Appendiceal goblet cell carcinoma: comparison of classification and staging systems with evaluation of the prognostic role of immunohistochemistry stains

Background: Goblet cell carcinoma (GCC) of the appendix is a unique lesion that exhibits features of both adenocarcinoma and neuroendocrine tumors. Due to the rarity of this cancer, multiple grading (e.g., Tang, Yozu, and Lee) and staging systems (e.g., tumor, lymph nodes, and metastasis [TNM]) have been developed for classification. This study aimed to compare commonly used classification systems and evaluate the prognostic effectiveness immunohistochemical staining may or may not have for appendiceal GCC. Methods: An electronic medical records review of patients who were diagnosed with GCC of the appendix in our hospital system from 2010 to 2020. The data were collected regarding the age at diagnosis, gender, initial diagnosis at presentation, operation(s) performed, final pathology results, current survival status, and year of recurrent disease or death year. Results: Ten patients were evaluated. Seventy percent of the patients were above the age of 50 years at diagnosis. Postdischarge survival ranged from 1 month to 109 months postdiagnosis. Two patients expired from GCC at 13- and 54-months following diagnosis. When comparing the classification systems, Lee categorized more patients as high risk than Tang and Yozu. Immunohistochemical staining was analyzed using four staining methods: Ki67, E-cadherin, Beta-catenin, and p53. Tumor, lymph nodes, and metastasis staging has supportive evidence for worsening prognosis and overall survival secondary to the depth of invasion of the tumor. Conclusion: Tumor, lymph nodes, and metastasis staging may be superior to the other classification systems in predicting overall mortality. Our study demonstrated that immunohistochemistry staining does not appear to have a significant impact in determining the prognosis for GCC of the appendix. (AU)

An unusual case of B-cell lymphoma of the scalp.

B-cell lymphoma is a lymphoproliferative non-Hodgkin lymphoma arising from B cells, a type of immune lymphocytes that produces antibodies in the follicles of lymph nodes. Primary cutaneous B-cell lymphoma (PCBCL), a subtype of B cell lymphoma, originates within cutaneous tissue without evidence of extracutaneous involvement. There are very few reports of PCBCLs originating in the scalp. The most common tumors of the scalp are usually benign with only 1%-2% being malignant, most being basal cell carcinoma, squamous cell carcinoma, or melanoma. Primary cutaneous follicular cell lymphoma (PCFCL) is regarded as the most common lymphoma of the skin with an indolent course and favorable prognosis due to the response rate to treatment methods such as surgical removal with local radiotherapy, topical drugs, and intralesional therapies. This report highlights a rare case of PCFCL originating in the scalp, to raise awareness of a topic that requires continued established management.

A 40-Year-Old Man with Anemia, Proctitis, Rectal Bleeding, and a Perianal Rash Due to Mpox (Monkeypox) Infection.

BACKGROUND A recently described zoonotic viral infection, mpox (monkeypox), is an Orthopoxvirus transmitted by close contact, which causes symptoms similar to smallpox, although less severe. This report presents the case of a 40-year-old man with anemia, proctitis, rectal bleeding, and a perianal rash due to mpox infection. CASE REPORT A 40-year-old man with a medical history of human immunodeficiency virus (HIV) and syphilis presented multiple times with progressive and painful perianal lesions. On initial presentation, swabbing of the lesions and polymerase chain reaction (PCR) testing confirmed a diagnosis of mpox infection, and treatment with a 14-day course of Tecovirimat was started. Nine days after initiating Tecovirimat, the patient presented again with worsening perianal pain and associated hematochezia resulting in acute symptomatic anemia. Despite a blood transfusion to treat his anemia, the patient's status declined as his viral symptoms progressed. Computed tomography (CT) investigation demonstrated significant proctitis with interval development of small perianal abscesses. A multidisciplinary approach for medical management and treatment was instituted. The resolution of the patient's anemia and mpox proctitis was confirmed on follow-up. CONCLUSIONS Despite treatment with antiviral agents, mpox infection can progress quickly; thus, swift management with a multidisciplinary approach and close follow-up is needed to treat and prevent secondary complications such as anemia and proctitis. Further data collection regarding the sexual practices of those with diagnoses of mpox as well as seminal, anorectal, and genital swabbing would be valuable to confirm the mode of transmission and cause of mpox proctitis.

