Thoracic lymphangiomatosis in a child

An 8-year-old boy who presented with a mediastinal mass, pulmonary infiltrates, and disseminated intravascular coagulation was diagnosed with lymphangiomatosis. Despite medical management, he developed multiple organ failure and died. The authors discuss the diagnostic findings, medical management, and pathology and review 52 additional cases of thoracic lymphangiomatosis from the literature. Patients presented with chylothorax (49%), a mass (47%), pulmonary infiltrates (45%), bone lesions (39%), splenic lesions (19%), cervical involvement (15%), disseminated intravascular coagulation (9%), and skin involvement (7%). Children (<16 years) had a worse prognosis than older patients (39% vs. 0% mortality). All patients who died had either parenchymal lung involvement or pleural effusion. Thoracic lymphangiomatosis should be included in the differential diagnosis of a mediastinal mass with pulmonary findings

Bioluminescent Mammalian Cells Grown in Sponge Matrices to Monitor Immune Rejection

The growth and bioluminescence of cells seeded in collagen and gelatin sponge matrices were compared in vitro under different conditions, and immune rejection was quantified and visualized directly in situ based on loss of bioluminescence activity. Mammalian cells expressing a Renilla luciferase complementary deoxyribonucleic acid (cDNA) were used to seed collagen and gelatin sponge matrices soaked in either polylysine or gelatin to determine optimal growth conditions in vitro. The sponges were incubated in tissue culture plates for 3 weeks and received 2, 9, or 15 injections of coelenterazine. Measurements of bioluminescence activity indicated that gelatin sponges soaked in gelatin emitted the highest levels of light emission, multiple injections of coelenterazine did not affect light emission significantly, and light emission from live cells grown in sponges could be measured qualitatively but not quantitatively. Histologic analysis of sponge matrices cultured in vitro showed that cells grew best in gelatin matrices. Visualization of subcutaneously implanted sponges in mice showed accelerated loss of light emission in immunocompetent BALB/c mice compared with immunodeficient BALB/c- scid mice, which was associated with increased cell infiltration. Our results indicate that sponge matrices carrying bioluminescent mammalian cells are a valid model system to study immune rejection in situ

Synovitis, acne, pustulosis, hyperostosis, and osteitis (SAPHO) syndrome presenting with a cervical vertebral fracture: A case report

Background context: Synovitis, acne, pustulosis, hyperostosis, and osteitis (SAPHO) syndrome is a rare condition that can be difficult to diagnose. There are no guidelines for the treatment of SAPHO syndrome, but newer modalities of medications show promising results. We present the case of a patient who presented with a pathologic fracture of her cervical spine who ended up being diagnosed with SAPHO syndrome. Case: A 51-year-old female presented with severe neck pain and a rash on her hands and feet. Imaging showed a C5 vertebral compression fracture and multiple sites of bony involvement concerning for malignancy or widespread infection. The patient underwent corpectomy and fusion to address the instability and cervical stenosis and was started on immunomodulating therapy. Based on the biopsy findings showing left shifted bone marrow versus mild acute inflammation, and in conjunction with the cutaneous findings, the patent was diagnosed with SAPHO syndrome. Outcome: At two year follow up, although posterior stabilization was required, her overall condition was improved. Nonetheless, she continued to have fatigue, malaise, and total body pain involving: the cervical spine, the mid thoracic spine, the left costal margin, bilateral sternoclavicular joints, and bilateral hips and knees. Conclusion: SAPHO syndrome can mimic infection and neoplasia. It should be suspected in patients presenting with multifocal osteitis and associated rash. Accurate and timely diagnosis is paramount as the treatment of this condition may require immunomodulating agents

Aggressive primary hepatic histiocytic sarcoma: case report and literature review

Histiocytic sarcoma is an uncommon non-Langerhans histiocyte disorder of mature tissue histiocytes. The authors presented an example of this rare tumor in a 14-year-old girl who presented with left upper quadrant pain, loss of appetite, and weight loss. A large 18 cm × 10 cm heterogeneous solid and cystic enhancing mass was found in the left lobe of the liver. Based on the histomorphology and positivity for histiocyte-specific markers in a needle biopsy, a diagnosis of histiocytic sarcoma was made. Chemotherapy was initiated, but the tumor did not respond well, and she died about 7 weeks following initial diagnosis with multi-organ failure. At autopsy, the tumor showed extensive necrosis, with no evidence of metastatic spread. In conclusion, the diagnosis of histiocytic sarcoma is challenging, and requires a high index of suspicion, with an appropriate panel of confirmatory immunohistochemical stains. Recognition of this rare tumor is important because of its poor response to chemotherapy and high mortality

