacement of cortico-medullary tissue by yellow nodular areas of fatty tissue and dilatation of the pelvicalyceal system. Thirty-six (90%) cases had nephrolithiasis. Histologically, the characteristic feature was the existence of lipid-laden foamy macrophages. Renal parenchymal involvement was diffuse in majority (31, 77.5%). Two (5.0%) of the patients had coexisting carcinoma in the same kidney. Histopathologic examination gives the definitive diagnosis of XGP which relies on the characteristic morphology. Surgical intervention in the form of nephrectomy is the treatment of choice and offers good treatment outcomes.

Full-house staining of glomeruli in renal pathology is highly suggestive of lupus nephritis. Other nonlupus entities can also present with a similar pattern on immune fluorescence. Different authors have used different names for this new entity with full house staining on immunofluorescence (IF) with negative serology for lupus. Some authors used the term full-house nephropathy for this new entity. The aim of our study is to define the clinicopathological spectrum and treatment outcomes of nonlupus "full-house" patterns. We retrospectively reviewed all renal biopsies performed between 2013 and 2017 in the nephrology department in a tertiary teaching hospital in south India. A total of 12 patients were found with full-house staining on IF, not fulfilling the American College of Rheumatology criteria for SLE. Out of 12 patients, eight patients (66%) presented with features suggestive of both nephrotic and nephritic syndrome, one patient (8%) with subnephrotic proteinuria, one patient (8%) with rapidly progressive glomerulonephritis, one patient (8%) with pure nephrotic syndrome, and one patient (8%) with pure nephritic syndrome. The most common histopathology pattern observed was diffuse proliferative glomerulonephritis (58%), followed by membranous nephropathy (16%), membranoproliferative glomerulonephritis (16%), and mesangioproliferative glomerulonephritis (8%). Irrespective of treatment regimen given, six patients (50%) achieved complete remission, three patients (25%) achieved partial remission, and three patients (25%) did not attain remission at the end of six months. Only one patient became ANA positive during follow-up. Thus, we can conclude that varied glomerular pathologies can occur with full house pattern on IF which respond well to immunosuppression.

BACKGROUND: Delay in operative management of small bowel obstruction (SBO) results in increased morbidity and mortality. The objective was to evaluate clinical presentation and treatment outcome of SBO METHOD: Prospective cohort study between 2013-2014. Adult patients presenting with SBO were included. Demographics, clinical details, investigations, operative findings, in-hospital progress and outcomes were documented RESULTS: There were 156 patients (median age 37 [IQR 27-54 years]) with early (44) and delayed (112) presentation. :F ratio was 1.4:1. Common causes of obstruction were adhesions (94; 60.3%) and hernias (31; 19.9%). Non-operative management was feasible in 59 patients (37.3%) with a success rate of 71.2%. Nonviable bowel was present in 45.1% (early 31%, delayed 50%; p = 0.078). Sixty-one patients (54%) underwent bowel resection; nine patients (20.5%) in the early presentation group and 52 (46.4%) in the delayed group (p = 0.003). Thirty-one patients needed ICU admission (early 5, delayed 26; p = 0.091). The delayed group had longer ICU stay (p = 0.018) and longer hospital stay (p &lt; 0.001). There were more complications (p = 0.084) and re-laparotomies (p = 0.156) in the delayed group. Eight patients died (5.1% CONCLUSION: The main causes of SBO were adhesions and hernias. Late presentation was associated with higher resection rate, higher critical care admission and longer hospital stay Keywords: small bowel obstruction, adhesive bowel obstruction, acute abdomen