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ABSTRACT Focal Cortical Dysplasia (FCD) is a group of focal developmental malformations of the cerebral cortex cytoarchitecture. FCD usually manifests as medically intractable epilepsy, especially in young children. Live patients are diagnosed by radiological examination such as magnetic resonance imaging (MRI), fluorodeoxyglucose positron emission tomography (FDG PET), magnetoencephalography (MEG), diffusion-tensor imaging (DTI), and intracranial electroencephalogram (EEG). While some cases can be missed by radiological examination, they are usually diagnosed on the histopathological examination of the surgically removed specimens of medically intractable epilepsy patients. We report a case of a young girl with cerebral palsy, mental retardation, and seizure disorder who died in her sleep. The deceased was diagnosed with FCD type III with hippocampal sclerosis on histopathological examination at autopsy. H & E stain and NeuN immunohistochemistry neuronal cell marker were used to demonstrate the findings of FCD.

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ABSTRACT Acute erythroid leukemia (AEL) is an exceedingly uncommon but distinct hematological malignancy that shows neoplastic proliferation of erythroid precursors with maturation arrest and no significant myeloblasts. We describe an autopsy case of this rare entity in a 62-year-old man with co-morbidities. He underwent a bone marrow (BM) examination for pancytopenia during the first outpatient department visit, which revealed an increased number of erythroid precursors with dysmegakaryopoiesis suggesting the possibility of Myelodysplastic syndromes (MDS). Thereafter, his cytopenia got worsened, warranting blood and platelet transfusions. Four weeks later on the second BM examination, AEL was diagnosed based on morphology and immunophenotyping. Targeted resequencing for myeloid mutations revealed TP53 and DNMT3A mutations. He was initially managed along febrile neutropenia with the stepwise escalation of antibiotics. He developed hypoxia attributed to anemic heart failure. Subsequently, he had hypotension and respiratory fatigue pre-terminally and succumbed to his Illness. A complete autopsy showed infiltration of various organs by AEL and leukostasis. Besides, there was extramedullary hematopoiesis, arterionephrosclerosis, diabetic nephropathy (ISN-RPS class II), mixed dust pneumoconiosis, and pulmonary arteriopathy. The histomorphology of AEL was challenging, and the differential diagnoses were many. Thus, this case highlights the autopsy pathology of AEL, an uncommon entity with a strict definition, and its relevant differentials.

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ABSTRACT Background Autopsy requests have been trending downward for a variety of factors. There are differences between pre- and postmortem diagnoses. Autopsies remain a tool for education, public health research, quality control, and closure for families. Objective We report two cases that illustrate the utility of autopsy for uncovering contributing factors in the death of these patients and highlight their ongoing importance. Design Clinical and autopsy investigation of two individuals and illustration of the importance of autopsy findings which, had they been diagnosed premortem, could have changed the outcome. Cases were evaluated using the Goldman criteria for discrepancies between premortem clinical diagnoses and postmortem autopsy findings. Results In the first case, the patient had been previously admitted due to a non-ST elevation myocardial infarction months before the fatal event. The autopsy showed an undiagnosed clear cell carcinoma of the ovary. She expired due to a massive myocardial infarction secondary to neoplasm induced hypercoagulable state. The degree of pre-mortem/postmortem diagnostic discrepancy makes this a Goldman Class I error. In the second case, the patient presented to the emergency department with symptoms of Guillain-Barré Syndrome (GBS), for which he was treated. Abdominal masses were discovered; however, the patient decompensated before workup was completed. A high-grade B-cell lymphoma was confirmed but would not have altered the outcome, making this a Goldman class II error. Conclusions The autopsy remains a relevant and necessary tool for physicians and society. It assists in the establishment of diagnoses, measurement of treatment quality, the providence of public health metrics, and closure to the survivors.

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ABSTRACT Sclerosing Mesenteritis (SM) is a rare diagnosis, particularly in pediatric patients, and is typically non-fatal when appropriately treated. Although molecular and immunohistochemical alterations have been described, no pathognomonic signature has been identified for this entity. This report presents a case of a seven-year-old boy who suffered sudden cardiorespiratory arrest. Upon autopsy, he was found to have multicentric SM on the upper mesentery, which led to bowel wall thinning and abdominal bleeding with bacterial translocation. We performed comprehensive morphological, immunohistochemical, and molecular analyses. SM is an atypical disorder with diverse clinical manifestations, including a rare but potentially fatal course. Early diagnosis is critical, given its potential severity. To our knowledge, this is the first case report of pediatric mortality linked to SM. Our findings emphasize the importance of increased awareness and early detection of SM in pediatric patients.

