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.
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.
Tumor lysis syndrome (TLS) is a life-threatening condition which could result in electrolyte imbalances, acute renal failure, seizures, arrhythmias and sudden death. It is mostly seen after the initiation of chemotherapy in hematological malignancies such as Burkitt's lymphoma and other Non-Hodgkin's Lymphoma. Solid tumors are rarely associated with Tumor Lysis Syndrome. TLS prior to the initiation of chemotherapy is known as spontaneous TLS, of which most of the literature involves hematological malignancies. There have been only a handful of case reports of spontaneous TLS occurring in solid tumors. We are presenting a patient who was newly diagnosed with metastatic small cell cancer of lung who developed spontaneous TLS. To our knowledge this is the first reported case of spontaneous TLS in small cell cancer of the lung. We will also review the literature on spontaneous TLS in solid tumors and whether as clinicians we can identify high-risk patients. Prompt diagnosis is essential in order to initiate necessary prophylactic precautions. After reviewing the few case reports it appears bulky tumors, site of metastases, prior renal functions may predict risk of suffering from TLS in solid tumors.
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.
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