Multiple myeloma is a relatively common clonal plasma cell disorder, comprising 17% of hematologic malignancies. One of the hallmark features of this disease is immunoparesis, which is characterized by the suppression of immunoglobulin polyclonality. Though not entirely elucidated, the mechanism behind this process can be attributed to the changes in the tumor microenvironment. All treating clinicians must consider potential complications related to immunoparesis in the management of multiple myeloma. Though not explicitly described in large data series, the increased risk of infection in multiple myeloma is likely, at least in part, due to immunoglobulin suppression. Additionally, the presence of immunoparesis serves as a prognostic factor, conveying poorer survival and a higher risk of relapse. Even in the era of novel agents, these findings are preserved, and immunoglobulin recovery also serves as a sign of improved outcome following autologous HSCT. Though not within the diagnostic criteria for multiple myeloma, the presence and degree of immunoparesis should be at diagnosis for prognostication, and immunoglobulin recovery should be tracked following myeloablative therapy and autologous HSCT.

Malignant peripheral nerve sheath tumors (MPNSTs) are growths that arise in conjunction with a peripheral nerve and are believed to originate from neural crest cells. These tumors can arise sporadically but are often associated with the cancer-predisposing genetic condition, neurofibromatosis type 1 (NF-1). The clinical presentation of an enlarging mass, pain, paresthesias, and neurologic deficits can mirror that of other soft tissue sarcomas. Thus, clinical suspicion should remain high for an MPNST when this aggregation of symptoms arises, particularly in those with a genetic proclivity. We report the case of metastatic MPNST in a 44-year-old female with a long history of NF-1. She was first seen for evaluation of progressive forearm and hand weakness associated with numbness and paresthesias in her second through fourth digits which prompted a need for an MRI. A forearm mass was discovered, and she underwent surgical intervention which revealed an MPSNT with positive margins. The patient completed radiation therapy for this lesion, but ultimately her forearm lesion recurred and progressed with metastasis to the lungs. Local recurrence was managed with a trans-humeral amputation and her systemic involvement necessitated chemotherapy. She was ultimately enrolled in a clinical trial for adult patients with recurrent advanced solid tumors. Given the potentially fatal course of NF-1-associated MPNSTs, clinical suspicion should remain high and early diagnosis and intervention with regular clinical surveillance are of utmost importance in this patient population.

Adrenal cortical carcinosarcomas are a rare and typically aggressive malignancy with few reported cases in medical literature. We present a case of a 78-year-old female who presented with complaints of fatigue and right shoulder pain. Imaging of the abdomen with computed tomography visualized a large mass in the right upper quadrant. The mass was radiologically described as a 22 × 17 × 13 cm heterogeneous mass with its epicenter in the area of the right adrenal gland, with medial and peripheral effacement of all structures in the right upper quadrant. Non-contrasted images demonstrated anterior mid-portion calcifications. The mass parasitized its blood supply from several surrounding structures, including the liver and right psoas muscle, and extensively invaded the psoas muscle. Resection of the mass was performed with pathology, which revealed a high mitotic index and nuclear atypia with two morphologically and immunophenotypically distinct components. One of these components stained positively for calretinin and inhibin, which is indicative of adrenal cortical carcinoma; the other exhibited strong expression of vimentin and desmin, which was concordant with sarcomatous change and confirmed the diagnosis of adrenal cortical carcinosarcoma. This unique histology with both carcinomatous and sarcomatous components presents a diagnostic challenge for clinicians. As such, adrenal carcinosarcomas should be kept on the differential when evaluating retroperitoneal masses. Additionally, this study includes a review of 34 previously reported cases of adrenal cortical carcinosarcomas along with a discussion about the future exploration of this pathology.