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ABSTRACT We report a fatal subdural empyema caused by Campylobacter rectus in a 66-year-old female who developed acute onset of confusion, dysarthria, and paresis in her left extremities. A CT scan showed hypodensity in a crescentic formation with a mild mid-line shift. She had a bruise on her forehead caused by a fall several days before admission, which initially raised subdural hematoma (SDH) diagnosis, and a burr hole procedure was planned. However, her condition deteriorated on the admission night, and she died before dawn. An autopsy revealed that she had subdural empyema (SDE) caused by Campylobacter rectus and Slackia exigua. Both microorganisms are oral microorganisms that rarely cause extra-oral infection. In our case, head trauma caused a skull bone fracture, and sinus infection might have expanded to the subdural space causing SDE. CT/MRI findings were not typical for either SDH or SDE. Early recognition of subdural empyema and prompt initiation of treatment with antibiotics and surgical drainage is essential for cases of SDE. We present our case and a review of four reported cases.

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ABSTRACT Hypoplastic coronary artery disease (HCAD) is a rare coronary artery anomaly that may be the cause of sudden death. It can involve a single or all coronary arteries. This anomaly may cause circulatory insufficiency leading to myocardial infarction. HCAD has no symptoms or may exhibit cardiovascular signs like syncope, dyspnea, chest discomfort, or dizziness. It is often diagnosed at autopsy, and early diagnosis is made with a coronary angiogram. We report HCAD as the cause of the sudden death of a 25-year-old female with a history of loss of consciousness following exertion. On autopsy, all the coronary arteries’ lumen was narrowed with thin vessel walls. Histopathological examination shows an underdeveloped and missing muscular layer of the left anterior descending and circumflex coronary arteries’ vascular wall. Many cases of HCAD diagnosed by radiographic imaging in living patients have been reported in the literature, but a structural anomaly of coronaries leading to HCAD has not been reported. We report a case of HCAD describing the histopathological examination findings of the vascular wall of coronary vessels illustrating the structural difference.

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ABSTRACT Candida pneumonia remains a difficult diagnosis and is most common in immunocompromised individuals. It has been rarely reported in immunocompetent individuals. We present a case of unsuspected Candida pneumonia associated with Candida esophagitis and gastritis discovered on postmortem examination in a presumably immunocompetent patient. The patient was a 71-year-old male who presented with chest pain and was subsequently found to have a myocardial infarction treated with angioplasty and drug-eluting stent placement. The patient’s recovery was complicated by pneumonia refractory to antibiotics, and he went on to experience acute hypoxic respiratory failure, sepsis, disseminated intravascular coagulation (DIC), and ultimately expired. Autopsy revealed evidence of myocardial infarction as well as unsuspected Candida albicans pneumonia, esophagitis, and gastritis. Our case highlights how a presumably immunocompetent individual can develop this infection and how Candida esophagitis and Candida gastritis can be seen in association with Candida pneumonia. Due to the difficulty in diagnosing Candida pneumonia antemortem, autopsies provide a key opportunity to better understand these cases and the factors that may contribute to their development.

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ABSTRACT Chronic rheumatic heart disease (RHD) is the most troublesome complication of rheumatic fever. Extensive valvular scarring and ventricular remodeling due to pressure and volume overload occur in chronic RHD. Deformed valves are at potential risk for developing infective endocarditis (IE) with further systemic embolism. We hereby describe a case of a patient diagnosed with chronic rheumatic heart disease and severe ventricular dysfunction, planned for aortic valve replacement. The patient developed septic shock during a hospital stay. The autopsy revealed infective endocarditis in the aortic valve with septic thromboembolism in the peripheral branches of the coronary artery and early multifocal myocardial infarction changes.

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ABSTRACT Extranodal NK/T- cell lymphoma (ENKTCL) is an aggressive lymphoma driven by Epstein-Barr virus (EBV) infection in genetically susceptible individuals. It was historically called a lethal midline granuloma. Due to the angio-destructive nature of ENKTCL, lymphoma cells are often accompanied and masked by necrosis and dense inflammation in the biopsy. Further, the biopsy may show vasculitis, which can mimic granulomatosis with polyangiitis. Due to these masquerades, ENKTCL is often misdiagnosed in the biopsy. Several biopsies may be required to establish the diagnosis. We describe the clinical course and autopsy findings of a young female who presented with a hard-palate ulcer. Antemortem biopsies failed to establish the diagnosis. The autopsy revealed an advanced nasal subtype of Extranodal NK/T-cell lymphoma with dissemination to the kidneys, adrenals, liver, spleen, and small intestine.

