Pulmonary Glomus Tumor.

Glomus tumors, which account for less than 2% of soft tissue tumors, are a rare benign soft tissue neoplasm. They originated from neuro-myo-arterial glomus tissue whose primary function is regulation of the body temperature. This tissue is commonly located in the dermis or subcutis in the subungual region; however, it can be extracutaneous such as in bones, the genitourinary tract, the gastrointestinal tract, and the respiratory tract. Histologically, a glomus tumor is made of proliferating rounded or cuboidal epithelioid cells in a meshwork of blood vessels. Although a benign growth, they can rarely show malignant features with infiltration of surrounding tissue with the rapid multiplication of cells in which case it is labeled as a malignant glomus tumor. Pulmonary glomus tumors are extremely rare and most commonly occur in middle-aged men. They are mostly asymptomatic, but a small percentage of patients may present with hemoptysis and cough if there is large airway involvement. We present an interesting case of a middle-aged man presenting with cough and occasional hemoptysis, found to have an endobronchial nodular lesion, and subsequently diagnosed with a pulmonary glomus tumor.

Blood Flow Restriction Therapy for 2 Weeks Prior to Anterior Cruciate Ligament Reconstruction Did Not Impact Quadriceps Strength Compared to Standard Therapy.

PURPOSE: To evaluate the efficacy of a 2-week home-based blood flow restriction (BFR) prehabiliation program on quadriceps strength and patient-reported outcomes prior to anterior cruciate ligament (ACL) reconstruction. METHODS: Patients presenting with an ACL tear were randomized into two groups, BFR and control, at their initial clinic visit. Quadriceps strength was measured using a handheld dynamometer in order to calculate peak force, average force, and time to peak force during seated leg extension at the initial clinic visit and repeated on the day of surgery. All patients were provided education on standardized exercises to be performed 5 days per week for 2 weeks between the initial clinic visit and date of surgery. The BFR group was instructed to perform these exercises with a pneumatic cuff set to 80% of limb occlusion pressure placed over the proximal thigh. Patient-Reported Outcome Measurement System Physical Function (PROMIS-PF), knee range of motion, and quadriceps circumference were gathered at the initial clinic visit and day of surgery, and patients were monitored for adverse effects. RESULTS: A total 45 patients met inclusion criteria and elected to participate. There were 23 patients randomized to the BFR group and 22 patients randomized into the control group. No significant differences were noted between the BFR and control groups in any demographic characteristics (48% vs 64% male [P = .271] and average age 26.5 ± 12.0 vs 27.0 ± 11.0 [P = .879] in BFR and control, respectively). During the initial clinic visit, there were no significant differences in quadriceps circumference, peak quadriceps force generation, time to peak force, average force, pain, and PROMIS scales (P > .05 for all). Following completion of a 2-week home prehabilitation protocol, all patients indeterminant of cohort demonstrated decreased strength loss in the operative leg compared to the nonoperative leg (P < .05 for both) However, there were no significant differences in any strength or outcome measures between the BFR and control groups (P > .05 for all). There were no complications experienced in either group, and both were compliant with the home-based prehabilitation program. CONCLUSIONS: A 2-week standardized prehabilitation protocol preceding ACL reconstruction resulted in a significant improvement in personal quadriceps peak force measurements, both with and without the use of BFR. No difference in quadriceps circumference, strength, or patient reported outcomes were found between the BFR and the control group. The home-based BFR prehabiliation protocol was found to be feasible, accessible, and well tolerated by patients. LEVEL OF EVIDENCE: Level II, randomized controlled trial with small effect size.

A TCR mimic CAR T cell specific for NDC80 is broadly reactive with solid tumors and hematologic malignancies.

