Hemophagocytic lymphohistiocytosis complicating septicemic melioidosis: A case report.

Melioidosis is an infectious disease caused by Burkholderia pseudomallei. People infected with B. pseudomallei experience fever and skin changes, pneumonia, abscesses, and septic shock that could cause death. Hemophagocytic lymphohistiocytosis is a severe inflammatory syndrome due to the excess activation of macrophages and T cells. We report a 50-year-old hypertensive and diabetic male patient presented with high-grade intermittent fever with loss of appetite and weight loss for two months and a history of jaundice, backache and swelling of both feet for 15 days. Blood and bone marrow culture grew Burkholderia pseudomallei. A liver biopsy revealed Kupffer cell hyperplasia and hemophagocytosis. The patient was treated with an injection of dexamethasone 4mg intravenous three times a day for five days and tapered over 15 days with ceftazidime 2 gm intravenous three times a day for six weeks. Early suspicion in the diagnosis of hemophagocytic lymphohistiocytosis in septicemia can prevent severe complications, even death.

Emerging insights into cephalic neural crest disorders: A single center experience.

Objectives: Neural crest cells (NCCs) are transient structures in the fetal life in vertebrates, which develop at the junctional site of the non-neural and neural ectoderm, sharing a common developmental origin for diverse diseases. After Epithelio-mesenchymal (EMT) of the NCCs within the neural tube, delamination of NCCs occurs. After delamination, the transformation of these cells into various cell lineages produces melanocytes, bones, and cartilage of the skull, cells of the enteric and peripheral nervous system. After the conversion, these cells migrate into various locations of the entire body according to the cell lineage. Abnormalities in neural crest (NC) formation and migration result in various malformations and tumors, known as neurocristopathy. Material and Methods: Herein, this case series describes a single-center experience in cephalic NC disorders over the past 3 years, including 17 cases of varying composition (i.e., vascular, dysgenetic, mixed, and neoplastic forms) involving the brain and occasionally skin, eyes, and face of the patients. Results: In our study of 17 patients with cephalic NC disease, 6 (35.3%) patients had vascular form, 5 (29.4%) had dysgenetic form, 4 (23.5%) had mixed form, and 2 (11.7%) had neoplastic form. Brain involvement in the form of vascular or parenchyma or both vascular and parenchymal was seen in all of our patients (100%), skin in 6 (35.3%) patients, eye in 2 (11.7%), and face in 1 (5.9%) patient. Treatment was planned according to the various manifestations of the disease. Conclusion: Neural crest diseases (NCDs) are a rare and under-recognized group of disorders in the literature and may have been under-reported due to a lack of awareness regarding the same. More such reporting may increase the repertoire of these rare disorders such that clinicians can have a high degree of suspicion leading to early detection and timely counseling and also improve preventive strategies and help in developing new drugs for these disorders or prevent them.

Effect of Preoperative Body Mass Index on Postoperative and Long-Term Outcomes in an East Indian Gastric Cancer Cohort.

BACKGROUND: Gastric cancer is a global health concern with varying clinical outcomes. This study aims to investigate the influence of preoperative Body Mass Index (BMI) on survival in patients who underwent curative resection for gastric cancer in Eastern India. METHODS: Data from a prospectively maintained Surgical Oncology database were analysed for patients who underwent curative resection for primary gastric adenocarcinoma between May 2016 and March 2022. Patients with incomplete data were excluded. Preoperative BMI was categorised into three groups: Underweight (< 18.5 kg/m2), Normal (18.5-22.9 kg/m2), and Overweight/Obese (=23 kg/m2). Clinicopathological details, short-term outcomes, and long-term oncological outcomes were assessed. Statistical analysis included survival estimates, Cox proportional hazard models, and subgroup analysis. RESULT: Of 162 patients, 145 met the inclusion criteria. Patients were predominantly male (68%) with middle or lower socioeconomic status. No significant differences amongst BMI groups were observed in performance score, tumour grade, clinical stage, or short-term outcomes. Postoperative complications and 30-day mortality were similar. However, underweight patients had poorer 4-year disease-free survival (DFS) compared to overweight/obese patients (14.3% vs. 39.7%, p = 0.03). Overweight/obese patients showed significantly better 4-year overall survival (OS) than underweight patients (47.8% vs. 20.4%, p = 0.03). CONCLUSIONS: In Eastern Indian gastric cancer patients undergoing curative resection, preoperative higher BMI (overweight/obese) was associated with better long-term survival. Understanding these findings could guide tailored interventions to improve outcomes in this population.

CK7+/CK20+/CDX2+ mucinous carcinoma, arising in a mature cystic teratoma of ovary, presenting with disseminated metastasis and pseudomyxoma peritonei.