AIDS-Associated BK Virus Nephropathy in Native Kidneys: A Case Report and Review of the Literature

BK virus (BKV) is a small DNA virus, a member of the polyomavirus family, that causes an opportunistic infection in immunocompromised patients, especially kidney transplant patients. This virus establishes a lifelong infection in most of the population, and once it reactivates in an immunocompromised state, leads to BKV nephropathy. This review seeks to assess the correlation between severe immunosuppression, evident by low CD4 cell counts in HIV-positive patients, and the reactivation of BKV, causing nephropathy. A literature review was conducted, extracting, and analyzing case reports of HIV-positive patients showing correlations between their degree of immunosuppression, as evidenced by their CD4 counts, and the degree of BKV infectivity, confirmed by kidney biopsy. A total of 12 cases of BKV nephropathy in HIV-infected patients were reviewed. A common finding was the presence of profound immunosuppression, with most patients having CD4 counts ≤50 cells/ mm3. A substantial number also had comorbid malignancies, with some undergoing chemotherapy, potentially increasing the risk of BKV reactivation. In addition to the HIV status and malignancies, other risk factors for BKV reactivation included older age, male gender, diabetes mellitus, Caucasian race, and ureteral stent placement. BKV nephropathy in HIV patients with native kidneys is closely correlated with severe immunosuppression. Although therapeutic strategies exist for post-transplant patients, aside from the treatment of HIV with highly active anti-retroviral therapy (HAART), which potentially helps with clearing BKV by increasing CD4 count, there is no definitive treatment for a native kidney BKV nephropathy in patients with AIDS. The complexity of the cases and severity of comorbidities indicate the need for further research to develop therapeutic strategies tailored to this population

Benign Giant Cell Lesion of C1 Lateral Mass: A Case Report and Literature Review

Primary osseous tumors of the spinal column account for approximately 1% of the total number of spinal tumors found in the pediatric patient population. The authors present a case of a C1 benign giant cell lesion that was incidentally found in a 15-year-old patient. A transoral biopsy was performed followed by treatment with denosumab, with definitive management in the form of transoral tumor resection with subsequent occiput-cervical three posterior instrumented fusion. The patient tolerated all of the procedures well, as there were no post-operative complications, discharged home neurologically intact and was eager to return to school when assessed during a follow-up visit in clinic. Osteolytic lesions affecting the cervical spine are rare in the pediatric population. It is of utmost importance to have sufficient background knowledge in order to formulate a differential diagnosis, as well as an understanding of principles underlying surgical techniques required to prevent occipital-cervical instability in this patient population. The information presented will guide surgical decision-making by identifying the patient population that would benefit from neurosurgical interventions to stabilize the atlantoaxial junction, in the context of rare osteolytic conditions affecting the cervical spine

Relationship of Post-Transplant Lymphoproliferative Disorders (PTLD) Subtypes and Clinical Outcome in Pediatric Heart Transplant Recipients: A Retrospective Single Institutional Analysis/Experience of 558 Patients

Post-transplant lymphoproliferative disorders (PTLD) are heterogenous lymphoproliferative disorders that develop as a consequence of immunosuppression in transplant recipients. We sought to determine if subtypes of PTLD correlated with different outcomes. We performed a retrospective review of PTLD occurring in pediatric heart transplant recipients. A total of 558 children and infants underwent cardiac transplantation at our institution between 1985 and 2019 and were followed until March 2021. Forty-nine of 558 patients developed PTLD (8.8%). As compared to older children (>one year of age), infant recipients (p = 0.04). Although not reaching statistical significance, patients with a shorter time to PTLD diagnosis showed a trend toward higher rates of M-PTLD or cHL. As expected, the overall survival (OS) of patients with M-PTLD or cHL was significantly lower than patients with non-destructive or polymorphic PTLD