Target identification for chimeric antigen receptor (CAR) T-cell therapies remains challenging due to the limited repertoire of tumor-specific surface proteins. Intracellular proteins presented in the context of cell surface HLA provide a wide pool of potential antigens targetable through T-cell receptor mimic antibodies. Mass spectrometry (MS) of HLA ligands from 8 hematologic and nonhematologic cancer cell lines identified a shared, non-immunogenic, HLA-A*02-restricted ligand (ALNEQIARL) derived from the kinetochore-associated NDC80 gene. CAR T cells directed against the ALNEQIARL:HLA-A*02 complex exhibited high sensitivity and specificity for recognition and killing of multiple cancer types, especially those of hematologic origin, and were efficacious in mouse models against a human leukemia and a solid tumor. In contrast, no toxicities toward resting or activated healthy leukocytes as well as hematopoietic stem cells were observed. This shows how MS can inform the design of broadly reactive therapeutic T-cell receptor mimic CAR T-cell therapies that can target multiple cancer types currently not druggable by small molecules, conventional CAR T cells, T cells, or antibodies.

Ketogenic Weight Loss Diet Rapidly Unmasks an Insulinoma.

The ketogenic diet, a diet high in fat and extremely low in carbohydrates, has recently gained momentum and is increasingly employed by patients in order to lose weight. We report a case of an otherwise healthy 47-year-old male who developed seizures and hypoglycemia shortly after initiating this diet. Biochemical testing confirmed hyperinsulinemic hypoglycemia and a subsequent abdomen MRI showed a pancreatic mass. The tumor was ultimately excised with pathology confirming an insulinoma. It was felt that his abrupt decrease in carbohydrate consumption led to the rapid unmasking of this tumor.

Pilot Study of the Mirabilis System Prototype for Rapid Noninvasive Uterine Myoma Treatment Using an Ultrasound-Guided Volumetric Shell Ablation Technique.

STUDY OBJECTIVE: The primary objective of this pilot study was to evaluate the safety and acute tissue ablation efficacy of a transabdominal high-intensity focused ultrasound (HIFU) prototype device that uses ultrasound imaging guidance for rapid noninvasive ablation of uterine myomas. The secondary objective was to assess preliminary myoma-related symptom improvement and myoma volume reduction at 3 to 6 months post-treatment in subsets of patients. DESIGN: Multicenter prospective single-arm pilot study (Canadian Task Force classification II-2). SETTING: University-affiliated teaching hospital and private community hospital. PATIENTS: Women with a diagnosis of symptomatic uterine myomas planning to undergo hysterectomy. INTERVENTIONS: Seventy-three women underwent transabdominal ultrasound-guided HIFU treatment using a volumetric ablation technique referred to as "shell ablation," in which the HIFU energy is deposited in patterns that partially encapsulate the peripheral region of the targeted myoma(s). Patients were divided into 2 sequential cohorts, the development cohort (the first 37 patients treated) and the validation cohort (the final 36 patients treated). Development cohort treatments were performed for dose-ranging purposes to identify the optimum HIFU treatment parameters, whereas the validation cohort treatments were performed to validate these final settings. Sixty-five patients (89.0%) received only prophylactic oral, sublingual, or intramuscular analgesia before treatment, sometimes with oral anxiolytics. The remaining 8 patients (11.0%) were anesthetized before treatment. Sixty-seven patients (91.8%) then had scheduled hysterectomies between 0 and 179 days after treatment completion. Adverse events were monitored until study exit, which ranged from 10 to 191 days post-treatment. MEASUREMENTS AND MAIN RESULTS: The primary efficacy endpoint measured in all 73 patients was the nonperfused volume (NPV) of tissue produced, which was assessed between 0 and 7 days post-treatment either by tissue sectioning after hysterectomy or by gadolinium-enhanced magnetic resonance imaging. Secondary efficacy endpoints were also measured in subsets of patients who were prospectively scheduled for delayed hysterectomies: Changes in menstrual blood loss (MBL), symptom severity (SS), and quality of life (QOL) scores were assessed using validated techniques at 3 months post-treatment in 10 patients and changes in treated myoma volume were assessed using magnetic resonance imaging at 3 to 6 months post-treatment in 14 patients. In all 73 patients, there were no reports of any serious adverse device effects, including no damage to any extrauterine collateral tissues or the abdominal skin. In the development cohort, a mean NPV of 17.9 ± 24.9 cm3 (range, 0-123.0) was produced in a mean total treatment time of 4.9 ± 2.4 minutes (range, 1.1-11.3). These metrics improved in the validation cohort, where a mean NPV of 44.9 ± 58.5 cm3 (range, 0-284.7) was produced in a mean total treatment time of 3.6 ± 2.1 minutes (range, 1.5-9.5). In the subsets of patients with data available, there was a significant improvement in QOL score (median, 16.5 point increase; p = .011), an improving trend in SS score (median, 13.5 point decrease; p = .254), and a significant improvement in treated myoma volume (mean, 24.0% decrease; p = .013). In 8 patients who had above-average MBL scores at baseline and regular menstrual cycle lengths during follow-up, there was also a significant improvement in MBL score (median, 40.8% decrease; p = .035). CONCLUSION: Ultrasound-guided HIFU ablation with the prototype device demonstrated an excellent safety profile and produced clinically relevant NPVs in a mean total treatment time of under 4 minutes using the final validated treatment settings. Short-term clinical efficacy metrics assessed in subsets of patients were encouraging, and larger studies should be conducted to confirm these results (ClinicalTrials.gov, NCT01946178).

