Uncommon Cause of Internal Mammary Artery Pseudoaneurysm.

Background: Internal mammary artery pseudoaneurysms most commonly develop from thoracic penetrating trauma or procedures. However, other important etiologies should not be overlooked. Case Report: A 27-year-old female presented with antiphospholipid antibody syndrome, thrombotic microangiopathy, end-stage renal disease on hemodialysis, and epilepsy. On admission, the patient had pulseless electrical activity and hypertensive emergency. After the patient was successfully resuscitated, she developed status epilepticus. Laboratory workup on admission revealed a subtherapeutic international normalized ratio, elevated C-reactive protein and sedimentation rate, and acute anemia. Imaging showed a right-sided subdural hematoma with a midline shift and likely internal mammary artery pseudoaneurysm. Angiography demonstrated aneurysmal dilation, segmental narrowing, and a string of beads appearance. Because of our patient's demographics, string of beads appearance on diagnostic angiography, history of renal disease, and negative hepatitis serology, fibromuscular dysplasia was considered the etiology of the internal mammary artery pseudoaneurysm. The family opted for 2 burr holes and a subdural drain but declined further diagnostic and therapeutic interventions because of anoxic brain injury and poor prognosis. Conclusion: In this patient, the etiology of the internal mammary artery pseudoaneurysm was attributed to fibromuscular dysplasia. Although this patient's family chose comfort measures, treatment methods are available for internal mammary artery pseudoaneurysms.

Concomitant occurrence of advanced fibrocavitary pulmonary sarcoidosis and chronic pulmonary aspergillosis.

An African American man in his 30s presented with haemoptysis associated with chronic productive cough, exertional dyspnoea, weight loss and skin lesions. Physical examination was notable for multiple cutaneous plaques over upper extremities and face. CT chest showed bilateral upper lobes cavitations and left upper lobe mass like consolidation. Further workup revealed positive serum aspergillus IgG, respiratory culture grew Aspergillus fumigatus, skin biopsy showed non-caseating granuloma. A final diagnosis of concomitant chronic pulmonary aspergillosis and advanced fibrocavitary pulmonary sarcoidosis with cutaneous involvement was made. The patient was initiated on antifungal therapy without steroids due to the concern of worsening the fungal infection. However, he presented later with worsening haemoptysis requiring bronchial artery embolisation. Surgical intervention was recommended but the patient eventually declined. The patient continued to be followed up closely in the clinic and repeated chest imaging showed stable findings 3 months after initial presentation.

Ignatzschineria spp. bacteremia from maggot infestation.

Ignatzschineria spp. bacteremia associated with maggot infestation is extremely rare in humans. There are only a few cases worldwide ever reported in the literature. We described a clinical case with a male patient who presented with maggot manifestation at his lower extremity, was found with bacteremia, and subsequently identified as Ignatzschineria spp by 16S rRNA sequencing.

Severe psoriasis presenting with rapidly progressive (crescentic) IgA-predominant glomerulonephritis.

IgA nephropathy (IgAN) is commonly associated with psoriasis; however, psoriasis presenting with crescentic IgAN is uncommon. A 49-year-old man with erythrodermic psoriasis with arthritis and stage 2 chronic kidney disease presented to the emergency department with worsening peripheral oedema and difficulty breathing. The patient had been hospitalised previously for a psoriasis flare. He was found to have an acute kidney injury on chronic kidney disease and was diagnosed with crescentic IgA glomerulonephritis on his first hospitalisation. He was treated with corticosteroids and was discharged stable with a plan to start cyclophosphamide in the outpatient setting. On his current hospitalisation, cyclophosphamide was added to his corticosteroids. Crescentic IgAN is rare. Its management has been based largely on observational studies. Our case highlights the importance of starting combined corticosteroids and cyclophosphamide early in crescentic IgAN and that corticosteroid monotherapy is insufficient in controlling disease progression.

Idiopathic linear IgA bullous dermatosis treated with prednisone.