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ABSTRACT Alcoholic foamy degeneration (AFD) is an uncommon presentation of alcoholic liver disease (ALD) with characteristic histologic findings of foamy-looking hepatocytes due to the presence of abundant microvesicles of fat within the cytoplasm predominantly in perivenular and midzonal regions without inflammation and fibrosis. It is underdiagnosed as the patients quickly recover after alcoholic abstinence and are rarely caught on biopsies. AFD has better prognosis than alcoholic hepatitis, and the injury mechanism is different, warranting a different diagnosis. We present an uncommon case of AFD incidentally diagnosed during autopsy in a chronic alcoholic and diabetic man.

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ABSTRACT Internal watershed infarcts (IWIs) occur at the junction of the deep and superficial perforating arterial branches of the cerebrum. Despite documentation in the radiology literature, IWIs are rarely encountered at the time of autopsy. Here, we report the case of a 59-year-old incarcerated male who was brought to the emergency department after being found unresponsive on the floor of his jail cell. Initial examination and imaging demonstrated right-sided hemiplegia, aphasia, right facial droop, and severe stenosis of the left middle cerebral artery, respectively. Repeat imaging 4 days after admission and 26 days before death demonstrated advanced stenosis of the intracranial, communicating segment of the right internal carotid artery, a large acute infarct in the right posterior cerebral artery territory, and bilateral deep white matter ischemic changes with a right-sided “rosary-like” pattern of injury that is typical of IWIs. Postmortem gross examination showed that the right deep white matter lesion had progressed to a confluent, “cigar-shaped” subacute IWI involving the right corona radiata. This is the first well-documented case of an IWI with radiologic imaging and photographic gross pathology correlation. This case uniquely highlights a rarely encountered lesion at the time of autopsy and provides an excellent visual representation of internal watershed neuroanatomy.

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ABSTRACT An enlarged left-sided supraclavicular node is a signal node for cancer metastasis. In such a case, the enlarged lymph node is often referred to as a Virchow node. The left-sided nature of the node is due to the drainage of the thoracic duct. So, the enlargement of a Virchow node is typically associated with malignancies, including gastrointestinal, pulmonary, and genitourinary carcinomas, in addition to lymphomas. This report documents a particularly unusual finding: bilateral Virchow nodes, representing metastasis of small-cell neuroendocrine carcinoma.

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ABSTRACT Necrotizing amebic colitis is an uncommon amebiasis complication associated with high mortality. We present a case of necrotizing amebic colitis in an old patient whose diagnosis was revealed at postmortem examination. An 81-year-old man died at home without medical attention. The postmortem examination revealed ulcers involving the entire colon and intestinal perforation. The ulcers were large, geographic, and necrotizing, extending from the cecum to the rectum. The histological examination disclosed the infectious etiology by showing amebic trophozoites at the base of the ulcers. No extra-intestinal lesions were found. No information about previous episodes of dysentery or travel could be obtained. The potential role of aging or drug-causing immunosuppression and the evolution of chronic and latent intestinal infection to a severe and invasive form of amebiasis is discussed. This case reinforces the value of postmortem examination for diagnosing diseases not clinically identified.

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ABSTRACT Toxic leukoencephalopathy (TLE) is a rare neurological debilitating and fatal condition. It has been previously associated with exposure to leukotoxic offenders such as chemotherapy, cranial radiation, certain drugs, and environmental factors. Currently, it is a commoner white matter syndrome resulting from increased substance abuse, classically by inhaled heroin and other opioids. Herein, we report a case of fatal TLE unveiled in an autopsy of a drug abuser. A 24-year-old male was found dead on the roadside. A day before, he was located in a state of delirium. In this case, the autopsy findings and histopathology characteristics of cerebral cortex involvement particularly directed to speculate the heroine as the principal offender.

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ABSTRACT Embryonal rhabdomyosarcoma (RMS) of the female genital tract is an uncommon malignancy, presenting mainly in the pediatric and adolescent populations, primarily affecting the first two decades of life. This malignancy presentation in adulthood is rare and is seldom seen. The incidence of this tumor affecting adult females is approximately 0.4 - 1%, with the common site being the vagina. This tumor infrequently involves the cervix. RMS has a poor survival rate and once diagnosed, it requires aggressive management by radical surgery accompanied by chemoradiation. We present a case of an anaplastic variant of embryonal RMS of the uterine cervix presenting as a cervical polyp in a 36-year-old female who complained of dyspareunia and post-coital bleeding.