Background: Neuroendocrine carcinomas (NECs) of the tubular gastrointestinal tract (GI-NECs) are rare and associated with worse clinical outcomes. This population-based study aims to highlight key demographics, clinicopathological factors, and survival outcomes in the US population. Methods: Data from 10,387 patients with GI-NECs were extracted from the Surveillance, Epidemiology, and End Result (SEER) database from 2000 to 2020. Results: Most patients were >40 years old at the time of presentation with a median age of 63 years old, with almost equal ethnic distribution per US population data. The most common primary tumor site was the small intestine (33.6%). The metastatic spread was localized in 34.8%, regional in 27.8%, and distant in 37.3% of cases, and the liver was the most common site of metastasis (19.9%) in known cases of metastases. Most NEC patients underwent surgery, presenting the highest 5-year overall survival of 73.2% with a 95% confidence interval (CI) (95% CI 72.0–74.4%), while chemotherapy alone had the lowest 5-year survival of 8.0% (95% CI 6.4–10.0%). Compared to men, women had a superior 5-year survival rate of 59.0% (95% CI 57.6–60.5%). On multivariate analysis, age > 65 (HR 2.49, 95% CI 2.36–2.54%, p ≤ 0.001), distant metastasis (HR 2.57, 95% CI 2.52–2.62%, p ≤ 0.001), tumor size > 4 mm (HR 1.98, 95%, CI 1.70–2.31%, p ≤ 0.001), esophageal (HR 1.49, 95% CI 0.86–2.58%, p ≤ 0.001), transverse colon (HR 1.95, 95% CI 1.15–3.33%, p ≤ 0.01), descending colon (HR 2.12, 95% CI 1.12, 3.97%, p = 0.02) anorectal sites, and liver or lung metastases were associated with worse survival. Surgical intervention and tumors located in the small intestine or appendix showed a better prognosis. Conclusion: GI-NECs are a group of rare malignancies associated with a poor prognosis. Therefore, epidemiological studies analyzing national databases may be the best alternative to have a more comprehensive understanding of this condition, assess the impact of current practices, and generate prognosis tools.

Background and Clinical Significance: Common Variable Immunodeficiency (CVID) is a prevalent manifestation of primary immunodeficiency disorder. The current mainstay of treatment is immunoglobulin replacement therapy; however, in patients with severe complications or refractory disease, hematopoietic stem cell transplant (HSCT) is indicated. Despite this, there has been little research regarding HSCT as a treatment for CVID, with few case reports demonstrating clinical benefit. Case presentation: We present a unique case of common variable immunodeficiency Type XII (CVID12) with rare NFKB mutation and its management. A 20-year-old female with autoimmune alopecia, eczema, and a congenital atrophic right kidney presented to the emergency department with a three-month history of intermittent fever, malaise, lymphadenopathy, mouth sores, diarrhea, and odynophagia, accompanied by a 5 lb. unintentional weight loss and night sweats. Previously, she received multiple steroid prescriptions for these symptoms, providing only temporary relief with each course. Lab findings revealed severe neutropenia and imaging demonstrated hepatosplenomegaly and lymphadenopathy. Flow cytometry revealed a slightly atypical CD8-positive T-cell population and bone marrow biopsy revealed variable cellular marrow with trilineage hematopoiesis. Genetic testing confirmed the diagnosis of Autosomal Dominant Common Variable Immunodeficiency Type XII with an NFKB1 mutation. Pre-transplant treatments included monthly IVIG, weekly rituximab, and daily filgrastim, all of which failed to improve her autoimmune neutropenia and hypogammaglobulinemia and failed to reduce her symptomatic burden. Given the patient’s young age and refractory autoimmune neutropenia, it was decided to manage them definitively with hematopoietic stem cell transplantation (HSCT). She ultimately underwent allogenic stem cell transplantation (haploidentical, donor was the mother) with 3.96 × 10⁸/kg TNC without immediate post-transplant complications. Conclusions: This article demonstrates a rare case of NFKB1-positive CVID that was successfully treated with HSCT and highlights the importance of considering transplant therapy in younger patients with clinically significant, refractory autoimmune cytopenia.

Basaloid squamous cell carcinoma (BSCC) is a variant of squamous cell carcinoma that is most often seen as a variety of primary head and neck cancers. BSCC of the lung is rare primary lung cancer and an uncommon histological subtype of non-small cell lung carcinoma. Due to its rarity, there is little published information and research available to direct disease-specific management of BSCC of the lung. Most published cases of basaloid carcinoma of the lung report on surgical management of eligible patients and even less information can be found for metastatic cases. We report a case of a 74-year-old male with stage four (IV) BSCC of the lung who experienced a complete metabolic with partial anatomic response to combined chemotherapy and immunotherapy with carboplatin/nab-paclitaxel/pembrolizumab and has continued to be in partial remission on maintenance immunotherapy with pembrolizumab despite PD-L1-negative status.