We present a case of a 43-year-old man with a medical history of paroxysmal atrial fibrillation that presented with acute onset generalised vesiculobullous rash of 1-week duration. The rash was initially noticed on his groin and then spread to his hands, feet and mucosal surfaces. Laboratory tests were unremarkable, including an extensive infection aetiology work-up. Punch biopsies were obtained of a fresh lesion and were stained with H&E and sent for direct immunofluorescence. Light microscopy and immunofluorescence study demonstrated a subepidermal blister with predominant neutrophilic infiltrates and a linear band of IgA at the dermoepidermal junction, respectively. The patient was diagnosed with linear IgA bullous dermatosis and was subsequently treated with 0.5 mg/kg of prednisone daily following previous case reports. At 1-week follow-up as an outpatient, the bullae became crusted, and the rash was nearly completely regressed.

Rare Cardiac Papillary Fibroelastoma: Right Atrial, Non-Valvular, Large, Symptomatic With Pulmonary Embolism.

INTRODUCTION: Primary cardiac tumors are rarely seen in the general population and only a subset are classified as cardiac papillary fibroelastoma. CASE PRESENTATION: A 59-year-old female that presented for unresponsiveness and cardiac arrest required 4 rounds of cardiopulmonary resuscitation and intubation. Laboratory investigations showed uncompensated respiratory acidosis, hyperkalemia, and elevated troponins. A chest computed tomography angiogram illustrated an acute right pulmonary embolism and a right atrial filling defect. Furthermore, an echocardiogram demonstrated a normal ejection fraction and a large, pedunculated, mobile, and non-valvular echodensity that was attached to the right atrium endocardium. Therefore, the patient was started on a heparin infusion and catheter-directed thrombolysis; however, the mass persisted. A surgical excision was performed, and a 40 mm was removed. The patient was diagnosed with a papillary fibroelastoma based on the clinical symptoms, imaging, and histological findings. CONCLUSION: This patient's papillary fibroelastoma had multiple rare features including right atrial origin, large size, non-valvular location, and developed symptoms. Although this disease can be initially fatal, the patients typically have a favorable prognosis after a successful excision.

Vascular endothelial growth factor receptor-3 promotes breast cancer cell proliferation, motility and survival in vitro and tumor formation in vivo.

Vascular endothelial growth factor receptor-3 is a receptor tyrosine kinase that is overexpressed in some human carcinomas, but its role in tumorigenesis has not been fully elucidated. We examined VEGFR-3 expression in normal, nonneoplastic and early stage malignant breast tissues and have shown that VEGFR-3 upregulation in breast cancer preceded tumor cell invasion, suggesting that VEGFR-3 may function as a survival signal. We characterized the biological effects of VEGFR-3 over-expression in human breast cancer cells based on two approaches: gain of function by overexpressing VEGFR-3 in MCF-7 breast cancer cells and loss of function by RNAi-mediated silencing of VEGFR-3 in MCF-7-VEGFR-3 and BT474 cells. VEGFR-3 overexpression increased cellular proliferation by 40% when MCF7-VEGFR-3 cells were compared to parental MCF7 cells, and proliferation was reduced by more than 40% when endogenous VEGFR-3 was downregulated in BT474 cells. VEGFR-3 overexpression promoted a three-fold increase in motility and invasion and both motility and invasion were inhibited by downregulation of VEGFR-3. Furthermore, VEGFR-3 overexpression promoted cellular survival under stress conditions induced by staurosporine treatment and led to anchorage-independent growth. VEGFR-3 overexpression dramatically increased tumor formation in both hormone-dependent and independent xenograft models. With estrogen stimulation, MCF7-VEGFR-3 xenografts were ten times larger than control xenografts. Finally, downregulation of VEGFR-3 expression in both xenograft model cell lines led to a significant reduction of tumor growth. For the first time, we have demonstrated that VEGFR-3 overexpression promotes breast cancer cell proliferation, motility, survival, anchorage-independent growth and tumorogenicity in the absence of ligand expression.