Intramedullary Spinal Cord Abscess Management: Case Series, Operative Video, and Systematic Review.

BACKGROUND: Intramedullary spinal cord abscess (ISCA) is an extremely rare disease, which has had fewer than 250 reported cases since its initial description in 1830. The condition is limited to level V evidence, limiting the ability for surgeons to characterize and treat it. OBJECTIVE: To report the cases of 2 patients with ISCA and their surgical management: a 59-year-old woman who presented with progressive right hemiparesis and a 69-old man who presented with acute gait instability and significant bilateral shoulder pain. In addition, to report findings from a systematic literature review and associated logistic regression analysis. METHODS: A MEDLINE and Embase search was conducted using the keywords "intramedullary," "spinal cord," "abscess," and "tuberculoma" and the results were screened for case reports. A logistic regression model was fit 100 times on data to retrieve predictor odds ratios. RESULTS: Two hundred case reports of ISCA were identified between 1965 and 2022. Logistic regression determined that the only variables of significance were age (P < 0.01) and antibiotics (P < 0.05). CONCLUSIONS: Treatment of ISCAs has significantly improved over the years. However, ISCAs are still poorly understood. Our recommendations can be used to guide diagnosis and treatment.

A Rare Case of Superior Vena Cava Syndrome in a Patient With Rheumatoid Arthritis and IgA Nephropathy.

Superior vena cava syndrome (SVCS) is a vascular condition resulting from an impaired venous return to the right atrium. The majority of SVCS cases are caused by mass effect in which extrinsic compression of the vessel leads to obstruction of blood flow. In less common cases of SVCS, thrombus formation and luminal narrowing can result in poor return through the SVC. Inflammatory causes of SVCS are even rarer and poorly documented. IgA nephropathy and rheumatoid arthritis (RA) are two autoimmune diseases with the potential to cause vasculitis, thus increasing the likelihood of intraluminal vessel occlusion. We report a rare case of SVCS in a 65-year-old female with a past medical history significant for atrial fibrillation, IgA nephropathy, chronic kidney disease stage IIIA, and RA who presented with headache, dizziness, and neck pain and swelling extending down the left upper extremity for three days. Inflammatory SVCS is uncommon and cases of SVCS secondary to RA and IgA nephropathy are underreported in the literature thus far. Our hope in presenting this case is to encourage a greater degree of suspicion for vascular complications, such as SVCS, in patients with autoimmune and inflammatory conditions.

Immune Checkpoint Inhibitor-Associated Thrombotic Thrombocytopenic Purpura in a Patient With Metastatic Non-Small-Cell Lung Cancer.

BACKGROUND: Immune-related adverse events (irAEs) are secondary reactions related to treatment with immune checkpoint inhibitors (ICIs). There have been six cases published reporting on an association between patients undergoing treatment with ICIs and the occurrence of acquired thrombotic thrombocytopenic purpura (TTP). CASE REPORT: We report a 61-year-old male receiving treatment with chemoimmunotherapy followed by pembrolizumab maintenance therapy for advanced non-small-cell lung cancer, presenting with bleeding symptoms, anemia, and thrombocytopenia. The patient received pembrolizumab seven times in total, in three-week cycles. Laboratory testing demonstrated hemolytic anemia, which, in combination with other findings, suggested thrombotic microangiopathy (TMA). PLASMIC scoring and specialized testing with ADAMTS13 activity and inhibitor confirmed a diagnosis of TTP. The patient was started on therapy with plasmapheresis and glucocorticoids, resulting in clinical improvement. The patient chose to leave the hospital under the care of home hospice and died approximately one month after being discharged. CONCLUSIONS: Of the six cases of ICI-induced TTP, only one other was treated with pembrolizumab to our knowledge to date. Our patient experienced an adverse reaction marked by thrombocytopenia and hematuria after drug exposure. With symptom improvement after ICI discontinuation and recurrence on readministration, a presumptive diagnosis of ICI-associated TTP was made. This case report and literature review emphasize the need for close observation of patients undergoing ICI therapy for potential rare irAEs. The further investigation aimed at the study of risk factors, disease severity, and treatment response to this form of secondary TTP is needed to guide treatment decisions.