Incidence and features of eosinophilic esophagitis in dysphagia: a prospective observational study.

OBJECTIVE: The incidence and symptoms associated with eosinophilic esophagitis (EoE) varies with geographic location, present in 7-15% dysphagic European or North American adults. We aimed to determine incidence and features of EoE in a dysphagic New Zealand population. MATERIALS AND METHODS: 101 consecutive patients presenting with dysphagia to a New Zealand teaching hospital completed a questionnaire (demographics and history) before upper gastrointestinal endoscopy and esophageal biopsies. RESULTS: The incidence of EoE was 14.1% in those having esophageal biopsies. Patients with EoE were younger (median age 38 years, cohort 58 years: OR 9.2 for age ≤ 40; p < 0.001), more frequently male (19.1% versus 7.4% of females: OR 4.7; p < 0.05), and had longer symptom duration (median 262 weeks versus 130.6 weeks: p = NS) with non-continuous symptoms (continuous symptoms 8.3% EoE versus 16.2% cohort: p = NS). Progressive symptoms, level of dysphagia and history of allergy/atopy occurred with almost identical frequency in those with and without EoE. Classic endoscopic features of EoE had a sensitivity and specificity of 30.6 and 93.2%, respectively. CONCLUSIONS: EoE occurs in an adult dysphagic population in New Zealand with similar frequency to that reported in Europe and North America. Demographics and features of history associated with EoE are described and the need to take esophageal biopsies in this population emphasized by the relatively low sensitivity of endoscopic features for the condition.

Likelihood of bone recurrence in prior sites of metastasis in patients with high-risk neuroblastoma.

PURPOSE/OBJECTIVES: Despite recent improvements in outcomes, 40% of children with high-risk neuroblastoma will experience relapse, facing a guarded prognosis for long-term cure. Whether recurrences are at new sites or sites of original disease may guide decision making during initial therapy. METHODS AND MATERIALS: Eligible patients were retrospectively identified from institutional databases at first metastatic relapse of high-risk neuroblastoma. Included patients had disease involving metaiodobenzylguanidine (MIBG)-avid metastatic sites at diagnosis and first relapse, achieved a complete or partial response with no more than one residual MIBG-avid site before first relapse, and received no total body irradiation or therapy with (131)I-MIBG before first relapse. Anatomically defined metastatic sites were tracked from diagnosis through first relapse to determine tendency of disease to recur at previously involved versus uninvolved sites and to assess whether this pattern was influenced by site irradiation. RESULTS: Of 159 MIBG-avid metastatic sites identified among 43 patients at first relapse, 131 (82.4%) overlapped anatomically with the set of 525 sites present at diagnosis. This distribution was similar for bone sites, but patterns of relapse were more varied for the smaller subset of soft tissue metastases. Among all metastatic sites at diagnosis in our subsequently relapsed patient cohort, only 3 of 19 irradiated sites (15.8%) recurred as compared with 128 of 506 (25.3%) unirradiated sites. CONCLUSIONS: Metastatic bone relapse in neuroblastoma usually occurs at anatomic sites of previous disease. Metastatic sites identified at diagnosis that did not receive radiation during frontline therapy appeared to have a higher risk of involvement at first relapse relative to previously irradiated metastatic sites. These observations support the current paradigm of irradiating metastases that persist after induction chemotherapy in high-risk patients. Furthermore, they raise the hypothesis that metastatic sites appearing to clear with induction chemotherapy may also benefit from radiotherapeutic treatment modalities (external beam radiation or (131)I-MIBG).