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ABSTRACT Introduction Endocarditis is a rare, often fatal complication of rat bite fever caused by Streptobacillus moniliformis. Only 39 cases have been reported (including this case) as of 2022. We describe a case and aim to perform this entit’s first systematic literature review. Methods We performed a systematic review in CENTRAL, EMBASE, MEDLINE, SciELO, and LILACS. The terms used were terms used were (but not limited to) rat bite fever, Streptobacillus moniliformis, Spirillum minus, and endocarditis. We included all abstracts and articles with patients with echocardiographic or histologic-proven endocarditis. In case of discordance, a third reviewer was involved. Our protocol was submitted to PROSPERO (CRD42022334092). We also performed searches for studies on the reference list of included articles. Results We retrieved 108 and included 36 abstracts and articles. A total of 39 patients (including our report) were identified. The mean age was 41.27, and 61.5% were males. The most common findings were fever, murmur, arthralgias, fatigue, splenomegaly, and rash. Underlying heart disease was present in 33%. Exposure to rats was noted in 71.8% of patients, with 56.4% recalling a rat bite. Anemia was seen in 57%, leukocytosis in 52%, and elevated inflammatory markers in 58% that had lab work performed. The mitral valve was most affected, followed by the aortic, tricuspid, and pulmonary valves. Surgical intervention was required in 14 (36%) cases. Of those, 10 required valve replacement. Death was reported in 36% of cases. Unfortunately, the literature available is limited to case series and reports. Conclusion Our review allows clinicians to suspect better, diagnose, and manage Streptobacillary endocarditis.

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ABSTRACT Pleomorphic adenoma (PA) of the external auditory canal (EAC) is a rare clinical entity with a few cases reported in the literature. The clinical diagnosis of these lesions can be formidable due to their rarity and unusual location. This tumor occurs at various other anatomical locations apart from the major salivary glands. A 30-year-old female presented with a two-year history of a gradually enlarging and painless mass in the left external auditory canal. The tumor was excised, and histopathological and immunohistochemical evaluation revealed a mixed tumor with both epithelial and stromal components of different proportions, recognized and classified today by the World Health Organization (WHO) as a pleomorphic adenoma. The post-operative course was uneventful, and at the 10-month follow-up, no recurrence of the pleomorphic adenoma was noted. We highlight the histological features and the immunohistochemical profile of the tumor and review the literature on glandular neoplasms of the EAC and their recent classification, emphasizing on the histogenesis, clinical presentations, and microscopic features of the tumor. In addition, we aim to discuss vital features in differentiating these tumors from other tumors of the external auditory canal to enable clinicians and pathologists to recognize this uncommon benign neoplasm.

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ABSTRACT Hemangiolymphangioma is a very rare vascular malformation that develops as a combination of dilated venous and lymphatic vessels. We describe an unusual case of hemangiolymphangioma of the tongue affecting an adult man who complained of an uncomfortable, slowly progressing exophytic irregular dark red-violaceous nodular mass on his tongue that impaired speech and swallowing for two weeks. The clinical differential diagnoses were Kaposi’s sarcoma and a COVID-19-related lesion. A complete blood count and serology for HIV-1 and 2 and RT-PCR for COVID-19 were requested and results were negative. An incisional biopsy was performed. Microscopically, the lesion exhibited several dilated vessels lined by normal-appearing endothelial cells, some filled with prominent intravascular erythrocytes and others containing proteinaceous eosinophilic material resembling lymphatic vessels, in close association with hyperkeratosis, papillomatosis, and acanthosis. From immunohistochemical analysis, most vessels were found to be CD34 positive, some highlighted by α-SMA, whereas D2-40 was focal. Positive staining for some lymphatic and blood vessel markers, i.e., D2-40 and CD34, respectively, indicates a mixed derivation of the lesion. HHV-8 was negative. Clinical features, the congested blood vessels with ectasia in intimate association with hyperplastic epithelium, and the immunohistochemical profile supported the final diagnosis of oral hemangiolymphangioma. The patient underwent minimally invasive surgical excision with no intercurrences. After 18 months of follow-up, there were no signs of relapse.

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ABSTRACT Parasitic infections rarely involve the oral and maxillofacial regions and pose a diagnostic challenge when they do. Hydatid cysts are parasitic cysts caused by Echinococcus granulosus. Intraosseous involvement is observed in 3% of cases, of which only 2-6% are in the maxillofacial region. A scientific literature search revealed only seven cases involving the mandible. We report a rare case in a 16-year-old female patient who presented with facial asymmetry and well-defined radiolucency of the ramus. Our findings will help in understanding the diagnostic issues caused by non-specific presentation and difficulties in suspecting such a rare diagnosis as echinococcosis of the oral or maxillofacial region. A thorough systemic investigation is essential as 20-30% of these cases show multiorgan involvement.

