An Unusual Cause of Adrenal Mass in Neurofibromatosis Type 1: Malignant Peripheral Nerve Sheath Tumor.

A malignant peripheral nerve sheath tumor (MPNST) is an aggressive tumor that can arise from the malignant transformation of benign neurofibromas in patients with neurofibromatosis type 1 (NF1). MPNST occurs in 2% of patients with NF1, contributing to significant mortality in these patients. Here, we report the case of a 67-year-old female with a known history of neurofibromatosis type 1 who was referred to general surgery after the discovery of a large left-sided adrenal mass on CT imaging five months earlier. Lab workup revealed elevated urine catecholamines, concerning pheochromocytoma. As pheochromocytoma is also common in those with NF-1, appropriate medical management followed by surgical resection was performed. The final pathology report revealed an MPNST.

Maintained Complete Response and Long-Term Survival in Epidermal Growth Factor Receptor Mutated Metastatic Non-Small Cell Lung Cancer with Erlotinib.

The prognosis of advanced non-small cell lung cancer (NSCLC) has significantly improved for certain patients with the development of epidermal growth factor receptor tyrosine kinase inhibitors (EGFR-TKIs). However, metastatic NSCLC patients with long-term survival are still rare. Our 66-year-old male patient was admitted to the hospital for treatment of pneumonia. A chest CT scan done revealed a left upper lobe mass; computed tomography (CT)-guided fine-needle aspiration (FNA) was done in 2010 revealing adenocarcinoma. A staging positron emission tomography (PET) scan did not reveal evidence of metastatic disease. He underwent left upper lobectomy and the pathologic stage was IB, moderately differentiated adenocarcinoma with positive angiolymphatic invasion. He was offered adjuvant systemic therapy, but he opted for surveillance. In 2012, a CT scan showed disease recurrence in the left upper lobe, which was confirmed with a biopsy. He was deemed non-surgical by thoracic oncology. Systemic therapy was initiated with carboplatin/pemetrexed and Avastin; after four cycles of treatment, the CT scan showed stable disease. Mutation analysis sent before chemotherapy revealed EGFR mutation for which chemotherapy was stopped and he was started on switch maintenance with erlotinib 150 mg in October 2012, then the dose was reduced to 100 mg secondary to grades 2-3 acneiform rash. Follow-up CT scans in January 2016 showed complete remission, which is maintained with no evidence of disease as of today. Non-small cell lung cancer (NSCLC) remains the leading cause of cancer-related mortality in the United States. Surgical excision is the standard treatment for stage I disease. Despite the long-term survival without adjuvant therapy, the disease recurrence rate ranges between 27% and 38% after resection. Different histologic subtypes vary in pathologic and molecular features, leading to differences in treatment and prognosis. In the adenocarcinoma subtype, five-year progression-free survival in patients with EGFR mutation treated with an EGFR-TKI is 14.6% as compared to less than 5% in unselected patients with distant-stage NSCLC. The association between exon 19 deletions, which represent about 45% of overall EGFR mutations and half of the sensitizing ones, and prolonged survival in patients with advanced NSCLC treated with EGFR-TKIs has been reported by several groups. Our case reports long-term survival in a patient with EGFR mutation-positive NSCLC with no evidence of disease for eight years since he started erlotinib treatment. Is there an option to discontinue maintenance erlotinib at this point? The answer to this question is not known, but this is a remarkably maintained response that is a good area to study patient's characteristics leading to differences in response.

A Rare Case of Small Bowel Extramedullary Plasmacytomas Presenting With Intestinal Obstruction.

Extramedullary plasmacytoma (EMP) is a plasma cell disorder involving soft tissues in the absence of clonal bone marrow involvement or destructive bone lesions. When present in the gastrointestinal (GI) tract, and specifically the small intestine, it can cause a wide range of symptoms including GI bleeding, obstruction, and abdominal pain. The diagnosis is challenging, as it can hold an indolent course, and is infrequently encountered in clinical practice. Diagnosis requires biopsy of the involved organ, which can be obtained during surgery or endoscopy, and other workup to rule out systemic disease and bone marrow involvement. Treatment depends on the primary site of disease involvement and the presence of other features of systemic disease. We report a case of multiple small bowel plasmacytomas in a 51-year-old female who presented with small bowel obstruction. She eventually underwent surgical resection and is currently on chemotherapy awaiting stem cell transplant.

Anti-PD-1 therapy using cemiplimab for advanced cutaneous squamous cell carcinoma in HIV patient: A case report.

This is a case of a 60-year-old man living with HIV who presented with advanced cutaneous squamous cell carcinoma. After workup, medical and surgical treatment, and disease recurrence, he achieved a complete response with no unexpected toxicities after immunotherapy with cemiplimab.

Tension Pneumothorax: Is it Sarcoma or Pazopanib?

Synovial sarcomas are rare malignant tumors that originate from primitive pluripotent mesenchymal stem cells that look similar to the developing synovium, but are histologically unrelated to it. Sarcomas commonly metastasize to the lungs and surrounding pleura, with a documented incidence as high as 85% for pleural-based metastases. The incidence of spontaneous pneumothorax in patients with sarcomas is only 1.9%, with synovial sarcoma being the third most common type of sarcoma associated with pneumothorax. While surgical resection is usually the treatment for localized primary synovial cell sarcoma, metastatic disease requires systemic therapy, mainly chemotherapy. Failure of chemotherapy calls for the use of targeted therapeutic agents such as pazopanib. Pazopanib has been linked to the incidence of spontaneous pneumothorax in previous case studies. However, primary research fails to establish a statistically significant causal association. Research shows that pneumothorax can result from lung metastases independent of therapeutic side effects. We report a case of synovial sarcoma of trapezius origin with secondary lung metastases, and development of pneumothorax after pazopanib treatment. We discuss the incidence of pneumothorax as a medication side effect versus independent effect of natural disease progression, and how this plays role in deciding when to continue using a medication in the face of complications.