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BACKGROUND: Aneurysmal bone cysts (ABCs) are benign, osteolytic lesions that can occur in long bones, vertebrae, or rarely, the skull. Here the authors present the case of a 15-year-old male with a primary ABC of the left frontoparietal skull along with a review of the literature to provide insight into the nature of this rare disease. OBSERVATIONS: An otherwise healthy 15-year-old male presented with a tense, painful lesion of the left frontoparietal scalp. He could not identify any inciting trauma, but first noted the lesion less than 2 weeks prior to presentation with progressive enlargement. Cranial imaging revealed a lytic skull lesion with fluid-fluid levels suggestive of ABC. Curative therapy was provided via wide excision of the lesion and calvarial reconstruction of the resultant skull defect. This was performed without complication, and histopathological evaluation confirmed the diagnosis of primary ABC. LESSONS: ABCs of the skull are rare entities and most often arise in the skull base versus the calvaria. Typically, these lesions are associated with an underlying bone pathology (secondary ABCs) but can be rarely seen as isolated lesions (primary ABCs). Clinical management consists of excision and adjuvant therapy for underlying pathology where appropriate.
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There is a known association between inflammatory bowel disease (IBD) and vasculitis, which can present with mesenteric ischemia or cutaneous manifestations. Infliximab, an anti-tumor necrosis factor (anti-TNF) used to treat IBD, has been implicated as a potential culprit. We present a unique case of a patient with ulcerative colitis who developed isolated celiac artery vasculitis presenting with abdominal pain and ileus after decreasing his dosage of azathioprine. Vasculitis resolved with steroids and increasing azathioprine dosage, while continuing anti-TNF therapy, suggesting that anti-TNF agents are not the only cause of vasculitis in patients with IBD or that thiopurines may be protective.
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Purpose: Our quality improvement study aimed to determine whether application of a neonatal early-onset sepsis calculator (NSC) among well-appearing infants born at ≥35 weeks' gestation to mothers with chorioamnionitis decreases the number of lab evaluations (LEs) and antibiotic treatments (Abxs) without missing early-onset sepsis. Methods: We compared 2 years (January 1, 2019-January 3, 2021) of data from a historical-control group before implementation of the NSC to 1 year (January 4, 2021-December 31, 2021) of data from a calculator group after implementation of the NSC to evaluate whether LE and Abx decreased following implementation of the NSC on January 4, 2021. A P-value of <0.05 was considered statistically significant for the chi-squared test, Fisher's exact test, Student's t-test, and Mann-Whitney U test used for the analyses. Results: In the historical-control group, 94% of infants received LE and Abx. Retrospective application of the NSC in the historical-control group decreased LE from 94% to 21% and Abx from 94% to 13%. In the calculator group, 14% and 5% of infants received LE and Abx, respectively, and none of the blood culture was positive. Median time from birth to antibiotic initiation was significantly longer (14.5 vs 3.8 hours; P=0.0037) with no increase in median length of stay (2.3 vs 2.4 days; P=0.02) after NSC implementation. No significant difference in neonatal intensive care unit admission was identified between groups (4% vs 1%; P=0.15). Conclusions: There was a significant decrease in LE and Abx among well-appearing infants born at ≥35 weeks' gestation to mothers with chorioamnionitis after implementation of the NSC without missing early-onset sepsis. There was no increase in neonatal intensive care unit admission or length of hospital stay in infants who received antibiotics later after they appeared equivocal or clinically ill in the calculator group. Larger prospective studies that include follow ups are needed to confirm that early-onset sepsis is not missed.
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Plasma cell neoplasms include various conditions ranging from indolent conditions such as monoclonal gammopathy of undetermined significance (MGUS) to more aggressive forms such as multiple myeloma (MM). The World Health Organization classifies plasmacytomas into two types: solitary osseous plasmacytoma (SOP) and extramedullary plasmacytoma (EMP). Most primary EMPs occur in the upper gastrointestinal tract, head and neck, upper respiratory system, central nervous system, lungs, liver, spleen, and kidneys. However, the occurrence of EMP involving the testis site is quite rare. Given the rarity of testicular plasmacytoma, there is no consensus on the standard of treatment for this diagnosis. Most EMP is radiosensitive, with few localized types responding to surgical intervention. Tumor recurrence and disseminated infiltration are treated with adjuvant chemotherapy after radiation or surgery. Our patient has a unique presentation of an individual who developed recurrent myeloma of the testis 12 years after his initial diagnosis of myeloma.