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ABSTRACT External auditory canal (EAC) cylindroma is a rare tumor that mainly presents as a painless mass over the lateral aspect of the ear canal. They have been designated under different nomenclatures in the literature, and controversies persist about their etiology and histogenesis. Moreover, a clinical diagnosis of EAC cylindroma is often challenging because of their rarity and a close resemblance with other adnexal benign and malignant tumors. None of the previous authors have extensively reviewed the dermal cylindroma of the EAC. We provide an extensive review involving PubMed and Google Scholar and report by Preferred Reporting Items for Systematic Reviews and Meta-Analyses standards. A total of 8 cases are included in the current study. The mean age is 55.13 years. There are six females and two males. The left and right ear are involved in 62.50% and 37.50% of cases, respectively. The most common sign/symptom is painless mass (50%). Five authors reported a primary lesion (62.50%), while the remaining 3 reported a recurrent tumor (37.50%). Benign versus malignant cylindroma is reported in 87.50% and 12.50% of cases, respectively. All, except one case, reported a solitary swelling. Surgical excision was employed in all the cases. Primary defect closure versus defect closure with local/distant skin graft /flap is utilized in 37.50% and 62.50% of cases, respectively.

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ABSTRACT Neuroendocrine neoplasm (NEN) of the cervix is a malignant tumor and is classified into low and intermediate-grade neuroendocrine tumor (NET), and high-grade small cell neuroendocrine carcinoma (SCNEC), and large cells neuroendocrine carcinoma (LCNEC). SCNEC of the cervix is an Infrequent tumor with an incidence of less than 1% of all gynecological malignancies. It is characterized by small to medium-sized tumor cells with scant cytoplasm and neuroendocrine differentiation. Most cases of SCNEC of the cervix manifest in pure forms, and only cases show coexisting, non-neuroendocrine component of HPV-associated adenocarcinoma or squamous cell carcinoma. In this report, reviewing the literature, we present one such unique case of SCNEC of the cervix with adenocarcinoma and high-grade squamous intraepithelial neoplasia.

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ABSTRACT Biliary adenofibroma (BAF) is an uncommon liver tumor with a high propensity for malignant transformation. The histomorphology of BAF with malignant transformation can show a spectrum of changes ranging from benign, dysplastic to frank malignancy. Thus, the diagnosis of BAF imposes the pursuit of dysplasia/ malignancy focus. We presented a case of intrahepatic cholangiocarcinoma arising from BAF in a 49-year-old woman with detailed histomorphology. We also performed a PubMed database search and tabulated all previously reported cases of BAF with dysplasia/ malignant transformation. A statistic comparison of age, sex ratio, size of the tumor, and survival following complete resection between BAFs with and without dysplasia/ malignancy from the retrieved data is presented. Our analysis did not highlight any statistically significant difference between BAFs with and without dysplasia/ malignancy in age, sex ratio, tumor size, and survival following complete surgical resection. Our study highlights the histopathology and immunohistochemistry of a case of BAF with malignant transformation and highlights the importance of this diagnosis in management. Further longitudinal studies on a larger cohort of patients are required to validate our findings.

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ABSTRACT Clostridioidesdifficile infection (CDI) is the culprit of millions of nosocomial infections in the United States. Programs that successfully decrease its incidence, therefore, render cost savings for the healthcare system. Toxic megacolon and perforation are two of the most significant complications with increased mortality rates. We report a 23-year-old nursing home resident hospitalized for fever, cough, and green sputum. After 3 days of antibiotic therapy, he developed abdominal distension, diarrhea, and vomiting and underwent a total colectomy. The colon was dilated to a maximum of 11 cm with markedly edematous mucosa and yellow pseudomembranes. Qualitative PCR of the stool detected Clostridioides difficile toxin B gene. While there is no consensus for the required interval between antibiotic treatment and CDI, this presentation 3 days after starting the antibiotic therapy is earlier than most proposed ranges.

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ABSTRACT Balloon cell melanoma is a rare presentation of malignant melanoma, usually on the skin, with less than 100 cases reported. Mucosal BCM is even rarer, with only one case of anorectal BCM reported in English literature. The diagnosis is based on the histopathologic findings of a tumor composed of large, foamy melanocytes, with or without pigmentation, and confirmed by immunohistochemical studies showing expression for melanocytic markers. The foam cell appearance of the tumor cells and the lack of melanin pigment lead to a diagnostic dilemma, mostly when presented at an unusual location. Herein, we report a case of balloon cell melanoma at the anorectal junction in a 73-year-old male patient complaining of constipation and bleeding per rectum. Surgical resection was performed with no evidence of recurrence after three years of close follow-up. We believe this case will raise awareness among the medical community to consider this tumor a differential diagnosis in rectal masses.