ABSTRACT: Mucinous carcinomas arising within an ovarian dermoid tumor are rare. Most of the cases reported in the literature show morphological features resembling an appendiceal mucinous neoplasm. They exhibit CK7-/CK20+ immunophenotype similar to carcinomas of the lower gastrointestinal tract. In this report, we have described a case of a well-differentiated mucinous carcinoma arising within a mature cystic teratoma. The mucinous carcinoma showed a spectrum of morphological patterns, including cystadenoma-like areas, proliferative/borderline areas, villous adenoma-like areas, and areas of invasive carcinoma. In addition, our case showed some unusual findings, namely, diffuse CK7 positivity, associated pseudomyxoma peritonei, and metastasis to lungs. These features were not demonstrated in any of the previously reported cases. Our case shows that the teratomatous mucinous neoplasm of the ovary may show CK7+/CK20+/CDX2 + immunoprofile making it immunohistochemically indistinguishable from a primary ovarian mucinous neoplasm or a metastatic mucinous carcinoma of the lower gastrointestinal tract.

Hepatic Vascular Variants in Hereditary Haemorrhagic Telangiectasia: Imaging findings.

Hereditary haemorrhagic telangiectasia (HHT) is an autosomal dominant disorder characterised by vascular dysplasia. Hepatic vascular malformations (VMs) range from small telangiectases to significant vascular shunting. Here we report two cases of HHT. Case 1 had diffuse ectasia of the hepatic artery along its intrahepatic and extrahepatic course with a hepatic arterial aneurysm. Case 2 presented with ileal and hepatic telangiectases. Knowledge of these vascular variants is indispensable for clinicians and radiologists in aiding diagnosis and surgical and interventional management.

Uterine Artery Pseudoaneurysm after Cervical Cerclage: A Rare Case and Its Management Through Uterine Artery Embolization.

Background: Uterine arterial pseudoaneurysm (UAP) is a rare cause of vaginal bleeding. Usually, UAP follows cesarean section, uterine curettage, vaginal delivery, or cervical conization and must be considered in the differential diagnosis. Here we report a very unusual case of UAP causing intra-abdominal and vaginal bleeding after cervical cerclage and its successful management through the endovascular route. Case Presentation: This 30-year-old P2L1A2 woman presented with vaginal bleeding after 7 days following hysterotomy for termination of pregnancy. At 16 weeks of her index pregnancy, she underwent cervical cerclage for cervical insufficiency, followed by an emergency hysterotomy at 20 weeks for profuse vaginal bleeding after failed medical management. On ultrasound and contrast-enhanced computed tomography (CECT), uterine dehiscence and a cervical pseudoaneurysm were detected. Dehiscence was repaired surgically, and pseudoaneurysm was successfully managed by uterine artery embolization. Conclusion: UAP is an uncommon cause of vaginal bleeding; however, it should be considered in the differential diagnosis of a woman with unusual vaginal bleeding, particularly in the postpartum or postoperative setting like cesarean section, uterine curettage, cervical conization, or cerclage. Endovascular uterine artery embolization is a minimally invasive, effective, preferred, and safe method for managing UAP.

Solitary Fibrous Tumor of the Kidney With Pure Round Cell Features: A Case Report With Review of Literature.

Solitary fibrous tumor (SFT) is a rare mesenchymal neoplasm known to occur at various soft tissue and visceral locations. Kidney is a rarely reported site for these tumors. Most of the SFTs described in the kidney exhibit a classical CD34-positive patternless spindle cell histology. Focal round cell morphology is seldom reported. Herein, we describe a 48-year-old male patient with renal SFT. This tumor had pure round cell morphology with a CD34-/STAT6+ immunophenotype. Fluorescent in situ hybridization and a multiplexed sequencing assay performed on an Illumina® HiSeq 4000 platform revealed NAB2 and STAT6 gene rearrangement. Renal tumors with round cell morphology are diagnostically challenging and SFT is not often considered in the differential diagnosis of a round cell tumor of the kidney. Moreover, a CD34-negative profile can be rather confounding while diagnosing such lesions. In such scenarios, a strong nuclear STAT6 immunostaining is extremely helpful in clinching the diagnosis. SFT should always be considered in the differential diagnosis of round cell tumors of the kidney due to significant diagnostic and therapeutic implications.

Giant Cell Arteritis Masquerading As Migraine: A Case Report.

