Benign Metastasizing Leiomyoma of the Uterus with Pulmonary and Bone Metastases.

Benign metastasizing leiomyoma (BML) is a rare spindle cell neoplasm seen in middle-aged women who have a history of leiomyoma of the uterus. The most common sites of metastases are the lungs; however, other sites of spread have been documented. These tumors by definition have no malignant features on histology and tend to be estrogen and progesterone positive. We present a middle-aged woman who was incidentally found to have multiple pulmonary nodules and a mass on her sternum after she was involved in a motor vehicle accident. She had a history of uterine leiomyoma and had undergone a hysterectomy ten years prior to the accident. Biopsies were performed of the lung nodules and sternum mass and compared to her hysterectomy specimen, and they were identical, and hence, she was diagnosed with BML. Due to the growing tumor of her sternum, she was started on tamoxifen with stability of her tumors. These tumors, since they are benign, tend to have an indolent course. However, in the instances that treatment is warranted, options include surgery or antiestrogen therapy. We will be discussing the pathogenesis, histological findings, and treatment options of this rare condition. Our case is unique because BML in general do not tend to spread to multiple organs and tend to be limited to one site of disease.

Extramedullary Solitary Plasmacytoma with Anaplastic Features of the Nasopharynx.

Extramedullary plasmacytomas (ESPs) are rare forms of plasma cell dyscrasias and usually are seen in the upper aerodigestive tract. ESPs with anaplastic features are extremely rare, and no treatment guidelines exist. We present a 75-year-old gentleman presented with left nasal blockage, and on examination, a polypoid left nasal mass was seen. He was, then, referred to ENT after a CT scan revealed a mass in the left nasopharynx for a biopsy. The preliminary reports suggested a high-grade lymphoma; however, after further testing, it was revealed to be an anaplastic plasma cell neoplasm. PET scan, bone marrow biopsy, serum and urine protein electropheresis, serum immunofixation and light chains were all unremarkable for systemic disease. Differential diagnosis included plasmablastic lymphoma, NK/T cell lymphoma-nasal type, and squamous cell cancers of the head and neck. He was treated with radiation alone given his comorbidities. Given there are no treatment guidelines, we would like to highlight this rare case and discuss different potential management options such as radiation, chemotherapy, surgery, or a combination of different modalities.

A Rare Case of Chronic Myelogenous Leukemia Presenting as T-Cell Lymphoblastic Crisis.

Chronic Myelogenous Leukemia in blast crisis can manifest as either myeloid (more common) or lymphoid blast crisis. Most lymphoblastic crises are of B-cell lineage. T-cell blast crisis is extremely rare, with only a few reported cases. We present a case of a middle-aged man who was diagnosed with CML on peripheral blood and bone marrow biopsy. Because of a generalized lymphadenopathy noted at the time of diagnosis, a lymph node biopsy was also performed, which revealed a T-cell lymphoblastic leukemia/lymphoma, BCR/ABL1 positive, with clonal evolution. This is a very rare manifestation of CML in blast crisis with no standard treatment and with poor outcomes despite chemotherapy or allogeneic stem cell transplant. Given its rarity, it would be difficult to develop standard chemotherapy protocols. We believe the treatment for this condition should be similar to any lymphoid blast crisis. The patient was treated with induction chemotherapy (hyper-CVAD regimen) plus dasatinib for 3 cycles followed by sibling-donor allogeneic stem cell transplant and is currently on maintenance dasatinib and has minimal residual disease at this time.

Management of Well-differentiated Gastroenteropancreatic Neuroendocrine Tumors (GEPNETs): A Review.

PURPOSE: Neuroendocrine tumors (NETs) are heterogeneous tumors that arise from the neuroendocrine cells of the digestive tract and other organs, such as the lung, ovary, and thyroid glands. They can be well differentiated or poorly differentiated, and management of these tumors differs for each histologic subtype. We have performed a review of NETs and focused on management of well-differentiated gastroenteropancreatic neuroendocrine tumors (GEPNETs) and carcinoid syndrome. METHODS: A PubMed search was performed to obtain articles on the management of well-differentiated NETs. Using the key words neuroendocrine tumors, carcinoid, pNET, octreotide, somatostatin analogues, and radiolabeled therapy, we reviewed Phase II and III trials that were published over the past 30 years. We also reviewed guidelines from the European Neuroendocrine Tumor Society, North America Neuroendocrine Tumor Society, and National Comprehensive Cancer Network in our search. FINDINGS: NETs are usually slow-growing tumors that remain asymptomatic for a long duration and can be either nonfunctioning or functioning. Surgical resection is recommended for locoregional disease, impending obstruction, symptom control, and advanced disease. Nonsurgical treatment options include somatostatin analogues (SSAs), multikinase inhibitors, targeted therapy, chemotherapy, and radiolabeled SSAs. Carcinoid syndrome is mainly treated with SSAs. IMPLICATIONS: Although GEPNETs are slow-growing tumors, most patients are diagnosed with metastatic disease, and therefore it is important that the management of each patient be discussed in a multidisciplinary setting to optimize the treatment strategy. Patients should be considered for clinical trials and refractory cases referred to a specialty center.

