Patient description: A 57-year-old man was admitted to the clinic because of weight loss and persistent dry cough 4 months ago. Chest computed topography showed bilateral multiple infiltrates in the upper lobes and thickened bronchial walls. There is a documented positive serum MPO-ANCA in his medical record. Transbronchial biopsy revealed necrotic granulomas with multinucleated giant cells and the Wegener's granulomatosis was diagnosed for him. He is treating with corticosteroid and cyclophosphamides since 4 months ago. His Birmingham Vasculitis Activity Score (BVAS) is above 4 since the beginning of his disease. His last physical exam and lab study was performed yesterday and showed the results bellow: A wellbeing, well-nourished man, non-icteric, cooperative and alert Weight: 73 kg Height: 177 BP: 120/80 HR: 90/min RR: 22/min Hgb: 13 g/dl WBC: 8000 /mm3 (Neutrophil: 2700/mm3) Plt: 300000 /ml AST: 40 U/L ALT: 56 U/L Alk P: 147 U/L Bill total: 1.2 mg/dl ESR: 120 mm/hr MPO-ANCA: 153 EU

Clinical trial eligibility criteria: eligible ages (years): 18.0-999.0,  Skin Squamous Cell Carcinoma Appendix Adenocarcinoma Rare Lesion Locally Advanced Malignant Neoplasm Locally Advanced Skin Squamous Cell Carcinoma Metastatic Malignant Neoplasm Metastatic Skin Squamous Cell Carcinoma Metastatic Small Intestinal Adenocarcinoma Rare Neoplastic Syndrome Refractory Malignant Neoplasm Stage IV Small Intestinal Adenocarcinoma AJCC v8 Unresectable Malignant Neoplasm Patients must be informed of the investigational nature of this study and must be willing to give written informed consent in accordance with institutional and federal guidelines. Patients must be able to comply with the requirements and assessments of the study protocol Must have histologically or cytologically documented rare tumor as defined per protocol that is metastatic or locally advanced and unresectable. Patients with locally advanced cutaneous squamous cell carcinoma that are technically resectable but in whom surgery is expected to lead to substantial function impairment or disfigurement are eligible Must be refractory or intolerant to standard lines of therapy Must have completed prior chemotherapy, immunotherapy, or radiation therapy at least 14 days prior to start of treatment and all toxicity must be resolved to Common Terminology for Adverse Events (CTCAE) v4.0 grade 1 (with the exception of CTCAE v4.0 grade 2 neuropathy) prior to start of treatment Presence of radiographically evaluable disease Eastern Cooperative Oncology Group (ECOG) performance status of =< 2 Tissue Parameters: a. Representative formalin-fixed paraffin-embedded (FFPE) tumor specimens in paraffin blocks (blocks are preferred) or at least 4 unstained slides, with an associated pathology report, for testing of tumor PD-L1 expression (tumor tissue from bone metastases is not evaluable for PD-L1 expression and is therefore not acceptable). b. Tumor tissue should be of good quality based on total and viable tumor content. Fine needle aspiration, brushing, cell pellet from pleural effusion, bone metastases, and lavage samples are not acceptable. For core-needle biopsy specimens, at least three cores should be submitted for evaluation. c. Patients who do not have tissue specimens meeting requirements must be willing to undergo a biopsy during the screening period Absolute neutrophil count (ANC) >= 1,000/mcL (obtained within 14 days prior to enrollment) Platelets >= 75,000/mcL (obtained within 14 days prior to enrollment) Hemoglobin >= 9 g/dL (obtained within 14 days prior to enrollment)  Presence of brain metastases (unless they have been adequately treated with radiotherapy or surgery and stable for at least 30 days prior to enrollment provided patient is neurologically asymptomatic and without corticosteroid treatment for at least 7 days prior to enrollment) Uncontrolled intercurrent illness including, but not limited to diabetes, hypertension, severe infection, severe malnutrition, unstable angina, class III-IV New York Heart Association (NYHA) congestive heart failure, ventricular arrhythmias, active ischemic heart disease, or myocardial infarction within 6 months prior to enrollment History of or evidence of retinal pathology on ophthalmologic examination that is considered a risk factor for neurosensory retinal detachment, central serous chorioretinopathy, retinal vein occlusion (RVO), or neovascular macular degeneration Patients will be excluded from study participation if they currently are known to have any of the following risk factors for RVO: a. Glaucoma with intraocular pressure >= 21 mmHg b. Grade >= 2 serum cholesterol c. Grade >= 2 hypertriglyceridemia d. Grade >= 2 or symptomatic hyperglycemia (fasting) e. Grade >= 2 uncontrolled hypertension (patients with a history of hypertension controlled with anti-hypertensive medication to grade =< 1 are eligible) Active malignancy (other than colorectal carcinoma [CRC]) or a history of prior malignancy within the past 3 years. Adequately treated basal cell or squamous cell skin cancer, in situ cervical cancer, ductal carcinoma in situ, other low grade lesions such as incidental appendix carcinoid, or any other cancer from which the patient has been disease and treatment free for two years are allowed. Prostate cancer patients on active surveillance are eligible Pregnant or nursing patients due to risk of fetal or nursing infant harm. Women/men of reproductive potential who do not agree to use an effective contraceptive method while on study and for at least 6 months after study treatment related to study medication (any cancer immunotherapy including CD137 agonists, anti-PD-1, anti-PD-L1, or anti-CTLA4 or any MEK or ERK inhibitor) Left ventricular ejection fraction (LVEF) < institutional lower limit of normal or < 50% History or risk of autoimmune disease, including but not limited to systemic lupus erythematosus, rheumatoid arthritis, inflammatory bowel disease, vascular thrombosis associated with antiphospholipid syndrome, Wegener's granulomatosis, Sjogren's syndrome, Bell's palsy, Guillain-Barre syndrome, multiple sclerosis, autoimmune thyroid disease, vasculitis, or glomerulonephritis a. Patients with a history of autoimmune hypothyroidism on a stable dose of thyroid replacement hormone may be eligible. b. Patients with controlled type 1 diabetes mellitus on a stable insulin regimen may be eligible. c. Patients with eczema, psoriasis, lichen simplex chronicus of vitiligo with dermatologic manifestations only (e.g., patients with psoriatic arthritis would be excluded) are permitted provided that they meet the following conditions Patients with psoriasis must have a baseline ophthalmologic exam to rule out ocular manifestations