Radiation-induced myofibroblastoma within the nasal sinus requiring maxillectomy and ethmoidectomy: A case report and literature review.

We report a case of radiation-induced myofibroblastoma of the right nasal cavity in a patient with a remote history of radiotherapy for pediatric retinoblastoma. The patient required maxillectomy and ethmoidectomy. To our knowledge, a rare number of cases have been reported in this location.

Post-kidney transplant cancers: Racial and ethnic differences in sun-exposed skin versus non-sun-exposed anogenital skin.

BACKGROUND: Transplant recipients have a 2- to 4-fold increased risk of developing malignancies over the general population. Cancer is the second most common cause of death for recipients. The magnitude of the risk depends on the cancer type and increases in viral-related malignancies. Skin cancer is the most common. However, data in most cancer registries is limited to cutaneous melanomas, thereby limiting the epidemiologic examination of cancer risk in non-melanoma skin cancer. Our goal was to evaluate post-kidney transplant cancer cases and sites in our population to guide screening recommendations. METHODS: Between 2009 and 2015, a retrospective study of adult kidney recipients transplanted at East Carolina University was conducted. The first cancer diagnosis after transplant through February 18, 2020, was captured and analyzed. Patient demographics, cancer sites, and histological diagnoses were analyzed and compared. p16 immunohistochemistry was used as a surrogate marker for high-risk human papillomavirus (HPV) infection. RESULTS: Retrospectively, kidney transplant recipients were analyzed (N = 439), the majority were non-Hispanic Black (NHB) individuals, 312 (71.1%), and 127 (28.9%) were non-Hispanic White (NHW) individuals. Of these, 59 (13.4%) developed a posttransplant malignancy, with the majority on sun-exposed skin found in NHW. NHB had all anogenital/mucosa skin cancers on non-sun-exposed skin. Of these detected in NHB, all were squamous cell carcinomas, with five out of six (83.3%) being positive for p16. CONCLUSIONS: Posttransplant malignancy differed significantly by race, site, and potential source of etiology. The majority of malignancies are likely explained by acceleration of precursor lesions from prior exposure to ultraviolet rays or HPV.

Primary malignant melanoma of the gastric antrum: A case report.

Primary melanoma of the antrum is a rare and aggressive disease. Diagnosis requires a correlation between the patient's history, tumor histopathology, and immunohistochemistry. Surgery and mutation-targeted treatments may improve overall survival.

Morbihan disease: A diagnostic dilemma: two cases with successful resolution.

Morbihan syndrome is a rare entity characterized by persistent erythema and solid edema of upper two-thirds of the face. Although its etiology is poorly understood, it is known to have a wide differential diagnosis and is frequently under-recognized.1-3 We report two such cases of Morbihan syndrome in patients that responded well to treatment with a combination of 2.5% hydrocortisone cream, brimonidine 0.33% topical gel, metronidazole gel and 100 mg doxycycline twice daily. This report emphasizes the necessity of biopsy for clinical correlation in cases of chronic facial edema. It also serves to highlight a potential association of Morbihan syndrome to diabetes mellitus through recently discovered pathophysiology of diabetes on the lymphatic system. It underscores the effectiveness of our therapeutic regimen in the context of other treatment regimen effectiveness. Finally, it highlights novel advances into the diagnosis and treatment of the disease.

Sipuleucel-T associated inflammatory cardiomyopathy: a case report and observations from a large pharmacovigilance database.

AIMS: The major cardiovascular (CV) adverse effects observed with sipuleucel-T from large multi-institutional clinical trials included thromboembolic events, myocardial infarction, and congestive heart failure in up to 0.3% of patients with CV risk factors. The incidence, outcomes, and mechanisms in real-world clinical settings of these CV adverse effects to date have not been fully elucidated. Our study identified a patient with sipuleucel-T-induced inflammatory cardiomyopathy, which led to the identification of CV adverse effects associated with sipuleucel-T from a large pharmacovigilance database and elucidation of its potential mechanisms. METHODS AND RESULTS: Using the MedDRA term 'cardiac disorders' (System Organ Class level), CV adverse events associated with sipuleucel-T versus all other drugs were reviewed from VigiBase, a large pharmacovigilance database. Disproportionality analysis was calculated by the information component (IC), a Bayesian disproportionality indicator. A positive IC025 (IC 95% lower end credibility interval) value (>0) is the traditional threshold used in statistical signal detection at the Uppsala Monitoring Centre. From VigiBase, the total number of CV adverse drug reaction reported with sipuleucel-T was 306 out of a total of 22 980 104 adverse drug reactions in VigiBase on 10/25/2020. MedDRA preferred terms levels were grouped into major CV adverse drug reaction categories where we observed significant reports of myocardial ischaemia, supraventricular tachycardia (particularly atrial fibrillation/atrial flutter), congestive heart failure, and valvular disorders. Myocardial ischemia included acute myocardial infarction (IC025 2.3) with n = 4/26 (15%) of these individual case safety reports considered fatal. Among patients with 'cardiac failure congestive' (IC025 1.5), 11 of these 43 cases (26%) were fatal with 42 (98%) of these cases considered to be solely due to sipuleucel-T. CONCLUSIONS: Patients with CV risk factors who are receiving sipuleucel-T may be at higher risk for congestive heart failure, myocardial ischemia, and supraventricular tachycardia. Electrocardiograms during weekly sipuleucel-T infusions and left ventricular function monitoring with echocardiogram should be considered in these patients. Our findings are suggestive of another rare presentation of T-cell-mediated CV toxicity with cancer immunotherapy.