Giant cell arteritis, or temporal arteritis, is a chronic granulomatous vasculitis that affects large- and medium-sized arteries. An elderly male of 61 years presenting with chronic headaches for the past one year had been misdiagnosed as having migraine because of the similarity in symptoms. General examination revealed the presence of bilateral large, tortuous temporal arteries without any scalp tenderness, diminished arterial pulsations, or skin changes over the dilated arteries. A temporal artery biopsy revealed giant cell arteritis and was treated with steroids. This case report highlights the importance of considering secondary headaches, especially giant cell arteritis, in the differential diagnosis of new-onset headaches or worsening headaches in the elderly.

Therapeutic potential of urine exosomes derived from rats with diabetic kidney disease.

Kidney disease is prevalent in diabetes. Urinary exosomes (uE) from animal models and patients with Diabetic nephropathy (DN) showed increased levels of miRs with reno-protective potential. We examined whether urinary loss of such miRs is associated with their reduced renal levels in DN patients. We also tested whether injecting uE can leverage kidney disease in rats. In this study (study-1) we performed microarray profiling of miRNA in uE and renal tissues in DN patients and subjects with diabetes without DN (controls). In study-2, diabetes was induced in Wistar rats by Streptozotocin (i.p. 50 mg/kg of body weight). Urinary exosomes were collected at 6th, 7th and 8th weeks, and injected back into the rats (100ug/biweekly, uE-treated n=7) via tail vein on weeks 9 and 10. Equal volume of vehicle was injected in controls (vehicle, n=7). uE from the human and rat showed the presence of exosome-specific proteins by immunoblotting. Microarray profiling revealed a set of 15 miRs having high levels in the uE, while lower in renal biopsies, from DN, compared to controls (n=5-9/group). Bioinformatic analysis also confirmed the Renoprotective potential of these miRs. Taqman qPCR confirmed the opposite regulation of miR-200c-3p and miR-24-3p in paired uE and renal biopsy samples from DN patients (n=15), relative to non-DN controls. A rise in 28 miRs levels, including miR-200c-3p, miR-24-3p, miR-30a-3p and miR-23a-3p were observed in the uE of DN rats, collected between 6th-8th weeks, relative to baseline (before diabetes induction). uE- treated DN rats had significantly reduced urine albumin-to-creatinine ratio, attenuated renal pathology, and lower miR-24-3p target fibrotic/inflammatory genes (TGF-beta, and Collagen IV), relative to vehicle treated DN rats. In uE treated rats, the renal expression of miR-24-3p, miR-30a-3p, let-7a-5p and miR-23a-3p was increased, relative to vehicle control. Patients with diabetic nephropathy had reduced renal levels, while higher uE abundance of miRs with reno-protective potential. Reverting the urinary loss of miRs by injecting uE attenuated renal pathology in diabetic rats.

Human umbilical cord blood-mesenchymal stem cell derived exosomes as an efficient nanocarrier for Docetaxel and miR-125a: Formulation optimization and anti-metastatic behaviour.

AIM: Exosomes, as a nanocarrier for the co-delivery of biologicals and small anticancer molecules is yet in its infancy. Herein, we investigated hUCBMSC derived exosomes as a biogenic nanocarrier for the co-delivery of tumor suppressor miR-125a and microtubule destabilizing Docetaxel (DTX) to target the proliferative and migratory aggressiveness of the murine TNBC 4T1 cells. MAIN METHODS: In this study, hUCBMSCs from the human umbilical cord blood cells (hUCB) were successfully transfected with miR-125a. Thereafter, DTX was encapsulated into both non-transfected and transfected exosomes by optimized mild sonication-incubation technique. The anticancer efficiency of hUCBMSC Exo-DTX and miR-125a Exo-DTX was compared by MTT and morphometric assay. The prominent anti-metastatic behaviour of the latter was confirmed by in-vitro wound healing and transwell invasion assay. Further, the synergistic effect of miR-125a and DTX was confirmed by F-actin and nuclear degradation by confocal and FESEM assay. KEY FINDINGS: hUCBMSC exosomes exhibited DTX payload of 8.86 ± 1.97 ng DTX/ µg exosomes and miRNA retention capacity equivalent to 12.31 ± 5.73 %. The co-loaded formulation (miR-125a Exo-DTX) exhibited IC50 at 192.8 ng/ml in 4T1 cells, which is almost 2.36 folds' lower than the free DTX IC50 (472.8 ng/ml). Additionally, miR-125a Exo-DTX treatment caused wound broadening upto 6.14±0.38 % while treatment with free DTX and miR-125a exosomes alone caused 18.71±4.5 % and 77.36±10.4 % of wound closure respectively in 36 h. miR-125a Exo-DTX treatment further exhibited significantly reduced invasiveness of 4T1 cells (by 3.5 ± 1.8 %) along with prominent cytoskeletal degradation and nuclear deformation as compared to the miR-125a exosomes treated group. The miR-125a expressing DTX loaded exosomal formulation clearly demonstrated the synergistic apoptotic and anti-migratory efficiency of the miR-125a Exo-DTX. SIGNIFICANCE: The synergistic anticancer and anti-metastatic effect of miR-125a Exo-DTX was observed due to presence of both DTX and miR-125a as the cargo of hUCBMSC derived exosomes.