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Intussusception in adults is a rare finding with a majority of cases occurring in the pediatric population. It occurs infrequently and its presentation, etiology, and treatment differ from childhood intussusception. When discovered in adults, it raises suspicion for a neoplastic process serving as the pathological lead point. Cross-sectional imaging is the primary study of choice for diagnosis, but at times, a more invasive approach involving an exploratory laparotomy is required posing an increased risk for morbidity and mortality. Here we present a 64-year-old male who was found to have jejunal-jejunal intussusception that was surgically removed with pathology revealing metastatic melanoma as the lead point. This case highlights a unique presentation of a melanoma that was previously eradicated with immunotherapy and now had metastasized to the intestine many years later.
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Many chemotherapeutic agents have been associated with drug-induced colitis (DIC). With newer agents' expansion of approval as first-line therapy for common cancers, it is important to be cognizant of their association with DIC. We present a case of brentuximab-associated DIC in an elderly woman with CD30+ Hodgkin lymphoma. Brentuximab's association with DIC was suspected by others in the literature, but a history of stem-cell transplant in them would blur the association with graft-vs-host disease. Lack of stem-cell transplant in our patient makes the link between brentuximab and DIC unambiguous.
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Immunoglobulin A (IgA) nephropathy is the most common cause of primary glomerulonephritis worldwide. IgA vasculitis (formerly known as Henoch-Schonlein purpura) typically presents with IgA nephropathy on renal biopsy in addition to extrarenal symptoms like purpura, abdominal pain, and arthritis. Diffuse alveolar hemorrhage (DAH) is the most common pulmonary complication, but this is rarely seen. In this case report, we describe a 35-year-old male with chronic untreated hepatitis B infection who presented with pulmonary-renal syndrome. He was found to have clinical findings of DAH and concomitant IgA nephropathy on renal biopsy, without having any other typical manifestations of IgA vasculitis. This shows that IgA nephropathy should be considered in the differential diagnosis of DAH and emphasizes the importance of a renal biopsy in patients presenting with pulmonary-renal syndrome.
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Vascular ectasia is a common cause of lower gastrointestinal (GI) bleeding in older patients. They typically present as flat or slightly raised fern-like bright red lesions. We report a rare case of a vascular ectasia presenting as a pedunculated polypoid lesion in a young patient with rectal prolapse. The pedunculated polyp was removed using hot snare polypectomy. This case highlights a unique presentation of a rare lesion and endoscopic management of these lesions.
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Immunoglobulin light chain (AL) amyloidosis is a systemic disease in which different systems such as kidneys, heart, and lungs are affected by the deposition of amyloid, a form of fibrillary protein. Usually, it occurs in patients with pre-existing diagnoses of plasma cell dyscrasias and is rarely seen in the concurrence of marginal zone lymphoma (MZL). Earlier interventions with cyclophosphamide and dexamethasone in conjunction with newer therapies such as bortezomib, carfilzomib or lenalidomide, and pomalidomide are being used to treat patients with AL amyloidosis. In this report, we are presenting a unique case of a patient who was diagnosed with AL amyloidosis several years prior to presenting with a soft tissue mass, which was subsequently noted to be an amyloid mass within an MZL. Overall, the occurrence of AL amyloidosis and MZL is rare with less than 20 patients reported. The MZL developed prior to or simultaneously with AL amyloidosis in the reported cases. Therefore, to our knowledge, this is the first time systemic amyloidosis has preceded MZL.
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Amyloidosis is a rare disease that involves the extracellular deposition of abnormally folded proteins, precipitating organ dysfunction. Pulmonary amyloidosis is frequently characterized by the AL amyloid subtype and can be localized or associated with systemic involvement, presenting in a nodular, diffuse alveolar-septal, or tracheobronchial pattern. Presentation of disease can vary from clinically silent to severe. Pulmonary amyloidosis is typically first suspected on CT scan of the chest. Diagnostic workup requires tissue biopsy and identification by immunohistochemical staining. Systemic treatment has evolved over recent years to include the combination of daratumumab, bortezomib, cyclophosphamide, and dexamethasone (dara-VCD) as first-line therapy, with the goal of quickly attaining complete hematologic response. Through clinical vignettes, we review pulmonary AL amyloidosis and discuss current treatment options.
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Acute myeloid leukemia (AML) is primarily a disease of older adults and can arise de novo, in relation to previous treatment or in the setting of underlying hematological disease. While it is known to arise from chemoradiation in the setting of breast cancer, little is known about the association between BRCA carriers and AML. We report a case of a young female BRCA carrier who develops de novo AML without prior chemoradiation treatment, and examine if there is a link between BRCA and developing leukemia.