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ABSTRACT Hydatid cyst is a parasitic infestation caused by Echinococcus larvae. Hydatid cyst of the ovary is a highly unusual presentation. Herein, we present a case of a young woman who complained of episodic lower abdominal pain. Ultrasound of the abdomen revealed a multi-cystic left adnexal mass measuring 86 mm x 67 mm. A possibility of ovarian cystic neoplasm was suggested. Unilateral salpingo-oophorectomy was performed. On histopathological examination, a cyst measuring 8.0 x 5.5 x 4.5 cm was found, replacing the entire ovary. The cyst cavity was filled with serous fluid and multiple pearly white membranous structures, giving a multiloculated appearance. Microscopic examination showed a cyst lined by a lamellar membrane containing protoscolices and hooklets. Hydatid disease is a zoonotic ailment caused by tapeworms (Echinococcus granulosus or, less commonly, Echinococcus multilocularis). The definitive hosts are carnivores. Humans are the accidental intermediate hosts. The hydatid cyst commonly affects the liver and the lungs. The primary hydatid cyst of the ovary is quite rare, with few case reports in the literature. In most cases, symptoms are vague, and the lesion is misdiagnosed as benign or malignant ovarian cystic neoplasm on clinical and radiological examination. Ovarian hydatid cyst is treated by surgery with ovarian cystectomy as the gold standard. The possibility of a hydatid cyst should be kept under differential diagnoses while evaluating the cystic diseases of the ovary.

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ABSTRACT Lipomas are mesenchymal neoplasms relatively uncommon in the oral cavity. Lipomas can exhibit histopathological features mimicking atypical lipomatous tumors (ALT) or dysplastic lipoma (DL) in the presence of degenerative changes. Relevantly, immunohistochemistry assists in the correct diagnosis. Herein, we present the case of a 54-year-old male with a sessile nodule located on the dorsum of the tongue. The histopathological analysis showed a diffuse, non-circumscribed adipocytic proliferation constituted by cells of variable size containing cytoplasmic vacuoles and displaced nuclei, some resembling lipoblasts supported by fibrous connective tissue stroma. By immunohistochemistry, tumor cells were positive for vimentin, S100, FASN, CD10, and p16. Rb expression was intact. Moreover, CD34, p53, MDM2, and CDK4 were negative. After 2-year of follow-up, no alteration or recurrence was observed. In conclusion, MDM2, CDK4, p53, and Rb immunomarkers can be used reliably to differentiate benign lipoma with degenerative changes from ALT and DL.

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ABSTRACT Colorectal carcinoma (CRC) is the third most commonly diagnosed cancer worldwide and is the second most common cause of cancer-related deaths. However, the Omani population shares the major burden as the most prevalent carcinoma. The disease is comparatively higher in males than females. Patients with pre-existing risk factors, including inflammatory bowel disease, are at increased risk of developing neoplasia. Among the various histopathological subtypes of adenocarcinoma in the rectum, signet ring cell carcinoma is the rarest and accounts for approximately 1% of the cases. Given the aggressive nature of this tumor, advanced presentation, stage, and poor prognosis, regular endoscopic surveillance is essential. Hereby, we report a rare case of signet ring cell carcinoma arising in the rectal stump in an already diagnosed and operated patient of Ulcerative colitis.

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ABSTRACT COVID-19 is commonly associated with high serum levels of pro-inflammatory cytokines, and the post-infection status can disturb self-tolerance and trigger autoimmune responses. We are reporting a 45-year-old male who was admitted with fatigue, jaundice, elevated liver enzymes (with cholestatic pattern), and acute kidney injury two weeks after recovering from a mild SARS-CoV-2 infection. Serologies for viral hepatitis and anti-mitochondrial antibody were negative, while anti-nuclear and anti-smooth muscle antibodies were positive. There were no signs of chronic liver disease, and a magnetic resonance cholangiography showed no dilatation of biliary ducts. Histologic evaluation of the liver evidenced numerous foci of lobular necrosis without ductopenia or portal biliary reaction. Considering the autoantibody profile and histologic changes, the medical team started oral prednisone, but there was a suboptimal biochemical response in the outpatient follow-up. Two months later, a second liver biopsy was performed and revealed non-suppurative destructive chronic cholangitis, extensive areas of confluent necrosis with hepatocytes regenerating into pseudorosettes, and numerous plasma cells. According to the Paris Criteria, the patient was then diagnosed with an autoimmune hepatitis-primary biliary cholangitis overlap syndrome (AIH-PBC-OS). After adding azathioprine and ursodeoxycholic acid to the treatment, there was a satisfactory response. This is the second worldwide report of an AIH-PBC-OS triggered by COVID-19, but the first case with a negative anti-mitochondrial antibody. In this setting, histologic evaluation of the liver by an experienced pathologist is a hallmark of achieving the diagnosis and correctly treat the patient.