A Case Study and Review of Literature of Eruptive Syringoma in a Six-Year-Old.

Syringomas are benign tumors originating from the intraepidermal portion of eccrine sweat ducts. A six-year-old African American female presented with multiple 2-3 mm hyperpigmented papules over the neck, upper chest, and axillae bilaterally. The lesions were non-tender, non-pruritic, and did not bleed when lightly scraped. A café-au-lait macule was incidentally found in the mid-back of the patient. Histopathologically, multiple small ducts displaying a tadpole-shaped/paisley-tie pattern with fibrotic stroma were identified on hematoxylin and eosin staining. Epithelium showing nests of cells with basaloid appearance and dilated glands filled with eosinophilic material were also identified. These histopathologic findings were consistent with a diagnosis of eruptive syringoma. The patient was treated conservatively, and the lesions subsided without intervention. In most patients requesting treatment, isotretinoin is used; however, this may be an unnecessary measure in many patients. Overall, this case was significant due to the patient's young age, ethnicity, and clinical improvement in the absence of treatment.

A Rare Case of Round Cell Sarcoma with CIC-DUX4 Mutation Mimicking a Phlegmon: Review of Literature.

BACKGROUND Undifferentiated small blue round cell sarcomas with CIC-DUX4 translocation differ morphologically and in clinical outcomes from other types of sarcoma. Although classified by the World Health Organization as undifferentiated sarcomas, it is unclear whether these tumors are variants of Ewing's sarcoma or a distinct entity. This report describes a round cell sarcoma with CIC-DUX4 translocation that presented clinically as a phlegmon. CASE REPORT A 31-year-old African American man presented with a mass in the right upper abdominal quadrant. Examination at a local hospital suggested an intra-abdominal abscess, and incision and drainage were performed. One week later, he returned with increased pain and bloody drainage from the incision site. Computed tomography showed a complex solid-cystic area measuring 7.8 cm suggesting a large phlegmon/abscess or neoplasm. Histologically, the sarcomatous malignancy was cellular, multinodular, and necrotic, with cells having round-ovoid to spindled nuclei and variable amounts of pale cytoplasm. Immunohistochemically, the mass was focally positive for CD99, but much less positive than an Ewing sarcoma. The mass also showed diffuse nuclear positivity for WT-1 and ETV4, but was negative for desmin. Fluorescence in-situ hybridization showed positivity for CIC-DUX4 gene fusion, resulting in a final diagnosis of round cell sarcoma with CIC-DUX4 translocation. The patient has completed 14 cycles of chemotherapy with no evidence of metastasis or local recurrence. CONCLUSIONS A round cell sarcoma with CIC-DUX4 translocation can present clinically as a phlegmon with pleomorphic morphology. Early tumor identification by molecular analysis and early initiation of treatment can improve patient prognosis.

Orbital mantle cell lymphoma presenting as myasthenia gravis.

A 69-year-old man, previously treated with pyridostigmine for myasthenia gravis (manifesting as ptosis and diplopia) was evaluated for several concomitant bilateral anterior orbital masses. Imaging revealed 3 discrete, solid masses within and around the orbits. An incisional biopsy demonstrated atypical lymphocytes positive for CD20 and Cyclin-D1, consistent with mantle cell lymphoma. The patient received induction chemotherapy with a rituximab-based regimen. He experienced resolution of his diplopia and ptosis after one cycle of chemotherapy and achieved complete remission of the orbital masses and myasthenia symptoms after 6 cycles. Myasthenia gravis is most commonly associated with thymoma, but may also be observed with other malignancies. Recognition that orbital lymphoma may coexist with myasthenia gravis will help in expediting the diagnosis of future cases and in guiding treatment decisions.