Acute liver dysfunction as a presentation of haemophagocytic lymphohistiocytosis.

Haemophagocytic lymphohistiocytosis (HLH) is an uncommon disorder of histiocyte function which presents with fever, cytopaenias and end-organ dysfunction. We report the case of a 62-year-old man with acute liver dysfunction as an initial presentation of HLH.

Cutaneous plasmablastic lymphoma in an immunocompetent patient with long-term pyrimethamine use for essential thrombocythemia: a case report and literature review.

We report a case of Epstein-Barr-virus-(EBV-) positive primary cutaneous plasmablastic lymphoma in a human-immunodeficiency-virus-(HIV-) negative, immunocompetent 62-year-old female patient. We postulate that her lymphoma development is due to the longstanding use of pyrimethamine for essential thrombocythemia. This has never been described in the literature.

Cap polyposis mistaken for Crohn's disease: case report and review of literature.

We report the case of a 19-year old male who presented with collapse and hypoglycemia associated with two weeks of frequent hard stools, abdominal pain relieved by defecation, postprandial vomiting and significant weight loss. Radiologically and endoscopically a diagnosis of Crohn's colitis was made and the patient was treated with steroids and immunosuppression. Following several hospital admissions treatment had to be escalated to include anti-TNF-α agents. Despite maximum therapy the patient continued to deteriorate symptomatically and biochemically with severe hypoalbuminemia and persistent anemia and a total colectomy was performed. Intra-operative finding was that of an inflamed large intestine and pseudo-polyposis but histology was reported as cap polyposis. The specimen was compared with the biopsies obtained from the earlier colonoscopies and it was felt that the previous samples were taken from areas of severely inflamed polypoid mucosa with histology mimicking colitis in inflammatory bowel disease.

A case of hemophagocytic lymphohistiocytosis in a patient with chronic lymphocytic leukemia after treatment with fludarabine, cyclophosphamide, and rituximab chemotherapy, with autopsy findings.

Hemophagocytic lymphohistiocytosis (HLH) is rarely described in association with chronic lymphocytic leukemia (CLL), mostly triggered by disease progression or concurrent infection. A 68-year-old male received 4 cycles of fludarabine, cyclophosphamide, and rituximab (FCR) for CLL and achieved a complete response. Twenty-four days after the last chemotherapy, he presented with febrile neutropaenia and was diagnosed with HLH. The diagnosis was based upon persistent fever, pancytopenia, hyperferritinemia, splenomegaly, and hemophagocytosis on bone marrow aspirate. He began treatment with dexamethasone, cyclosporine, and etoposide. Fever resolved and hyperferritinemia improved but pancytopenia persisted. He died 13 days later from septic shock with positive blood cultures. A limited postmortem examination was performed and biopsies were taken from bone marrow, liver, and spleen. Biopsies demonstrated abundant hemophagocytosis by the activated macrophage as stained by CD68. There was no evidence of residual CLL as demonstrated by the lack of lymphocytes which was confirmed by the negative staining of CD79a. Chemotherapy appears to be responsible for the development of HLH in this patient. This is the second reported case of HLH developing after a rituximab-containing chemotherapy.

Solitary fibrous tumor of small bowel mesentery: a case report and review of the literature.

Solitary fibrous tumor is an extremely rare clinical entity, most commonly manifested in the pleura and peritoneum. An increasing number of cases are found to occur at extrapleural sites. We report a case of solitary fibrous tumor arising from the mesentery of the small intestine. A 53-year-old man presented with a large abdominal mass which was initially thought to be a gastrointestinal stromal tumor. The tumor was successfully removed surgically and the diagnosis of solitary fibrous tumor was confirmed on immunohistochemical analysis. To our knowledge this is the first reported case of a solitary fibrous tumor arising from the small bowel mesentery.