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ABSTRACT Glomangiopericytoma is a rare vascular neoplasm of the nasal cavity and paranasal sinuses that occurs during the sixth or seventh decade of life. It is categorized as a borderline tumor with low malignant potential and classified as a distinct entity of sinonasal tumors with perivascular myoid phenotype by the World Health Organization (WHO). We report the case of a 50-year-old woman with nasal obstruction and severe epistaxis. The nasal sinuses computed tomography (CT), and magnetic resonance imaging (MRI) demonstrated a 3.1 cm soft tissue mass occupying the upper part of the left nasal cavity invading the left paranasal sinuses and nasal septum, and the left eye medial rectus muscle. A total mass resection was performed by nasal endoscopy. The histological and immunohistochemical examination yielded the diagnosis of glomangiopericytoma. This case report aims to contribute to the knowledge of nasal neoplasms. The need for more data on this entity is the main obstacle to developing standardized treatment guidelines.

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ABSTRACT Chronic myeloid leukemia (CML) accounts for 2-3% of childhood leukemias. About 5% of cases present in a blastic phase of CML which clinically and morphologically mimics more common acute leukemias of childhood. We report a case of a 3-year-old male who presented with gradual onset swelling of the abdomen and extremities along with generalized weakness. Examination revealed massive splenomegaly, pallor, and pedal edema. Initial workup showed anemia, thrombocytopenia, and leukocytosis (120,000/uL) with a blast percentage of 35%. Blasts were positive for CD13, CD33, CD117, CD34 and HLA-DR, and stained negative for Myeloperoxidase and Periodic Acid Schiff. Fluorescence in situ hybridization was positive for b3a2/e14a2 junction BCR-ABL1 transcript and negative for RUNX1-RUNX1T1/t(8;21), clinching the diagnosis of CML in myeloid blast crisis. The patient expired within 17 days of diagnosis and initiation of therapy.

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ABSTRACT Xanthogranulomatous inflammation is a rare benign inflammatory lesion characterized by sheets of lipid-laden foamy histiocytes. It has been reported in various organs, mainly the kidney and gall bladder. Xanthogranulomatous endometritis (XGE) is sporadic, with only a few cases reported in the English medical literature. Herein, we report a case of xanthogranulomatous endometritis with the formation of stones in a 50-year-old female patient with a prolapsed uterus. Grossly the endometrium was irregular, and the uterine cavity was filled with a yellow friable material, a polypoid growth, and yellowish stones. The microscopy showed sheets of histiocytes with few preserved endometrial glands. In this case, the xanthogranulomatous inflammation may mimic a clear cell carcinoma involving the endometrium and myometrium. One of the important differential diagnoses is malakoplakia. Immunohistochemistry and special stains are helpful in diagnosis.

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ABSTRACT Splenogonadal fusion is an infrequent cause of testicular or scrotal swelling with less than 250 cases reported. We report the case of a 27-year-old male who presented with painless scrotal swelling. The sonography showed a homogeneous, well-encapsulated left extratesticular mass, which was surgically removed. The gross examination revealed a grey-brown tissue below the left testis. The microscopy of the grey-brown mass revealed splenic tissue, and the testis showed maturation arrest, resulting in the diagnosis of splenogonadal fusion. These can be easily mistaken for a tumor, especially in this age group. Reporting such an entity increases awareness among clinicians, radiologists and pathologists, which will aid in preventing an orchiectomy for these patients.

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ABSTRACT Background Acantholytic squamous cell carcinoma (ASCC) is an uncommon histological variation of oral squamous cell carcinoma (OSCC), accounting for fewer than 4% of all occurrences. The tumor shows a slight masculine predisposition, with the lower lip being the most commonly affected location. ASCC is reported to have a diverse biologic behavior, which explains its ability to metastasize to distant places and, thus, its poor prognosis. Similarly, clear cell change in OSCC is a rare occurrence with an unknown etiology that suggests its aggressive nature. Method and Results Histopathology reveals central acantholytic cells with numerous duct-like features. The presence of distinct cytological atypia contributes to the diagnosis of SCC. Special stains and IHC aid in distinguishing tumor from other histopathologically similar entities. Conclusion The case of a 29-year-old male presented here with an updated literature review highlights the need for histological study of the unique and seldom seen oral ASCC with clear cell change, which can be ignored because of similarities with other entities. Because recurrence rates are so high for ASCC, amalgamated clear cell change makes it critical for proper treatment initiation with a definite diagnosis. To the best of our knowledge, this is the first documented occurrence. Our experience with the present case suspected a more aggressive behavior due to a high Ki-67 index, anticipating a poorer prognosis in the oral cavity considering the patient's young age.