Use of molecular studies for treatment of metastatic pleomorphic large cell pancreatic cancers-a novel strategy.

Pleomorphic large cell pancreatic cancer is a rare and more aggressive variant with no proven treatment in the metastatic setting. It constitutes about 1% of the total pancreatic cancer cases. In the absence of any standard of care, we aim to increase awareness amongst clinical practitioners that molecular level testing, using immunohistochemistry, next-generation sequencing and chromogenic in-situ hybridization can help in making chemotherapeutic decisions for this variant of pancreatic cancer. We present a 50-year-old male who presented to our hospital complaining of persistent abdominal pain. CT scan revealed a pancreatic tail mass that was invading the splenic flexure causing high-grade obstruction. There was evidence of peritoneal studding. He underwent exploratory laparotomy with biopsy of the pancreatic mass and omentum which revealed metastatic undifferentiated pleomorphic large cell pancreatic cancer. Since there is no proven treatment for this particular entity, his specimen was sent for molecular testing. The molecular studies revealed positive mutations of TLE3 gene, EGFR, KRAS, PD1 gene, TP53 and TOP2A gene. The tumor was found to be sensitive to gemcitabine, paclitaxel, docetaxel, temozolamide, dacarbazine and doxorubicin. He was initiated on gemcitabine and nab-paclitaxel. The patient was treated based on these recommendations. The patient completed 5 cycles of gemcitabine and nab-paclitaxel. Treatment had to be held because of gemcitabine induced hemolytic uremic syndrome. Serial CT scans have shown stable disease and currently it has been 10 months since his diagnosis. Molecular level testing can be an important instrument in not only diagnosing but also be an important aid in deciding about the chemotherapeutic agents to be used in cases of metastatic pleomorphic large cell pancreatic cancer. Availability a knowledge of the novel tools like immunohistochemistry, next-generation sequencing and chromogenic in-situ hybridization can be prudent and treating some rare forms of pancreatic cancer as in this patient.

WITHDRAWN: A Unique Case of Perivascular Epithelioid Cell Tumor Associated With Birt-Hogg-Dube Syndrome.

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Spine and scapular pain: an unusual presentation of colon adenocarcinoma.

We are presenting an elderly Caucasian woman who was admitted to our hospital for severe lower back pain and scapular pain for 8 days. She had a history of spinal stenosis and we believed this was the aetiology of her pain. However, her pain was not subsiding and hence an MRI spine was performed. It revealed spinal stenosis and abnormal signal in the lower thoracic and lumbar vertebrae. Hence a bone biopsy was performed that came back as metastatic adenocarcinoma. She underwent a bone scan and revealed diffuse disease in thoracic, lumbar, sacral spine as well as third and 10th ribs. Her CT chest/abdomen/pelvis revealed no primary tumour. A mass was detected on her colonscopy, and pathology revealed adenocarcinoma. She underwent palliative radiation and one cycle of chemotherapy. She was discharged to home hospice care. It is very rare to have osseous metastases as the only manifestation of colon cancer.

Post-transfusion purpura: a rare and life-threatening aetiology of thrombocytopenia.

We present a middle-aged man with history of lung adenocarcinoma, who was admitted with massive haemoptysis secondary to severe thrombocytopenia. Two weeks prior he was started on enoxaparin for a newly diagnosed pulmonary embolus and at that time required blood transfusions for anaemia. Our initial diagnosis was heparin-induced thrombocytopenia. His platelet count, however, did not improve despite receiving argatroban and platelet transfusions. Hence, we suspected post-transfusion purpura (PTP) and started him on intravenous immunoglobulin which brought his platelet count to normal levels. The serotonin-release assay was negative and platelet-antibody test was positive confirming PTP as our diagnosis. The patient eventually was transferred to hospice care because of the advanced stage lung cancer and died of respiratory failure.

Bacteroides fragilis endocarditis in a patient with Crohn's disease.

Bacteroides fragilis is an uncommon cause of endocarditis and its occurrence in Crohn's disease has never been reported. We present a case of a B fragilis bacteraemia and endocarditis caused by seeding of left ventricular thrombus formed secondary to severe left ventricular dysfunction. A 44-years-old man with a history of persistent bloody diarrhoea for many years presented with 1-month duration of generalised weakness, malaise, fever and chills. The patient also developed right foot pain associated with cyanotic discolouration. On examination, he was cachectic and his right foot was pulseless, cold and blue in colour. Echocardiogram showed three intraventricular echo densities. Colonoscopy revealed multiple fistulous openings and blood cultures grew B fragilis. He was treated with intravenous metronidazole and underwent a proctocolectomy with ileostomy. Biopsy of the specimen confirmed Crohn's disease. This case emphasises the importance of identifying anaerobic bacteria as an uncommon but important cause of endocarditis and recognise likely source.