Adult-Onset Multifocal Cutaneous Myofibromas: A Case Report of a Rare Entity.

Myofibromas are observed in both infantile and adult presentations, with key differences in the number and severity of lesions between these two groups. Infantile presentations encompass both indolent, isolated cutaneous lesions, as well as aggressive, multicentric presentations with visceral involvement. Adult myofibromas appear to be characterized by a single isolated cutaneous lesion, generally asymptomatic and following a benign clinical course. The occurrence of adult multifocal myofibromas has not yet been described in the literature. Here, we report a case of a 57-year-old female who presented with two minimally symptomatic soft tissue lesions on her right leg, with the pathologic findings of each lesion consistent with a cutaneous myofibroma. This case report describes a rare presentation of adult-onset multifocal cutaneous myofibromas.

Defect in Automated Antigen Excess Detection Discovered after Reviewing Serum Free Light Chain Results in Context with Clinical Findings.

Serum κ and λ free light chains can be markedly elevated in monoclonal gammopathies; consequently, serum free light chain (sFLC) immunoassays are susceptible to inaccuracies caused by antigen excess. As a result, diagnostics manufacturers have attempted to automate antigen excess detection. A 75-year-old African-American woman had laboratory findings consistent with severe anemia, acute kidney injury, and moderate hypercalcemia. Serum and urine protein electrophoresis and sFLC testing were ordered. The sFLC results initially showed mildly elevated free λ light chains and normal free κ. The pathologist noted that sFLC results were discrepant with the bone marrow biopsy, electrophoresis, and immunofixation results. After manual dilution of the serum, repeat sFLC testing revealed significantly higher λ sFLC results. Antigen excess causing falsely low sFLC quantitation may not be detected by immunoassay instruments as intended. Correlation with clinical history, serum and urine protein electrophoresis results, and other laboratory findings is essential when interpreting sFLC results.

Podocytopathies related to either COVID-19 infection or its vaccination, our experience and literature review.

Coronavirus disease 2019 (COVID-19) affects several organs including the kidney resulting in acute kidney injury (AKI) and variants of podocytopathies. From the beginning to the middle period of the COVID-19 pandemic, we have collected eight renal biopsies with various renal diseases including 4 podocytopathies. In addition, from the middle period to the near end of the COVID-19 pandemic, we have seen two of the patients who developed nephrotic syndrome following COVID-19 vaccination. Three of 4 podocytopathies were collapsing glomerulopathy (also called collapsing focal segmental glomerulosclerosis) and the fourth was a minimal change disease (MCD). Two of three collapsing glomerulopathy were found in African American patients, one of who was tested positive for having the high-risk allele APOL-1 G1. In addition, the two renal biopsies showed either MCD or replaced MCD following COVID-19 vaccination. MCD can be a rare complication following COVID-19 infection and COVID-19 vaccination, raising the question if there are similar antigens induced by the infection or by the vaccination that trigger the MCD. This article reports our experience of diagnosing podocytopathies related to either COVID-19 infection or its vaccination and provides a literature review regarding the incidence and potential pathophysiology in the field.

Overlapping Tubulointerstitial Lupus Nephritis and Immunoglobulin G4-Related Disease: A Case Report.

Immunoglobulin G4-related disease (IgG4-RD) in conjunction with systemic lupus erythematosus (SLE) is a rare occurrence. In this case report, we present the case of a 50-year-old female who was diagnosed with SLE based on clinical and laboratory criteria. The patient exhibited pericardial effusion necessitating pericardiocentesis, pleural effusion requiring thoracentesis, and impaired renal function necessitating dialysis. Renal biopsy revealed findings consistent with tubulointerstitial lupus nephritis and IgG4-related disease. Additionally, elevated levels of serum IgG4 were detected. The patient received intravenous pulse dose steroids and oral steroids, which were tapered gradually, followed by daily hydroxychloroquine treatment and two doses of rituximab every two weeks. Consequently, the patient experienced an improvement in renal function and no longer needed dialysis. To our knowledge, only a few reports of this overlap exist. This late diagnosis of SLE could be explained by the fact that IgG4 is associated with milder renal disease in lupus patients, due to its inability to activate the classical complement pathway. IgG4-RD/SLE overlap patients usually respond well to a combination of steroids and other immunosuppressants used to treat SLE. However, our experience with treating this disease overlap remains limited due to its extreme rarity.

Multiple Endocrine Neoplasia Type 1 with Concomitant Existence of Malignant Insulinoma: A Rare Finding.

Multiple endocrine neoplasia type 1 (MEN1) is a rare syndrome of autosomal dominant inheritance defined by co-occurrence of two or more tumors originating from the parathyroid gland, pancreatic islet cells, and/or anterior pituitary. Insulinoma which has an incidence of 0.4% is a rare pancreatic neuroendocrine tumor. Malignant insulinoma is extremely rare, while primary hyperparathyroidism is a common occurrence in MEN1. We present a case of MEN1 syndrome with 2.6 cm insulinoma in the pancreatic head and parathyroid adenoma in a 56-year-old female who presented with symptoms suggestive of hypoglycemia like multiple episodes of loss of consciousness for four years. Classical pancreaticoduodenectomy was carried out, and the postoperative period was uneventful. Later, subtotal parathyroidectomy was performed, which showed parathyroid adenoma. Patients presenting with features of hypoglycemia should be vigilantly assessed for the presence of a sinister pathology.

Pancreatic Neuroendocrine Tumor with Benign Serous Cystadenoma: A Rare Entity.

Mixed serous-neuroendocrine neoplasm constitutes pancreatic serous cystic neoplasms and pancreatic neuroendocrine tumor, two tumor components with different underlying pathologies. The differentiation of these tumors is important as the management and prognosis depend on the pancreatic neuroendocrine tumor component. We report a case of mixed serous-neuroendocrine neoplasm in a 47-year-old female who presented with epigastric pain abdomen for two years. Imaging studies, tumor markers, thorough systemic evaluation, surgical resection, histopathological examination, and timely follow-up constituted our management approach. A 4 cm × 4 cm mass in the distal pancreas with multiple cysts in the pancreatic parenchyma containing serous fluid on distal pancreatectomy and splenectomy was found. The histopathological examination revealed combined benign serous cystadenoma and neuroendocrine tumor. She did not have any recurrence or metastasis by four years of follow-up.

Neuromyelitis Optica in a Nepalese Man.

BACKGROUND: Neuromyelitis optica is a severely disabling inflammatory disorder of the central nervous system of autoimmune etiology that mainly affects the optic nerves and spinal cord. Here, we present a case report detailing a patient with tingling and weakness of right upper and lower limbs who was neuromyelitis optica immunoglobulin G-positive. CASE PRESENTATION: A 46-year-old Nepalese man presented to the hospital with a history of tingling and weakness of right upper and lower limbs that developed over a period of two months. Clinical evaluation showed diminished power across all major muscle groups in the right upper and lower limbs. Magnetic resonance imaging of his cervical spine showed T1 iso- to hypointense signal and T2 hyperintense signal in central cervical spinal cord from first to sixth cervical level, probably suggestive of myelitis or demyelination. The patient was immediately started on intravenous methylprednisolone. The diagnosis of neuromyelitis optica was later confirmed with strongly positive neuromyelitis optica immunoglobulin G. CONCLUSION: In resource limited setting, in the absence of tests for neuromyelitis optica immunoglobulin G, treatment was started and the patient's condition started to get better. Hence, early initiation of aggressive immunosuppressive treatment is essential in such cases.