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ABSTRACT Giant cell arteritis (GCA) is a type of chronic vasculitis that affects medium and large-caliber arteries, frequently related to aortic involvement and, consequently, to aneurysm formation. However, associated valvulitis with giant cells is uncommon. We describe the case of a 50-year-old female patient with aortic aneurysm and valvular insufficiency, whose anatomopathological examination revealed giant-cell aortic valvulitis associated with giant cell aortitis.

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ABSTRACT Non-insulinoma pancreatogenous hypoglycemia syndrome (NIPHS), without previous bariatric surgery, is a rare form of hypoglycemia in adult patients and is associated with nesidioblastosis. Adult-onset nesidioblastosis in diabetic patients is rare and histologically identical to "non-insulinoma pancreatogenous hypoglycemia syndrome (NIPHS)". Nesidioblastosis is rare in adults and clinically and biochemically mimics Insulinoma. In the literature, there have only been four cases of adult nesidioblastosis that followed diabetes mellitus. We report a case of nesidioblastosis in a 36-year-old diabetic female presenting with dizziness, sweating, and palpitations for three years. Selective non-invasive techniques failed to detect a tumor. Based on the pursuit of an insulinoma, a distal pancreatectomy specimen was received at our laboratory, and a diagnosis of nesidioblastosis was made. She is currently on follow-up with a favorable outcome. The definitive diagnosis of nesidioblastosis is made on a histological basis. The preferred form of treatment is pancreatic surgical resection. Nesidioblastosis should be taken into consideration in cases where diabetes transforms into hyperinsulinemic hypoglycemia.

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ABSTRACT The gingival cyst of the adult (GCA) is a rare odontogenic cyst, consisting of 0.3% of all odontogenic cysts. This case report, based on CARE guidelines for case reports, aims to present a case of a 52-year-old female patient with a symptomatic translucent nodule in the upper left anterior gingiva, measuring approximately 6mm. Excisional biopsy was performed, and the histological examination revealed multiple cystic cavities lined by the squamous epithelium of varying thickness with focal areas of nodular thickenings. The presence of clusters of cells with clear cytoplasm within epithelial thickenings was observed. PAS staining was negative in clear cells. The diagnosis of the GCA was established. Despite its rarity, GCA should be considered in the differential diagnosis of gingival lesions. Conservative surgical treatment proved to be effective, with no signs of recurrence.

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ABSTRACT First described by J Rosai and R F Dorfman in 1969, Rosai-Dorfman disease (RDD) is a benign, self-limiting histiocytosis of unknown etiology. It is usually seen in the first two decades of life. The most frequent clinical presentation is painless, bilateral cervical lymphadenopathy accompanied by fever, weight loss, and an elevated ESR. However, RDD without nodal involvement is extremely rare, and the most common extranodal location is the head and neck region, mainly affecting the nasal cavity, pharynx, and paranasal sinuses. Oral location of RDD is occasional; according to our knowledge, only 17 cases of oral Rosai-Dorfman disease without lymph node involvement have been found in the literature. Because of the rarity of these isolated oral presentations, the clinical and radiological aspects need to be more studied. This article aims to present a rare case of oral Rosai-Dorfman disease without nodal involvement, detail the clinical and radiological signs, and the treatment strategy used in our patient.

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ABSTRACT Streptococcus constellatus is usually a benign, commensal bacteria but has increased incidence in blood cultures and abscesses. This pathogenic involvement is most prevalent in individuals with underlying medical conditions, such as solid tumors and type 2 diabetes mellitus, as well as in cases of community-acquired infections. We report a 43-year-old male with a right medial thigh ulcer and necrotic scrotal skin. The wound culture from surgical debridement grew Streptococcus constellatus, and histology was consistent with stage III necrotizing fasciitis. Regardless of etiology, the mortality rate of patients with necrotizing fasciitis is greatly decreased with early intervention and thorough surgical debridement.

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ABSTRACT Introduction the autopsy is an essential medical procedure; however, its use has declined over the decades. In autoimmune and rheumatological diseases, anatomical and microscopic diagnosis is critical to diagnose of the cause of death. For this reason, our objective is to describe the cause of death in patients diagnosed with autoimmune and rheumatic diseases who underwent an autopsy in a Pathology reference center in Colombia. Materials and methods a retrospective and descriptive study of autopsy reports. Results between January 2004 and December 2019, 47 autopsies of patients with autoimmune and rheumatological diseases were performed. Systemic lupus erythematosus and rheumatoid arthritis were the most common diseases. The leading cause of death was related to infections, being opportunistic infections in the majority of the cases. Conclusions our autopsy-based study was focused on patients with autoimmune and rheumatological conditions. Infections are the leading cause of death, particularly opportunistic infections, diagnosed mainly by microscopy. Thus, the autopsy should continue to be considered the “gold standard” to determine the cause of death in this population.