Isolated metastatic extremity liposarcoma to the liver, an uncommon and transient finding.

BACKGROUND: Extremity liposarcomas can metastasize to different areas of the body but have rarely been demonstrated to metastasize to the liver. Due to the unusual occurrence of isolated metastatic extremity liposarcoma to the liver, the optimal treatment of this condition is unknown. CASE PRESENTATION: Less than one year after resection of a myxoid/round cell liposarcoma of the left lateral calf, a 61-year-old male presented with a CT scan showing a 2 cm low-density lesion in the right lobe of the liver. The lesion tripled in size over the next few months. An extensive evaluation revealed isolated disease to the liver. The lesion was surgically removed with a right hepatic lobectomy and the pathology was consistent with metastatic myxoid/round cell liposarcoma. CONCLUSION: Although extremity liposarcoma rarely metastasizes solely to the liver, the best chance at cure is with complete resection. Unfortunately, cure rates are very low in the setting of metastatic disease. As expected, the patient experienced progression of disease at sites outside of the liver 5 months after the liver resection.

Vascular endothelial growth factor receptor-3 and focal adhesion kinase bind and suppress apoptosis in breast cancer cells.

Focal adhesion kinase (FAK) and vascular endothelial growth factor receptor-3 (VEGFR-3) are protein tyrosine kinases that are overexpressed in human cancer and play an important role in survival signaling. In addition to its involvement with cell survival, VEGFR-3 is a primary factor in lymphatic angiogenesis. Because FAK function is regulated by its COOH terminus (FAK-CD), we used FAK-CD as a target to identify binding partners. We isolated a peptide from a phage library that bound to FAK-CD, specifically the focal adhesion targeting domain of FAK and was homologous to VEGFR-3, suggesting these two tyrosine kinases physically interact. We have also shown that VEGFR-3 is overexpressed in human breast tumors and cancer cell lines. For the first time, we have shown the physical association of FAK and VEGFR-3. The association between the NH(2) terminus of VEGFR-3, containing the peptide identified by phage display, and the COOH terminus of FAK was detected by in vitro and in vivo binding studies. We then coupled a 12-amino-acid VEGFR-3 peptide, AV3, to a TAT cellular penetration sequence and showed that AV3 and not control-scrambled peptide caused specific displacement of FAK from the focal adhesions and affected colocalization of FAK and VEGFR-3. In addition, AV3 peptide decreased proliferation and caused cell detachment and apoptosis in breast cancer cell lines but not in normal breast cells. Thus, the FAK/VEGFR-3 interaction may have a potential use to develop novel molecular therapeutics to target the signaling between FAK and VEGFR-3 in human tumors.

Neoadjuvant chemotherapy of breast cancer.

The use of neoadjuvant chemotherapy in the treatment of breast cancer has been evolving during the past two decades. Fisher's group accomplished the scientific rationale in mouse models in the 1970s. The Milan group was the first to use neoadjuvant therapy in locally advanced breast cancer and also determined that adjuvant sequential doxorubicin with cyclophosphamide/methotrexate/fluorouracil (CMF) offered improved survival when compared to alternating CMF with doxorubicin. Other groups (M. D. Anderson and NSABP) have evaluated similar doxorubicin-based neoadjuvant therapies in locally advanced breast cancer (LABC) and in early disease. These studies have shown an increase in breast-conserving therapy (BCT) and an increase in pathologic complete response (pCR) when neoadjuvant therapy was used. Due to anthracycline resistance, taxanes were added to the doxorubicin-based neoadjuvant chemotherapy regimen with an increase in BCT and pCR than when an anthracycline regimen was used alone. Overall survival has yet to be determined when comparing an anthracycline-based regimen to the same regimen with the addition of a sequential taxane. Therefore, a combined treatment of an anthracycline/cyclophosphamide/taxane regimen is the recommended neoadjuvant chemotherapy for patients with breast cancer.