Left Gastric Artery Pseudoaneurysm Complicating Chronic Calcifying Pancreatitis in a Child.

A left gastric artery pseudoaneurysm is a rare complication of pancreatitis and is associated with significant morbidity and mortality. We report a 14-year-old male with severe abdominal pain and a palpable upper abdominal mass, earlier diagnosed as chronic idiopathic calcifying pancreatitis, and awaiting surgical intervention. Computed tomography showed a pseudocyst and a pseudoaneurysm in the lesser sac near the left gastric artery. The patient underwent successful angiographic coiling of the left gastric artery and definitive pancreatic surgery weeks thereafter. The early detection and interventional radiologic management of the vascular complication averted a life-threatening hemorrhage without emergency surgery in a pediatric patient.

Solitary Fibrous Tumor of the Adrenal Gland.

OBJECTIVES: Solitary fibrous tumor (SFT) is a mesenchymal neoplasm that can arise at various anatomic locations. It is characterized by inv12(q13q13)-derived NAB2::STAT6 fusion, resulting in the nuclear expression of STAT6. Primary SFT of the adrenal gland is rare. We launched a multi-institutional collaboration to comprehend the overarching demographics, clinical and follow-up, macroscopic, microscopic, IHC, and FISH features of 9 patients with SFT of the adrenal gland. METHODS: We added a series of 9 patients to the collection of adrenal SFTs where the clinicopathologic parameters, including clinical presentation, imaging, histopathology, IHC, molecular profiles, and management and follow-up data, were analyzed comprehensively. A modified 4-variable risk stratification model, including age, tumor size, and necrosis, was applied. RESULTS: Our series consisted of 6 male and 3 female patients, ranging in age from 19 to 64 years (mean, 49.3 years). Abdominal pain (4) and fever with abdominal pain (1) were the presenting symptoms in 5 patients. In the remaining 4 patients, the tumors were detected by abdominal imaging for hypertension and diabetes. The size of the tumor ranged from 2 cm to 10.5 cm in maximum dimension. All tumors exhibited the morphology of a spindle cell SFT with a patternless architecture; 3 had a focal storiform arrangement. STAT6 positivity was observed in all tumors, and 7 were positive for CD34. Surgical resection was the primary modality of treatment. No adjuvant therapy was administered. Follow-up ranging from 7 months to 23 months was available for 7 patients. All were alive without disease recurrence or metastasis. Risk stratification placed 8 (88.9%) patients into a low-risk category and 1 into an intermediate-risk category. CONCLUSIONS: This series is the largest of adrenal SFTs to date. These tumors of the adrenal gland are predominantly spindle cell neoplasms with indolent behavior, with a wide age distribution and a slight male preponderance. Combining our cohort with the previously published cases, the majority of tumors fall into the low-risk category for the propensity to develop metastases. Owing to the rarity and age distribution associated with these tumors, the differential diagnosis is wide and requires a systematic approach for ruling out key differential diagnoses aided by STAT6 IHC.

Hydatid cyst of femur, radiologically mimicking a sarcoma.

Hydatid disease is a rare pathology, but relatively common in the Mediterranean, the Middle East, Central Asia, and East Africa. It may develop in almost any part of the body, although most hydatid cysts occur in the liver or in the lung. Bone involvement is rare, and accounts for only 0.5% to 4% of all locations. Most of the hydatid cyst are diagnosed on the basis of their clinical and radiological presentation. However, in cases with an atypical presentation, FNAC may be used to evaluate the nature of lesion.

Reticular schwannoma mimicking myxoid sarcoma.

Reticular/microcystic schwannoma is a recently described rare distinctive variant of schwannoma with a predilection for gastrointestinal tract. Its occurrence in soft tissue is extremely rare. We report a case of reticular/microcystic variant of schwannoma in a 55-year-old Indian woman who presented with a painless slow-growing swelling in the right forearm for the past 6 months. MRI findings suggested myxoid sarcoma. However, histological and immunohistochemical findings in the excised specimen were consistent with reticular/microcystic variant of schwannoma. This case report emphasises that the diagnosis of reticular/microcystic schwannoma should always be considered in cases where myxoid sarcomas are suspected as it can mimic malignant myxoid sarcomas, clinically and radiologically, thereby avoiding aggressive intervention and overtreatment.